Biomedical subjects
Publications and source records attributed to W R Gibb.
A case of probable neuroleptic malignant syndrome (NMS) complicating L-dopa withdrawal in striatonigral degeneration is described. This case suggests that the hyperthermia of NMS is due to peripheral heat production associated with severe muscular rigidity secondary to withdrawal of striatal dopamine drive, rather than being centrally mediated by the hypothalamus, which is not involved in striatonigral degeneration.
We compared 46 patients having onset of Parkinson's disease before age 45 years with 52 having onset after age 70. Young-onset cases more often presented with muscular stiffness (43%) and old-onset with difficulty walking (33%). One-third of young-onset cases had off-period dystonia, mostly affecting the legs, but no dystonia was recorded in old-onset cases. Presentation with rest tremor occurred in 41% of young-onset and 63% of old-onset. There were no differences in the number of affected relatives, endocrine disease, personality characteristics, dementia, or dyskinesia. A pathological study of 12 young-onset and 22 old-onset cases showed 24% greater nigral cell loss in the young, but no differences in the basic Lewy body pathology. Median disease duration in young cases was 5 years longer in the clinical study and 12 years longer in the pathological study. These studies show that the Parkinson's disease process is similar in young- and old-onset cases.
In 12 cases of post-encephalitic parkinsonian syndrome (PEPS) starting at a mean age of 25.1 years, the mean disease duration of 35.8 years was twice as long as in 12 young patients with Lewy body-Parkinson's disease (LB-PD), whose disease started at a mean age of 39.9 years (mean duration 16.5 years). In PEPS the rate of progression of disease was extremely slow. The groups were of a similar age at death, but the post-encephalitic cases had 70% fewer cells in the substantia nigra than in idiopathic Parkinson's disease (PD) and 92% fewer than in controls. Nigral cell destruction was not found in PEPS, but active cell breakdown and abundant extraneuronal melanin were present in PD. A second group of 12 LB-PD cases with mean onset at 58.9 years survived for a mean of 10.1 years, but the number of remaining substantia nigra cells was the same as in the young onset cases of PD. This clinical and pathological evidence does not support the notion that progression of disease in PD can be attributed to the superadded effects of normal ageing on top of a primary acute noxious insult sustained in earlier life.
Four patients with severe dementia and a parkinsonian syndrome are described. Dysphasia, dyscalculia, dyspraxia, visual and verbal memory disturbance and psychosis, usually of depressive type, occurred early in the course of the illness. Pathologically they were characterized by the presence of numerous Lewy bodies throughout both the cerebral cortex and brainstem. Three cases also had severe neurofibrillary tangle change or senile plaques in the neocortex, compatible with Alzheimer's disease, but the cortical tangle distribution did not always match that of the Lewy bodies. This disorder may form part of the spectrum of pathology in Parkinson's disease, where it may be one possible cause of dementia.
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The administration of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) to marmosets resulted in parkinsonism and degeneration of the substantia nigra pars compacta. Examination of the hypothalamus showed neuronophagic-like rosettes of glial cells and diffuse increase in glial numbers in areas of the dopaminergic A11-A14 groups. These findings suggest that MPTP may be toxic to dopamine neurons of the hypothalamus as well as to the substantia nigra.
The subjective and motor phenomena of neuroleptic-induced akathisia were studied in two different populations of psychiatric patients. Thirty nine (41%) of 95 patients attending community psychiatric centres and psychiatric day hospitals experienced a compulsion to move about, and 52 (55%) complained of restlessness of the body. Of 842 psychiatric in-patients 159 found to have marked hyperkinesis were divided into three groups; group 1 with motor restlessness, and a subjective desire to move about or marching on the spot (27 patients), group 2 with choreo-athetotic movements and motor restlessness (79 patients) and an indeterminate group 3 (53), bearing more similarities to group 1 than group 2. Motor disturbances associated with akathisia were repeated leg crossing, swinging of one leg, lateral knee movements, sliding of the feet and rapid walking.
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A 60 year old woman with idiopathic Parkinson's disease had been prescribed thioridazine for schizophrenia. Five months after this was stopped, Sinemet also considered of dubious therapeutic value, was withdrawn. One week later she developed features of the neuroleptic malignant syndrome (NMS) accompanied by myoglobinuric renal failure. Post-mortem examination confirmed Lewy body degeneration in the substantia nigra. It is proposed that NMS may be caused by levodopa withdrawal in Parkinson's disease, and that it is withdrawal of dopaminergic drive that causes the syndrome.
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Four patients presented with the nephrotic syndrome. The histological appearances on renal biopsy were in three characteristic and in one suggestive of lupus nephritis. These patients did not initially have other clinical features of SLE, but three had a positive ANA and one a raised DNA titre. Remission occurred in two patients, in one spontaneously and in another following corticosteroid therapy, but two developed renal failure. During follow-up all developed elevated DNA binding levels and arthralgia or lymphopenia. The ARA classification criteria for lupus were only fulfilled at this late stage.
A patient with active giant cell arteritis developed paraparesis and dissociated sensory loss due to infarction in the anterior spinal artery territory at the level of T12. Three days later fatal basilar artery thrombosis occurred. No occlusive lesion was found to explain the anterior spinal artery syndrome but this was associated with active arteritis. Alternative possibilities are that thrombus was present in involved cervical feeding vessels, or that emboli arose from intimal involvement in larger vessels, or that the event was related to thrombocytosis.
A case of coma due to Wernicke's encephalopathy complicated by respiratory failure is described. Ventilation and thiamine administration lead to recovery, although Korsakoff's psychosis and ataxia persisted. A review of similar cases of coma emphasizes the absence of diagnostic features, but that if structural disease is excluded the presence of pupillary and ocular signs may support a diagnosis of Wernicke's encephalopathy. The response to thiamine may be diagnostic as in 8 of these patients who received it, but the long-term morbidity remains high.