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Título

FOCAL AWARE STATUS EPILEPTICUS IN PATIENT WITH STROKE-LIKE MIGRAINE ATTACKS AFTER RADIATION THERAPY (SMART) SYNDROME: A CASE REPORT

RESUMO

CASE PRESENTATION: A 56-year-old woman was admitted with a three-day history of complex visual hallucinations.
She had previous history of right temporo-occipital grade III ependymoma, diagnosed 10 years before, and treated with surgery and radiotherapy. Repeated neuroimaging exams showed no signs of tumor recurrence. She reported neither epileptic seizures nor use of antiseizure medications (ASM).
On admission, she reported recurrent episodes of complex visual hallucinations, described as altered depth perception and image distortion, associated with left lateral gaze deviation, without impairment of consciousness. Episodes occurred several times a day and lasted up to an hour.
On admission, neurological examination revealed left homonymous hemianopsia, with no other abnormalities.
Magnetic resonance imaging (MRI) of the head demonstrated cortical gyriform enhancement surrounding the surgical site in right temporo-parieto-occipital region, virtually pathognomonic of stroke-like migraine attacks after radiation therapy (SMART) syndrome.
Continuous electroencephalogram (cEEG) monitoring showed repeated focal epileptic seizures, with ictal onset over posterior regions of the right hemisphere. Clinically, the patient referred visual symptoms; in some of the seizures, she had eye and head deviation to the left. Seizures lasted up to 77 minutes, leading to diagnosis of focal status epilepticus (SE) without impairment of consciousness. She was treated with intravenous lacosamide plus oral clobazam, which led to seizure cessation.
MRI was repeated one month later and showed complete resolution of previous findings. A new EEG demonstrated absence of epileptiform abnormalities.
DISCUSSION: SMART syndrome is a rare reversible syndrome that occurs as a late effect of brain radiotherapy. Classical features include migraine-like headaches, focal neurologic signs and/or seizures, associated with typical imaging findings. Episodes last days to weeks and usually occur years or decades after radiotherapy.
We report a case of a patient with SMART syndrome who presented with focal aware SE with visual symptoms, which is an atypical presentation of this syndrome. After treatment with ASM symptoms regressed, as well as MRI abnormalities, demonstrating the reversible character of the syndrome.
FINAL REMARKS: Albeit rare, SMART syndrome should be considered in patients with history of brain radiotherapy and typical clinical and neuroimaging findings.

Palavras Chave

SMART syndrome; status epilepticus; radiotherapy

Área

Epilepsia