63:
32:
441:
Poduri, Annapurna; Evrony, Gilad D.; Cai, Xuyu; Elhosary, Princess
Christina; Beroukhim, Rameen; Lehtinen, Maria K.; Hills, L. Benjamin; Heinzen, Erin L.; Hill, Anthony; Hill, R. Sean; Barry, Brenda J.; Bourgeois, Blaise F.D.; Riviello, James J.; Barkovich, A. James; Black, Peter M.; Ligon, Keith L.;
794:
716:
787:
235:
Surgery is usually performed as soon as possible to minimize damage caused by seizures. However, a trial with drugs can be attempted for a few months before surgery, and there is a slim chance of it succeeding. Because
780:
146:
Seizures are the main symptom. There can be as many as hundreds of seizures a day. Seizures tend to begin soon after birth, but may sometimes commence during later infancy or, rarely, during early childhood.
134:
affecting all or a part of a cerebral hemisphere. It causes severe seizures, which are often frequent and hard to control. A minority might have seizure control with medicines, but most will need
240:
decreases with age, the earlier in life the surgery is completed, the more likely it is that the remaining hemisphere will adapt to perform tasks previously completed by the missing hemisphere.
1080:
355:
1550:
1535:
1073:
1329:
1066:
1136:
979:
1131:
553:
1231:
212:, the affected part is distorted and enlarged. It can be diagnosed prenatally, but a lot of cases go undiagnosed until seizures begin.
1344:
1269:
196:
formation. The excessive proliferation is postulated to occur early and to possibly continue beyond the normal proliferative period.
1369:
1334:
1540:
931:
1431:
138:
as the best chance. Uncontrolled, they often cause progressive intellectual disability and brain damage and stop development.
1279:
1236:
1126:
1339:
1221:
1459:
1251:
954:
1395:
1191:
1146:
654:
Trounce, J. Q.; Rutter, N.; Mellor, D. H. (1991). "Hemimegalencephaly: diagnosis and treatment. - Semantic
Scholar".
1294:
1201:
1390:
1274:
888:
1349:
1186:
1098:
731:
772:
382:
314:
Sato, N; Yagishita, A; Oba, H; Miki, Y; Nakata, Y; Yamashita, F; Nemoto, K; Sugai, K; Sasaki, M (April 2007).
1259:
197:
1264:
600:
1545:
1089:
760:
1498:
1486:
1470:
1442:
1166:
1106:
936:
1454:
1438:
1426:
1414:
62:
1321:
1309:
862:
803:
131:
1156:
1116:
1033:
1004:
912:
824:
679:
636:
200:
is thought to play an important role in the excessive proliferation and the pathogenesis of HME.
71:
570:
491:
Abdel Razek, A.A.K.; Kandell, A.Y.; Elsorogy, L.G.; Elmongy, A.; Basett, A.A. (7 August 2008).
1419:
1206:
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1121:
902:
671:
628:
620:
575:
549:
543:
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417:
337:
296:
47:
40:
1503:
1479:
1359:
1354:
1216:
1161:
1038:
663:
612:
514:
504:
463:
455:
327:
286:
276:
265:"Cranio-orbital-temporal neurofibromatosis: an uncommon subtype of neurofibromatosis type-1"
237:
229:
224:
Although there have been a few reports of medical treatment, the main treatment is radical:
1491:
1475:
1463:
1447:
1374:
1241:
1226:
1211:
922:
243:
225:
193:
135:
316:"Hemimegalencephaly: a study of abnormalities occurring outside the involved hemisphere"
208:
It should be suspected in infants or children with intractable, frequent seizures. On a
1512:
1364:
1111:
974:
965:
941:
807:
667:
519:
492:
468:
443:
332:
291:
264:
124:
1529:
1178:
1043:
907:
883:
857:
616:
189:
683:
1284:
1058:
1017:
1012:
960:
878:
850:
640:
128:
56:
736:
459:
444:"Somatic Activation of AKT3 Causes Hemispheric Developmental Brain Malformations"
1304:
995:
832:
815:
1299:
917:
725:
213:
105:
624:
281:
845:
52:
528:
477:
341:
300:
31:
708:
675:
632:
1288:
1151:
755:
840:
509:
315:
209:
228:. However, it has a high mortality , and there have been reports of a
720:
599:
Bonioli, Eugenio; Palmieri, Antonella; Bellini, Carlo (1994-03-01).
601:"Surgical vs. medical treatment of seizures in hemimegalencephaly"
188:
It is a disorder related to excessive neuronal proliferation and
176:
1062:
776:
356:"Hemimegalencephaly - Why hemispherectomy is usually required"
263:
Acharya N, Reddy MS, Paulson CT, Prasanna D (January 2014).
232:
and seizures resuming, this time in the healthy hemisphere.
571:"Radical surgery offers hope for baby racked by seizures"
16:
Disorder affecting development of one side of the brain
542:
Harvey B. Sarnat; Paolo
Curatolo (26 September 2007).
493:"Disorders of Cortical Formation: MR Imaging Features"
180:
causes hemispheric developmental brain malformations.
698:
1404:
1383:
1320:
1250:
1177:
1097:
1026:
1003:
994:
871:
823:
814:
746:
702:
136:
removal or disconnection of the affected hemisphere
104:
96:
88:
80:
70:
46:
24:
39:Left-sided hemimegalencephaly in a person with
383:"Gene Mutations Cause Massive Brain Asymmetry"
1074:
788:
418:"Hemimegalencephaly & Cortical Dysplasia"
76:Frequent seizures often resistant to medicine
8:
216:can display asymmetrical brain hemispheres.
1081:
1067:
1059:
1000:
820:
795:
781:
773:
699:
656:Developmental Medicine and Child Neurology
61:
30:
21:
518:
508:
467:
331:
290:
280:
320:AJNR. American Journal of Neuroradiology
980:Bilateral frontoparietal polymicrogyria
255:
1551:Syndromes affecting the nervous system
1536:Congenital disorders of nervous system
226:remove or disconnect the affected side
165:Progressive blindness of half the body
594:
592:
162:Progressive weakness of half the body
7:
569:Hennessy-Fiske, Molly (2012-06-15).
442:Walsh, Christopher A. (April 2012).
412:
410:
408:
406:
404:
402:
377:
375:
545:Malformations of the Nervous System
806:malformations and deformations of
668:10.1111/j.1469-8749.1991.tb05116.x
497:American Journal of Neuroradiology
14:
1330:Bannayan–Riley–Ruvalcaba syndrome
932:Agenesis of the corpus callosum
1137:Bonnet–Dechaume–Blanc syndrome
1:
1132:Sakati–Nyhan–Tisdale syndrome
269:Oman Journal of Ophthalmology
156:Asymmetrical or enlarged head
1370:Tatton-Brown–Rahman syndrome
1340:Benign symmetric lipomatosis
617:10.1016/0387-7604(94)90060-4
460:10.1016/j.neuron.2012.03.010
422:hemifoundation.homestead.com
360:www.brainrecoveryproject.org
246:might control the seizures.
1460:Branchio-oto-renal syndrome
1335:Beckwith–Wiedemann syndrome
898:other reduction deformities
1567:
1432:Zimmermann–Laband syndrome
1384:Laurence–Moon–Bardet–Biedl
1345:Klippel–Trénaunay syndrome
1295:Caudal regression syndrome
1270:Klippel–Trénaunay syndrome
1232:Smith–Lemli–Opitz syndrome
1202:Cornelia de Lange syndrome
1280:Rubinstein–Taybi syndrome
192:overgrowth affecting the
38:
29:
1350:Neurofibromatosis type I
1237:Snyder–Robinson syndrome
1187:1q21.1 deletion syndrome
1127:Saethre–Chotzen syndrome
884:Congenital hydrocephalus
387:UC Health - UC San Diego
282:10.4103/0974-620X.127934
1260:Adducted thumb syndrome
1222:Silver–Russell syndrome
548:. Newnes. p. 154.
198:Epidermal growth factor
1541:Neurological disorders
1396:Laurence–Moon syndrome
1192:Aarskog–Scott syndrome
1147:Baller–Gerold syndrome
1090:Congenital abnormality
174:Somatic activation of
1391:Bardet–Biedl syndrome
1275:Nail–patella syndrome
1167:Pierre Robin sequence
1107:Acrocephalosyndactyly
937:Septo-optic dysplasia
889:Dandy–Walker syndrome
605:Brain and Development
1322:Overgrowth syndromes
110:Anti-epileptic drugs
1310:VACTERL association
863:Chiari malformation
159:Developmental delay
132:congenital disorder
1265:Holt–Oram syndrome
1157:Goldenhar syndrome
1117:Carpenter syndrome
1034:Currarino syndrome
1005:Neural tube defect
947:Hemimegalencephaly
913:Microlissencephaly
825:Neural tube defect
747:External resources
510:10.3174/ajnr.A1223
142:Symptoms and signs
117:Hemimegalencephaly
25:Hemimegalencephaly
1523:
1522:
1420:Feingold syndrome
1207:Dubowitz syndrome
1197:Cockayne syndrome
1122:Pfeiffer syndrome
1056:
1055:
1052:
1051:
990:
989:
903:Holoprosencephaly
770:
769:
576:Los Angeles Times
555:978-0-08-055984-1
123:), or unilateral
114:
113:
41:neurofibromatosis
19:Medical condition
1558:
1504:Donohue syndrome
1480:Timothy syndrome
1360:Proteus syndrome
1355:Perlman syndrome
1217:Robinow syndrome
1162:Moebius syndrome
1083:
1076:
1069:
1060:
1039:Diastomatomyelia
1001:
821:
797:
790:
783:
774:
700:
688:
687:
651:
645:
644:
596:
587:
586:
584:
583:
566:
560:
559:
539:
533:
532:
522:
512:
488:
482:
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471:
438:
432:
431:
429:
428:
414:
397:
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393:
379:
370:
369:
367:
366:
352:
346:
345:
335:
311:
305:
304:
294:
284:
260:
238:brain plasticity
230:vegetative state
66:
65:
34:
22:
1566:
1565:
1561:
1560:
1559:
1557:
1556:
1555:
1526:
1525:
1524:
1519:
1492:Marfan syndrome
1476:Keutel syndrome
1464:CHARGE syndrome
1448:Fraser syndrome
1406:
1405:Combined/other,
1400:
1379:
1375:Weaver syndrome
1316:
1246:
1242:Turner syndrome
1227:Seckel syndrome
1212:Noonan syndrome
1173:
1093:
1087:
1057:
1048:
1022:
986:
923:Hydranencephaly
867:
810:
801:
771:
766:
765:
742:
741:
711:
697:
692:
691:
653:
652:
648:
598:
597:
590:
581:
579:
568:
567:
563:
556:
541:
540:
536:
490:
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485:
440:
439:
435:
426:
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364:
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313:
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308:
262:
261:
257:
252:
244:Benzodiazepines
222:
206:
186:
184:Pathophysiology
172:
153:
144:
100:Hemispherectomy
60:
20:
17:
12:
11:
5:
1564:
1562:
1554:
1553:
1548:
1543:
1538:
1528:
1527:
1521:
1520:
1518:
1517:
1516:
1515:
1513:Fryns syndrome
1507:
1495:
1483:
1467:
1451:
1435:
1423:
1410:
1408:
1402:
1401:
1399:
1398:
1393:
1387:
1385:
1381:
1380:
1378:
1377:
1372:
1367:
1365:Sotos syndrome
1362:
1357:
1352:
1347:
1342:
1337:
1332:
1326:
1324:
1318:
1317:
1315:
1314:
1313:
1312:
1307:
1302:
1297:
1282:
1277:
1272:
1267:
1262:
1256:
1254:
1248:
1247:
1245:
1244:
1239:
1234:
1229:
1224:
1219:
1214:
1209:
1204:
1199:
1194:
1189:
1183:
1181:
1175:
1174:
1172:
1171:
1170:
1169:
1164:
1159:
1154:
1149:
1141:
1140:
1139:
1134:
1129:
1124:
1119:
1114:
1112:Apert syndrome
1103:
1101:
1095:
1094:
1088:
1086:
1085:
1078:
1071:
1063:
1054:
1053:
1050:
1049:
1047:
1046:
1041:
1036:
1030:
1028:
1024:
1023:
1021:
1020:
1015:
1009:
1007:
998:
992:
991:
988:
987:
985:
984:
983:
982:
975:Polymicrogyria
971:
970:
969:
968:
966:Schizencephaly
963:
950:
949:
944:
942:Megalencephaly
939:
934:
928:
927:
926:
925:
920:
915:
910:
905:
894:
893:
892:
891:
881:
875:
873:
869:
868:
866:
865:
860:
855:
854:
853:
848:
843:
838:
829:
827:
818:
812:
811:
808:nervous system
802:
800:
799:
792:
785:
777:
768:
767:
764:
763:
751:
750:
748:
744:
743:
740:
739:
728:
712:
707:
706:
704:
703:Classification
696:
695:External links
693:
690:
689:
646:
588:
561:
554:
534:
483:
433:
398:
371:
347:
306:
254:
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251:
248:
221:
218:
205:
202:
185:
182:
171:
168:
167:
166:
163:
160:
157:
152:
151:Other symptoms
149:
143:
140:
125:megalencephaly
112:
111:
108:
102:
101:
98:
94:
93:
90:
86:
85:
82:
78:
77:
74:
68:
67:
50:
44:
43:
36:
35:
27:
26:
18:
15:
13:
10:
9:
6:
4:
3:
2:
1563:
1552:
1549:
1547:
1546:Rare diseases
1544:
1542:
1539:
1537:
1534:
1533:
1531:
1514:
1511:
1510:
1508:
1505:
1501:
1500:
1496:
1493:
1489:
1488:
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1481:
1477:
1473:
1472:
1468:
1465:
1461:
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1444:
1440:
1436:
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1411:
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1403:
1397:
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1373:
1371:
1368:
1366:
1363:
1361:
1358:
1356:
1353:
1351:
1348:
1346:
1343:
1341:
1338:
1336:
1333:
1331:
1328:
1327:
1325:
1323:
1319:
1311:
1308:
1306:
1303:
1301:
1298:
1296:
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1292:
1290:
1286:
1283:
1281:
1278:
1276:
1273:
1271:
1268:
1266:
1263:
1261:
1258:
1257:
1255:
1253:
1249:
1243:
1240:
1238:
1235:
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1230:
1228:
1225:
1223:
1220:
1218:
1215:
1213:
1210:
1208:
1205:
1203:
1200:
1198:
1195:
1193:
1190:
1188:
1185:
1184:
1182:
1180:
1179:Short stature
1176:
1168:
1165:
1163:
1160:
1158:
1155:
1153:
1150:
1148:
1145:
1144:
1142:
1138:
1135:
1133:
1130:
1128:
1125:
1123:
1120:
1118:
1115:
1113:
1110:
1109:
1108:
1105:
1104:
1102:
1100:
1096:
1091:
1084:
1079:
1077:
1072:
1070:
1065:
1064:
1061:
1045:
1044:Syringomyelia
1042:
1040:
1037:
1035:
1032:
1031:
1029:
1025:
1019:
1016:
1014:
1011:
1010:
1008:
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1002:
999:
997:
993:
981:
978:
977:
976:
973:
972:
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964:
962:
959:
958:
957:
956:
952:
951:
948:
945:
943:
940:
938:
935:
933:
930:
929:
924:
921:
919:
916:
914:
911:
909:
908:Lissencephaly
906:
904:
901:
900:
899:
896:
895:
890:
887:
886:
885:
882:
880:
877:
876:
874:
870:
864:
861:
859:
858:Encephalocele
856:
852:
849:
847:
844:
842:
839:
836:
835:
834:
831:
830:
828:
826:
822:
819:
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813:
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805:
798:
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791:
786:
784:
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778:
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752:
749:
745:
738:
734:
733:
729:
727:
723:
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713:
710:
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701:
694:
685:
681:
677:
673:
669:
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602:
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565:
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547:
546:
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511:
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502:
498:
494:
487:
484:
479:
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453:
449:
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403:
399:
388:
384:
378:
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372:
361:
357:
351:
348:
343:
339:
334:
329:
326:(4): 678–82.
325:
321:
317:
310:
307:
302:
298:
293:
288:
283:
278:
274:
270:
266:
259:
256:
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241:
239:
233:
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227:
219:
217:
215:
211:
203:
201:
199:
195:
191:
190:hamartomatous
183:
181:
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169:
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49:
45:
42:
37:
33:
28:
23:
1497:
1485:
1469:
1453:
1437:
1425:
1413:
1285:Gastrulation
1099:Craniofacial
1018:Rachischisis
1013:Spina bifida
961:Porencephaly
953:
946:
897:
879:Microcephaly
851:Iniencephaly
754:
730:
715:
662:(3): 261–6.
659:
655:
649:
608:
604:
580:. Retrieved
574:
564:
544:
537:
500:
496:
486:
454:(1): 41–48.
451:
447:
436:
425:. Retrieved
421:
390:. Retrieved
386:
363:. Retrieved
359:
350:
323:
319:
309:
272:
268:
258:
242:
234:
223:
207:
187:
175:
173:
145:
120:
116:
115:
57:rheumatology
1407:known locus
1305:Sirenomelia
996:Spinal cord
833:Anencephaly
503:(1): 4–11.
275:(1): 43–5.
81:Usual onset
1530:Categories
1300:Ectromelia
918:Pachygyria
804:Congenital
611:(2): 169.
582:2018-04-12
427:2018-04-14
392:2018-04-12
365:2018-04-12
250:References
214:Ultrasound
106:Medication
84:Congenital
1509:Multiple
1092:syndromes
846:Acalvaria
625:0387-7604
220:Treatment
204:Diagnosis
97:Treatment
92:Long term
53:Neurology
48:Specialty
1289:mesoderm
1152:Cyclopia
955:CNS cyst
837:Acephaly
756:Orphanet
684:45836490
529:18687750
478:22500628
342:17416820
301:24799805
194:cortical
170:Genetics
89:Duration
72:Symptoms
841:Acrania
737:D065705
676:1902803
641:2442788
633:8048711
520:7051699
469:3460551
333:7977333
292:4008903
210:CT scan
127:, is a
1143:Other
682:
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517:
476:
466:
448:Neuron
340:
330:
299:
289:
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1252:Limbs
1027:Other
872:Other
816:Brain
761:99802
726:Q04.5
680:S2CID
637:S2CID
732:MeSH
672:PMID
629:PMID
621:ISSN
550:ISBN
525:PMID
474:PMID
338:PMID
297:PMID
177:AKT3
129:rare
717:ICD
664:doi
613:doi
515:PMC
505:doi
464:PMC
456:doi
328:PMC
287:PMC
277:doi
121:HME
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1487:15
1478:,
1471:12
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