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Landau–Kleffner syndrome

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303:, or emotional/behavioral problems. An EEG (electroencephalogram) test is imperative to a diagnosis. Many cases of patients exhibiting LKS will show abnormal electrical brain activity in both the right and left hemispheres of the brain; this is exhibited frequently during sleep. Even though an abnormal EEG reading is common in LKS patients, a relationship has not been identified between EEG abnormalities and the presence and intensity of language problems. In many cases however, abnormalities in the EEG test has preceded language deterioration and improvement in the EEG tracing has preceded language improvement (this occurs in about half of all affected children). Many factors inhibit the reliability of the EEG data: neurologic deficits do not closely follow the maximal EEG changes in time. 251:. This is demonstrated in patients in multiple ways including the inability to recognize familiar noises and the impairment of the ability to lateralize or localize sound. In addition, receptive language is often critically impaired, however in some patients, impairment in expressive language is the most profound. In a study of 77 cases of Landau–Kleffner syndrome, 6 were found to have this type of aphasia. Because this syndrome appears during such a critical period of language acquisition in a child's life, speech production may be affected just as severely as language comprehension. The onset of LKS is typically between 18 months and 13 years, the most predominant time of emergence being between 3 and 7 years. 177: 33: 501:
with LKS consists of a neurologist, a neuropsychologist, and a speech pathologist or audiologist. Some children with behavioral problems may also need to see a child psychologist and a psychopharmacologist. Speech therapy begins immediately at the time of diagnosis along with medical treatment that may include steroids and anti-epileptic or anti-convulsant medications.
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Various hospitals contain programs designed to treat conditions such as LKS like the Children's Hospital Boston and its Augmentative Communication Program. It is known internationally for its work with children or adults who are non-speaking or severely impaired. Typically, a care team for children
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used 7 patients in his study (all males, aged 8–27 years of age) with LKS. On long-term followup, most of his patients did not demonstrate total epilepsy remission and language problems continued. Out of the seven patients, one reported a normal quality of life while the other six reported aphasia
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Often, behavioral and neuropsychologic disturbances accompany the progression of LKS. Behavioral issues are seen in as many as 78% of all cases. Hyperactivity and a decreased attention span are observed in as many as 80% of patients as well as rage, aggression, and anxiety. These behavior patterns
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The prognosis for children with LKS varies. Some affected children may have a permanent severe language disorder, while others may regain much of their language abilities (although it may take months or years). In some cases, remission and relapse may occur. The prognosis is improved when the onset
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Most cases of LKS do not have a known cause. Occasionally, the condition may be induced secondary to other diagnoses, such as low-grade brain tumors, closed-head injuries, Neurocysticercosis, and Demyelinating Disease. Central Nervous System vasculitis may be associated with this condition as well.
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in which results were reported for 14 patients with acquired epileptic aphasia who underwent multiple subpial transections. Seven of the fourteen patients recovered age-appropriate speech and no longer required speech therapy. Another 4 of the 14 displayed improvement of speech and understanding
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Seizures, especially during the night, are a heavily weighted indicator of LKS. The prevalence of clinical seizures in acquired epileptic aphasia (LKS) is 70–85%. In one third of patients, only a single episode of a seizure was recorded. The seizures typically appear between the ages of 4 and 10
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The most effective way of confirming LKS is by obtaining overnight sleep EEGs, including EEGs in all stages of sleep. Many conditions like demyelination and brain tumors can be ruled out by using magnetic resonance imaging (MRI). In LKS, fluorodeoxyglucose (FDG) and positron emission tomography
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Typically, children with LKS develop normally, but then lose their language skills. While many affected individuals have clinical seizures, some only have electrographic seizures, including electrographic status epilepticus of sleep (ESES). The first indication of the language problem is usually
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Generally, earlier manifestation of the disease correlates with poorer language recovery, and with the appearance of night seizures that last for longer than 36 months. LKS has a wide range of symptom differences and lacks a uniformity in diagnostic criteria between cases, and many studies don't
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of the disorder is after age 6 and when speech therapy is started early. Seizures generally disappear by adulthood. Short-term remissions are not uncommon in LKS but they create difficulties in evaluating a patient's response to various therapeutic modalities.
461:, which should be started early. Some patients improve with the use of corticosteroids or adrenocorticotropin hormone (ACTH) which lead researches to believe that inflammation and vasospasm may play a role in some cases of acquired epileptic aphasia. 1346: 1331: 656:
Globally, more than 200 cases of acquired epileptic aphasia have been described in the literature. Between 1957 and 1980, 81 cases of acquired epileptic aphasia were reported, with 100 cases generally being diagnosed every 10 years.
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study is one of few that features long-term follow up reports of LKS and utilizes EEG testing, MRIs, the Vineland Adaptive Behavior Scales, the Connor's Rating Scales-revised, and a Short-Form Health Survey to analyze its patients.
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Patient education has also proved to be helpful in treating LKS. Teaching them sign language is a helpful means of communication and if the child was able to read and write before the onset of LKS, that is extremely helpful too.
1457: 488:. The cortex is sliced in parallel lines to the midtemporal gyrus and perisylvian area to attenuate the spread of the epileptiform activity without causing cortical dysfunction. There is a study by Morrell 673: 1450: 1242: 1443: 243:). The disorder usually occurs in children between the ages of 3 and 7 years. There appears to be a male dominance in the diagnosis of the syndrome (ratio of 1.7:1, men to women). 258:
Language deterioration in patients typically occurs over a period of weeks or months. However, acute onset of the condition has also been reported as well as episodic aphasia.
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Most cases of LKS do not have a known cause. Occasionally, the condition may be induced secondary to other diagnoses such as low-grade brain tumors, closed-head injury,
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are considered secondary to the language impairment in LKS. Impaired short-term memory is a feature recorded in long-standing cases of acquired epileptic aphasia.
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instructions given verbally, but they still required speech therapy. Eleven patients had language dysfunction for two or more years. Another study by Sawhney
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Sinclair DB, Snyder TJ (May 2005). "Corticosteroids for the treatment of Landau–Kleffner syndrome and continuous spike-wave discharge during sleep".
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The table below demonstrates the extensive and differential diagnosis of acquired epileptic aphasia along with Cognitive and Behavioral Regression:
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Morrell F, Whisler WW, Smith MC, et al. (December 1995). "Landau-Kleffner syndrome. Treatment with subpial intracortical transection".
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The following table demonstrate the Long-Term Follow-up of Acquired Epileptic Aphasia across many different instrumental studies:.
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Note: EEG = electroencephalographic; ESES = electrical status epilepticus of sleep; RL = receptive language; S = sociability
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Tütüncüoğlu S, Serdaroğlu G, Kadioğlu B (October 2002). "Landau-Kleffner syndrome beginning with stuttering: case report".
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reported improvement in all three of their patients with acquired epileptic aphasia who underwent the same procedure.
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Duran MH, Guimarães CA, Medeiros LL, Guerreiro MM (January 2009). "Landau-Kleffner syndrome: long-term follow-up".
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Landau WM, Kleffner FR (November 1998). "Syndrome of acquired aphasia with convulsive disorder in children. 1957".
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include follow-ups on the patients, so no other relationships between symptoms and recovery have been made known.
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The Landau–Kleffner syndrome is characterized by the sudden or gradual development of
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Lower rates of good outcomes have been reported, ranging between 14% and 50%. Duran
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Expressive language, RL, S, verbal and nonverbal communication, possibly behavioral
1377: 933: 235:(EEG). LKS affects the parts of the brain that control comprehension and speech ( 1581: 32: 1388: 1293: 1120: 1586: 1553: 1418: 1340: 1088: 1071: 454: 202: 170: 287:, pervasive developmental disorder, hearing impairment, learning disability, 1407: 771: 477: 166: 1301: 1212: 1097: 1056: 1015: 980: 941: 862: 743: 1177: 1159: 1128: 779: 436:
Continuous spike and wave of slow-wave sleep (>85% of slow-wave sleep).
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Spike-and-wave discharges on the left temporocentral and frontal regions
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Sawhney IM, Robertson IJ, Polkey CE, Binnie CD, Elwes RD (March 1995).
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A controversial treatment option involves a surgical technique called
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The syndrome can be difficult to diagnose and may be misdiagnosed as
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Santos LH, Antoniuk SA, Rodrigues M, Bruno S, Bruck I (June 2002).
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Developmental dysphasia (developmental expressive language disease)
141: 473: 231:(the inability to understand or express language) and an abnormal 219:, who characterized it in 1957 with a diagnosis of six children. 1518: 1366: 965:"Landau–Kleffner syndrome: an analysis of 10 cases in Venezuela" 481: 1741: 1439: 692:"Landau Kleffner Syndrome - Symptoms, Causes, Treatment | NORD" 807:"Landau-Kleffner Syndrome (LKS or Infantile Acquired Aphasia)" 357:
Expressive language, RL, S, verbal and nonverbal communication
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Expressive language, RL, S, verbal and nonverbal communication
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Treatment for LKS usually consists of medications, such as
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Rotenberg J, Pearl PL (2003). "Landau–Kleffner syndrome".
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Number of Patients with Normal or Mild Language Problems
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Left or right temporal or parietal spikes, possibly ESES
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National Institute of Neurological Disorders and Stroke
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and disappear before adulthood (around the age of 15).
1313: 1144:"Multiple subpial transection: a review of 21 cases" 2120: 2084: 2030: 2007: 1949: 1883: 1874: 1851: 1820: 1779: 1714: 1648: 1623: 1602: 1567: 1484: 1477: 1398: 1317: 827:Pearl PL, Carrazana EJ, Holmes GL (November 2001). 160: 134: 57:. Unsourced material may be challenged and removed. 907:Medscape Part of WebMD Health Professional Network 814:Medscape part of WebMD Health Professional Network 1070:Grote CL, Van Slyke P, Hoeppner JA (March 1999). 468:in which multiple incisions are made through the 963:Guevara-Campos J, González-de Guevara L (2007). 1031:"Landau–Kleffner syndrome: study of four cases" 423:Expressive language, no verbal-auditory agnosia 1753: 1451: 801: 799: 797: 149:Landau–Kleffner syndrome is characterized by 8: 1524:Mixed receptive-expressive language disorder 894: 892: 390:Expressive language, RL, possibly behavioral 1239:"Landau–Kleffner syndrome information page" 890: 888: 886: 884: 882: 880: 878: 876: 874: 872: 401:No; lack of expressive language acquisition 1880: 1760: 1746: 1738: 1481: 1458: 1444: 1436: 1314: 175: 140: 131: 1167: 1087: 1046: 852: 733: 648:to be a substantial struggle. The Duran 117:Learn how and when to remove this message 2128:Citizens United for Research in Epilepsy 519: 328: 293:attention deficit hyperactivity disorder 666: 716:Landau WM, Kleffner FR (August 1957). 1666:Learning problems in childhood cancer 379:Expressive language, oromotor apraxia 376:Acquired expressive epileptic aphasia 7: 2045:Dentatorubral–pallidoluysian atrophy 409:Disintegrative epileptiform disorder 55:adding citations to reliable sources 2092:Sudden unexpected death in epilepsy 1610:Developmental coordination disorder 1008:10.1016/j.pediatrneurol.2004.12.006 766:(5): 1241, 8 pages following 1241. 2022:Complex partial status epilepticus 25: 2107:Psychogenic non-epileptic seizure 1979:Benign familial neonatal seizures 1931:Sleep-related hypermotor epilepsy 2189:Specific developmental disorders 1471:specific developmental disorders 1148:J. Neurol. Neurosurg. Psychiatry 845:10.1046/j.1535-7597.2001.00012.x 343:Autistic epileptiform regression 199:aphasia with convulsive disorder 31: 1048:10.1590/s0004-282x2002000200010 42:needs additional citations for 2067:Early myoclonic encephalopathy 2040:Progressive myoclonus epilepsy 1615:Developmental verbal dyspraxia 1592:Disorder of written expression 1534:Speech and language impairment 1205:10.1016/j.braindev.2008.09.007 829:"The Landau–Kleffner Syndrome" 187:Landau–Kleffner syndrome (LKS) 1: 291:/verbal processing disorder, 2017:Epilepsia partialis continua 1631:Auditory processing disorder 1529:Specific language impairment 1499:Expressive language disorder 1245:. 2007-02-13. Archived from 934:10.1177/08830738020170101808 900:"Acquired Epileptic Aphasia" 679:Dorland's Medical Dictionary 472:of the affected part of the 466:multiple subpial transection 404:Temporal or parietal spikes 2072:Juvenile myoclonic epilepsy 2050:Unverricht–Lundborg disease 1636:Sensory processing disorder 555:Mantovani and Landau (1980) 2210: 1989:Myoclonic astatic epilepsy 1294:10.1001/archneur.60.7.1019 1262:"Landau–Kleffner syndrome" 922:Journal of Child Neurology 674:"Landau–Kleffner syndrome" 365:Acquired epileptic aphasia 195:acquired epileptic aphasia 191:infantile acquired aphasia 66:"Landau–Kleffner syndrome" 2140:Epilepsy Action Australia 148: 139: 2102:Landau–Kleffner syndrome 1936:Panayiotopoulos syndrome 1681:Multisensory integration 1509:Landau–Kleffner syndrome 1424:Landau–Kleffner syndrome 1121:10.1093/brain/118.6.1529 1115:. 118 ( Pt 6): 1529–46. 484:tracts in the subjacent 135:Landau–Kleffner syndrome 18:Landau-Kleffner syndrome 1984:Lennox–Gastaut syndrome 1866:Epilepsy and employment 1089:10.1093/brain/122.3.561 772:10.1212/wnl.51.5.1241-a 368:RL, possibly behavioral 297:intellectual disability 249:auditory verbal agnosia 1916:Temporal lobe epilepsy 1836:Electroencephalography 1676:Management of dyslexia 597:Robinson et al. (2001) 382:Centrotemporal spikes 349:Centrotemporal spikes 322:Differential diagnosis 1921:Frontal lobe epilepsy 1549:Speech sound disorder 1160:10.1136/jnnp.58.3.344 316:demyelinating disease 201:—is a rare childhood 2157:Epilepsy Research UK 1941:Vertiginous epilepsy 1861:Epilepsy and driving 1812:Epilepsy in children 1727:People with dyslexia 233:electroencephalogram 51:improve this article 2145:Epilepsy Foundation 2112:Epilepsy in animals 1792:Aura (warning sign) 1722:Dyslexia in fiction 1696:Reading acquisition 1569:Learning disability 735:10.1212/wnl.7.8.523 354:Autistic regression 2032:Myoclonic epilepsy 2009:Status epilepticus 1399:External resources 1035:Arq Neuropsiquiatr 526:Number of Patients 312:neurocysticercosis 223:Signs and symptoms 211:It is named after 2171: 2170: 2085:Related disorders 2080: 2079: 1926:Rolandic epilepsy 1735: 1734: 1656:Dyslexia research 1644: 1643: 1559:Tip of the tongue 1514:Language disorder 1433: 1432: 641: 640: 529:Mean Follow-up, y 430: 429: 184: 183: 129:Medical condition 127: 126: 119: 101: 16:(Redirected from 2201: 2162:Epilepsy Society 2151:Epilepsy Outlook 1994:Epileptic spasms 1906:Gelastic seizure 1881: 1807:Neonatal seizure 1762: 1755: 1748: 1739: 1504:Infantile speech 1486:Speech, language 1482: 1460: 1453: 1446: 1437: 1315: 1305: 1276: 1274: 1273: 1257: 1255: 1254: 1225: 1224: 1188: 1182: 1181: 1171: 1139: 1133: 1132: 1108: 1102: 1101: 1091: 1067: 1061: 1060: 1050: 1026: 1020: 1019: 991: 985: 984: 960: 954: 953: 917: 911: 910: 904: 896: 867: 866: 856: 824: 818: 817: 811: 803: 792: 791: 755: 737: 713: 707: 706: 704: 702: 696:rarediseases.org 688: 682: 671: 520: 329: 180: 179: 153:and an abnormal 144: 132: 122: 115: 111: 108: 102: 100: 59: 35: 27: 21: 2209: 2208: 2204: 2203: 2202: 2200: 2199: 2198: 2174: 2173: 2172: 2167: 2134:Epilepsy Action 2116: 2076: 2026: 2003: 1999:Febrile seizure 1964:Absence seizure 1945: 1901:Complex partial 1870: 1853:Personal issues 1847: 1832:Investigations 1828:Anticonvulsants 1816: 1802:Epileptogenesis 1797:Postictal state 1775: 1766: 1736: 1731: 1710: 1686:Neuropsychology 1640: 1619: 1598: 1563: 1539:Speech disorder 1489: 1473: 1464: 1434: 1429: 1428: 1394: 1393: 1326: 1312: 1279: 1271: 1269: 1260: 1252: 1250: 1237: 1234: 1232:Further reading 1229: 1228: 1190: 1189: 1185: 1141: 1140: 1136: 1110: 1109: 1105: 1069: 1068: 1064: 1041:(2–A): 239–41. 1028: 1027: 1023: 996:Pediatr. 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1040: 1036: 1032: 1025: 1022: 1017: 1013: 1009: 1005: 1001: 997: 990: 987: 982: 978: 975:(11): 652–6. 974: 970: 966: 959: 956: 951: 947: 943: 939: 935: 931: 928:(10): 785–8. 927: 923: 916: 913: 909:. 2019-03-05. 908: 903:(medicinenet) 901: 895: 893: 891: 889: 887: 885: 883: 881: 879: 877: 875: 873: 869: 864: 860: 855: 850: 846: 842: 838: 834: 833:Epilepsy Curr 830: 823: 820: 815: 810:(medicinenet) 808: 802: 800: 798: 794: 789: 785: 781: 777: 773: 769: 765: 761: 753: 749: 745: 741: 736: 731: 728:(8): 523–30. 727: 723: 719: 712: 709: 697: 693: 687: 684: 681: 680: 675: 670: 667: 660: 658: 654: 651: 646: 636: 634: 631: 628: 627: 623: 620: 617: 614: 610: 609: 605: 602: 599: 596: 595: 591: 588: 585: 582: 581: 577: 574: 571: 568: 567: 563: 560: 557: 554: 553: 549: 546: 543: 540: 536: 535: 531: 528: 525: 522: 521: 518: 515: 508: 506: 502: 498: 496: 491: 487: 483: 479: 475: 471: 467: 462: 460: 456: 452: 448: 440: 435: 434: 433: 425: 422: 419: 418: 414: 411: 408: 407: 403: 400: 397: 396: 392: 389: 386: 385: 381: 378: 375: 374: 370: 367: 364: 363: 359: 356: 353: 352: 348: 345: 342: 341: 338:EEG Patterns 337: 335:Deterioration 334: 331: 330: 327: 321: 319: 317: 313: 308: 304: 302: 301:schizophrenia 298: 294: 290: 286: 278: 276: 269: 267: 263: 259: 256: 252: 250: 244: 242: 238: 234: 230: 222: 220: 218: 214: 209: 207: 204: 200: 196: 192: 189:—also called 188: 178: 172: 168: 165: 163: 159: 156: 152: 147: 143: 138: 133: 121: 118: 110: 107:February 2015 99: 96: 92: 89: 85: 82: 78: 75: 71: 68: –  67: 63: 62:Find sources: 56: 52: 46: 45: 40:This article 38: 34: 29: 28: 19: 2101: 1959:Tonic–clonic 1544:Speech error 1508: 1469:and related 1417: 1406: 1382: 1371: 1360: 1345: 1330: 1285: 1281: 1270:. Retrieved 1266:the original 1251:. Retrieved 1247:the original 1199:(1): 58–63. 1196: 1192: 1186: 1154:(3): 344–9. 1151: 1147: 1137: 1112: 1106: 1082:(3): 561–6. 1079: 1075: 1065: 1038: 1034: 1024: 1002:(5): 300–6. 999: 995: 989: 972: 968: 958: 925: 921: 915: 906: 839:(2): 39–45. 836: 832: 822: 813: 763: 759: 725: 721: 711: 699:. Retrieved 695: 686: 677: 669: 655: 649: 644: 642: 612: 583:Rossi (1999) 538: 516: 512: 503: 499: 494: 489: 486:white matter 476:beneath the 463: 444: 431: 325: 309: 305: 299:, childhood 282: 273: 264: 260: 257: 253: 245: 237:Broca's area 226: 210: 203:neurological 198: 194: 190: 186: 185: 113: 104: 94: 87: 80: 73: 61: 49:Please help 44:verification 41: 1951:Generalised 1582:Dyscalculia 1282:Arch Neurol 637:18 (28.6%) 2178:Categories 1974:Automatism 1821:Management 1587:Dysgraphia 1554:Stuttering 1478:Conditions 1419:Patient UK 1384:DiseasesDB 1272:2007-08-23 1253:2007-08-23 969:Rev Neurol 661:References 455:prednisone 420:Stuttering 171:psychiatry 77:newspapers 2194:Syndromes 1413:neuro/182 1408:eMedicine 1193:Brain Dev 760:Neurology 722:Neurology 509:Prognosis 478:pia mater 453:(such as 441:Treatment 332:Diagnosis 279:Diagnosis 167:Neurology 162:Specialty 1912:Epilepsy 1892:Seizures 1773:epilepsy 1769:Seizures 1701:Spelling 1671:Literacy 1577:Dyslexia 1467:Dyslexia 1302:12873863 1221:25248883 1213:18930363 1098:10094262 1057:12068352 1016:15866429 981:17557221 942:12546439 863:15309183 788:45332481 744:13451887 701:21 April 537:Soprano 289:auditory 206:syndrome 1624:Sensory 1378:D018887 1178:7897419 1169:1073374 1129:8595482 950:3173640 780:9867583 752:2093377 457:), and 360:Normal 229:aphasia 151:aphasia 91:scholar 1780:Basics 1488:, and 1367:245570 1300:  1219:  1211:  1176:  1166:  1127:  1096:  1055:  1014:  979:  948:  940:  861:  854:320814 851:  786:  778:  750:  742:  650:et al. 645:et al. 615:(2009) 613:et al. 611:Duran 541:(1994) 539:et al. 495:et al. 490:et al. 482:axonal 470:cortex 387:(ESES) 314:, and 285:autism 173:  93:  86:  79:  72:  64:  1885:Focal 1715:Lists 1603:Motor 1389:31407 1356:345.8 1341:F80.3 1217:S2CID 1113:Brain 1076:Brain 946:S2CID 784:S2CID 748:S2CID 629:Total 523:Study 474:brain 415:ESES 393:ESES 270:Cause 98:JSTOR 84:books 2164:(UK) 2153:(UK) 2147:(US) 2136:(UK) 2130:(US) 1771:and 1519:Lisp 1373:MeSH 1362:OMIM 1351:9-CM 1298:PMID 1209:PMID 1174:PMID 1125:PMID 1094:PMID 1053:PMID 1012:PMID 977:PMID 938:PMID 859:PMID 776:PMID 740:PMID 703:2024 449:and 239:and 215:and 70:news 1347:ICD 1332:ICD 1290:doi 1201:doi 1164:PMC 1156:doi 1117:doi 1084:doi 1080:122 1043:doi 1004:doi 930:doi 849:PMC 841:doi 768:doi 730:doi 676:at 621:9.5 603:5.6 589:9.7 575:8.1 197:or 155:EEG 53:by 2180:: 1422:: 1411:: 1387:: 1376:: 1365:: 1354:: 1339:: 1336:10 1296:. 1286:60 1284:. 1241:. 1215:. 1207:. 1197:31 1195:. 1172:. 1162:. 1152:58 1150:. 1146:. 1123:. 1092:. 1078:. 1074:. 1051:. 1039:60 1037:. 1033:. 1010:. 1000:32 998:. 973:44 967:. 944:. 936:. 926:17 924:. 905:. 871:^ 857:. 847:. 835:. 831:. 812:. 796:^ 782:. 774:. 764:51 762:. 746:. 738:. 724:. 720:. 694:. 632:63 624:1 606:3 600:18 592:2 586:11 578:3 564:6 561:22 550:3 544:12 295:, 208:. 193:, 169:, 1761:e 1754:t 1747:v 1459:e 1452:t 1445:v 1349:- 1334:- 1324:D 1304:. 1292:: 1275:. 1256:. 1223:. 1203:: 1180:. 1158:: 1131:. 1119:: 1100:. 1086:: 1059:. 1045:: 1018:. 1006:: 983:. 952:. 932:: 865:. 843:: 837:1 816:. 790:. 770:: 754:. 732:: 726:7 705:. 618:7 572:6 558:9 547:8 120:) 114:( 109:) 105:( 95:· 88:· 81:· 74:· 47:. 20:)

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Landau-Kleffner syndrome

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aphasia
EEG
Specialty
Neurology
psychiatry
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neurological
syndrome
William Landau
Frank Kleffner
aphasia
electroencephalogram
Broca's area
Wernicke's area
auditory verbal agnosia
autism
auditory

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