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ISSN 1561-3119 (Electronic)

2015, Number 4

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Rev Cubana Pediatr 2015; 87 (4)

Hirayama disease observed in an adolescent

Vargas DJ, García ML, García NY, Hernández DZ, Barroso GE, Gutiérrez GJV, Novoa LLM

Language: Spanish
References: 19
Page: 522-528
PDF size: 210.40 Kb.


ABSTRACT

Hirayama disease or monomelic amyotrophy is a low frequent, barely reported illness at pediatric ages. Here is the clinical case of 15 years-old boy that presented with reduced muscular strength and loss of muscle mass; this condition began at the age of 10 year in his left hand and then affected the forearm. The illness progressed for three years and then remained stable. The needle electromyogram showed a lesion in C7-T1 myotome-depending motor axons or motoneurons and to less extent in those C5-C6 depending ones. The venous contrast computed tomography on a marked cervical cord flexion position; it was observed an evident asymmetric postmedullary vein ectasia from the C7-T2 myotomes, mainly on the left side, caused by the internal posterior vertebral vein plexus congestion. There was spontaneous remission of the disease in this patient, but it is necessary in other cases to limit the cervical cord motility with the use of a collar and to only perform surgery in the most rapidly evolving and severe cases.


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