The classification of neurological disorders in the 11th revision of the International Classification of Diseases (ICD-11)

摘要

Facial onset sensory and motor neurono-pathy (FOSMN) syndrome may represent an amyotrophic lateral sclerosis pheno-type as indicated by associated with the heterozygous D90A superoxide dismu-tase-1 (SOD-1) gene mutation. Facial onset sensory and motor neuronopa-thy (FOSMN) syndrome is a rare and slowly progressive neurological disorder heralded by development of sensory symptoms within the face (trigeminal nerve distribution), and followed by evolution of sensory and motor deficits in a rostral-caudal direction.1 The motor deficits are characterised by lower motor neurone features including muscle weakness and wasting, cramps and fasciculations, with absence of upper motor neurone signs.The pathophysiological mechanisms underlying FOSMN syndrome remain to be fully elucidated, although neu-rodegenerative and autoimmune mechanisms have been proposed.