Peripheral ataxia is reported in a juvenile case of Alpers-Huttenlocher disease (AHD). Neurophysiological and neuropathological investigations revealed a central-peripheral axonopathy, affecting the deep sensation carried by the peripheral nerve fibres and the posterior tracts of the cord, due to neuronal loss of the sensory ganglia. Involvement of the sensory pathways is regarded as a major feature of juvenile AHD.

Central-peripheral sensory axonopathy in a juvenile case of Alpers-Huttenlocher disease

SIMONATI, Alessandro;TOMELLERI, Giuliano;TONIN, PAOLA;RIZZUTO, Nicolo'
2003

Abstract

Peripheral ataxia is reported in a juvenile case of Alpers-Huttenlocher disease (AHD). Neurophysiological and neuropathological investigations revealed a central-peripheral axonopathy, affecting the deep sensation carried by the peripheral nerve fibres and the posterior tracts of the cord, due to neuronal loss of the sensory ganglia. Involvement of the sensory pathways is regarded as a major feature of juvenile AHD.
Alpers' Disease; Peripheral Ataxia; Gangliopathy; Neuropathology; Mitochondrial Diseases
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11562/305323
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