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

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. Received: 28 October 2002, Received in revised form: 2 January 2003, Accepted: 15 January 2003 Present address: A. Polo, MD, Neurology Unit, Hospital of Piove di Sacco (PD), Italy Correspondence to: A. Simonati, MD