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

Martikainen, Mika H.; Roine, Susanna

doi:10.1007/s10194-011-0400-y

Cited by 20 publications

(11 citation statements)

References 9 publications

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“…However, their EEG data did not show ictal seizure activity, although the recordings were apparently brief and not continuous [4] . Similar case reports can be found, again lacking adequate EEG data [5] , [6] . It is possible that the diagnosis of nonconvulsive SE was missed in at least some of these cases, suggesting that this complication of CADASIL is underdiagnosed.…”

Section: Discussionsupporting

confidence: 73%

Recurrent status epilepticus as the primary neurological manifestation of CADASIL: A case report

Haddad

Ikard

Hiatt

et al. 2015

Epilepsy & Behavior Case Reports

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Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) often presents with a history of migraine with aura and eventual manifestations of dementia with unrelenting, repeated cerebral vascular insults. Only 6–10% of patients with CADASIL have been reported to develop seizures, and status epilepticus (SE) is exceedingly rare. Here, we describe a patient who presented with recurrent SE, with eventual biopsy diagnosis of CADASIL.An 80-year-old woman presented to our hospital three times in two years with decreased level of consciousness and subtle intermittent right-sided upper extremity and facial twitching. There was no known significant family history and no past medical history for seizures, stroke, migraine headache, or overt dementia. Electroencephalography revealed recurrent focal seizures with left hemispheric onset and evolution, fulfilling the criteria for focal SE each time. All three admissions required sedation with midazolam to control seizure activity, in addition to high doses of multiple antiepileptic drugs. Brain MRI repeatedly showed extensive abnormalities in the periventricular and deep white matter, subcortical white matter, and bilateral basal ganglia. Skin biopsy was obtained on the third admission, and electron microscopy showed numerous deposits of granular osmiophilic material, which are pathognomonic for CADASIL. Detailed investigations failed to reveal any other etiology for the patient's condition.This case illustrates the potential for nonconvulsive SE to be the sole manifestation of CADASIL. With the appropriate brain MRI findings, CADASIL should be added to the list of rare causes of SE.

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Section: Discussionsupporting

confidence: 73%

Recurrent status epilepticus as the primary neurological manifestation of CADASIL: A case report

Haddad

Ikard

Hiatt

et al. 2015

Epilepsy & Behavior Case Reports

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“…There is little published data on treatment responses to migraine in CADASIL. [ 9 – 12 ] We found a significant proportion of patients with migraine did not take medication due to the migraine episodes being infrequent. However in those that did, treatment responses seemed to be similar to that seen in the general population.…”

Section: Discussionmentioning

confidence: 96%

“…[ 5 – 8 ] Furthermore, little is known about how it responds to therapy and whether the management of migraine in CADASIL should differ from that of migraine in the general population. [ 9 – 12 ] Epidemiological studies have demonstrated that migraine, in particular migraine with aura, is an independent risk factor for stroke in the general population. [ 13 – 16 ] However, whether the presence of migraine with aura in CADASIL is predictive of an earlier onset of stroke and a more severe phenotype is unknown.…”

Section: Introductionmentioning

confidence: 99%

CADASIL: Migraine, Encephalopathy, Stroke and Their Inter-Relationships

Tan

Markus

2016

PLoS ONE

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BackgroundMigraine is common in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) but its treatment responses are not well described, and its relationship to stroke risk unknown. Encephalopathy is a less common presentation; it has been suggested it is related to migraine. We characterised migraine patterns and treatment responses in CADASIL, and examined associations between migraine and both stroke risk and encephalopathy.Methods300 symptomatic CADASIL patients were prospectively recruited from a national referral clinic over a nineteen year period, from 1996 to 2015. Data was collected using a standardised questionnaire. Migraine was classified according to the International Classification of Headache Disorders, 3rd edition (beta version). A cross-sectional analysis was carried out on the data collected.ResultsMigraine was present in 226 (75.3%), and the presenting feature in 203 (67.7%). It was usually accompanied by aura (89.8%). Patients showed variable responses to a variety of drugs for migraine. Of 24 given triptans, 45.5% had consistent or partial responses. None had complications following triptans. Thirty-three (11.0%) patients experienced encephalopathy lasting on average 8.1 ± 3.4 days. Patients with migraine with aura had higher odds of encephalopathy (OR = 5.4; 95%CI 1.6–28.4; p = 0.002). Patients with confusional aura had higher odds of encephalopathy than those with other aura types (OR = 2.5, 95%CI = 1.0–5.8, p = 0.04). There was also no increase in risk of encephalopathy with sex or age at onset of migraine. Migraineurs had a lower stroke risk than non-migraineurs (HR = 0.46, 95%CI 0.3–0.6, p = 2.1x10-6).ConclusionsMigraine with aura is a prominent feature of CADASIL. Treatment responses are similar to those seen in the general migraine population and no complications were observed with triptans. Migraine with aura was associated with increased risk of encephalopathy suggesting they may share pathophysiological mechanisms. There was no increased stroke risk associated with migraine, but risk appeared to be reduced although this finding needs confirming.

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“… 25 In rats, valproate activates the NOTCH3 /c-FLIP signaling cascade and promotes vascular smooth muscle cell survival, attenuating development of white matter hyperintensities. 26 It has also been reported that valproate controls migraines in patients with CADASIL, 27 raising the possibility that valproate, the classic mood-stabilizing drug, may represent a novel drug therapy for CADASIL. 26 …”

Section: Discussionmentioning

confidence: 99%

Bipolar II disorder as the initial presentation of CADASIL: an underdiagnosed manifestation

Wang

Kong

et al. 2017

NDT

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Mood disturbances have been documented in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). The highly varied morbidity indicates that the affective symptoms in CADASIL have not been cataloged systematically, leading to ineffective treatment, affecting the patients’ quality of life, and possibly resulting in suicide. We present a case of CADASIL with bipolar II disorder as the first manifestation. A middle-aged female reported recurrent depressive episodes and appeared treatment resistant to adequate dosages and durations of antidepressants. Following a structured psychiatric interview and neuropsychological assessment, a past episode of hypomania was identified. Added treatment with sodium valproate alleviated most symptoms. Considering late-onset bipolar disorder with unexplained decline in cognition, a medical history of migraine, and a suspected family history of stroke, further cranial magnetic resonance imaging scan was performed and revealed severe leukoencephalopathy, prompting further investigation. The diagnosis was revised to CADASIL after Arg587Cys NOTCH3 mutation was confirmed. This case highlights the evolving process of affective disorder diagnosis and underlying organic etiologies. Based on the overlap of white matter hyperintensities, NOTCH3 mutation, and valproate therapy in bipolar disorder and CADASIL, bipolar II depression may be a poorly recognized manifestation of CADASIL. Well-designed clinical trials are warranted to verify the current findings.

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

Cited by 20 publications

References 9 publications

Recurrent status epilepticus as the primary neurological manifestation of CADASIL: A case report

Recurrent status epilepticus as the primary neurological manifestation of CADASIL: A case report

CADASIL: Migraine, Encephalopathy, Stroke and Their Inter-Relationships

Bipolar II disorder as the initial presentation of CADASIL: an underdiagnosed manifestation

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