No abstract available.
Creutzfeldt-Jakob Syndrome*

No abstract available.
Animals ; Neurons* ; Oxidopamine* ; Rats* ; Substantia Nigra*

Hemimasticatory spasm is a rare neurologic disorder characterized by unilateral, paroxysmal involuntary contraction of the masticatory muscles. It can be confused with other unilateral facial spasms. In this report, we present two patients with hemimasticatory spasm who were initially misdiagnosed with other disorders. The first patient was a 54-year-old man with a 9-year history of right facial spasm. He underwent microvascular decompression, without benefit, at another hospital with the diagnosis of hemifacial spasm two years prior to presenting at our hospital.On examination, right masseter and temporalis muscles had irregular contractions with twitches and prolonged spasms. His brain magnetic resonance imaging was normal.He showed a good response to carbamazepine. The second case was a 60-year-old man presented with a 10-year history of jaw-closing movement. Previous treatment for temporomandibular joint disorder was ineffective. He was previously diagnosed as oromandibular dystonia at another hospital, and biperiden and diazepam treatment resulted in slight improvement. There was tonic contraction and hypertrophy of the left masseter. He improved with carbamazepine. These cases illustrate the importance of hemimasticatory spasm as differential diagnosis, and the good response to carbamazepine.
Nervous System Diseases

No abstract available.
Arm* ; Dyskinesias* ; Hand* ; Multiple Sclerosis*

Beta-fluoroethylacetate has been extensively used as the rodenticide in Korea. In some patients with acute poisoning, beta-fluoroethylacetate caused cerebellar dysfunction as a single and persistent neurologic sequela after a period of an acute neurological disorder which is characterized by mental deterioration, seizures, and respiratory failure. But there has been no report of pathological findings to explain neurological deficit. We tried to verify the histologic changes of the central nervous systems in beta-fluoroethylacetate poisoned rats. Silver staining(Gallyas) was used to evaluate the histology. In acute intoxication experiment with LD50(7mg/Kg), beta-fluoroethylacetate elicited acute onset of consciousness deterioration, generalized tonic-clonic seizures and large amplitude tremulous activity involving whole body with full recovery after 24 hours. There was no discernible pathologic change in CNS in acutely poisoned rats. However, when poisoned with sublethal dose(5mg/Kg) daily for five days, a moderate degree of nerve cell degeneration was found selectively in dentate nucleus, Purkinie cell layer, vestibulo-cochlear nucleus and striatum. This change was not seen in hippocampus, cerebral cortex or cerebellar cortex. These findings were well correlated with the previous reports of selective pathology in human 5-FU intoxication cases. Our preliminary results suggest that beta-fluoroethylacetate, a kind of cellular metabolism inhibitor may induce selective neuropathology mainly involving cerebellar output pathway in rats.
Humans ; Rats ; Animals ; Poisons

Myoclonus epilepsy and ragged-red fiber (MERRF) syndrome is one of the common etiologies of progressive myoclonus epilepsy. The clinical features of MERRF syndrome are myoclonus, seizure, dementia, ataxia, neuropathy, myopathy, deafness, and lipouta. The patients with MERRF syndrome have a point mutation in mitochondrial DNA at 8344 or 8356 nucleotide. We are reporting a patient who developed myoclonus and seizure at the age of eighteen. He later showed cerebellar ataxia, peripheral neuropathy, and cognitive dysfunction. Skeletal muscle biopsy failed to demonstrate ragged-red fibers. He was diagnosed as MERRF syndrome by the mitochondrial DNA analysis. He had 86% mutant mitochondrial genomes (A-)G(8%) mutation) in leukocytes, and his asymptomatic mother had 66%. The absence of ragged-red fibers does not rule out the possibility of MERRF syndrome. Demonstration of mitochondrial DNA mutation is the most convincing method for establishing the diagnosis of MERRF.
Ataxia ; Biopsy ; Cerebellar Ataxia ; Deafness ; Dementia ; Diagnosis ; DNA, Mitochondrial ; Epilepsies, Myoclonic* ; Genome, Mitochondrial ; Humans ; Leukocytes ; MERRF Syndrome ; Mothers ; Muscle, Skeletal ; Muscular Diseases ; Myoclonic Epilepsies, Progressive ; Myoclonus* ; Peripheral Nervous System Diseases ; Point Mutation ; Seizures

No abstract available.
Olivopontocerebellar Atrophies*

We present the first case report of fragile X-associated tremor ataxia syndrome (FXTAS) in the Republic of Korea. A 75-year-old male developed progressive gait ataxia, parkinsonism, and a mood disorder. Magnetic resonance imaging revealed T2 high signal intensity within the middle cerebellar peduncles. Analysis of the fragile X mental retardation 1 gene revealed a CGG trinucleotide repeat number of 136. FXTAS should be considered when a patient has atypical parkinsonism, cerebellar ataxia, and specific MRI abnormalities.

Both genetic and environmental factors play important roles in the pathogenesis of Parkinson Disease (PD). The contribution of many environmental factors including dietary factor remains unproven. The purpose the study was to investigate the dietary habits, nutrient intake and dietary quality of Korean PD patients according to the duration of disease. PD patients were recruited from K and S university hospitals from May 2005 to January 2006. This study was carried out after approval by the Institute Review Board (IRB). British Brain Bank criteria was used to diagnose PD. The subjects were classified into 2 groups based on the duration of PD: < 25 months and > or = 25 months groups. General characteristics, anthropometric measurements, food habits and dietary intakes were investigated. The results of this study were as follows: 1) The mean age of < 25 months group (66.9 +/-8.0 yr) was significantly higher than that of > or = 25 months group (62.2 +/- 8.8 yr) (p < 0.05). No significant differences were found for academic background, occupation, living status and social activity, however, numbers of diseases, exercise and family history of PD were significantly different. 2) Anthropometric measurements were not different between the two groups. 3) The frequency of taking snacks was significantly higher in <25 months group and the amounts of alcohol consumption were significantly higher in > or = 25 months group. 4) Daily intakes of most nutrients were very low compared with DRI. 5) The MAR score was significantly lower in < 25 months group (p < 0.05;) however, the scores of DVS, DDS and DQI were not significantly different. As a conclusion an overall nutrient intake and dietary quality of the Parkinson's Disease patients need to be improved regardless of duration of the disease and a well-balanced diet should be emphasized.
Alcohol Drinking ; Brain ; Diet ; Food Habits ; Hospitals, University ; Humans ; Nutritional Status ; Occupations ; Parkinson Disease ; Snacks

Both genetic and environmental factors play important roles in the pathogenesis of Parkinson Disease (PD). The contribution of many environmental factors including dietary factor remains unproven. The purpose the study was to investigate the dietary habits, nutrient intake and dietary quality of Korean PD patients according to the duration of disease. PD patients were recruited from K and S university hospitals from May 2005 to January 2006. This study was carried out after approval by the Institute Review Board (IRB). British Brain Bank criteria was used to diagnose PD. The subjects were classified into 2 groups based on the duration of PD: < 25 months and > or = 25 months groups. General characteristics, anthropometric measurements, food habits and dietary intakes were investigated. The results of this study were as follows: 1) The mean age of < 25 months group (66.9 +/-8.0 yr) was significantly higher than that of > or = 25 months group (62.2 +/- 8.8 yr) (p < 0.05). No significant differences were found for academic background, occupation, living status and social activity, however, numbers of diseases, exercise and family history of PD were significantly different. 2) Anthropometric measurements were not different between the two groups. 3) The frequency of taking snacks was significantly higher in <25 months group and the amounts of alcohol consumption were significantly higher in > or = 25 months group. 4) Daily intakes of most nutrients were very low compared with DRI. 5) The MAR score was significantly lower in < 25 months group (p < 0.05;) however, the scores of DVS, DDS and DQI were not significantly different. As a conclusion an overall nutrient intake and dietary quality of the Parkinson's Disease patients need to be improved regardless of duration of the disease and a well-balanced diet should be emphasized.
Alcohol Drinking ; Brain ; Diet ; Food Habits ; Hospitals, University ; Humans ; Nutritional Status ; Occupations ; Parkinson Disease ; Snacks