Atrofia monomiélica distal de extremidad superior. Caso clínico
Author
dc.contributor.author
Matamala Capponi, José Manuel
Author
dc.contributor.author
Cea Muñoz, Gabriel
Author
dc.contributor.author
Salinas Ríos, Rodrigo
Author
dc.contributor.author
Vidal Téllez, Aarón
Author
dc.contributor.author
López, Ingeborg
Author
dc.contributor.author
Marileo, Roberto
Author
dc.contributor.author
Stuardo, Andrés
Admission date
dc.date.accessioned
2021-09-10T18:53:29Z
Available date
dc.date.available
2021-09-10T18:53:29Z
Publication date
dc.date.issued
2021
Cita de ítem
dc.identifier.citation
Revista Médica de Chile 2021; 149: 142-146
es_ES
Identifier
dc.identifier.issn
0034-9887
Identifier
dc.identifier.uri
https://repositorio.uchile.cl/handle/2250/181965
Abstract
dc.description.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.