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We have seen 32 patients with "apraxia of lid opening" (ALO) in the following clinical settings: as an isolated condition (3 patients), idiopathic blepharospasm (BSP, 20 patients, including 4 familial cases), progressive supranuclear palsy (PSP, 7 patients), and dystonic parkinsonian syndrome (2 patients). Twenty-nine patients treated with botulinum toxin(More)
Eight patients with Friedreich's ataxia showed profound reduction in the density of large myelinated fibers in sural nerve biopsies. The density of small myelinated fibers was normal, but the axonal size and myelin thickness were reduced. Demyelination, presumably secondary to axonal dysfunction, was observed in 3% of the isolated fibers. There was axonal(More)
The rates of spontaneous remission and progression of dystonia to other sites were studied in 72 patients who first presented with adult-onset torticollis, and who were followed up for a mean of 7.7 years. Dystonia had progressed to sites other than the neck (mainly the face and upper limbs) in 23 patients (32%). The latter cases were not differentiated(More)
The occurrence of involuntary movements of stumps following amputation is described in two patients. Although recognised for over 100 years, this phenomenon has received little attention in the modern literature. The condition appears to represent a variant within the spectrum of movement disorders induced by injury to the peripheral nervous system.
Twenty-three children (aged less than 18 years) and 17 adults with severe widespread dystonia were treated with high doses of benzhexol (up to 130 mg daily introduced slowly over many weeks). Children tolerated higher doses (median 30 mg/day) than adults (median 20 mg/day). 52% of the children gained useful benefit, many (43%) without unwanted side effects.(More)
Pharmacokinetic studies in parkinsonian patients and healthy volunteers have shown that Madopar HBS behaves as a slow-release formulation of L-dopa and benserazide. In comparison with standard Madopar the rate of absorption is reduced, providing lower peak concentrations of L-dopa. The drug is released and absorbed over a period of 4-5 h, thus maintaining(More)
The short-term efficacy of continuous intravenous infusions of levodopa (with oral peripheral decarboxylase inhibitor) in abolishing or reducing "on-off" fluctuations in patients with Parkinson's disease is well established. However, there are suggestions that clinical response may be less good with longer infusions, or infusions on consecutive days. We(More)
In a open study in 13 patients with Parkinson's disease with 'on-off' fluctuations, all (n = 3) or part (n = 10) of the usual intake of levodopa (with peripheral decarboxylase inhibitor) was replaced by Madopar HBS for 5-122 days (median 35). Time 'off' increased in 2 of the 3 monotherapy subjects. With combined therapy, time 'on' was increased in 6,(More)