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Rapid improvement of a complex migrainous episode with sodium valproate in a patient with CADASIL

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited disease of small arteries caused by mutations in the Notch3 gene. Complex migrainous episodes, such as acute confusional migraine, status migrainosus with persisting aura, and “CADASI...

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Autores principales: Martikainen, Mika H., Roine, Susanna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Milan 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3253158/
https://www.ncbi.nlm.nih.gov/pubmed/22065121
http://dx.doi.org/10.1007/s10194-011-0400-y
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author Martikainen, Mika H.
Roine, Susanna
author_facet Martikainen, Mika H.
Roine, Susanna
author_sort Martikainen, Mika H.
collection PubMed
description Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited disease of small arteries caused by mutations in the Notch3 gene. Complex migrainous episodes, such as acute confusional migraine, status migrainosus with persisting aura, and “CADASIL coma” have been described in patients with CADASIL. However, there are few descriptions of effective treatment of such episodes. We describe a 44-year-old male with CADASIL, who presented with sudden-onset aphasia and decreased responsiveness after prolonged, severe migraine attack. Subsequently, the patient had two generalized seizures. A subtle status epilepticus was suspected because of drowsiness and seizures, and intravenous sodium valproate medication was initiated. EEG recording showed left hemispheric attenuation but no spike discharges, thus not confirming epileptic mechanism. The clinical status of the patient improved markedly after the initiation of valproate. The patient started speaking again; drowsiness and headache subsided. In repeated EEG recording, the left hemispheric attenuation disappeared. Diffusion weighted MR imaging showed no signs of recent ischemic events. The patient recovered fully from the episode with no further seizures. We suggest that CADASIL patients with acute complex migrainous episodes may benefit from intravenous sodium valproate.
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spelling pubmed-32531582012-01-20 Rapid improvement of a complex migrainous episode with sodium valproate in a patient with CADASIL Martikainen, Mika H. Roine, Susanna J Headache Pain Brief Report Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited disease of small arteries caused by mutations in the Notch3 gene. Complex migrainous episodes, such as acute confusional migraine, status migrainosus with persisting aura, and “CADASIL coma” have been described in patients with CADASIL. However, there are few descriptions of effective treatment of such episodes. We describe a 44-year-old male with CADASIL, who presented with sudden-onset aphasia and decreased responsiveness after prolonged, severe migraine attack. Subsequently, the patient had two generalized seizures. A subtle status epilepticus was suspected because of drowsiness and seizures, and intravenous sodium valproate medication was initiated. EEG recording showed left hemispheric attenuation but no spike discharges, thus not confirming epileptic mechanism. The clinical status of the patient improved markedly after the initiation of valproate. The patient started speaking again; drowsiness and headache subsided. In repeated EEG recording, the left hemispheric attenuation disappeared. Diffusion weighted MR imaging showed no signs of recent ischemic events. The patient recovered fully from the episode with no further seizures. We suggest that CADASIL patients with acute complex migrainous episodes may benefit from intravenous sodium valproate. Springer Milan 2011-11-08 /pmc/articles/PMC3253158/ /pubmed/22065121 http://dx.doi.org/10.1007/s10194-011-0400-y Text en © The Author(s) 2011 https://creativecommons.org/licenses/by/4.0/This article is distributed under the terms of the Creative Commons Attribution License which permits any use, distribution and reproduction in any medium, provided the original author(s) and source are credited.
spellingShingle Brief Report
Martikainen, Mika H.
Roine, Susanna
Rapid improvement of a complex migrainous episode with sodium valproate in a patient with CADASIL
title Rapid improvement of a complex migrainous episode with sodium valproate in a patient with CADASIL
title_full Rapid improvement of a complex migrainous episode with sodium valproate in a patient with CADASIL
title_fullStr Rapid improvement of a complex migrainous episode with sodium valproate in a patient with CADASIL
title_full_unstemmed Rapid improvement of a complex migrainous episode with sodium valproate in a patient with CADASIL
title_short Rapid improvement of a complex migrainous episode with sodium valproate in a patient with CADASIL
title_sort rapid improvement of a complex migrainous episode with sodium valproate in a patient with cadasil
topic Brief Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3253158/
https://www.ncbi.nlm.nih.gov/pubmed/22065121
http://dx.doi.org/10.1007/s10194-011-0400-y
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