Abstract Details

Title Pediatric SMART Syndrome in a 10-year-old boy: A Case-Report

Topic Child Neurology and Developmental Neurology

Presentation(s) P5 - Poster Session 5 (8:00 AM-9:00 AM)

Poster/Presentation
Number 6-005

Objective Stroke like migraine attacks after radiation therapy (SMART) syndrome is a rare complication in the pediatric population post cranial radiation therapy. It commonly occurs on average 20 years post radiation. We review a unique case of SMART syndrome in a 10-year-old male occurring 17 months after radiation.

Background 10-year-old with FOXR2-activated CNS neuroblastoma in the left temporal lobe with history of subtotal resection, craniospinal radiation (total dose of 55.8 Gy) and chemotherapy with no residual weakness after surgery. 17 months after initial tumor-radiation, he began experiencing left sided headaches, unresponsiveness, right upper extremity (RUE) weakness, sensory loss and slurred speech. CT brain was normal and MRI brain with and without contrast did not show any acute findings. Symptoms initially resolved after 7 hours. He re-presented the next day with recurrence of headache, photophobia and phonophobia. Examination revealed RUE weakness, right facial droop and eyes deviated to the left. EEG showed left temporal slowing, without epileptiform activity and no seizures. Repeat MRI brain with and without contrast showed leptomeningeal vessel prominence with no diffusion restriction. CSF analysis was not indicative of tumor recurrence or CNS infection.

Design/Methods Not Applicable

Results Not Applicable

Conclusions

Neurotoxicity post cranial radiation can occur secondary to injury to the intracranial vascular endothelium, neuroglial cells and precursor cells. SMART syndrome is a rare, delayed complication of cranial radiation therapy above 50 Gy. It has a male predominance, and symptoms usually develop 20 years after radiation therapy. Clinical features include migrainous headache, aphasia, hemiparesis, hemisensory changes and seizures. Unilateral gyral gadolinium enhancement on MRI brain sparing white matter may be present. Most symptoms usually resolve within hours to weeks with recovery usually occurring within 1.5-2.5 months. There may be persistent neurological deficits such as weakness and aphasia.

Treatment is generally symptom management and preventative measures. Aspirin is sometimes considered for residual deficits.

Authors/Disclosures
Janine E. Taitt, MBBS (Medical College of Wisconsin and Affiliated Hospitals) PRESENTER	Dr. Taitt has nothing to disclose.
Avantika Singh, MD (Medical College of Wisconsin)	Dr. Singh has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ceribell.
Namrata Patel, MD (University of Washington)	No disclosure on file
Nancy E. Bass, MD (Children's Wisconsin)	Dr. Bass has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elseviert.

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