Man-in-the-barrel syndrome, a symmetrical proximal brachial amyotrophic diplegia related to motor neuron diseases: a survey of nine cases

Abstract

SummaryObjective. To report on 9 patients presenting with sporadic motor neuron disease , who over a long period of time evolved with a symmetrical proximal brachial amyotrophic diplegia. methOdS. Nine patients were followed-up who , displayed, since onset, a progressive limitation of arm flexion/abduction resulting in a peculiar posture with both hands hanging loosely beside the trunk. Electrophysiological test results were consistent with lower motor neuron disease. Cervical MRI was performed in all patients. reSultS. Nine male subjects with ages ranging from 38 to 73 years at onset of symptoms, developed bilateral and symmetric paresis and atrophy of upper limb muscles. Proximal muscles were more involved than the distal groups. In most patients tendon reflexes were absent or hypoactive in the upper limbs. Needle electromyography (EMG) revealed positive sharp waves and fibrillations and high amplitude polyphasic potentials with an incomplete recruitment pattern in most upper limb muscles. EMG of lower limb muscles was normal in some cases while abnormal in others. MRC did not disclose cervical spinal cord abnormalities from C5-T1. cOncluSiOn. Attention is called to the Man-in-the-Barrel syndrome in some motor neuron diseases, especially in patients with progressive spinal atrophy and amyotrophic lateral sclerosis Key words: motor neuron diseases. man-in-the-Barrel Syndrome. Brachial amyotrophic diplegia. Progressive spinal atrophy. Amyotrophic lateral sclerosis.

Comparison between Flail Arm Syndrome and Upper Limb Onset Amyotrophic Lateral Sclerosis: Clinical Features and Electromyographic Findings

“…Patients with FAS showed a male predominance pattern as compared to patients with UL-ALS (5:1 vs. 1.8:1, p=0.129). These results are consistent with those reported in previous studies [3, 8, 9]; however, the small sample size of the current study may have impacted our ability to observe a statistically significant difference. Another remarkable finding was that UMN signs showed no significant between-group differences.…”

Amyotrophic Lateral Sclerosis Regional Variants (Brachial Amyotrophic Diplegia, Leg Amyotrophic Diplegia, and Isolated Bulbar Amyotrophic Lateral Sclerosis)

“…Unlike arm-onset ALS which usually presents with distal weakness, the majority of BAD patients have proximal weakness at presentation (70%). [8, 10, 11] In most series patients with BAD have only lower motor neuron involvement at presentation, with decreased or absent reflexes (47–90%), and some series define this group as a variant of progressive muscular atrophy. Progression of symptoms after 12 months has been variable from case series to case series.…”

“…[5] Other case series have shown a clear benefit in survival: in the UK and Melbourne study of patients with BAD limited to one region for at least 12 months they found mean survival of 76.8–79.9 months, with 52–52.6% alive at 5 years, and 13–15.8% alive at 10 years; in the US study there were no deaths in 3–11 years of follow up (n=10 patients);[8] and in two additional smaller studies showed no deaths in 2–10 years of follow up. [10, 11] There is increased 5 and 10 year survival in patients with BAD compared to classic ALS …”

Reversible man-in-the-barrel syndrome in myasthenia gravis

“…The etiology of MBS is varied, ranging in localization from central, spinal cord to peripheral including myopathic and neuropathic etiologies. [ 1 2 3 ] Herein we describe a patient who had MBS as a presenting symptom of myasthenia gravis among other atypical features.…”