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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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38-year-old man who was treated 5 years previously for a right temporal anaplastic astrocytoma with chemo- and radiation therapy, now presenting with increasing epileptic activity and gradually progressive left sided hemiparesis and dysarthria. T2 weighted FLAIR MR images (a, b) showing gyral swelling and signal increase in the temporal lobe. T2 weighted MR image (c, d) showing gyral swelling and signal increase in the temporal lobe. A post-operative parenchymal defect is seen in the area of the previously treated astrocytoma. T1 weighted MR images after gadolinium administration (e, f) abnormal gyral and leptomeningeal enhancement in the temporal lobe and in the basal frontal areas. No focal enhancement in post-operative parenchymal defect. MR images 3 months later. g, h T1 weighted MR image after gadolinium administration showing persistent gyral abnormalities but disappearance of abnormal enhancement. MR images 6 months after treatment for recurrent anaplastic astrocytoma using chemotherapy. i T2 weighted FLAIR MR image and j T1 weighted MR image after gadolinium administration showing persistent gyral abnormalities but disappearance of abnormal enhancement. MR images 6 months after treatment for recurrent anaplastic astrocytoma using chemotherapy. k T2 weighted MR image and l T1 weighted MR image after gadolinium administration resolution of T2 abnormalities and disappearance of abnormal enhancement in the deep temporal area
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Fig4: 38-year-old man who was treated 5 years previously for a right temporal anaplastic astrocytoma with chemo- and radiation therapy, now presenting with increasing epileptic activity and gradually progressive left sided hemiparesis and dysarthria. T2 weighted FLAIR MR images (a, b) showing gyral swelling and signal increase in the temporal lobe. T2 weighted MR image (c, d) showing gyral swelling and signal increase in the temporal lobe. A post-operative parenchymal defect is seen in the area of the previously treated astrocytoma. T1 weighted MR images after gadolinium administration (e, f) abnormal gyral and leptomeningeal enhancement in the temporal lobe and in the basal frontal areas. No focal enhancement in post-operative parenchymal defect. MR images 3 months later. g, h T1 weighted MR image after gadolinium administration showing persistent gyral abnormalities but disappearance of abnormal enhancement. MR images 6 months after treatment for recurrent anaplastic astrocytoma using chemotherapy. i T2 weighted FLAIR MR image and j T1 weighted MR image after gadolinium administration showing persistent gyral abnormalities but disappearance of abnormal enhancement. MR images 6 months after treatment for recurrent anaplastic astrocytoma using chemotherapy. k T2 weighted MR image and l T1 weighted MR image after gadolinium administration resolution of T2 abnormalities and disappearance of abnormal enhancement in the deep temporal area

Mentions: In 1999 at the age of 34 years, this man suffered from an epileptic seizure caused by a right temporal anaplastic astrocytoma. The tumour was resected and he subsequently had focal brain radiation (50 Gy in 20 fractions). In the following 5 years he had infrequent focal seizures of the left arm but no tumour recurrence was observed on repeated MRI. Five years after initial treatment, however, he developed a progressive clumsy left hand, left-sided facial paresis and dysarthria in combination with an increase of focal seizures. An EEG showed nearly continuous epileptic discharges diffusely over the right hemisphere. He was treated with intravenous clonazepam and valproate. The MRI demonstrated no tumour recurrence but a gyriform enhancement and swelling of the right parieto-temporal cortex (Fig. 4a–f).Fig. 4


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)

38-year-old man who was treated 5 years previously for a right temporal anaplastic astrocytoma with chemo- and radiation therapy, now presenting with increasing epileptic activity and gradually progressive left sided hemiparesis and dysarthria. T2 weighted FLAIR MR images (a, b) showing gyral swelling and signal increase in the temporal lobe. T2 weighted MR image (c, d) showing gyral swelling and signal increase in the temporal lobe. A post-operative parenchymal defect is seen in the area of the previously treated astrocytoma. T1 weighted MR images after gadolinium administration (e, f) abnormal gyral and leptomeningeal enhancement in the temporal lobe and in the basal frontal areas. No focal enhancement in post-operative parenchymal defect. MR images 3 months later. g, h T1 weighted MR image after gadolinium administration showing persistent gyral abnormalities but disappearance of abnormal enhancement. MR images 6 months after treatment for recurrent anaplastic astrocytoma using chemotherapy. i T2 weighted FLAIR MR image and j T1 weighted MR image after gadolinium administration showing persistent gyral abnormalities but disappearance of abnormal enhancement. MR images 6 months after treatment for recurrent anaplastic astrocytoma using chemotherapy. k T2 weighted MR image and l T1 weighted MR image after gadolinium administration resolution of T2 abnormalities and disappearance of abnormal enhancement in the deep temporal area
© Copyright Policy
Related In: Results  -  Collection

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Fig4: 38-year-old man who was treated 5 years previously for a right temporal anaplastic astrocytoma with chemo- and radiation therapy, now presenting with increasing epileptic activity and gradually progressive left sided hemiparesis and dysarthria. T2 weighted FLAIR MR images (a, b) showing gyral swelling and signal increase in the temporal lobe. T2 weighted MR image (c, d) showing gyral swelling and signal increase in the temporal lobe. A post-operative parenchymal defect is seen in the area of the previously treated astrocytoma. T1 weighted MR images after gadolinium administration (e, f) abnormal gyral and leptomeningeal enhancement in the temporal lobe and in the basal frontal areas. No focal enhancement in post-operative parenchymal defect. MR images 3 months later. g, h T1 weighted MR image after gadolinium administration showing persistent gyral abnormalities but disappearance of abnormal enhancement. MR images 6 months after treatment for recurrent anaplastic astrocytoma using chemotherapy. i T2 weighted FLAIR MR image and j T1 weighted MR image after gadolinium administration showing persistent gyral abnormalities but disappearance of abnormal enhancement. MR images 6 months after treatment for recurrent anaplastic astrocytoma using chemotherapy. k T2 weighted MR image and l T1 weighted MR image after gadolinium administration resolution of T2 abnormalities and disappearance of abnormal enhancement in the deep temporal area
Mentions: In 1999 at the age of 34 years, this man suffered from an epileptic seizure caused by a right temporal anaplastic astrocytoma. The tumour was resected and he subsequently had focal brain radiation (50 Gy in 20 fractions). In the following 5 years he had infrequent focal seizures of the left arm but no tumour recurrence was observed on repeated MRI. Five years after initial treatment, however, he developed a progressive clumsy left hand, left-sided facial paresis and dysarthria in combination with an increase of focal seizures. An EEG showed nearly continuous epileptic discharges diffusely over the right hemisphere. He was treated with intravenous clonazepam and valproate. The MRI demonstrated no tumour recurrence but a gyriform enhancement and swelling of the right parieto-temporal cortex (Fig. 4a–f).Fig. 4

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