Atrofia monomiélica distal de extremidad superior. Caso clínico / Monomelic amyotrophy. Report of one case
Rev. méd. Chile
; 149(1): 142-146, ene. 2021. ilus
Article
em Es
| LILACS
| ID: biblio-1389350
Biblioteca responsável:
CL126.2
ABSTRACT
Monomelic amyotrophy, also known as Hirayama disease, is a rare lower motor neuron syndrome due to localized lower motor neuron loss in the spinal cord at the cervical level. Clinically, monomelic amyotrophy is defined by the insidious onset of unilateral atrophy and weakness involving the hand and forearm, typically beginning in the second or third decade of life. We report 19-year-old man with a two years history of slowly progressive unilateral weakness and atrophy of his right-hand muscles. Neurological examination revealed weakness and atrophy in his intrinsic hand muscles, with sparing of the abductor pollicis brevis muscle. Also, mild atrophy of the ulnar aspect of the forearm was detected with sparing of the brachioradialis muscle. Electromyography showed active and chronic neurogenic changes affecting C8 and T1 myotomes, with mild chronic neurogenic changes on C7 myotome. Magnetic resonance imaging of his cervical spine revealed spinal cord atrophy involving C5 to C7 segments, associated with forward displacement of the posterior wall of the dura in cervical spine flexion. The clinical features associated with the imaging and electrophysiological findings support the diagnosis of monomelic amyotrophy.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
LILACS
Assunto principal:
Atrofias Musculares Espinais da Infância
Limite:
Adult
/
Humans
/
Male
Idioma:
Es
Revista:
Rev. méd. Chile
Assunto da revista:
MEDICINA
Ano de publicação:
2021
Tipo de documento:
Article
País de afiliação:
Chile
País de publicação:
Chile