F Bompaire1, L Zinchenko2, M Lahutte3, K Mokhtari4, D Psimaras5, C Gaultier2, A Monjour2, J-Y Delattre6, D Ricard7. 1. Service de neurologie, HIA du Val-de-Grâce, 74, boulevard de Port-Royal, 75005 Paris, France. Electronic address: fbompaire@gmail.com. 2. Service de neurologie, hôpital Pasteur, 39, avenue de la Liberté, 68000 Colmar, France. 3. Service de radiologie, HIA Percy, rue du Lieutenant-Raoul-Batany, 92140 Clamart, France. 4. Laboratoire de neuropathologie R. Escourolle, groupe hospitalier Pitié-Salpêtrière, AP-HP, 75013 Paris, France; Inserm, U 1127, ICM, 75013 Paris, France; CNRS, UMR 7225, ICM, 75013 Paris, France; Institut du cerveau et de la moelle épinière ICM, 75013 Paris, France. 5. Service de neurologie 2-Mazarin, groupe hospitalier Pitié-Salpêtrière, AP-HP, 75013 Paris, France. 6. Inserm, U 1127, ICM, 75013 Paris, France; CNRS, UMR 7225, ICM, 75013 Paris, France; Service de neurologie 2-Mazarin, groupe hospitalier Pitié-Salpêtrière, AP-HP, 75013 Paris, France; UM 75, ICM, Sorbonne universités, UPMC université Paris 06, 75013 Paris, France. 7. Service de neurologie, HIA du Val-de-Grâce, 74, boulevard de Port-Royal, 75005 Paris, France; Service de neurologie 2-Mazarin, groupe hospitalier Pitié-Salpêtrière, AP-HP, 75013 Paris, France; UMR8257 MD4 Cognac G, CNRS, service de santé des armées, université Paris Descartes, 75005 Paris, France; École du Val-de-Grâce, 1, place Alphonse-Laveran, 75005 Paris, France.
Abstract
BACKGROUND: Stroke-like migraine attacks after radiation therapy (SMART) syndrome is a rare complication of cerebral radiation therapy that usually presents>10 years after treatment as reversible paroxysmal episodes of neurological dysfunction associated with headaches. CASES: We report here on two cases of SMART syndrome in long-term survivors of high-grade glioma for whom neuropathological data were available. The course of the disease was unfavorable. Although the clinico-radiological picture of SMART syndrome clearly differs from classic cerebral radionecrosis, the gross neuropathological lesions observed in our two patients appeared to be similar to those described in focal radionecrosis. CONCLUSION: SMART syndrome may progress from a benign reversible form to a severe and eventually irreversible form. This severe course may also be confused with tumor progression, and lead to permanent disability and inadequate antitumor treatment. Clinicians should be aware of this latter atypical presentation.
BACKGROUND:Stroke-like migraine attacks after radiation therapy (SMART) syndrome is a rare complication of cerebral radiation therapy that usually presents>10 years after treatment as reversible paroxysmal episodes of neurological dysfunction associated with headaches. CASES: We report here on two cases of SMART syndrome in long-term survivors of high-grade glioma for whom neuropathological data were available. The course of the disease was unfavorable. Although the clinico-radiological picture of SMART syndrome clearly differs from classic cerebral radionecrosis, the gross neuropathological lesions observed in our two patients appeared to be similar to those described in focal radionecrosis. CONCLUSION: SMART syndrome may progress from a benign reversible form to a severe and eventually irreversible form. This severe course may also be confused with tumor progression, and lead to permanent disability and inadequate antitumor treatment. Clinicians should be aware of this latter atypical presentation.