• Annals of Clinical Neurophysiology
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• v.2 no.2
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• pp.125-129
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• 2000

Benign monomelic amyotrophy(BMA) is an uncommon cause of progressive mildly disabling atrophy and weakness of a limb. It predominantly affects the distal upper limb of young men. I present two men with BMA of the lower extremities. Although the disorder seemed clinically confined to a arm or leg, I confirmed by electromyography evidence of denervation in the other extremities. I reviewed the literatures and discuss the differential diagnosis. BMA is a diagnosis of exclusion that requires consideration in men with unilateral arm or leg atrophy and weakness.

• Annals of Clinical Neurophysiology
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• v.14 no.2
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• pp.72-75
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• 2012

Ischemic monomelic neuropathy and myopathy are rare complications of peripheral arterial occlusive disease. We report a case of ischemic monomelic neuropathy of the right sural, common peroneal and posterior tibial nerves and ischemic myopathy of the right tibialis anterior resulting from the occlusion of the right common femoral arteries despite successful revascularization. Ischemic monomelic neuropathy and myopathy can occur as a result of occlusion of the specific peripheral artery.

• Clinical Pain
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• v.19 no.2
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• pp.101-105
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• 2020

Benign monomelic amyotrophy (BMA) is a benign motor neuron disease in which amyotrophic change is confined to either the upper or the lower extremities. Numerous cases of BMA have been reported from Japan and India. However, only a few cases have been reported from other regions, including South Korea. Here we report a rare case of late-onset BMA in Korean male using conventional diagnostic approach with magnetic resonance imaging and electromyography. The patient received ten sessions of manual therapy, which focused on strengthening of the left ankle. At two-month follow up, weakness was still isolated to the patient's left ankle. There were no signs of disease progression.

• Genomics & Informatics
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• v.9 no.2
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• pp.64-68
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• 2011

Monomelic amyotrophy (MA), also known as Hirayama disease, occurs mainly in young men and manifests as weakness and wasting of the muscles of the distal upper limbs. Here, we sought to identify a genetic basis for MA. Given the predominance of MA in males, we focused on candidate neurological disease genes located on the X chromosome, selecting two X-linked candidate genes, androgen receptor (AR ) and ubiquitin-like modifier activating enzyme 1 (UBA1). Screening for genetic variants using patients' genomic DNA revealed three known genetic variants in the coding region of the AR gene: one nonsynonymous single-nucleotide polymorphism (SNP; rs78686797) encoding Leu57Gln, and two variants of polymorphic trinucleotide repeat segments that encode polyglutamine (CAG repeat; rs5902610) and polyglycine (GGC repeat; rs3138869) tracts. Notably, the Leu57Gln polymorphism was found in two patients with MA from 24 MA patients, whereas no variants were found in 142 healthy male controls. However, the numbers of CAG and GGC repeats in the AR gene were within the normal range. These data suggest that the Leu57Gln polymorphism encoded by the X-linked AR gene may contribute to the development of MA.

• The Journal of the Korean bone and joint tumor society
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• v.12 no.1
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• pp.71-77
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• 2006

Chondrosarcoma is one of the most common types of primary bone sarcoma. With the exception of the mesenchymal subtype, chondrosarcomas are usually low-grade lesions and rarely show multicentricity or distant metastasis. Only rare cases of multicentric chondrosarcomas have been reported in association with Ollier's disease and Maffucci's syndrome. To our knowledge, no report has been issued of a synchronous multicentric chondrosarcoma occurrence across a joint. We experienced a 30-year-old man with a synchronous monomelic juxta-articular multicentric chondrosarcoma across a shoulder joint in the absence of pulmonary and visceral metastases. He was treated by curettage and cement filling with allograft in the acromion and wedge resection with cement filling in the proximal humerus. At the 18-month follow-up, there was no evidence of recurrence, and the patient had full range of motion without pain.