Abstract Details

To describe a case of SMART syndrome presenting as non-convulsive status epilepticus.

Stroke-like Migraine Attacks After Radiation Therapy (SMART) syndrome is a rare, delayed complication of cranial irradiation characterized by stroke-like events, migrainous headaches, and seizures. Underlying pathophysiology is multifactorial and includes radiation-induced vascular injury, impaired autoregulation, and cortical hyperexcitability. 

A 41-year-old female with a past medical history of anaplastic oligoastrocytoma, World Heath Organization grade III status post resection, radiation-therapy (59.4Gy), and chemotherapy 15 years ago, depression and anxiety presented with expressive aphasia, headache with migrainous features and visual hallucinations. She had a prior hospitalization 3 years ago of expressive aphasia with negative MRI and EEG, that had resolved in few days.

On this admission, patient presented with similar presentation, of headache and expressive aphasia. Cerebrospinal fluid analysis did not reveal any evidence of infection or inflammation. Brain magnetic resonance image (MRI) demonstrated gyriform cortical and adjacent leptomeningeal enhancement in the left temporal, occipital and parietal regions.  Electroencephalogram revealed intractable left temporoparietal seizures, consistent with non-convulsive status epilepticus, refractory to multiple antiseizure medications.  She was treated with high dose steroids with marked clinical and electrographic improvement, with resolution of her symptoms.

This case illustrates SMART syndrome as a delayed radiation-induced complication that can masquerade as non-convulsive status epilepticus. This case is particularly notable because the patient presented with a constellation of symptoms including migrainous headaches, aphasia, and visual hallucination, that resembled her previous presentations associated with seizures and the initial presentation of her cancer. However, unlike prior episodes, the diagnostic workup, including brain MRI and EEG, was strongly suggestive of SMART syndrome, making this a unique case, and highlights the importance of diagnostic consideration in patient with prior cranial radiation.