Hemiconvulsion-hemiplegia-epilepsy syndrome: Current understandings

被引:44
作者
Auvin, Stephane [1 ,2 ,3 ]
Bellavoine, Vanina [3 ]
Merdariu, Dana [3 ]
Delanoe, Catherine [4 ]
Elmaleh-Berges, Monique [5 ]
Gressens, Pierre [1 ,2 ,3 ]
Boespflug-Tanguy, Odile [2 ,3 ]
机构
[1] INSERM, U676, Paris, France
[2] Univ Paris 07, Fac Med Denis Diderot, Paris, France
[3] Hop Robert Debre, APHP, Serv Neurol Pediat, F-75019 Paris, France
[4] Robert Debre Children Hosp, APHP, Dept Neurophysiol, Paris, France
[5] Robert Debre Children Hosp, APHP, Dept Radiol, Paris, France
关键词
Hemiconvulsion; Hemiplegia; Febrile seizure; Status epilepticus; Edema; CACNA1A; Epileptogenesis; CACNA1A GENE MUTATION; STATUS EPILEPTICUS; VASCULAR CHANGES; CALCIUM-CHANNEL; MIGRAINE; BRAIN; ASSOCIATION; INJURY; CONSEQUENCES; HYPERTHERMIA;
D O I
10.1016/j.ejpn.2012.01.007
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Hemiconvulsion-Hemiplegia (HH) syndrome is an uncommon consequence of prolonged focal febrile convulsive seizures in infancy and early childhood. It is characterized by the occurrence of prolonged clonic seizures with unilateral predominance occurring in a child and followed by the development of hemiplegia. Neuroradiological studies showed unilateral edematous swelling of the epileptic hemisphere at the time of initial status epilepticus (SE). This acute phase is followed by characteristic cerebral hemiatrophy with subsequent appearance of epilepsy, so called Hemiconvulsion-Hemiplegia-Epilepsy (HHE) syndrome. The etiologies and the underlying mechanisms remain to be understood. Using a review of the literature, we summarized the data of the last 20 years. It appears that idiopathic HH/HHE syndrome is the most common reported form. The basic science data suggest that immature brain is relatively resistant to SE-induced cell injury. Several factors might contribute to the pathogenesis of HH/HHE syndrome: 1. prolonged febrile seizure in which inflammation may worsen the level of cell injury; 2. inflammation and prolonged ictal activity that act on blood-brain-barrier permeability; 3. predisposing factors facilitating prolonged seizure such as genetic factors or focal epileptogenic lesion. However, these factors cannot explain the elective involvement of an entire hemisphere. We draw new hypothesis that may explain the involvement of one hemisphere such as maturation of brain structure such as corpus callosum or genetic factors (CACNA1A gene) that are specifically discussed. An early diagnosis and a better understanding of the underlying mechanisms of HHE are needed to improve the outcome of this condition. (c) 2012 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
引用
收藏
页码:413 / 421
页数:9
相关论文
共 49 条
[11]   Decreased hemispheric water mobility in hemiplegic migraine related to mutation of CACNA1A gene [J].
Chabriat, H ;
Vahedi, K ;
Clark, CA ;
Poupon, C ;
Ducros, A ;
Denier, C ;
Le Bihan, D ;
Bousser, MG .
NEUROLOGY, 2000, 54 (02) :510-512
[12]  
Chauvel P, 2005, EPILEPTIC SYNDROMES, P277
[13]   Association between the α1a calcium channel gene CACNA1A and idiopathic generalized epilepsy [J].
Chioza, B ;
Wilkie, H ;
Nashef, L ;
Blower, J ;
McCormick, D ;
Sham, P ;
Asherson, P ;
Makoff, AJ .
NEUROLOGY, 2001, 56 (09) :1245-1246
[14]  
Franzoni E, 2010, NEUROL SCI
[15]   Hemiconvulsion-hemiplegia-epilepsy syndrome: Characteristic early magnetic resonance imaging findings [J].
Freeman, JL ;
Coleman, LT ;
Smith, LJ ;
Shield, LK .
JOURNAL OF CHILD NEUROLOGY, 2002, 17 (01) :10-16
[16]  
GASTAUT H, 1960, EPILEPSIA, V1, P418
[17]   Resistance of immature hippocampus to morphologic and physiologic alterations following status epilepticus or kindling [J].
Haas, KZ ;
Sperber, EF ;
Opanashuk, LA ;
Stanton, PK ;
Moshé, SL .
HIPPOCAMPUS, 2001, 11 (06) :615-625
[18]  
HOSSMANN KA, 1995, CEREBROVAS BRAIN MET, V7, P187
[19]   Human epilepsy associated with dysfunction of the brain P/Q-type calcium channel [J].
Jouvenceau, A ;
Eunson, LH ;
Spauschus, A ;
Ramesh, V ;
Zuberi, SM ;
Kullmann, DM ;
Hanna, MG .
LANCET, 2001, 358 (9284) :801-807
[20]   CRANIAL COMPUTED TOMOGRAPHIC AND ELECTROENCEPHALOGRAPHIC ABNORMALITIES IN CHILDREN WITH POST-HEMICONVULSIVE HEMIPLEGIA [J].
KATAOKA, K ;
OKUNO, T ;
MIKAWA, H ;
HOJO, H .
EUROPEAN NEUROLOGY, 1988, 28 (05) :279-284