A carregar...
Publicação
FOSMN: the facial expression of ALS
Autores
Orientador(es)
Resumo(s)
Facial onset sensory and motor neuronopathy (FOSMN syndrome) was described as a new neurological condition 16 years ago, in a study including 4 patients (Vucic et al., 2006). The disease was characterized by sensory changes in the face, chest and arms, associated with progressive weakness in the bulbar region, neck and upper limbs, and fasciculations. Nerve conduction studies disclosed low sensory nerve action potential (SNAP) amplitudes in upper limbs, and needle EMG confirmed neurogenic signs in bulbar and cervical anatomical regions. The suspected neurodegenerative origin was supported by the loss of lower motor neurons in the hypoglossal nucleus and cervical anterior horns, and the loss of sensory neurons in the dorsal root ganglia and trigeminal sensory nucleus in the post-mortem study of one of the included patient. In another study with 4 patients, including one woman, the authors proposed a link to amyotrophic lateral sclerosis (ALS), since one patient progressed to respiratory failure and another had neurogenic motor unit potentials in muscles innervated by the spinal cord lumbar region (Fluchere et al., 2011).
Descrição
© 2022 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.
Palavras-chave
Contexto Educativo
Citação
Clinical Neurophysiology 140 (2022) 167-168
Editora
Elsevier