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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ovine GM-1 gangliosidosis is an inherited lysosomal storage disease. Nine lambs affected with the disease were studied to characterize clinical signs and to determine if there were any pathognomonic clinicopathologic abnormalities. Evaluation included physical, ophthalmic, and neurologic examinations, complete blood counts, serum enzyme and electrolyte analyses, urinalyses, cerebrospinal fluid analyses, blood gas analyses, roentgenograms, electromyograms, and electrocardiograms. Two affected lambs had clinicopathologic tests performed before and after the onset of clinical signs. The only consistent abnormalities recognized were nonspecific signs referable to the central nervous system; predominantly ataxia, conscious proprioceptive deficit most severe in the hind limbs, blindness, and recumbency. Lambs continued to eat and drink, though at diminished levels and with loss of body condition. It was concluded that there are no pathognomonic clinicopathologic abnormalities associated with ovine GM-1 gangliosidosis, and antemortem diagnosis requires enzyme assay of leukocytes or cultured fibroblasts, or lectin histochemistry of tissues obtained by biopsy. Lysosomal storage diseases should be considered among the differential diagnoses in young animals presenting with early neonatal death or with nonspecific neurological signs, in concert with an absence of diagnostic clinicopathologic findings.
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PMID:Clinical and clinicopathologic characteristics of ovine GM-1 gangliosidosis. 806 59

The case of a chronic alcoholic patient with Wernicke's encephalopathy accompanied by passing blindness is reported and the alcoholic amblyopia is discussed in this study. The patient was a 39 year-old male who had been a heavy drinker for 13 years, and was habitually inebriated for the last one year. Disturbance of consciousness ataxia of gait, nystagmus and blindness were manifested on admission. Decreased level of serum vitamin B1 was also recognized at admission. The symptoms diminished from about a month after admission except for horizontal nystagmus. Since the patient had racket-like scotoma in his central visual field, his blindness was thought to be alcoholic amblyopia. Although alcohol dependence is associated with many physical disabilities, there are few reports about Wernicke's encephalopathy with alcoholic amblyopia. This case demonstrates the importance of careful physical examination for understanding alcohol-related disabilities and alcohol dependence.
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PMID:[A case of Wernicke's encephalopathy which accompanied a passing blindness]. 819 11

A flock of 9 1/2-week-old commercial tom turkeys experienced high mortality after consuming a complete feed containing an unidentified toxic substance. Initially, turkeys were found dead. Clinically, the birds were calm and still but became hyperexcitable with noise. A small percentage of birds exhibited torticollis, opisthotonos, circling, ataxia, and blindness. Findings at necropsy and upon microscopic examination were bilaterally symmetrical areas of necrosis of the cerebral hemispheres in the area of the neostriatum that were well demarcated from the surrounding normal neuropil. A feeding trial with the suspect feed in twelve 4-week-old turkey hens induced clinical disease and gross and microscopic brain changes similar to those observed in the field case. Analyses for the following substances in the suspect feed were either negative or within acceptable limits: salt, selenium, furazolidone, monensin, amprolium, 3-nitro-4-hydroxyphenylarsonic acid, aflatoxin, deoxynivalenol, zearalenone, T-2 toxin, ochratoxin, fumonisin, organophosphates, chlorinated hydrocarbons, and carbamates. The toxic component of the feed remains unidentified.
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PMID:Cerebral encephalomalacia in commercial turkeys. 825 95

This study was carried out on 170 children admitted to the University Hospital of Brazzaville (Congo) for cerebral malaria between January 1, 1988 and June 30, 1989. The selection criteria were 1) unarousable coma, cerebrospinal fluid without microorganisms or a marked cellular reaction, and the absence of other causes, and 2) that the children lived in Brazzaville. The case fatality rate was 15%. In 75% of the cases, death occurred within the first 48 hr. The prognosis worsened with the stage of the coma and a younger age. At discharge from the hospital, 9% of the cases presented with sequelae. The postcerebral malaria mortality was high; indeed, death occurred in six (7%) of 90 children discharged from the hospital whose parents were contacted between nine and 27 months later. Two deaths were directly related to neurologic sequelae. Among the 58 children examined under satisfactory conditions between nine and 27 months (mean 16.9 months) after discharge, 50% (3 of 6) still presented with attenuated forms of the sequelae observed immediately after the episode of cerebral malaria (cortical blindness had regressed completely, unlike ataxia and loss of balance). Disorders that may have been related to the episode of cerebral malaria were observed in 31% of these 58 cases.
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PMID:Mortality and sequelae due to cerebral malaria in African children in Brazzaville, Congo. 844 26

Toxic chemicals in the environment can cause a wide range of neurological disease. High-dose exposures to environmental neurotoxicants have produced encephalopathy in children ingesting chips of lead-based paint, blindness in persons who ingested methanol, blindness and ataxia in persons who consumed organic mercury, spinal cord degeneration and peripheral neuropathy in persons exposed to tri-ortho-cresyl phosphate (TOCP), and Parkinsonism in persons exposed to MPTP or to manganese. Environmental neurotoxicants have also been shown to produce a wide range of subclinical neurotoxic effects, including reduction in intelligence, impairment in reasoning ability, shortening of attention span, and alternation of behavior. The first step in the prevention of environmental neurotoxicity is to test chemicals for their toxic potential. More than 70,000 chemicals are currently in commerce. However, except for pharmaceuticals, fewer than 10% of these chemicals have been tested for neurotoxicity. A logical approach to neurotoxicologic assessment of chemical substances will build on and extend currently available test systems. It will have a tiered structure. The first or screening tier will consist of tests to measure obvious structural and functional changes, often a functional observational battery. Subsequent levels of testing will be guided by the results of initial screening. Toxicologic testing must be supplemented by epidemiologic surveillance of populations exposed to known and suspect neurotoxicants. Screening programs in these populations designed to detect excessive absorption of a neurotoxic agent or subclinical neurological dysfunction can be useful in identifying affected individuals before severe disability occurs.
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PMID:Strategies for the prevention of environmental neurotoxic illness. 847 70

Many neurological disorders have been described in combination with sensorineural hearing loss and pigmentary retinopathy. We present the clinicopathological case of such a combination, associated with premature atherosclerosis of large cerebral arteries. In the literature dealing with the combination of deafness and pigmentary retinopathy, none of the many described syndromes was associated with premature atherosclerosis. The mitochondrial myopathy, encephalopathy, lactic acidosis, early atherosclerosis and stroke-like episodes (MELAS) syndrome can include deafness and blindness. In this syndrome small cerebral arteries are affected. In our case we did not find electron microscopic evidence of mitochondrial myopathy. Also the syndrome with encephalopathy, deafness, blindness and ataxia in young women is attributed to microangiopathy with small brain infarcts and retinal infarcts. In contrast, in our case, large cerebral arteries are affected. In the reverse order, none of the conditions with early atherosclerosis has been reported in combination with sensorineural deafness and pigmentary retinopathy. There is some similarity of our case to cases of Usher syndrome, type II. In the Usher syndrome, plasma lipid disturbances have been described and neuroradiological evidence of decreased circulation in the posterior cerebral circulation has been published. We suggest that in cases of congenital or acquired oto-ophthalmo-neurological disease the cerebral circulation and the lipid metabolism should be analyzed.
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PMID:A congenital syndrome of mental deficiency, gait disturbance, sensorineural deafness and pigmentary retinopathy associated with premature atherosclerosis. 852 24

We reviewed the records and radiologic studies of eight patients who developed new focal neurologic abnormalities while receiving interleukin-2 (IL2)-based immunotherapy for malignancy or HIV infection. Initial confusion and delirium in the patients evolved into coma, ataxia, hemiparesis, seizures, and cortical syndromes including aphasia, apraxia, and cortical blindness. Imaging studies showed multiple white and gray matter lesions with a predilection for the occipital poles, centrum semiovale, and cerebellum. After cessation of IL2 treatment, seven patients improved to normal or near-normal neurologic function paralleled by resolution of the lesions on scans. One patient improved only minimally. Possible etiologies for the lesions include an IL2-induced cerebral vasculopathy, a direct toxic effect of IL2, or immunologically mediated damage.
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PMID:Multiple cerebral lesions complicating therapy with interleukin-2. 875 14

An 11-month-old female Vietnamese pot-bellied pig was examined for severe dehydration and neurologic signs including disorientation, ataxia, blindness, and involuntary twitching of the muscles of the neck and head. Biochemical analyses of serum revealed hypernatremia, hyperchloremia, hyperkalemia, azotemia, hyperphosphatemia, hyperalbuminemia, and high activities of aspartate transaminase and creatine kinase. A diagnosis of salt toxicosis/water deprivation was made. Medical management consisted of intravenous administration of a high-sodium crystalloid solution, anti-inflammatory drugs, and other supportive care. Sodium concentration of fluids administered intravenously was adjusted to be slightly less than the pig's serum sodium concentration so that the serum sodium concentration was reduced gradually over 48 hours. Resolution of clinical signs was rapid and the pig was discharged after 8 days of hospitalization. Fourteen days after the initial examination, the pig appeared healthy except for visual deficits. Historically, prognosis with conventional treatment of salt toxicosis/water deprivation is poor; however, this alternative approach to treating this condition appears promising.
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PMID:High-sodium crystalloid solution for treatment of hypernatremia in a Vietnamese pot-bellied pig. 883 48

Vitamin E is one of the most important lipid-soluble antioxidant nutrients. Severe vitamin E deficiency can have a profound effect on the central nervous system. Cystic fibrosis, chronic cholestatic liver disease, abetalipoproteinemia, short bowel syndrome, isolated vitamin E deficiency syndrome and other malabsorption syndromes all may cause varying degrees of neurologic deficits due to related vitamin deficiencies. The classic abnormalities in vitamin E deficiency progress from hyporeflexia, ataxia, limitations in upward gaze and strabismus to long-tract defects, profound muscle weakness and visual field constriction. Patients with severe, prolonged deficiency may develop complete blindness, dementia and cardiac arrhythmias. Treatment must be tailored to the underlying cause of vitamin E deficiency and may include oral or parenteral vitamin supplementation. The more advanced the deficits, the more limited the response to therapy. Therefore, a good neurologic examination and periodic serum vitamin E levels are essential in patients at risk of vitamin E deficiency.
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PMID:Neurologic findings in vitamin E deficiency. 901 78

Transcranial Doppler, electroencephalography, and single photon emission computed tomography were performed in a case of basilar migraine during the different phases of the attack. In the aura phase, the patient had bilateral blindness and ataxia. Doppler ultrasound studies showed a reduction in the mean flow velocity of the posterior cerebral arteries, electroencephalography showed slow activity confined to the posterior regions, and single photon emission computed tomography, an area of hypoperfusion in the right parietal and occipital regions. During the headache phase, when the neurological examination was normal, transcranial Doppler showed an increase in the mean flow velocity of both posterior cerebral arteries and the electroencephalogram revealed an increase in the slow activity over the occipital regions. When the pain subsided, the electroencephalogram showed a progressive reduction of the slow abnormalities and transcranial Doppler was reported as normal. After a week, single photon emission computed tomography and cranial magnetic resonance imaging were normal. After a month, a follow-up electroencephalogram was also normal. All these findings indicated a transient focal reduction of cerebral blood flow during the aura phase.
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PMID:Basilar artery migraine: transcranial Doppler EEG and SPECT from the aura phase to the end. 904 23


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