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From the Departments of Neurology (Drs. Katz and Andersson) at Palo Alto VA Medical Center and Stanford University, Palo Alto, CA; University of Texas (Drs. Wolfe, Elliott, Nations, Bryan, and Barohn), Southwestern Medical Center, Dallas, TX; and Wilford Hall Medical Center (Dr. Saperstein), San Antonio, TX.
Address correspondence and reprint requests to Dr. Jonathan Katz, Department of Neurology (127), Department of Veterans Affairs, 3801 Miranda Ave., Palo Alto, CA 94304.
OBJECTIVE: To describe a sporadic motor neuron disorder that remains largely restricted to the upper limbs over time.
BACKGROUND: Progressive amyotrophy that is isolated to the upper limbs in an adult often suggests ALS. The fact that weakness can remain largely confined to the arms for long periods of time in individuals presenting with this phenotype has not been emphasized.
METHODS: We reviewed the records of patients who had a neurogenic "man-in-the-barrel" phenotype documented by examination at least 18 months after onset. These patients had severe bilateral upper-extremity neurogenic atrophy that spared lower-extremity, respiratory, and bulbar musculature.
RESULTS: Nine of 10 patients meeting these criteria had a purely lower motor neuron disorder. During follow-up periods ranging from 3 to 11 years from onset, only three patients developed lower-extremity weakness, and none developed respiratory or bulbar dysfunction or lost the ability to ambulate.
CONCLUSION: Patients presenting with severe weakness that is fully isolated to the upper limbs, without pyramidal signs, may have a relatively stable variant of motor neuron disease.
Key words: Man-in-the-barrel syndrome—Amyotrophic lateral sclerosis—Focal amyotrophy—Motor neuron disease—Brachial amyotrophic diplegia.
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