Familial autosomal dominant dopa responsive Parkinson's disease in three living generations showing extreme anticipation and childhood onset.

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.

High-dose intravenous immunoglobulin in the treatment of Guillain-Barré syndrome: a preliminary open study.

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.

Diffuse Lewy body disease: correlative neuropathology using anti-ubiquitin immunocytochemistry.

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.

Diffuse Lewy body disease: clinical features in 15 cases.

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.

Diffuse Lewy body disease: an important differential diagnosis in dementia with extrapyramidal features.

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.

Smoking and Parkinson's disease.

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.

Transient global amnesia in four brothers.

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.

The assessment of drug treatment of spastic gait.

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.

Oxpentifylline in Parkinson's disease.

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.

Hypersecretion of anti-diuretic hormone due to tuberculous meningitis.

Three patients with tuberculous meningitis complicated by hypersecretion of anti-diuretic hormone are described. The recognition of this complication is important in the treatment and prognosis of tuberculous meningitis.

An unusual neurological disorder of copper metabolism clinically resembling Wilson's disease but biochemically a distinct entity.

A patient with progressive neurological disease resembling Wilson's disease but in whom Kayser-Fleischer rings were absent, was given 67Cu and 64Cu, orally and intravenously, to measure the rate of absorption of copper using a convolution integral. The data show an abnormal distribution of body copper resulting in low copper concentrations in plasma, urine and liver but with an accumulation in the lower bowel probably due to a defect in mucosal transport. The importance of differentiating this condition from Wilson's disease is stressed.

The treatment of Parkinson's disease.

The treatment of Parkinson's disease is discussed. Levodopa is the most effective drug in this therapy. The place of other agents is discussed.