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115 SMART: A rare syndrome with potentially devastating consequences
We report three cases with differing neurological presentations and outcomes. A 57 year old female with previous cranial radiotherapy for a frontal astrocytoma treated 9 years earlier presented with a two week history of headache, aphasia and right sided hemiparesis. MRI Brain demon- strated new extensive left hemispheric enhancement with extensive effacement of the ipsilateral cerebral sulci. 40 days following her presentation she started to improve and is now in rehabilitation. A 33 year old female with a background of medulloblastoma treated with cranio-spinal radiotherapy 19 years earlier presented with worsening confu...
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 27, 2022 Category: Neurosurgery Authors: Baheerathan, A., Lynch, D., Pednekar, N., Blindheim, C., Smyth, D., Stockford, L., Rees, J. Tags: Poster Presentations Source Type: research

Atypical imaging findings of presumed stroke-like migraine attacks after radiation therapy syndrome in the brainstem
AbstractThe authors present an atypical case of presumed stroke-like migraine attacks after radiation therapy (SMART) syndrome in the brainstem. A 29-year-old male, who had been treated with resection and subsequent craniospinal radiation for posterior fossa medulloblastoma 21 years before, presented with subacute progressive left hemiparesis evolving over 4 days. Hematological findings, cerebrospinal fluid (CSF), and electroencephalogram (EEG) were unremarkable. Magnetic resonance imaging (MRI) showed a round area of hyperintense FLAIR signal centered within the pons associated with central restricted diffusion, periphera...
Source: Neuroradiology - March 11, 2021 Category: Radiology Source Type: research

SMART Syndrome Identification and Successful Treatment
We report a 41-year-old male patient admitted to our emergency room with a reduced level of consciousness and global aphasia. One month prior to admission, he started with frequent headache attacks of moderate intensity and paroxysmal behavioral alterations, advancing to confusion, gait instability, language impairment, and somnolence. He had a history of medulloblastoma treated with surgical resection followed by craniospinal irradiation 21 years before symptom onset. After excluding more frequent causes for the patient ’s symptoms along with a suggestive image pattern, we started treatment for SMART syndrome with high-...
Source: Case Reports in Neurology - January 25, 2021 Category: Neurology Source Type: research

Sulfonylurea receptor 1 expression is variable in adult and pediatric brain tumors.
CONCLUSION: SUR1 is a putative therapeutic target to reduce neuroinflammation in adult and pediatric brain tumors. Inhibition of SUR1 may result in neuronal stabilization in glioblastoma, cerebral metastases, and posterior fossa ependymoma and reduced edema in medulloblastoma.
. PMID: 30079884 [PubMed - as supplied by publisher]
Source: Clinical Neuropathology - August 6, 2018 Category: Pathology Authors: Thompson EM, Halvorson K, McLendon R Tags: Clin Neuropathol Source Type: research

A 15-year-old girl with acute and reversible neurologic symptoms after cranial irradiation for medulloblastoma
A 15-year-old girl with history of medulloblastoma was evaluated for headache and acute onset neurologic deficits 5 years after completion of initial radiation therapy and 3 years following completion of re-irradiation. Neurological examination was notable for new onset left hemianopia, hemiparesis and neglect. MRI showed extensive areas of cortical T2 prolongation and thickening involving the right parietal, occipital and temporal lobes, with associated extensive gyral enhancement. Upon spontaneous resolution of her weakness after 4 days, repeat MRI showed resolution of the edema and gyral enhancement, suggestive of the d...
Source: Seminars in Pediatric Neurology - April 1, 2017 Category: Neurology Authors: Elizabeth Duke, Nicole J Ullrich Source Type: research

A 15-Year-Old Girl With Sudden Onsent Reversible Neurologic Symptoms After Cranial Irradiation for Medulloblastoma
A 15-year-old girl with history of medulloblastoma was evaluated for headache and neurologic deficits 5 years after completion of initial radiation therapy and 3 years following completion of reirradiation. Neurologic examination was notable for new-onset left hemianopia, hemiparesis, and neglect. Magnetic resonance imaging showed extensive areas of cortical T2 prolongation and thickening involving the right parietal, occipital, and temporal lobes with associated extensive gyral enhancement. Upon spontaneous resolution of her weakness after 4 days, repeat magnetic resonance imaging showed resolution of the edema and gyral ...
Source: Seminars in Pediatric Neurology - April 1, 2017 Category: Neurology Authors: Elizabeth Duke, Nicole J. Ullrich Source Type: research

Cerebral Hyperperfusion in a Child with Stroke-Like Migraine Attacks after Radiation Therapy Syndrome
We report an 11-year-old girl with a primary diagnosis of medulloblastoma presented with acute-onset severe headache and left-sided weakness, 20 months after completing cranial radiotherapy. MRI demonstrated unilateral cortical swelling and concomitant leptomeningeal, gyral contrast enhancement, and MR perfusion imaging showed increased cortical perfusion in the right temporo-parieto-occipital region. Her symptoms resolved spontaneously over several days.SMART syndrome appears to be a reversible, long-term complication of cranial radiotherapy. So far, a limited number of pediatric patients with SMART syndrome have been rep...
Source: Neuropediatrics - April 21, 2016 Category: Neurology Authors: Ardicli, DidemGocmen, RahsanOguz, Kader K.Varan, AliYalnizoglu, Dilek Tags: Short Communication Source Type: research

Status Epilepticus in Patients with SMART (P5.012)
CONCLUSIONS We describe three patients with SMART presenting with SE. Our cases raise the concern that SMART is not necessarily a migraine phenomenon but can be a form of focal SE associated with stroke-like symptoms. In patients with radiographic suspicion of SMART we suggest a low threshold for vEEG monitoring and aggressive anti-epileptic management.Disclosure: Dr. Fan has nothing to disclose. Dr. Gabriel has nothing to disclose. Dr. Gerard has nothing to disclose. Dr. Schuele has received personal compensation for activities with Sunovion and Eisai as a speaker.
Source: Neurology - April 3, 2016 Category: Neurology Authors: Fan, P., Gabriel, H., Gerard, E., Schuele, S. Tags: Neuromuscular Disease and Epilepsy ePoster Session Source Type: research

Late-Onset Radiation-Induced Vasculopathy and Stroke in a Child With Medulloblastoma
We report a case of a 15-year-old boy who presented to our institution with left-sided weakness and slurred speech. He had a history of medulloblastoma diagnosed at 3 years of age, status postsurgical resection and craniospinal radiation. Magnetic resonance imaging (MRI) of brain revealed a right paramedian pontine infarction, suspected secondary to late-onset radiation-induced vasculopathy of the vertebrobasilar system. Radiation to the brain is associated with increased incidence of ischemic stroke. Clinicians should have a high index of suspicion for stroke when these patients present with new neurologic symptoms.
Source: Journal of Child Neurology - April 15, 2015 Category: Neurology Authors: Bansal, L. R., Belair, J., Cummings, D., Zuccoli, G. Tags: Brief Communication Source Type: research

Teaching NeuroImages: Postoperative bifocal stroke of the pontine tegmentum: Why don't you smile anymore?
A 7-year-old boy underwent resection of a posterior fossa medulloblastoma. Two days later, he developed facial diplegia, left abducens nerve palsy, and mild hypalgesia below the neck. MRI documented 2 dot-like ischemic foci in the pons (figure). We diagnosed the unusual bilateral occurrence of Gasperini syndrome, a lesion of the sixth and seventh cranial nerve nuclei and the lateral spinothalamic tract that produces ipsilateral cranial nerve palsies with contralateral hemisensory deficits.1 We hypothesized a mechanism of postoperative spasm of long circumferential branches of the basilar artery. This mechanism should be co...
Source: Neurology - May 12, 2014 Category: Neurology Authors: Frassanito, P., Massimi, L., Tamburrini, G., Di Rocco, C., Caldarelli, M. Tags: Childhood stroke, MRI, Clinical neurology examination, Pediatric stroke; see Cerebrovascular Disease/ Childhood stroke RESIDENT AND FELLOW SECTION Source Type: research