cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy ( cadasil ) is a disease of the vascular smooth muscle typically caused by a mutation of the notch3 gene . cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy often presents with a history of migraine with aura and eventual manifestations of dementia with unrelenting , repeated cerebral vascular insults , . diagnoses have been made singularly or by a combination of genetic testing and skin biopsy . only 610% of patients with cadasil have been reported to develop seizures , and status epilepticus ( se ) is exceedingly rare , , . here , we describe a patient who presented three times in two years with se of an unknown cause , with eventual biopsy diagnosis of cadasil . an 80-year - old woman presented to our hospital three times in two years with a decreased level of consciousness and subtle intermittent right - sided upper extremity and facial twitching . her past medical history was only significant for well - controlled hypertension , with no history of seizures , migraine headaches , strokes , or overt dementia . electroencephalography revealed recurrent focal seizures with left hemispheric onset and evolution , fulfilling the criteria for focal se each time ( fig .  1 ) . brain mri repeatedly showed extensive abnormalities in the periventricular and deep white matter , subcortical white matter , and bilateral basal ganglia ( fig .  2 ) . cerebrospinal fluid studies consistently revealed culture - negative , predominantly neutrophilic pleocytosis ( range : 27 to 92 ) . otherwise , detailed and repetitive investigations failed to reveal any clear etiology ( metabolic , infectious , inflammatory , paraneoplastic , etc . ) for the patient 's condition . all three admissions required sedation with midazolam to control seizure activity in addition to high doses of multiple antiepileptic drugs . the patient made a slow but full recovery after the first and second episodes , following five - week and four - week hospital stays , respectively . the third episode occurred despite the regular intake of antiepileptic drugs ( valproate and levetiracetam ) , and the patient remained minimally responsive afterwards . the patient did eventually require tracheostomy and peg tube placement and was discharged to a nursing home . months after discharge , she is still in a nursing home with unimproved mental status . on the third admission , skin biopsy was obtained and returned positive ; electron microscopic images showed gaps and discontinuities in the perivascular smooth muscle layer with numerous deposits of granular osmiophilic material ( fig .  3 ) . to our knowledge , this is the first reported case of cadasil presenting as nonconvulsive se , without the other typical manifestations . seizures are seen in a small percentage of patients with cadasil , but they often follow a stroke and are not typically the first manifestation of the disease , . status epilepticus is also very rare in this disease ; we found only one published case report of eeg - proven nonconvulsive se in a patient with established diagnosis of cadasil . described six patients with cadasil with a reversible acute encephalopathy ; four of them had seizures during their hospitalization . however , their eeg data did not show ictal seizure activity , although the recordings were apparently brief and not continuous . 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 . manifestations of cadasil usually start in early or mid - adulthood , with gradual accumulation of deficits . however , the phenotypic and prognostic variability has already been emphasized with 14% of biopsy - proven patients over 60  years of age having no disability at all . the consistent csf pleocytosis in this case is unusual for cadasil , but the co - occurrence of se may be a confounding factor . 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 , regardless of a subject 's advanced age .
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 610% 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 .