Posstreptococcal neuropathy.
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Biomedical subjects
Publications and source records attributed to R B Godwin-Austen.
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We describe a three generation family with Parkinson's disease showing autosomal dominant inheritance with extreme anticipation. Familial Parkinson's disease in three living generations is extremely rare, and anticipation is an unusual and interesting feature. Anticipation was shown in all generations and may have involved previous generations. Some cases of familial Parkinson's disease may therefore involve a trinucleotide repeat gene as part of the causal mechanism.
We report the effects of intravenous gamma-globulin (IVGG) in severe Guillain-Barré syndrome (GBS) in a small open study. Seven patients were given IVGG (0.4 g/kg/day) for 5 consecutive days. At the start of treatment all had progressing limb weakness and none could stand unaided. Within 24 h all but one patient started to improve, without the expected plateau phase, and progress was maintained with a mean time to independent walking of 14 days. One patient started treatment 9 h prior to ventilation for 15 days but was walking independently 31 days after admission. This study provides further evidence that IVGG is a promising therapeutic alternative to plasmapheresis in GBS. The rapidity of the response to IVGG raises the possibility that a single dose regimen might be equally or more effective.
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Diffuse Lewy body disease is an important pathological substrate of the common syndrome of parkinsonian dementia. The new technique of anti-ubiquitin immunocytochemistry has been used in a correlative quantitative neuropathological study of fifteen cases of diffuse Lewy body disease, showing that the severity of dementia is related to cortical Lewy body density, whilst subcortical abnormalities make a much less significant contribution. Cortical senile plaques also appear to be part of the pathology of diffuse Lewy body disease and should not therefore be used as an isolated diagnostic criterion for Alzheimer's disease. Diagnostic criteria for diffuse Lewy body disease are discussed.
Fifteen cases of diffuse Lewy body disease were diagnosed on pathological grounds during a single year in one health district. The range and frequency of clinical features contrast strikingly with previous reports. The majority of cases presented with classical levodopa-responsive Parkinson's disease either alone (6 cases) or with mild cognitive impairment (3 cases); the remaining 6 cases presented with cognitive impairment alone. In time almost all patients developed both dementia and Parkinsonism. The dementia was cortical in type, but unusual in that most (12 cases) showed day-to-day fluctuation in severity at some point in their illness. These findings suggest that diffuse Lewy body disease is not rare, and that it presents in a range of ways from dementia with subsequent Parkinsonism to Parkinson's disease with subsequent dementia. The latter mode of presentation suggests that it should be considered as a significant pathological substrate of dementia in Parkinson's disease.
Fifteen cases of diffuse Lewy body disease were identified in a systematic survey of all 216 brains referred to this hospital from a single health district in a single year. These cases presented with Parkinson's disease (40%), cognitive impairment (40%) and both (20%). Quantitative neuropathological studies using anti-ubiquitin immunocytochemistry revealed that dementia severity was related to cortical Lewy body density. The prevalence of diffuse Lewy body disease may have been underestimated in the past because of the neuropathological difficulties in making the diagnosis. Firstly, cortical Lewy bodies have a subtle appearance and are easy to overlook. Secondly, senile plaques are a common feature of diffuse Lewy body disease and may lead the unwary to make an erroneous diagnosis of Alzheimer's disease or "plaque-only Alzheimer's disease'. Diffuse Lewy body disease is a common and important cause of parkinsonian dementia, including the dementia of Parkinson's disease itself.
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The case histories are described of four brothers who suffered attacks of transient global amnesia. The coincidence of this disorder in four siblings suggests that the causative factors in transient global amnesia may have a familial basis and that its incidence is more common than generally realised.
In a case control study of the relationship between smoking habits and Parkinson's disease a negative association was demonstrated with a relative risk of 0 x 52. A history of smoking up to 20 years earlier was associated with a risk of developing Parkinson's disease equal to about half that in non-smokers. The type of disease, age of onset and rate of progression were associated with a similar reduction in risk implying that in respect of smoking history the disease is homogeneous. The positive correlation of degenerative vascular disease with smoking is further evidence that arteriosclerosis is not involved in the causation of Parkinson's disease. The negative association between Parkinson's disease and smoking is confirmed and is independent of other associated factors. The known and serious risks of smoking far outweigh the possible benefit of lowered risk of Parkinson's disease.
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The technique of polarised light goniometry was used to quantify objectively parameters of the spastic gait during a double-blind cross-over trial comparing the spasmolytic effects of DS103-282, baclofen and placebo. Only minimal objective and subjective changes in gait were found when the results of treatment with DS103-282 or baclofen were compared with those of treatment with placebo.
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The effects of oxpentifylline were assessed in a double-blind trial in 11 patients with Parkinson's disease already under treatment. No significant improvement was noted. Eight patients developed involuntary movements or a worsening of movements if already present. The significance of this unexpected finding is discussed.