Headache Medicine
ISSN 2178-7468
e
-ISSN 2763-6178
v.11
Supplement
p. 56
October 2020.
56
ASAA
DOI: 10.48208/HeadacheMed.2020.Supplement.56
Costa, B.; Carvalho, F.; Kubota, G.; Andrade, D.; Fortini, I.
Headache Medicine
Case report: migraine with aura in patient with cadasil
Bárbara Costa, Felipe Carvalho, Gabriel Kubota, Daniel Andrade, Ida Fortini
HC- FMUSP
Introduction
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most frequent hereditary
cause of brain ischemic small vessel disease (1). Migraine with aura (MA) is typically the presenting and most common clinical feature of
CADASIL. The estimated prevalence of MA in CADASIL ranges from 20 to 40% (2).
Case Report
43-year-old woman, with previous history of hypertension, diabetes, and episodic migraine with visual aura, suffered three episodes of
hemihyposthesia in 2016, 2017 and 2018 (one on her right and the other two on her left). In the last of these episodes, she reported
worsening of the visual acuity of her right eye, and developed chronic migraine. Brain MRI showed extensive areas of conuent T2
hyperintensity in the white matter, as well as in the nucleocapsular and bilateral thalamic regions. In all three episodes the patient was
treated with IV or oral corticosteroids and had partial improvement of the symptoms. The patient had familiar history of relatives who
had suffered multiple strokes.
Discussion
The diagnosis of multiple sclerosis was rstly considered due to the evolution in clinical attacks and the response to corticotherapy.
However, the patient’s family history, MRI ndings and previous diagnosis of migraine with visual aurea lead to the suspicion of
CADASIL. This diagnosis was ultimately conrmed through genetic testing that showed C> T variation in NOTCH3 gene. Migraine was
successfully treated with greater occipital nerve blocks and topiramate.
Final Comment
CADASIL stroke-like attacks remain a therapeutic challenge. It is possible that corticosteroid treatment may benet these patients by
reducing the inammatory process that results from blood-brain barrier breakdown. More studies are needed to evaluate the efcacy and
safety of corticotherapy in this population.
Keywords:
CADASIL, migraine, aura, Leukoencephalopathy