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Abstract
Background: To assess the role of 2-deoxy-2(18F)-fluoro-D-glucose positron emission tomography (FDG-PET) scans in the comprehensive evaluation and surgical decision-making in patients with schizencephaly. Methods: We evaluated 11 patients (8M) with schizencephaly (mean follow-up: 4.5 years), including detailed clinical, MRI, FDG-PET, EEG, surgical and neuropathology data. Results: Eight patients had unilateral and three had bilateral clefts on MRI. Mean age at seizure onset was 20 months, with seizure being frequent in 10 and rare in one. Multiple seizure types were noted, with complex partial seizures being the most common (n=8) followed by infantile spasms (n=6). FDG-PET showed larger area of involvement than MRI in all the patients which corresponded better with the electrophysiological changes. Five patients (with unilateral disease on MRI) underwent epilepsy surgery (4 hemispherectomy and 1 multilobar resection). Two patients with focal defect on MRI underwent hemispherectomy due to larger area of abnormality revealed by FDG-PET. One patient was excluded from the surgery due to bilateral abnormalities on FDG PET. Six patients (4 with surgery) were seizure-free at last follow-up (average seizure-free duration: 70 months). One patient who underwent hemispherectomy due to apparently unilateral disease on both video-EEG and MRI but having bilateral abnormality on PET continued to have seizures. ACTH treatment had only a brief (1 month to 1 year) or no response in the six infantile spasms patients. Conclusions: FDG-PET typically shows a much larger area of involvement than MRI thus supplementing MRI in defining the full extent of malformation and assessing the functional integrity of the contralateral hemisphere. FDG-PET may prove to be a useful tool to aid in surgical decision-making and predicting surgical outcome, as patients with contralateral abnormality on FDG-PET may have poor surgical outcomes. When the malformation is unilateral with an intact contralateral hemisphere, surgery (usually hemispherectomy) may be curative of the epilepsy.
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Copyright (c) 2017 Tuhina Govil-Dalela, Ajay Kumar, Praneetha Konka, Harry T Chugani
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References
1. Guerrini R, Dobyns WB. Malformations of cortical development: clinical features and genetic causes. The Lancet Neurology. 2014;13:710-26.
2. Haverkamp F, Zerres K, Ostertun B, Emons D, Lentze M. Familial schizencephaly: further delineation of a rare disorder. Journal of medical genetics. 1995;32:242-4.
3. Curry CJ, Lammer EJ, Nelson V, Shaw GM. Schizencephaly: heterogeneous etiologies in a population of 4 million California births. Am J Med Genet A. 2005;137:181-9. doi:10.1002/ajmg.a.30862.
4. Howe DT, Rankin J, Draper ES. Schizencephaly prevalence, prenatal diagnosis and clues to etiology: a register-based study. Ultrasound Obstet Gynecol. 2012;39:75-82. doi:10.1002/uog.9069.
5. Barkovich A, Kuzniecky R, Jackson G, Guerrini R, Dobyns W. A developmental and genetic classification for malformations of cortical development. Neurology. 2005;65:1873-87.
6. Dies KA, Bodell A, Hisama FM, Guo C-Y, Barry B, Chang BS, Barkovich AJ, Walsh CA. Schizencephaly Association With Young Maternal Age, Alcohol Use, and Lack of Prenatal Care. Journal of child neurology. 2012:0883073812467850.
7. Denis D, Chateil JF, Brun M, Brissaud O, Lacombe D, Fontan D, Flurin V, Pedespan J. Schizencephaly: clinical and imaging features in 30 infantile cases. Brain Dev. 2000;22:475-83.
8. Luat AF, Bernardi B, Chugani HT. Congenital perisylvian syndrome: MRI and glucose PET correlations. Pediatric neurology. 2006;35:21-9.
9. Leventer RJ, Guerrini R, Dobyns WB. Malformations of cortical development and epilepsy. Dialogues Clin Neurosci. 2008;10:47-62.
10. Granata T, Battaglia G, D'Incerti L, Franceschetti S, Spreafico R, Battino D, Savoiardo M, Avanzini G. Schizencephaly: neuroradiologic and epileptologic findings. Epilepsia. 1996;37:1185-93.
11. Barkovich AJ, Kjos BO. Schizencephaly: correlation of clinical findings with MR characteristics. AJNR Am J Neuroradiol. 1992;13:85-94.
12. Packard AM, Miller VS, Delgado MR. Schizencephaly: correlations of clinical and radiologic features. Neurology. 1997;48:1427-34.
13. Suchet I. Schizencephaly: antenatal and postnatal assessment with colour-flow Doppler imaging. Canadian Association of Radiologists journal= Journal l'Association canadienne des radiologistes. 1994;45:193-200.
14. Choi HY, Koh EJ. Long-term outcome of surgical treatment of patients with intractable epilepsy associated with schizencephaly. Acta Neurochir (Wien). 2013;155:1717-24. doi:10.1007/s00701-013-1791-0.
15. Granata T, Freri E, Caccia C, Setola V, Taroni F, Battaglia G. Schizencephaly: clinical spectrum, epilepsy, and pathogenesis. J Child Neurol. 2005;20:313-8.
16. Hayashi N, Tsutsumi Y, Barkovich AJ. Morphological features and associated anomalies of schizencephaly in the clinical population: detailed analysis of MR images. Neuroradiology. 2002;44:418-27. doi:10.1007/s00234-001-0719-1.
17. Nishio S, Morioka T, Mihara F, Fukui M. Neuroimaging and neuropathology in epilepsy: With special reference to focal epileptogenic abnormalities. Neuropathology. 1999;19:238-46.
18. Lee N, Radtke RA, Gray L, Burger PC, Montine TJ, DeLong GR, Lewis DV, Oakes WJ, Friedman AH, Hoffman JM. Neuronal migration disorders: positron emission tomography correlations. Annals of neurology. 1994;35:290-7.
19. Salamon N, Kung J, Shaw S, Koo J, Koh S, Wu J, Lerner J, Sankar R, Shields W, Engel J. FDG-PET/MRI coregistration improves detection of cortical dysplasia in patients with epilepsy. Neurology. 2008;71:1594-601.
20. Chugani HT, Shewmon DA, Khanna S, Phelps ME. Interictal and postictal focal hypermetabolism on positron emission tomography. Pediatric neurology. 1993;9:10-5.
21. Bittar R, Andermann F, Olivier A, Dubeau F, Dumoulin S, Pike G, Reutens D. Interictal spikes increase cerebral glucose metabolism and blood flow: a PET study. Epilepsia. 1999;40:170-8.
22. Redecker C, Lutzenburg M, Gressens P, Evrard P, Witte O, Hagemann G. Excitability changes and glucose metabolism in experimentally induced focal cortical dysplasias. Cerebral Cortex. 1998;8:623-34.
23. Bairamian D, Di Chiro G, Theodore WH, Holmes MD, Dorwart RH, Larson SM. MR imaging and positron emission tomography of cortical heterotopia. Journal of computer assisted tomography. 1985;9:1137-9.
24. Chugani HT. Role of PET in detection of cerebral dysgenesis. In: Kotagal P LH, editor. The epilepsies: Etiologies and prevention. New York: Spectrum; 1999. p. 29-36.
25. Ji-soo Kim S-kL, Dong-soo Lee, Ki-hyun Chang. Neuronal Migration Disorders: Correlation of MRI, SPECT and PET Findings. Epilepsia. 1996;37.
26. Colombo N, Salamon N, Raybaud C, Özkara Ç, Barkovich AJ. Imaging of malformations of cortical development. Epileptic Disorders. 2009;11:194-205.
27. Stanescu L, Ishak GE, Khanna PC, Biyyam DR, Shaw DW, Parisi MT. FDG PET of the brain in pediatric patients: Imaging spectrum with MR imaging correlation. Radiographics. 2013;33:1279-303.
References
1. Guerrini R, Dobyns WB. Malformations of cortical development: clinical features and genetic causes. The Lancet Neurology. 2014;13:710-26.
2. Haverkamp F, Zerres K, Ostertun B, Emons D, Lentze M. Familial schizencephaly: further delineation of a rare disorder. Journal of medical genetics. 1995;32:242-4.
3. Curry CJ, Lammer EJ, Nelson V, Shaw GM. Schizencephaly: heterogeneous etiologies in a population of 4 million California births. Am J Med Genet A. 2005;137:181-9. doi:10.1002/ajmg.a.30862.
4. Howe DT, Rankin J, Draper ES. Schizencephaly prevalence, prenatal diagnosis and clues to etiology: a register-based study. Ultrasound Obstet Gynecol. 2012;39:75-82. doi:10.1002/uog.9069.
5. Barkovich A, Kuzniecky R, Jackson G, Guerrini R, Dobyns W. A developmental and genetic classification for malformations of cortical development. Neurology. 2005;65:1873-87.
6. Dies KA, Bodell A, Hisama FM, Guo C-Y, Barry B, Chang BS, Barkovich AJ, Walsh CA. Schizencephaly Association With Young Maternal Age, Alcohol Use, and Lack of Prenatal Care. Journal of child neurology. 2012:0883073812467850.
7. Denis D, Chateil JF, Brun M, Brissaud O, Lacombe D, Fontan D, Flurin V, Pedespan J. Schizencephaly: clinical and imaging features in 30 infantile cases. Brain Dev. 2000;22:475-83.
8. Luat AF, Bernardi B, Chugani HT. Congenital perisylvian syndrome: MRI and glucose PET correlations. Pediatric neurology. 2006;35:21-9.
9. Leventer RJ, Guerrini R, Dobyns WB. Malformations of cortical development and epilepsy. Dialogues Clin Neurosci. 2008;10:47-62.
10. Granata T, Battaglia G, D'Incerti L, Franceschetti S, Spreafico R, Battino D, Savoiardo M, Avanzini G. Schizencephaly: neuroradiologic and epileptologic findings. Epilepsia. 1996;37:1185-93.
11. Barkovich AJ, Kjos BO. Schizencephaly: correlation of clinical findings with MR characteristics. AJNR Am J Neuroradiol. 1992;13:85-94.
12. Packard AM, Miller VS, Delgado MR. Schizencephaly: correlations of clinical and radiologic features. Neurology. 1997;48:1427-34.
13. Suchet I. Schizencephaly: antenatal and postnatal assessment with colour-flow Doppler imaging. Canadian Association of Radiologists journal= Journal l'Association canadienne des radiologistes. 1994;45:193-200.
14. Choi HY, Koh EJ. Long-term outcome of surgical treatment of patients with intractable epilepsy associated with schizencephaly. Acta Neurochir (Wien). 2013;155:1717-24. doi:10.1007/s00701-013-1791-0.
15. Granata T, Freri E, Caccia C, Setola V, Taroni F, Battaglia G. Schizencephaly: clinical spectrum, epilepsy, and pathogenesis. J Child Neurol. 2005;20:313-8.
16. Hayashi N, Tsutsumi Y, Barkovich AJ. Morphological features and associated anomalies of schizencephaly in the clinical population: detailed analysis of MR images. Neuroradiology. 2002;44:418-27. doi:10.1007/s00234-001-0719-1.
17. Nishio S, Morioka T, Mihara F, Fukui M. Neuroimaging and neuropathology in epilepsy: With special reference to focal epileptogenic abnormalities. Neuropathology. 1999;19:238-46.
18. Lee N, Radtke RA, Gray L, Burger PC, Montine TJ, DeLong GR, Lewis DV, Oakes WJ, Friedman AH, Hoffman JM. Neuronal migration disorders: positron emission tomography correlations. Annals of neurology. 1994;35:290-7.
19. Salamon N, Kung J, Shaw S, Koo J, Koh S, Wu J, Lerner J, Sankar R, Shields W, Engel J. FDG-PET/MRI coregistration improves detection of cortical dysplasia in patients with epilepsy. Neurology. 2008;71:1594-601.
20. Chugani HT, Shewmon DA, Khanna S, Phelps ME. Interictal and postictal focal hypermetabolism on positron emission tomography. Pediatric neurology. 1993;9:10-5.
21. Bittar R, Andermann F, Olivier A, Dubeau F, Dumoulin S, Pike G, Reutens D. Interictal spikes increase cerebral glucose metabolism and blood flow: a PET study. Epilepsia. 1999;40:170-8.
22. Redecker C, Lutzenburg M, Gressens P, Evrard P, Witte O, Hagemann G. Excitability changes and glucose metabolism in experimentally induced focal cortical dysplasias. Cerebral Cortex. 1998;8:623-34.
23. Bairamian D, Di Chiro G, Theodore WH, Holmes MD, Dorwart RH, Larson SM. MR imaging and positron emission tomography of cortical heterotopia. Journal of computer assisted tomography. 1985;9:1137-9.
24. Chugani HT. Role of PET in detection of cerebral dysgenesis. In: Kotagal P LH, editor. The epilepsies: Etiologies and prevention. New York: Spectrum; 1999. p. 29-36.
25. Ji-soo Kim S-kL, Dong-soo Lee, Ki-hyun Chang. Neuronal Migration Disorders: Correlation of MRI, SPECT and PET Findings. Epilepsia. 1996;37.
26. Colombo N, Salamon N, Raybaud C, Özkara Ç, Barkovich AJ. Imaging of malformations of cortical development. Epileptic Disorders. 2009;11:194-205.
27. Stanescu L, Ishak GE, Khanna PC, Biyyam DR, Shaw DW, Parisi MT. FDG PET of the brain in pediatric patients: Imaging spectrum with MR imaging correlation. Radiographics. 2013;33:1279-303.