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Vol. 2. Núm. 2.
Páginas 81-86 (enero 1991)
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Vol. 2. Núm. 2.
Páginas 81-86 (enero 1991)
Tumores multiplos do sistema nervoso na neurofibromatose
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1782
P. Breia
Serviço de Neurologia do Hospital de Egas Moniz. Lisboa
S. Figueiredo*, M. Campos*
* Serviço de Neurocirurgia do Hospital de Egas Moniz. Lisboa
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Resumo

São apresentados dois doentes com tumores múltiplos do sistema nervoso e estigmas cutâneos de neurofibromatose. Em ambos havia história familiar da doença e a coexistência de neurinoma do acústico bilateral. O primeiro doente, com história de surdez bilateral progressiva com dez años de evolução, hemiparesia esquerda e confusão mental recentes, apresentava meningeoma e quisto aracnoideu da convexidade fronto-parietal direita e menigeoma da convexidade cerebelosa esquerda. A segunda doente, con um quadro de hipertensão intracraniana há dez meses e hipocusia esquerda, tinha à data do diagnóstico apenas um neurinoma do acústico esquerdo. Posteriormente surgiram outros tumores com o diagnóstico provável de neurinoma do acústico direito, meningeoma do seio cavernoso direito, ependimoma, neurinoma da cauda equina e ainda siringobulbia e siringomielia, clinicamente silenciosos.

Palabras chave:
Neurofibromatose
Neurinoma do acústico
Tumores múltiplos
Siringobulbia
Siringomielia
Summary

Two patients with multiple tumors of the nervous system and cutaneous features of neurofibromatosis are described. Both had a family history of this disease and bilateral acoustic neurinoma. The first patient had a history of progressive bilateral deafness for ten years and recent left hemiparesis and confusional state. He presented with a meningioma and arachnoid cyst of the frontoparietal right convexity and another meningioma of cerebellar convexity. The other patient had intracraneal hypertension for ten months, left deafness and just a left acoustic neurinoma at the time of diagnosis. Other probable tumors, syringobulbia and syringomielia were diagnosed later on.

Key words:
Neurofibromatosis
Acoustic neurinoma
Multiple tumors
Syringobulbia
Syringomielia

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