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Biomedical subjects

K W Heathfield

Publications and source records attributed to K W Heathfield.

At least 19 recordsLinked to original sources

Dementia in Huntington's disease is associated with neurochemical deficits in the caudate nucleus, not the cerebral cortex.

Choline acetyltransferase (ChAT) and the neurotransmitter amino acids gamma-aminobutyric acid (GABA) and glutamic acid were measured in brain tissue taken post-mortem from control subjects and from patients with Huntington's disease (HD). Two subgroups of HD patients were defined with either severe dementia or no dementia. It was found that ChAT exhibited no greater decrease in cortical tissue from severely demented patients. While there were also no significant deficits associated with dementia in cortical concentrations of the amino acids, a substantial and regionally-specific decrease in both GABA and glutamate was observed in the caudate nucleus of severely demented HD patients.

Adult↗

Pallidal GABA and chorea in Huntington's disease.

Neurochemical correlates of chorea in Huntington's disease were studied using striatal and pallidal tissue taken post mortem from patients with mild and severe chorea. While GABA was decreased in all these areas in Huntington's disease, patients with mild chorea had significantly less GABA in the medial pallidum than did those with severe chorea. There was no relationship between the degree of chorea and concentrations of dopamine or its metabolite. Thus the chorea of Huntington's disease may relate to the balance of residual GABAergic innervation between specific areas of the basal ganglia, consistent with primate models of dyskinesias.

Adult↗

Quadriceps myopathy: a variant of the limb-girdle dystrophy syndrome.

The clinical and pathological features in a patient with quadriceps myopathy are presented. The pattern of progression of the disorder, during a period of 18 years observation, suggests that it represents an unusual and perhaps specific syndrome within the clinical spectrum of the limb-girdle muscular dystophies.

Humans↗

Prognosis of the Ramsay Hunt syndrome.

Thirty-six cases of herpes zoster complicated by facial paralysis (the Ramsay Hunt syndrome) seen over 10 years were reviewed to determine the subjective degree of recovery (ascertained by questionnaire) and residual disability (ascertained by re-examination). Eighteen patients made a full recovery, most within three months; 14 patients were left with only mild residual signs. In only four patients was the final result regarded as unsatisfactory. Outcome was not adversely influenced by age. The facial paralysis of the Ramsay Hunt syndrome thus carries a generally favourable prognosis.

Adult↗

Valproate sodium in Huntington chorea.

An open trial of valproate sodium in 14 patients suffering from Huntington chorea showed that the drug was ineffective in the two treatment schedules employed.

Adolescent↗

An unusual intranuclear structure (? viral nucleocapsid) in the brain in subacute sclerosing panencephalitis.

A brain biopsy was examined from a 7 year old boy with subacute sclerosing panencephalitis. Intranuclear deposits of measles antigens were demonstrated in parenchymal cells by immunofluorescence. Electron microscopy showed viral nucleocapsids in nucleoliform inclusions and unusual rod-like intranuclear structures which resemble structures previously described only in tissue cultures infected with measles virus.

Antigens, Viral↗

Differential diagnosis of transient amnesia.

In a group of 39 consecutive patients attending neurological clinics with transient amnesia patients with transient global amnesia formed the largest group; others suffered from epilepsy, migraine, temporal lobe encephalitis, or psychogenic fugues. In most cases the clinical features suggested the aetiology, but an electroencephalogram recorded as soon after the attack as possible may help in distinguishing amnesia due to temporal lobe epilepsy.

Adolescent↗

The EEG in Huntington's chorea: a clinical and neuropathological study.

The EEGs are reported on a group of 95 patients with Huntington's chorea. Thirty one showed little activity of any kind, and in particular no alpha rhythm above 10 μV in amplitude was seen. Only those records which still met these criteria when re-examined were included in the `low voltage' category. EEGs in this category occurred significantly more frequently in institutionalized patients and in those with a positive family history of Huntington's chorea, dementia, and choreiform movements together. Computer averaged responses to light and sound were found in the three patients examined, though their routine EEGs were low voltage. Neuropathological examination confirmed a clinical diagnosis of Huntington's chorea in 14 patients investigated. There was a statistically significant association between cortical atrophy, including the frontal lobe, and a `low voltage' EEG. It was concluded that the low voltage record, though not specific for Huntington's chorea, was rare in other neurological disorders. The EEG is therefore of value in patients suspected of having Huntington's chorea as well as in various presenile dementias.

Adult↗

Treatment of involuntary movement disorders with tetrabenazine.

Seventeen patients with choreiform, athetoid, or ballistic involuntary movements, or with spasmodic torticollis, were treated with tetrabenazine in doses of 25 to 200 mg daily for periods varying from two weeks to more than six months. Randomized ciné film of the patients' involuntary movements, taken before, during, and after treatment was assessed individually by seven `blind' observers. Eight patients were judged improved; two had Huntington's chorea, two athetosis, two dystonia musculorum deformans, one hemiballismus, and one spasmodic torticollis. Four of the eight improved patients have continued taking the drug for longer than six months. In a second study seven patients with Huntington's chorea were treated for two weeks each with tetrabenazine (50 mg t.d.s.) and with amantadine (100 mg t.d.s.) and the results assessed by the same method. The choreiform movements of six of these patients were strikingly improved with tetrabenazine therapy, but amantadine had no effect. Tetrabenazine is an effective agent for the suppression of choreiform and ballistic involuntary movements. It is only slightly effective in the treatment of athetosis and spasmodic torticollis. Drowsiness, insomnia, and depression were the most conspicuous unwanted effects, and these may limit the clinical usefulness of the drug.

Adolescent↗