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Título

Síndrome do homem no barril como apresentação de doença do neurônio motor: relato de caso

RESUMO

MAN IN THE BARREL SYNDROME AS A PRESENTATION OF MOTOR NEURON DISEASE: CASE REPORT

Pedro Thiago Simões Ferreira, Juliana Oliveira de Almeida, João Vitor Nunes Sobreira Cruz, Allef Roberto Gomes Bezerra, Kirsten Araujo Melo, Patrícia Pereira Nunes, Simone de Cassia Silveira da Silva Lucena, Fernando Temório Gameleira, Mariana Cota Bastos.

Main author's email: pedro_thiago22@hotmail.com

Presentation of the case

Male, 52 years old, history of fasciculations and sudden biparesis of the limbs, asymmetric, for 3 years, evolving with mild paresis of the LLL with progression to RLL in 10 days, without prejudice on gait, associated with dysphonia and intermittent dysphagia. The condition followed with a gradual worsening of the tetraparesis, with a severe predominance in the upper limbs, with no complaints of sensitivity. Personal Background: systemic arterial hypertension. On examination: alert, oriented, dysarthria and dysphonia, isochoric and photoreactive pupils, plegia and bilateral hypotrophy of the upper limbs, generalized hypotonia, with patellar hyperreflexia, Babinski's sign on the right, Hoffman's sign on the left. Neuroaxis imaging exams without alterations. Electroneuromyography (ENMG): findings consistent with motor neuron disease.

Discussion:

Clinical signs of upper and lower motor neuron lesions, with a clear predominance of involvement in the upper limbs, with a characteristic phenotype of man-in-the-barrel syndrome, required the need for an electroneuromyographic study, which showed alterations compatible with sensory preganglionic involvement, showing signs of chronic denervation and reinnervation in at least two body segments, corroborating the hypothesis of motor neuron disease / amyotrophic lateral sclerosis (ALS).

Final comments:

The nomenclature “man in the barrel syndrome” (MBS) was first used in 1983, since then, many cases have been reported, although the vast majority of motor neuron pathologies have a classically different etiopathogenesis from this description. Still, MBS is not exclusive to ALS, as a considerable part of the reports are caused by Progressive Spinal Amyotrophy, differential diagnosis that requires neurophysiological evaluation for clarification, given its lower severity compared to ALS. The patient above presented an atypical clinic of upper limb biplegia, and the ENMG was consistent with the diagnosis of ALS manifested with MBS.

Palavras Chave

Síndrome do homem no barril