School of Medicine and Health Sciences Poster Presentations

Title

The Elusive Diagnosis of SMART Syndrome Demystified

Poster Number

254

Document Type

Poster

Status

Postdoc

Abstract Category

Health Sciences

Keywords

SMART Syndrome, MRI, stroke, recurrent tumor, restricted diffusion, gyral enhancement

Publication Date

Spring 2018

Abstract

A 32-year-old woman presented to the Emergency Department (ED) with severe headaches, acute neurologic changes and altered mental status. Her past medical history was significant for medulloblastoma treated with radiation and chemotherapy 24 years prior to this admission. Admitting diagnoses included recurrent tumor and stroke. Brain MRI showed areas of restricted diffusion, sulcal effacement and gyral +/- leptomeningeal enhancement in the left temporal lobe, and in short did not further narrow the differential diagnoses. The patient was treated symptomatically. The patient and her MRI findings subsequently returned to baseline. A couple of years later, she presented to the ED with similar clinical history and near identical MRI findings. The constellation of the recurring MRI findings led to the diagnosis of SMART syndrome (stroke-like migraine attacks after radiation therapy). The clinical presentation of patients with SMART syndrome can be diverse and difficult to hone, requiring MRI imaging for further characterization. The MRI imaging can also be confusing to those unfamiliar to this entity. Restricted diffusion, which is one of the MRI findings in these cases, is typically associated with irreversible cell death either caused by compromised blood supply or less likely infectious agent. On the other hand, gyriform pattern of enhancement, which is another MRI finding in these cases, is typically not seen in the setting of acute cell death. Instead it is typically associated with the subacute phase of cytotoxic edema. As such, the concomitant presence of these two findings can appear as a challenge to fundamental rules of MRI interpretation. In this presentation, the etiology of the MRI findings, their pathophysiology and how they reflect the SMART syndrome in a patient with previous cranial radiation treatment will be reviewed. This understanding will expedite diagnosis and treatment planning and decrease morbidity and mortality.

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The Elusive Diagnosis of SMART Syndrome Demystified

A 32-year-old woman presented to the Emergency Department (ED) with severe headaches, acute neurologic changes and altered mental status. Her past medical history was significant for medulloblastoma treated with radiation and chemotherapy 24 years prior to this admission. Admitting diagnoses included recurrent tumor and stroke. Brain MRI showed areas of restricted diffusion, sulcal effacement and gyral +/- leptomeningeal enhancement in the left temporal lobe, and in short did not further narrow the differential diagnoses. The patient was treated symptomatically. The patient and her MRI findings subsequently returned to baseline. A couple of years later, she presented to the ED with similar clinical history and near identical MRI findings. The constellation of the recurring MRI findings led to the diagnosis of SMART syndrome (stroke-like migraine attacks after radiation therapy). The clinical presentation of patients with SMART syndrome can be diverse and difficult to hone, requiring MRI imaging for further characterization. The MRI imaging can also be confusing to those unfamiliar to this entity. Restricted diffusion, which is one of the MRI findings in these cases, is typically associated with irreversible cell death either caused by compromised blood supply or less likely infectious agent. On the other hand, gyriform pattern of enhancement, which is another MRI finding in these cases, is typically not seen in the setting of acute cell death. Instead it is typically associated with the subacute phase of cytotoxic edema. As such, the concomitant presence of these two findings can appear as a challenge to fundamental rules of MRI interpretation. In this presentation, the etiology of the MRI findings, their pathophysiology and how they reflect the SMART syndrome in a patient with previous cranial radiation treatment will be reviewed. This understanding will expedite diagnosis and treatment planning and decrease morbidity and mortality.