Man-in-the-barrel syndrome, a symmetrical proximal brachial amyotrophic diplegia related to motor neuron diseases: a survey of nine cases (original) (raw)
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Man-in-the-barrel syndrome as an atypical form of Amyotrophic Lateral Sclerosis
Revista Neurociências, 2019
The “Man-in-the-barrel syndrome” makes reference to a picture of bilateral brachial muscular weakness, which incapacitates the patients in functional activities with upper limbs elevation. Since its description, there are several cases described of patients with this syndrome, whose etiology differs from the first one described. We read the article by Sasaki et al. “Atypical form of amyotrophic lateral sclerosis: a new term to define a previously well-known form of ALS” (2000), concerning the atypical form of amyotrophic lateral sclerosis (ALS). The pattern of muscular atrophy in these patients differed from that of typical ALS in that severe muscle involvement was confined to the upper limbs, predominantly the proximal portion and shoulder girdle.
Monomelic amyotrophy with proximal upper limb involvement: a case report
Journal of Medical Case Reports, 2016
Background: Monomelic amyotrophy is an uncommon, benign, unilateral disorder of the lower motor neurons, affecting predominantly the hand and forearm muscles. Proximal involvement of the arm and shoulder muscles is an unusual presentation that has been rarely reported in the literature. Case presentation: A 28-year-old white man presented with insidious-onset, slowly progressive, unilateral weakness and atrophy of his left shoulder girdle and deltoid muscles. A neurological examination revealed weakness and atrophy in his left deltoid, infraspinatus and supraspinatus muscles. Electromyography demonstrated an active and chronic neurogenic pattern affecting his left C5 and C6 myotomes; magnetic resonance imaging of his cervical spine was normal. He did well with conservative treatment. Conclusions: Upper limb proximal form of monomelic amyotrophy is a rare clinical entity with a wide differential diagnosis. Physicians, especially neurologists, should be familiar with this benign condition to avoid inappropriately labeling patients as having amyotrophic lateral sclerosis and other disorders with less favorable outcomes.
Muscle & Nerve, 2008
Previous studies suggest that in amyotrophic lateral sclerosis (ALS) the abductor pollicis brevis (APB) and first dorsal interosseous (FDI) are more severely involved than abductor digiti minimi (ADM). To elucidate the pattern, frequency, extent, and specificity of such dissociated muscle atrophy in ALS, compound muscle action potentials recorded from APB, FDI, and ADM were analyzed in 77 ALS patients, 171 normal controls, and 196 disease controls. Compared with normal controls, ALS patients had a reduced APB/ADM amplitude ratio (P Ͻ 0.001) and FDI/ADM ratio (P Ͻ 0.001), whereas patients with other anterior horn diseases showed similar APB/ADM and FDI/ADM ratios to normal values. Decreased APB/ADM ratio was found in 41% of ALS patients, 5% of normal controls, and 4% of disease controls. Prominent muscle atrophy in APB and FDI, with relatively preserved ADM, appears to be specific to ALS. Dissociated hand muscle atrophy presumably reflects part of the pathophysiology and supports the diagnosis of ALS.
A Challenging Rare Motor Neuron Disease to Diagnose
https://www.ijrrjournal.com/IJRR\_Vol.9\_Issue.12\_Dec2022/IJRR-Abstract03.html, 2022
Mills hemiplegic variant is a progressive motor neuron disease characterized by spastic ascending or descending hemiparesis or hemiplegia with no sensory involvement. We presented a 53years old male patient with a history of present complaints was difficulty on walking, and difficulty in speech then he developed left lower limb weakness followed by left upper limb weakness for the last 6 months. Electromyography showed a neurogenic pattern with denervation with reinnervation. Then, he successfully accomplished his treatment. The biggest challenge in making a diagnosis is ruling out other conditions that may have symptoms similar to this disease entity.
Clinical features and associations of 560 cases of motor neuron disease
Journal of Neurology, Neurosurgery & Psychiatry, 1990
In 560 cases of motor neuron disease, studied retrospectively from their casenotes in three teaching centres, the age at onset ranged from 13 to 87 years (mean 56 years), and the mean duration of illness until death was 2-6 years. In the subgroup of the disease presenting with progressive bulbar palsy presenting after age 59 years, there was a previously unrecognised excess of females sufficient to equalise the sex ratio of incidence of the disease in this age group. No potentially causative clinical associations emerged; no relation was noted between occupational exposure to leather products, trauma or surgical procedures and the disease. There was a trend for patients with motor neuron disease to give a history of abstention from alcohol. The cause of motor neuron disease is unknown, but epidemiological studies are important in studying new approaches to this inexorably progressive and fatal disease. In all such studies it is important to assess the accuracy of diagnosis, 2as well as evaluating the clinical patterns of the disease so that possible associations that might prove important in assessing new notions of causation can be tested. We have reviewed the clinical data on 560 consecutive patients investigated at three neurological centres in the United Kingdom and report our observations here.
Benign monomelic amyotrophy with proximal upper limb involvement: case report
Arquivos de Neuro-Psiquiatria, 2007
Monomelic amyotrophy (MA) is a rare disorder in which neurogenic amyotrophy is restricted to a superior or inferior limb, generally sporadic, and characterized by gradual, often insidious onset 1-2 . It differs from other lower motor neuron disorders in that it involves only one limb. Upper motor neuron signs are usually absent, and progression slowly evolves over months or even years, followed by clinical stabilization of symptoms 3 . MA affects younger individuals (second to third decade of life) and predominantly males, also characterized by a typical geographic distribution 4 . The majority of cases are encountered in Asia, mainly in Japan and India. There are few reports of MA in western countries .
Electro Clinical Profiles of Motor Neuron Disease and Atypical Motor Neuron Disorders: A Case Series
Journal of National Institute of Neurosciences Bangladesh, 2018
Amyotrophic lateral sclerosis (ALS) is the commonest MND phenotype. Although many of the atypical motor neuron disorders share some features with ALS, they often can be distinguished by their clinical and electrophysiologic characteristics. Here we present five different cases with varied clinical findings. All the patients were referred from outpatient department to neurophysiology laboratory where electrodiagnostic (EDX) correlations helped to come to a conclusion. The nerve conduction study protocol for a suspected atypi¬cal motor neuron disorder is the same as that for ALS. Akin to the nerve conduction studies, the EMG evaluation of patients with suspected atypical motor neuron disorders is similar to that of ALS. An extensive study is indicated, often of all four limbs, the paraspinal muscles, and the bulbar muscles to reach a possible diagnosis. History, clinical findings and electrophysiological correlation often help to differentiate these atypical motor neuron disorders. Correct diagnosis is needed for further evaluation and prognosis. In this case series five (5) cases have described who are referred from outpatient department to neurophysiology laboratory for electrodiagnostic (EDX) correlations.