Surgically Induced SMART Syndrome: Case Report and Review of the Literature

doi:10.1016/j.wneu.2013.01.028

World Neurosurgery

Volume 82, Issues 1–2, July–August 2014, Pages 240.e7-240.e12

https://doi.org/10.1016/j.wneu.2013.01.028 Get rights and content

Background

Strokelike migraine attack after radiation therapy is a recently described clinical entity characterized by transient hemispheric dysfunction manifesting as, but not limited to, visuospatial deficits, confusion, hemisensory deficits, hemiparesis, aphasia, seizures, and, most prominently, headache in patients with a history of remote external beam radiation therapy to the brain. The radiographic hallmark on magnetic resonance imaging is the presence of transient, diffuse, unilateral gadolinium enhancement of the cortex with white matter sparing, usually corresponding to the previous radiation field.

Case Description

We present a case of strokelike migraine attacks after radiation therapy syndrome diagnosed immediately following a craniotomy and temporal lobectomy for recurrent metastatic tumor resection after prior gamma knife radiosurgery and whole-brain radiation therapy.

Conclusion

SMART syndrome should be considered in the differential diagnosis of postsurgical patients with remote history of cranial irradiation and significant, new transient neurologic deficits not explainable by any other mechanism. It is possible that manipulation of the trigeminal ganglion, or the dura of the Meckel cave, contributed to triggering the manifestations of this syndrome in our patient during the immediate postoperative period.

Section snippets

Clinical Presentation

A 56-year-old right-handed woman presented in April 2005 with a history of stage 4 infiltrating adenocarcinoma of the right breast. At the time of diagnosis she was also discovered to have lung metastases. Following lumpectomy, she was treated with paclitaxel (Taxol), followed by six cycles of docetaxel (Taxotere) and trastuzumab (Herceptin). She was then maintained on trastuzumab and letrozole (Femara). She responded well and her malignancy was considered to be in remission by February 2006.

Discussion

The SMART syndrome was first described by Bartleson et al. in two patients with prior history of radiation therapy (RT) (2). They later proposed the diagnostic criteria listed in Table 1 (3). To date, there have been 25 suspected cases described in the literature (articles restricted to English); 8 women and 17 men (Tables 2 and 3) 2, 3, 4, 5, 11, 16, 18, 19, 21. None of them have been temporarily related or attributed to recent intracranial surgery, although many cases have history of prior

Conclusion

This is the first report of a case of SMART syndrome occurring in the acute postoperative period after an uncomplicated neurosurgical procedure. SMART syndrome should be considered in the differential diagnosis of postsurgical patients with remote history of cranial irradiation and significant, new transient neurologic deficits not explainable by any other mechanism. It is possible that manipulation of the trigeminal ganglion, or the dura of the Meckel cave, contributed to triggering the

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Cited by (17)

SMART syndrome
2021, Neurologia
Uncommon radiation-induced neurological syndromes
2020, Neuro-Oncology for the Clinical Neurologist
Cranial irradiation is used in settings ranging from definitive treatment of primary brain and head and neck cancers to palliation of metastatic disease and central nervous system prophylaxis, as a single modality or in combination with surgery or systemic therapy. A comprehensive understanding of potential complications of cranial irradiation is necessary for optimal multidisciplinary management of oncology patients. Common side-effect considerations include neurocognitive and inflammatory effects, as well as damage to critical brain structures in the irradiated field, and are discussed elsewhere in this text. Here, we review rare complications of cranial irradiation including radiation myelitis, stroke-like migraine attacks after radiation therapy (SMART syndrome), hypothalamic dysfunction, post-radiation vasculopathy and stroke, and radiation-induced secondary malignancies including meningioma and glioma. Along with case presentations, we review the diagnosis, impact of radiation dose and field, and multidisciplinary management for these complications. Efforts to optimize radiation delivery to minimize these complications include modern radiation therapy techniques, such as intensity-modulated radiation therapy, stereotactic radiosurgery, and proton beam therapy, which allow for highly conformal dose distributions and relative sparing of critical normal tissues and have been adopted in the treatment of central nervous system malignancies. In the rapidly evolving oncology landscape, multidisciplinary management of patients is necessary for both cancer treatment and management of complications.
SMART (stroke-like migraine attacks after radiation therapy) syndrome responded to steroid pulse therapy: Report of a case and review of the literature
2018, eNeurologicalSci
This report presents a case of stroke-like migraine attacks after radiation therapy (SMART) syndrome in a 31-year-old man in whom symptoms and radiological findings resolved with steroid pulsed therapy and reviews the literatures with special emphasis on the use of steroids against SMART syndrome. The patient had a past history of left temporal anaplastic astrocytoma and was treated with surgery followed by local 72 Gy radiation therapy and chemotherapy using Nimustine Hydrochloride. Four years after the surgery, he was suffering from subacute progressing symptoms of headache, right hemianopia, right hemiparesis and aphasia from 2 to 4 days before admission to our hospital. At first he was diagnosed as symptomatic epilepsy but after extensive examination, the final diagnosis was SMART syndrome. His symptoms soon improved with steroid pulse therapy. In the literature, steroid pulse therapy is not necessarily a standard of care for SMART syndrome, but it seemed to decrease the need of biopsy. As the lesions of SMART syndrome require differential diagnosis from recurrences, biopsy was performed in some cases. However, lack of benefit and possible detriment is reported with biopsy of SMART lesions. Through this experience we suggest that steroid pulse therapy may provide speedy recovery from symptoms, and it should be considered before other invasive investigations or treatments.
Stroke-like migraine attacks after radiation therapy syndrome: a case report and literature review
2017, Radiology Case Reports
Citation Excerpt :
For long-term prophylaxis of episodes of SMART syndrome, aspirin and verapamil have been used to good effect [3,4,10–12]. The efficacy of steroids has shown mixed results [5,13,14]. Although initially described as a temporary or reversible condition, a recent case series [5] demonstrated that reversibility is not universal, with nearly half of cases showed incomplete resolution.
Stroke-like migraine attacks after radiation therapy syndrome is a late complication of cranial radiation. It typically presents as reversible, unilateral cortical signs and symptoms such as confusion, hemiparesis, seizures, and headaches. Magnetic resonance imaging is also required for diagnosis, demonstrating cortical linear gadolinium enhancement. Typically, these magnetic resonance imaging findings resolve as patients experience partial or complete improvement in their symptoms and signs after a few weeks. Although a very rare condition, it is becoming increasingly observed as survival rates from brain tumors improve. In this report, we describe a typical case of stroke-like migraine attacks after radiation therapy syndrome and present a review of the literature.
Strategies to improve the quality of survival for childhood brain tumour survivors
2015, European Journal of Paediatric Neurology
Citation Excerpt :
CRT-related LE range from transient and treatable to permanent and progressive impairments.86 The risk of many LE is, as shown in Table 2,87–145 dependent of CRT-region, -dose, -technique and multiple covariates. The role of the CRT-dose-volume-ratio still needs validation for already examined entities such as ependymoma and LGG70,146 and also further analysis for other CBT-subgroups and different CRT techniques.
Tumours of the central nervous system (CNS) are the most frequent solid tumours and the second most frequent type of cancer in children and adolescents. Overall survival has continuously improved in Germany, since an increasing number of patients have been treated according to standardised, multicentre, multimodal treatment recommendations, trials of the German Paediatric Brain Tumour Consortium (HIT-Network) or the International Society of Paediatric Oncology-Europe (SIOP-E) during the last decades. Today, two out of three patients survive. At least 8000 long-term childhood brain tumour survivors (CBTS) are currently living in Germany. They face lifelong disease- and treatment-related late effects (LE) and associated socioeconomic problems more than many other childhood cancer survivors (CCS).
We review the LE and resulting special needs of this particular group of CCS.
Despite their increasing relevance for future treatment optimisation, neither the diversity of chronic and cumulative LE nor their pertinent risk factors and subsequent impact on quality of survival have yet been comprehensively addressed for CBTS treated according to HIT- or SIOP-E-protocols. Evidence-based information to empower survivors and stakeholders, as well as medical expertise to manage their individual health care, psychosocial and educational/vocational needs must still be generated and established.
The establishment of a long-term research- and care network in Germany shall contribute to a European platform, that aims at optimising CBTSs' transition into adulthood as resilient individuals with high quality of survival including optimal levels of activity, participation and acceptance by society.
Stroke-Like Migraine Attacks After Radiation Therapy (SMART) Syndrome: A Comprehensive Review
2021, Current Pain and Headache Reports

View all citing articles on Scopus

: Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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Peer-Review Short ReportSurgically Induced SMART Syndrome: Case Report and Review of the Literature

Background

Case Description

Conclusion

Section snippets

Clinical Presentation

Discussion

Conclusion

J Clin Neurosci

Curr Opin Pharmacol

World Neurosurg

The International Classification of Headache Disorders: 2nd edition

Cephalalgia

Reversible, strokelike migraine attacks in patients with previous radiation therapy

Neuro-oncology

SMART: stroke-like migraine attacks after radiation therapy

Cephalalgia

Neurocognitive recovery in SMART syndrome: a case report

Cephalalgia

Normal cerebrovascular reactivity in stroke-like migraine attacks after radiation therapy syndrome

Clin Nucl Med

Migraine-associated seizure: a case of reversible MRI abnormalities and persistent nondominant hemisphere syndrome

Headache

Perioperative posterior reversible encephalopathy syndrome in 2 pediatric neurosurgery patients with brainstem ependymoma

J Neurosurg Pediatr

Posterior reversible encephalopathy syndrome associated with hemodynamic augmentation in aneurysmal subarachnoid hemorrhage

Neurocrit Care

SMART syndrome: a late reversible complication after radiation therapy for brain tumours

J Neurol

Peer-Review Short Report
Surgically Induced SMART Syndrome: Case Report and Review of the Literature