Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0007758 (cerebellar ataxia)
 3,609 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In both Minamata disease (MD) and HTLV-I associated myelopathy (HAM), sensory disturbance is one of the most characteristic clinical symptoms. We have examined median nerve SSEPs (MN-SEP) and posterior tibial nerve SEPs (PTN-SEP) of both patient groups, and reported their specific abnormalities. MN-SEP of MD patients never showed any conduction delay nor conduction block at the cervical cord. However, they demonstrated the initial positive cortical response with low amplitude instead of the initial negative response (N20) seen in healthy subjects. In PTN-SEP, MD patients showed the initial positive cortical response with significantly shorter latency and lower amplitude than healthy subjects. These findings have never been seen in any other diseases. On the other hand, the conduction delay and conduction block on peripheral nerve, spinal cord and/or intracranial sensory tracts have been demonstrated in many cases with HAM. The patient was a 60-year-old man. About 40 years ago, he suffered with typical clinical symptoms of MD such as cerebellar ataxia, intention tremor and sensory disturbance of upper and lower extremities, and then his condition was complicated with progressive spastic paraplegia and urinary bladder dysfunction since 30 years ago. Both MN-SEP and PTN-SEP were studied so that we could make the electrophysiological differential diagnosis of MD and HAM. His MN-SEP indicated both the conduction delay at his cervical cord and intracranial sensory tract and the initial positive potential from cephalic recording. Furthermore, his PTN-SEP demonstrated severe conduction block at the spinal cord and neither cervical response (N28) nor cortical response (P37) was evoked. In conclusion, the clinical electrophysiologic studies supported our notion that the case might be affected with both MD and HAM.
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PMID:[Neurophysiological studies of minamata disease with HTLV-I associated myelopathy--a case report]. 897 28

The Minamata disease was discovered in the Minamata region, Kumamoto Prefecture, Japan, in 1956. Symptoms of this disease included cerebellar ataxia, sensory disturbance, narrowing of the visual field, and hearing and speech disturbances. In 1965, similar conditions were identified in persons living around the Agano River area, Niigata Prefecture, Japan and accordingly termed as the Niigata Minamata disease or the second Minamata disease. Both the diseases have been attributed to poisoning with methyl mercury that was generated during the production of acetaldehyde using mercury as a catalyst. The discharged methyl mercury accumulated in fishes and shellfishes and caused poisoning on consumption. This review discusses the history, clinical presentation including atypical forms, and autopsy findings of the Niigata Minamata disease. In addition, it highlights the problems about criteria for official recognition and the therapeutic trial for this disease.
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PMID:[Clinical aspects of the Niigata Minamata disease]. 2558 33

Organic mercury, especially methylmercury, poisoning causes chronic neurological disease predominantly affecting the brain. There have been documented exposures from eating fish from contaminated waters in Japan and in northwestern Ontario and in Iraq from eating bread made from seed wheat treated with methylmercuric fungicide. The neurological disease is called Minamata disease in Japan. Visual field constriction due to involvement of the calcarine cortex, sensory disturbance due to involvement of the somatosensory cortex, and cerebellar ataxia due to involvement of granule cell neurons of the cerebellum are common and characteristic features due to methylmercury poisoning. Other neurological features include dysarthria, postural and action tremor, cognitive impairment, and hearing loss and dysequilibrium. In contrast, peripheral nerve disease is more characteristic of inorganic mercury intoxication. Similarly, psychosis is more typical of exposure to inorganic mercury, which has been documented in the felt hat industry ("mad hatter"). Laboratory tests (e.g., on blood and hair and toenail samples) are of limited value in the assessment of chronic neurological disease due to mercury poisoning because they may not reflect remote neuronal injury due to mercury. Methylmercury also causes injury to fetal brains during development. There is no effective treatment.
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PMID:Chronic Neurological Disease Due to Methylmercury Poisoning. 3027 52

Minamata disease (MD) is a form of intoxication involving the central nervous system and is caused by ingesting seafood from methylmercury-contaminated areas in Japan. In MD, cerebellar ataxia is a cardinal feature observed in approximately 80% of MD patients. Although cerebellar transcranial magnetic stimulation (TMS) has recently been used for treating cerebellar ataxia, the optimal stimulation conditions remain unclear. Here, we report the first case of cerebellar ataxia in an MD patient that was significantly improved after high-frequency cerebellar TMS. To determine the optimal stimulation conditions, we examined the excitability of the primary motor cortex (M1) using resting-state functional magnetic resonance imaging (rs-fMRI). rs-fMRI revealed M1 hyperconnectivity, which was indicative of activation of the dentato-thalamo-cortical (DTC) pathway. Thus, high-frequency cerebellar TMS was applied to inhibit the DTC pathway. Improvement of cerebellar ataxia was only observed after real TMS, not sham stimulation. As this effect was consistent with inhibition of hyperconnectivity of M1, the effectiveness of high-frequency cerebellar TMS for cerebellar ataxia was thought to be caused by inhibition of the DTC pathway. Therefore, we suggest that the evaluation of M1 excitability using rs-fMRI can be effective for determining the optimal TMS stimulation conditions for cerebellar ataxia.
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PMID:Cerebellar Transcranial Magnetic Stimulation Improves Ataxia in Minamata Disease. 3154 98