Hirayama disease, or juvenile amyotrophy of distal upper extremity, is a benign, self-limiting cervical myelopathy consisting of selective unilateral weakness of the hand and forearm. The weakness slowly progresses until spontaneous arrest occurs within 5 years of onset. The condition predominantly affects Asian males and is thought to be secondary to spinal cord compression during neck flexion, because of a forward displacement of the posterior dural sac. The authors present what is to their knowledge the first reported case of a Caucasian male with a severe form of Hirayama disease, suffering from weakness of the leg as well as the forearm. An abnormal range of cervical flexion was observed at the C5–6 level. The patient was successfully treated by anterior cervical discectomy and fusion.

A 51-year-old woman had an attack of severe hemifacial pain with autonomic features as the presenting symptom of a lateral medullary infarction. A bilateral vertebral artery dissection was demonstrated. The existence of secondary cases may lead to a better understanding of the pathophysiology of trigeminal autonomic cephalalgias.

RESUMENEn 1875 M Raymond describió la aparición de una atrofia y debilidad muscular progresiva en 3 individuos supervivientes de una poliomielitis aguda en la infancia. Jean-Martin Charcot, sugirió que la lesión inicial debía dejar a las neuronas de estos individuos más sensibles para desarrollar enfermedades medulares posteriores y que la nueva debilidad era consecuencia del sobreuso de los músculos afectos. En 1979, tras la publicación de la descripción realizada por un paciente de 57 años de las dificultades motoras que desarrolló tras el padecimiento de una poliomielitis en la infancia, se produjo un incremento muy importante de comentarios de otros individuos con síntomas similares, llegando a acuñarse en los años 80 el término de síndrome post-polio (SPP). El término se reserva para describir el desarrollo de nuevos síntomas neurológicos, en especial para el desarrollo de una debilidad muscular, atrofia muscular y fatiga muscular nueva que no son explicables por otra causa médica, y que aparecen después de más de 15 años de la infección aguda. Se estima que afecta del 20 al 85% de individuos con antecedentes de poliomielitis en la infancia. En el año 2000 se describieron los primeros criterios diagnósticos. El SPP condiciona una alteración de la capacidad funcional del individuo.Su etiopatogenia es desconocida, pudiendo estar relacionada con el envejecimiento. También podría deberse a un cuadro inflamatorio persistente o estar influenciado por factores genéticos. No existe tratamiento farmacoló-gico eficaz, por lo que sólo se puede recomendar tratamiento sintomático y de entrenamiento muscular moderado.Palabras clave: Poliovirus. Poliomielitis. Síndrome postpolio. ABSTRACT Polio paralytic. New Problems: Postpolio SyndromeIn 1875 M Raymond described a progressive muscle wasting and weakness in 3 individuals survivors of childhood acute poliomyelitis. Jean-Martin Charcot suggested that the initial injury should let these guys neurons more sensitive to develop posterior spinal diseases and new weakness was the result of overuse of the affected muscles. In 1979, after the publication of the description given by a 57 year old patien on motor difficulties that developed after suffering of polio in childhood, there was a very significant increase of comments of other individuals with similar symptoms, reaching wedged in the 80s the term of post-polio syndrome.The term is reserved for describing the development of new neurological symptoms, especially for the development of muscle weakness, muscle atrophy and new muscle fatigue not explained by other medical causes, and appear after more than 15 years of infection acute. Is estimated to affect 20 to 85% of individuals with a history of polio in childhood. In 2000 first described the diagnostic criteria. This syndrome determines a change in the functional abilities. Its pathogenesis is unknown, may be associated with aging. It could also be due to an inflammatory persistent or be influenced by genetic factors. There is no effective drug treatment, so I can only rec...

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