we present a case of status epilepticus due to cerebral amyloid angiopathy - related inflammation , successfully treated with immunomodulation .
a 52-year - old man with a history of hypertension and alcohol abuse , presented in january of 2015 with new - onset seizures .
the patient 's family first noted new - onset irritability , paranoia , and confusion that were thought to be atypical for him .
two days later , the patient noted a 45-minute episode of elementary visual hallucinations consisting of bright colors and stars in his right visual field with an associated expressive aphasia .
he reported that he could understand questions and formulate responses in his head but not actually speak .
he was brought to an outside hospital where he had a witnessed seizure during which he had deviation of his head to the left , evolving to a generalized tonic - convulsion seizure lasting 40  seconds , with associated tongue biting and postictal confusion but no incontinence .
his exam was notable only for a right inferior quadrant visual field defect , which was homonymous but more profound in the right eye .
a lumbar puncture was performed , and cerebrospinal fluid was acellular with normal protein and glucose , with no oligoclonal bands , and with negative cytology and flow cytometry .
routine electroencephalograms ( eegs ) showed spike  wave discharges with several electrographic seizures in the left occipital region . magnetic resonance imaging ( mri )
revealed left occipital white matter and cortical t2 hyperintensity with mild focal mass effect , without enhancement but with petechial hemorrhages on gradient echo ( gre ) imaging and with a second smaller focus of t2 hyperintensity in the left anterior insula ( fig .
he was loaded with levetiracetam and was discharged from the hospital . upon initial formulation ,
the patient 's history , exam , and studies were thought to be most consistent with a multifocal glioma , less likely an infectious or inflammatory process , and even less likely an old stroke .
his levetiracetam dose was increased because of a recurrent episode of visual symptoms and aphasia , and he was scheduled for a biopsy of the left occipital lesion .
however , a repeat mri performed in march 2015 in preparation for the biopsy showed significant improvement of the left occipital and left anterior insular lesions ( fig .
these shifting lesions were thought to be more consistent with a demyelinating process such as acute disseminated encephalomyelitis ( adem ) .
the brain biopsy was aborted , and the patient was instead admitted for an expedited inflammatory workup .
a repeat lumbar puncture was again unrevealing ( including negative oligoclonal bands , cytology , flow cytometry , and igh gene rearrangement ) . a computed tomography ( ct ) scan of the chest , abdomen , and pelvis revealed multiple 1- to 2-cm hepatic lesions with peripheral nodular enhancements , which were then confirmed as hepatic hemangiomas on abdominal mri .
magnetic resonance imaging of the cervical and thoracic spine showed only mild degenerative disc disease , and ct angiogram of the head and neck was unremarkable .
the patient was again discharged home , but follow - up mri in june 2015 showed worsening of the left occipital t2 hyperintensity ( fig .
2c ) and new left frontal t2 hyperintensities , as well as new enhancement of the lesion in the right anterior temporal lobe ( not shown ) .
the biopsy showed reactive changes and thickened blood vessels with beta - amyloid deposition in vessel walls ( fig .
he was then brought to the emergency department for a sudden clinical deterioration with global aphasia and confusion .
he was unable to name objects , repeat phrases , or follow commands ; made frequent paraphasic errors ; and was noted to have decreased blink to threat on the right .
an eeg was obtained , revealing continuous lateralized periodic discharges with overlying fast activity over the left posterior quadrant ( fig .
4b ) , consisting of 9-hz rhythmic sharp waves , without clinical return to baseline between seizures .
he was therefore determined to be in nonconvulsive status epilepticus ( ncse ) and was admitted .
over the course of approximately 24  h , he was started on lacosamide , phenytoin , and valproic acid in addition to levetiracetam , and ncse was aborted .
g 's clinical status improved only slightly with the arrest of seizures , and he continued to demonstrate significant global aphasia and confusion .
positron emission tomography ( pet ) scan of the brain and whole body revealed increased fluorodeoxyglucose ( fdg ) uptake in the left occipital and left temporal regions , consistent with the known lesions on mri .
these areas of increased fdg uptake were thought to be most likely due to an inflammatory process and less likely due to recent seizure activity . at this point ,
the diagnosis of cerebral amyloid angiopathy - related inflammation was advanced , and he was started on a 5-day course of methylprednisolone , 1000  mg daily as a diagnostic and therapeutic maneuver .
clinically , his speech difficulties and confusion resolved almost entirely , and he was able to name , repeat , and follow commands normally .
he was able to discontinue phenytoin and valproic acid without recurrence of seizures ( either clinically or on eeg ) .
following pulse - dose steroids , he was discharged on a slow taper of oral prednisone .
repeat imaging in september of 2015 showed marked improvement of all t2 hyperintense lesions and complete resolution of all enhancement ( fig .
cerebral amyloid angiopathy ( caa ) consists of -amyloid deposition in small and medium arteries of the brain and leptomeninges .
it is found in 2357% of the asymptomatic elderly population and at increased rates in those with dementia and intracerebral hemorrhage .
cerebral amyloid angiopathy can more rarely cause an inflammatory reaction , known as caa - related inflammation ( caa - i ) .
this has historically been described as primary angiitis of the central nervous system associated with caa , cerebral amyloid angiitis , and cerebral amyloid inflammatory vasculopathy , , .
cerebral amyloid angiopathy - related inflammation presents with subacute cognitive decline , headaches , and seizures rather than the chronic dementia or hemorrhagic strokes classically associated with cerebral amyloid angiopathy .
the mean age at onset is approximately 68  years , significantly younger than for hemorrhagic caa .
magnetic resonance imaging findings in caa - i include shifting multifocal white matter t2 hyperintensities abnormalities colocalized with petechial hemorrhages on swi .
cerebrospinal fluid is typically bland , though protein may be elevated , and more rarely , pleiocytosis has been observed .
the apoe 4/4 genotype is present at increased rates  71% of patients in one case series .
histopathologic findings include amyloid deposition within vessel walls and perivascular , transmural , or intramural inflammation , including perivascular multinucleated giant cells .
several published cases and case series describe treatment with immunosuppressive therapy , including corticosteroids with or without additional immunosuppressive therapy such as methotrexate , mycophenolate mofetil , or most commonly cyclophosphamide .
thirty - eight of 53 published cases showed improvement with immunosuppressive treatment , as did mr .
our patient 's case was notable for a typically subacute fluctuating clinical course and multifocal fluctuating radiographic findings , classically bland csf , and a biopsy demonstrating amyloid deposition within artery walls .
inflammatory infiltrate was not seen on biopsy , and this was attributed to the biopsied lesion being an older ,  burned out 
cerebral amyloid angiopathy - related inflammation is an unusual but highly treatable cause of new - onset seizures in the middle - aged and elderly population and should be considered in the differential diagnosis of new - onset seizures after the age of 40 , associated with fluctuating multifocal t2 hyperintensities and petechial hemorrhages on mri .
 this report discusses a case of nonconvulsive status epilepticus , caused by cerebral amyloid angiopathy - related inflammation . 
 brain biopsy demonstrated cerebral amyloid angiopathy , with clinical and radiographic features indicative of a fluctuating inflammatory process . 
 immunomodulatory treatment with pulse steroids resulted in rapid and dramatic clinical and radiographic improvement . 
 cerebral amyloid angiopathy - related inflammation should be considered in the differential diagnosis of new - onset seizures after the age of 40 , when associated with fluctuating multifocal t2 hyperintensities and petechial hemorrhages on gradient echo ( gre ) or susceptibility - weighted ( swi ) mri sequences . 
Case report
Discussion