Atrofia monomiélica distal de extremidad superior. Caso clínico

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...

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Autores principales:	Matamala,José Manuel, Cea,Gabriel, Salinas,Rodrigo, Vidal,Aarón, López,Ingeborg, Marileo,Roberto, Stuardo,Andrés
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2021
Materias:	Muscular Atrophy Spinal Spinal Cord Diseases
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872021000100142
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spelling	oai:scielo:S0034-988720210001001422021-05-09Atrofia monomiélica distal de extremidad superior. Caso clínicoMatamala,José ManuelCea,GabrielSalinas,RodrigoVidal,AarónLópez,IngeborgMarileo,RobertoStuardo,Andrés Muscular Atrophy Muscular Atrophy Spinal Spinal Cord Diseases 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.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.149 n.1 20212021-01-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872021000100142es10.4067/S0034-98872021000100142
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Muscular Atrophy Muscular Atrophy Spinal Spinal Cord Diseases
spellingShingle	Muscular Atrophy Muscular Atrophy Spinal Spinal Cord Diseases Matamala,José Manuel Cea,Gabriel Salinas,Rodrigo Vidal,Aarón López,Ingeborg Marileo,Roberto Stuardo,Andrés Atrofia monomiélica distal de extremidad superior. Caso clínico
description	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.
author	Matamala,José Manuel Cea,Gabriel Salinas,Rodrigo Vidal,Aarón López,Ingeborg Marileo,Roberto Stuardo,Andrés
author_facet	Matamala,José Manuel Cea,Gabriel Salinas,Rodrigo Vidal,Aarón López,Ingeborg Marileo,Roberto Stuardo,Andrés
author_sort	Matamala,José Manuel
title	Atrofia monomiélica distal de extremidad superior. Caso clínico
title_short	Atrofia monomiélica distal de extremidad superior. Caso clínico
title_full	Atrofia monomiélica distal de extremidad superior. Caso clínico
title_fullStr	Atrofia monomiélica distal de extremidad superior. Caso clínico
title_full_unstemmed	Atrofia monomiélica distal de extremidad superior. Caso clínico
title_sort	atrofia monomiélica distal de extremidad superior. caso clínico
publisher	Sociedad Médica de Santiago
publishDate	2021
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872021000100142
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Atrofia monomiélica distal de extremidad superior. Caso clínico

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