Benign fasciculation syndrome:- Latest research articles

"Benign fasciculation syndrome is NOT dangerous but may provoke fear regarding the possibility of ALS. Latest research publications are scanned daily from major neurology journals and updated here"

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Fulltext access to articles relating to benign fasciculation syndrome

  • Intracelluar Ligands of NCAM.
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    Intracelluar Ligands of NCAM.

    Neurochem Res. 2008 Feb 21;

    Authors: ( Benign fasciculation syndrome )] Büttner B, Horstkorte R

    The neural cell adhesion molecule (NCAM) was identified as one of the first members of the immunoglobulin superfamily. It is implicated in numerous cellular functions. Thus, during embryonic development it controls tissue formation (e.g. neurulation) and is involved in many processes of neuronal development such as migration of neuroblasts, regulated outgrowth and fasciculation of neurites and the formation of communication contacts, for example nerve-muscle cell interactions. Furthermore, NCAM is involved in synaptogenesis and synaptic plasticity, learning and memory processes as well as regeneration. NCAM is a Ca(2+)-independent homophilic cell adhesion molecule, existing in three major isoforms, which are generated by alternative splicing of a single gene. With reference to their apparent molecular weights, they are termed NCAM 120, NCAM 140 and NCAM 180. NCAM 120 is anchored to the membrane via glycosyl-phosphatidyl-inositol, whereas NCAM 140 and NCAM 180 are transmembrane glycoproteins with large intracellular domains of different length. The purpose of this overview is to summarize the current knowledge on the intracellular binding partners of NCAM 140 and NCAM 180.

    PMID 18288608 [Pubmed ( Benign fasciculation syndrome )- as supplied by publisher]

  • Lateral neuron-glia interactions steer the response of axons to the Robo code.
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    Lateral neuron-glia interactions steer the response of axons to the Robo code.

    Neuron Glia Biol. 2004 May 1;1(2):101-112

    Authors: ( Benign fasciculation syndrome )] Kinrade EF, Hidalgo A

    Glia are required for axon pathfinding along longitudinal trajectories, but it is unknown how this relates to the molecular paradigm of axon guidance across the midline. Most interneuron axons in bilateral organisms cross the midline only once. Preventing them from recrossing the midline requires the expression of Robo receptors on the axons. These sense the repulsive signal Slit, which is produced by the midline. The lateral positioning of longitudinal axons depends on the response to Slit by the combination of Robo receptors expressed by the axons, on selective fasciculation, and on longitudinal (lateral) glia. Here, we analyse how longitudinal glia influence reading of the 'Robo code' by axons. We show that whereas loss of robo1 alone only affects the most medial axons, loss of both glial cells missing (gcm) and robo1 causes a severe midline collapse of longitudinal axons, similar to that caused by the loss of multiple Robo receptors. Furthermore, whereas ectopic expression of robo2 is sufficient to displace the medial MP2 axons along a more lateral trajectory, this does not occur in gcm-robo1 double-mutant embryos, where axons either do not extend at all or they misroute exiting the CNS. Hence, lateral neuron-glia interactions steer the response of axons to the Robo code.

    PMID 18273400 [Pubmed ( Benign fasciculation syndrome )- as supplied by publisher]

  • The role of glial cells in axon guidance, fasciculation and targeting.
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    The role of glial cells in axon guidance, fasciculation and targeting.

    Adv Exp Med Biol. 2007;621:156-66

    Authors: ( Benign fasciculation syndrome )] Learte AR, Hidalgo A

    Axons navigate step-wise, from one intermediate target to the next, until they reach their final destination target. In the central nervous system, intermediate targets are often ial cells, and final targeting is also aided by glia. In the peripheral nervous system, however, glial cells most often follow axons, which therefore navigate following other, nonglial clues. Even in the central nervous system, interactions between axons and glia are dynamic and reciprocal, as the neurons regulate migration, survival and proliferation of the glia cells they need for guidance. We review here the experimental evidence investigating roles of glia in axon guidance. Some molecules are known to influence either the neurons or the glia, but the molecular mechanisms underlying axon-glia interactions during pathfinding are only beginning to emerge.

    PMID 18269218 [Pubmed ( Benign fasciculation syndrome )- in process]

  • Firing pattern of fasciculations in ALS: evidence for axonal and neuronal origin.
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    Firing pattern of fasciculations in ALS: evidence for axonal and neuronal origin.

    Neurology. 2008 Jan 29;70(5):353-9

    Authors: ( Benign fasciculation syndrome )] Kleine BU, Stegeman DF, Schelhaas HJ, Zwarts MJ

    BACKGROUND: In amyotrophic lateral sclerosis (ALS), the origin of fasciculations is disputed. We hypothesized that the discharge pattern of fasciculation potentials (FPs) would be different for FPs arising in the motor axon or in the spinal motor neuron. METHOD: FPs were recorded by high-density surface EMG of the biceps brachii or vastus lateralis muscle for 15 minutes in 10 patients with ALS. Records were decomposed into different FP waveforms and their firing moments. Interspike interval (ISI) histograms were constructed for FPs that fired more than 100 times. RESULTS: Two types of ISI histograms were found. 1) In 23 of 30 different FPs with a total of 8,597 ISIs, the refractory period was 3 to 4 msec. ISIs longer than 15 msec had a Poisson distribution. Five of these 23 FPs discharged doublets with an ISI of approximately 5 msec, indicative of supernormality. This is consistent with the FPs arising in motor axons. 2) In the other 7 FPs, accounting for 11,266 ISIs, the refractory period was 17 to 46 msec. The preferred ISI duration was around 80 msec. Both timing factors are consistent with origin in the spinal motor neuron. CONCLUSIONS: Firing pattern analysis, based on high-density surface EMG, can detect fasciculation potentials (FPs) of axonal and neuronal origin in amyotrophic lateral sclerosis. The two FP types coexist within the same muscle. The recognition that clinically identical fasciculations conceal the existence of two types of FP that can be studied in a noninvasive manner will introduce a new aspect in the research of motor neuron disease.

    PMID 18227416 [Pubmed ( Benign fasciculation syndrome )- in process]

  • Neurite arborization and mosaic spacing in the mouse retina require DSCAM.
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    Neurite arborization and mosaic spacing in the mouse retina require DSCAM.

    Nature. 2008 Jan 24;451(7177):470-4

    Authors: ( Benign fasciculation syndrome )] Fuerst PG, Koizumi A, Masland RH, Burgess RW

    To establish functional circuitry, retinal neurons occupy spatial domains by arborizing their processes, which requires the self-avoidance of neurites from an individual cell, and by spacing their cell bodies, which requires positioning the soma and establishing a zone within which other cells of the same type are excluded. The mosaic patterns of distinct cell types form independently and overlap. The cues that direct these processes in the vertebrate retina are not known. Here we show that some types of retinal amacrine cells from mice with a spontaneous mutation in Down syndrome cell adhesion molecule (Dscam), a gene encoding an immunoglobulin-superfamily member adhesion molecule, have defects in the arborization of processes and in the spacing of cell bodies. In the mutant retina, cells that would normally express Dscam have hyperfasciculated processes, preventing them from creating an orderly arbor. Also, their cell bodies are randomly distributed or pulled into clumps rather than being regularly spaced mosaics. Our results indicate that mouse DSCAM mediates isoneuronal self-avoidance for arborization and heteroneuronal self-avoidance within specific cell types to prevent fasciculation and to preserve mosaic spacing. These functions are analogous to those of Drosophila DSCAM (ref. 6) and DSCAM2 (ref. 7). DSCAM may function similarly in other regions of the mammalian nervous system, and this role may extend to other members of the mammalian Dscam gene family.

    PMID 18216855 [Pubmed ( Benign fasciculation syndrome )- in process]

  • The effect of gallium nitride on long-term culture induced aging of neuritic function in cerebellar granule cells.
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    The effect of gallium nitride on long-term culture induced aging of neuritic function in cerebellar granule cells.

    Biomaterials. 2008 Apr;29(11):1573-82

    Authors: ( Benign fasciculation syndrome )] Chen CR, Young TH

    Gallium nitride (GaN) has been developed for a variety of microelectronic and optical applications due to its unique electric property and chemical stability. In the present study, n-type and p-type GaN were used as substrates to culture cerebellar granule neurons to examine the effect of GaN on cell response for a long-term culture period. It was found that GaN could rapidly induce cultured neurons to exhibit a high phosphorylated Akt level after 20h of incubation. It was assumed that the anti-apoptotic effect of Akt phosphorylation could be correlated with cell survival, neurite growth and neuronal function for up to 35 days of incubation. Morphological studies showed GaN induced larger neuronal aggregates and neurite fasciculation to exhibit a dense fiber network after 8 days of incubation. Western blot analysis and immunocytochemical characterization showed that GaN still exhibited the expression of neurite growth and function, such as high levels of GAP-43, synapsin I and synaptophysin even after 35 days of incubation. In addition, survival of cerebellar granule neurons on GaN was improved by the analysis of lactate dehydrogenase (LDH) release from damaged cells. These results indicated that neuronal connections were formed on GaN by a gradual process from Akt activation and cell aggregation to develop neurite growth, fasciculation and function. Therefore, GaN offers a good model system to identify a well-characterized pattern of neuronal behavior for a long-term culture period, consistent with the development of a neurochip requiring the integration of biological system and semiconductor material.

    PMID 18194814 [Pubmed ( Benign fasciculation syndrome )- in process]

  • Forme fruste or incipient form of widespread-type amyotrophic lateral sclerosis, or motor neuron disease with pallido-nigro-luysian atrophy? An autopsy case report.
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    Forme fruste or incipient form of widespread-type amyotrophic lateral sclerosis, or motor neuron disease with pallido-nigro-luysian atrophy? An autopsy case report.

    Neuropathology. 2007 Dec 29;

    Authors: ( Benign fasciculation syndrome )] Hashimoto T, Matsubara S, Mochizuki Y, Tsuji S, Mizutani T, Oyanagi K

    We describe a 52-year-old man with body weight loss and bulbar palsy, who exhibited muscle atrophy and weakness with fasciculation especially in the respiratory muscles 4 years prior to death, necessitating respiratory support for 4 years, but who was able to walk until the end-stage. He had no significant family history. Neuropathological examination revealed severe loss of motor neurons in the spinal cord and brainstem, and ubiquitin-positive skein-like inclusions and Bunina bodies in the remaining neurons. In addition, prominent degeneration of the anterolateral funiculus and severe loss of neurons in the intermediate zone of the spinal cord were evident, without marked alteration of the corticospinal tracts. Degeneration of the subthalamic nucleus, increased iron deposition in the substantia nigra, and axonal swelling, residual nodules and acidophilic granules in the spinal ganglia were found. The patient's condition was considered to have been a forme fruste or incipient form of widespread-type amyotrophic lateral sclerosis (ALS) or motor neuron disease (MND) with pallido-nigro-luysian atrophy (PNLA). The neuropathological features of the present case appear to be important for understanding the nature of widespread-type ALS and MND with PNLA.

    PMID 18179405 [Pubmed ( Benign fasciculation syndrome )- as supplied by publisher]

  • Electrodiagnostic criteria for diagnosis of ALS.
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    Electrodiagnostic criteria for diagnosis of ALS.

    Clin Neurophysiol. 2007 Dec 26;

    Authors: ( Benign fasciculation syndrome )] de Carvalho M, Dengler R, Eisen A, England JD, Kaji R, Kimura J, Mills K, Mitsumoto H, Nodera H, Shefner J, Swash M

    A consensus meeting was held to determine the best use and interpretation of electrophysiological data in the diagnosis of ALS. The utility of needle EMG and nerve conduction studies was affirmed. It is recommended that electrophysiological evidence for chronic neurogenic change should be taken as equivalent to clinical information in the recognition of involvement of individual muscles in a limb. In addition, in the context of a suspected clinical diagnosis of ALS, fasciculation potentials should be taken as equivalent to fibrillation potentials and positive sharp waves in recognising denervation. The importance of searching for instability in fasciculation potentials and in motor unit potentials in ALS is stressed. These changes in the interpretation of electrophysiological data render obsolete the category Probable Laboratory-Supported ALS in the modified El Escorial diagnostic criteria for ALS. Methods for detection of upper motor neuron abnormality appear sensitive but require further study, particularly regarding their value when clinical signs of upper motor neuron lesion are uncertain.

    PMID 18164242 [Pubmed ( Benign fasciculation syndrome )- as supplied by publisher]

  • Olfactory sensory neuron-specific and sexually dimorphic expression of protocadherin 20.
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    Olfactory sensory neuron-specific and sexually dimorphic expression of protocadherin 20.

    J Comp Neurol. 2008 Mar 1;507(1):1076-86

    Authors: ( Benign fasciculation syndrome )] Lee W, Cheng TW, Gong Q

    Olfactory sensory axons navigate from the nasal cavity to the olfactory bulb and sort from among 1,000 different odorant receptor-expressing types to converge upon the same two or three glomeruli. To achieve this task during development, it is likely that multiple classes of regulatory molecules, including cell adhesion molecules, are involved. Cell adhesion molecules have been shown to be important in controlling axon guidance, fasciculation, and synapse formation. To gain further understanding of the involvement of adhesion molecules in olfactory circuitry development, we examined the dynamic and cell type specific expression of a novel protocadherin, PCDH20, in the olfactory system. PCDH20 is specifically expressed in newly differentiated olfactory sensory neurons and their axons during development. PCDH20 expression is down-regulated in the adult olfactory system, except in a small olfactory sensory neuron population. These small, discrete numbers of PCDH20-positive glomeruli in the adult olfactory bulb are consistently clustered in the ventral-caudal region in both male and female mice. However, adult males have higher numbers of PCDH20-positive glomeruli with a broader distribution, whereas adult females have fewer PCDH20-positive glomeruli with a more restricted distribution. The gender difference in PCDH20 expression may reflect olfactory receptor expression differences for gender-specific social discrimination.

    PMID 18095321 [Pubmed ( Benign fasciculation syndrome )- in process]

  • Endovascular treatment of a basilar artery dissecting aneurysm.
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    Endovascular treatment of a basilar artery dissecting aneurysm.

    Arq Neuropsiquiatr. 2007 Dec;65(4A):1015-7

    Authors: ( Benign fasciculation syndrome )] Forcelini CM, Rotta FT, Posenato N, Rovani JS, Crusius PS, Mallmann AB, Seibert CA, Crusius MU, Carazzo C, Crusius CU, Goellner E, Ragnini J, Wayhs SY

    Fasciculations are symptoms present in a broad spectrum of conditions, ranging from normal manifestations to motor neuron diseases. They also represent the main picture of Benign fasciculation syndrome . We report a case of such syndrome: a 48-years-old woman complaining about fasciculations for three decades who remained with the symptoms even after the compensation of a disclosed hyperthyroidism. The introduction of gabapentin rendered control of her fasciculations. The available data in the literature about the therapeutic approaches for fasciculations are revised, as long as the rare reports of evolution from patients with "benign" fasciculations to cases of amyotrophic lateral sclerosis, underlining the importance of following the patients with fasciculations.

    PMID 18094867 [Pubmed ( Benign fasciculation syndrome )- in process]

  • Cross-midline interactions between mouse commissural hindbrain axons contribute to their efficient decussation.
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    Cross-midline interactions between mouse commissural hindbrain axons contribute to their efficient decussation.

    Dev Neurobiol. 2008 Feb 15;68(3):349-64

    Authors: ( Benign fasciculation syndrome )] Sandoval-Minero T, Varela-Echavarría A

    Information from both sides of the brain is integrated by axons that project across the midline of the central nervous system via numerous commissures present at all axial levels. Despite the accumulated experimental evidence, questions remain regarding the formation of commissures in the presence of strong repulsive signals in the ventral midline. Studies from invertebrates suggest that interaction at the midline between homologous axons of specific decussating neurons contributes to efficient midline crossing, but such evidence is lacking in vertebrate systems. We performed experiments to determine whether commissural axons of the caudal region of the hindbrain interact with their contralateral counterparts at the ventral midline and to evaluate the relevance of this reciprocal interaction. Double anterograde axon labeling with lipophilic tracers revealed close apposition between growth cones of contralateral pioneer decussating axons at the midline. Later, we detected fasciculation between contralateral axons that is maintained even after they have crossed the midline. Blocking axon projections unilaterally with a solid mechanical barrier decreased dramatically the midline crossing of the equivalent population from the contralateral side. Decussation was also blocked by a unilateral barrier permeable to diffusible molecules but not by an axon-permeable barrier. These results suggest that in the caudal region of the hindbrain, midline crossing is facilitated by interactions between decussating contralateral axon partners. (c) 2007 Wiley Periodicals, Inc. Develop Neurobiol, 2008.

    PMID 18085564 [Pubmed ( Benign fasciculation syndrome )- in process]

  • Cadherin-8 and N-cadherin differentially regulate pre- and postsynaptic development of the hippocampal mossy fiber pathway.
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    Cadherin-8 and N-cadherin differentially regulate pre- and postsynaptic development of the hippocampal mossy fiber pathway.

    Hippocampus. 2007 Dec 6;

    Authors: ( Benign fasciculation syndrome )] Bekirov IH, Nagy V, Svoronos A, Huntley GW, Benson DL

    Cells sort into regions and groups in part by their selective surface expression of particular classic cadherins during development. In the nervous system, cadherin-based sorting can define axon tracts, restrict axonal and dendritic arbors to particular regions or layers, and may encode certain aspects of synapse specificity. The underlying model has been that afferents and their targets hold in common the expression of a particular cadherin, thereby providing a recognition code of homophilic cadherin binding. However, most neurons express multiple cadherins, and it is not clear whether multiple cadherins all act similarly in shaping neural circuitry. Here we asked how two such cadherins, cadherin-8 and N-cadherin, influence the guidance and differentiation of hippocampal mossy fibers. Using organotypic hippocampal cultures, we find that cadherin-8 regulates mossy fiber fasciculation and targeting, but has little effect on CA3 dendrites. In contrast, N-cadherin regulates mossy fiber fasciculation, but has little impact on axonal growth and targeting. However, N-cadherin is essential for CA3 dendrite arborization. Both cadherins are required for formation of proper numbers of presynaptic terminals. Mechanistically, such differential actions of these two cadherins could, in theory, reflect coupling to distinct intracellular binding partners. However, we find that both cadherins bind beta-catenin in dentate gyrus (DG). This suggests that cadherins may engage different intracellular signaling cascades downstream of beta-catenin, coopt different extracellular binding partners, or target distinct subcellular domains. Together our findings demonstrate that cadherin-8 and N-cadherin are critical for generating the mossy fiber pathway, but that each contributes differentially to afferent and target differentiation, thereby complementing one another in the assembly of a synaptic circuit. (c) 2007 Wiley-Liss, Inc.

    PMID 18064706 [Pubmed ( Benign fasciculation syndrome )- as supplied by publisher]

  • Matrix metalloproteinases promote motor axon fasciculation in the Drosophila embryo.
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    Matrix metalloproteinases promote motor axon fasciculation in the Drosophila embryo.

    Development. 2008 Jan;135(1):95-109

    Authors: ( Benign fasciculation syndrome )] Miller CM, Page-McCaw A, Broihier HT

    Matrix metalloproteinases (MMPs) are a large conserved family of extracellular proteases, a number of which are expressed during neuronal development and upregulated in nervous system diseases. Primarily on the basis of studies using pharmaceutical inhibitors, MMPs have been proposed to degrade the extracellular matrix to allow growth cone advance during development and hence play largely permissive roles in axon extension. Here we show that MMPs are not required for axon extension in the Drosophila embryo, but rather are specifically required for the execution of several stereotyped motor axon pathfinding decisions. The Drosophila genome contains only two MMP homologs, Mmp1 and Mmp2. We isolated Mmp1 in a misexpression screen to identify molecules required for motoneuron development. Misexpression of either MMP inhibits the regulated separation/defasciculation of motor axons at defined choice points. Conversely, motor nerves in Mmp1 and Mmp2 single mutants and Mmp1 Mmp2 double mutant embryos are loosely bundled/fasciculated, with ectopic axonal projections. Quantification of these phenotypes reveals that the genetic requirement for Mmp1 and Mmp2 is distinct in different nerve branches, although generally Mmp2 plays the predominant role in pathfinding. Using both an endogenous MMP inhibitor and MMP dominant-negative constructs, we demonstrate that MMP catalytic activity is required for motor axon fasciculation. In support of the model that MMPs promote fasciculation, we find that the defasciculation observed when MMP activity is compromised is suppressed by otherwise elevating interaxonal adhesion -- either by overexpressing Fas2 or by reducing Sema-1a dosage. These data demonstrate that MMP activity is essential for embryonic motor axon fasciculation.

    PMID 18045838 [Pubmed ( Benign fasciculation syndrome )- in process]

  • Expression profile of the cadherin family in the developing Drosophila brain.
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    Expression profile of the cadherin family in the developing Drosophila brain.

    J Comp Neurol. 2008 Jan 20;506(3):469-88

    Authors: ( Benign fasciculation syndrome )] Fung S, Wang F, Chase M, Godt D, Hartenstein V

    The Drosophila genome encodes 17 members of the cadherin family of adhesion molecules, which in vertebrates has been implicated in patterning the nervous system through cell and axon sorting. With only a few exceptions all cadherins show widespread expression in the larval brain. What expression patterns have in common is that 1) they are global, in the sense that all lineages of the central brain or optic lobe, or both, show expression; and 2) expression is stage-specific: some cadherins are expressed only in primary neurons (located closest to the neuropile), others in early secondary neurons (near the brain surface), or primaries plus late secondaries. The Fat-like cadherins, Fat and Dachsous, as well as Cad96Ca and Cad74A, are expressed in the epithelial optic lobe anlagen, which matches the widespread epithelial expression of these molecules in the embryo. DE-cadherin is restricted to immature secondary neurons and glia; by contrast, DN-cadherin, Flamingo, Cad87A, Cad99C, and Calsyntenin-1 appear in differentiating primary neurons and, at a later stage, some or all secondary neurons. Cad87A is strongly enriched apically in epithelia and in neuronal dendrites. Fat-like, Cad86C, Cad88C, Cad89D, and Dret are expressed ubiquitously in embryonic and larval brains at low or moderate levels. We conclude from this analysis that cadherins are likely to play a role in 'generic' neural functions, such as neurite fasciculation, branching, and synapse formation.

    PMID 18041774 [Pubmed ( Benign fasciculation syndrome )- in process]

  • Development of the corticospinal tract in Semaphorin3A- and CD24-deficient mice.
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    Development of the corticospinal tract in Semaphorin3A- and CD24-deficient mice.

    Neuroscience. 2007 Dec 19;150(4):898-904

    Authors: ( Benign fasciculation syndrome )] Sibbe M, Taniguchi M, Schachner M, Bartsch U

    Mutations in the gene encoding the neural recognition molecule L1 result in hypoplasia of the corticospinal tract and path finding errors of corticospinal axons at the pyramidal decussation. Candidate molecules that have been implicated in L1-dependent guidance of corticospinal axons from the ventral medullary pyramids to the contralateral dorsal columns of the cervical spinal cord include Semaphorin3A and CD24. In the present study, we anterogradely labeled corticospinal axons from the sensorimotor cortex of young postnatal Semaphorin3A- and CD24-deficient mice to elucidate potential functions of both proteins during formation of this long axon projection. Our results indicate that elongation, collateralization, fasciculation and path finding of corticospinal axons in mice proceed normally in the absence of Semaphorin3A or CD24.

    PMID 18022325 [Pubmed ( Benign fasciculation syndrome )- in process]

  • L1 cell adhesion molecule is not required for small-diameter primary afferent sprouting after deafferentation.
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    L1 cell adhesion molecule is not required for small-diameter primary afferent sprouting after deafferentation.

    Neuroscience. 2007 Dec 19;150(4):959-69

    Authors: ( Benign fasciculation syndrome )] Runyan SA, Roy RR, Zhong H, Phelps PE

    L1 is a cell adhesion molecule associated with axonal outgrowth and fasciculation during spinal cord development and may reiterate its developmental role in adults following injury; L1 is upregulated on certain sprouting and regenerating axons in adults, but it is unclear if L1 expression is necessary for, or contributes to, regrowth of axons. This study asks if L1 is required for small-diameter primary afferents to sprout by conducting unilateral dorsal rhizotomies (six segments; T10-L2) on both wild-type and L1 mutant mice. First we determined that L1 co-localizes substantially with the peptidergic (calcitonin gene-related peptide; CGRP) but minimally with the nonpeptidergic (isolectin B4; IB4) primary afferents in intact wild-type and L1 mutant mice. However, we encountered a complication using IB4 to identify primary afferents post-rhizotomy; we detected extensive abnormal IB4 expression in the dorsal horn and dorsal columns. Much of this aberrant IB4 labeling is associated with fibrous astrocytes and microglia. Five days after dorsal rhizotomy a large decrease in peptidergic and nonpeptidergic afferents is evident on the deafferented side in both wild-type and L1 mutants. Three months after surgery the peptidergic primary afferents sprouted into the center of the denervated dorsal horn in both wild-type and mutant mice, and quantitative analyses confirmed a sprouting density of similar magnitude in both genotypes. In contrast, we did not detect sprouting in the nonpeptidergic primary afferents in either genotype. These results suggest that the absence of L1 neither diminishes nor enhances sprouting of peptidergic small-diameter primary afferent axons following a dorsal rhizotomy.

    PMID 18022323 [Pubmed ( Benign fasciculation syndrome )- in process]

  • Pathophysiologic insights into motor axonal function in Kennedy disease.
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    Pathophysiologic insights into motor axonal function in Kennedy disease.

    Neurology. 2007 Nov 6;69(19):1828-35

    Authors: ( Benign fasciculation syndrome )] Vucic S, Kiernan MC

    OBJECTIVE: Kennedy disease (KD), or spinobulbomuscular atrophy, is a slowly progressive inherited neurodegenerative disorder, marked by prominent fasciculations that typically precede the development of other symptoms. Although the genetic basis of KD relates to triplet (CAG) repeat expansion in the androgen receptor (AR) gene on the X chromosome, the mechanisms underlying the clinical presentation in KD have yet to be established. Consequently, the present study applied axonal excitability techniques to investigate the pathophysiologic mechanisms associated with KD. METHODS: Peripheral nerve excitability studies were undertaken in 7 patients with KD with compound muscle action potentials (CMAP) recorded from the right abductor pollicis brevis. RESULTS: Strength-duration time constant (KD 0.54 +/- 0.03 msec; controls, 0.41 +/- 0.02 msec, p < 0.01) and the hyperpolarizing current/threshold gradient (KD 0.42 +/- 0.01; controls, 0.37 +/- 0.01, p < 0.05) were significantly increased in KD. Strength-duration time constant correlated with the CMAP amplitude (R = 0.68) and the fasciculation frequency (R = 0.62). Threshold electrotonus revealed greater changes in response to subthreshold depolarizing (KD TEd [90 to 100 msec], 50.75 +/- 1.98%; controls TEd [90 to 100 msec], 45.67 +/- 0.67%, p < 0.01) and hyperpolarizing (KD TEh [90 to 100 msec], 128.5 +/- 6.9%; controls TEh [90 to 100 msec], 120.5 +/- 2.4%) conditioning pulses. Measurements of refractoriness, superexcitability, and late subexcitability changed appropriately for axonal hyperpolarization, perhaps reflecting the effects of increased ectopic activity. CONCLUSION: In total, the increase in the strength-duration time constant may be the primary event, occurring early in course of the disease, contributing to the development of axonal hyperexcitability in Kennedy disease, and thereby to the generation of fasciculations, a characteristic hallmark of the disease.

    PMID 17984450 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • [Altered axonal ion channel function in amyotrophic lateral sclerosis]
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    [Altered axonal ion channel function in amyotrophic lateral sclerosis]

    Brain Nerve. 2007 Oct;59(10):1109-15

    Authors: ( Benign fasciculation syndrome )] Kuwabara S, Kanai K

    Fasciculation is a characteristic feature of amyotrophic lateral sclerosis (ALS). The ectopic firing of motor units usually arises from the motor nerve terminals, and occasionally from the motor neurons, indicating a wide-spread abnormality in axonal excitability properties. ALS is a multi-factorial disease; some gene abnormalities and environmental factors lead to a cell death through a complex cascade, including oxidative stress, mitochondrial dysfunction, excitotoxicity, and impaired axonal transport. It is important to elucidate the pathophysiology of axonal excitability in ALS, because increased axonal excitability would enhance oxidative stress and excitotoxicity, and therefore contribute to motor neuronal death. So far, two kinds of axonal ion channel abnormalities have been found; (1) increased persistent sodium currents, and (2) reduced potassium currents, both increasing axonal excitability and responsible for generation of fasciculations. In excitability testing, findings in ALS are characterized prolonged strength-duration time constant, suggesting increased persistent sodium currents, and greater threshold changes in depolarizing threshold electrotonus and greater supernormality, suggestive of impaired potassium channels. In relation to disease stage, persistent sodium currents increase in the early phase of the disease, possibly associated with collateral sprouting, and then, potassium currents decline. These serial changes in axonal properties could provide new insights into the pathophysiology of ALS, and implications for future therapeutic options.

    PMID 17969351 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Spontaneous rhythmic activity in early chick spinal cord influences distinct motor axon pathfinding decisions.
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    Spontaneous rhythmic activity in early chick spinal cord influences distinct motor axon pathfinding decisions.

    Brain Res Rev. 2008 Jan;57(1):77-85

    Authors: ( Benign fasciculation syndrome )] Hanson MG, Milner LD, Landmesser LT

    During embryonic development chick and mouse spinal cords are activated by highly rhythmic episodes of spontaneous bursting activity at very early stages, while motoneurons are still migrating and beginning to extend their axons to the base of the limb. While such spontaneous activity has been shown to be important in refining neural projections once axons have reached their targets, early pathfinding events have been thought to be activity independent. However, in-ovo pharmacological manipulation of the transmitter systems that drive such early activity has shown that early motor axon pathfinding events are highly dependent on the normal pattern of bursting activity. A modest decrease in episode frequency resulted in dorsal-ventral pathfinding errors by lumbar motoneurons, and in the downregulation of several molecules required to successfully execute this guidance decision. In contrast, increasing the episode frequency was without effect on dorsal-ventral pathfinding. However, it prevented the subsequent motoneuron pool specific fasciculation of axons and their targeting to appropriate muscles, resulting in marked segmental pathfinding errors. These observations emphasize the need to better evaluate how such early spontaneous electrical activity may influence the molecular and transcription factor pathways that have been shown to regulate the differentiation of motor and interneuron phenotypes and the formation of spinal cord circuits. The intracellular signaling pathways by which episode frequency affects motor axon pathfinding must now be elucidated and it will be important to more precisely characterize the patterns with which specific subsets of motor and inter-neurons are activated normally and under conditions that alter spinal circuit formation.

    PMID 17920131 [Pubmed ( Benign fasciculation syndrome )- in process]

  • Reliability of a novel neurostimulation method to study involuntary muscle phenomena.
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    Reliability of a novel neurostimulation method to study involuntary muscle phenomena.

    Muscle Nerve. 2008 Jan;37(1):90-100

    Authors: ( Benign fasciculation syndrome )] Minetto MA, Botter A, Ravenni R, Merletti R, De Grandis D

    Experimental methods involving painful electrical stimulation of a peripheral nerve showed the existence of a minimum stimulation frequency capable of inducing cramp, termed "threshold frequency" (TF). Our aim was to test an alternative method to induce fasciculations and cramps electrically. Two daily sessions of electrical stimulation of the abductor hallucis muscle were performed in 19 volunteers on 3 days: stimulation trains of 150 monophasic square pulses (duration 152 micros) of increasing frequency (current intensity 30% higher than maximal; frequency of the first trial, 4 pps; recovery between trials, 1 min) were delivered to the main muscle motor point until a cramp developed. Once a cramp was induced the protocol was repeated after 30 min. To verify by electromyography that cramp occurred, a surface electrode array was placed between the motor point and the distal tendon. Ambient and skin temperature were kept constant in all sessions. Fasciculations and cramps were elicited in all subjects. We observed the following median (interquartile range) values of TF: day 1 (session 1), 13 (6) pps; day 1 (session 2), 16 (4) pps; day 2 (session 1), 16 (6) pps; day 2 (session 2), 18 (6) pps; day 3 (session 1), 17 (4) pps; day 3 (session 2), 18 (8) pps. TF intersession intraclass correlation coefficients were 0.82, 0.92, and 0.90 for days 1, 2, and 3, respectively. TF interday intraclass correlation coefficient was 0.85. The absence of pain due to the stimulation and the demonstration of TF reliability support the use of our method for the study of involuntary muscle phenomena.

    PMID 17912751 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • [Protective effects of vigabatrin and atropine against dimethoate induced-intoxication in mice]
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    [Protective effects of vigabatrin and atropine against dimethoate induced-intoxication in mice]

    Zhonghua Lao Dong Wei Sheng Zhi Ye Bing Za Zhi. 2007 Jul;25(7):389-93

    Authors: ( Benign fasciculation syndrome )] Wu QE, Ban TT, Yao XM, Chang XL, Wu Q, Zhou ZJ

    OBJECTIVE: To investigate the protective effects of vigabatrin and atropine against the acute toxicity of dimethoate in male Kun-min mice. METHODS: The therapeutic schedules of vigabatrin (50 or 100 mg/kg) and (or) atropine (2.5 or 5.0 mg/kg) were performed according to the L(9) (3(4)) orthogonal test table. The survival time, the righting reflex and the onset of muscle fasciculations were observed after the administration of dimethoate. RESULTS: First, the main effects of vigabatrin, atropine and the interaction between them were statistically significant in the Univariate analysis of the survival time at the alpha level of 0.05 (F(V)= 4.73, P(V)= 0.015, F(A)= 50.88, P(A)= 0.000, F(VxA)= 4.17, P(VxA)= 0.007). The range of atropine was more than double of that of vigabatrin or their interaction (R(A)> 2RV or 2R(VxA)). So not only atropine and vigabatrin but also their combination interaction protected mice against dimethoate lethality. The atropine played the major role in diminishing the lethality induced by dimethoate. Second, only vigabatrin, while not atropine, delayed the mice from dimethoate-induced muscle fasciculation according its statistical results (F(V)= 6.87, P(V)= 0.003, F(A)= 0.03, P(A)= 0.968, F(VxA)= 1.134, P(VxA)= 0.356). It should be noted that vigabatrin could not completely prevent dimethoate induced-muscle fasciculation in the test. At last, both atropine and vigabatrin could maintain the righting reflex in the intoxication, however there was no interaction between them (F(V)= 5.81, P(V)= 0.006, F(A)= 9.05, P(A)= 0.001, F(VxA)= 1.34, P(VxA)= 0.257). CONCLUSION: Combined treatment with atropine and vigabatrin in the organophosphates intoxication seems reasonable and acceptable.

    PMID 17908425 [Pubmed ( Benign fasciculation syndrome )- in process]

  • The effects of magnesium sulphate-pretreatment on suxamethonium-induced complications during induction of general endotracheal anaesthesia.
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    The effects of magnesium sulphate-pretreatment on suxamethonium-induced complications during induction of general endotracheal anaesthesia.

    Afr J Med Med Sci. 2007 Mar;36(1):43-7

    Authors: ( Benign fasciculation syndrome )] Danladi KY, Sotunmbi PT, Eyelade OR

    To determine the effects of Magnesium-Sulphate-pretreatment on Suxamethonium-induced complications (serum potassium rise, fasciculations and apnea). Eighty-four adult patients were selected and randomly allocated into two study groups during induction of general endotracheal anaesthesia. Endotracheal intubation was facilitated with suxamethonium in group A, while in group B magnesium sulphate pretreatment and suxamethonium. Blood sample for serum potassium estimation was taken before induction and at 5 min after induction. Degree of fasciculations and duration of apnea were assessed clinically. Anaesthetic technique and monitoring of patient was standardized. This study showed statistically significant increase in serum potassium of Group A patients {average 0.34 mmol/L} from baseline value p value 0.00. Magnesium sulphate pretreatment significantly reduced suxamethonium-induced hyperkalaemia by an average of 0.3 mmol/L (p-value 0.01). The severity of fasciculations was also significantly reduced (p-value 0.00). There was no significant effect of magnesium pretreatment on duration of apnea during endotracheal intubation (p-value 0.41). Fourteen point six percent (14.6%) of patients that received magnesium pretreatment complained of feeling of heat or warmth but there was no life threatening dysrrhythmias observed in any of the eighteen patients that had continuous ECG monitoring. The study shows that magnesium sulphate pretreatment has significantly reduced suxamethonium-induced hyperkalaemia and severity of fasciculations during induction of general endotracheal anaesthesia, however there was no significant effect on the duration of apnea. The average of 0.034 mmol/L in Group B was not significant {p value 0.06}. We advocate the use of magnesium pretreatment in all patients at risk of these complications.

    PMID 17876916 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Rattlesnake envenomation.
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    Rattlesnake envenomation.

    Compend Contin Educ Vet. 2007 Mar;29(3):166-76; quiz 176-7

    Authors: ( Benign fasciculation syndrome )] Najman L, Seshadri R

    Snake envenomation has been widely reported throughout the human and veterinary literature. The effects of venom include coagulation disorders, neurotoxicity, and tissue effects, such as local swelling and necrosis. Significant progress has been made in understanding the pathophysiology of envenomation, leading to changes in treatment protocols. Recent developments include the production of a new antivenin and a canine rattlesnake vaccine.

    PMID 17726937 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Interspike interval analysis in a patient with peripheral nerve hyperexcitability and potassium channel antibodies.
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    Interspike interval analysis in a patient with peripheral nerve hyperexcitability and potassium channel antibodies.

    Muscle Nerve. 2008 Feb;37(2):269-74

    Authors: ( Benign fasciculation syndrome )] Kleine BU, Stegeman DF, Drost G, Zwarts MJ

    Neuromyotonia or Isaacs' syndrome is a rare peripheral nerve hyperexcitability disorder caused by antibodies against potassium channels of myelinated axons. We present the high-density surface electromyographic (EMG) recordings of a patient with fasciculations and cramps due to neuromyotonia. To characterize the time course of hyperexcitability, we analyzed the interspike intervals (ISIs) between fasciculation potentials, doublet, and multiplet discharges. ISI duration increased within each burst. The ISI histograms found can be explained by the recovery cycle of the myelinated axon and its dependency on the slow potassium conductance. We conclude that ISI analysis is a useful tool to understand the membrane dynamics underlying abnormal motor unit activity. Muscle Nerve, 2007.

    PMID 17636480 [Pubmed ( Benign fasciculation syndrome )- in process]

  • Fasciculation potentials in high-density surface EMG.
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    Fasciculation potentials in high-density surface EMG.

    J Clin Neurophysiol. 2007 Jun;24(3):301-7

    Authors: ( Benign fasciculation syndrome )] Drost G, Kleine BU, Stegeman DF, van Engelen BG, Zwarts MJ

    Fasciculation potentials (FPs) are observed in healthy individuals, but also in patients with neurogenic disorders. The exact site of origin and the clinical relevance in distinguishing, for example, amyotrophic lateral sclerosis (ALS) from other neurogenic diseases based on specific characteristics of the FPs is still a matter of debate and needs further exploration. This report describes the use of high-density surface EMG (HD-sEMG), with multiple electrodes in a compact grid to noninvasively record FPs. The technique provides both temporal and spatial information of fasciculations. Examples of the FPs of a patient diagnosed with definite ALS are presented. FPs are shown in different electrode montages and the unique spatial characteristics of different FPs are presented. During 30-second recordings, 137 FPs were detected that via a decomposition algorithm could be assigned to 11 different underlying sources. It is concluded that HD-sEMG, both because of its noninvasive character and the unique spatiotemporal information, is very suitable to examine fasciculations. It allows long stable recording times and provides quantitative information. This electrophysiologic tool is expected to expand the existing knowledge of FP properties.

    PMID 17545837 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Rattlesnake envenomation with neurotoxicity refractory to treatment with crotaline Fab antivenom.
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    Rattlesnake envenomation with neurotoxicity refractory to treatment with crotaline Fab antivenom.

    Clin Toxicol (Phila). 2007 Jun-Aug;45(5):472-5

    Authors: ( Benign fasciculation syndrome )] Richardson WH, Goto CS, Gutglass DJ, Williams SR, Clark RF

    INTRODUCTION: Neurotoxicity following rattlesnake envenomation is reported with certain crotaline species. In some instances, crotaline Fab antivenom therapy that effectively halts progression of local tissue edema and hemotoxicity fails to reverse neurologic venom effects. CASE SERIES: A 50-year-old man presented following a rattlesnake envenomation to the left ring finger. He had swelling and pain in the affected hand and complained of dyspnea and dysphonia. Significant fasciculations were seen in the face, tongue, neck, trunk, and arms. The patient received crotaline Fab antivenom but continued to develop worsening respiratory distress. His respiratory insufficiency requiring ventilatory support appeared related to respiratory muscle incoordination as extremity motor function remained intact. Initial control of local edema progression and hematologic parameters was achieved with antivenom, but diffuse fasciculations involving the entire body worsened despite aggressive antivenom treatment. In another case, a 9-year-old boy was envenomated by a rattlesnake on the left thenar eminence. He presented with pain and swelling up to the forearm and fasciculations of the tongue, face, and upper extremities. The progression of edema was halted at the mid-bicep level and hematologic parameters normalized with crotaline Fab antivenom. However, fasciculations continued for two days despite antivenom treatment. CONCLUSION: We describe two cases of neurotoxicity following rattlesnake envenomation in which treatment with crotaline Fab antivenom adequately obtained initial control of local swelling and hematologic effects, but neurotoxic venom effects remained refractory to antivenom therapy. This phenomenon is anecdotally recognized following certain crotaline species envenomations.

    PMID 17503249 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Comparison of clinical methods for fasciculation detection in amyotrophic lateral sclerosis.
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    Comparison of clinical methods for fasciculation detection in amyotrophic lateral sclerosis.

    Muscle Nerve. 2007 Sep;36(3):404-5

    Authors: ( Benign fasciculation syndrome )] Mateen FJ, Sorenson EJ, Daube JR

    PMID 17471569 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Fibration, fibrillation, and fasciculation: say what you see.
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    Fibration, fibrillation, and fasciculation: say what you see.

    Clin Neurophysiol. 2007 Jun;118(6):1418-20

    Authors: ( Benign fasciculation syndrome )] van Baalen A, Stephani U

    PMID 17452004 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Intraretinal projection of retinal ganglion cell axons as a model system for studying axon navigation.
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    Intraretinal projection of retinal ganglion cell axons as a model system for studying axon navigation.

    Brain Res. 2008 Feb 4;1192:165-77

    Authors: ( Benign fasciculation syndrome )] Bao ZZ

    The initial step of retinal ganglion cell (RGC) axon pathfinding involves directed growth of RGC axons toward the center of the retina, the optic disc, a process termed "intraretinal guidance". Due to the accessibility of the system, and with various embryological, molecular and genetic approaches, significant progress has been made in recent years toward understanding the mechanisms involved in the precise guidance of the RGC axons. As axons are extending from RGCs located throughout the retina, a multitude of factors expressed along with the differentiation wave are important for the guidance of the RGC axons. To ensure that the RGC axons are oriented correctly, restricted to the optic fiber layer (OFL) of the retina, and exit the eye properly, different sets of positive and negative factors cooperate in the process. Fasciculation mediated by a number of cell adhesion molecules (CAMs) and modulation of axonal response to guidance factors provide additional mechanisms to ensure proper guidance of the RGC axons. The intraretinal axon guidance thus serves as an excellent model system for studying how different signals are regulated, modulated and integrated for guiding a large number of axons in three-dimensional space.

    PMID 17320832 [Pubmed ( Benign fasciculation syndrome )- in process]

  • Dysesthesia and fasciculation: unusual complications following face-lift with cog threads.
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    Dysesthesia and fasciculation: unusual complications following face-lift with cog threads.

    Dermatol Surg. 2007 Feb;33(2):253-5; discussion 255

    Authors: ( Benign fasciculation syndrome )] Lee CJ, Park JH, You SH, Hwang JH, Choi SH, Kim CH

    PMID 17300616 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Lidocaine or diazepam can decrease fasciculation induced by succinylcholine during induction of anesthesia.
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    Lidocaine or diazepam can decrease fasciculation induced by succinylcholine during induction of anesthesia.

    Middle East J Anesthesiol. 2006 Jun;18(5):929-31

    Authors: ( Benign fasciculation syndrome )] Hassani M, Sahraian MA

    Succinylcholine is used during induction of anesthesia, and it may induce fasciculations. In this study we demonstrated that intravenous diazepam (1 mg/kg) or lidocaine (1.5 mg/kg) can decrease fasciculations induced by succinylcholine. There is no significant difference between these two drugs in reducing fasciculations moreover, these drugs can also prevent raised blood pressure and heart rate during intubation.

    PMID 17094530 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Pretreatment with magnesium sulphate is associated with less succinylcholine-induced fasciculation and subsequent tracheal intubation-induced hemodynamic changes than precurarization with vecuronium during rapid sequence induction.
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    Pretreatment with magnesium sulphate is associated with less succinylcholine-induced fasciculation and subsequent tracheal intubation-induced hemodynamic changes than precurarization with vecuronium during rapid sequence induction.

    Acta Anaesthesiol Belg. 2006;57(3):253-7

    Authors: ( Benign fasciculation syndrome )] Sakuraba S, Serita R, Kosugi S, Eriksson LI, Lindahl SG, Takeda J

    Although it has side effects, succinylcholine is still widely used in rapid sequence induction. The aim of the present study is to evaluate the effects of pretreat ment with magnesium and precurarization of vecuroni um on succinylcholine-induced fasciculation and subse quent tracheal intubation-induced hemodynamic changes during rapid sequence induction. Fifty-five patients were allocated to three groups by a blinded randomization: Group M received saline 100 ml with magnesium 40 mg x kg(-1) for 5 min at 6.5 min before induction and sub sequently administered saline 1-2 ml at 1.5 min before induction; Group V received saline 100 ml for 5 min at 6.5 min before induction and subsequently administered vecuronium 0.02 mg x kg(-1) at 1.5 min before induction; Group C received saline 100 ml for 5 min at 6.5 min before induction and then saline 1-2 ml at 1.5 min before induction. Fasciculation scores and mean percent changes of heart rate, systolic blood pressure and rate pressure product between baseline and after induction were significantly lower in group M than those in group C and group V. Pretreatment with magnesium is more effective to limit succinylcholine-induced fasciculation and subsequent tracheal intubation-induced hemody namic changes in rapid sequence induction compared with vecuronium pretreatment, although magnesium does not prevent the elevation of serum potassium con centration after induction.

    PMID 17067136 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Endometrial damage and apoptosis in rats induced by dichlorvos and ameliorating effect of antioxidant vitamins E and C.
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    Endometrial damage and apoptosis in rats induced by dichlorvos and ameliorating effect of antioxidant vitamins E and C.

    Reprod Toxicol. 2006 Nov;22(4):783-90

    Authors: ( Benign fasciculation syndrome )] Oral B, Guney M, Demirin H, Ozguner M, Giray SG, Take G, Mungan T, Altuntas I

    We aimed to investigate the effect of subchronic administration of dichlorvos (DDVP) on endometrium and to evaluate ameliorating effects of a combination of Vitamins E and C against DDVP toxicity in the rat. Three groups of rats were used in the experiment. The first group was treated with 4 mg/kg DDVP; the second group was treated with 4 mg/kg body weight DDVP plus Vitamins E and C (DDVP+Vit); the third group was given only corn oil (control). DDVP and DDVP+Vit groups were given DDVP by gavage 5 days a week for 4 weeks at a dose level of 4 mg/kg day by using corn oil as the vechicle. Vitamins E and C were injected at doses of 50 mg/kg i.m. and 20 mg/kg body weight i.p. Histopathological and immunohistochemical examinations for caspase-3 and caspase-9 were accomplished in the endometrium. The level of malondialdehyde (MDA) increased significantly in the DDVP group compared with the control group (p<0.05). MDA significantly decreased in the DDVP+Vit group compared with the DDVP group (p<0.05). Administration of Vitamins E and C along with DDVP significantly reduced the histopathological changes and the extent of apoptosis. In conclusion, subchronic DDVP administration caused endometrial damage and that treatment with a combination of Vitamins E and C reduced endometrial damage caused by DDVP.

    PMID 16973328 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Myokymia (fasciculation) of the tongue as a unique presentation of mucoepidermoid carcinoma.
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    Myokymia (fasciculation) of the tongue as a unique presentation of mucoepidermoid carcinoma.

    Int J Oral Maxillofac Surg. 2007 Jan;36(1):79-81

    Authors: ( Benign fasciculation syndrome )] Andrews KV, Eveson JW

    Neural invasion is a relatively common feature of squamous cell carcinomas of the head and neck and malignant salivary gland tumours. The symptoms depend on the location and the particular nerves involved, and include pain, anaesthesia, paraesthesia and cranial nerve palsy. The present case appears to be unique. A mucoepidermoid carcinoma showed evidence of neural involvement and presented with nerve stimulation inducing myokymia, rather than nerve destruction, which is the usual consequence of tumoral nerve invasion.

    PMID 16965900 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • BMP signaling regulates murine enteric nervous system precursor migration, neurite fasciculation, and patterning via altered Ncam1 polysialic acid addition.
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    BMP signaling regulates murine enteric nervous system precursor migration, neurite fasciculation, and patterning via altered Ncam1 polysialic acid addition.

    Dev Biol. 2006 Nov 1;299(1):137-50

    Authors: ( Benign fasciculation syndrome )] Fu M, Vohra BP, Wind D, Heuckeroth RO

    The enteric nervous system (ENS) forms from migrating neural crest-derived precursors that differentiate into neurons and glia, aggregate into ganglion cell clusters, and extend neuronal processes to form a complex interacting network that controls many aspects of intestinal function. Bone morphogenetic proteins (BMPs) have diverse roles in development and influence the differentiation, proliferation, and survival of ENS precursors. We hypothesized that BMP signaling might also be important for the ENS precursor migration, ganglion cell aggregation, and neurite fasciculation necessary to form the enteric nervous system. We now demonstrate that BMP signaling restricts murine ENS precursors to the outer bowel wall during migration. In addition, blocking BMP signaling causes faster colonization of the murine colon, reduces ganglion cell aggregation, and reduces neurite fasciculation. BMP signaling also influences patterns of neurite extension within the developing bowel wall. These effects on ENS precursor migration and neurite fasciculation appear to be mediated at least in part by increased polysialic acid addition to neural cell adhesion molecule (Ncam1) in response to BMP. Removing PSA enzymatically reverses the BMP effects on ENS precursor migration and neurite fasciculation. These studies demonstrate several novel roles for BMP signaling and highlight new functions for sialyltransferases in the developing ENS.

    PMID 16952347 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Laser Doppler spectroscopy towards the detection of spontaneous muscle activity.
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    Laser Doppler spectroscopy towards the detection of spontaneous muscle activity.

    Clin Neurophysiol. 2006 Oct;117(10):2279-83

    Authors: ( Benign fasciculation syndrome )] Stambouli D, Stamboulis E, Papazoglou TG, Siafakas A, Fotakis C

    OBJECTIVE: We used laser Doppler spectroscopy to detect hyperactivity of muscle fibers caused by the presence of fibrillations, positive waves and fasciculations. METHODS: The proposed method relies upon the dynamic scattering of light by moving particles, which causes a Doppler shift of the original frequency. For resolution of small alterations in frequency the heterodyne detection was used. One hundred seventy-three normal and 109 EMG diagnosed denervated first dorsal interosseous muscles were examined. RESULTS: Denervated muscles with fasciculations showed significant differentiation from control muscles, whereas denervated muscles without fasciculations were not significantly differentiated. The smallest differentiation compared to controls was seen in chronic denervated muscles without spontaneous activity. CONCLUSIONS: Presence of fasciculations in denervated muscles allowed Doppler spectroscopy to differentiate them from normal muscles. Presence of atrophy reduced the diagnostic potential of the method. SIGNIFICANCE: Our findings can be used as a basis for further improving painless and noninvasive laser Doppler spectroscopy to be used clinically as an alternative to painful and invasive EMG.

    PMID 16931147 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • The C. elegans gene dig-1 encodes a giant member of the immunoglobulin superfamily that promotes fasciculation of neuronal processes.
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    The C. elegans gene dig-1 encodes a giant member of the immunoglobulin superfamily that promotes fasciculation of neuronal processes.

    Dev Biol. 2006 Nov 1;299(1):193-205

    Authors: ( Benign fasciculation syndrome )] Burket CT, Higgins CE, Hull LC, Berninsone PM, Ryder EF

    The adhesion of growing neurites into appropriate bundles or fascicles is important for the development of correct synaptic connectivity in the nervous system. We describe fasciculation defects of animals with mutations in the C. elegans gene dig-1 and show that dig-1 encodes a giant molecule (13,100 amino acids) of the immunoglobulin superfamily. Five new alleles of dig-1 were isolated in a screen for mutations affecting the morphology or function of several classes of head sensory neurons. Mutants showed process defasciculation of several classes of neurons. Analysis of a temperature-sensitive allele revealed that dig-1 is required during embryogenesis for normal process fasciculation of one class of head sensory neuron. Partial sequencing of two alleles, RNA interference (RNAi) and rescuing experiments showed that dig-1 encodes a giant molecule of the immunoglobulin superfamily. DIG-1 protein contains many domains associated with adhesion, is likely secreted, and has some features of proteoglycans. dig-1 mutants were originally isolated due to their displaced gonads [Thomas, J.H., Stern, M.J., Horvitz, H.R., 1990. Cell interactions coordinate the development of the C. elegans egg-laying system. Cell 62, 1041-52]; thus, dig-1 alleles were also characterized for their effects on gonad placement. Mutant phenotypes suggest that DIG-1 may mediate cell movement as well as process fasciculation and that different regions of the protein may mediate these functions.

    PMID 16928366 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Predictors of delay in the diagnosis and clinical trial entry of amyotrophic lateral sclerosis patients: a population-based study.
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    Predictors of delay in the diagnosis and clinical trial entry of amyotrophic lateral sclerosis patients: a population-based study.

    J Neurol Sci. 2006 Dec 1;250(1-2):45-9

    Authors: ( Benign fasciculation syndrome )] Zoccolella S, Beghi E, Palagano G, Fraddosio A, Samarelli V, Lamberti P, Lepore V, Serlenga L, Logroscino G,

    BACKGROUND: The El Escorial and the revised Airlie House diagnostic criteria for amyotrophic lateral sclerosis (ALS) were introduced to select patients for clinical trials. Heterogeneity of clinical presentation at onset and delay in diagnosis may decrease the likelihood for trial entry. OBJECTIVE: Identify risk factors for delay in the diagnosis and trial exclusion. METHODS: ALS incident cases were identified with El Escorial (EEC) and Airlie House criteria (AHC) through a population-based registry established in Puglia, Southern Italy, in the years 1998-99. RESULTS: 130 ALS incident cases were diagnosed with a median interval between onset of symptoms and diagnosis of 9.3 months and not different across both EEC and AHC categories. Twenty percent of cases were not eligible for clinical trials according to the AHC. About 5% of subjects in this series died with only lower motor neuron signs. Predictors for delay in the diagnosis were age between 65 and 75 years and spinal onset while fasciculations and cramps as first symptoms were predictors of exclusion from trials. CONCLUSIONS: In this population-based series, diagnostic delay was longer in subjects with spinal onset and age between 65 and 75 and fasciculation as first symptoms. About 80% of incident cases were trial eligible with AHC criteria. However, a significant number of subjects with ALS, characterized by a limited spread of signs, were not trial eligible while alive.

    PMID 16920152 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • A 52-year-old man with daytime sleepiness, sialorrhea, and facial fasciculations.
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    A 52-year-old man with daytime sleepiness, sialorrhea, and facial fasciculations.

    Chest. 2006 Jul;130(1):287-90

    Authors: ( Benign fasciculation syndrome )] Ahuja A, Gothi D, Joshi JM

    PMID 16840414 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Is the dose-related reduction in succinylcholine-induced myalgia due to cointervention?
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    Is the dose-related reduction in succinylcholine-induced myalgia due to cointervention?

    Anesthesiology. 2006 Jul;105(1):222; author reply 223

    Authors: ( Benign fasciculation syndrome )] Kettler RE

    PMID 16810018 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Reversible tremor, myoclonus, and fasciculations associated with topiramate use for migraine.
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    Reversible tremor, myoclonus, and fasciculations associated with topiramate use for migraine.

    Clin Neuropharmacol. 2006 May-Jun;29(3):157-9

    Authors: ( Benign fasciculation syndrome )] Alonso-Navarro H, Jiménez-Jiménez FJ

    OBJECTIVE: To report a patient with tremor, multifocal myoclonus, and fasciculations, possibly induced by the antiepileptic drug topiramate. CASE REPORT: A 46-year-old woman with frequent episodes of migraine without aura that were not controlled with other prophylactic drug, developed a reversible clinical picture of postural tremor, multifocal myoclonus, weight loss, amyotrophia, and fasciculations after exposure to topiramate. These symptoms disappeared after topiramate withdrawal. CONCLUSIONS: Tremor, myoclonus, and fasciculations should be considered as a possible adverse effect of topiramate.

    PMID 16772816 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Retroviral misexpression of cVax disturbs retinal ganglion cell axon fasciculation and intraretinal pathfinding in vivo and guidance of nasal ganglion cell axons in vivo.
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    Retroviral misexpression of cVax disturbs retinal ganglion cell axon fasciculation and intraretinal pathfinding in vivo and guidance of nasal ganglion cell axons in vivo.

    Dev Biol. 2006 Sep 1;297(1):59-73

    Authors: ( Benign fasciculation syndrome )] Mühleisen TW, Agoston Z, Schulte D

    The transcription factor cVax (Vax2) is expressed in the ventral neural retina and restricted expression is a prerequisite for at least three prominent aspects of retinal dorsal-ventral patterning: polarized expression of EphB/B-ephrin molecules, the retinotectal projection and the distribution of rod photoreceptors across the retina. In the chick retina, the fasciculation pattern of ganglion cell axons also differs between the dorsal and ventral eye. To investigate the molecular mechanisms involved, the nerve fiber layer was analyzed after retroviral misexpression of several factors known to regulate the positional specification of retinal ganglion cells. Forced cVax expression ventralized the fasciculation pattern and caused axon pathfinding errors near the optic disc. Ectopic expression of different ephrin molecules indicated that axon fasciculation is, at least in part, mediated by the EphB system. Finally, we report that retroviral misexpression of cVax increased the pool of EphA4 receptors phosphorylated on tyrosine residues and altered the guidance preference of nasal axons in vitro. These results identify novel functions for cVax in intraretinal axon fasciculation and pathfinding as well as suggest a mechanism to explain how restricted cVax expression may influence map formation along the dorso-ventral and antero-posterior axes of the optic tectum.

    PMID 16769047 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Urticaria and lip fasciculation may be prodromal signs of brain malignancy.
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    Urticaria and lip fasciculation may be prodromal signs of brain malignancy.

    Dermatol Online J. 2006;12(3):23

    Authors: ( Benign fasciculation syndrome )] Shamsadini S, Varesvazirian M, Shamsadini A

    The association of urticaria and cancer usually is seen with lymphoreticular system malignancies. Recalcitrant intra-nostril pruritus has been associated with fourth-ventricle tumors of the brain. Rarely, urticaria has been described with cancer of the lung, usually small-cell adenocarcinoma. We describe a girl who suffered with chronic urticaria for 3 months before lip fasciculation began to be observed. CT scan revealed a brain tumor adjacent to the cerebellum, which was diagnosed as astrocytoma grade II. Because of the location, the tumor was not operable, but after one course of radiotherapy, both the urticaria and lip fasciculation disappeared.

    PMID 16638437 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Infectious mononucleosis and unilateral tongue writhing.
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    Infectious mononucleosis and unilateral tongue writhing.

    Neurology. 2006 Apr 11;66(7):1110

    Authors: ( Benign fasciculation syndrome )] van Baalen A, Petersen B, Stephani U

    PMID 16606931 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Usefulness of transcervical approach for surgical treatment of hypoglossal schwannoma with paraspinal extension: case report.
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    Usefulness of transcervical approach for surgical treatment of hypoglossal schwannoma with paraspinal extension: case report.

    Surg Neurol. 2006 Apr;65(4):397-401, discussion 401

    Authors: ( Benign fasciculation syndrome )] Sato K, Shimizu S, Oka H, Nakahara K, Utsuki S, Fujii K

    BACKGROUND: Usefulness of transcervical approach to hypoglossal schwannoma with paraspinal extension is described herein. CASE DESCRIPTION: A 54-year-old woman presented with gradually worsening left hypoglossal nerve palsy. The findings were of a tumor lying in the left hypoglossal canal and paraspinal region and were consistent with hypoglossal schwannoma. Subtotal intracapsular removal of the tumor was performed via transcervical approach. The symptoms improved, and no additional symptoms were noted. CONCLUSION: The transcervical approach and intracapsular removal of the tumor under electrophysiological monitoring provided for successful minimally invasive surgery in this case of hypoglossal schwannoma.

    PMID 16531208 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Altered axonal excitability properties in amyotrophic lateral sclerosis: impaired potassium channel function related to disease stage.
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    Altered axonal excitability properties in amyotrophic lateral sclerosis: impaired potassium channel function related to disease stage.

    Brain. 2006 Apr;129(Pt 4):953-62

    Authors: ( Benign fasciculation syndrome )] Kanai K, Kuwabara S, Misawa S, Tamura N, Ogawara K, Nakata M, Sawai S, Hattori T, Bostock H

    Fasciculations are a characteristic feature of amyotrophic lateral sclerosis (ALS), and can arise proximally or distally in the motor neuron, indicating a widespread disturbance in membrane excitability. Previous studies of axonal excitability properties (i.e. threshold electrotonus, strength-duration time constant) have suggested respectively that change in potassium or sodium channels may be involved. To reinvestigate these changes and explore their correlation with disease stage, multiple axonal excitability properties (threshold electrotonus, strength-duration time constant, recovery cycle and current-threshold relationship) were measured for the median nerve at the wrist in 58 ALS patients, and compared with 25 age-matched controls. In ALS, there were greater changes in depolarizing threshold electrotonus (i.e. less accommodation) (P < 0.001) and greater supernormality in the recovery cycles (P < 0.001). These abnormalities were more prominent in patients with moderately reduced CMAP (1-5 mV). Modelling the excitability changes in this group supported the hypothesis that axonal potassium conductances are reduced, resulting in increased supernormality despite membrane depolarization. The tendency for strength-duration time constant to be prolonged in ALS was only significant for patients with normal CMAP amplitude (>5 mV). Patients with severely reduced CMAP (<1 mV) alone showed reduced threshold changes to hyperpolarizing current. These results suggest a changing pattern of abnormal membrane properties with disease progression. First, persistent Na+ conductance increases, possibly associated with collateral sprouting, and then K(+) conductances decline. Both changes cause axonal hyperexcitability, and may contribute to the generation of fasciculations. These serial changes in axonal properties could provide insights into the pathophysiology of ALS, and implications for future therapeutic options.

    PMID 16467388 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • fasciculations, autonomic symptoms and limbic encephalitis: a thymoma-associated Morvan's-like syndrome.
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    fasciculations, autonomic symptoms and limbic encephalitis: a thymoma-associated Morvan's-like syndrome.

    Eur Neurol. 2005;54(4):235-7

    Authors: ( Benign fasciculation syndrome )] Deymeer F, Akca S, Kocaman G, Parman Y, Serdaroglu P, Oktem-Tanor O, Coban O, Vincent A

    PMID 16401901 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • Myasthenia gravis--a rare presentation with tongue atrophy and fasciculation.
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    Myasthenia gravis--a rare presentation with tongue atrophy and fasciculation.

    Age Ageing. 2006 Jan;35(1):87-8

    Authors: ( Benign fasciculation syndrome )] Burch J, Warren-Gash C, Ingham V, Patel M, Bennett D, Chaudhuri KR

    We report the case of an unusual presentation of myasthenia gravis with tongue atrophy and fasciculation. Myasthenia gravis is an autoimmune condition associated with weakness and fatigability of voluntary muscles. In >50%, the initial symptoms and signs are related to extraocular muscle weakness, such as diplopia or ptosis [Tsung K, Seggev JS. An unusual cause of dysphagia. West J Med 1995; 163: 159-60]. Rarely, it is known to affect bulbar muscles and can lead to dysphagia and respiratory compromise.

    PMID 16364941 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

  • De novo GMP synthesis is required for axon guidance in Drosophila.
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    De novo GMP synthesis is required for axon guidance in Drosophila.

    Genetics. 2006 Mar;172(3):1633-42

    Authors: ( Benign fasciculation syndrome )] Long H, Cameron S, Yu L, Rao Y

    Guanine nucleotides are key players in mediating growth-cone signaling during neural development. The supply of cellular guanine nucleotides in animals can be achieved via the de novo synthesis and salvage pathways. The de novo synthesis of guanine nucleotides is required for lymphocyte proliferation in animals. Whether the de novo synthesis pathway is essential for any other cellular processes, however, remains unknown. In a search for genes required for the establishment of neuronal connectivity in the fly visual system, we identify the burgundy (bur) gene as an essential player in photoreceptor axon guidance. The bur gene encodes the only GMP synthetase in Drosophila that catalyzes the final reaction of de novo GMP synthesis. Loss of bur causes severe defects in axonal fasciculation, retinotopy, and growth-cone morphology, but does not affect photoreceptor differentiation or retinal patterning. Similar defects were observed when the raspberry (ras) gene, encoding for inosine monophosphate dehydrogenase catalyzing the IMP-to-XMP conversion in GMP de novo synthesis, was mutated. Our study thus provides the first in vivo evidence to support an essential and specific role for de novo synthesis of guanine nucleotides in axon guidance.

    PMID 16322525 [Pubmed ( Benign fasciculation syndrome )- in process]

  • Sensory symptoms in acquired neuromyotonia.
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    Sensory symptoms in acquired neuromyotonia.

    Neurology. 2005 Oct 25;65(8):1330-1

    Authors: ( Benign fasciculation syndrome )] Herskovitz S, Song H, Cozien D, Scelsa SN

    PMID: 16247076 [Pubmed ( Benign fasciculation syndrome )- indexed for MEDLINE ( Benign fasciculation syndrome )]

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