Latest neuropathy articles

"Latest neuropathy articles are scanned daily from major neurology journals and updated here"

Assessing subclinical tactual deficits in the hand function of diabetic blind persons at risk for peripheral neuropathy. Related Articles

Assessing subclinical tactual deficits in the hand function of diabetic blind persons at risk for peripheral neuropathy.

Arch Phys Med Rehabil. 2007 Dec;88(12):1662-72

Latest neuropathy articles Authors: Travieso D, Lederman SJ

OBJECTIVE: To assess subclinical impairments in tactual hand function produced by diabetes mellitus in late-blind adults with diabetic retinopathy. DESIGN: The survey compares diabetic blind with nondiabetic blind and blindfolded sighted controls in terms of their performance on a battery of tests that assess tactual hand function. SETTING: Subjects were evaluated at their rehabilitation program center in Madrid. PARTICIPANTS: Nine (referred) diabetic blind subjects affected by diabetic retinopathy versus 10 (referred) nondiabetic blind subjects versus 10 blindfolded sighted volunteers, all right-handed and matched for age. Subjects were referred by the training professionals of the rehabilitation program center and asked to volunteer. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Cutaneous force and spatial resolution thresholds, haptic psychophysical functions for perceived roughness, weight, and size, and both accuracy and response times for haptic classification of 3-dimensional common objects. Measures of joint mobility, muscular strength, and motor dexterity were also included. RESULTS: The diabetic blind performed significantly poorer than the controls in terms of force sensitivity (distal and proximal finger pads, and palm), spatial resolution (distal finger pad only), motor dexterity, perceived roughness, and finally, haptic object classification response times for texture-diagnostic objects. CONCLUSIONS: Subclinical disturbances in the tactual hand function of the diabetic blind subjects were only documented in perceptual and motor tasks for which cutaneous, as opposed to kinesthetic, information was particularly relevant.

Latest neuropathy articles PMID: 18047883 [PubMed - in process]A hypothesis: can erythropoietin administration affect the severity of retinopathy in diabetic patients with renal failure? Related Articles

A hypothesis: can erythropoietin administration affect the severity of retinopathy in diabetic patients with renal failure?

Am J Med Sci. 2007 Oct;334(4):260-4

Latest neuropathy articles Authors: Diskin CJ, Stokes TJ, Dansby LM, Radcliff L, Carter TB

BACKGROUND: Before the clinical availability of erythropoietin, diabetic retinopathy was known to stabilize on dialysis. Recently erythropoietin has been shown to be a potent angiogenic factor. Therefore, we chose to examine whether severity and progression of diabetic retinopathy has been accelerated by the administration of recombinant erythropoietin to patients with chronic renal failure. METHODS: Records of the patients followed by the Hypertension Nephrology, Dialysis, and Transplantation Clinic, the regional nephrology referral center for Eastern Alabama, from 1982 through 2005 were reviewed. Funduscopic examination at the time of ESRD was ranked according to the proposed international scale for severity of clinical diabetic retinopathy. Forty-five patients from the era before the availability of erythropoietin were matched to 45 patients from 2002 to 2004 who had been given erythropoietin but had similar prevalence of proliferative retinopathy, neuropathy, and years of diabetes before the onset of end-stage renal disease. Progression of retinopathy was compared according to multivariate analysis with 2-tailed Pearson correlation coefficient. RESULTS: There was significantly greater deterioration of retinopathy at 1 year in the patients who had received erythropoietin (P = 0.004). Although the presence of retinopathy at ESRD correlated with known traditional risk factors such as years of diabetes, age, and serum cholesterol, the deterioration of retinopathy after the initiation of hemodialysis correlated only with hematocrit (P = 0.042) and most significantly total dose of erythropoietin (P = 0.001). CONCLUSIONS: The prevalence and severity of proliferative retinopathy appear to have increased and are most closely associated with the erythropoietin dosing.

Latest neuropathy articles PMID: 18030182 [PubMed - in process]Percutaneous endovascular repair of ruptured abdominal aortic aneurysms. Related Articles

Percutaneous endovascular repair of ruptured abdominal aortic aneurysms.

Arch Surg. 2007 Nov;142(11):1049-52

Latest neuropathy articles Authors: Najjar SF, Mueller KH, Ujiki MB, Morasch MD, Matsumura JS, Eskandari MK

HYPOTHESIS: Percutaneous endovascular repair of ruptured abdominal aortic aneurysms (RAAAs) has better outcomes than traditional open surgical repair. DESIGN: Single-center retrospective review. SETTING: University hospital tertiary referral center. PATIENTS: Thirty-seven RAAAs treated using endovascular repair (n = 15) or open surgery (n = 22). INTERVENTIONS: From January 1, 2000, through December 31, 2005, 15 RAAAs were treated with endovascular stent graft exclusion using commercially available systems. Twenty-two other patients undergoing standard open surgical repair during the same interval comprised a control group for comparison. MAIN OUTCOME MEASURES: Early outcomes of percutaneous endovascular repair of RAAAs. RESULTS: Among the endovascular group, the mean +/- SD age was 73 +/- 9.8 years, 86.6% were men (n = 13), and 20.0% had a preoperative systolic blood pressure of 80 mm Hg or lower (n = 3). An entirely percutaneous procedure was performed in the final 11 patients using arterial closure systems. Technical success of attempted endovascular exclusion was 100.0%. The mean +/- SD procedure time (107 +/- 30 minutes), transfusion requirements (6.6 +/- 4.7 U), and length of stay (3.0 +/- 6.8 days) were statistically significantly reduced compared with open surgery. The 30-day mortality was 6.7% (1 of 15) compared with an open surgery 30-day mortality of 13.6% (3 of 22). No late complications (pseudoaneurysm, infection, lymphocele, or neuropathy) occurred after a completely percutaneous technique during a mean follow-up of 12 months. CONCLUSION: Percutaneous endovascular repair of RAAAs is a more expedient and less morbid alternative than open surgical repair.

Latest neuropathy articles PMID: 18025332 [PubMed - indexed for MEDLINE]Onodi cell mucocele: rare cause of optic compressive neuropathy. Related Articles

Onodi cell mucocele: rare cause of optic compressive neuropathy.

Arch Otolaryngol Head Neck Surg. 2007 Nov;133(11):1153-6

Latest neuropathy articles Authors: Toh ST, Lee JC

Latest neuropathy articles PMID: 18025322 [PubMed - in process]Lessons from the ischemic optic neuropathy decompression trial: a decade later. Related Articles

Lessons from the ischemic optic neuropathy decompression trial: a decade later.

Arch Ophthalmol. 2007 Nov;125(11):1570-1

Latest neuropathy articles Authors: Levin LA

Latest neuropathy articles PMID: 17998522 [PubMed - in process]The ischemic optic neuropathy decompression trial. Related Articles

The ischemic optic neuropathy decompression trial.

Arch Ophthalmol. 2007 Nov;125(11):1568-70

Latest neuropathy articles Authors: Newman NJ

Latest neuropathy articles PMID: 17998521 [PubMed - in process]Midterm results with a bipolar radial head prosthesis: radiographic evidence of loosening at the bone-cement interface. Related Articles

Midterm results with a bipolar radial head prosthesis: radiographic evidence of loosening at the bone-cement interface.

J Bone Joint Surg Am. 2007 Nov;89(11):2469-76

Latest neuropathy articles Authors: Popovic N, Lemaire R, Georis P, Gillet P

BACKGROUND: Metal prostheses are useful for restoring elbow and forearm stability when the radial head cannot be fixed after a fracture. Because the anatomy of the radial head is difficult to reproduce with a prosthesis, two different options have been proposed: a bipolar prosthesis with a fixed stem and a mobile head, and a monoblock prosthesis with a smooth stem that is intentionally fixed loosely in the neck of the radius. One concern with a fixed-stem implant with a mobile head has been the risk of osteolysis. The purpose of this study was to evaluate radiographic changes reflecting or suggesting progressive osteolysis in patients with a bipolar radial head prosthesis. METHODS: The functional and radiographic outcomes following treatment of fifty-one comminuted fractures of the radial head with a bipolar radial head prosthesis in fifty-one consecutive patients were evaluated at a mean of 8.4 years postoperatively. There were eleven isolated comminuted fractures involving the entire radial head. Thirty-four fractures were associated with a posterior elbow dislocation, and six patients had a posterior Monteggia lesion. RESULTS: According to the Mayo Elbow Performance Index, fourteen elbows were graded as excellent; twenty-five, as good; nine, as fair; and three, as poor. Radiographic changes reflecting or suggesting progressive osteolysis were present in thirty-seven patients. Complications occurred in ten patients, but only one underwent surgical treatment, for an ulnar neuropathy. CONCLUSIONS: Although satisfactory midterm functional results were achieved in thirty-nine of the fifty-one patients, the high prevalence of adverse radiographic changes suggesting periprosthetic osteolysis should alert clinicians to this possible drawback of the use of bipolar radial head prostheses, especially in young and/or active patients.

Latest neuropathy articles PMID: 17974891 [PubMed - in process]Multifocal motor neuropathy: the diagnostic spectrum and response to treatment. Related Articles

Multifocal motor neuropathy: the diagnostic spectrum and response to treatment.

Neurology. 2007 Oct 23;69(17):1680-7

Latest neuropathy articles Authors: Slee M, Selvan A, Donaghy M

OBJECTIVE: To define the clinical spectrum in a large cohort of patients with multifocal motor neuropathy (MMN) and the effectiveness of IVIg treatment. We also test two neurophysiologic criteria for conduction block (CB) for relevance to treatment responsiveness. METHODS: Retrospective case cohort analysis of 47 patients with MMN followed for up to 12 years. RESULTS: A total of 32 (70%) had an upper-limb onset with most showing clinical features of conduction block: weakened but non-wasted muscles (67%) and differential weakness across muscles supplied by a common terminal motor nerve (54%). Differential weakness of finger extension was a characteristic early sign. Application of consensus criteria for definite CB would have denied a trial of treatment to 6 patients with a typical phenotype compared with new criteria. No association was found between CB and presence of anti-GM1 ganglioside antibody. A total of 24 (51%) patients were treated with IVIg, which was associated with a marked initial improvement in self-reported disability in most patients. The magnitude of initial disability improvement was not sustained in all patients over time. However, the majority of treated patients reported significantly less disability at last follow-up than prior to treatment. Patients converted to a domiciliary IVIg program maintained function at least as well as hospital treated patients. CONCLUSION: The importance of the clinical phenotype of multifocal motor neuropathy (MMN) is emphasized. Neither conduction block (CB) nor antibody status is a reliable predictor of treatment responsiveness. Over-reliance upon consensus CB criteria can deny IVIg to patients with MMN who are treatment responsive.

Latest neuropathy articles PMID: 17954783 [PubMed - indexed for MEDLINE]The diabetic foot. Related Articles

The diabetic foot.

Surg Clin North Am. 2007 Oct;87(5):1149-77, x

Latest neuropathy articles Authors: Andersen CA, Roukis TS

Lower extremity complications are common in patients with diabetes and include neuropathy, ulceration, infection, and peripheral arterial disease. Foot infections represent the single most common cause of hospitalization and lower extremity amputation in persons with diabetes. Foot ulceration as a result of diabetic peripheral sensory neuropathy, rigid osseous deformities and soft-tissue contractures, repetitive trauma from unprotected ambulation, and peripheral vascular disease can all lead to a limb- or life-threatening infection. Antibiotic therapy for diabetic soft-tissue and osseous infections is usually inadequate as an isolated form of therapy. The mainstay of treatment involves well-planned surgical procedures, including extensive and properly placed incisions to perform adequate drainage of abscesses and débridement of necrotic soft-tissue and osseous structures from which deep cultures are obtained for specific antibiotic coverage. Initial antibiotic therapy should provide broad-spectrum coverage, and when final culture results are available the regimen should be revised to organism-specific coverage. Detailed and timely evaluation of the vascularity of the limb is paramount, followed by timely vascular reconstruction involving various endovascular and open surgical procedures to restore pulsatile flow to the full extent of the limb.

Latest neuropathy articles PMID: 17936480 [PubMed - in process]Proportion of individuals with low serum vitamin B-12 concentrations without macrocytosis is higher in the post folic acid fortification period than in the pre folic acid fortification period. Related Articles

Proportion of individuals with low serum vitamin B-12 concentrations without macrocytosis is higher in the post folic acid fortification period than in the pre folic acid fortification period.

Am J Clin Nutr. 2007 Oct;86(4):1187-92

Latest neuropathy articles Authors: Wyckoff KF, Ganji V

BACKGROUND: Large intakes of folic acid may delay the diagnosis of vitamin B-12 deficiency, which could lead to irreversible neuropathy. OBJECTIVE: The objective of this study was to determine whether the proportion of individuals with low serum vitamin B-12 without macrocytosis (undiagnosed vitamin B-12 deficiency) has increased in the post-folic acid fortification period. DESIGN: Individuals aged >or=19 y with low serum vitamin B-12 (<258 pmol/L) and mean corpuscular volume (MCV) measured between 1995 and 2004 were identified from medical records. The proportion and odds ratios of individuals with low serum vitamin B-12 without macrocytosis by sex, race, and age according to prefortification (n = 86), perifortification (n = 138), and postfortification (n = 409) periods were determined. RESULTS: MCV was significantly lower in the postfortification period (88.6 fL) than in the prefortification (94.4 fL; P < 0.001) and perifortification (90.6 fL; P = 0.007) periods. The proportion of subjects with low serum vitamin B-12 without macrocytosis was significantly higher in the postfortification (approximately 87%) and perifortification (approximately 85%) periods than in the prefortification period (approximately 70%; P < 0.001). In a sex-, race-, and age-adjusted analysis, the odds ratio for having low serum vitamin B-12 without macrocytosis was 3.0 (95% CI: 1.7, 5.2) in the postfortification period relative to the prefortification period. CONCLUSIONS: Subjects with low serum vitamin B-12 were likely to be without macrocytosis in the postfortification period. MCV should not be used as a marker for vitamin B-12 insufficiency. It is possible that folic acid fortification may have led to a correction of macrocytosis associated with vitamin B-12 insufficiency.

Latest neuropathy articles PMID: 17921401 [PubMed - indexed for MEDLINE]Recessive Twinkle mutations in early onset encephalopathy with mtDNA depletion. Related Articles

Recessive Twinkle mutations in early onset encephalopathy with mtDNA depletion.

Brain. 2007 Nov;130(Pt 11):3032-40

Latest neuropathy articles Authors: Hakonen AH, Isohanni P, Paetau A, Herva R, Suomalainen A, Lönnqvist T

Twinkle is a mitochondrial replicative helicase, the mutations of which have been associated with autosomal dominant progressive external ophthalmoplegia (adPEO), and recessively inherited infantile onset spinocerebellar ataxia (IOSCA). We report here a new phenotype in two siblings with compound heterozygous Twinkle mutations (A318T and Y508C), characterized by severe early onset encephalopathy and signs of liver involvement. The clinical manifestations included hypotonia, athetosis, sensory neuropathy, ataxia, hearing deficit, ophthalmoplegia, intractable epilepsy and elevation of serum transaminases. The liver showed mtDNA depletion, whereas the muscle mtDNA was only slightly affected. Alpers-Huttenlocher syndrome has previously been associated with mutations of polymerase gamma, a replicative polymerase of mtDNA. We show here that recessive mutations of the close functional partner of the polymerase, the Twinkle helicase, can also manifest as early encephalopathy with liver involvement, a phenotype reminiscent of Alpers syndrome, and are a new genetic cause underlying tissue-specific mtDNA depletion.

Latest neuropathy articles PMID: 17921179 [PubMed - in process]Complementary therapies and integrative oncology in lung cancer: ACCP evidence-based clinical practice guidelines (2nd edition). Related Articles

Complementary therapies and integrative oncology in lung cancer: ACCP evidence-based clinical practice guidelines (2nd edition).

Chest. 2007 Sep;132(3 Suppl):340S-354S

Latest neuropathy articles Authors: Cassileth BR, Deng GE, Gomez JE, Johnstone PA, Kumar N, Vickers AJ,

BACKGROUND: This chapter aims to differentiate between "alternative" therapies, often promoted falsely as viable options to mainstream lung cancer treatment, and complementary therapies, adjunctive, effective techniques that treat symptoms associated with cancer and its mainstream treatment, and to describe the evidence base for use of complementary therapies.Methods and design: A multidisciplinary panel of experts in oncology and integrative medicine evaluated the evidence for complementary (not alternative) therapies in the care of patients with lung cancer. Because few complementary modalities are geared to patients with only a single cancer diagnosis, symptom-control research conducted with other groups of patients with cancer was also included. Data on complementary therapies such as acupuncture, massage therapy, mind-body therapies, herbs and other botanicals, and exercise were evaluated. Recommendations were based on the strength of evidence and the risk-to-benefit ratio. RESULTS: Patients with lung and other poor-outlook cancers are particularly vulnerable to heavily promoted claims for unproved or disproved "alternatives." Inquiring about patients' use of these therapies should be routine because these practices may be harmful and can delay or impair treatment. Mind-body modalities and massage therapy can reduce anxiety, mood disturbance, and chronic pain. Acupuncture assists the control of pain and other side effects and helps reduce levels of pain medication required. Trials of acupuncture for chemotherapy-induced neuropathy and postthoracotomy pain show promising results. Herbal products and other dietary supplements should be evaluated for side effects and potential interactions with chemotherapy and other medications. CONCLUSIONS: Complementary therapies have an increasingly important role in the control of symptoms associated with cancer and cancer treatment.

Latest neuropathy articles PMID: 17873179 [PubMed - indexed for MEDLINE]Differences and similarities between atypical facial pain and trigeminal neuropathic pain. Related Articles

Differences and similarities between atypical facial pain and trigeminal neuropathic pain.

Neurology. 2007 Oct 2;69(14):1451-9

Latest neuropathy articles Authors: Forssell H, Tenovuo O, Silvoniemi P, Jääskeläinen SK

OBJECTIVE: To investigate contribution of neuropathic mechanisms to clinically diagnosed atypical facial pain (AFP) using neurophysiologic and thermal quantitative sensory testing (QST) and comparing findings in AFP with those in definite trigeminal neuropathic pain (TNP). METHODS: Twenty patients with AFP and 12 patients with TNP participated after thorough clinical diagnostic workup. All patients underwent blink reflex (BR) recordings, habituation of the BR, and (except one patient with TNP) thermal QST. The results were compared with the reference values of our laboratory for normality. RESULTS: Of the patients with AFP, 75% showed abnormal findings. The BR responses were abnormal in three (15%) AFP patients (in two patients, the findings were compatible with a peripheral neuropathy and in one with a brainstem lesion), and in seven (58%) TNP patients. Seven (35%) patients with AFP and four (33%) with TNP showed increased excitability of the BR in the form of deficient habituation. Thermal QST indicated abnormal small fiber function in 11 (55%) patients with AFP and in all patients with TNP tested. QST showed thermal hypoesthesia in 45% and warm allodynia in 10% of patients with AFP. In TNP, all findings indicated thermal hypoesthesia. Abnormalities in BR and thermal QST were less frequent in AFP than TNP, but when present, type and pattern of findings were similar in both conditions. CONCLUSIONS: Clinical diagnosis of atypical facial pain represents a heterogeneous entity and seems to form a continuum regarding the level and extent of neuropathic involvement. Without detailed neurophysiologic and quantitative sensory examinations, neuropathic cause of chronic orofacial pain may be overlooked.

Latest neuropathy articles PMID: 17909158 [PubMed - indexed for MEDLINE]Patterns of Guillain-Barre syndrome in children: results from a Mexican population. Related Articles

Patterns of Guillain-Barre syndrome in children: results from a Mexican population.

Neurology. 2007 Oct 23;69(17):1665-71

Latest neuropathy articles Authors: Nachamkin I, Barbosa PA, Ung H, Lobato C, Rivera AG, Rodriguez P, Briseno AG, Cordero LM, Perea LG, Perez JC, Ribera M, Veitch J, Fitzgerald C, Cornblath D, Pinto MR, Griffin JW, Willison HJ, Asbury AK, McKhann GM

BACKGROUND: Guillain-Barré syndrome (GBS) is an acute, immune-mediated flaccid paralysis frequently associated with Campylobacter infection. Of two predominant GBS subtypes, a demyelinating subtype (acute inflammatory demyelinative polyneuropathy [AIDP]) predominates in the United States and Europe, and axonal subtype (acute motor axonal neuropathy [AMAN]) is the predominant form in China. Previous clinical studies suggested that AMAN also occurs in Mexican children. The purpose of this study was to describe the subtypes of GBS in children from Mexico City. METHODS: We prospectively studied 121 children admitted to two pediatric hospitals in Mexico City from 1996 to 2002. Clinical histories were obtained, electrophysiologic studies were performed to determine GBS subtype, and microbiologic studies were performed. RESULTS: Of the 121 children, 46 had AMAN and 32 had AIDP. The male to female ratio was 1.3 for AMAN cases (mean age = 6.3) and 3.0 for AIDP cases (mean age = 7.0). There was a strong seasonal distribution of AMAN cases in July to September. Children with AMAN, but not AIDP, had worsening of illness during hospitalization as judged by peak severity scores. Vomiting was more likely in AIDP (28.1%) vs AMAN (6.5%) (p = 0.012) and diarrhea was more common in AMAN (32.6%) than AIDP (12.5%) (p = 0.06). IgG anti-GM1 antibody titers were higher in patients with AMAN vs AIDP (p = 0.067). Anti-GD1a antibodies were equally present in both groups. Anti GQ1b titers were higher in AMAN vs AIDP (p = 0.009). Campylobacter antibody responses were positive in 44.1% of patients with AMAN and 37.0% of patients with AIDP. Twenty patients (14 = AMAN, 6 = AIDP) had positive stool cultures for C jejuni. Two serotypes, HS:19 and HS:41, accounted for 6 of 10 Campylobacter isolates available for serotyping from these cases. CONCLUSIONS: This study confirms that acute motor axonal neuropathy is an important Guillain-Barré syndrome subtype in Mexican children, is associated with diarrhea, and occurs seasonally.

Latest neuropathy articles PMID: 17898327 [PubMed - indexed for MEDLINE]Denervation of skin in neuropathies: the sequence of axonal and Schwann cell changes in skin biopsies. Related Articles

Denervation of skin in neuropathies: the sequence of axonal and Schwann cell changes in skin biopsies.

Brain. 2007 Oct;130(Pt 10):2703-14

Latest neuropathy articles Authors: Ebenezer GJ, McArthur JC, Thomas D, Murinson B, Hauer P, Polydefkis M, Griffin JW

We compared the pathological changes in cutaneous axons and Schwann cells of individuals with nerve transection to the changes in patients with chronic neuropathies. Following axotomy there was segmentation of axons in the epidermis and dermis on the first day, and loss of axons from the skin was virtually complete by Day 11. Epidermal and small superficial dermal axons were lost before larger caliber and deeper dermal axons. Within the first 50 days following nerve transection, the denervated Schwann cells in the dermis were easily identified by their markers p75 and S100, but by 8 months they had largely disappeared. The chronic neuropathy patients had distally predominant fibre loss, with greater loss of epidermal and dermal fibres in the distal regions of the leg than proximal regions. Several patients had large axonal swellings, often alternating with axonal attenuation, even in regions with normal or nearly normal fibre densities. By electron microscopy the swellings contained accumulations of mitochondria and other particulate organelles as well as neurofilaments. These swellings are likely to represent predegenerative changes in sites of impaired axonal transport, and previous data indicate that the swellings presage fibre loss in the subsequent months. Some of the severely denervated regions had remaining Schwann cells, as judged by immunocytochemistry and by electron microscopy, but others lacked Schwann cells. By analogy with animal experiments, these regions are likely to have had more prolonged denervation. The distribution of axonal loss, the axonal swellings and the changes in Schwann cells all have implications for the design of clinical trials of agents intended to protect cutaneous innervation and to promote regeneration of cutaneous axons in peripheral neuropathies.

Latest neuropathy articles PMID: 17898011 [PubMed - indexed for MEDLINE]Distal truncation of KCC3 in non-French Canadian HMSN/ACC families. Related Articles

Distal truncation of KCC3 in non-French Canadian HMSN/ACC families.

Neurology. 2007 Sep 25;69(13):1350-5

Latest neuropathy articles Authors: Salin-Cantegrel A, Rivière JB, Dupré N, Charron FM, Shekarabi M, Karéméra L, Gaspar C, Horst J, Tekin M, Deda G, Krause A, Lippert MM, Willemsen MA, Jarrar R, Lapointe JY, Rouleau GA

BACKGROUND: Hereditary motor and sensory neuropathy with agenesis of the corpus callosum (HMSN/ACC) is a severe and progressive autosomal recessive polyneuropathy. Mutations in the potassium-chloride cotransporter 3 gene (KCC3) were identified as responsible for HMSN/ACC in the French Canadian (FC) population. In the present study, the authors were interested in finding new mutations in non-FC populations, assessing the activity of mutant proteins and refining genotype-phenotype correlations. METHODS: The authors screened KCC3 for mutations using direct sequencing in six non-FC HMSN/ACC families. They then assessed the functionality of the most common mutant protein using a flux assay in Xenopus laevis oocytes. RESULTS: The authors identified mutations in exon 22 of KCC3: a novel mutation (del + 2994-3003; E1015X) in one family, as well as a known mutation (3031C-->T; R1011X) found in five unrelated families and associated with two different haplotypes. The function of the cotransporter was abolished, although a limited amount of mutant proteins were correctly localized at the membrane. CONCLUSIONS: KCC3 mutations in exon 22 constitute a recurrent mutation site for hereditary motor and sensory neuropathy with agenesis of the corpus callosum (HMSN/ACC), regardless of ethnic origin, and are the most common cause of HMSN/ACC in the non-French Canadian (FC) families analyzed so far. Therefore, for genetic analysis, exon 22 screening should be prioritized in non-FC populations. Finally, the R1011X mutation leads to the abrogation of KCC3's function in Xenopus laevis oocytes, likely due to impaired transit of the cotransporter.

Latest neuropathy articles PMID: 17893295 [PubMed - indexed for MEDLINE]Bone marrow B-cell clonal expansion in type II mixed cryoglobulinaemia: association with nephritis. Related Articles

Bone marrow B-cell clonal expansion in type II mixed cryoglobulinaemia: association with nephritis.

Rheumatology (Oxford). 2007 Nov;46(11):1657-61

Latest neuropathy articles Authors: Quartuccio L, Fabris M, Salvin S, Isola M, Soldano F, Falleti E, Beltrami CA, De Re V, De Vita S

OBJECTIVES: To investigate the relationship between the pattern of bone marrow (BM) B-cell expansion and the clinical features of mixed cryoglobulinaemia (MC) syndrome. METHODS: Fifty-five patients with type II MC syndrome were analysed. Their median age was 64 yrs (range 24-82), the median disease duration was 6 yrs (range 1-26) and the mean follow-up after BM analysis was 2.65 yrs (s.d. = 1.33). Peripheral neuropathy was present in 33 patients (60%), nephritis in 14 (25.4%), skin ulcers in 14 (25.4%) and lymphoma or atypical lymphoproliferative disorder (LPD) in 17/55 (30.9%). Anti-HCV antibodies were found in 43/55 patients (78.2%). BM B-cell expansion was evaluated by a semi-nested PCR amplification of the V-D-J region of the IgH genes. RESULTS: A clonal B-cell expansion in the BM was found in 33/55 (60%) patients, while a polyclonal pattern in 22/55 (40%). A BM pattern of clonal B-cell expansion increased the risk of nephritis of about 10 times [odds ratio (OR) = 10.11, CI95%1.52-67.31], if compared to a polyclonal pattern. In contrast, the risk of skin ulcers was decreased in BM clonal cases (OR = 0.09, CI95%0.02-0.49). Overt lymphomas did not emerge from patients with BM monoclonal expansion (without clinical or histopathological features of lymphoproliferation; or with LPD) in a short-term, consistent with the finding that monoclonality was associated with nephritis and not with an underlying, not recognized lymphoma. CONCLUSION: BM clonal B-cell expansion is associated with nephritis in MC syndrome. Particular B-cell clones may be preferentially expanded and may play a pathogenic role in MC nephritis.

Latest neuropathy articles PMID: 17893101 [PubMed - in process]Prescribing amiodarone: an evidence-based review of clinical indications. Related Articles

Prescribing amiodarone: an evidence-based review of clinical indications.

JAMA. 2007 Sep 19;298(11):1312-22

Latest neuropathy articles Authors: Vassallo P, Trohman RG

CONTEXT: Although amiodarone is approved by the US Food and Drug Administration only for refractory ventricular arrhythmias, it is one of the most frequently prescribed antiarrhythmic medications in the United States. OBJECTIVE: To evaluate and synthesize evidence regarding optimal use of amiodarone for various arrhythmias. EVIDENCE ACQUISITION: Systematic search of MEDLINE to identify peer-reviewed clinical trials, randomized controlled trials, meta-analyses, and other studies with clinical pertinence. The search was limited to human-participant, English-language reports published between 1970 and 2007. Amiodarone was searched using the terms adverse effects, atrial fibrillation, atrial flutter, congestive heart failure, electrical storm, hypertrophic cardiomyopathy, implantable cardioverter-defibrillator, surgery, ventricular arrhythmia, ventricular fibrillation, and Wolff-Parkinson-White. Bibliographies of identified articles and guidelines from official societies were reviewed for additional references. Ninety-two identified studies met inclusion criteria and were included in the review. EVIDENCE SYNTHESIS: Amiodarone may have clinical value in patients with left ventricular dysfunction and heart failure as first-line treatment for atrial fibrillation, though other agents are available. Amiodarone is useful in acute management of sustained ventricular tachyarrythmias, regardless of hemodynamic stability. The only role for prophylactic amiodarone is in the perioperative period of cardiac surgery. Amiodarone may be effective as an adjunct to implantable cardioverter-defibrillator therapy to reduce number of shocks. However, amiodarone has a number of serious adverse effects, including corneal microdeposits (>90%), optic neuropathy/neuritis (< or =1%-2%), blue-gray skin discoloration (4%-9%), photosensitivity (25%-75%), hypothyroidism (6%), hyperthyroidism (0.9%-2%), pulmonary toxicity (1%-17%), peripheral neuropathy (0.3% annually), and hepatotoxicity (elevated enzyme levels, 15%-30%; hepatitis and cirrhosis, <3% [0.6% annually]). CONCLUSION: Amiodarone should be used with close follow-up in patients who are likely to derive the most benefit, namely those with atrial fibrillation and left ventricular dysfunction, those with acute sustained ventricular arrhythmias, those about to undergo cardiac surgery, and those with implantable cardioverter-defibrillators and symptomatic shocks.

Latest neuropathy articles PMID: 17878423 [PubMed - indexed for MEDLINE]Anterior ischemic optic neuropathy in patients younger than 50 years. Related Articles

Anterior ischemic optic neuropathy in patients younger than 50 years.

Am J Ophthalmol. 2007 Dec;144(6):953-60

Latest neuropathy articles Authors: Preechawat P, Bruce BB, Newman NJ, Biousse V

PURPOSE: To characterize anterior ischemic optic neuropathy (AION) in patients younger than 50 years. DESIGN: Retrospective study. METHODS: Records of all AION patients seen between 1989 and 2006 were reviewed. Patients younger than 50 years when initial visual loss occurred were included. RESULTS: Of 727 consecutive patients with AION, 169 (23%) were younger than 50 years (median, 43 years; range, 13 to 49 years; 58% men; 93% White). Involvement was unilateral in 59% of patients and bilateral in 41%. At least one cardiovascular risk factor was found in 74% of patients. Hypercoagulable states and vasculitis were found in 8%. An underlying small or anomalous optic disk was found in 92% of eyes (210/230). Isolated disk anomalies (without systemic risk factors) were present in 26% of eyes. Final visual acuities were 20/40 or better in 64% of eyes and 20/200 or worse in 22%. Among patients with bilateral involvement, final visual acuity was similar in the two eyes in 70% of patients. Anemia and type I diabetes were associated significantly with fellow eye involvement. Recurrent AION in the same eye occurred in 6% of patients. CONCLUSIONS: AION in younger patients is not uncommon and represents 23% of AION patients in a tertiary neuro-ophthalmic service. Except for giant cell arteritis, ocular and systemic risk factors and associated disorders are similar to those described in older AION patients. Younger AION patients have better visual acuity outcomes but a higher risk of fellow eye involvement than older AION patients.

Latest neuropathy articles PMID: 17854756 [PubMed - in process]Efficacy of gabapentin in the management of chemotherapy-induced peripheral neuropathy: a phase 3 randomized, double-blind, placebo-controlled, crossover trial (N00C3). Related Articles

Efficacy of gabapentin in the management of chemotherapy-induced peripheral neuropathy: a phase 3 randomized, double-blind, placebo-controlled, crossover trial (N00C3).

Cancer. 2007 Nov 1;110(9):2110-8

Latest neuropathy articles Authors: Rao RD, Michalak JC, Sloan JA, Loprinzi CL, Soori GS, Nikcevich DA, Warner DO, Novotny P, Kutteh LA, Wong GY,

BACKGROUND: The antiepileptic agent, gabapentin, has been demonstrated to relieve symptoms of peripheral neuropathy due to various etiologies. On the basis of these data, a multicenter, double-blind, placebo-controlled, crossover, randomized trial was conducted to evaluate the effect of gabapentin on symptoms of chemotherapy-induced peripheral neuropathy (CIPN). METHODS: Patients with symptomatic CIPN who complained of 'average' daily pain scores of either 1) >/=4 on a 0-10 numerical rating scale (NRS); or 2) >/=1 on the 0-3 Eastern Cooperative Oncology Group neuropathy scale (ENS) were eligible (higher numbers indicate greater severity of symptoms in both scales). Patients were randomized to receive gabapentin (target dose, 2700 mg) or placebo for 6 weeks. Crossover occurred after a 2-week washout period. CIPN-related symptoms were evaluated weekly by questionnaires. Statistical methods followed established methods for crossover designs, including Student t tests to compare average intrapatient differences between treatments and linear models to adjust for potential concomitant covariates. RESULTS: There were 115 patients who were randomly assigned to the treatment or control arm. Both groups were well matched by symptoms at study entry. Changes in symptom severity were statistically similar between the 2 groups during the study. Adverse events were mild and similar in both groups. CONCLUSIONS: This trial failed to demonstrate any benefit to using gabapentin to treat symptoms caused by CIPN.

Latest neuropathy articles PMID: 17853395 [PubMed - in process]Methanol poisoning in Tunisia: report of 16 cases. Related Articles

Methanol poisoning in Tunisia: report of 16 cases.

Clin Toxicol (Phila). 2007 Sep;45(6):717-20

Latest neuropathy articles Authors: Brahmi N, Blel Y, Abidi N, Kouraichi N, Thabet H, Hedhili A, Amamou M

Methanol poisoning continues to be a public health problem in Tunisia in spite of the different legislative measures. We report a series of 16 cases of methanol poisoning admitted to our Intensive Care Unit between December 2003 and April 2004. The patients' median age was 21.5 years (range 16 to 53 years) with a median SAPS II of 14 (range 12 to 84) and an APACHE II of 8 (range 6 to 36). The median latent period was 9.5 hours (range 4 to 24 hours) with a delay to medical consultation of 36 hours (range 6 to 48 hours), and a median serum methanol concentration of 1.4 g/L (range 0.19 to 3.62 g/L). Clinical signs included central nervous system symptoms (69%), gastrointestinal complaints (87%), visual disturbances (69%) and metabolic acidosis (94%). Three patients (19%) required mechanical ventilation because of deep coma or shock and died within 6 hours. Hemodialysis was performed in eleven patients (69%) because of visual disturbances and/or metabolic acidosis. One patient developed irreversible bilateral blindness and another unilateral blindness secondary to optic neuropathy. Statistical significant risk factors for the developing of visual disturbances were found to be the ingested quantity of methanol, the latent period, acidosis and serum methanol concentration on admission.

Latest neuropathy articles PMID: 17849250 [PubMed - indexed for MEDLINE]Background noise: the experience of chemotherapy-induced peripheral neuropathy. Related Articles

Background noise: the experience of chemotherapy-induced peripheral neuropathy.

Nurs Res. 2007 Sep-Oct;56(5):323-31

Latest neuropathy articles Authors: Bakitas MA

BACKGROUND: A substantial portion of the 1.3 million persons diagnosed annually with cancer receive neurotoxic chemotherapy that may produce distressful symptoms and changes in functional ability. However, little is known about the symptom experience and daily life effects of chemotherapy-induced peripheral neuropathy (CIPN). OBJECTIVE: To describe the CIPN symptom experience and the influence of symptoms on everyday life. METHODS: This was a qualitative, exploratory, interpretive, descriptive study with semistructured interviews. A purposive sample of 28 participants was recruited from a rural National Cancer Institute-designated comprehensive cancer center. Interviews were audiotaped, transcribed verbatim, and imported into Atlas.ti software. Content analysis and constant comparative method were used to analyze the data. RESULTS: Participants represented diversity in age (46-81 years), cancer type, time since diagnosis (3-198 months), neuropathy severity, and neurotoxic chemotherapy agents received. Content analysis yielded a rich, thick description of CIPN symptoms and the influence of the symptoms on functional ability and everyday life. Further interpretive analysis provided a description of the symptom experience through an overarching metaphor, Background Noise, and four major themes: (a) Becoming Aware; (b) Learning New Lyrics; (c) Functional, Emotional, and Social Role Cacophony; and (d) Learning to Live With It. Participants described significant physical limitations, emotional distress, and social role impairments due to CIPN. CONCLUSIONS: Having CIPN results in diverse symptom patterns and degrees of physical symptom distress from mild to severe, emotional distress, alterations in functional ability, and social role impairment. Comprehensive clinical and research measures are needed to assess the full spectrum of CIPN effects on everyday life.

Latest neuropathy articles PMID: 17846553 [PubMed - indexed for MEDLINE]Intraneural perineurioma: a systematic review with illustrative cases. Related Articles

Intraneural perineurioma: a systematic review with illustrative cases.

Arch Pathol Lab Med. 2007 Sep;131(9):1382-92

Latest neuropathy articles Authors: Boyanton BL, Jones JK, Shenaq SM, Hicks MJ, Bhattacharjee MB

CONTEXT: Intraneural perineurioma may be confused with other "onion bulb" Schwann cell entities (localized hypertrophic neuropathy, reactive/demyelinating processes, or inherited polyneuropathies of Charcot-Marie-Tooth/Dejerine Sottas) due to similar clinical, radiologic, and histologic features. Perineurial and Schwann cells can only be differentiated by ultrastructure and immunohistochemsitry. OBJECTIVE: To identify and summarize the clinicopathologic features of true cases of intraneural perineurioma from the English language literature. DATA SOURCES: A systematic review was performed on definitive intraneural perineuriomas identified through Medline. Baylor College of Medicine-affiliated hospitals' anatomic pathology databases yielded 2 illustrative intraneural perineurioma cases. STUDY SELECTION: Intraneural perineurioma inclusion criteria consisted of characteristic histology and confirmation of perineurial cell lineage by either immunohistochemistry (epithelial membrane antigen positive, S100 protein negative) and/or ultrastructural analysis (thin cytoplasmic processes with an incomplete basal lamina, poorly formed tight junctions, and pinocytotic vesicles). DATA EXTRACTION: Clinicopathologic data were extracted from all identified articles, with subsequent statistical analysis of the following parameters: age, sex, race, tumor location, tumor size, duration of symptoms prior to diagnosis, treatment modalities and outcomes measures, follow-up assessment for tumor recurrence and metastasis, clinical features (history of trauma, motor/sensory abnormalities, clinical/family history), and diagnostic workup (routine histology, immunohistochemistry, ultrastructural analysis, and molecular/cytogenetic characteristics). CONCLUSIONS: Intraneural perineurioma is a neoplastic proliferation of perineurial cells with unique immunohistochemistry and ultrastructural features, and it is distinct from other onion bulb Schwann cell-derived entities. Despite harboring molecular abnormalities of the long arm of chromosome 22, intraneural perineurioma has not been associated with neurofibromatosis. Intraneural perineurioma is a benign peripheral nerve sheath tumor that does not recur or metastasize.

Latest neuropathy articles PMID: 17824794 [PubMed - indexed for MEDLINE]Scapular winging: an unusual complication of bracing in idiopathic scoliosis. Related Articles

Scapular winging: an unusual complication of bracing in idiopathic scoliosis.

Clin Orthop Relat Res. 2007 Aug;461:258-61

Latest neuropathy articles Authors: Debeer P, Van Den Eede E, Moens P

We describe a 15-year-old girl who had winging of the right scapula develop after incorrect use of a thoracolumbar orthosis. The girl was treated for idiopathic scoliosis, but after 2 years of bracing, progressive scapular winging and diminished range of motion in the right shoulder was observed. The girl reported that the superior part of the brace frequently hooked under the tip of the right scapula. This resulted in complete neuropathy of the dorsal scapular nerve. When using a thoracolumbar orthosis in the treatment of children with scoliosis, physicians must consider potential compressive injuries to the dorsal scapular nerve.

Latest neuropathy articles PMID: 17806153 [PubMed - indexed for MEDLINE]Characterization of rodent models of HIV-gp120 and anti-retroviral-associated neuropathic pain. Related Articles

Characterization of rodent models of HIV-gp120 and anti-retroviral-associated neuropathic pain.

Brain. 2007 Oct;130(Pt 10):2688-702

Latest neuropathy articles Authors: Wallace VC, Blackbeard J, Segerdahl AR, Hasnie F, Pheby T, McMahon SB, Rice AS

A distal symmetrical sensory peripheral neuropathy is frequently observed in people living with Human Immunodeficiency Virus Type 1 (HIV-1). This neuropathy can be associated with viral infection alone, probably involving a role for the envelope glycoprotein gp120; or a drug-induced toxic neuropathy associated with the use of nucleoside analogue reverse transcriptase inhibitors as a component of highly active anti-retroviral therapy. In order to elucidate the mechanisms underlying drug-induced neuropathy in the context of HIV infection, we have characterized pathological events in the peripheral and central nervous system following systemic treatment with the anti-retroviral agent, ddC (Zalcitabine) with or without the concomitant delivery of HIV-gp120 to the rat sciatic nerve (gp120+ddC). Systemic ddC treatment alone is associated with a persistent mechanical hypersensitivity (33% decrease in limb withdrawal threshold) that when combined with perineural HIV-gp120 is exacerbated (48% decrease in threshold) and both treatments result in thigmotactic (anxiety-like) behaviour. Immunohistochemical studies revealed little ddC-associated alteration in DRG phenotype, as compared with known changes following perineural HIV-gp120. However, the chemokine CCL2 is significantly expressed in the DRG of rats treated with perineural HIV-gp120 and/or ddC and there is a reduction in intraepidermal nerve fibre density, comparable to that seen in herpes zoster infection. Moreover, a spinal gliosis is apparent at times of peak behavioural sensitivity that is exacerbated in gp120+ddC as compared to either treatment alone. Treatment with the microglial inhibitor, minocycline, is associated with delayed onset of hypersensitivity to mechanical stimuli in the gp120+ddC model and reversal of some measures of thigmotaxis. Finally, the hypersensitivity to mechanical stimuli was sensitive to systemic treatment with gabapentin, morphine and the cannabinoid WIN 55,212-2, but not with amitriptyline. These data suggests that both neuropathic pain models display many features of HIV- and anti-retroviral-related peripheral neuropathy. They therefore merit further investigation for the elucidation of underlying mechanisms and may prove useful for preclinical assessment of drugs for the treatment of HIV-related peripheral neuropathic pain.

Latest neuropathy articles PMID: 17761732 [PubMed - indexed for MEDLINE]Homoplasmy, heteroplasmy, and mitochondrial dystonia. Related Articles

Homoplasmy, heteroplasmy, and mitochondrial dystonia.

Neurology. 2007 Aug 28;69(9):911-6

Latest neuropathy articles Authors: McFarland R, Chinnery PF, Blakely EL, Schaefer AM, Morris AA, Foster SM, Tuppen HA, Ramesh V, Dorman PJ, Turnbull DM, Taylor RW

BACKGROUND: In clinical practice, mitochondrial disease is seldom considered until a variable combination of seizures, alteration in tone, muscle weakness, and developmental problems is evident. However, it is not uncommon for one symptom to occur in isolation and dominate the clinical phenotype. We report six patients from two families where dystonia was the principal clinical manifestation. A mitochondrial etiology was considered in each case because of the association of dystonia with other less prominent clinical features such as epilepsy. METHODS: Histochemical and biochemical analyses were undertaken in skeletal muscle biopsies from individuals in both families. Sequencing of skeletal muscle mtDNA was also performed and suspected mutations were quantified by hot last cycle PCR-RFLP or primer extension assay. Functional consequences of one of the mutations were investigated by measurement of steady state levels of mitochondrial tRNA. RESULTS: Two distinct mitochondrial pathologies were identified: a novel, homoplasmic mitochondrial tRNA(Cys) (MTTC) mutation and the primary, m.11778G>A Leber hereditary optic neuropathy (LHON) mutation. The mild nature of both mutations has permitted very high levels of mutated mtDNA to accumulate. Patients with the mutation in the MTTC gene have no wild type mtDNA detectable and although the LHON mutation is heteroplasmic in the patients we report, it is commonly observed to be homoplasmic. CONCLUSIONS: The mitochondrial etiology identified in these patients emphasizes the pathologic potential of homoplasmic mutations and has important implications for the investigation and genetic counseling of families where dystonia is the principal clinical feature. We advocate that mitochondrial disease should be given serious consideration in patients with familial, progressive dystonia, particularly when additional neurologic features such as epilepsy are present.

Latest neuropathy articles PMID: 17724295 [PubMed - indexed for MEDLINE]Volumetric brain changes in females with fragile X-associated tremor/ataxia syndrome (FXTAS). Related Articles

Volumetric brain changes in females with fragile X-associated tremor/ataxia syndrome (FXTAS).

Neurology. 2007 Aug 28;69(9):851-9

Latest neuropathy articles Authors: Adams JS, Adams PE, Nguyen D, Brunberg JA, Tassone F, Zhang W, Koldewyn K, Rivera SM, Grigsby J, Zhang L, DeCarli C, Hagerman PJ, Hagerman RJ

BACKGROUND: Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late-onset neurodegenerative disorder occurring in male and rare female carriers of a premutation expansion (55 to 200 CGG repeats) of the fragile X mental retardation 1 (FMR1) gene. METHODS: Volumetric MRI studies, clinical staging, cognitive testing, and molecular analysis were conducted in 15 female premutation carriers affected by FXTAS (age 59.5 +/- 10.3 years), 20 unaffected female carriers (43.3 +/- 11.2 years), 11 genetically normal female controls (51.0 +/- 10.3 years), 36 affected male carriers (65.0 +/- 5.6 years), 25 unaffected male carriers (53.5 +/- 12.5 years), and 39 male controls (58.0 +/- 15.0 years). Female and male carriers with FXTAS were matched on duration of disease. RESULTS: We found less pronounced reductions of cerebellar volume and a lower incidence of involvement (symmetric high T2 signal) of the middle cerebellar peduncles (MCP sign) in females affected by FXTAS (13%) compared with affected males (58%). We found reduced brain volumes and increased white matter disease associated with the presence of FXTAS in females compared with female controls. We also observed significant associations between reduced cerebellar volume and both increased severity of FXTAS symptoms and increased length of the CGG repeat expansion in male premutation carriers, but not in females. CONCLUSIONS: Females affected by fragile X-associated tremor/ataxia syndrome (FXTAS) demonstrated milder brain changes than affected males, although they showed a similar pattern of radiologic findings consistent with brain atrophy and white matter disease. FXTAS should be considered (by ordering fragile X DNA testing) in females who present with late-onset ataxia, action tremor, or neuropathy, particularly in those with a family history of mental retardation, autism, or premature ovarian failure.

Latest neuropathy articles PMID: 17724287 [PubMed - indexed for MEDLINE]Protective effects of cyclooxygenase-2 gene inactivation against peripheral nerve dysfunction and intraepidermal nerve fiber loss in experimental diabetes. Related Articles

Protective effects of cyclooxygenase-2 gene inactivation against peripheral nerve dysfunction and intraepidermal nerve fiber loss in experimental diabetes.

Diabetes. 2007 Dec;56(12):2997-3005

Latest neuropathy articles Authors: Kellogg AP, Wiggin TD, Larkin DD, Hayes JM, Stevens MJ, Pop-Busui R

OBJECTIVE: Activation of the cyclooxygenase (COX) pathway with secondary neurovascular deficits are implicated in the pathogenesis of experimental diabetic peripheral neuropathy (DPN). The aim of this study was to explore the interrelationships between hyperglycemia, activation of the COX-2 pathway, and oxidative stress and inflammation in mediating peripheral nerve dysfunction and whether COX-2 gene inactivation attenuates nerve fiber loss in long-term experimental diabetes. RESEARCH DESIGN AND METHODS: Motor and sensory digital nerve conduction velocities, sciatic nerve indexes of oxidative stress, prostaglandin content, markers of inflammation, and intraepidermal nerve fiber (IENF) density were measured after 6 months in control and diabetic COX-2-deficient (COX-2(-/-)) and littermate wild-type (COX-2(+/+)) mice. The effects of a selective COX-2 inhibitor, celecoxib, on these markers were also investigated in diabetic rats. RESULTS: Under normal conditions, there were no differences in blood glucose, peripheral nerve electrophysiology, markers of oxidative stress, inflammation, and IENF density between COX-2(+/+) and COX-2(-/-) mice. After 6 months, diabetic COX-2(+/+) mice experienced significant deterioration in nerve conduction velocities and IENF density and developed important signs of increased oxidative stress and inflammation compared with nondiabetic mice. Diabetic COX-2(-/-) mice were protected against functional and biochemical deficits of experimental DPN and against nerve fiber loss. In diabetic rats, selective COX-2 inhibition replicated this protection. CONCLUSIONS: These data suggest that selective COX-2 inhibition may be useful for preventing or delaying DPN.

Latest neuropathy articles PMID: 17720896 [PubMed - in process]Heart rate variability and heart rate turbulence in patients with type 2 diabetes mellitus with versus without cardiac autonomic neuropathy. Related Articles

Heart rate variability and heart rate turbulence in patients with type 2 diabetes mellitus with versus without cardiac autonomic neuropathy.

Am J Cardiol. 2007 Sep 1;100(5):890-3

Latest neuropathy articles Authors: Balcioğlu S, Arslan U, Türkoğlu S, Ozdemir M, Cengel A

Cardiac autonomic neuropathy (CAN) is an important complication of diabetes mellitus (DM) and confers an increased cardiovascular risk. The aim of this study was to disclose the place of heart rate (HR) variability and HR turbulence for the detection of CAN in patients with type 2 DM and no obvious heart disease. Ninety patients who were <75 years old and had type 2 DM for >/=2 years were studied. CAN was diagnosed with a battery of cardiovascular reflex tests and the degree of neuropathic involvement was graded by the Ewing score. Time-domain HR variability and HR turbulence parameters were assessed on 24-hour digital Holter recordings. Thirty-five patients were found to have CAN. The clinical characteristics of patients with and without CAN were similar, except that the mean duration of DM and the number of patients using insulin were significantly increased in the group with CAN. All time-domain HR variability parameters were significantly lower in the group with CAN. Of the 2 HR turbulence parameters studied, turbulence onset was similar but turbulence slope was significantly lower in the group with CAN. The Ewing score significantly correlated negatively with all HR variability parameters and turbulence slope, and among all, turbulence slope was the most strongly correlated (r = -0.617, p <0.01). Receiver-operating characteristics analysis revealed a sensitivity of 97% and a specificity of 71% at a turbulence slope cut-off value of 3.32 for the detection of CAN. In conclusion, time-domain HR variability and HR turbulence parameters, except turbulence, onset were found to be significantly depressed in patients with type 2 DM and CAN. Decreases in all these parameters were found to correlate significantly with degree of neuropathic involvement. The most strongly correlated parameter, turbulence slope, was found to be highly sensitive and specific for the detection of CAN at a cut-off value of 3.32.

Latest neuropathy articles PMID: 17719340 [PubMed - indexed for MEDLINE]MRI of the distal biceps femoris muscle: normal anatomy, variants, and association with common peroneal entrapment neuropathy. Related Articles

MRI of the distal biceps femoris muscle: normal anatomy, variants, and association with common peroneal entrapment neuropathy.

AJR Am J Roentgenol. 2007 Sep;189(3):549-55

Latest neuropathy articles Authors: Vieira RL, Rosenberg ZS, Kiprovski K

OBJECTIVE: The objectives of our study were to describe the previously unreported normal MR anatomy of the distal biceps femoris muscle and its relationship with the common peroneal nerve and to present a case in which previously unreported MR evidence of an anatomic variation in the distal biceps femoris muscle was associated with common peroneal entrapment neuropathy. MATERIALS AND METHODS: One hundred consecutive 1.5-T knee MR studies of 97 asymptomatic patients were retrospectively reviewed by two observers in consensus for, first, normal anatomy of the distal biceps femoris muscle; second, anatomic variations of the muscle; and, third, the relationship of the muscle to the common peroneal nerve. Measurements of the distal and posterior extents of the short and long heads of the biceps femoris were performed. An MR study of a symptomatic patient with clinical evidence of common peroneal neuropathy associated with a surgically proven anatomic variation of the distal biceps femoris was reviewed. RESULTS: Two MR anatomic patterns were seen in the asymptomatic patient group: First, in 77 knees (77%), the common peroneal nerve was located within abundant fat posterolateral to the biceps femoris; and, second, in 23 cases (23%), the common peroneal nerve traversed within a narrow fatty tunnel between the biceps femoris and lateral head of the gastrocnemius muscles. There was a positive correlation between the distal and posterior extents of the short head of the biceps femoris muscle and the presence of the tunnel. CONCLUSION: Variations in the posterior and distal extents of the biceps femoris muscle can produce a tunnel in which the common peroneal nerve travels. We also described a case of common peroneal neuropathy secondary to tunnel formation.

Latest neuropathy articles PMID: 17715099 [PubMed - indexed for MEDLINE]Endoscopic optic nerve decompression for nontraumatic optic neuropathy. Related Articles

Endoscopic optic nerve decompression for nontraumatic optic neuropathy.

Arch Otolaryngol Head Neck Surg. 2007 Aug;133(8):780-3

Latest neuropathy articles Authors: Pletcher SD, Metson R

OBJECTIVE: To determine the efficacy of endoscopic optic nerve decompression for the treatment of patients with nontraumatic optic neuropathy. DESIGN: Retrospective case series. SETTING: Academic medical center. PATIENTS: Ten optic nerve decompressions were performed on 7 patients with nontraumatic optic neuropathy caused by various pathologic entities, including meningioma, lymphangioma, fibro-osseous lesions (fibrous dysplasia and osteoma), mucopyocele, and Graves orbitopathy. INTERVENTIONS: Endoscopic instrumentation was used in a transnasal fashion to decompress the optic nerve. MAIN OUTCOME MEASURES: Visual acuity and complication rates. RESULTS: Mean visual acuity improved from 20/300 preoperatively to 20/30 after surgery. Visual acuity improved by at least 2 lines on the Snellen chart following 7 of the 10 decompressions. Median operative time was 133 minutes, and median length of stay was less than 24 hours. Complications were limited to postoperative hyponatremia and corneal abrasions, both of which resolved with conservative therapy. Mean follow-up time was 6.1 months. CONCLUSION: Endoscopic optic nerve decompression appears to be an effective treatment for restoring visual acuity in select patients who present with compressive optic neuropathy.

Latest neuropathy articles PMID: 17709616 [PubMed - indexed for MEDLINE]Spinal cord magnetic resonance imaging for investigation of nonneurogenic lower urinary tract dysfunction--can the yield be improved? Related Articles

Spinal cord magnetic resonance imaging for investigation of nonneurogenic lower urinary tract dysfunction--can the yield be improved?

J Urol. 2007 Oct;178(4 Pt 2):1748-50; discussion 1750-1

Latest neuropathy articles Authors: Afshar K, Blake T, Jaffari S, MacNeily AE, Poskitt K, Sargent M

PURPOSE: Magnetic resonance imaging has been used to detect occult neuropathy in patients with nonneurogenic lower urinary tract dysfunction. There is substantial controversy surrounding the role of this test for lower urinary tract dysfunction. We identified factors associated with positive magnetic resonance imaging to improve patient selection. MATERIALS AND METHODS: A case-control study was done in all pediatric patients referred to our radiology department for spinal magnetic resonance imaging primarily because of lower urinary tract symptoms between 1995 and 2004. Patients with known neurological disorders or anomalies associated with neurogenic bladder (overt spinal dysraphism, imperforate anus, etc) were excluded. A total of 80 patients with a median age of 6.5 years (range 4 to 17) were identified, of whom 47 (59%) were female. Bivariate analysis was used to evaluate the association of certain variables with positive magnetic resonance imaging findings, including patient age, gender, type of urinary symptoms, fecal soiling, abnormal neuro-orthopedic examination, lumbar cutaneous findings, resistance to medical management and urodynamic findings. RESULTS: Magnetic resonance imaging revealed spinal abnormalities in 6 cases (7.5%), including intradural arachnoid cyst in 1, sacral dysgenesis in 3, syrinx/hydromyelia in 1 and tethered cord in 1. An abnormal lumbar cutaneous finding was the only variable associated with positive magnetic resonance imaging (Fisher's exact test p = 0.002). CONCLUSIONS: Spinal magnetic resonance imaging has a low impact in the management of lower urinary tract dysfunction. With proper patient selection the pretest probability of positive magnetic resonance imaging may be increased and, therefore, many unnecessary studies may be avoided. Abnormal cutaneous findings are associated with abnormal magnetic resonance imaging.

Latest neuropathy articles PMID: 17707435 [PubMed - indexed for MEDLINE]Painful neuropathy due to intraneural leukemic spread in a patient with acute myeloid leukemia. Related Articles

Painful neuropathy due to intraneural leukemic spread in a patient with acute myeloid leukemia.

Neurology. 2007 Aug 14;69(7):707

Latest neuropathy articles Authors: Platten M, Opitz CA, Kohlhof P, Hegenbart U, Ho AD, Wick W

Latest neuropathy articles PMID: 17698797 [PubMed - indexed for MEDLINE]Dynamic phases of peroneal and tibial intraneural ganglia formation: a new dimension added to the unifying articular theory. Related Articles

Dynamic phases of peroneal and tibial intraneural ganglia formation: a new dimension added to the unifying articular theory.

J Neurosurg. 2007 Aug;107(2):296-307

Latest neuropathy articles Authors: Spinner RJ, Amrami KK, Wolanskyj AP, Desy NM, Wang H, Benarroch EE, Skinner JA, Rock MG, Scheithauer BW

OBJECT: The pathogenesis of intraneural ganglia has been a controversial issue for longer than a century. Recently the authors identified a stereotypical pattern of occurrence of peroneal and tibial intraneural ganglia, and based on an understanding of their pathogenesis provided a unifying articular explanation. Atypical features, which occasionally are observed, have offered an opportunity to verify further and expand on the authors' proposed theory. METHODS: Three unusual cases are presented to exemplify the dynamic features of peroneal and tibial intraneural ganglia formation. RESULTS: Two patients with a predominant deep peroneal nerve deficit shared essential anatomical findings common to peroneal intraneural ganglia: namely, 1) joint connections to the anterior portion of the superior tibiofibular joint, and 2) dissection of the cyst along the articular branch of the peroneal nerve and proximally. Magnetic resonance (MR) images obtained in these patients demonstrated some unusual findings, including the presence of a cyst within the tibial and sural nerves in the popliteal fossa region, and spontaneous regression of the cysts, which was observed on serial images obtained weeks apart. The authors identified a clinical outlier, a case that could not be understood within the context of their previously reported theory of intraneural ganglion cyst formation. Described 32 years ago, this patient had a tibial neuropathy and was found at surgery to have tibial, peroneal, and sciatic intraneural cysts without a joint connection. The authors' hypothesis about this case, based on their unified theory, was twofold: 1) the lesion was a primary tibial intraneural ganglion with proximal extension followed by sciatic cross-over and distal descent; and 2) a joint connection to the posterior aspect of the superior tibiofibular joint with a remnant cyst within the articular branch would be present, a finding that would help explain the formation of different cysts by a single mechanism. The authors proved their hypothesis by careful inspection of a recently obtained postoperative MR image. CONCLUSIONS: These three cases together with data obtained from a retrospective review of the authors' clinical material and findings reported in the literature provide firm evidence for mechanisms underlying intraneural ganglia formation. Thus, expansion of the authors' unified articular theory permits understanding and elucidation of unusual presentations of intraneural cysts. Whereas an articular connection and fluid following the path of least resistance was pivotal, the authors now incorporate dynamic aspects of cyst formation due to pressure fluxes. These basic principles explain patterns of ascent, cross-over, and descent down terminal nerve branches based on articular connections, paths of diminished resistance to fluid flow within recognized anatomical compartments, and the effects of fluctuating pressure gradients.

Latest neuropathy articles PMID: 17695383 [PubMed - indexed for MEDLINE]Signs and symptoms predictive of respiratory failure in patients with foodborne botulism in Thailand. Related Articles

Signs and symptoms predictive of respiratory failure in patients with foodborne botulism in Thailand.

Am J Trop Med Hyg. 2007 Aug;77(2):386-9

Latest neuropathy articles Authors: Wongtanate M, Sucharitchan N, Tantisiriwit K, Oranrigsupak P, Chuesuwan A, Toykeaw S, Suputtamongkol Y

We conducted a clinical study of 137 patients with home-canned bamboo shoot botulism at Nan Hospital, northern Thailand. The median age of the patients was 44 years (range = 14-74 years) and 36.2% were male. The median incubation period was 2 days (range = 1-8 days). Forty-three patients (31.4%) developed respiratory failure, but there were no deaths. Patients who did not have either nausea or vomiting and did not have urinary retention that required Foley catheterization was less likely to develop respiratory failure. This clinical predictor rule had a sensitivity of 75.5% and a specificity of 90.7%. The clinical syndrome most predictive of respiratory failure was nausea or vomiting and any cranial neuropathy with urinary retention or difficulty swallowing. This clinical syndrome had a sensitivity of 69.8% and a specificity of 93.6%. These clinical characteristics could help triage large numbers of patient in the event of a future outbreak.

Latest neuropathy articles PMID: 17690419 [PubMed - indexed for MEDLINE]Ocular manifestations of systemic lupus erythematosus. Related Articles

Ocular manifestations of systemic lupus erythematosus.

Rheumatology (Oxford). 2007 Dec;46(12):1757-62

Latest neuropathy articles Authors: Sivaraj RR, Durrani OM, Denniston AK, Murray PI, Gordon C

Ocular manifestations of lupus are fairly common, may be the presenting feature of the disease and can be sight-threatening. Almost any part of the eye and visual pathway can be affected by inflammatory or thrombotic processes. Ocular pain and visual impairment require urgent assessment by an ophthalmologist. Infection should be excluded. Optic neuritis and ischaemic optic neuropathy may be difficult to distinguish. Scleritis and severe retinopathy require systemic immunosuppression but episcleritis, anterior uveitis and dry eyes can usually be managed with local eye drops. Vaso-occlusive disease, particularly in the presence of antiphospholipid antibodies, requires treatment with anticoagulation and proliferative retinopathy is treated with laser therapy. Hydroxychloroquine rarely causes ocular toxicity at doses under 6.5 mg/kg/day. When this has occurred, it has been associated with more than 5 years of drug exposure.

Latest neuropathy articles PMID: 17681981 [PubMed - in process]Neuropathy in multiple myeloma treated with thalidomide: a prospective study. Related Articles

Neuropathy in multiple myeloma treated with thalidomide: a prospective study.

Neurology. 2007 Aug 7;69(6):573-81

Latest neuropathy articles Authors: Plasmati R, Pastorelli F, Cavo M, Petracci E, Zamagni E, Tosi P, Cangini D, Tacchetti P, Salvi F, Bartolomei I, Michelucci R, Tassinari CA

BACKGROUND: Thalidomide is effective as a first-line therapy for the treatment of multiple myeloma (MM), but its use is limited by peripheral neurotoxicity. OBJECTIVE: To study the occurrence of both myeloma-related neuropathy and thalidomide-induced neuropathy in 31 patients with newly diagnosed MM. METHODS: Clinical and electrophysiologic examinations were performed in 31 patients with newly diagnosed MM before and after 4 months of therapy with thalidomide (200 mg/day, total dose: 21 g) aimed at debulking MM, before autologous transplantation. After transplantation, the patients took thalidomide, 200 mg/day for another 3 months (total dose over three months: 18 g) and then underwent a final clinical and electrophysiologic checkup. RESULTS: At baseline, four patients presented a mild sensorimotor peripheral neuropathy related to MM, which tended to worsen slightly during treatment with thalidomide. At the end of treatment, 83% of the patients had clinical and electrophysiologic evidence of a mild sensory rather than motor, axonal, length-dependent polyneuropathy, whereas 100% of the patients showed improvement to the basic pathology (>or=partial response). CONCLUSIONS: Peripheral neuropathy, sometimes subclinical, and mild in our patients, is a common, early side effect of thalidomide therapy. The high doses (21 g) used in all patients for a relatively short time (4 months) rule out any correlations between neuropathy, total dose, and duration of treatment.

Latest neuropathy articles PMID: 17679676 [PubMed - indexed for MEDLINE]Ataxic vs painful form of paraneoplastic neuropathy. Related Articles

Ataxic vs painful form of paraneoplastic neuropathy.

Neurology. 2007 Aug 7;69(6):564-72

Latest neuropathy articles Authors: Oki Y, Koike H, Iijima M, Mori K, Hattori N, Katsuno M, Nakamura T, Hirayama M, Tanaka F, Shiraishi M, Yazaki S, Nokura K, Yamamoto H, Sobue G

OBJECTIVE: To characterize the clinicopathologic features of ataxic and painful forms of paraneoplastic neuropathy. METHODS: Clinical, electrophysiologic, and histopathologic findings were assessed in 17 patients with paraneoplastic neuropathy. RESULTS: Clinical features can be categorized into two groups: one group (13 patients) with predominantly deep sensory disturbance and a second group (4 patients) with predominantly superficial sensory disturbance. The former group showed severe sensory ataxia and predominantly large myelinated fiber loss in the sural nerve. The latter group showed marked pain, in particular, severe mechanical hyperalgesia, and predominantly small myelinated and unmyelinated fiber loss. Nerve conduction assessment indicated an axonal neuropathy pattern in both groups, while sensory action potentials were more markedly diminished in the sensory ataxic form. Anti-Hu antibodies were detected in half of the patients in both groups. Treatment for cancer was effective to improve or stabilize neuropathic symptoms in some cases from both groups. Immunotherapy was effective only for a short time. CONCLUSIONS: Paraneoplastic neuropathy can be characterized into two groups by the presence of sensory ataxia or severe spontaneous pain and severe mechanical hyperalgesia. Preferential small myelinated and unmyelinated fiber loss correlated to the cases of severe pain.

Latest neuropathy articles PMID: 17679675 [PubMed - indexed for MEDLINE]Nitrofurantoin neuropathy: a forgotten adverse effect. Related Articles

Nitrofurantoin neuropathy: a forgotten adverse effect.

Obstet Gynecol. 2007 Aug;110(2 Pt 2):510-2

Latest neuropathy articles Authors: Kammire LD, Donofrio PD

BACKGROUND: Nitrofurantoin is a widely prescribed antibiotic used to treat uncomplicated lower urinary tract infections. Unknown in the obstetric and gynecologic literature is the complication of peripheral neuropathy as an adverse effect. CASE: We describe a patient who developed a severe sensory neuropathy after taking nitrofurantoin intermittently and then continuously over a 7-year period. She recovered almost completely after its discontinuation. CONCLUSION: Peripheral neuropathy is a rare and potentially reversible adverse effect, unreported in the obstetric and gynecologic literature, and commonly unrecognized by physicians who prescribe it.

Latest neuropathy articles PMID: 17666646 [PubMed - indexed for MEDLINE]Antiviral therapy for cryoglobulinemic neuropathy: comment on the article by Saadoun et al. Related Articles

Antiviral therapy for cryoglobulinemic neuropathy: comment on the article by Saadoun et al.

Arthritis Rheum. 2007 Aug;56(8):2810; author reply 2810-1

Latest neuropathy articles Authors: Gemignani F, Brindani F, Marbini A

Latest neuropathy articles PMID: 17665398 [PubMed - indexed for MEDLINE]Gamma knife radiosurgery for choroidal hemangioma. Related Articles

Gamma knife radiosurgery for choroidal hemangioma.

Am J Ophthalmol. 2007 Aug;144(2):319-22

Latest neuropathy articles Authors: Kong DS, Lee JI, Kang SW

PURPOSE: To determine the feasibility of gamma knife radiosurgery (GKS) in the treatment of symptomatic choroidal hemangioma (CH). DESIGN: Observational case series. METHODS: Three patients who had CH were treated with GKS. Ophthalmologic examinations revealed submacular fluid collection and retinal detachment associated with CH in all cases. GKS was performed as an initial treatment in two cases of circumscribed CH and as secondary treatment after failure of photocoagulation in the other diffuse CH. RESULTS: The follow-up period ranged from 18 to 36 months, and visual acuity of the affected eyes was improved in all cases. The volume of CHs and submacular fluid collection were decreased. There were no adverse effects of radiation such as retinopathy or optic neuropathy. CONCLUSIONS: GKS may be a useful alternative treatment for symptomatic CHs that are difficult to be managed with a standard therapeutic option such as photocoagulation.

Latest neuropathy articles PMID: 17659973 [PubMed - indexed for MEDLINE]Neurotoxicity of bortezomib therapy in multiple myeloma: a single-center experience and review of the literature. Related Articles

Neurotoxicity of bortezomib therapy in multiple myeloma: a single-center experience and review of the literature.

Cancer. 2007 Sep 1;110(5):1042-9

Latest neuropathy articles Authors: Badros A, Goloubeva O, Dalal JS, Can I, Thompson J, Rapoport AP, Heyman M, Akpek G, Fenton RG

BACKGROUND: Bortezomib is active in heavily pretreated multiple myeloma patients; the dose-limiting toxicity is peripheral neuropathy (PN). METHODS: The authors retrospectively reviewed the incidence, severity, and risk factors for PN in 78 patients who received bortezomib. The median age was 57 years (range, 33-80 years), 62% of patients were men, and 37% of patients were African Americans. Seventeen patients (22%) had diabetes mellitus (DM), and 66 patients (85%) had received thalidomide. Before bortezomib treatment, 37% of the patients reported subjective, grade 1 or 2 PN. Patients received bortezomib alone (n = 10 patients) plus dexamethasone (n = 36 patients) and thalidomide (n = 20 patients) or chemotherapy (n = 12 patients). PN affected 52% of patients, including grade 3 and 4 PN in 15% and 7%, respectively. RESULTS: Twelve patients stopped bortezomib because of side effects that included PN (n = 9 patients), diarrhea (n = 2 patients) and cytomegalovirus pneumonia (n = 1 patient); 11 patients had dose reductions because of PN. Grade 4 PN affected 6 patients (sensory, n = 4 patients; motor/sensory, n = 2 patients). The onset of grade 4 PN was sudden rather than cumulative. Factors that were predictive of PN grade were baseline PN (P = .002), prior thalidomide use (P = .03), and the presence of DM (P = .03). Multiple myeloma responses included complete, near complete, and partial responses in 5% of patients, 10% of patients, and 27% of patients, respectively. Responses were independent of PN and of whether bortezomib was combined with chemotherapy or thalidomide. Patients remained on therapy longer for a median of 5 cycles (range, 2-36 cycles) when they received bortezomib plus thalidomide versus 3 cycles (range, 1-19 cycles) for the other combinations. PN therapy was mostly supportive. It was noteworthy that 6 of 9 patients with PN who received lenalidomide as salvage therapy after bortezomib had significant improvement in their symptoms. CONCLUSIONS: The risk of bortezomib-related PN was greater in patients who had PN and DM at baseline. The authors concluded that an unexpected, symptomatic improvement of PN on lenalidomide is worth further investigation.

Latest neuropathy articles PMID: 17654660 [PubMed - indexed for MEDLINE]Two novel mutations in dynamin-2 cause axonal Charcot-Marie-Tooth disease. Related Articles

Two novel mutations in dynamin-2 cause axonal Charcot-Marie-Tooth disease.

Neurology. 2007 Jul 17;69(3):291-5

Latest neuropathy articles Authors: Fabrizi GM, Ferrarini M, Cavallaro T, Cabrini I, Cerini R, Bertolasi L, Rizzuto N

BACKGROUND: Recently, mutations affecting different domains of dynamin-2 (DNM2) were associated alternatively with autosomal dominant centronuclear myopathy or dominant intermediate (demyelinating and axonal) Charcot-Marie-Tooth disease (CMT) type B. OBJECTIVE: To assess the etiologic role of DNM2 in CMT. METHODS: We performed a mutational screening of DNM2 exons 13 through 16 encoding the pleckstrin homology domain in a large series of CMT patients with a broad range of nerve conduction velocities and without mutations in more common genes. RESULTS: We identified two novel DNM2 mutations that cosegregated with purely axonal CMT in two pedigrees without clinical evidence of primary myopathy. CONCLUSION: Patients with axonal Charcot-Marie-Tooth disease type 2 neuropathy without mutations in more common genes should undergo investigation for DNM2 pleckstrin homology.

Latest neuropathy articles PMID: 17636067 [PubMed - indexed for MEDLINE]Microvascular tissue plasminogen activator is reduced in diabetic neuropathy. Related Articles

Microvascular tissue plasminogen activator is reduced in diabetic neuropathy.

Neurology. 2007 Jul 17;69(3):268-74

Latest neuropathy articles Authors: Hafer-Macko CE, Ivey FM, Sorkin JD, Macko RF

BACKGROUND: Abnormalities of endogenous fibrinolysis are linked to diabetic macrovascular disease; whether key vascular endothelial regulatory proteins, such as tissue plasminogen activator (tPA), are altered in diabetic neuropathy microvasculature is unknown. This neuropathologic case: control study investigates the hypothesis that tPA expression is regionally deficient in microvessels in human diabetic neuropathy. METHODS: tPA and von Willebrand factor (vWF), a vascular endothelial cell marker, are detected on vascular endothelium by immunoperoxidase methods with specific antibodies on formalin fixed paraffin embedded sural nerve biopsies from six diabetic and six axonal neuropathy control nerves without vasculopathy. The proportion of microvessels in each nerve region expressing tPA is determined by the ratio of tPA positive vessels/total vWF positive vessels on serial sections. RESULTS: tPA expression is lower in diabetic neuropathy cases compared to controls in all regions, including epineurial (62.4 +/- 8.6% vs 91.0 +/- 1.6%, p < 0.02) and endoneurial microvessels (51.7 +/- 7.1% vs 91.5 +/- 2.9%, p < 0.001). CONCLUSIONS: These results demonstrate a four- to sixfold increase in the number of peripheral nerve microvessels lacking immunodetectable tissue plasminogen activator in the epineurial and endoneurial vessels in diabetes, suggesting that impaired endogenous fibrinolysis might contribute to microvascular ischemia in human diabetic neuropathy.

Latest neuropathy articles PMID: 17636064 [PubMed - indexed for MEDLINE]Docetaxel and cisplatin in patients with metastatic androgen independent prostate cancer and circulating neuroendocrine markers. Related Articles

Docetaxel and cisplatin in patients with metastatic androgen independent prostate cancer and circulating neuroendocrine markers.

J Urol. 2007 Sep;178(3 Pt 1):844-8; discussion 848

Latest neuropathy articles Authors: Culine S, El Demery M, Lamy PJ, Iborra F, Avancès C, Pinguet F

PURPOSE: A link between neuroendocrine cell differentiation and resistance to androgen deprivation has been observed in prostate cancer, suggesting the possible efficacy of specific treatments. We assessed the efficacy and toxicity of a chemotherapy regimen combining docetaxel and cisplatin in men with androgen independent prostatic adenocarcinoma and circulating neuroendocrine markers. MATERIALS AND METHODS: A total of 41 patients were treated with a combination of 75 mg/m(2) docetaxel and 75 mg/m(2) cisplatin every 3 weeks for a maximum of 6 cycles. The primary study end point was the neuroendocrine response rate, defined as a decrease in neuron specific enolase and/or chromogranin A to 50% or greater of the supranormal baseline serum value. Median followup was 40 months. RESULTS: A median of 6 cycles per patient was delivered. A neuroendocrine response was observed in 13 patients (33%). The median response duration was 4 months (range 2 to 10). The prostate specific antigen response rate was 48%. A clinical benefit was observed in 45% of patients who required analgesics at study entry. The objective response rate was 41% in 29 patients with measurable metastases. Five patients had to stop therapy due to toxicity. The main side effects were cumulative asthenia and sensitive neuropathy. Median survival was 12 months (range 1 to 38). CONCLUSIONS: Regarding the disappointing efficacy and significant toxicity observed in this study, the combination of docetaxel and cisplatin cannot be recommended in daily practice. Further studies are necessary to determine whether patients with circulating neuroendocrine markers require specific therapeutic approaches.

Latest neuropathy articles PMID: 17631339 [PubMed - indexed for MEDLINE]High-fat diet induced neuropathy of pre-diabetes and obesity: effects of "healthy" diet and aldose reductase inhibition. Related Articles

High-fat diet induced neuropathy of pre-diabetes and obesity: effects of "healthy" diet and aldose reductase inhibition.

Diabetes. 2007 Oct;56(10):2598-608

Latest neuropathy articles Authors: Obrosova IG, Ilnytska O, Lyzogubov VV, Pavlov IA, Mashtalir N, Nadler JL, Drel VR

OBJECTIVE: Subjects with dietary obesity and pre-diabetes have an increased risk for developing both nerve conduction slowing and small sensory fiber neuropathy. Animal models of this type of neuropathy have not been described. This study evaluated neuropathic changes and their amenability to dietary and pharmacological interventions in mice fed a high-fat diet (HFD), a model of pre-diabetes and alimentary obesity. RESEARCH DESIGN AND METHODS: Female C57BL6/J mice were fed normal diets or HFDs for 16 weeks. RESULTS: HFD-fed mice developed obesity, increased plasma FFA and insulin concentrations, and impaired glucose tolerance. They also had motor and sensory nerve conduction deficits, tactile allodynia, and thermal hypoalgesia in the absence of intraepidermal nerve fiber loss or axonal atrophy. Despite the absence of overt hyperglycemia, the mice displayed augmented sorbitol pathway activity in the peripheral nerve, as well as 4-hydroxynonenal adduct nitrotyrosine and poly(ADP-ribose) accumulation and 12/15-lipoxygenase overexpression in peripheral nerve and dorsal root ganglion neurons. A 6-week feeding with normal chow after 16 weeks on HFD alleviated tactile allodynia and essentially corrected thermal hypoalgesia and sensory nerve conduction deficit without affecting motor nerve conduction slowing. Normal chow containing the aldose reductase inhibitor fidarestat (16 mg x kg(-1) x day (-1)) corrected all functional changes of HFD-induced neuropathy. CONCLUSIONS: Similar to human subjects with pre-diabetes and obesity, HFD-fed mice develop peripheral nerve functional, but not structural, abnormalities and, therefore, are a suitable model for evaluating dietary and pharmacological approaches to halt progression and reverse diabetic neuropathy at the earliest stage of the disease.

Latest neuropathy articles PMID: 17626889 [PubMed - indexed for MEDLINE]Mycophenolate mofetil as adjunctive therapy for MMN patients: a randomized, controlled trial. Related Articles

Mycophenolate mofetil as adjunctive therapy for MMN patients: a randomized, controlled trial.

Brain. 2007 Aug;130(Pt 8):2004-10

Latest neuropathy articles Authors: Piepers S, Van den Berg-Vos R, Van der Pol WL, Franssen H, Wokke J, Van den Berg L

Multifocal motor neuropathy (MMN) is an immune-mediated disorder characterized by slowly progressive asymmetrical limb weakness. Treatment with immunoglobulins (IVIg) leads to improvement of muscle strength. Anecdotal evidence suggests that immunosuppressive drugs as adjunctive therapy may be beneficial. Mycophenolate mofetil (MMF) is a potent and safe immunosuppressant. Safety and efficacy of MMF as adjunctive therapy for MMN patients receiving IVIg maintenance treatment were evaluated in a randomized controlled trial. MMN patients responding to IVIg treatment were eligible for randomization. Muscle strength and functional status were assessed at monthly intervals for 1 year. Three months after the start of MMF or placebo treatment, IVIg doses were reduced stepwise, until a deterioration of functioning or decline in muscle strength could be observed. An IVIg dose reduction of 50% during adjunctive treatment was defined as a primary endpoint. Secondary outcome measures were improvement in muscle strength and functional status after 3 months and reduction of anti GM1-IgM titres after 12 months of MMF treatment. Twenty-eight patients were randomized. One patient allocated to MMF reached the primary endpoint of 50% IVIg dose reduction. After 12 months IVIg reduction did not differ significantly between the two treatment groups. Patients did not experience drug toxicity and none of the patients showed significant disease progression after 12 months. Muscle strength and functional scores after 3 months and anti GM1-IgM titres after 12 months did not change. Adjunctive treatment of MMN patients with MMF at a dose of 1 g twice daily is safe but does not alter disease course or allow significant reduction of IVIg doses.

Latest neuropathy articles PMID: 17626040 [PubMed - indexed for MEDLINE]Primary spinal cord neurodegeneration in Leber hereditary optic neuropathy. Related Articles

Primary spinal cord neurodegeneration in Leber hereditary optic neuropathy.

Neurology. 2007 Jul 10;69(2):214-6

Latest neuropathy articles Authors: Jaros E, Mahad DJ, Hudson G, Birchall D, Sawcer SJ, Griffiths PG, Sunter J, Compston DA, Perry RH, Chinnery PF

Latest neuropathy articles PMID: 17620555 [PubMed - indexed for MEDLINE]Stoichiometric alteration of PMP22 protein determines the phenotype of hereditary neuropathy with liability to pressure palsies. Related Articles

Stoichiometric alteration of PMP22 protein determines the phenotype of hereditary neuropathy with liability to pressure palsies.

Arch Neurol. 2007 Jul;64(7):974-8

Latest neuropathy articles Authors: Li J, Ghandour K, Radovanovic D, Radovanovic D, Shy RR, Krajewski KM, Shy ME, Nicholson GA

BACKGROUND: Hereditary neuropathy with liability to pressure palsies (HNPP) is caused by a 1.4-megabase deletion at chromosome 17p11.2, which bears the PMP22 gene and other genes. However, whether other genes besides PMP22 contribute to the phenotype is unknown. Whether any mutation within the coding region of the PMP22 gene ultimately causes HNPP by reducing the amount of peripheral myelin protein 22 (PMP22) expressed in myelin is also unknown. OBJECTIVE: To determine whether affected patients develop a phenotype identical to that found in HNPP and whether the leucine 7 frameshift (Leu7fs) mutation reduces PMP22 levels in myelin. DESIGN: We evaluated affected family members by neurological examination, electrophysiology, and skin biopsies. We identified a large family with a Leu7fs mutation of PMP22 (11 affected members across 3 generations) that predicts truncation of the protein prematurely and eliminates PMP22 expression from the mutant allele. RESULTS: We found that PMP22 levels were reduced in peripheral nerve myelin in dermal skin biopsies in patients with an Leu7fs mutation. Through clinical and electrophysiological evaluation, we also found that patients with the Leu7fs mutation were indistinguishable from patients with HNPP caused by deletion. We also found that a length-dependent axonal loss became pronounced in elderly patients with Leu7fs mutations, similar to what has been described in heterozygous knockout mice (pmp22 +/-). CONCLUSIONS: Taken together, these results confirm that the phenotypic expression is identical in patients with Leu7fs mutation and patients with HNPP caused by chromosome 17p11.2 deletion. They also demonstrate that reduction of PMP22 is sufficient to cause the full HNPP phenotype.

Latest neuropathy articles PMID: 17620487 [PubMed - indexed for MEDLINE]Skin denervation and cutaneous vasculitis in eosinophilia-associated neuropathy. Related Articles

Skin denervation and cutaneous vasculitis in eosinophilia-associated neuropathy.

Arch Neurol. 2007 Jul;64(7):959-65

Latest neuropathy articles Authors: Chao CC, Hsieh ST, Shun CT, Hsieh SC

BACKGROUND: Eosinophilia is frequently associated with peripheral neuropathy, and neuropathic pain is a major presentation. Little is known about the involvement of sensory nerve terminals and the vasculature in the skin of patients with eosinophilia. OBJECTIVES: To investigate the skin innervation and the pathological abnormalities of the cutaneous vasculature and their clinical significance in eosinophilia-associated neuropathy. DESIGN: Case series. SETTING: National Taiwan University Hospital, Taipei, Taiwan. Patients Twelve patients with neuropathy and concomitant eosinophilia (with an eosinophilic ratio of white blood cell classification > 10% or absolute eosinophil count of > 1000/microL). INTERVENTIONS: Clinical assessments of neurological deficits, laboratory tests, nerve conduction studies, and a skin biopsy specimen 3 mm in diameter taken from the distal leg without active skin lesions. MAIN OUTCOME MEASURES: Quantitation of epidermal innervation, immunopathological findings of the cutaneous vasculature, and motor disability grade. RESULTS: Six patients fulfilled the criteria of Churg-Strauss syndrome, and the other 6 patients were categorized as having primary eosinophilia. All of the 12 patients had mononeuropathy multiplex or polyneuropathy with sensory symptoms as the initial manifestation. Intraepidermal nerve fiber densities were reduced in 10 patients (83.3%), being significantly lower than in the controls (mean +/- SD, 2.12 +/- 2.30 vs 10.56 +/- 3.69 fibers/mm, respectively; P < .001) and negatively correlated with the disability grade (P = .003). Nine patients (75.0%), including all of the 6 patients with Churg-Strauss syndrome, had cutaneous vasculitis, and two-thirds of the 9 patients had perivascular infiltration of eosinophils. CONCLUSION: Skin denervation with cutaneous vasculitis is a major manifestation of eosinophilia-associated neuropathy.

Latest neuropathy articles PMID: 17620485 [PubMed - indexed for MEDLINE]

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