Study of monomelic amyotrophy of the lower limbs in the territory of the Western Balkans: Case series.

RATIONALE

Monomelic amyotrophy is a rare form of motor neuron disease in which the neurogenic atrophy is restricted to 1 limb, mostly the distal part of the arm. The disease most often occurs in Asia, especially in Japan and India, while in European countries, this disease is rarely recognized. Registration and publication of new cases of this disease aims to increase the awareness of clinicians about the existence of this disease in European countries, and with the aim of easier recognition and faster diagnosis of this essentially benign disorder.

PATIENT CONCERNS

Five patients with signs of atrophy of the muscles of 1 leg were examined at our Institution.

DIAGNOSES

The criteria for selecting patients were as follows: clinical evidence of wasting and weakness confined to the 1 lower limb; progressive course, or initial progression followed by stationary course; absence of any definite sensory loss or central nervous system involved; no evidence of compression lesion of the spinal cord.

INTERVENTIONS

The clinical characteristics of our patients were similar to those previously described in the literature. The characteristic clinical features were sporadic occurrence, predominance in males with an initially progressive course for 2 to 5 years followed by a stationary state. There was no family history of neuromuscular disease.

OUTCOMES

The electromyographic finding was consistent with a chronic neuropathic disorder. Magnetic resonance imaging of the lumbosacral spine excluded intraspinal pathologies and root compression in all cases.

LESSONS SUBSECTION

Monomelic amyotrophy of the lower limb is a rare disease that should be considered in cases of slow progressive unilateral amyotrophy of a single leg, especially in younger and middle-aged men, not only in Asia but also in the Western Balkans and Europe.