Abstract Details

Title A "SMART" Diagnosis Indeed!

Topic Neuro-oncology

Presentation(s) P06 - (-)

Poster/Presentation
Number 148

Objective

Background BACKGROUND: A 67 year old male presented with a week of transient left sided headache, visual field loss and right sided hemiparesis. Medical history included a traumatic brain injury, epilepsy, hypertension, and a malignant occipital meningioma treated with radiation therapy. Neurological exam was significant for expressive aphasia, right homonymous hemianopia and right hemiparesis. MRI revealed a gadolinium enhancing lesion in left pareito-occipital region. EEG did not show any electrographic seizures. Lumbar puncture revealed markedly elevated protein 236 mg/dL. CSF cytology and flow cytometry was negative for malignant cells. He had similar past presentations with transient headache and hemiparesis and similar MRI and CSF findings that had resolved prior to this presentation. He was diagnosed with SMART syndrome.

Design/Methods

Results

Conclusions CONCLUSIONS: Stroke-like migraine attacks after radiation therapy is an uncommon complication of cerebral radiation therapy. Patients present years after radiation therapy with prolonged stroke-like episodes associated with neurological dysfunction, headache and are often preceded by an migraine-like aura and occasionally seizures. Symptoms may last from hours to months. Hallmark MRI findings include transient gyral enhancement and CSF protein may also be elevated. Pathogenesis is likely a post-radiation cortical vasculopathy. This precipitates the reversible neurological symptoms and the dramatic transient cortical enhancement of the affected hemisphere. The differential diagnosis includes tumor recurrence, post-ictal changes, peri-ictal pseudo progression and radiation necrosis. Treatment consists of symptomatic management of the headaches. Some authors suggest antiplatelet therapy as used in stroke patients. Recognition of this syndrome can help avoid invasive testing including a brain biopsy.

Authors/Disclosures
Shilpi Mittal, MD (Thomas Jefferson University) PRESENTER	Dr. Mittal has nothing to disclose.
Daniel O. Claassen, MD, FAAN (Vanderbilt University Medical Center)	Dr. Claassen has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Alterity. Dr. Claassen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Lundbeck. Dr. Claassen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Teva. Dr. Claassen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for AskBio. Dr. Claassen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for University of Michigan. Dr. Claassen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cognition Therapeutics . Dr. Claassen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amylyx. The institution of Dr. Claassen has received research support from NIH. The institution of Dr. Claassen has received research support from CHDI. The institution of Dr. Claassen has received research support from HDSA. The institution of Dr. Claassen has received research support from Department of Defense. The institution of Dr. Claassen has received research support from CHDI.
Matthew Imperioli, MD (Associated Neurologist)	Dr. Imperioli has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenix. Dr. Imperioli has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Ultragenyx Pharmaceuticals . Dr. Imperioli has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Law offices of Scott Doody.
Alexandra Flowers, MD (Hartford Hospital)	No disclosure on file

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