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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.45 no.3 São Paulo Sept. 1987

http://dx.doi.org/10.1590/S0004-282X1987000300011 

Mielinolise pontina central e extra-pontinas: apresentação de um caso autopsiado e revisão da literatura

 

Central pontine and extrapontine myelinolysis: report of an autopsied case and review of the literature

 

 

José Eymard Homem Pittella; Helenice Gobbi

Laboratório de Neuropatologia, Departamento de Anatomia Patológica e Medicina Legal, Faculdade de Medicina da Universidade Federal de Minas Gerais, Belo Horizonte

 

 


RESUMO

Relata-se um caso autopsiado de mielinólise pontina central e extra-pontina em paciente diabética de 16 anos, desidratada, que recebeu grande quantidade de solução salina isotônica intravenosa, diariamente, a partir do primeiro dia da internação hospitalar. Nos três primeiros dias houve aumento em mais de 30 mEq/1 do sódio plasmático em relação ao valor inicial. Após ligeira queda nos dias subsequentes, o sódio novamente aumentou e persistiu com níveis acima do normal por mais 12 dias. No sexto dia desta nova e persistente elevação do sódio surgiram manifestações neurológicas progressivas caracterizadas por mutismo, impossibilidade de movimentação da cabeça, tronco e membros, recusa de dieta oral e dificuldade de eliminar as secreções das vias aéreas respiratórias, que permaneceram até o óbito. O exame neuropatológico, além da mielinólise pontina central maciça, revelou lesões desmielinizantes focais múltiplas, com quadro histopatológico semelhante ao da ponte, na substância branca subcortical do lobo temporal esquerdo, trato óptico direito, cápsulas externa e extrema à direita, fascículo mamilar principal e substância branca subcortical do hemisfério cerebelar esquerdo. Faz-se revisão dos casos de mielinólise extra-pontina registrados na literatura, o presente caso representando a 309. Comenta-se o quadro clínico e a etiopatogênese da mielinólise pontina central e extra-pontina, destacando-se como um dos prováveis fatores causais, além da correção rápida e persistente do sódio plasmático, a oscilação dos níveis deste eletrólito em pacientes com hiponatremia e/ou desidratação.


SUMMARY

An autopsied case of central pontine and extrapontine myelinolysis in a 16-year-old diabetic girl is described. Due to dehydration she was treated vigorously with daily intravenous isotonic saline, from the first day of hospitalization. In the first three days the serum sodium level increased by more than 30 mEq/1 when compared with the initial level. By the next days the serum sodium level, after a mild drop, rose again and was maintained above normal range for a further 12 days. On the sixth day of this new and sustained serum sodium increase, the patient presented progressive neurological manifestations that remained until her death, characterized by mutism, inability to eat, to move her head, trunk, and members and, in addition, retention of respiratory secretions. The neuropathological examination showed massive central pontine myelinolysis and similar myelinolytic lesions in the subcortical white matter of the temporal lobe, the right optic tract, the external and extreme capsules to the right, the main mammillary tract and the subcortical white matter of the left cerebellar hemisphere. The review of the literature on central pontine and extrapontine myelinolysis shows that the present case is the 30th of such condition. The clinical picture and the etiopathogenesis of central pontine and extrapontine myelinolysis are commented upon. It is suggested, as possible causative factors, the persistent and rapid correction of serum sodium concentration as well as its flutuation in patients with hyponatremia and/or dehydration.


 

 

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