Epilepsy in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy

Haan, Joost; Oberstein, Saskia A J Lesnik; Ferrari, Michel D.

doi:10.1159/000106518

Cited by 11 publications

(6 citation statements)

References 12 publications

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“…The initial seizures are usually generalized tonic-clonic. 4,5 CADASIL EEG Epilepsy Migraine Neurogenetics Stoke A B S T R A C T Recurrent transient ischemic attacks, migraine and dementia represent the principal symptoms of cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL). During the course of the disease, about 10% of patients may experience epileptic seizures, mainly related to the presence of an ischemic stroke.…”

Section: Discussionmentioning

confidence: 99%

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Focal epilepsy as first symptom in CADASIL

Velizarova

Mourand

Serafini

et al. 2011

Seizure

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Recurrent transient ischemic attacks, migraine and dementia represent the principal symptoms of cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL). During the course of the disease, about 10% of patients may experience epileptic seizures, mainly related to the presence of an ischemic stroke. We present a 30-year-old woman with new-onset focal epilepsy leading to the diagnosis of CADASIL. The neuropsychological testing revealed no cognitive impairment. Interictal EEG demonstrated spikes and polyspikes with low amplitude over the right occipital region during NREM sleep. MRI showed white-matter hyperintensities on both hemispheres with confluent lesions at the right parieto-occipital junction, with juxtacortical components. Like in multiple sclerosis, we can suppose that this type of white matter lesions, close to the cortex, may be causative of seizures.

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Section: Discussionmentioning

confidence: 99%

“…2 Rarely, seizures may precede the ischemic events and the cognitive impairment. 3,4 We present the case of new-onset focal epilepsy leading to the diagnosis of CADASIL.…”

Section: Introductionmentioning

confidence: 99%

Focal epilepsy as first symptom in CADASIL

Velizarova

Mourand

Serafini

et al. 2011

Seizure

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“…From the present study, one can conclude that seizures in patients with a lacunar stroke/infarct are more closely linked to the cognitive status than to the cerebrovascular disease. It is unclear whether this is the same in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy who develop epilepsy [22]. …”

Section: Discussionmentioning

confidence: 99%

Cognitive Impairment and Seizures in Patients with Lacunar Strokes

Reuck

Maele²

2008

Eur Neurol

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Background: Lacunar infarcts and white matter changes have been linked to cognitive impairment. Patients with lacunar strokes can also develop seizures, although the relationship between the two remains unclear. The present study investigates whether seizures in patients with lacunar infarcts are related to the strokes or to an underlying neurodegenerative disorder leading to cognitive impairment. Methods: The demographic features, vascular risk factors and scores on the National Institutes of Health Stroke Scale (NIHSS) on admission for the stroke and on the modified Rankin scale on discharge, as well as on the Mini-Mental State Examination (MMSE), were determined in patients with a lacunar stroke. They were compared between 44 patients with and 248 without subsequent seizures. Results: Patients with seizures had a lower main NIHSS score (p = 0.00133) and a more severe MMSE score (p < 0.001). They remained significantly more dependent (p = 0.019) after hospital discharge. Smoking, as a vascular risk factor, appeared to occur less frequently in seizure patients (p = 0.039). On logistic regression analysis, only NIHSS and MMSE scores remained independent variables. Conclusions: Seizure occurrence in patients with a lacunar infarct is not related to the severity of the stroke but rather to the degree of cognitive impairment. The present study suggests that the seizures are not due to lacunar infarcts but are more probably the expression of an underlying neurodegenerative process that is also responsible for the mental deterioration.

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“…2 Epilepsy is rare in CADASIL, and it has only been reported in 10% of patients. 3,4 The postulated pathophysiology is cortical or juxtacortical involvement-that is, subcortical lacunar infarcts secondary to angiopathy at the gray-white matter junction ( Figure 1C). [3][4][5] In a case series with 102 patients with CADASIL, 10 patients had epileptic seizures and nine of them had a history of strokes.…”

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confidence: 99%

“…3,4 The postulated pathophysiology is cortical or juxtacortical involvement-that is, subcortical lacunar infarcts secondary to angiopathy at the gray-white matter junction ( Figure 1C). [3][4][5] In a case series with 102 patients with CADASIL, 10 patients had epileptic seizures and nine of them had a history of strokes. 3 Our patient was transitioned to oral levetiracetam at a dose of 500 mg twice daily with no further seizures.…”

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confidence: 99%