Stroke‐like events after brain radiotherapy: a large series with long‐term follow‐up

Abstract

Background and purpose Patients with a history of brain radiotherapy can experience acute stroke‐like syndromes related to the delayed effects of brain radiation, including stroke‐like migraine attacks after radiation therapy syndrome, peri‐ictal pseudoprogression and acute late‐onset encephalopathy after radiation therapy syndrome. The aim of this study was to collect evidence on the long‐term outcome and treatment of these conditions, whose knowledge is undermined by their rarity and fragmented description. Methods Cases were collected, both prospectively and retrospectively, amongst six neuro‐oncology departments. Inclusion criteria were as follows: (i) history of brain radiotherapy (completed at least 6 months before the acute episode); (ii) new onset of acute/subacute neurological symptoms; (iii) exclusion of all etiologies unrelated to brain irradiation. A review of current literature on stroke‐like syndromes was performed to corroborate our findings. Results Thirty‐two patients with acute neurological conditions attributed to the delayed effects of radiation were identified, including 26 patients with stroke‐like syndromes. Patients with stroke‐like syndromes commonly presented with a mosaic of symptoms, including focal deficits (77%), encephalopathy (50%), seizures (35%) and headache (35%). Seventy‐three percent of them had acute consistent magnetic resonance imaging alterations. Treatment included high‐dose steroids in 65% of cases. Twenty‐two patients recovered completely (85%). Sixteen patients (62%) experienced relapses (median follow‐up 3.5 years). A literature review identified 87 additional stroke‐like cases with similar characteristics. Conclusions Stroke‐like events related to brain irradiation may be associated with permanent sequelae. Steroids are often administered on empirical grounds, as they are thought to accelerate recovery. Relapses are common, highlighting the need to elaborate adequate prevention strategies.

SMART syndrome: retrospective review of a rare delayed complication of radiation

“…Di Stefano et al [3] recently presented a series of 32 patients with spells of acute neurologic dysfunction attributed to remote irradiation including 15 patients with SMART syndrome, seven patients with ALERT syndrome (acute late‐onset encephalopathy after radiation therapy), and three patients with short‐lasting focal deficits. These authors did not separately report the data for SMART syndrome cases as they considered these three different entities to belong to a common pathophysiologic spectrum, although acute late‐onset encephalopathy after radiotherapy (ALERT) syndrome primarily manifests with encephalopathy and does not have the classic MRI findings of SMART.…”

“…Patients with SMART syndrome have a history of a neoplasm and may have multiple relapses [3]. Consequently, we questioned whether SMART could be a manifestation of a paraneoplastic or autoimmune syndrome.…”

“…SMART is most often considered a delayed complication of brain radiation which can occur decades after therapy [1,2]. Although it is a rare condition, improvement in cancer survival rates and better awareness has resulted in a rise in the frequency of this diagnosis [3]. Case reports of these syndromic stroke‐like episodes occurring in pediatric and adult patients with remote cerebral irradiation have been described for over two decades in multiple countries.…”

Cerebral Autoregulation and Neurovascular Coupling after Craniospinal Irradiation in Long-Term Survivors of Malignant Pediatric Brain Tumors of the Posterior Fossa

“…24 Migraine attacks have been reported after irradiation. 25 In migraine, the autoregulatory index Dx was altered in patients with migraine with aura. 26 No alterations of cerebrovascular reactivity had been demonstrated in three patients with stroke-like migraine attacks after irradiation.…”

Stroke-Like Migraine Attacks After Radiation Therapy Syndrome

“…18 Di Stefano et al reported full recovery in 85% (22/26) of patients, while 15% were left with permanent symptoms. 19 Black et al reported that 45% (5/11) of patients in their case series had longterm symptoms, including cognitive impairment, hemiparesis, and dysphasia. 1 Permanent imaging findings of cortical laminar necrosis were present in 27% of patients.…”