Variants in the CACNA1A gene on chromosome 19p13 result in a spectrum of neurological phenotypes ranging from familial or sporadic hemiplegic migraine to congenital or progressive encephalopathies. Patients with CACNA1A variants often show acute attacks with ataxia or hemiplegia till coma, sometimes related to unilateral brain oedema. No guidelines for the medical management of these attacks are available since treatment is empiric, and many cases do not respond to common antimigraine drugs.

Combined early treatment in hemiplegic attacks related to CACNA1A encephalopathy with brain oedema: Blocking the cascade? / Camia, Francesca; Pisciotta, Livia; Morana, Giovanni; Schiaffino, Maria Cristina; Renna, Salvatore; Carrera, Paola; Ferrari, Maurizio; Baglietto, Maria Giuseppina; Veneselli, Edvige; Siri, Laura; Mancardi, Maria Margherita. - In: CEPHALALGIA. - ISSN 0333-1024. - 37:12(2017), pp. 1202-1206. [10.1177/0333102416668655]

Combined early treatment in hemiplegic attacks related to CACNA1A encephalopathy with brain oedema: Blocking the cascade?

FERRARI, MAURIZIO;
2017-01-01

Abstract

Variants in the CACNA1A gene on chromosome 19p13 result in a spectrum of neurological phenotypes ranging from familial or sporadic hemiplegic migraine to congenital or progressive encephalopathies. Patients with CACNA1A variants often show acute attacks with ataxia or hemiplegia till coma, sometimes related to unilateral brain oedema. No guidelines for the medical management of these attacks are available since treatment is empiric, and many cases do not respond to common antimigraine drugs.
2017
Hemiplegic migraine; brain oedema; case report; cerebellar atrophy; recurrent coma; steroids
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.11768/61233
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