Man-in-the-Barrel Syndrome as an Atypical form of Amyotrophic Lateral Sclerosis

TMF, 58 years old, retired, reports that for approximately two years she has been showing amyotrophy, fasciculations and paresis in the upper limbs and pectoral girdle (Figures 1-2). Lower limbs with muscle strength grading 4, in addition to live reflexes. The electroneuromyography demonstrated a pre-ganglionic\purely motor chronic peripheral neurogenic involvement in the four limbs without conduction block. After excluding other possible diagnoses, it is an atypical case of Amyotrophic Lateral Sclerosis presenting as “Barrel Man” syndrome. Such nomenclature refers to a picture of bilateral brachial paresis that disables patients in basic and instrumental activities of daily life that requires elevation of the upper limbs. Since its description, there are some cases of patients with this syndrome, of varying etiologies (Figure 1 & 2).

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