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Brain MRI and CP

The latest research articles relating to the use of brain MRI and CP are accessible below (updated daily).

Some original research articles are available in full at the bottom of the page.

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  • MR imaging in a child with scurvy: a case report.
    Related Articles

    MR imaging in a child with scurvy: a case report.

    Korean J Radiol. 2007 Sep-Oct;8(5):443-7

    Authors: Choi SW, Park SW, Kwon YS, Oh IS, Lim MK, Kim WH, Suh CH

    Scurvy is very rare disease in industrialized societies. Nevertheless, it still exists in higher risk groups including economically disadvantaged populations with poor nutrition, such as the elderly and chronic alcoholics. The incidence of scurvy in the pediatric population is very low. This study reports a case of scurvy in a 5-year-old girl with cerebral palsy and developmental delay based on MRI findings.

    PMID: ( MRI and CP ) ] 17923788 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Analysis of the correlation between three methods used in the assessment of children with cerebral palsy.
    Related Articles

    Analysis of the correlation between three methods used in the assessment of children with cerebral palsy.

    Funct Neurol. 2007 Jan-Apr;22(1):17-21

    Authors: Romei M, Galli M, Fazzi E, Maraucci I, Schwartz M, Uggetti C, Crivellini M

    The primary aim of this study was to assess the correlations between gait analysis, magnetic resonance imaging (MRI), and Gross Motor Function Measure (GMFM) scores in children with cerebral palsy (CP). These common diagnostic tools were used to evaluate 21 children affected by CP (mean age: 6 years, range: 5-13 years; 8 females and 13 males; 5 left hemiplegics, 4 right hemiplegics, 12 diplegics). In particular, in order to compare gait analysis data with other diagnostic evaluations, the Normalcy Index (NI) was used. The results showed a good correlation between the NI and the results of MRI, and between NI and the GMFM score (r=-0.76). Therefore, this investigation demonstrated that there exists a strong relationship between gait analysis and other clinical evaluation tools.

    PMID: ( MRI and CP ) ] 17509239 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Ruptured anterior communicating artery aneurysm causing bilateral oculomotor nerve palsy: a case report.
    Related Articles

    Ruptured anterior communicating artery aneurysm causing bilateral oculomotor nerve palsy: a case report.

    J Korean Med Sci. 2007 Feb;22(1):173-6

    Authors: Kang SD

    A rare case of bilateral third cranial nerve palsy due to a ruptured anterior communicating artery aneurysm is presented. A 68-yr-old woman was semicomatose with bilaterally fixed dilated pupil, abducted eyes, and ptosis. A computed tomography demonstrated extensive hemorrhage spreading around the both Sylvian and interhemisheric fissure without focal mass effect. Intracranial pressure via extraventricular drainage before surgery was 15-50 mmHg. Three months later, brain MRI showed infarction of left posterior cerebral artery territory and lacuna infarction of the pons. Eleven months after aneurysm repair, nerve palsy improved slowly and recovered partially. The patient communicated well with simple words. The author reviewed and discussed the possible mechanism of this rare neuro-ophthalmological manifestation in view of a false localizing sign.

    PMID: ( MRI and CP ) ] 17297277 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Periventricular leukomalacia with late-onset circulatory dysfunction of premature infants: correlation with severity of magnetic resonance imaging findings and neurological outcomes.
    Related Articles

    Periventricular leukomalacia with late-onset circulatory dysfunction of premature infants: correlation with severity of magnetic resonance imaging findings and neurological outcomes.

    Tohoku J Exp Med. 2006 Dec;210(4):333-9

    Authors: Kobayashi S, Fujimoto S, Fukuda S, Hattori A, Iwaki T, Koyama N, Tanaka T, Kokubo M, Okanishi T, Togari H

    The incidence of late-onset circulatory dysfunction (LCD) of premature infants, which is characterized by sudden hypotension and oliguria, has recently increased in Japan. This condition suddenly occurs after several days of age without obvious causes in preterm infants with stable respiration and circulation. Intravenous steroids frequently improve the hypotension. The main problem with LCD is the subsequent and frequent onset of periventricular leukomalacia (PVL), and neurological development appears to be worse in PVL patients with LCD than those without LCD. The aim of this study was to determine whether the severity of magnetic resonance imaging (MRI) findings and neurological outcomes differ between infants who developed PVL after LCD and those who developed PVL without LCD. We retrospectively studied preterm infants who were delivered at less than 33 weeks of gestation between the years 2000 and 2003. During the study period, 10 and 26 infants developed PVL with and without LCD, respectively. The incidence of severe or moderate MRI findings was significantly higher in PVL patients with LCD (100%) than those without LCD (50%; p < 0.05). The incidence of severe cerebral palsy was 88% in PVL infants with LCD and 43% in PVL infants without LCD (p < 0.05). Moreover, the incidence of visual disorders was significantly higher in PVL infants with LCD (63%) than those without LCD (9%; p < 0.01). In conclusion, neurological outcomes are worse in preterm infants who develop PVL with LCD than those without LCD, which is well correlated to the severity judged by MRI findings.

    PMID: ( MRI and CP ) ] 17146199 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Does perinatal asphyxia impair cognitive function without cerebral palsy?
    Related Articles

    Does perinatal asphyxia impair cognitive function without cerebral palsy?

    Arch Dis Child Fetal Neonatal Ed. 2006 Nov;91(6):F454-9

    Authors: Gonzalez FF, Miller SP

    Some studies on neurodevelopmental outcomes after neonatal encephalopathy have suggested that cognitive deficits do not occur in the absence of cerebral palsy. It is increasingly apparent that childhood survivors of overt neonatal encephalopathy may have cognitive impairments, even in the absence of functional motor deficits. The risk of cognitive deficits is related to the severity of neonatal encephalopathy and the pattern of brain injury on neuroimaging, particularly the watershed pattern of injury. A better understanding of the risk factors for cognitive abnormalities after neonatal encephalopathy will ultimately lead to interventions to prevent these deficits. Identifying the full spectrum of neurodevelopmental outcomes after neonatal encephalopathy will also allow care givers to identify children requiring early intervention to maximise their potential for independent function throughout development.

    PMID: ( MRI and CP ) ] 17056843 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Clinical and MRI correlates of cerebral palsy: the European Cerebral Palsy Study.
    Related Articles

    Clinical and MRI correlates of cerebral palsy: the European Cerebral Palsy Study.

    JAMA. 2006 Oct 4;296(13):1602-8

    Authors: Bax M, Tydeman C, Flodmark O

    CONTEXT: Magnetic resonance imaging (MRI) findings have been reported for specific clinical cerebral palsy (CP) subgroups or lesion types but not in a large population of children with all CP subtypes. Further information about the causes of CP could help identify preventive strategies. OBJECTIVE: To investigate the correlates of CP in a population sample and compare clinical findings with information available from MRI brain studies. DESIGN AND SETTING: Cross-sectional, population-based investigative study conducted in 8 European study centers (North West London and North East London, England; Edinburgh, Scotland; Lisbon, Portugal; Dublin, Ireland; Stockholm, Sweden; Tübingen, Germany; and Helsinki, Finland). PARTICIPANTS: Five hundred eighty-five children with CP were identified who had been born between 1996 and 1999; 431 children were clinically assessed and 351 had a brain MRI scan. MAIN OUTCOME MEASURES: Standardized clinical examination results, parental questionnaire responses, MRI results, and obstetric, genetic, and metabolic data from medical records. RESULTS: Important findings include the high rate of infections reported by mothers during pregnancy (n = 158 [39.5%]). In addition, 235 children (54%) were born at term while 47 children (10.9%) were very preterm (<28 weeks). A high rate of twins was found, with 51 children (12%) known to be from a multiple pregnancy. Clinically, 26.2% of children had hemiplegia, 34.4% had diplegia, 18.6% had quadriplegia, 14.4% had dyskinesia, 3.9% had ataxia, and 2.6% had other types of CP. Brain MRI scans showed that white-matter damage of immaturity, including periventricular leukomalacia (PVL), was the most common finding (42.5%), followed by basal ganglia lesions (12.8%), cortical/subcortical lesions (9.4%), malformations (9.1%), focal infarcts (7.4%), and miscellaneous lesions (7.1%). Only 11.7% of these children had normal MRI findings. There were good correlations between the MRI and clinical findings. CONCLUSIONS: These MRI findings suggest that obstetric mishaps might have occurred in a small proportion of children with CP. A systematic approach to identifying and treating maternal infections needs to be developed. Multiple pregnancies should be monitored closely, and the causes of infant stroke need to be investigated further so preventive strategies can be formulated. All children with CP should have an MRI scan to provide information on the timing and extent of the lesion.

    PMID: ( MRI and CP ) ] 17018805 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Neonatal MRI to predict neurodevelopmental outcomes in preterm infants.
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    Neonatal MRI to predict neurodevelopmental outcomes in preterm infants.

    N Engl J Med. 2006 Aug 17;355(7):685-94

    Authors: Woodward LJ, Anderson PJ, Austin NC, Howard K, Inder TE

    BACKGROUND: Very preterm infants are at high risk for adverse neurodevelopmental outcomes. Magnetic resonance imaging (MRI) has been proposed as a means of predicting neurodevelopmental outcomes in this population. METHODS: We studied 167 very preterm infants (gestational age at birth, 30 weeks or less) to assess the associations between qualitatively defined white-matter and gray-matter abnormalities on MRI at term equivalent (gestational age of 40 weeks) and the risks of severe cognitive delay, severe psychomotor delay, cerebral palsy, and neurosensory (hearing or visual) impairment at 2 years of age (corrected for prematurity). RESULTS: At two years of age, 17 percent of infants had severe cognitive delay, 10 percent had severe psychomotor delay, 10 percent had cerebral palsy, and 11 percent had neurosensory impairment. Moderate-to-severe cerebral white-matter abnormalities present in 21 percent of infants at term equivalent were predictive of the following adverse outcomes at two years of age: cognitive delay (odds ratio, 3.6; 95 percent confidence interval, 1.5 to 8.7), motor delay (odds ratio, 10.3; 95 percent confidence interval, 3.5 to 30.8), cerebral palsy (odds ratio, 9.6; 95 percent confidence interval, 3.2 to 28.3), and neurosensory impairment (odds ratio, 4.2; 95 percent confidence interval, 1.6 to 11.3). Gray-matter abnormalities (present in 49 percent of infants) were also associated, but less strongly, with cognitive delay, motor delay, and cerebral palsy. Moderate-to-severe white-matter abnormalities on MRI were significant predictors of severe motor delay and cerebral palsy after adjustment for other measures during the neonatal period, including findings on cranial ultrasonography. CONCLUSIONS: Abnormal findings on MRI at term equivalent in very preterm infants strongly predict adverse neurodevelopmental outcomes at two years of age. These findings suggest a role for MRI at term equivalent in risk stratification for these infants.

    PMID: ( MRI and CP ) ] 16914704 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Topography of dopamine transporter availability in progressive supranuclear palsy: a voxelwise [123I]beta-CIT SPECT analysis.
    Related Articles

    Topography of dopamine transporter availability in progressive supranuclear palsy: a voxelwise [123I]beta-CIT SPECT analysis.

    Arch Neurol. 2006 Aug;63(8):1154-60

    Authors: Seppi K, Scherfler C, Donnemiller E, Virgolini I, Schocke MF, Goebel G, Mair KJ, Boesch S, Brenneis C, Wenning GK, Poewe W

    BACKGROUND: Dopaminergic loss can be visualized by means of iodine I 123-labeled 2beta-carbomethoxy-3beta-(4-iodophenyl)tropane ([(123)I]beta-CIT) single-photon emission computed tomography (SPECT) in several neurodegenerative parkinsonian disorders. Most previous SPECT studies have adopted region-of-interest methods for analysis, which are subjective and operator dependent. OBJECTIVE: To objectively localize the cerebral dopamine transporter status in the early stages of progressive supranuclear palsy (PSP). DESIGN: Prospective study. SETTING: Parkinson disease outpatient clinic. PATIENTS: Fourteen patients with PSP, 17 with Parkinson disease (PD), 15 with Parkinson-variant multiple-system atrophy (MSA-P), and 13 healthy control subjects, matched for age and disease duration. INTERVENTIONS: Statistical parametric mapping applied to [(123)I]beta-CIT SPECT. MAIN OUTCOME MEASURES: Differences in [(123)I]beta-CIT uptake. RESULTS: All patients with the different parkinsonian disorders showed a significant decrease in striatal [(123)I]beta-CIT uptake without any overlap with the control group. In patients with MSA-P and PSP, an additional reduction in brainstem [(123)I]beta-CIT signal compared with controls and patients with PD was identified with statistical parametric mapping. Midbrain [(123)I]beta-CIT uptake discriminated atypical parkinsonian disorders from PD with an overall correct classification of 91.3%. On the other hand, [(123)I]beta-CIT SPECT failed to discriminate PSP and MSA-P. CONCLUSION: By applying statistical parametric mapping to [(123)I]beta-CIT SPECT images of patients with PSP, a widespread decline of monoaminergic transporter availability including the striatum and brainstem was localized in PSP, discriminating patients with PSP from patients with PD, but not from those with MSA-P. Quantification of midbrain dopamine transporter signal may therefore enhance the utility of SPECT imaging in the differential diagnosis of patients with parkinsonism.

    PMID: ( MRI and CP ) ] 16908744 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Cerebral palsy in a term population: risk factors and neuroimaging findings.
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    Cerebral palsy in a term population: risk factors and neuroimaging findings.

    Pediatrics. 2006 Aug;118(2):690-7

    Authors: Wu YW, Croen LA, Shah SJ, Newman TB, Najjar DV

    OBJECTIVE: The purpose of this work was to study risk factors and neuroimaging characteristics of cerebral palsy in term and near-term infants. PATIENTS AND METHODS: Among a cohort of 334,339 infants > or = 36 weeks' gestation born at Kaiser Permanente Medical Care Program in northern California in 1991-2003, we identified infants with cerebral palsy and obtained clinical data from electronic and medical charts. Risk factors for cerebral palsy among infants with different brain abnormalities were compared using polytomous logistic regression. RESULTS: Of 377 infants with cerebral palsy (prevalence: 1.1 per 1000), 273 (72%) received a head computed tomography or MRI. Abnormalities included focal arterial infarction (22%), brain malformation (14%), and periventricular white matter abnormalities (12%). Independent risk factors for cerebral palsy were maternal age > 35, black race, and intrauterine growth restriction. Intrauterine growth restriction was more strongly associated with periventricular white matter injury than with other neuroimaging findings. Nighttime delivery was associated with cerebral palsy accompanied by generalized brain atrophy but not with cerebral palsy accompanied by other brain lesions. CONCLUSIONS: Cerebral palsy is a heterogeneous syndrome with focal arterial infarction and brain malformation representing the most common neuroimaging abnormalities in term and near-term infants. Risk factors for cerebral palsy differ depending on the type of underlying brain abnormality.

    PMID: ( MRI and CP ) ] 16882824 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • A new autosomal recessive spastic ataxia associated with frequent white matter changes maps to 2q33-34.
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    A new autosomal recessive spastic ataxia associated with frequent white matter changes maps to 2q33-34.

    Brain. 2006 Sep;129(Pt 9):2332-40

    Authors: Thiffault I, Rioux MF, Tetreault M, Jarry J, Loiselle L, Poirier J, Gros-Louis F, Mathieu J, Vanasse M, Rouleau GA, Bouchard JP, Lesage J, Brais B

    Recessive ataxias are a heterogeneous group of diseases. We identified a group of 23 French-Canadian cases belonging to 17 families affected by an autosomal recessive spastic ataxia associated with frequent white matter changes. The fact that 59% of these families have a genealogical relationship to the Portneuf County of Quebec suggests that this is a new form of ataxia with a regional founder effect. All cases present with cerebellar ataxia and spasticity. There is great intrafamilial and interfamilial variability, as illustrated by the spectrum of age of diagnosis (range: 2-59 years, mean: 15.0) and the presence of white matter changes on MRI in 52.4% of cases. The more severe cases have spasticity from birth, scoliosis, dystonia and cognitive impairment and were considered cases of cerebral palsy. Brain MRI constantly shows cerebellar atrophy, which in some cases may be associated with cortical atrophy, leucoencephalopathy and corpus callosum thinning. A genome wide scan uncovered linkage of three families to marker D2S2321 localized on chromosome 2q33-34. Linkage analysis confirmed that all families are linked to the same region [multipoint log of the odds (LOD) score of 5.95]. Haplotype analysis and allele sharing suggest that one common mutation may account for 97% of carrier chromosomes in Quebec. The uncovering of the mutated gene may point to a common pathway for pyramidal and cerebellar degeneration as both are often observed in recessive ataxias and complicated paraplegias.

    PMID: ( MRI and CP ) ] 16672289 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Sydenham's chorea in western Pennsylvania.
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    Sydenham's chorea in western Pennsylvania.

    Pediatrics. 2006 Apr;117(4):e675-9

    Authors: Zomorrodi A, Wald ER

    OBJECTIVE: Chorea is characterized by involuntary, fleeting, irregular, nonrhythmic movements that flow from 1 body region to another. There are many causes of childhood chorea, including cerebrovascular accidents, collagen vascular diseases, drug intoxication, hyperthyroidism, Wilson's disease, Huntington's disease, and infectious agents. Although Sydenham's chorea (SC), a nonsuppurative sequela of group A streptococcal infection, is known to be a common cause of chorea, multiple laboratory and radiographic studies are often obtained to determine the cause of pediatric chorea. We conducted a retrospective chart review to determine the causes of childhood chorea seen in a large children's hospital in an area endemic for acute rheumatic fever (ARF). The utility of neuroimaging in establishing a final diagnosis of SC is discussed. METHODS: Patients who received a diagnosis of chorea between 1980 and 2004 at the Children's Hospital of Pittsburgh were identified from databases that are maintained by the divisions of Infectious Diseases and Cardiology and from the hospital's medical records department. Charts were abstracted retrospectively. All patients who had new-onset chorea and did not have any underlying neurologic disorders were included in this study. Patient demographic, clinical, laboratory, and imaging information was analyzed. Follow-up information was not found consistently and therefore was not included. Charts of patients with questionable diagnoses were reviewed with a neurologist. RESULTS: A total of 144 patients met the search criterion. Eleven patients had incomplete charts, and 6 charts could not be located. Thirty patients were excluded because they had preexisting neurologic diagnoses, eg, cerebral palsy. Fifteen patients were excluded because they were miscoded as having chorea. Eighty-two patients had new-onset chorea. The cause was SC (n = 79), postoperative cerebral ischemia (n = 1), and basal ganglion infarct (n = 2). Seventy-six (71%) children with SC were female. The mean age of presentation was 9.8 years (range: 5-14.5 years). Chorea was unilateral in 23 (30%) patients. Family history of ARF existed in 30% of patients. Neurologic symptoms of SC included behavior change (46%), dysarthria (67%), gait change (51%), deterioration of handwriting (29%), and headache (11%). Nonneurologic manifestations of ARF were carditis (44%), arthritis (11%), erythema marginatum (3%), and subcutaneous nodules (0%). Antecedent group A streptococcal infection was documented in 99% of patients who were tested by an elevated antistreptolysin O titer (n = 53), an elevated anti-deoxyribonuclease B titer (n = 7), a positive streptozyme (n = 53), or acute throat infection with Streptococcus pyogenes (n = 19). A total of 52 neuroimaging tests were obtained from 46 patients with SC. In patients with SC, brain MRI was abnormal in 8 of 32 patients, and brain computed tomography was abnormal in 1 of 20 patients. Abnormalities did not aid in diagnosis and included nonspecific increased signal (n = 2), nonspecific punctate lesions (n = 2), asymmetry of the hippocampal fissures, unrelated petrous bone anomaly, Arnold Chiari malformation, and medulloblastoma in a macrocephalic patient. Three patients with chorea that was not attributed to ARF had atypical presentations: 1 developed chorea after a perioperative hypoxic/ischemic central nervous system insult; 1 had an episode of disorientation, aphasia, and transient facial droop (angiography showed basal ganglia infarct); and 1 with hemichorea had basal ganglion infarct seen on MRI. CONCLUSIONS: Ninety-six percent of children who had acute chorea and presented to a large children's hospital in an area that is endemic for ARF had SC. These patients had characteristic demographic and clinical features of SC. The most common concurrent major Jones criterion was carditis. Arthritis, erythema marginatum, and subcutaneous nodules were uncommon in this population. Neuroimaging was obtained in 58% of patients with SC and did not aid in any of their diagnoses. The 3 patients with chorea that was not caused by SC had histories that were atypical for SC and warranted neuroimaging. SC can be readily diagnosed on the basis of history, physical examination, and laboratory evaluation; neuroimaging is not necessary and should be reserved for patients who have an atypical presentation, including hemichorea.

    PMID: ( MRI and CP ) ] 16533893 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Venous infarction secondary to septic cavernous sinus thrombosis.
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    Venous infarction secondary to septic cavernous sinus thrombosis.

    Intern Med. 2006;45(1):25-7

    Authors: Kamouchi M, Wakugawa Y, Okada Y, Kishikawa K, Matsuo R, Toyoda K, Yasumori K, Inoue T, Ibayashi S, Iida M

    A 65-year-old woman with poorly controlled diabetes presented bilateral miosis, bilateral abducens nerve palsy, and left hemiparesis. On MRI, cavernous sinus thrombosis, subdural empyema and hemorrhagic infarction in the frontotemporal lobe were detected. Cerebral angiogram revealed filling defect in the cavernous sinus with venous congestion but no involvement of internal carotid artery. Postmortem examination demonstrated hemorrhagic infarction in the right frontotemporal lobe as well as hemorrhagic necrosis of the pituitary gland. It should be noted that venous congestion due to cavernous sinus thrombosis may cause these complications.

    PMID: ( MRI and CP ) ] 16467601 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • EEG spectral analysis and coherence in children with hemiparetic cerebral palsy.
    Related Articles

    EEG spectral analysis and coherence in children with hemiparetic cerebral palsy.

    Med Sci Monit. 2005 Sep;11(9):CR449-55

    Authors: Kułak W, Sobaniec W, Boćkowski L

    BACKGROUND: Hemiparetic cerebral palsy (HCP) is described as having two main forms: arm-dominant, associated with large cortical/subcortical lesions and leg-dominant, associated with central white matter lesions. MATERIAL/METHODS: Twelve children with HCP underwent clinical assessment and imaging studies. For each child, 20 artifact-free EEG epochs, each of 2s duration, were selected for spectral analysis and coherence functions. The objective of this research was to estimate EEG spectral power, interhemispheric coherence (ICoh), and intrahemispheric (Hcoh) coherence in children with HCP as compared to healthy children. RESULTS: Significant differences between the HCP and control children were detected in the distribution of alpha, theta, delta and beta rhythms over the left and right hemispheres. The ICoh values in the alpha band in the temporal, parietal and occipital regions were significantly lower in the HCP patients than in the controls. There was a significant ICoh increase in the HCP in the theta and delta band, involving frontal and temporal derivations. The significantly lower ICoh values in the HCP children in the beta band involved the frontal, central, parietal and occipital derivations. A higher HCoh value in the HCP children in the alpha band was detected at the right hemisphere. CONCLUSIONS: The lower ICoh at the temporal, parietal and occipital derivations in the alpha band implies hypoconnectivity between the right and left hemispheres. HCoh asymmetry, which implies relative hypoconnectivity within the left hemisphere as compared with the right, suggests that functional hemispheric differentiation may be diminished.

    PMID: ( MRI and CP ) ] 16127366 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Congenital bilateral severe microphthalmia with mental retardation and cerebral palsy: chromosome aberration, 46, XY, t (2;6)(q31;q24).
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    Congenital bilateral severe microphthalmia with mental retardation and cerebral palsy: chromosome aberration, 46, XY, t (2;6)(q31;q24).

    J Nippon Med Sch. 2005 Aug;72(4):242-4

    Authors: Hirayama T, Kobayashi T, Fujino O

    Congenital bilateral anophthalmia and microphthalmia are rare conditions, with overall prevalence in one study set at 1.0 per 10,000 births. We report here a case of congenital bilateral severe microphthalmia with mental retardation and cerebral palsy. The patient was man aged 38 years with a chromosome aberration, namely a balanced translocation: 46, XY, t (2;6)(q31;q24). He had no other malformations apart from the severe microphthalmia. CT of the head showed no significant abnormal findings in the brain, but rudimentary eyeballs and external ocular muscles in the bilateral orbits. There was no family history of anophthalmia, microphthalmia, mental retardation or cerebral palsy. His mother had not used any medications or excessive alcohol during gestation. Putative genes of anophthalmia and microphthalmia reported to date include PAX6 (Glaser T et al 1994) and CHX10 (Ferda Percin E et al 2000). Further, some loci of these conditions have been reported (Graham CA et al 1991; Bessant DAR et al 1998; Morle L et al 2000: Forrester S et al 2001: Ng D et al 2002). To our knowledge, however, this is the first report of nonsyndromic microphthalmia or anophthalmia with chromosome 2q31 or 6q24 aberration. We consider that the putative gene may be located on the brake points of chromosome 2 and 6.

    PMID: ( MRI and CP ) ] 16113496 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Systemic prenatal insults disrupt telencephalon development: implications for potential interventions.
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    Systemic prenatal insults disrupt telencephalon development: implications for potential interventions.

    Epilepsy Behav. 2005 Nov;7(3):345-63

    Authors: Robinson S

    Infants born prematurely are prone to chronic neurologic deficits including cerebral palsy, epilepsy, cognitive delay, behavioral problems, and neurosensory impairments. In affected children, imaging and neuropathological findings demonstrate significant damage to white matter. The extent of cortical damage has been less obvious. Advances in the understanding of telencephalon development provide insights into how systemic intrauterine insults affect the developing white matter, subplate, and cortex, and lead to multiple neurologic impairments. In addition to white matter oligodendrocytes and axons, other elements at risk for perinatal brain injury include subplate neurons, GABAergic neurons migrating through white matter and subplate, and afferents of maturing neurotransmitter systems. Common insults including hypoxia-ischemia and infection often affect the developing brain differently than the mature brain, and insults precipitate a cascade of damage to multiple neural lineages. Insights from development can identify potential targets for therapies to repair the damaged neonatal brain before it has matured.

    PMID: ( MRI and CP ) ] 16061421 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Quantitative diffusion tensor imaging in cerebral palsy due to periventricular white matter injury.
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    Quantitative diffusion tensor imaging in cerebral palsy due to periventricular white matter injury.

    Brain. 2005 Nov;128(Pt 11):2562-77

    Authors: Thomas B, Eyssen M, Peeters R, Molenaers G, Van Hecke P, De Cock P, Sunaert S

    Periventricular white matter injury (PWI) is a major form of brain injury observed in congenital hemiparesis. The aim of this study is to determine the usefulness of diffusion tensor imaging (DTI) and fibre tracking in delineating the primary and secondary degenerative changes in cerebral white matter and deep grey matter in patients with spastic cerebral palsy due to PWI and to look for any possible reorganization of the axonal architecture. Five hemiparetic cerebral palsy patients (median age 14 years) with known PWI were prospectively studied with DTI of the brain at 1.5T and quantitatively compared with five age and sex matched controls. Fibre tracts for various corticofugal, thalamocortical and association tracts were generated and analysed for the DTI fibre count and for diffusion parameters. A region of interest based analysis was performed for the directionally averaged mean diffusivity (D(av)) and fractional anisotropy (FA) values in various white matter locations in the brain and the brainstem and in the deep grey matter nuclei. Group statistics were performed for these parameters using Mann-Whitney U-test comparing the affected sides in patients with either side in controls and the unaffected side in hemiparetics. There was significant reduction in DTI fibre count on the lesional side involving corticospinal tract (CST), corticobulbar tract (CBT) and superior thalamic radiation in the patient group compared with controls. Also there was an increase in DTI fibre count in the unaffected side of the hemiparetic patients in CST and CBT, which reached statistical significance only in CBT. The corpus callosum, cingulum, superior longitudinal fasciculus and middle cerebellar peduncle failed to show any significant change. ROI measurements on the primary site of white matter lesion and the thalamus revealed a significant increase in D(av) and decrease in FA, suggesting primary degeneration. The CST in the brainstem, the body of corpus callosum and the head of caudate and lentiform nuclei showed features of secondary degeneration on the affected side. The CST on the unaffected side of hemiparetics was found to have a significant decrease in D(av) and an increase in FA. Thus the degeneration of various motor and sensory pathways, as well as deep grey matter structures, appears to be important in determining the pathophysiological mechanisms in patients with congenital PWI. Also evidence suggesting the reorganization of sensorimotor tracts in the unaffected side of spastic hemiparetic patients was noted.

    PMID: ( MRI and CP ) ] 16049045 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Isolated oculomotor nerve palsy in Churg-Strauss syndrome.
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    Isolated oculomotor nerve palsy in Churg-Strauss syndrome.

    Intern Med. 2005 Jun;44(6):638-40

    Authors: Tsuda H, Ishikawa H, Majima T, Sawada U, Mizutani T

    A 30-year-old man with bronchial asthma complained of horizontal diplopia. Partial oculomotor nerve palsy with restrictions of elevation and adduction, and mydriasis was observed in the left eye. Cranial magnetic resonance imaging demonstrated an infarct lesion in the territory of the left superior median mesencephalic branch of the posterior cerebral artery. Based on bronchial asthma, hypereosinophilia, mononeuropathy multiplex, pulmonary eosinophilia and positive perinuclear antineutrophil cytoplasmic antibody in the serum, the patient was diagnosed as having Churg-Strauss syndrome. This is the first case of oculomotor nerve palsy due to midbrain infarction associated with Churg-Strauss syndrome.

    PMID: ( MRI and CP ) ] 16020896 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Prognostic clinical variables in childhood tuberculous meningitis: an experience from Mumbai, India.
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    Prognostic clinical variables in childhood tuberculous meningitis: an experience from Mumbai, India.

    Neurol India. 2005 Jun;53(2):191-5; discussion 195-6

    Authors: Karande S, Gupta V, Kulkarni M, Joshi A

    BACKGROUND: In India, tuberculous meningitis (TBM) is still a major cause of neurological disabilities and death. AIM: To identify the clinical variables which predict the outcome in childhood TBM. SETTING: Tertiary teaching hospital. DESIGN: Prospective observational study. MATERIALS AND METHODS: Thirty-six clinical variables were analyzed in 123 consecutive children with TBM admitted between May 2000 and August 2003. The outcome was assessed in terms of survival or death. Survival meant that the patient was discharged from hospital having made a complete recovery, or with disability. RESULTS: Twenty-five (20%) children recovered completely, 70 (57%) survived with disability, and 28 (23%) died. Employing univariate analysis nine variables correlated with survival with disability outcome: presence of tonic motor posturing, cranial nerve palsy, focal neurological deficit, hypertonia, moderate to severe hydrocephalus, cerebral infarction on cranial CT, and requiring shunt surgery, and absence of extracranial tuberculosis and no antituberculous-related hepatotoxicity; two variables correlated with fatal outcome: presence of deep coma (Glasgow coma scale score P = 0.012, d.f. = 1, OR 0.12, 95% CI 0.02-0.62) correlated with survival with disability outcome, and presence of deep coma (P = 0.030, d.f. = 1, OR 0.35, 95% CI 0.14-0.90) with fatal outcome. CONCLUSION: In children with TBM, the presence of hypertonia at admission is an independent predictor of neurological sequelae in survivors, and deep coma is an independent predictor of mortality.

    PMID: ( MRI and CP ) ] 16010058 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Apraxia in movement disorders.
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    Apraxia in movement disorders.

    Brain. 2005 Jul;128(Pt 7):1480-97

    Authors: Zadikoff C, Lang AE

    The definition of apraxia specifies that the disturbance of performed skilled movements cannot be explained by the more elemental motor disorders typical of patients with movement disorders. Generally this does not present a significant diagnostic problem when dealing with 'higher-level' praxic disturbances (e.g. ideational apraxia), but it can be a major confound in establishing the presence of limb-kinetic apraxia. Most motor disturbances characteristic of extrapyramidal disorders, particularly bradykinesia and dystonia, will compromise the ability to establish the presence of loss of dexterity and deftness that constitutes this subtype. The term 'apraxia' has also been applied to other motor disturbances, such as 'gait apraxia' and 'apraxia of eyelid opening', that perhaps are misnomers, demonstrating the lack of a coherent nomenclature in this field. Apraxia is a hallmark of corticobasal degeneration (CBD) and historically this has received the most attention among the movement disorders. Corticobasal degeneration is characterized by various forms of apraxia affecting limb function, particularly ideomotor apraxia and limb-kinetic apraxia, although buccofacial and oculomotor apraxia can be present as well. The syndrome of parkinsonism and prominent apraxia, designated the 'corticobasal syndrome' (CBS), may be caused by a variety of other central nervous system pathologies including progressive supranuclear palsy (PSP), Alzheimer's disease, dementia with Lewy bodies and frontotemporal dementias. Distinct from the CBS, PSP and Parkinson's disease can demonstrate varying degrees of apraxia on selected tests, especially in those patients with more severe cognitive dysfunction. Diseases that cause the combination of apraxia and a primary movement disorder most often involve a variety of cerebral cortical sites as well as basal ganglia structures. Clinical-pathological correlates and functional imaging studies are compromised by both this diffuse involvement and the confusion experienced in the clinical evaluation of apraxia in the face of the additional elemental movement disorders. Finally, although apraxia results in clear disability in patients with the CBS, it is not clear how milder ideomotor apraxia found on specific testing contributes to patients' overall day-to-day motor disability.

    PMID: ( MRI and CP ) ] 15930045 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Clinical deficits correlate with regional cerebral atrophy in progressive supranuclear palsy.
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    Clinical deficits correlate with regional cerebral atrophy in progressive supranuclear palsy.

    Brain. 2005 Jun;128(Pt 6):1259-66

    Authors: Cordato NJ, Duggins AJ, Halliday GM, Morris JG, Pantelis C

    Most cerebral imaging studies of patients with progressive supranuclear palsy (PSP) have noted subtle atrophy, although the full extent of atrophy and any correlates to clinical features have not been determined. We used voxel-based morphometry analysis of grey matter, white matter and CSF on MRI brain scans to map the statistical probability of regional tissue atrophy in 21 patients with PSP, 17 patients with Parkinson's disease and 23 controls. PSP and Parkinson's disease cohorts were selected to approximate the mid-stages of their respective disease courses. Where regions of significant tissue atrophy were identified in a disease group relative to controls, the probability of tissue loss within those regions was correlated with global indices of motor disability, and behavioural and cognitive disturbance for that disease group. Minimal regional atrophy was observed in Parkinson's disease. PSP could be distinguished from both controls and Parkinson's disease by symmetrical tissue loss in the frontal cortex (maximal in the orbitofrontal and medial frontal cortices), subcortical nuclei (midbrain, caudate and thalamic) as well as periventricular white matter. For PSP, motor deficits correlated with atrophy of the caudate and motor cingulate, while behavioural changes related to atrophy in the orbitofrontal cortex and midbrain. These data suggest that intrinsic neurodegeneration of specific subcortical nuclei and frontal cortical subregions together contribute to motor and behavioural disturbances in PSP and differentiate this disorder from Parkinson's disease within 2-4 years of symptom onset.

    PMID: ( MRI and CP ) ] 15843423 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Primary cerebral angiitis containing marked xanthoma cells with massive intraparenchymal involvement--case report--.
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    Primary cerebral angiitis containing marked xanthoma cells with massive intraparenchymal involvement--case report--.

    Neurol Med Chir (Tokyo). 2005 Mar;45(3):156-60

    Authors: Ishikawa E, Tsuboi K, Takano S, Kimura H, Aoki T, Mashiko R, Nagata M

    A 27-year-old woman was referred to our hospital with mild disorientation, bilateral abducens nerve palsy, and mild left hemiparesis. Magnetic resonance (MR) imaging revealed diffuse mass lesions resembling malignant glioma in the right frontal intraparenchymal region, with enhancement of multiple meningeal and intraparenchymal nodules. Partial resection of the frontal lesion was performed. Histological examination revealed that the specimens consisted of brain tissue, with marked perivascular infiltration of histiocytes and sheets of xanthomatous cells. The diagnosis was primary cerebral angiitis containing marked xanthoma cells. Steroid therapy was administered over 1 week. MR imaging showed that the remaining lesions resolved gradually, and had disappeared 2 years after surgery. No neurological symptoms or recurrence of the tumor has been observed during the 6-year period since the operation.

    PMID: ( MRI and CP ) ] 15782008 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Incomplete oculomotor nerve palsy caused by an unruptured internal carotid-anterior choroidal artery aneurysm--case report--.
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    Incomplete oculomotor nerve palsy caused by an unruptured internal carotid-anterior choroidal artery aneurysm--case report--.

    Neurol Med Chir (Tokyo). 2005 Mar;45(3):143-7

    Authors: Kurokawa Y, Ishizaki E, Inaba K

    A 59-year-old woman visited our institute with the chief complaint of dizziness which persisted whenever she tried to focus on objects. She had not experienced apparent double vision and had no history of intracranial bleeding. Neurological examination revealed no abnormality except for exotropia at the mid-position and at upper gaze. Cerebral angiography revealed that the intracranial portion of the left internal carotid artery ran more horizontally and also identified an unruptured left internal carotid-anterior choroidal artery (IC-AChA) aneurysm of 3.0 mm diameter. The aneurysm at the origin of the AChA was confirmed during surgery. The proximal lateral wall of the aneurysm was in contact with the oculomotor nerve. This contact was released after complete obliteration of the aneurysm. The exotropia resolved 3 months later. Oculomotor nerve palsy usually indicates the presence of internal carotid-posterior communicating artery (IC-PcomA) aneurysm. Since sacrifice of the AChA will result in severe neurological deficits, accurate neuroimaging information is needed prior to the operation. Conventional angiography and/or three-dimensional computed tomography angiography should be performed to ascertain whether the aneurysm is an IC-PcomA or IC-AChA aneurysm, even if some neurosurgeons insist that conventional angiography is not always needed before surgery for an unruptured aneurysm.

    PMID: ( MRI and CP ) ] 15782005 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Infantile spasms: neurological and developmental follow-up--a comparison between two ethnic groups: Israeli Jews and Bedouin in the South of Israel.
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    Infantile spasms: neurological and developmental follow-up--a comparison between two ethnic groups: Israeli Jews and Bedouin in the South of Israel.

    Med Sci Monit. 2005 Mar;11(3):CR117-22

    Authors: Vardi G, Snapir SM, Merick J, Shorer Z, Levy J, Friger M, Galil A

    BACKGROUND: The aim of this study was to examine all cases of infantile spasms (IS) diagnosed at the Soroka University Medical Center, Ben Gurion University, Israel during 1981--1997. MATERIAL/METHODS: 31 children, 17 (55%) males. 17 (55%) were Jews and 14 (45%) Bedouins. Four (13%) died. Data was gathered from hospital files, neuropediatric unit and Zusman Child Development Center. Demographic and ethnic data, characteristics of the disease type of seizures, EEG pattern imaging studies, type of treatment, psychomotor development, rehabilitation and educational services were analysed. RESULTS: Mean age at diagnosis 7.22 months. Etiology for one third was pre- or perinatal insult, one third postnatal and one third unknown. 26 (84%) were symptomatic and five (16%) cryptogenic. Significant statistical difference was found with more Bedouin children symptomatic with moderate or severe mental retardation, cerebral palsy with severe motor difficulties and recurrence of the disease. Statistically significant correlation existed between poor response to initial treatment and placement in special education, recurrence of disease and cerebral palsy with moderate or severe motor difficulties, the appearence of a different type of epilepsy during follow-up and placement in special education. 18 children (58%) received first treatment with ACTH, 10 (32%) children with IVIG (immunoglobolin iv), 3 (10%) with neither ACTH nor IVIG. Regarding therapy response we found no significant difference between Jews and Bedouins. CONCLUSIONS: No significant statistical difference was found between the two treatments concerning clinical course or developmental follow-up. Due to small numbers multi-center research is needed.

    PMID: ( MRI and CP ) ] 15735563 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Maternal and infant characteristics associated with perinatal arterial stroke in the infant.
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    Maternal and infant characteristics associated with perinatal arterial stroke in the infant.

    JAMA. 2005 Feb 9;293(6):723-9

    Authors: Lee J, Croen LA, Backstrand KH, Yoshida CK, Henning LH, Lindan C, Ferriero DM, Fullerton HJ, Barkovich AJ, Wu YW

    CONTEXT: Perinatal arterial ischemic stroke (PAS) is a common cause of hemiplegic cerebral palsy. Risk factors for this condition have not been clearly defined. OBJECTIVE: To determine maternal and infant characteristics associated with PAS. DESIGN, SETTING, AND PATIENTS: Case-control study nested within the cohort of all 199,176 infants born from 1997 through 2002 in the Kaiser Permanente Medical Care Program, a managed care organization providing care for more than 3 million residents of northern California. Case patients were confirmed by review of brain imaging and medical records (n = 40). Three controls per case were randomly selected from the study population. MAIN OUTCOME MEASURE: Association of maternal and infant complications with risk of PAS. RESULTS: The population prevalence of PAS was 20 per 100,000 live births. The majority (85%) of infants with PAS were delivered at term. The following prepartum and intrapartum factors were more common among case than control infants: primiparity (73% vs 44%, P = .002), fetal heart rate abnormality (46% vs 14%, P<.001), emergency cesarean delivery (35% vs 13%, P = .002), chorioamnionitis (27% vs 11%, P = .03), prolonged rupture of membranes (26% vs 7%, P = .002), prolonged second stage of labor (25% vs 4%, P<.001), vacuum extraction (24% vs 11%, P = .04), cord abnormality (22% vs 6%, P = .01), preeclampsia (19% vs 5%, P = .01), and oligohydramnios (14% vs 3%, P = .01). Risk factors independently associated with PAS on multivariate analysis were history of infertility (odds ratio [OR], 7.5; 95% confidence interval [CI], 1.3-45.0), preeclampsia (OR, 5.3; 95% CI, 1.3-22.0), prolonged rupture of membranes (OR, 3.8; 95% CI, 1.1-12.8), and chorioamnionitis (OR, 3.4; 95% CI, 1.1-10.5). The rate of PAS increased dramatically when multiple risk factors were present. CONCLUSIONS: Perinatal arterial ischemic stroke in infants is associated with several independent maternal risk factors. How these complications, along with their potential effects on the placenta and fetus, may play a role in causing perinatal stroke deserves further study.

    PMID: ( MRI and CP ) ] 15701914 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Diffusion-tensor MR imaging and fiber tractography: a new method of describing aberrant fiber connections in developmental CNS anomalies.
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    Diffusion-tensor MR imaging and fiber tractography: a new method of describing aberrant fiber connections in developmental CNS anomalies.

    Radiographics. 2005 Jan-Feb;25(1):53-65; discussion 66-8

    Authors: Lee SK, Kim DI, Kim J, Kim DJ, Kim HD, Kim DS, Mori S

    Congenital anomalies of the central nervous system (CNS) often demonstrate aberrant white matter connections, which may be better characterized with diffusion-tensor imaging (DTI) and fiber tractography (FT) than with conventional magnetic resonance (MR) imaging. DTI-FT demonstrates abnormal hemispheric fiber connections in callosal agenesis or acquired disease of the corpus callosum. Decreased anisotropy of white matter adjacent to the malformed cortex and an aberrant course of major fiber pathways due to dysplastic white matter are common findings in cortical dysplasia. Increased anisotropy of dysplastic gray matter in heterotopia supports the hypothesis that developing neurons migrate from the ependyma to the cortex with a radial growth pattern. In periventricular leukomalacia, DTI-FT demonstrates an intact corticospinal tract and decreased thalamocortical sensory connections, which are responsible for the spasticity of cerebral palsy owing to impairment of inhibitory function. Joubert syndrome comprises malformation of the cerebellar vermis and an aberrant connection between the cerebellum and the cerebral cortex via an elongated and abnormally shaped superior cerebellar peduncle, which are well visualized with DTI-FT. In developmental CNS disease, DTI-FT demonstrates additional findings beyond those seen with conventional MR imaging. Future studies will focus on determining the significance of the aberrant fiber connections and their relationships to the clinical manifestations of CNS anomalies.

    PMID: ( MRI and CP ) ] 15653586 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Characteristics of six newborn infants with postnatal findings of severe intracranial haemorrhage.
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    Characteristics of six newborn infants with postnatal findings of severe intracranial haemorrhage.

    Ann Acad Med Singapore. 2004 Nov;33(6):789-92

    Authors: Wong JP, Seow WT, Yeo GS

    OBJECTIVE: The objective of this study was to study the characteristics of newborn infants with postnatal findings of severe neonatal intracranial haemorrhage. METHODS: All the records of babies who underwent surgery from 1997 to 2002 for intracranial haemorrhage were reviewed. These were correlated with their antenatal records to see if fetal intracranial haemorrhage had been detected at the 20 weeks' screening scan or any other incidental scan e.g. growth scan. The perinatal records were also reviewed to see if there was associated birth trauma such as instrumentation or obstetric manoeuvres at delivery. RESULTS: Six cases of severe intracranial haemorrhage were diagnosed postnatally. Of these, only 1 case was detected antenatally on ultrasound scan. None of the cases were due to birth trauma. Three babies were found to have clotting factor deficiency. One of them subsequently developed cerebral palsy. One baby was diagnosed to have alloimmune thrombocytopenia. One case underwent an emergency Caesarean section for non-reassuring fetal status. Extensive intracranial haemorrhage, attributed to hypoxia, was found. The baby died. CONCLUSIONS: Our study suggests that neonatal intracranial haemorrhages are not exclusively due to birth trauma. The study also shows that fetal intracranial haemorrhage may not be detected antenatally by the routine practice. The causes in our study included clotting deficiency, alloimmune thrombocytopenia and hypoxia.

    PMID: ( MRI and CP ) ] 15608840 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Correlation between antemortem magnetic resonance imaging findings and pathologically confirmed corticobasal degeneration.
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    Correlation between antemortem magnetic resonance imaging findings and pathologically confirmed corticobasal degeneration.

    Arch Neurol. 2004 Dec;61(12):1881-4

    Authors: Josephs KA, Tang-Wai DF, Edland SD, Knopman DS, Dickson DW, Parisi JE, Petersen RC, Jack CR, Boeve BF

    BACKGROUND: Slowly progressive asymmetric parkinsonism and cortical dysfunction clinically characterize corticobasal syndrome (CBS). Various pathologic findings, including corticobasal degeneration (CBD), progressive supranuclear palsy, and frontotemporal degenerations, underlie CBS. OBJECTIVE: To determine if regional cortical and corpus callosum atrophy and subcortical and periventricular white matter (SPWM) signal changes on head magnetic resonance imaging were specific to CBD. DESIGN: Historical review of autopsy cases. SETTING: Subspecialized behavioral neurology and movement disorder clinics within a neurology department of a tertiary referral center. PATIENTS: Seventeen patients with CBS who had an autopsy-confirmed diagnosis of CBD or another neurodegenerative disease. MAIN OUTCOME MEASURES: Regional cerebral cortical atrophy, regional corpus callosum atrophy, and SPWM signal changes. RESULTS: Similar patterns of regional atrophy and SPWM signal changes were found in the patients with autopsy-proven CBD and in the patients with other neurodegenerative diseases. CONCLUSION: Neither cortical nor corpus callosum atrophy nor SPWM signal changes on head magnetic resonance imaging are specific to CBD.

    PMID: ( MRI and CP ) ] 15596608 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Presentation and outcome of rhino-orbital-cerebral mucormycosis in patients with diabetes.
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    Presentation and outcome of rhino-orbital-cerebral mucormycosis in patients with diabetes.

    Postgrad Med J. 2004 Nov;80(949):670-4

    Authors: Bhansali A, Bhadada S, Sharma A, Suresh V, Gupta A, Singh P, Chakarbarti A, Dash RJ

    AIM: To report presentation and outcome of rhino-orbital-cerebral mucormycosis (ROCM) exclusively in patients with diabetes mellitus. METHODS: Retrospective, non-comparative, interventional analysis of the medical records of 35 patients with ROCM among 22 316 patients with diabetes seen over the last 12 years. RESULTS: A cohort of 23 men and 12 women with a mean (SD) age of 47.3 (14.4) years (range 18-70 years) was studied. Five patients had type 1 diabetes mellitus, 29 had type 2 diabetes mellitus, and one had secondary diabetes. Nine patients had ROCM as the first clinical manifestation of diabetes. The mean (SD) blood glucose at presentation was 20.6 (8.3) mmol/l (range 10.0 to 53.3 mmol/l) and 17 patients had ketosis/ketoacidosis. Ophthalmic symptoms and signs were pronounced: external ophthalmoplegia (89%), proptosis (83%), visual loss (80%), chemosis (74%), and eye lid gangrene (14%). Non-ophthalmic manifestations included sinusitis (100%), nasal discharge/ulceration (74%), infranuclear VI nerve palsy (46%), palatal necrosis (29%), cerebral lobe involvement (20%), and hemiparesis (17%). Computed tomography/magnetic resonance imaging showed involvement of paranasal sinuses in all patients with ethmoid (86%) and maxillary (80%) sinuses being most frequently involved. Orbital involvement was observed in 80% of patients with cavernous sinus thrombosis in 11%, and internal carotid occlusion and hydrocephalus in 3% each. All were treated with amphotericin B (3-3.5 g) and 26 (74%) patients underwent appropriate surgery. Twenty one patients (68%) survived with a mean (SD) follow up of 39.6 (34.1) months (range 10 months to 11 years). Factors related to poor survival included delay in diagnosis and treatment (p<0.05), facial and/or eye lid gangrene (p<0.05), hemiplegia (p<0.05), cerebral invasion by mucorales (p<0.05), and treatment with amphotericin B alone (p<0.05). CONCLUSIONS: In patients with diabetes and ROCM, ROCM was the presenting manifestation in one fourth of the patients. Ophthalmic and extensive cerebral involvement predominated in the clinical picture. Delay in treatment due to late presentation and associated complications were major determinants of the survival outcome in these patients.

    PMID: ( MRI and CP ) ] 15537854 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Neonatal brain magnetic resonance imaging before discharge is better than serial cranial ultrasound in predicting cerebral palsy in very low birth weight preterm infants.
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    Neonatal brain magnetic resonance imaging before discharge is better than serial cranial ultrasound in predicting cerebral palsy in very low birth weight preterm infants.

    Pediatrics. 2004 Oct;114(4):992-8

    Authors: Mirmiran M, Barnes PD, Keller K, Constantinou JC, Fleisher BE, Hintz SR, Ariagno RL

    OBJECTIVE: To compare the value of serial cranial ultrasound (US) with a single magnetic resonance imaging (MRI) before discharge in very low birth weight preterm infants to predict cerebral palsy (CP). METHODS: Infants who weighed <1250 g at birth and were <30 weeks' gestational age underwent conventional brain MRI at near term (36-40 weeks' postmenstrual age) using 1.5 Tesla MRI scanner. Sagittal and axial T1 and T2 fluid attenuated inversion recovery and gradient recalled echo images were obtained. Cranial US was also obtained at least twice during the first 2 weeks of life. MRI and US images were interpreted by 2 independent radiologists, who were masked to clinical outcome, and scored as follows: category 1, no abnormality; category 2, subependymal hemorrhage or mineralization; category 3, moderate to severe ventriculomegaly; category 4, focal parenchymal abnormality with or without ventriculomegaly. For the purpose of this study, 1 and 2 were categorized as "normal," and 3 and 4 were categorized as "abnormal." The infants were assessed at a mean age of 20 and 31 months using the Amiel-Tison standardized neurodevelopmental examination. RESULTS: The sensitivity and specificity of MRI for predicting CP were 71% and 91% at 20 month and 86% and 89% at 31 months, respectively. The sensitivity and specificity of US for predicting CP were 29% and 86% at 20 months and 43% and 82% at 31 months. CONCLUSIONS: As a predictor of outcome for CP, MRI at near-term in very low birth weight preterm neonates is superior to US. However, both US and MRI demonstrate high specificity.

    PMID: ( MRI and CP ) ] 15466096 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Cerebral metabolism in severe neonatal hyperbilirubinemia.
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    Cerebral metabolism in severe neonatal hyperbilirubinemia.

    Pediatrics. 2004 Jul;114(1):291-4

    Authors: Groenendaal F, van der Grond J, de Vries LS

    The metabolism of the basal ganglia was examined by using proton magnetic resonance spectroscopy in 5 neonates with severe hyperbilirubinemia. A decreased N-acetylaspartate/choline ratio, indicating neuronal injury, and an abnormally high lactate/N-acetylaspartate ratio were found only in the neonate with neonatal magnetic resonance imaging abnormalities and subsequent cerebral palsy.

    PMID: ( MRI and CP ) ] 15231949 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Chorioamnionitis and cerebral palsy in term and near-term infants.
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    Chorioamnionitis and cerebral palsy in term and near-term infants.

    JAMA. 2003 Nov 26;290(20):2677-84

    Authors: Wu YW, Escobar GJ, Grether JK, Croen LA, Greene JD, Newman TB

    CONTEXT: Half of all cases of cerebral palsy (CP) occur in term infants, for whom risk factors have not been clearly defined. Recent studies suggest a possible role of chorioamnionitis. OBJECTIVE: To determine whether clinical chorioamnionitis increases the risk of CP in term and near-term infants. DESIGN, SETTING, AND PATIENTS: Case-control study nested within a cohort of 231 582 singleton infants born at 36 or more weeks' gestation between January 1, 1991, and December 31, 1998, in the Kaiser Permanente Medical Care Program, a managed care organization providing care for more than 3 million residents of northern California. Case patients were identified from electronic records and confirmed by chart review by a child neurologist, and comprised all children with moderate to severe spastic or dyskinetic CP not due to postnatal brain injury or developmental abnormalities (n = 109). Controls (n = 218) were randomly selected from the study population. MAIN OUTCOME MEASURE: Association between clinical chorioamnionitis and increased risk of CP in term and near-term infants. RESULTS: Most CP cases had hemiparesis (40%) or quadriparesis (38%); 87% had been diagnosed by a neurologist and 83% had undergone neuroimaging. Chorioamnionitis, considered present if a treating physician made a diagnosis of chorioamnionitis or endometritis clinically, was noted in 14% of cases and 4% of controls (odds ratio [OR], 3.8; 95% confidence interval [CI], 1.5-10.1; P =.001). Independent risk factors identified in multiple logistic regression included chorioamnionitis (OR, 4.1; 95% CI, 1.6-10.1), intrauterine growth restriction (OR, 4.0; 95% CI, 1.3-12.0), maternal black ethnicity (OR, 3.6; 95% CI, 1.4-9.3), maternal age older than 25 years (OR, 2.6; 95% CI, 1.3-5.2), and nulliparity (OR, 1.8; 95% CI, 1.0-3.0). The population-attributable fraction of chorioamnionitis for CP is 11%. CONCLUSION: Our data suggest that chorioamnionitis is an independent risk factor for CP among term and near-term infants.

    PMID: ( MRI and CP ) ] 14645309 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Diffusion tensor brain imaging findings at term-equivalent age may predict neurologic abnormalities in low birth weight preterm infants.
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    Diffusion tensor brain imaging findings at term-equivalent age may predict neurologic abnormalities in low birth weight preterm infants.

    AJNR Am J Neuroradiol. 2003 Sep;24(8):1646-53

    Authors: Arzoumanian Y, Mirmiran M, Barnes PD, Woolley K, Ariagno RL, Moseley ME, Fleisher BE, Atlas SW

    BACKGROUND AND PURPOSE: Low birth weight preterm infants are at high risk of brain injury, particularly injury to the white matter. Diffusion tensor imaging is thought to be more sensitive than conventional MR imaging for detecting subtle white matter abnormalities. The objective of this study was to examine whether diffusion tensor imaging could detect abnormalities that may be associated with later neurologic abnormalities in infants with otherwise normal or minimally abnormal conventional MR imaging findings. METHODS: We prospectively studied 137 low birth weight (<1800 g) preterm infants. Neonatal conventional MR imaging and diffusion tensor imaging were performed near term-equivalent age before discharge, and neurologic development of the infants was later followed up at 18 to 24 months of age. RESULTS: Among the preterm infants who were fully studied, 63 underwent normal conventional MR imaging. Three of these infants developed cerebral palsy, and 10 others showed abnormal neurologic outcome. Diffusion tensor imaging results for these infants showed a significant reduction of fractional anisotropy in the posterior limb of the internal capsule in neurologically abnormal infants (including those with cerebral palsy) compared with control preterm infants with normal neurologic outcomes. CONCLUSION: These results suggest that neonatal diffusion tensor imaging may allow earlier detection of specific anatomic findings of microstructural abnormalities in infants at risk for neurologic abnormalities and disability. The combination of conventional MR imaging and diffusion tensor imaging may increase the predictive value of neonatal MR imaging for later neurologic outcome abnormalities and may become the basis for future interventional clinical studies to improve outcomes.

    PMID: ( MRI and CP ) ] 13679287 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Reversible ischemic neurological deficit (RIND) due to exercise testing for the diagnosis of angina pectoris.
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    Reversible ischemic neurological deficit (RIND) due to exercise testing for the diagnosis of angina pectoris.

    Jpn Heart J. 2003 Jul;44(4):575-81

    Authors: Nanke T, Matsumoto N, Wakimoto H, Nakazawa K, Miyake F, Watanabe H, Horiuchi M, Takahashi Y

    A 57 year old Japanese male with chest oppression due to exercise is presented. This symptom was likely due to effort angina pectoris. Master's double two-step test revealed ischemic ST segment depression on the electrocardiogram, thus, a Tl201 myocardial stress imaging test using a bicycle ergometer was undertaken. Immediately following the exercise test, the patient experienced dizziness and palsy in his left upper and lower limbs. Cerebral angiography demonstrated 70% stenosis at the right internal carotid artery, but no abnormal findings were demonstrated on a cranial x-ray CT scan and magnetic resonance imaging. His palsy in the left limbs completely recovered within 3 weeks. Thus this event was diagnosed as a reversible ischemic neurological deficit (RIND). Major but non-cardiogenic complications during exercise testing are very rare, and RIND has not been reported thus far to the best of our knowledge.

    PMID: ( MRI and CP ) ] 12906039 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Perception and production of biological movement in patients with early periventricular brain lesions.
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    Perception and production of biological movement in patients with early periventricular brain lesions.

    Brain. 2003 Mar;126(Pt 3):692-701

    Authors: Pavlova M, Staudt M, Sokolov A, Birbaumer N, Krägeloh-Mann I

    Recent neuroimaging and psychophysical findings suggest that perception and production of human body motion share a common representational network. In the present study, we address the issue of whether early disorders in production of biological movement correspond to impairment in biological motion perception. By using the simultaneous masking paradigm, we examined visual sensitivity to biological motion in adolescents (aged 13-16 years) who were born very preterm (at 27-33 gestational weeks). In a confidence rating procedure, the presence of a point-light walking figure embedded in a moving mask was judged. The participants differed in their locomotion ability, ranging from normal to a complete walking disability exhibiting signs of leg-dominated bilateral spastic cerebral palsy (BS-CP) caused by periventricular leukomalacia (PVL). Irrespective of an ability to produce movement, patients with a similar extent of PVL in the parieto-occipital complex exhibit nearly the same sensitivity to biological motion. Sensitivity correlates negatively with the extent of PVL over the parieto-occipital complex, whereas neither the severity of motor disorder nor the severity of pyramidal tract affection relate significantly to the sensitivity index. The data suggest that perception of biological motion is not substantially affected by an observer's early restrictions in body movement. Instead, the findings favour the assumption that the common network for perception and production of biological motion might be inherent for the brain. Motor experience per se does not appear to be necessary for the visual analysis of human movement.

    PMID: ( MRI and CP ) ] 12566289 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • A clinicopathological study of vascular progressive supranuclear palsy: a multi-infarct disorder presenting as progressive supranuclear palsy.
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    A clinicopathological study of vascular progressive supranuclear palsy: a multi-infarct disorder presenting as progressive supranuclear palsy.

    Arch Neurol. 2002 Oct;59(10):1597-601

    Authors: Josephs KA, Ishizawa T, Tsuboi Y, Cookson N, Dickson DW

    BACKGROUND: Clinical features suggesting a diagnosis of progressive supranuclear palsy (PSP) include early falls, axial rigidity, vertical supranuclear ophthalmoplegia, and levodopa unresponsiveness. When these clinical features are present, the diagnosis is almost always PSP, yet vascular disease sometimes has a similar presentation, referred to as vascular PSP. OBJECTIVE: To evaluate clinical and pathologic features of cases of vascular PSP submitted to a PSP brain bank. DESIGN: Review of gross and microscopic neuropathological features, determination of tau haplotype, and medical record review of 4 patients with an antemortem diagnosis of PSP who did not meet the pathologic criteria for PSP and instead had vascular pathologic abnormalities. RESULTS: All patients had vertical supranuclear ophthalmoplegia, a history of falls, and a gradually progressive disease course. Falls began 1 year after symptom onset, and all patients had asymmetric findings on a neurological examination. A magnetic resonance imaging scan revealed lacunar basal ganglia infarcts in one patient and an increased T2-weighted signal in the corona radiata and centrum semiovale in another. Gross and microscopic neuropathological studies demonstrated infarcts in the cerebral cortex (n = 4), thalamus (n = 4), basal ganglia (n = 3), and cerebellum (n = 4). The brainstem was affected in one patient, but no infarcts were detected in the subthalamic nucleus or substantia nigra. Of the 4 patients, 3 carried an H2 tau haplotype, a rare occurrence in the general population. CONCLUSIONS: Asymmetric signs, falls after 1 year of symptom onset, vascular lesions on a magnetic resonance imaging scan, and an H2 tau haplotype may help differentiate vascular PSP from PSP. Thalamic and basal ganglia infarcts are common in patients with vascular PSP and, when present, may contribute to misdiagnosis.

    PMID: ( MRI and CP ) ] 12374498 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Does parenchymal brain injury affect biobehavioral pain responses in very low birth weight infants at 32 weeks' postconceptional age?
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    Does parenchymal brain injury affect biobehavioral pain responses in very low birth weight infants at 32 weeks' postconceptional age?

    Pediatrics. 2002 Sep;110(3):570-6

    Authors: Oberlander TF, Grunau RE, Fitzgerald C, Whitfield MF

    OBJECTIVE: Children with neurologic impairments have shown diminished pain response compared with control subjects; however, it remains unclear what mechanisms underlie this response or when it develops. If this were also true with premature infants who undergo neonatal intensive care, then infants with parenchymal brain injury (PBI) would be at increased risk of underrecognition and undertreatment of procedural pain. The purpose of this study was to determine whether infants with PBI display altered responses to acute procedural pain at 32 weeks' postconceptional age (PCA), compared with control subjects. METHODS: We compared responses to blood collection by heel lance at 32 weeks' PCA in 12 very low birth weight infants (mean [range] birth weight: 876 g [630-1240 g]; gestational age: 26.3 weeks (24-28 weeks) who had sustained PBI in the neonatal period, with 12 control subjects matched for gestational age at birth and gender (838 g [625-990 g]; 26.3 weeks [24-28 weeks[) who had normal neonatal brain imaging. PBI was defined as cerebral parenchymal infarction (grade 4 intraventricular hemorrhage) or cystic periventricular leukomalacia on serial cranial ultrasound scans conducted in the neonatal period. Biobehavioral responses to pain were measured using facial activity (Neonatal Facial Coding System) and measures of heart rate (HR) variability (low-frequency [LF] power [0.04-0.15], high-frequency [HF] power [0.15-0.8 Hz], and LF/HF ratio) as a measure of cardiac autonomic modulation. Neurodevelopmental follow-up was undertaken at 18 months. RESULTS: The infants with PBI had significantly higher illness severity scores at day 1 compared with day 3 (Score of Neonatal Acute Physiology II: 32.1 vs 19.8) but similar previous pain experiences (109 vs 115) and total morphine exposure (0.29 vs 0.30 mg/kg). Both groups of children mounted similar responses to heel lance at 32 weeks' PCA with no difference in facial response or HR variability. Mean HR and facial action scores increased from baseline to the lance, whereas LF, HF, and the LF/HF ratio decreased significantly. No group differences were found. The only statistically significant difference between groups was that infants with PBI had more tongue protrusion at lance. Neurodevelopmental follow-up showed 8 of 11 toddlers with PBI had cerebral palsy compared with 0% of control toddlers. Psychomotor Developmental Index score on the Bayley Scales of Infant Development II was significantly lower in the PBI group. Five of 11 toddlers with PBI had Mental Developmental Index score <2 standard deviations below mean compared with 0% of the control toddlers. CONCLUSION: Contrary to expectations, we did not find any evidence of an altered pain response pattern in infants with proven brain injury in the neonatal period. Although most infants with PBI developed cerebral palsy, these findings suggest that cerebral injury predominantly to the central white matter leaves brainstem responses intact in the neonatal period. Furthermore, it seems that the injured brain of the preterm infant has not yet expressed the identifiable differences in pain display and the functional impairment observed at later ages.

    PMID: ( MRI and CP ) ] 12205262 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • An outbreak of meningitis caused by Angiostrongylus cantonensis in Kaohsiung.
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    An outbreak of meningitis caused by Angiostrongylus cantonensis in Kaohsiung.

    J Microbiol Immunol Infect. 2001 Mar;34(1):50-6

    Authors: Tsai TH, Liu YC, Wann SR, Lin WR, Lee SJ, Lin HH, Chen YS, Yen MY, Yen CM

    Eight Thai laborers developed meningitis after eating raw snails (Ampullarium canaliculatus) during the period from September 27 to October 6, 1998. The diagnosis of Angiostrongylus cantonensis infection was established in all patients by serologic studies of serum and cerebral spinal fluid (CSF). Clinical manifestations included meningitis, radiculitis and cranial nerve palsy. Symptoms included fever, headache, orbital pain, gastrointestinal upset, hyperesthesia, muscle weakness, skin rash and diplopia. Laboratory abnormalities included peripheral eosinophilia, CSF eosinophilia, transient elevation of liver enzymes and creatinine phosphokinase, elevation of IgE. No space occupying lesions were detected by magnetic resonance imaging of the brain. None of the patients developed severe sequelae during the 6-month follow-up except for occasional headache in one patient. This report also provides evidence that third stage larvae were present in the intermediate host, A. canaliculatus, which the laborers had eaten.

    PMID: ( MRI and CP ) ] 11321128 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Hemiparetic cerebral palsy: etiological risk factors and neuroimaging.
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    Hemiparetic cerebral palsy: etiological risk factors and neuroimaging.

    Arq Neuropsiquiatr. 2001 Mar;59(1):29-34

    Authors: Piovesana AM, Moura-Ribeiro MV, Zanardi Vd , Gonçalves VM

    The purpose of this paper, which was conducted on 175 children with hemiparetic cerebral palsy (H-CP), was to verify the etiological risk period for this disease. Etiological risk factors (ERF) were detected through anamnesis: 23% in the prenatal period, 18% in the perinatal period and 59% of the patients the period was undefined (ERF in the prenatal and perinatal period was 41% and no ERF was 18% of the cases. The computerized tomographic scan (CT) and MRI were performed on all the patients, who were then classified according to their etiopathogenic data: CT1= normal (18%); CT 2= unilateral ventricular enlargement (25%); CT 3= cortical/ subcortical cavities (28%); CT4= hemispheric atrophy and other findings (14%); CT 5= malformations (15%). CT 5 was associated with physical malformations beyond the central nervous system and with prenatal ERF's, while CT 2 was associated with the perinatal ERF's, mainly in premature births. Magnetic resonance imaging was performed on 57 patients and demonstrated a good degree of concordance with the CT. Etiology remained undefined in only 37% of the cases after neuroimaging was related to ERF. A high perinatal RF frequency (59%) was observed and emphasized the need for special care during this period.

    PMID: ( MRI and CP ) ] 11299427 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Diabetes is an independent risk factor for in-hospital mortality from acute spontaneous intracerebral hemorrhage.
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    Diabetes is an independent risk factor for in-hospital mortality from acute spontaneous intracerebral hemorrhage.

    Diabetes Care. 2000 Oct;23(10):1527-32

    Authors: Arboix A, Massons J, García-Eroles L, Oliveres M, Targa C

    OBJECTIVE: We tested the hypothesis that diabetes is an independent determinant of outcome after intracerebral hemorrhage (ICH). RESEARCH DESIGN AND METHODS: This was a hospital-based prospective study The setting was an acute care 350-bed hospital in the city of Barcelona, Spain. Spontaneous ICH was diagnosed in 229 (11%) of 2,000 consecutive stroke patients included in a prospective stroke registry during a 10-year period. Main outcome measures were frequency of demographic variables, risk factors, clinical events, neuroimaging data, and outcome in ICH patients with and without diabetes. Variables related to vital status at discharge (alive or dead) in the univariate analysis plus age were studied in 4 logistical regression models. RESULTS: A total of 35 patients (15.3%) had diabetes. The overall in-hospital mortality rate was 54.3% in the diabetic group and 26.3% in the nondiabetic group (P < 0.001). Previous cerebral infarction, altered consciousness, sensory symptoms, cranial nerve palsy, multiple topography of the hematoma, intraventricular hemorrhage, and infectious complications were significantly more frequent in diabetic patients than in nondiabetic patients. The presence of diabetes was a significant predictive variable in the model based on demographic variables and cardiovascular risk factors (odds ratio 2.98 [95% CI 1.37-6.46]) and in the models based on these variables plus clinical variables (5.76 [2.01-16.51]), neuroimaging variables (5.59 [1.87-16.691), and outcome data (6.10 [2.04-18.291). CONCLUSIONS: Diabetes is an independent determinant of death after ICH. ICH in diabetic individuals presents some different clinical features compared with ICH in nondiabetic patients.

    PMID: ( MRI and CP ) ] 11023147 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Worster-Drought syndrome, a mild tetraplegic perisylvian cerebral palsy. Review of 47 cases.
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    Worster-Drought syndrome, a mild tetraplegic perisylvian cerebral palsy. Review of 47 cases.

    Brain. 2000 Oct;123 ( Pt 10):2160-70

    Authors: Clark M, Carr L, Reilly S, Neville BG

    A retrospective case-note analysis was undertaken of 47 children with a congenital upper motor neurone bulbar palsy (excluding pure speech dyspraxia) to clarify the phenotype of Worster-Drought syndrome (WDS) and to record its associated features and complications. The results revealed that the study children had significant bulbar problems (with 80% still needing a modified diet and a similar number using augmentative communication methods at last review). There were also high rates of predictable bulbar complications (86% had dribbling, 60% had glue ear, gastro-oesophageal reflux in 40%, history of poor nutrition in 40% and aspiration in 40%). Most of the children had additional complex impairments (91% had mild pyramidal tetraplegia, 81% learning difficulties, 60% congenital defects, 41% neuropsychiatric problems and 28% epilepsy). Over half of the children had significant medical problems in the first year, but mean age at diagnosis was 6 years. There were no obvious causes in pregnancy or birth. Six children had a family history of WDS and 32% (12/37) had abnormal neuroimaging including five with bilateral perisylvian polymicrogyria. In our experience, WDS is not uncommon, is relatively easily diagnosed and is crucial not to miss as the management of these children's multiple impairments is complex and requires a careful team approach. WDS falls clearly within the cerebral palsies as a syndrome that includes motor impairment arising from static damage to the brain in early life. The common presence of cognitive, behavioural and seizure impairments strongly supports the cerebral cortical (presumably perisylvian) localization. Its core elements are a suprabulbar paresis, a mild spastic tetraplegia and a significant excess of cognitive and behavioural impairments and epilepsy. The complete overlap in phenotype between WDS and the bilateral perisylvian syndrome leads us to propose that they are the same condition. WDS is startlingly absent from epidemiological studies of the cerebral palsies and rarely diagnosed, presumably because of lack of clinical awareness of the condition and lack of major gross motor impairments.

    PMID: ( MRI and CP ) ] 11004132 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Arg133Cys mutation of Notch3 in two unrelated Japanese families with CADASIL.
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    Arg133Cys mutation of Notch3 in two unrelated Japanese families with CADASIL.

    Intern Med. 2000 Sep;39(9):732-7

    Authors: Uyama E, Tokunaga M, Suenaga A, Kotorii S, Kamimura K, Takahashi K, Tabira T, Uchino M

    OBJECTIVE: More than 80 unrelated, but all Caucasian, patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), originating from various communities around the world, have been molecularly identified. To clarify the occurrence of CADASIL in Orientals, we investigated Japanese families presenting as CADASIL. METHODS: We performed the PCR-SSCP and sequence analyses using genomic DNA, isolated from venous blood of participants under informed consent. PATIENTS: We identified two unrelated Japanese families with CADASIL, including 5 affected members through 2 generations. RESULTS: Each of the affected individuals developed recurrent strokes without risk factors resulting in progressive dementia, pseudobulbar palsy, and gait disturbances which started after the fifth decade of life. Although affected individuals had no vascular risk factors, they showed various degrees of narrowing of retinal arteries. Their MRI/CTs showed characteristics of the disease; bilateral small infarcts in the thalamus, basal ganglia, brain stem, and deep white matter in addition to the findings of leukoaraiosis. On SPECT imaging, there was severe hypoperfusion in the cortex as well as in the white matter. Ultrastructural studies revealed an abnormal deposition of granular osmiophilic materials (GOM) within the basal lamina of pericytes in muscular capillaries. On PCR-SSCP and sequence analyses, a heterozygous Arg133Cys mutation was present, in the affected individuals, in the exon 4 of Notch3 gene which is the hot spot region for CADASIL mutations in Caucasian families. None of the non-affected members nor the 50 Japanese normal controls revealed this mutation. CONCLUSION: Thus, our results confirm that CADASIL is a geographically widespread disorder caused by a Notch3 mutation.

    PMID: ( MRI and CP ) ] 10969905 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Serum immunoreactivity to S-100 in children with cerebral palsy and delayed development and in their healthy parents.
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    Serum immunoreactivity to S-100 in children with cerebral palsy and delayed development and in their healthy parents.

    Yonsei Med J. 2000 Jun;41(3):328-32

    Authors: Park ES, Park CI, Baek SY, Kim SW, Baek SK, Kim HO

    The passive immunization of pregnant female rats to S-100 protein often leads to ultra-structural abnormalities in the brain glial structures of the offspring of these rats and induces signs of delayed development in the fetal brain. Additionally passive immunization of pregnant animals with certain antigens induces permanent Ag-specific changes in the immune response of their offspring. The purpose of this study was to investigate serum immunoreactiviy (SIR) to S-100 in cerebral-palsied and developmentally-delayed children as well as in their healthy parents and to evaluate its significance related to radiologic findings of brain MRI and single photon emission computed tomography (SPECT). The subjects were children with cerebral palsy and delayed development that had abnormal findings on brain MRI or Brain SPECT. SIR to S-100 protein was measured by ELISA method in the patients, their healthy parents, 20 normal adult controls and 22 normally developed children. The SIR to S-100 protein was significantly higher in the cerebral-palsied and developmentally-delayed children when compared to that of the normal control group children. Increased SIRs were detected in healthy mothers but not in their fathers. There was no difference of SIR between the cerebral-palsied and developmentally-delayed children or any significant difference of SIRs according to the findings of the brain MRI or to developmental quotients. But, the SIRs to S-100 protein were higher in the group of more abnormal findings on brain SPECT.

    PMID: ( MRI and CP ) ] 10957886 [Pubmed :- ( MRI and CP ) - indexed for MEDLINE ( MRI and CP ) ]

  • Abnormal vertical optokinetic nystagmus in infants and children.
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    Abnormal vertical optokinetic nystagmus in infants and children.

    Br J Ophthalmol. 2000 May;84(5):451-5

    Authors: Garbutt S, Harris CM

    AIMS: To determine if testing vertical optokinetic nystagmus (VOKN) has a role in the clinical assessment of infants and children. METHODS: A large field projection system was developed with which optokinetic nystagmus (OKN) could be stimulated in any direction. Gross abnormalities in the response were detected simply by observation. RESULTS: VOKN wa