Biomedical subjects
Publications and source records attributed to R Sandyk.
A 47-year-old woman presented with paralysis of upward gaze associated with Addison's pernicious anaemia. Administration of cyanocobalamin resulted in significant improvement of eye movements and haematological parameters. Isolated paralysis of upward gaze may be a feature of vitamin B12 deficiency.
We report the occurrence of a partially reversible Parkinson-like extrapyramidal syndrome in a woman chronically exposed to vapours from an industrial solvent mixture based on trichlorotrifluoroethane and nitromethane. The fluoroalkane group of compounds has not been previously reported to produce such effects in man, but mixtures containing nitromethane may not be innocent. Under certain industrial conditions the conversion of nitromethane to methylisocyanide may result in toxicity particularly in the basal ganglia and cerebellum following chronic low level exposure.
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In a patient with idiopathic blepharospasm treatment with the gamma-aminobutyric acid (GABA)-mimetic combination of baclofen and sodium valproate resulted in complete and sustained remission of symptoms and signs. It is suggested that blepharospasm results from a relative deficiency of GABA-ergic neurons and dopamine predominance in the striatum, and that treatment with the GABA-mimetic agents (baclofen and sodium valproate) represents a more physiological means of reducing dopaminergic predominance in the striatum. This report, together with previous reports, suggests that the combination of these two agents should be tried in the initial management of this syndrome.
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Toxic irreversible encephalopathic syndromes developed in 2 patients treated with lithium carbonate and haloperidol. Symptoms consisted of lethargy, fever, tremulousness, confusion, and extrapyramidal and cerebellar dysfunction, accompanied by leucocytosis and elevated serum enzyme, blood urea nitrogen, creatinine and fasting blood glucose levels. One patient suffered widespread irreversible brain damage; the other was left with persistent dyskinesias. Although causal factors have not been identified, this report and others in the literature suggest that diffuse irreversible encephalopathy may occasionally develop in individuals with abnormal brain sensitivity to the lithium carbonate/haloperidol combination. Evidence for this is based on the fact that in our patients and others mentioned in the literature the dosage and blood levels of lithium were not high.
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In a patient suffering from essential tremor, treatment with aminophylline resulted in complete and sustained remission of symptoms. This observation supports a central origin of essential tremor and suggests that brain nucleotides (cyclic adenosine monophosphate, cyclic guanosine monophosphate) may be important in the pathogenesis of this condition.
A 52-year-old Black woman on phenytoin therapy for post-traumatic epilepsy developed transient hemiparesis contralateral to the injury. The episode appeared to have been precipitated by toxicity due to ingestion of a large amount of phenytoin. A possible mechanism for focal neurological deficit in brain-damaged patients on phenytoin therapy is discussed.
Progressive multifocal leuco-encephalopathy (PML) is a demyelinating viral infection of the central nervous system, usually fatal and characterized by widespread lesions mainly in the cerebral hemisphere. PML is a disease of adult life, usually occurring in a setting of chronic neoplastic disease and less often non-neoplastic granulomatous disease. The disorder usually evolves rapidly. Of all laboratory investigations electro-encephalography is the most useful for indicating the severity of pathological involvement. PML is believed to result from infection with papovaviruses, an SV40-like agent and a JC virus. Both viruses are oncogenic in hamsters. There is no effective therapy.
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Bickerstaff's brainstem encephalitis is a condition in which the brainstem is affected out of proportion to the rest of the brain. Despite severe involvement of brainstem structures, complete recovery over a period ranging from weeks to months is the rule. We describe a case suggestive of this condition which occurred in Johannesburg and in which the progression of neurological signs and symptoms culminated in complete recovery. To our knowledge there has been no previous report of such a case in South Africa.
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A 52-year-old Black man with therapy-resistant parkinsonism, gait apraxia and dementia but no endocrine abnormality was found to have extensive intracerebral calcifications which included the basal ganglia. Although most patients with radiological evidence of calcifications in the basal ganglia remain asymptomatic, a small group may present with an extrapyramidal syndrome that is usually progressive, resistant to therapy, and not associated with an endocrine disorder. Plain skull radiography and computed tomography should therefore be performed in patients presenting with a parkinsonian syndrome unresponsive to therapy.
Unilateral hemichorea-hemiballismus developed suddenly in a 60-year-old man. Computed tomography revealed a small area of low density in the region of the left anterior limb of the internal capsule and the head of the caudate nucleus which was consistent with a lacunar infarction. Haloperidol gradually abolished the symptoms within 7 days of their onset. No further choreoballistic movements were experienced. This report reveals that ballistic movements may occasionally be caused by lesions lying some distance from the subthalamic nucleus of Luys.