Treatment of early onset Parkinson's disease with ropinirole.
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Humans↗
Biomedical subjects
J R Ponsford
Publications and source records attributed to J R Ponsford.
Tactile spatial resolution in blind braille readers.
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Blindness↗
Brown-Vialetto-Van Laere syndrome.
The clinical and pathological findings of a male with the Brown-Vialetto-Van Laere syndrome are described. This rare and fatal affection of the nervous system involves mainly the brain stem with the prominent and early manifestation of sensorineural deafness. Increased awareness and documentation of this disorder has added information on the mode of inheritance.
Adolescent↗
Chiari malformation in adults: relation of morphological aspects to clinical features and operative outcome.
To determine whether clinical features attributed to cerebellar ectopia could be related to the severity of the malformation, and if morphological features could be related to operative outcome, a retrospective study of 141 patients with the adult Chiari malformation was carried out, 81 receiving operative treatment. Morphological parameters derived from preoperative clinical imaging were compared with presenting clinical features and postoperative outcomes. Patients with the most severe cerebellar malformation, defined as descent of the cerebellar tonsils to or below the axis, had disabling ataxia and nystagmus more frequently. Those with brainstem compression had limb weakness and muscle wasting more frequently. Operative outcome was significantly less favourable in patients with severe cerebellar ectopia (12% improved, 69% deteriorated) than in those with minor ectopia (50% improved, 17% deteriorated). Patients with a distended cervical syrinx had a more favourable outcome than those without. Morphological features help predict operative risk.
Adolescent↗
Methacholine pupillary responses in third nerve palsy and Adie's syndrome.
In order to examine the clinical usefulness of methacholine in assessing the site of ocular parasympathetic lesions, pupillary responses in man were measured in postganglionic (Adie's syndrome) and preganglionic third nerve lesions involving the pupil and in controls. From previous work with methacholine it might have been expected that greater constriction would occur in the postganglionic lesions but similar responses were found in both. Corneal hypoxia due to ptosis appeared unlikely to affect corneal permeability significantly and it is probable that these results reflect an increase responsiveness of the iris at, or distal to, the site of muscarinic acetylcholine receptors. Pupils contralateral to third nerve palsy, when tested on a separate occasion, also constricted by an amount approximately proportional to that of the clinically abnormal pupil. The possibilities that this may result in some way from reduction in total retinal illumination, or from retrograde changes in preganglionic pupilloconstrictor neurons affecting contralateral pupilloconstrictor neurons via central pathways, are discussed. It is concluded that supersensitivity to methacholine, tested carefully in the manner described, is a useful guide to the presence of parasympathetic denervation or decentralization, but that it is not reliable in distinguishing between the two sites.
Adie Syndrome↗
X-linked recessive bulbospinal neuronopathy: a report of ten cases.
A form of adult onset 'bulbospinal muscular atrophy' of X-linked recessive inheritance is described in 10 patients from eight families. Muscle weakness in the limbs was mainly proximal and developed in the third to fifth decades of life, often preceded by muscle cramps on exertion and tremor of the hands. Weakness and fasciculation of the facial muscles and tongue were prominent. All the patients had gynaecomastia and some were infertile. Two had diabetes mellitus. Motor nerve conduction studies were normal but most patients had small or unrecordable sensory action potentials in the absence of clinical sensory loss. Plasma creatine kinase levels were considerably elevated and muscle biopsies showed neurogenic atrophy together with secondary myopathic changes. The importance of recognising this distinctive disorder in single cases (six of the present series) is emphasised.
Biopsy↗
Transient visual obscurations, without papilloedema.
Two cases of frontal, space occupying tumour without papilloedema are reported. Both presented with frequent, stereotyped attacks of visual disturbance with orbital headache, neck pain and unsteadiness of gait. Intermittent occipital lobe ischaemia, related to compression of the posterior cerebral artery against the tentorium by distorted, herniating brain, seems a probable explanation.
Adult↗
Defective PGE reactivity in leucocytes of multiple sclerosis patients.
Leucocyte migration inhibition in vitro, in response to antigen or mitogen, is suppressed by PGE2 (0.01-1.0 mug/ml). The susceptibility of leucocytes to such inhibition by PGE2 has been compared using cell preparations obtained from normal individuals, multiple sclerosis patients and from patients with other neurological diseases. The results indicate defective reactivity of leucocytes from multiple sclerosis patients.
Adult↗
Mental change as an early feature of multiple sclerosis.
Five patients with mental change as a prominent and early feature of an illness which appeared to be multiple sclerosis are reported. All the patients had in addition clinical signs of predominant brain stem involvement and the cerebrospinal fluid findings were similar. It is emphasised that mental change may be an early feature of multiple sclerosis even in those patients in whom the onset of the disease is insidious.
Adult↗