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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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47-year-old man who was treated 5 years previously for a left parietal oligodendrolioma with chemo- and radiation therapy, now presenting with right hemianopia and mild paresis of his right arm and leg. T2 weighted FLAIR MR images a showing gyral swelling and signal increase in the temporal lobe. b After gadolinium administration, diffuse gyral enhancement is seen in the occipital, temporal and insular lobe. Proton density (c) and T1 post gadolinium (d) MR imaging 8 days later showing normalization of signal intensity in the temporal lobe and marked reduction in abnormal gyral enhancement. After 3 years, the patient presented again with right sided paresis. MR imaging again shows signal increase in the temporal lobe on PD weighted images (e) while on T1 post gadolinium imaging (f) abnormal gyral enhancement is again visible. Like after the previous episode, the MR abnormalities disappeared (g, h)
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Fig3: 47-year-old man who was treated 5 years previously for a left parietal oligodendrolioma with chemo- and radiation therapy, now presenting with right hemianopia and mild paresis of his right arm and leg. T2 weighted FLAIR MR images a showing gyral swelling and signal increase in the temporal lobe. b After gadolinium administration, diffuse gyral enhancement is seen in the occipital, temporal and insular lobe. Proton density (c) and T1 post gadolinium (d) MR imaging 8 days later showing normalization of signal intensity in the temporal lobe and marked reduction in abnormal gyral enhancement. After 3 years, the patient presented again with right sided paresis. MR imaging again shows signal increase in the temporal lobe on PD weighted images (e) while on T1 post gadolinium imaging (f) abnormal gyral enhancement is again visible. Like after the previous episode, the MR abnormalities disappeared (g, h)

Mentions: Three years thereafter he was again taken into hospital with focal epileptic seizures of the right side of his face. He suffered from headache, progressive aphasia, right hemianopia and mild paresis of his right arm and leg. An MRI scan now showed diffuse cortical enhancement of the left occipital and temporal cortex (Fig. 3a, b). This enhancement disappeared on a subsequent MRI performed 8 days later (Fig. 3c, d). An EEG showed no epileptic activity. Clinically, he recovered substantially.Fig. 3


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)

47-year-old man who was treated 5 years previously for a left parietal oligodendrolioma with chemo- and radiation therapy, now presenting with right hemianopia and mild paresis of his right arm and leg. T2 weighted FLAIR MR images a showing gyral swelling and signal increase in the temporal lobe. b After gadolinium administration, diffuse gyral enhancement is seen in the occipital, temporal and insular lobe. Proton density (c) and T1 post gadolinium (d) MR imaging 8 days later showing normalization of signal intensity in the temporal lobe and marked reduction in abnormal gyral enhancement. After 3 years, the patient presented again with right sided paresis. MR imaging again shows signal increase in the temporal lobe on PD weighted images (e) while on T1 post gadolinium imaging (f) abnormal gyral enhancement is again visible. Like after the previous episode, the MR abnormalities disappeared (g, h)
© Copyright Policy
Related In: Results  -  Collection

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

Fig3: 47-year-old man who was treated 5 years previously for a left parietal oligodendrolioma with chemo- and radiation therapy, now presenting with right hemianopia and mild paresis of his right arm and leg. T2 weighted FLAIR MR images a showing gyral swelling and signal increase in the temporal lobe. b After gadolinium administration, diffuse gyral enhancement is seen in the occipital, temporal and insular lobe. Proton density (c) and T1 post gadolinium (d) MR imaging 8 days later showing normalization of signal intensity in the temporal lobe and marked reduction in abnormal gyral enhancement. After 3 years, the patient presented again with right sided paresis. MR imaging again shows signal increase in the temporal lobe on PD weighted images (e) while on T1 post gadolinium imaging (f) abnormal gyral enhancement is again visible. Like after the previous episode, the MR abnormalities disappeared (g, h)
Mentions: Three years thereafter he was again taken into hospital with focal epileptic seizures of the right side of his face. He suffered from headache, progressive aphasia, right hemianopia and mild paresis of his right arm and leg. An MRI scan now showed diffuse cortical enhancement of the left occipital and temporal cortex (Fig. 3a, b). This enhancement disappeared on a subsequent MRI performed 8 days later (Fig. 3c, d). An EEG showed no epileptic activity. Clinically, he recovered substantially.Fig. 3

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