Variante faringo-cérvico-braquial del síndrome de Guillain-Barré

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Abstract

El síndrome de Guillain-Barré es una polineuropatía desmielinizante inflamatoria aguda, existiendo variantes locorregionales poco frecuentes como la faringo-cérvico-braquial. Presentamos el caso de un varón de 63 años de edad que acudió a urgencias por debilidad de extremidades superiores y a nivel cervical, con incapacidad para deglutir y masticar, y una insuficiencia respiratoria aguda rápidamente progresiva por debilidad de músculos respiratorios, todo ello secundario a la variante faringo-cérvico-braquial del síndrome de Guillain-Barré. Esta variante, aunque poco frecuente, presenta un patrón clínico y unos criterios diagnósticos bien definidos, importantes de reconocer para poder así iniciar de forma precoz el tratamiento y mejorar el pronóstico, no siempre favorable, de estos pacientes.

Translated abstract

Guillain-Barré syndrome is an acute inflammatory demyelinating polyneuropathy. Infrequent loco-regional variants, like the pharyngeal-cervical-brachial, have been described. We report the case of a 63-year-old male admitted to the emergency department with cervical and upper limb weakness, inability to swallow and chew, he also presented a rapidly progressive acute respiratory failure due to weakness of the respiratory muscles secondary to the pharyngeal-cervical-brachial variant of Guillain-Barré syndrome. This variant, although unusual, presents a well-defined clinical pattern and diagnostic criteria, which is important in order to start an early treatment to improve the prognosis, not always favorable, to these patients.

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Unusual clinical variants and signs in Guillain-Barré syndrome.

A Ropper (1986)

Limited regional forms of the Guillain-Barré syndrome (GBS) and unusual focal signs or symptoms that resemble other illnesses are described: pharyngeal-cervical-brachial weakness with ptosis, sparing power, and reflexes in the legs; paraparesis with normal power and reflexes in the arms; early severe ptosis without other signs of oculomotor weakness; and acute severe midline back pain at the onset. The first two variants did not progress to typical generalized GBS, delaying the proper diagnosis. Regional and functional variants suggest that the pathologic, and perhaps immunologic abnormalities of GBS can be localized and selective.

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[Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a well-defined clinical condition with a heterogeneous immunological profile].

C Más-Lázaro, A García-Pastor, S Díaz-Insa … (2008)

The pharyngeal-cervical-brachial variant of Guillain-Barre syndrome (GBS) is rare. This variant has its own specific clinical aspects but a heterogeneous immunological profile.