motor neuron disease/seizures

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Chorea-acanthocytosis presenting as motor neuron disease.

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BACKGROUND Chorea-acanthocytosis (ChAc) is a rare autosomal recessive disease characterized by involuntary movements, seizures, cognitive changes, myopathy, and axonal neuropathy. METHODS We report a patient who presented with gait impairment and dysarthria. Clinical and neurophysiological

Attempts to reproduce amyotrophic lateral sclerosis in laboratory animals by inoculation of Schu virus isolated from a patient with apparent amyotrophic lateral sclerosis.

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A virus isolated from the CSF of a patient who had amyotrophic lateral sclerosis for 7 years, and prolonged pleocytosis in the CSF, was adapted to suckling mouse brain by subsequent serial blind passages. This Schu virus belongs to the tick-borne encephalitis complex of the genus Flavivirus

Familial pontocerebellar hypoplasia type I with anterior horn cell disease.

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We report the association of pontocerebellar hypoplasia and anterior horn cell disease in three female siblings. One child presented with the classical clinical and neuropathological features of pontocerebellar hypoplasia with associated anterior horn cell disease, described by Barth as

Spontaneous degenerative polioencephalomyelopathy in feeder pigs--a new motor neuron disease?

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A central nervous disorder occurred spontaneously in a herd of feeder pigs characterized by muscle fasciculations, convulsions, squealing, and acute death in numerous animals. Histopathology revealed a degenerative poliomyeloencephalopathy of brain stem and spinal cord consisting of neuronal

Short-term outcome of olfactory ensheathing cells transplantation for treatment of amyotrophic lateral sclerosis.

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OBJECTIVE To determine whether transplanting olfactory ensheathing cells (OECs) is effective in controlling or reversing the deterioration caused by amyotrophic lateral sclerosis (ALS). METHODS Between February 2003 and April 2006, 327 patients (241 males and 86 females) with probable or definite

Creatine Revealed Anticonvulsant Properties on Chemically and Electrically Induced Seizures in Mice.

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Creatine exerts beneficial effects on a variety of pathologies in which energy metabolism and oxidative stress play an etiological role. Creatine supplements have shown beneficial effects on neurological disorders including Parkinson׳s disease, Huntington›s disease, amyotrophic lateral sclerosis, as

Epileptic seizure, cataract, and tongue atrophy during the 8 years after electrical brain injury.

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As a delayed neurologic complication following a high-voltage electrical injury, motor neuron disease-like spinal cord injury has often been reported. However, epileptic seizure as a delayed complication of electrical brain injury has not been reported. We report a 32-year-old man, who developed

Specific antagonism of behavioral action of "uncommon" amino acids linked to motor-system diseases.

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Beta-N-methylamino-L-alanine (BMAA) and beta-N-oxalylamino-L-alanine (BOAA) are chemically related amino acids present in the seeds of Cycas circinalis and Lathyrus sativus, respectively. Consumption of these seeds has been linked to Guam amyotrophic lateral sclerosis (BMAA) and lathyrism (BOAA; a

Seizures and neurodegeneration induced by 4-aminopyridine in rat hippocampus in vivo: role of glutamate- and GABA-mediated neurotransmission and of ion channels.

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Infusion of the K(+) channel blocker 4-aminopyridine in the hippocampus induces the release of glutamate, as well as seizures and neurodegeneration. Since an imbalance between excitation and inhibition, as well as alterations of ion channels, may be involved in these effects of 4-aminopyridine, we

A new mouse line with reduced GluA2 Q/R site RNA editing exhibits loss of dendritic spines, hippocampal CA1-neuron loss, learning and memory impairments and NMDA receptor-independent seizure vulnerability.

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Calcium (Ca²⁺)-permeable AMPA receptors may, in certain circumstances, contribute to normal synaptic plasticity or to neurodegeneration. AMPA receptors are Ca²⁺-permeable if they lack the GluA2 subunit or if GluA2 is unedited at a single nucleic acid, known as the Q/R site. In

A special kind of anterior horn cell involvement in juvenile myoclonic epilepsy demonstrated by macro electromyography.

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Juvenile myoclonic epilepsy (JME) is not an uncommon seizure disorder, occurring in 5-10% of epileptic patients. A subclinical anterior horn cell involvement has been suggested in some JME patients by concentric needle electromyography (EMG) and turn/amplitude analysis. In this study, 22 JME

Hirayama disease with juvenile myoclonic epilepsy: A case report.

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Hirayama disease (HD) is rare, but benign anterior horn cell disease, predominantly affecting young men. One of the symptoms, besides weakness, is abnormal movement in the hand. Juvenile myoclonic epilepsy (JME) is one of the most common types of generalized epilepsies and can be recognized by a

Survival and Motor Phenotypes in FVB C9-500 ALS/FTD BAC Transgenic Mice Reproduced by Multiple Labs

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Mordes et al. (2020) did not detect the survival or motor phenotypes in C9orf72 BAC transgenic mice originally described by Liu et al. (2016). We discuss methodological differences between the Mordes and Liu studies, several additional studies in which survival and motor phenotypes were found, and

[Human supplementary motor area: a role in voluntary movements and its clinical significance].

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There were two hypotheses of functions of supplementary motor area (SMA): supplementary vs. supramotor, in 1980s. Clinically, SMA can develop a very intractable seizure focus characterized by unique ictal motor symptoms, and its dysfunction is also strongly related to the cardinal clinical features

Huntington's disease-like 2: a phenocopy not to miss

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A 67-year-old Brazilian man of African ancestry and his 60-year-old sister both presented with choreiform movements, although in the man these were significantly overshadowed by additional parkinsonism. The man also had a history of four epileptic seizures. Neurological examination in each also

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