Absence of clinical or physiological changes during short-term cerebellar stimulation in a patient with Lafora body disease.

Wright, G D; McLellan, D L

doi:10.1136/jnnp.44.4.364

Journal of Neurology, Neurosurgery & Psychiatry

1981

DOI: 10.1136/jnnp.44.4.364

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Absence of clinical or physiological changes during short-term cerebellar stimulation in a patient with Lafora body disease.

G D Wright¹,

D L McLellan²

Abstract: A 17-year-old girl with Lafora body disease received short-term cerebellar stimulation but her epilepsy failed to improve and no changes were detected in her EEG. Neither visual nor somatosensory evoked potentials were changed by short bursts of cerebellar stimulation.

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2010

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Cited by 2 publications

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A double-blind trial of chronic cerebellar stimulation in twelve patients with severe epilepsy.

Wright¹,

McLellan²,

Brice³

1984

Journal of Neurology, Neurosurgery & Psychiatry

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187

110

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SUMMARY Twelve patients with severe intractable epilepsy were treated by chronic cerebellar stimulation under double-blind conditions for six months. No reduction in seizure frequency occurred that could be attributed to stimulation, though eleven of the patients considered that the trial had helped them. One patient experienced fewer episodes of incontinence during stimulation. Cerebellar stimulation in its present form cannot be recommended for the treatment of severe intractable epilepsy.Therapeutic effects of cerebellar stimulation in severe epilepsy were first reported by Cooper.' A later report summarised the outcome in a total of 32 patients.2 Seizure frequency fell to half or less of the pre-operative rate in 18 patients and nine were regarded as therapeutic failures. One died at operation and four others died subsequently in their sleep. Two of these had not responded to stimulation, one had been free of grand mal and petit mal attacks for the six week period between implantation and death and the response of the fourth was not described. Treatment was uncontrolled and assessment open.Several other reports3-6 have since appeared which have supported the concept that cerebellar stimulation improves epilepsy, although the effect was less pronounced than in Cooper's series. Only one double-blind study7 has been performed. This involved five patients, of whom three, on the criteria recommended by Cooper et al,8 might not have been expected to respond favourably.Severe epilepsy has an appreciable morbidity. Mortality is also increased, especially in young adult males. Lennox9 quotes an overall death rate of 11-4 in the age group 25-45 compared with a rate of 3-1 in the general population. Therefore it is extremely important to establish whether or not this treatment should be recommended for occasional cases of severe intractable epilepsy. The present uncertainty

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A double-blind trial of chronic cerebellar stimulation in twelve patients with severe epilepsy.

Wright¹,

McLellan²,

Brice³

1984

Journal of Neurology, Neurosurgery & Psychiatry

Self Cite

187

110

View full text Add to dashboard Cite

show abstract

Lafora Disease

Monaghan

Delanty

2010

CNS Drugs

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Lafora disease is a rare, fatal, autosomal recessive, progressive myoclonic epilepsy. It may also be considered as a disorder of carbohydrate metabolism because of the formation of polyglucosan inclusion bodies in neural and other tissues due to abnormalities of the proteins laforin or malin. The condition is characterized by epilepsy, myoclonus and dementia. Diagnostic findings on MRI and neurophysiological testing are not definitive and biopsy or genetic studies may be required. Therapy in Lafora disease is currently limited to symptomatic management of the epilepsy, myoclonus and intercurrent complications. With a greater understanding of the pathophysiological processes involved, there is justified hope for future therapies.

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Absence of clinical or physiological changes during short-term cerebellar stimulation in a patient with Lafora body disease.

Abstract: A 17-year-old girl with Lafora body disease received short-term cerebellar stimulation but her epilepsy failed to improve and no changes were detected in her EEG. Neither visual nor somatosensory evoked potentials were changed by short bursts of cerebellar stimulation.

Cited by 2 publications

References 2 publications

A double-blind trial of chronic cerebellar stimulation in twelve patients with severe epilepsy.

A double-blind trial of chronic cerebellar stimulation in twelve patients with severe epilepsy.

Lafora Disease

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