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

Kerklaan JP, Lycklama á Nijeholt GJ, Wiggenraad RG, Berghuis B, Postma TJ, Taphoorn MJ - J. Neurol. (2011)

Bottom Line: MRI demonstrated typical cortical swelling and contrast enhancement, primarily in the parieto-occipital region.On follow-up both clinical and MRI features improved spontaneously.The pathophysiology of the SMART syndrome is poorly understood but bears similarities with the posterior reversible encephalopathy syndrome (PRES).

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Medical Centre Haaglanden, Postbus 432, 2501 CK, The Hague, The Netherlands, J.Kerklaan@mchaaglanden.nl.

ABSTRACT
With intensified treatment leading to longer survival, complications of therapy for brain tumours are more frequently observed. Regarding radiation therapy, progressive and irreversible white matter disease with cognitive decline is most feared. We report on four patients with reversible clinical and radiological features occurring years after radiation for brain tumours, suggestive for the so called SMART syndrome (stroke-like migraine attacks after radiation therapy). All four patients (males, age 36-60 years) had been treated with focal brain radiation for a primary brain tumour or with whole-brain radiation therapy for brain metastases. Ranging from 2 to 10 years following radiation therapy patients presented with headache and focal neurological deficits, suggestive for tumour recurrence. Two patients also presented with focal seizures. MRI demonstrated typical cortical swelling and contrast enhancement, primarily in the parieto-occipital region. On follow-up both clinical and MRI features improved spontaneously. Three patients eventually proved to have tumour recurrence. The clinical and radiological picture of these patients is compatible with the SMART syndrome, a rare complication of radiation therapy which is probably under recognized in brain tumour patients. The pathophysiology of the SMART syndrome is poorly understood but bears similarities with the posterior reversible encephalopathy syndrome (PRES). These four cases underline that the SMART syndrome should be considered in patients formerly treated with radiation therapy for brain tumours, who present with new neurologic deficits. Before the diagnosis of SMART syndrome can be established other causes, such as local tumour recurrence, leptomeningeal disease or ischemic disease should be ruled out.

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60-year-old man who was treated 10 years previously for brain metastases of bronchial carcinoma with chemo- and radiation therapy, now presenting with gradually progressive headache and acute dysphasis. a T2 weighted MR image showing diffuse gyral swelling and signal increase in the left parietal lobe. b T1 weighted MR image after gadolinium administration showing abnormal regional leptomeningeal enhancement. c T2 weighted FLAIR MR image showing diffuse gyral swelling and signal increase in the left occipital lobe. d T1 weighted MR image after gadolinium administration showing abnormal leptomeningeal enhancement. MR images after 4 months. e T2 weighted MR image no residual signal abnormalities. f T1 weighted MR image after gadolinium administration showing no abnormal leptomeningeal enhancement
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Fig2: 60-year-old man who was treated 10 years previously for brain metastases of bronchial carcinoma with chemo- and radiation therapy, now presenting with gradually progressive headache and acute dysphasis. a T2 weighted MR image showing diffuse gyral swelling and signal increase in the left parietal lobe. b T1 weighted MR image after gadolinium administration showing abnormal regional leptomeningeal enhancement. c T2 weighted FLAIR MR image showing diffuse gyral swelling and signal increase in the left occipital lobe. d T1 weighted MR image after gadolinium administration showing abnormal leptomeningeal enhancement. MR images after 4 months. e T2 weighted MR image no residual signal abnormalities. f T1 weighted MR image after gadolinium administration showing no abnormal leptomeningeal enhancement

Mentions: In 1997 at age 50 years, this man was treated for small cell lung cancer and two brain metastases with systemic chemotherapy and whole-brain radiation (30 Gy in 10 fractions). Ten years later he developed slowly progressive headache, and a few weeks later he woke up with an expressive dysphasia. During hospitalization he developed a right sided hemiparesis with right hemianopia and he suffered from frequent focal epileptic seizures of the right arm and leg, for which he received valproate and levetiracetam. MRI showed focal gyral thickening and enhancement of the left parieto-occipital lobe where metastases had not been observed previously (Fig. 2a–d). Apart from diffuse leukoencephalopathy, presumably due to the earlier brain radiotherapy, there were no signs of recurrent brain metastases. Repeated CSF analysis showed a slightly elevated protein level, but no leptomeningeal metastases (Table 1). There were no clues for extracranial tumour recurrence. During the hospital stay he had a gradual clinical improvement and was discharged fully recovered. A follow-up MRI 3 months after discharge also showed complete resolution of the abnormalities in the left parieto-occipital cortex (Fig. 2e, f).Fig. 2


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

Kerklaan JP, Lycklama á Nijeholt GJ, Wiggenraad RG, Berghuis B, Postma TJ, Taphoorn MJ - J. Neurol. (2011)

60-year-old man who was treated 10 years previously for brain metastases of bronchial carcinoma with chemo- and radiation therapy, now presenting with gradually progressive headache and acute dysphasis. a T2 weighted MR image showing diffuse gyral swelling and signal increase in the left parietal lobe. b T1 weighted MR image after gadolinium administration showing abnormal regional leptomeningeal enhancement. c T2 weighted FLAIR MR image showing diffuse gyral swelling and signal increase in the left occipital lobe. d T1 weighted MR image after gadolinium administration showing abnormal leptomeningeal enhancement. MR images after 4 months. e T2 weighted MR image no residual signal abnormalities. f T1 weighted MR image after gadolinium administration showing no abnormal leptomeningeal enhancement
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3101343&req=5

Fig2: 60-year-old man who was treated 10 years previously for brain metastases of bronchial carcinoma with chemo- and radiation therapy, now presenting with gradually progressive headache and acute dysphasis. a T2 weighted MR image showing diffuse gyral swelling and signal increase in the left parietal lobe. b T1 weighted MR image after gadolinium administration showing abnormal regional leptomeningeal enhancement. c T2 weighted FLAIR MR image showing diffuse gyral swelling and signal increase in the left occipital lobe. d T1 weighted MR image after gadolinium administration showing abnormal leptomeningeal enhancement. MR images after 4 months. e T2 weighted MR image no residual signal abnormalities. f T1 weighted MR image after gadolinium administration showing no abnormal leptomeningeal enhancement
Mentions: In 1997 at age 50 years, this man was treated for small cell lung cancer and two brain metastases with systemic chemotherapy and whole-brain radiation (30 Gy in 10 fractions). Ten years later he developed slowly progressive headache, and a few weeks later he woke up with an expressive dysphasia. During hospitalization he developed a right sided hemiparesis with right hemianopia and he suffered from frequent focal epileptic seizures of the right arm and leg, for which he received valproate and levetiracetam. MRI showed focal gyral thickening and enhancement of the left parieto-occipital lobe where metastases had not been observed previously (Fig. 2a–d). Apart from diffuse leukoencephalopathy, presumably due to the earlier brain radiotherapy, there were no signs of recurrent brain metastases. Repeated CSF analysis showed a slightly elevated protein level, but no leptomeningeal metastases (Table 1). There were no clues for extracranial tumour recurrence. During the hospital stay he had a gradual clinical improvement and was discharged fully recovered. A follow-up MRI 3 months after discharge also showed complete resolution of the abnormalities in the left parieto-occipital cortex (Fig. 2e, f).Fig. 2

Bottom Line: MRI demonstrated typical cortical swelling and contrast enhancement, primarily in the parieto-occipital region.On follow-up both clinical and MRI features improved spontaneously.The pathophysiology of the SMART syndrome is poorly understood but bears similarities with the posterior reversible encephalopathy syndrome (PRES).

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Medical Centre Haaglanden, Postbus 432, 2501 CK, The Hague, The Netherlands, J.Kerklaan@mchaaglanden.nl.

ABSTRACT
With intensified treatment leading to longer survival, complications of therapy for brain tumours are more frequently observed. Regarding radiation therapy, progressive and irreversible white matter disease with cognitive decline is most feared. We report on four patients with reversible clinical and radiological features occurring years after radiation for brain tumours, suggestive for the so called SMART syndrome (stroke-like migraine attacks after radiation therapy). All four patients (males, age 36-60 years) had been treated with focal brain radiation for a primary brain tumour or with whole-brain radiation therapy for brain metastases. Ranging from 2 to 10 years following radiation therapy patients presented with headache and focal neurological deficits, suggestive for tumour recurrence. Two patients also presented with focal seizures. MRI demonstrated typical cortical swelling and contrast enhancement, primarily in the parieto-occipital region. On follow-up both clinical and MRI features improved spontaneously. Three patients eventually proved to have tumour recurrence. The clinical and radiological picture of these patients is compatible with the SMART syndrome, a rare complication of radiation therapy which is probably under recognized in brain tumour patients. The pathophysiology of the SMART syndrome is poorly understood but bears similarities with the posterior reversible encephalopathy syndrome (PRES). These four cases underline that the SMART syndrome should be considered in patients formerly treated with radiation therapy for brain tumours, who present with new neurologic deficits. Before the diagnosis of SMART syndrome can be established other causes, such as local tumour recurrence, leptomeningeal disease or ischemic disease should be ruled out.

Show MeSH
Related in: MedlinePlus