Русский журнал детской неврологии. 2012; 7: 17-30
СИНДРОМ ГЕМИКОНВУЛЬСИВНЫХ ПРИСТУПОВ, ГЕМИПЛЕГИИ И ЭПИЛЕПСИИ (СИНДРОМ HHE) (ЛЕКЦИЯ)
https://doi.org/10.17650/2073-8803-2012-7-4-17-30Аннотация
Синдром гемиконвульсивных приступов, гемиплегии и эпилепсии (синдромHHE) представляет собой по- лиэтиологичное заболевание, дебютирующее в детском возрасте и характеризующееся триадой признаков: 1) продолжительными судорожными приступами (обычно на фоне температуры), захватывающими половину тела (гемиконвульсии); 2) остро возникающим сразу после приступов гемипарезом (на стороне судорог); 3) последующим развитием симптоматической фокальной эпилепсии. Авторы подробно рассматривают различные аспекты заболевания, включая этиологию и патогенез, диагностические критерии и дифференциальный диагноз, а также подходы к терапии (профилактика и лечение заболевания) и прогноз.
Список литературы
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6. Auvin S., Devism L., Maurage C.A., Soto-Ares G., Cuisset J.M., Leclerc F., et al. Neuropathological and MRI findings in an presentation of HHE: a report with pathophysiological implications // Seizure. — 2007. — V. 16(4). — P. 371-6.
7. Bahi-Buisson N., Kossorotoff M., Barnerias C., Boddaert N., Bourgeois M., Dulac O., Plouin P., Chiron C., Hertz- Pannier L. Atypical case of hemiconvulsions-hemiplegia-epilepsy syndrome revealing contralateral focal cortical dysplasia // Dev. Med. Child Neurol. — 2005. — V. 47(12). — P. 830-834.
8. Baram T.Z., Schinnar Sh. Febrile seizures. — San Diego, Academic press, 2002. — 337 P.
9. Bernheim M., Girard P., Larbre F. Le role des phlebites cerebrales dans les "encephalitis aigues de l'enfant" // Semaine Hop Paris. — 1955. — V. 31. — P. 1-14.
10. Bruni J. Valproate // In: The treatment of epilepsy / Eds.: S. Shorvon, F. Dreifuss, D. Fish, D. Thomas. — Oxford, 1996. — P. 482-490.
11. Chauvel P., Dravet Ch. The HHE syndrome // In: Epileptic syndromes in infancy, childhood and adolescence. 4th edition / Eds. J. Roger, M. Bureau, Ch. Dravet, P. Genton, C.A. Tassinari, P. Wolf — John Libbey Eurotext, 2005. — P. 277-293.
12. Chauvel P., Dravet Ch., Di Leo M., Roger J., Bancaud J., Talairach J. The HHE syndrome // In: Epilepsy surgery / Eds. H. Luders — N.Y.: Raven Press, 1991. — P.183-196.
13. Dulac O., Aubourg P., Checoury A., Devictor D., Plouin P., Arthuis M. Etats de mal convulsifs du nourrisson: aspects etiologiques et pronostiques // Rev. EEG Neurophysiol. — 1984. — V. 14. — P. 255-262.
14. Engel J.Jr. Principles of epilepsy surgery // In: The treatment of epilepsy / Eds.: S. Shorvon, F. Dreifuss, D. Fish, D. Thomas. — Oxford, 1996. — P. 519-529.
15. Freeman J.L. Coleman L.T, Smith L.J., Shield L.K. HHE Syndrome; characteristic early magnetic resonance imaging findings // J Child Neurol. — 2002. — V. 17 (1). — P. 10-60.
16. Gastaut H., Poirier F., Payan H., Salamon G., Toga M., Vigouroux M. HHE syndrome: hemiconvulsions, hemiplegia, epilepsy // Epilepsia. — 1959. — V. 1. — P. 418-447.
17. Gastaut H., Vigouroux M., Trevisan C., Regis H. Le syndrome "Hemiconvulsion - Hemiplegie - Epilepsie" (Syndrome HHE) // Rev. Neurol. — 1957. — V. 97. — P. 37-52.
18. Hiremath R., Pattar R., Pol M., Anegundi T.J., Rudrappa K. Role of Diffusion Weighted Imaging in the Detection of the Initial Phase of the Hemiplegia Hemiconvulsion Epilepsy Syndrome: A Case Report // Journal of Clinical and Diagnostic Research. — 2012. — V. 6(1). — P. 101-102.
19. Kim D.W., Kim K.K., Chu K., Chung C.K., Lee S.K. Surgical treatment of delayed epilepsy in hemiconvulsion- hemiplegia-epilepsy syndrome // Neurology. — 2008. — V. 70 (22 Pt 2). — P. 2097-2098.
20. Lee C., Born M., Salomons G.S., Jakobs C., Woelfle J. HHE Syndrome as a presenting feature L-2-hydroxyglutar- ic aciduria // J Child Neurol. — 2006. — V. 21(6). — P. 538-40.
21. Marie P. Hemiplegie cerebrale infantile et maladies infectieuses // Prog. Med. — 1885. — V. 2. — P. 167.
22. Mirsattari S., Wilde N., Pigott S. Long-term cognitive outcome of hemiconvulsion-hemiplegia-epilepsy syndrome affecting the left cerebral hemisphere // Epilepsy Behav. — 2008. — V. 13. — P. 678-680.
23. Mondal R.K., Chakravorty D., Das S. Hemiconvulsion, hemiplegia, epilepsy syndrome and inherited protein S deficiency // Indian J. Pediatr. — 2006. — V. 73(2). — P. 157-159.
24. Osborn A.G., Blaser S.I., Salzman K.L. et al. Diagnostic imaging brain // Salt Lake City, Amirsys, 2004. — I-10. — P. 54-57.
25. Osler W. Cerebral palsies of children // Philadelphia: Blakiston, 1889.
26. Panayiotopoulos C.P. A clinical guide to epileptic syndromes and their treatment // Bladon Medical Publishing, Oxford, 2002. - P. 36-49.
27. Panayiotopoulos C.P. Severe Neocortical Epileptic Syndromes in Infancy and Childhood // in The Epilepsies: Seizures, Syndromes and Management. — Bladon Medical Publishing, Oxford, 2005. — 541 p.
28. Roger J., Dravet Ch., Bureau M. Unilateral seizures: hemiconvulsions - hemiplegia syndrome (HH) and hemi- convulsions - hemiplegia epilepsia syndrome (HHE) // In: Henri Gastaut and the Marseilles school's contribution to the neurosciences / Eds.: R.J. Broughton — EEG Suppl. — 1982. — № 35. — P. 211-221.
29. Roger J., Lob H., Tassinari C.A. Status epilepticus // In: The epilepsies / Eds.: Vinken P.J., Bruyn C.W. — Handbook of neurology, 1974. — V. 15. — P. 145-188.
30. Sakakibara T., Nakagawa E., Saito Y., Sakuma H., Komaki H., Sugai K., Sasaki M., Kurahashi H., Hirose S. Hemiconvulsion-hemiplegia syndrome in a patient with severe myoclonic epilepsy in infancy //Epilepsia. — 2009. — V. 50(9). — P. 2158-2162.
31. Salih M.A., Kabiraj M., Al-Jarallah A.S., El Desouki M., Othman S., Palkar V.A. Hemiconvulsion-hemiplegia-epilep- sy syndrome A clinical, electroencephalographic and neuroradiological study // Child's Nervous System. — 1997. — V. 13, № 5. — P. 257-263.
32. Soffer D., Melamed E., Assaf Y. et al. Hemispheric brain damage in unilateral status epilepticus // Ann. Neurol. — 1986. — V. 20. — P. 737-740.
33. Tan N., Urich H. Postictal cerebral hemiatrophy with a contribution to the problem of crossed cerebellar atrophy // Acta Neuropathol. (Berlin). — 1984. — V. 62. — P.332-339.
34. Tanaka Y., Nakanishi Y., Hamano S., Nara T., Aihara T. Brain perfusion in acute infantile hemiplegia studied with single photon emission computed tomography // No. To. Hattatsu. — 1994. — V. 26. — P. 68-73.
35. Tenney J.R., Schapiro M.B. Child neurology: hemiconvulsion-hemiplegia-epilepsy syndrome // Neurology. — 2012. — V. 79(1). — e1-4.
36. Toldo I., Calderone M., Boniver C., Dravet C.H., Guerrini R., Laverda A.M. HHE Syndrome: early MRI findings // Brain Dev — 2007. — V. 29 (2). — P. 109-111.
37. Toorn R., Janse van Rensburg P., Solomons R., Ndondo A.P., Schoeman J.F. Hemiconvulsion-hemiplegia-epilep- sy syndrome in South African children: insights from a retrospective case series // Eur J Paediatr Neurol. — 2012. — V. 16(2). — P. 142-8.
38. Vivaldi J. J. Les crises hemicloniques de l'enfant: modalites evolutives // These, Marseille, 1976.
39. Wakamoto H., Ohta M., Nakano N. Hypercytokinemia in hemiconvulsions-hemiplegia syndrome associated with dual infection with varicella zoster and Epstein-Barr viruses // Neuropediatrics. — 2002. — V. 33(5). — P. 262-265.
40. Walker M.C., Shorvon S.D. Treatment of status epilepticus and serial seizures // In: The treatment of epilepsy / Eds.: S. Shorvon, F. Dreifuss, D. Fish, D. Thomas. — Oxford, 1996. — P. 269-285.
41. Yamazaki S., Ikeno K., Abe T., Tohyama J., Adachi Y. Hemiconvulsion-hemiplegia-epilepsy syndrome associated with CACNA1A S218L mutation // Pediatr Neurol. —2011. — V. 45(3). — P. 193-6.
42. Young G.B. Status epilepticus and brain damage: pathology and pathophysiology // Advances in neurology. — 2006. — V. 97. — P. 217-220.
Russian Journal of Child Neurology. 2012; 7: 17-30
HEMICONVULSION-HEMIPLEGIA-EPILEPSY SYNDROME (HHE SYNDROME) (A LECTURE)
https://doi.org/10.17650/2073-8803-2012-7-4-17-30Abstract
Hemiconvulsion-hemiplegia-epilepsy syndrome (HHE syndrome) — polyetiologic disease, with onset in childhood, characterized by the triad of symptoms: 1) prolonged convulsive seizures (usually on background of fever), involving one half of the body (hemiconvulsions); 2) acute hemiparesis occurring immediately after seizures (on the side of convulsions); 3) symptomatic focal epilepsy, occurring in the sequel. The authors discuss in detail different aspects of the disease, including etiology, pathogenesis, diagnostic approaches, differential diagnosis, and approaches to treatment (including prevention and therapy of the disease) and outcome.
References
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4. Arzimanoglou A., Guerrini R., Aicardi J. Aicardi's epilepsy in children — 3-rd edition. — Lippincott, Philadelphia, 2004. — P. 176-187.
5. Auvin S., Bellavoine V., Merdariu D., Delanoe C., Elmaleh-Berges M., Gressens P., Boespflug-Tanguy O. Hemiconvulsion-hemiplegia-epilepsy syndrome: Current understandings // Eur J Paediatr Neurol. — 2012. — V. 16(5). — P. 413-21.
6. Auvin S., Devism L., Maurage C.A., Soto-Ares G., Cuisset J.M., Leclerc F., et al. Neuropathological and MRI findings in an presentation of HHE: a report with pathophysiological implications // Seizure. — 2007. — V. 16(4). — P. 371-6.
7. Bahi-Buisson N., Kossorotoff M., Barnerias C., Boddaert N., Bourgeois M., Dulac O., Plouin P., Chiron C., Hertz- Pannier L. Atypical case of hemiconvulsions-hemiplegia-epilepsy syndrome revealing contralateral focal cortical dysplasia // Dev. Med. Child Neurol. — 2005. — V. 47(12). — P. 830-834.
8. Baram T.Z., Schinnar Sh. Febrile seizures. — San Diego, Academic press, 2002. — 337 P.
9. Bernheim M., Girard P., Larbre F. Le role des phlebites cerebrales dans les "encephalitis aigues de l'enfant" // Semaine Hop Paris. — 1955. — V. 31. — P. 1-14.
10. Bruni J. Valproate // In: The treatment of epilepsy / Eds.: S. Shorvon, F. Dreifuss, D. Fish, D. Thomas. — Oxford, 1996. — P. 482-490.
11. Chauvel P., Dravet Ch. The HHE syndrome // In: Epileptic syndromes in infancy, childhood and adolescence. 4th edition / Eds. J. Roger, M. Bureau, Ch. Dravet, P. Genton, C.A. Tassinari, P. Wolf — John Libbey Eurotext, 2005. — P. 277-293.
12. Chauvel P., Dravet Ch., Di Leo M., Roger J., Bancaud J., Talairach J. The HHE syndrome // In: Epilepsy surgery / Eds. H. Luders — N.Y.: Raven Press, 1991. — P.183-196.
13. Dulac O., Aubourg P., Checoury A., Devictor D., Plouin P., Arthuis M. Etats de mal convulsifs du nourrisson: aspects etiologiques et pronostiques // Rev. EEG Neurophysiol. — 1984. — V. 14. — P. 255-262.
14. Engel J.Jr. Principles of epilepsy surgery // In: The treatment of epilepsy / Eds.: S. Shorvon, F. Dreifuss, D. Fish, D. Thomas. — Oxford, 1996. — P. 519-529.
15. Freeman J.L. Coleman L.T, Smith L.J., Shield L.K. HHE Syndrome; characteristic early magnetic resonance imaging findings // J Child Neurol. — 2002. — V. 17 (1). — P. 10-60.
16. Gastaut H., Poirier F., Payan H., Salamon G., Toga M., Vigouroux M. HHE syndrome: hemiconvulsions, hemiplegia, epilepsy // Epilepsia. — 1959. — V. 1. — P. 418-447.
17. Gastaut H., Vigouroux M., Trevisan C., Regis H. Le syndrome "Hemiconvulsion - Hemiplegie - Epilepsie" (Syndrome HHE) // Rev. Neurol. — 1957. — V. 97. — P. 37-52.
18. Hiremath R., Pattar R., Pol M., Anegundi T.J., Rudrappa K. Role of Diffusion Weighted Imaging in the Detection of the Initial Phase of the Hemiplegia Hemiconvulsion Epilepsy Syndrome: A Case Report // Journal of Clinical and Diagnostic Research. — 2012. — V. 6(1). — P. 101-102.
19. Kim D.W., Kim K.K., Chu K., Chung C.K., Lee S.K. Surgical treatment of delayed epilepsy in hemiconvulsion- hemiplegia-epilepsy syndrome // Neurology. — 2008. — V. 70 (22 Pt 2). — P. 2097-2098.
20. Lee C., Born M., Salomons G.S., Jakobs C., Woelfle J. HHE Syndrome as a presenting feature L-2-hydroxyglutar- ic aciduria // J Child Neurol. — 2006. — V. 21(6). — P. 538-40.
21. Marie P. Hemiplegie cerebrale infantile et maladies infectieuses // Prog. Med. — 1885. — V. 2. — P. 167.
22. Mirsattari S., Wilde N., Pigott S. Long-term cognitive outcome of hemiconvulsion-hemiplegia-epilepsy syndrome affecting the left cerebral hemisphere // Epilepsy Behav. — 2008. — V. 13. — P. 678-680.
23. Mondal R.K., Chakravorty D., Das S. Hemiconvulsion, hemiplegia, epilepsy syndrome and inherited protein S deficiency // Indian J. Pediatr. — 2006. — V. 73(2). — P. 157-159.
24. Osborn A.G., Blaser S.I., Salzman K.L. et al. Diagnostic imaging brain // Salt Lake City, Amirsys, 2004. — I-10. — P. 54-57.
25. Osler W. Cerebral palsies of children // Philadelphia: Blakiston, 1889.
26. Panayiotopoulos C.P. A clinical guide to epileptic syndromes and their treatment // Bladon Medical Publishing, Oxford, 2002. - P. 36-49.
27. Panayiotopoulos C.P. Severe Neocortical Epileptic Syndromes in Infancy and Childhood // in The Epilepsies: Seizures, Syndromes and Management. — Bladon Medical Publishing, Oxford, 2005. — 541 p.
28. Roger J., Dravet Ch., Bureau M. Unilateral seizures: hemiconvulsions - hemiplegia syndrome (HH) and hemi- convulsions - hemiplegia epilepsia syndrome (HHE) // In: Henri Gastaut and the Marseilles school's contribution to the neurosciences / Eds.: R.J. Broughton — EEG Suppl. — 1982. — № 35. — P. 211-221.
29. Roger J., Lob H., Tassinari C.A. Status epilepticus // In: The epilepsies / Eds.: Vinken P.J., Bruyn C.W. — Handbook of neurology, 1974. — V. 15. — P. 145-188.
30. Sakakibara T., Nakagawa E., Saito Y., Sakuma H., Komaki H., Sugai K., Sasaki M., Kurahashi H., Hirose S. Hemiconvulsion-hemiplegia syndrome in a patient with severe myoclonic epilepsy in infancy //Epilepsia. — 2009. — V. 50(9). — P. 2158-2162.
31. Salih M.A., Kabiraj M., Al-Jarallah A.S., El Desouki M., Othman S., Palkar V.A. Hemiconvulsion-hemiplegia-epilep- sy syndrome A clinical, electroencephalographic and neuroradiological study // Child's Nervous System. — 1997. — V. 13, № 5. — P. 257-263.
32. Soffer D., Melamed E., Assaf Y. et al. Hemispheric brain damage in unilateral status epilepticus // Ann. Neurol. — 1986. — V. 20. — P. 737-740.
33. Tan N., Urich H. Postictal cerebral hemiatrophy with a contribution to the problem of crossed cerebellar atrophy // Acta Neuropathol. (Berlin). — 1984. — V. 62. — P.332-339.
34. Tanaka Y., Nakanishi Y., Hamano S., Nara T., Aihara T. Brain perfusion in acute infantile hemiplegia studied with single photon emission computed tomography // No. To. Hattatsu. — 1994. — V. 26. — P. 68-73.
35. Tenney J.R., Schapiro M.B. Child neurology: hemiconvulsion-hemiplegia-epilepsy syndrome // Neurology. — 2012. — V. 79(1). — e1-4.
36. Toldo I., Calderone M., Boniver C., Dravet C.H., Guerrini R., Laverda A.M. HHE Syndrome: early MRI findings // Brain Dev — 2007. — V. 29 (2). — P. 109-111.
37. Toorn R., Janse van Rensburg P., Solomons R., Ndondo A.P., Schoeman J.F. Hemiconvulsion-hemiplegia-epilep- sy syndrome in South African children: insights from a retrospective case series // Eur J Paediatr Neurol. — 2012. — V. 16(2). — P. 142-8.
38. Vivaldi J. J. Les crises hemicloniques de l'enfant: modalites evolutives // These, Marseille, 1976.
39. Wakamoto H., Ohta M., Nakano N. Hypercytokinemia in hemiconvulsions-hemiplegia syndrome associated with dual infection with varicella zoster and Epstein-Barr viruses // Neuropediatrics. — 2002. — V. 33(5). — P. 262-265.
40. Walker M.C., Shorvon S.D. Treatment of status epilepticus and serial seizures // In: The treatment of epilepsy / Eds.: S. Shorvon, F. Dreifuss, D. Fish, D. Thomas. — Oxford, 1996. — P. 269-285.
41. Yamazaki S., Ikeno K., Abe T., Tohyama J., Adachi Y. Hemiconvulsion-hemiplegia-epilepsy syndrome associated with CACNA1A S218L mutation // Pediatr Neurol. —2011. — V. 45(3). — P. 193-6.
42. Young G.B. Status epilepticus and brain damage: pathology and pathophysiology // Advances in neurology. — 2006. — V. 97. — P. 217-220.
