This study identifies a rare neurological condition causing muscle wasting and weakness in a single limb. The condition, affecting younger adults, progresses slowly and remains confined to the affected limb.
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Single-limb atrophy is a rare condition characterized by muscle wasting and weakness.
Previous studies have not fully elucidated the clinical and electrophysiological features of this condition.
Purpose of the Study:
To describe the clinical, electrophysiological, and histological features of patients with single-limb atrophy.
To investigate the progression and long-term outcomes of this condition.
Main Methods:
A retrospective analysis of 23 patients diagnosed with single-limb atrophy between 1977 and 1981.
Clinical examination, electromyography (EMG), and muscle biopsy were performed.
Main Results:
The study identified 23 patients (13 upper-limb, 10 lower-limb) with insidious onset in their second and third decades.
Key features included male preponderance, sporadic occurrence, and weakness confined to one limb, with no cranial nerve, central nervous system, or sensory involvement.
Electromyography and histology indicated an anterior horn cell lesion, with slow progression over 2-4 years followed by a stable phase.
Conclusions:
Single-limb atrophy is a distinct neurological entity likely originating from anterior horn cell damage.
The condition presents with a characteristic pattern of localized weakness and slow progression, remaining confined to one limb even after 10-15 years.