A study of posterior column function in familial spastic paraplegia.

A family is described in which affected members have clinical features consistent with the late onset form of Strümpell's Familial Spastic Paraplegia which is of dominant inheritance. Abnormalities in cortical somatosensory to peroneal nerve stimulation were found in all affected members of the family and in several who were clinically unaffected. In some cases responses were better defined at slow rates of stimulation. Peripheral nerve conduction velocity was normal. These changes are consistent with previous findings of degeneration in the posterior columns at necroscopy and with a dying back process in the first sensory neuron. Clinically unaffected members of the family with abnormalities in the somatosensory response may represent asymptomatic heterozygotes.

Peripheral nerve function in patients with bronchial carcinoma. Comparison with matched controls and effects of treatment.

Clinical examination of 80 patients with bronchial carcinoma showed minor neurological abnormalities but in only a few cases were these considered to be due to neuromyopathy. Spontaneous activity in the EMG was shown in 35%, consistent with a mild degree of partial denervation; when 50 of the patients were matched with 50 controls the patients showed a small but significant impairment of nerve conduction velocity in comparison with the controls. These findings accord with subclinical neuropathy in a high proportion of patients with bronchial carcinoma consistent with primary axonal change. Thirty patients participated in a prospective study of the effects of treatment. Of these nine were reassessed following surgery or radiotherapy. Although there was a trend towards improvement in sensory conduction there was no consistent change in the electromyographic findings.

Muscle fibre excitability and resting membrane potential in hamster muscular dystrophy.

Measurements of resting potential carried out in vivo in the dystrophic hamster and also measurements of muscle excitability showed no difference between dystrophy and healthy animals.

Habituation of the orbicularis oculi reflex in dementia and dyskinetic states.

The habituation index is a quantitative expression of the ability of the orbicularis oculi (blink reflex) to adapt to a series of electrical stimuli applied to the supraorbital region. This parameter has been studied in a group of normal control subjects, and the results compared with those in cases of idiopathic and drug-induced Parkinsonism, states of dementia, and dyskinesias such as Huntington's chorea and senile chorea. Patients with Huntington's chorea showed a tendency for the reflex to habituate readily in contrast to patients with dementia caused by cortical atrophy and those with Parkinson's disease. Younger patients with Huntington's chorea had indices within the normal range. It seems unlikely that this test will prove of value in the detection of clinically unaffected relatives. Where dementia was associated with a reversible intracranial lesion, the habituation index was studied before and after treatment. Failure of habituation in this condition appears to be due to the release of a primitive protective reflex.

Urinary monoamine metabolite excretion in disorders of movement. Effects of amantadine and levodopa.

We have studied the urinary excretion of 1,4-methylhistamine (1,4-MeHm), 5-hydroxyindole-3-acetic acid (5-HIAA) and homovanillic acid (HVA) in patients with Parkinson's disease, choreiform movements and essential tremor. The effect of amantadine on urinary excretion has been measured in each group of patients as well as the effect of levodopa in patients with Parkinson's disease. In patients with Parkinson's disease, excretion of 1,4-MeHm and HVA was significantly lower than in controls. Patients with choreiform movements had a reduced excretion of HVA but trends toward low levels of 1,4-MeHm and, in patients with Huntington's chorea, elevated excretion of 5-HIAA, were not significant. In patients with essential tremor, urinary excretion of the amine metabolities studied did nof differ significantly from controls. Administration of amantadine to patients with Parkinson's disease was not followed by increased excretion of monoamine metabolites except in those patients who were already receiving anticholinergic drugs. This increase is not significant and there was no effect in other groups of patients. These findings lend no support to the view that amantadine has a general amine-releasing action although there is limited evidence for such an effect in Parkinson's disease. In addition to the expected increase in HVA excretion, administration of levodopa to Parkinsonian patients was followed by significantly reduced excretion of 1,4-MeHm and 5-HIAA. However, if amantadine and levodopa were given together, excretion of 5-HIAA was still reduced, but that of 1,4-MeHm was normal. Levodopa may thus modify the turnover of histamine, which appears to be reduced in Parkinson's disease, and this effect may be modified by amantadine.

Neurological investigation of dementia.

Fifty-two patients thought to be suffering from dementia when first seen as neurological out-patients were fully investigated. Four of them were found to have tumours, whilst in five the dementia was due to a treatable cause. Neither a long history nor an age above sixty-five precludes a dementia being due to a treatable cause.

Cluster headaches associated with vascular malformations.

A vascular malformation was demonstrated in a migrainous female who had developed cluster headaches. The patient responded well to oral dihydroergotamine 1 mg twice daily.

Effect of magnesium on nerve conduction velocity during regular dialysis treatment.

Serial nerve conduction velocities in the peroneal and ulnar nerves have been measured in 10 patients on regular dialysis treatment over a three year period. Each patient alternated between phases on dialysis with magnesium-containing dialysate (1·5-1·7 m-equiv/l.) and phases on `magnesium-free' dialysate (0·2 m-equiv/l.). Plasma magnesium concentrations were high both pre- and post-dialysis during magnesium-containing dialysis, and normal to low on magnesium-free dialysis. All patients had defects in nerve conduction, mainly asymptomatic. Increases in nerve conduction velocity coincided with magnesium-free dialysis, and decreases occurred when the patients reverted to magnesium-containing dialysate. The significance of the correlation by the sign test was P<0·0005. It is concluded that extracellular magnesium levels can influence the rate of nerve conduction in vivo.