Peripheral Neuropathy in Leigh's Encephalomyelopathy

Published on Nov 12, 2008in Developmental Medicine & Child Neurology3.532
路 DOI :10.1111/j.1469-8749.1975.tb03529.x
A. Moosa3
Estimated H-index: 3
(Hammersmith Hospital)
SUMMARY An infant with subacute necrotising encephalopathy is described, in whom slow motor-nerve conduction velocities suggested the presence of peripheral neuropathy. Peripheral nerves are not invariably involved in patients with this disease, but as they are involved in thiamine-deficiency states it is conjectured that the presence or absence of peripheral neuropathy in patients with subacute necrotising encephalopathy may distinguish those with thiamine triphosphate deficiency from those in whom the disease is due to other causes. RESUME Neuropathie peripherique dans l'encephalomyelopathie de Leigh L'auteur rapporte le cas d'un nourrisson presentant une encephalopathie necrosante subaigue chez qui des vitesses de conduction lentes dans les nerfs moteurs suggeraient l'existence d'une neuropathie peripherique. Les nerfs peripheriques ne sont pas obligatoirement atteints dans l'affection envisagee, mais comme ils le sont dans les etats de deficience en thiamine, il est suppose que la presence ou l'absence de neuropathie peripherique chez les malades atteints d'encephalopathie necrosante subaigue peut differencier les cas lies a une deficience en thiamine triphosphate de ceux dus a une autre cause. ZUSAMMENFASSUNG Periphere Neuropathie bei der Leigh'schen Enzephalopathie Es wird uber einen Saugling mit subakuter nekrotisierender Enzephalopathie berichtet, bei dem eine verlangsamte Nervenleitgeschwindigkeit auf eine periphere Neuropathie hinwies. Die peripheren Nerven sind bei Patienten mit dieser Erkrankung nicht immer betroffen, aber da sie betroffen sind bei Fallen mit Thiamin Mangel wird vermutet, das das Vorliegen bzw. nicht Vorliegen einer peripheren Neuropathie bei Patienten mit subakuter nekrotisierender Enzephalopathie die Falle mit einem Thiamintriphosphat Mangel gegen solche abgrenzt, bei denen die Erkrankung andere Ursachen hat. RESUMEN Neuropatia periferica en la encefalomielopatia de Leigh Se describe un nino con una encefalopatia necrotizante subaguda, en que la reduccion en la velocidad de conduction sugirio la presencia de una neuropatia periferica. Los nervios perifericos no estan invariablemente afectados en pacientes con esta enfermedad, pero como lo estan en estados de deficiencia de tiamina, se piensa que la presencia o ausencia de neuropatia periferica en pacientes con encefalopatia necrotizante subaguda puede distinguir los casos con deficiencia de trifosfato de tiamina de aquellos debidos a otras causas.
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