Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0028738 (nystagmus)
 7,431 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 66-year-old man was admitted to Tokyo Metropolitan Geriatric Hospital because of progressive speech disturbance and ataxic gait. He had no history of abdominal surgery, liver disease, heavy drinking nor blood transfusion. One day before the admission, he showed confusional behavior for 6 hours, but he had no other history of consciousness disturbance. Neurological examination revealed slurred speech, fixation nystagmus, limb and gait ataxia, and hyperreflexia with pathological reflexes. Extrapyramidal signs were not identified. Laboratory examination showed marked hyperammonemia (118-292 micrograms/dl) with poor ICG excretion (ICG15 min = 30.8%). Percutaneous portography showed a large shunt vessel between the portal vein and the left hepatic vein. The plasma ammonium level of the right hepatic vein is normal (15 micrograms/dl), but that of the left one is very high (298 micrograms/dl). Therefore we concluded that hyperammonemia of the systemic circulation was resulted from portal-systemic shunt. A T2 weighted MRI image demonstrated symmetrical high signal intensity in the deep cerebral white matter. Unique lesions were observed in the bilateral middle cerebellar peduncles, and shown as low signal intensity in T1 weighted image and as high signal intensity in T2 weighted image. Hyperammonemia and neurological impairments of this patient did not improve with medical treatment. Three months after occlusion of the shunt vessel, fixation nystagmus and extensor plantar responses abolished and unsteadiness gait improved. Hyperammonemia might cause the cerebellar ataxia in the present case.
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PMID:[A case of portal-systemic shunt with progressive ataxia]. 833 93

Two cases of alcoholic cerebellar degeneration with pyramidal sign were reported. Patient 1 with alcohol dependence syndrome was a 46-year-old woman. After the alcohol abuse of about eight years, she complained of gait disturbance. The gait disturbance progressively worsened in about two months and she could not ambulate freely by herself. Neurological examination revealed nystagmus, ataxic and spastic gait, slight weakness and spasticity of the lower extremities, hyperreflexia of the extremities, bilateral Babinski's signs, and incoordination of the lower extremities. Examination of liver function and serum B12 was normal. Cranial CT scan and MRI revealed atrophy of the cerebellar vermis and dorsal part of the cerebellum. Though neurological signs slightly improved after the admission to our hospital and the abstinence from alcohol abuse, ataxic gait and hyperreflexia of the extremities have continued. Patient 2 was a 58-year-old man. He was a heavy drinker, but was not a patient with alcohol dependence syndrome. After the heavy drinking of about 40 years, he complained of gait disturbance. The gait disturbance had progressively worsened in about four months. Neurological examination revealed ataxic gait, hyperreflexia of the lower extremities, and bilateral Babinski's signs. Laboratory examination revealed slight liver dysfunction with minimal GPT and moderate gamma-GTP elevation. Examination of serum B12 was normal. Cranial CT scan and MRI revealed atrophy of the cerebellar vermis. Though bilateral Babinski's signs disappeared after the abstinence from heavy drinking, ataxic gait and hyperreflexia of the lower extremities have continued. Alcoholic myelopathy without hepatic cirrhosis was rarely reported. In the relation of alcoholic cerebellar degeneration to alcoholic myelopathy, our cases are interesting and important.
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PMID:[Alcoholic cerebellar degeneration with pyramidal sign--in relation to alcoholic myelopathy]. 847 68

This study was performed in 37 patients with alcohol dependency. The mean age was 46.3 years and mean duration of heavy drinking was 23.8 years. The mean duration of temperance to the initial examination was 21.4 days and that to the second examination was 82.0 days. The results of the test on body sway in patients with alcohol dependency may summarized as follows. In patients with alcohol dependency the length of and the area covered by the body sway were significantly larger than in the normal group (p < 0.05). This significant difference from the normal group disappeared open eyes, but remained with eyes closed (p < 0.05). The Romberg quotients of both the length and the environmental area tended to be larger than the normal group mean (p < 0.05). The oscillation power spectrum obtained with eyes open and that obtained with eyes closed tended to be low at levels below 0.2Hz and high at range of 0.2Hz to 0.5Hz in the right left direction. The power spectrum in the anterior-posterior direction obtained with eyes closed was lower than that in the normal group at levels less than 0.2Hz, and higher than that in the normal group at range of 2.0Hz to 3.0Hz on the first examination. After temperance on the second examination, these significant differences had disappeared at all levels. The results of the present study on ocular movements in patients with alcohol dependency may be summarized as follows. Using the oculomotor test, abnormalities appeared in more than 40% of the test subjects for ETT, OKN, OKAN, VS and lateral gaze nystagmus test. According to the report of Matsuoka in 1979, some head positional nystagmus was noted in about 30% of patients with alcohol dependency. In the present study, the rate of appearance was as low as 8%. Ataxic pursuit, vertical positioning nystagmus, decrease of VS, disturbance of OKN and abnormalities of OKAN were found. These results suggested lesions between the brain stem and cerebellum, especially around the upper and lower vermis of cerebellum. After about 3 months of temperance, each test revealed a tendency towards some improvement, but the disturbance appeared to be fixed in some cases. It was important that some improvement in the oculomotor and the central vestibular system was found after about 3 months temperance. These results are quite promising for treatment and recovery in patients with alcohol dependency.
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PMID:[Neurotological examination of patients with alcohol dependency]. 883 Dec 34