Moises Dominguez1, Rachna Malani2. 1. Department of Neurology, Weill Cornell Medicine & New York-Presbyterian, New York, NY, USA. mod9040@nyp.org. 2. Department of Neurology, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
Abstract
PURPOSE OF REVIEW: SMART syndrome is a delayed complication of cranial irradiation that can be misconstrued as tumor recurrence or some other intracranial neurological disease. Recognition of this clinical syndrome is imperative as it can obviate the need for invasive diagnostic testing and can provide reassurance to both the patient and their loved ones. RECENT FINDINGS: SMART syndrome is generally considered a reversible clinical syndrome; however, neurological deficits may become permanent. Pathophysiology of SMART syndrome may involve cerebrovascular autoregulation impairment, neuronal dysfunction leading to trigeminovascular system impairment and/or cortical spreading depression, and seizures. In addition to MRI brain with gadolinium, other imaging modalities, such as CT perfusion, MR perfusion, MR spectroscopy, and FDG PET/CT, aid in arriving to the diagnosis sooner. Patients should also undergo electroencephalogram in order to promptly identify and treat seizures. There are currently no clear guidelines on how to effectively treat SMART syndrome, but treatment may involve anti-seizure medication, anti-hypertensives, anti-platelet, and steroid therapy. This review provides a comprehensive understanding of the clinical characteristics of SMART syndrome from presentation to diagnostic evaluation. We also discuss radiographic features and treatment strategies for this rare disease. With increased radiotherapy utilization, prompt clinical recognition of SMART syndrome and further development of a comprehensive diagnostic approach to SMART syndrome utilizing newer radiographic modalities as well as treatment algorithms to effectively treat this clinical condition will be imperative.
PURPOSE OF REVIEW: SMART syndrome is a delayed complication of cranial irradiation that can be misconstrued as tumor recurrence or some other intracranial neurological disease. Recognition of this clinical syndrome is imperative as it can obviate the need for invasive diagnostic testing and can provide reassurance to both the patient and their loved ones. RECENT FINDINGS: SMART syndrome is generally considered a reversible clinical syndrome; however, neurological deficits may become permanent. Pathophysiology of SMART syndrome may involve cerebrovascular autoregulation impairment, neuronal dysfunction leading to trigeminovascular system impairment and/or cortical spreading depression, and seizures. In addition to MRI brain with gadolinium, other imaging modalities, such as CT perfusion, MR perfusion, MR spectroscopy, and FDG PET/CT, aid in arriving to the diagnosis sooner. Patients should also undergo electroencephalogram in order to promptly identify and treat seizures. There are currently no clear guidelines on how to effectively treat SMART syndrome, but treatment may involve anti-seizure medication, anti-hypertensives, anti-platelet, and steroid therapy. This review provides a comprehensive understanding of the clinical characteristics of SMART syndrome from presentation to diagnostic evaluation. We also discuss radiographic features and treatment strategies for this rare disease. With increased radiotherapy utilization, prompt clinical recognition of SMART syndrome and further development of a comprehensive diagnostic approach to SMART syndrome utilizing newer radiographic modalities as well as treatment algorithms to effectively treat this clinical condition will be imperative.
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