Abstract

Hereditary Motor & Sensory Neuropathies (HMSN) are autosomal inherited disorders with clinical features of peroneal muscular atrophy. The disorder of peroneal muscular atrophy with pyramidal features has been referred to as HMSN Type V by Dyck. We have seen a 44 year-old male patient who complained of hypesthesia below the lovel of umbilicus and gait disturbance. Physical examination reveals increased knee jerk with extensor plantar reflex, pretibial muscle atrophy and weakness and hypesthesia below the level of T₁₀. He was diagnosed as HMSN Type V by clinical manifestation, electrodiagnostic study and sural nerve biopsy.

• Keywords: Hereditary motor and sensory neuropathy, Pyramidal features, Autosomal dominance