Landau-Kleffner综合征：一例报道及文献复习
Landau-Kleffner Syndrome: A Case Report and Literature Review
DOI: 10.12677/acm.2025.1582313, PDF,    科研立项经费支持
作者: 陈直蔚, 胡禄英, 徐韵婷, 戴燕红, 陈卓铭*：暨南大学附属第一医院康复科，广东 广州
关键词: Landau-Kleffner综合征；癫痫；获得性失语；脑电图；Landau-Kleffner Syndrome； Epilepsy； Acquired Aphasia； EEG
摘要: 目的：探讨Landau-Kleffner综合征(Landau-Kleffner Syndrome, LKS)的病因、临床特征、治疗方法等特点，以提高对该疾病的认识。方法：通过对我院收治的一例LKS患儿的临床资料及短期随访结果进行分析，并结合文献复习进行探讨。结果：患儿表现为急性病程，起病前智力及语言发育正常，无癫痫家族史，表现为失语及脑电图异常(癫痫样放电)，诊断为LKS，经激素、营养神经、改善认知药物治疗及计算机辅助语言认知康复训练后，患儿语言能力及社会适应能力明显改善。结论：Landau-Kleffner综合征是一种罕见的以癫痫发作和获得性失语和脑电图异常为特点的与年龄相关的癫痫综合征，早期干预治疗及通过计算机辅助言语认知康复训练可改善失语状况。
Abstract: Purpose: To explore the etiology, clinical features and treatment of Landau-Kleffner syndrome (LKS), in order to improve the understanding of the disease. Method: The clinical data and short-term follow-up results of a child with LKS admitted to our hospital were analyzed, and the literature was reviewed. Result: The patient presented with acute course of disease, normal intelligence and language development before onset, no family history of epilepsy, aphasia and abnormal EEG (epileptiform discharge), and was diagnosed as LKS. After hormone, nerve nutrition, cognitive drug therapy and computer-assisted language cognitive rehabilitation training, the language ability and social adaptability of the child were significantly improved. Conclusion: Landau-Kleffner syndrome (Landau-Kleffner syndrome) is a rare age-related epilepsy syndrome characterized by seizures and acquired aphasia and abnormal electroencephalogram. Early intervention and computer-assisted speech cognitive rehabilitation training can improve aphasia.
文章引用：陈直蔚, 胡禄英, 徐韵婷, 戴燕红, 陈卓铭. Landau-Kleffner综合征：一例报道及文献复习[J]. 临床医学进展, 2025, 15(8): 897-904. https://doi.org/10.12677/acm.2025.1582313

参考文献

[1] Landau, W.M. and Kleffner, F.R. (1957) Syndrome of Acquired Aphasia with Convulsive Disorder in Children. Neurology, 7, 523-523. [Google Scholar] [CrossRef] [PubMed]
[2] Proposal for Revised Classification of Epilepsies and Epileptic Syndromes (1989) Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia, 30, 389-399.
[3] Engel Jr., J. (2001) A Proposed Diagnostic Scheme for People with Epileptic Seizures and with Epilepsy: Report of the ILAE Task Force on Classification and Terminology. Epilepsia, 42, 796-803. [Google Scholar] [CrossRef] [PubMed]
[4] Kaga, M., Inagaki, M. and Ohta, R. (2014) Epidemiological Study of Landau-Kleffner Syndrome (LKS) in Japan. Brain and Development, 36, 284-286. [Google Scholar] [CrossRef] [PubMed]
[5] Muzio, M.R., Cascella, M. and Khalili Y.A. (2025) Landau-Kleffner Syndrome. StatPearls Publishing.
http://www.ncbi.nlm.nih.gov/books/NBK547745/
[6] Carvill, G.L., Regan, B.M., Yendle, S.C., O’Roak, B.J., Lozovaya, N., Bruneau, N., et al. (2013) GRIN2A Mutations Cause Epilepsy-Aphasia Spectrum Disorders. Nature Genetics, 45, 1073-1076. [Google Scholar] [CrossRef] [PubMed]
[7] Lemke, J.R., Lal, D., Reinthaler, E.M., Steiner, I., Nothnagel, M., Alber, M., et al. (2013) Mutations in GRIN2A Cause Idiopathic Focal Epilepsy with Rolandic Spikes. Nature Genetics, 45, 1067-1072. [Google Scholar] [CrossRef] [PubMed]
[8] Li, X., Xie, L., Han, W., Hong, S., Ma, J., Wang, J., et al. (2020) Clinical Forms and GRIN2A Genotype of Severe End of Epileptic-Aphasia Spectrum Disorder. Frontiers in Pediatrics, 8, Article ID: 574803. [Google Scholar] [CrossRef] [PubMed]
[9] Turner, S.J., Morgan, A.T., Perez, E.R. and Scheffer, I.E. (2015) New Genes for Focal Epilepsies with Speech and Language Disorders. Current Neurology and Neuroscience Reports, 15, Article No. 35. [Google Scholar] [CrossRef] [PubMed]
[10] Yang, X., Qian, P., Xu, X., Liu, X., Wu, X., Zhang, Y., et al. (2018) GRIN2A Mutations in Epilepsy-Aphasia Spectrum Disorders. Brain and Development, 40, 205-210. [Google Scholar] [CrossRef] [PubMed]
[11] Strehlow, V., Heyne, H.O., Vlaskamp, D.R.M., et al. (2019) GRIN2A-Related Disorders: Genotype and Functional Consequence Predict Phenotype. A Journal of Neurology, 142, 80-92.
[12] Husari, K.S. and Dubey, D. (2019) Autoimmune Epilepsy. Neurotherapeutics, 16, 685-702. [Google Scholar] [CrossRef] [PubMed]
[13] Mikati, M.A., Saab, R., Fayad, M.N. and Choueiri, R.N. (2002) Efficacy of Intravenous Immunoglobulin in Landau-Kleffner Syndrome. Pediatric Neurology, 26, 298-300. [Google Scholar] [CrossRef] [PubMed]
[14] Connolly, A.M., Chez, M., Streif, E.M., Keeling, R.M., Golumbek, P.T., Kwon, J.M., et al. (2006) Brain-Derived Neurotrophic Factor and Autoantibodies to Neural Antigens in Sera of Children with Autistic Spectrum Disorders, Landau-Kleffner Syndrome, and Epilepsy. Biological Psychiatry, 59, 354-363. [Google Scholar] [CrossRef] [PubMed]
[15] Gogou, M., Papadopoulou-Alataki, E., Spilioti, M., Alataki, S. and Evangeliou, A. (2017) Clinical Applications of Intravenous Immunoglobulins in Child Neurology. Current Pharmaceutical Biotechnology, 18, 628-637. [Google Scholar] [CrossRef] [PubMed]
[16] Granata, T., Cross, H., Theodore, W. and Avanzini, G. (2011) Immune‐Mediated Epilepsies. Epilepsia, 52, 5-11. [Google Scholar] [CrossRef] [PubMed]
[17] Michałowicz, R., Jóźwiak, S., Szwabowska-Orzeszko, E., et al. (1989) The Landau-Kleffner Syndrome. Wiadomosci Lekarskie, 42, 256-259.
[18] Otero, E., Cordova, S., Diaz, F., Garcia‐Teruel, I. and Brutto, O.H.D. (1989) Acquired Epileptic Aphasia (The Landau‐kleffner Syndrome) Due to Neurocysticercosis. Epilepsia, 30, 569-572. [Google Scholar] [CrossRef] [PubMed]
[19] Pascual-Castroviejo, I., Martín, V.L., Bermejo, A.M. and Higueras, A.P. (1992) Is Cerebral Arteritis the Cause of the Landau-Kleffner Syndrome? Four Cases in Childhood with Angiographic Study. Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques, 19, 46-52. [Google Scholar] [CrossRef] [PubMed]
[20] Fameen, R., Rinsha Pravin, K., Pooja, S., Rashmi, V., Bhattarai, B. and Abhishek, B.P. (2022) Acquired Childhood Aphasia as a Consequence of COVID‐19 and Its Differential Diagnosis from Speech-Language Pathologist Perspective: A Case Study. Clinical Case Reports, 10, e6587. [Google Scholar] [CrossRef] [PubMed]
[21] van der Meulen, I., Pangalila, R.F. and van de Sandt-Koenderman, W.M.E. (2021) Cognitive Linguistic Treatment in Landau Kleffner Syndrome: Improvement in Daily Life Communication. Child Neurology Open, 8, 2329048X211022196.
[22] Caraballo, R.H., Cejas, N., Chamorro, N., Kaltenmeier, M.C., Fortini, S. and Soprano, A.M. (2014) Landau-Kleffner Syndrome: A Study of 29 Patients. Seizure-European Journal of Epilepsy, 23, 98-104. [Google Scholar] [CrossRef] [PubMed]
[23] Stefanatos, G. (2011) Changing Perspectives on Landau-Kleffner Syndrome. The Clinical Neuropsychologist, 25, 963-988. [Google Scholar] [CrossRef] [PubMed]
[24] de Guevara, L. and Campos, J. (2015) Landau-Kleffner Syndrome. Journal of Pediatric Neurology, 5, 93-99. [Google Scholar] [CrossRef] 
[25] Tuft, M., Årva, M., Bjørnvold, M., et al. (2015) Landau-Kleffner Syndrome. Tidsskrift for Den Norske Laegeforening: Tidsskrift for Praktisk Medicin, 135, 2061-2064.
https://tidsskriftet.no/en/2015/12/landau-kleffner-syndrome
[26] Althagafi, L., Al Fuhayd, R., Almeathem, F.K., Almeshal, R.A., Al-Amri, L.J. and Mustafa, W.A. (2024) Landau-kleffner Syndrome, Attention-Deficit/Hyperactivity Disorder (ADHD), and Viral/Autoimmune Encephalitis: Challenges in the Diagnosis and Management of a Six-Year-Old Boy. Cureus, 16, e52133. [Google Scholar] [CrossRef] [PubMed]
[27] Besag, F., Aldenkamp, A., Caplan, R., et al. (2025) Psychiatric and Behavioural Disorders in Children with Epilepsy: An ILAE Task Force Report. Epileptic Disorders, 18, S1.
https://onlinelibrary.wiley.com/doi/10.1684/epd.2016.0809
[28] Smith, M.C. and Hoeppner, T.J. (2003) Epileptic Encephalopathy of Late Childhood: Landau-Kleffner Syndrome and the Syndrome of Continuous Spikes and Waves during Slow-Wave Sleep. Journal of Clinical Neurophysiology, 20, 462-472. [Google Scholar] [CrossRef] [PubMed]
[29] Kotagal, P. (2017) Current Status of Treatments for Children with Electrical Status in Slow-Wave Sleep (ESES/CSWS). Epilepsy Currents, 17, 214-216. [Google Scholar] [CrossRef] [PubMed]
[30] Duda, P., Duda, N., Kostelecka, K., Woliński, F., Góra, J., Granat, M., et al. (2024) Multiple Subpial Transection for the Treatment of Landau-Kleffner Syndrome—Review of the Literature. Journal of Clinical Medicine, 13, Article 7580. [Google Scholar] [CrossRef] [PubMed]
[31] Hughes, J.R. (2011) A Review of the Relationships between Landau-Kleffner Syndrome, Electrical Status Epilepticus during Sleep, and Continuous Spike-Waves during Sleep. Epilepsy & Behavior, 20, 247-253. [Google Scholar] [CrossRef] [PubMed]
[32] Nariai, H., Duberstein, S. and Shinnar, S. (2017) Treatment of Epileptic Encephalopathies: Current State of the Art. Journal of Child Neurology, 33, 41-54. [Google Scholar] [CrossRef] [PubMed]
[33] Geva-Dayan, K., Shorer, Z., Menascu, S., Linder, I., Goldberg-Stern, H., Heyman, E., et al. (2012) Immunoglobulin Treatment for Severe Childhood Epilepsy. Pediatric Neurology, 46, 375-381. [Google Scholar] [CrossRef] [PubMed]
[34] Arts, W.F.M., Aarsen, F.K., Scheltens‐de Boer, M. and Catsman‐Berrevoets, C.E. (2009) Landau‐Kleffner Syndrome and CSWS Syndrome: Treatment with Intravenous Immunoglobulins. Epilepsia, 50, 55-58. [Google Scholar] [CrossRef] [PubMed]
[35] Fainberg, N., Harper, A., Tchapyjnikov, D. and Mikati, M.A. (2016) Response to Immunotherapy in a Patient with Landau‐Kleffner Syndrome and GRIN2A Mutation. Epileptic Disorders, 18, 97-100. [Google Scholar] [CrossRef] [PubMed]
[36] Deonna, T., Prelaz‐Girod, A., Mayor‐Dubois, C. and Roulet‐Perez, E. (2009) Sign Language in Landau‐Kleffner Syndrome. Epilepsia, 50, 77-82. [Google Scholar] [CrossRef] [PubMed]

为你推荐