Treatment of Progressive Myoclonic Epilepsy with Mephenesin

Kelly, R.E.; Laurence, D. R.

doi:10.1136/bmj.1.4911.456

Cited by 7 publications

(1 citation statement)

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“…Kelly & Laurence (1955) reported disappearance of spike activity from the EEG and simultaneous cessation of myoclonus after intravenous infusion of mephenesin. Kelly & Laurence (1955) reported disappearance of spike activity from the EEG and simultaneous cessation of myoclonus after intravenous infusion of mephenesin.…”

Section: Personal Investigationsmentioning

confidence: 98%

Clinical and Electroencephalographic Studies of Responses to Lidocaine and Chlormethiazole in Progressive Myoclonus Epilepsy

Petersén

1966

Acta Psychiatr Scand

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Around the turn of this century descriptions were given-in the works of Unuerricht (1891) and, notably, Lundborg (1903)--of a progressive, often familial, clinical syndrome in which the triad of epilepsy, disabling myoclonia, and dementia were the cardinal symptoms. The onset occurred during adolescence, and death from marasmus generally followed after several years, Since those first reports, symptomatologies which include the above-mentioned triad have been the subject of extensive research and considerable attention in the 1iterature.A great diversity of lesions have been demonstrated histopathologically. A substantial number of cases have exhibited Lafora bodies: intra-and extracellular deposits whose chemical definition remains in some measure debatable but which are stated to include mucopolysaccharides, glucoproteins and mucoproteins. In other instances there have been "lipoidotic" cellular abnormalities; and in yet another group non-specific degenerative and atrophic changes. These lesions have been found often widely scattered within the CNS, with particularly severe affection of the dentate nucleus, the olivary bodies, substantia nigra, thalamus and cortex. In cases with Lafora bodies the specific lesions have even been found in spinal nerves, myocardium, liver, and striated muscle (vide e. g. Harriman & M i l l a r 1955, Schwarz & ranoff 1965).From the diverse histopathological findings it seems clear that in the sPdrome of progressive myoclonus epilepsy a uniform pathogenesis or clinical symptomatology cannot be expected. Indeed, there are clinical differences even between the cases described by Unverricht and those of Lundborg. Later Case presentationsCase J. E. E.Male, 27, with no known consanguinuity of ancestors. Father had suspected epilepsy (? psychomotor absences), Patient began to have srand ma1 seizures at age 16 and myoclonic spasms at age 18. The myoclonus, by virtue of its intensity and extension, soon became so disabling that patient was obliged to abandon his schooling and, at age 20, to seek disability pension. Six years after onset, mental symptoms attributable to cerebral lesions necessitated observation for a time in a psychiatric ward. Pneumoencephalographic findings at this point were normal. Ophthalmological examination

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Section: Personal Investigationsmentioning

confidence: 98%