Peripheral nerve involvement in Batten-Spielmeyer-Vogt's disease

Abstract

Electromyography and sensory and motor nerve conduction were studied in 23 patients with Batten-Spielmeyer-Vogt's disease ("juvenile amaurotic idiocy", cerebral ceroidlipofuscinosis). A slight to moderate slowing of the sensory conduction velocity was found in the median as well as in the sural nerve, more pronounced in the distal than in the proximal segments. The findings are interpreted as evidence of impaired transmission of the peripheral nerves in Batten-Spielmeyer-Vogt's disease.