PURPOSE: We intended to describe the clinical features including characteristic etiologies, therapeutic approaches and outcomes for Landau-Kleffner syndrome(LKS). METHODS: A retrospective chart reviews were done to reveal the clinical and electrophysiological features in 5 patients who were diagnosed as LKS and undergone extensive diagnostic work-up and various therapeutic interventions. RESULTS: Among five LKS patients, 2 patients were males and 3 were females. All patients showed well controlled seizure outcomes but cognitive function including auditory and/or expressive aphasia were not improved in spite of using various anticonvulsants. Only two patients responded to steroid therapy but one patient showed repeated deterioration after discontinuation of the durg. Among 3 patients who were on ketogenic diet(KD), one patient showed a dramatic improvement in liguistic and cognitive functions in spite of underlying mitochondrial complex I deficiency. Multiple subpial transections (MST) were done in the other two patients because of incomplete recovery from ketogenic diet, and only one patient showed fairly successful improvement. CONCLUSION: Cognitive regression induced by LKS could be successfully improved by various therapeutic modalities including steroid, KD and palliative MST in most patients.
Anticonvulsants ; Aphasia, Broca ; Female ; Humans ; Ketogenic Diet ; Landau-Kleffner Syndrome* ; Male ; Retrospective Studies ; Seizures

Conventional anti-epileptic treatments have limitations in treating many epileptic patients. Although there have been great advances in the management of epilepsy by application of newly developed anti-convulsants and surgery, some patients still remain in an intractable condition. Other supplementary treatments including immunoglobulin, steroids, and ketogenic diet have significant anti-epileptic potencies. Immunoglobulin treatment shows benefits in some autoimmune-related epileptic conditions such as Rasmussen's encephalitis, however, still has limitations in long-term efficacy. Steroids show significant improvements in many epileptic syndromes including infantile spasm, Lennox-Gastaut syndrome, Landau-Kleffner syndrome, and epilepsy with continuous spikes and waves during slow wave sleep, however, some of them recur during or after tapering of the drug. Ketogenic diet has become one of the most reliable treatments for intractable epilepsy especially in children. Ketogenic diet is difficult to maintain because of poor palatability, but shows a very high anti-epileptic efficacy. These supplementary treatments should be considered in various epileptic conditions especially in intractable patients.
Child ; Encephalitis ; Epilepsy* ; Humans ; Immunoglobulins ; Infant ; Infant, Newborn ; Ketogenic Diet ; Landau-Kleffner Syndrome ; Spasms, Infantile ; Steroids

Landau-Kleffner syndrome, or acquired epileptiform aphasia, is an epilepsy syndrome with variable disruption of acquired language and epileptiform discharges on electroencephalograph (EEG). Auditory agnosia can deteriorate into total unresponsiveness and impaired expressive communication. In spite of the presence of this condition, the pure tone audiometry, otoacoustic emission and brainstem evoked auditory potential could be normal. Therefore, in the above hearing tests which are the mainstream methods for pediatric hearing evaluation, otolaryngologists should have suspicion for the presence of this syndrome, although it is rare.
Agnosia ; Audiometry ; Brain Stem ; Epilepsy ; Hearing ; Hearing Tests ; Landau-Kleffner Syndrome*

PURPOSE: The efficacy of ketogenic diet in intractable childhood epilepsy has been reported in Korea. The aim of this retrospective study is to elicit the clinical and electrophysiological characteristics of intractable chilhood epilepsy, who can be successfully treated by ketogenic diet and to determine the favorable outcome group for ketogenic diet in intractable chilhood epilepsy. METHODS: We investigated clinical seizure type, epilepsy classification, underlying etiologies, clinical characteristics, EEG findings, response to treatments, and prognosis were studied in 41 patients with complete seizure remission over 6 months after ketogenic diet as compared with unsuccessful group among 96 patients of trial from July 1995 to October 1999 at the pediatric department of and epilepsy center at Sang-gye Paik Hospital, Inje University. RESULTS: 1) Complete seizure remission over 6 months were obtained in 15 patients of infantile spasms (IS), 6 cases of Lennox-Gastaut syndrome (LGS), 4 cases of periodic spasms, 3 cases of severe myoclonic epilepsy in infancy (SMEI), 1 case of Landau-Kleffner syndrome, 8 cases of other type of partial epilepsies. 2) Ketogenic diet was more effective in infantile spasms and periodic spasms but least effective in Lennox-Gastaut syndome. 3) In same types of epilepsies, ketogenic diet was more effective in patients with less frequent seizures, less severe EEG findings and earlier controlling seizures. CONCLUSIONS: Ketogenic diet was most effective in infantile spasms, patients with less frequent seizure in same type of epilepsy, less severe EEG findings in same seizure type, and patients who seizures stopped sooner after administration of ketogenic diet.
Classification ; Electroencephalography ; Epilepsies, Myoclonic ; Epilepsies, Partial ; Epilepsy* ; Humans ; Infant ; Infant, Newborn ; Ketogenic Diet* ; Korea ; Landau-Kleffner Syndrome ; Prognosis ; Retrospective Studies ; Seizures* ; Spasm ; Spasms, Infantile

Anticonvulsants ; therapeutic use ; Brain ; pathology ; physiopathology ; Brain Waves ; physiology ; Child ; Electroencephalography ; Humans ; Landau-Kleffner Syndrome ; physiopathology ; Sleep ; physiology ; Sleep Stages ; physiology ; Status Epilepticus ; epidemiology ; etiology ; physiopathology ; therapy

Electrical status epilepticus during sleep (ESES), an EEG defined syndrome characterized by the occurrence of almost continuous spike and/or slow waves during nonREM sleep, is considered to be rare in incidence. It broadly overlaps with benign rolandic epilepsy, pseudo-Lennox syndrome, and Landau-Kleffner syndrome. A 13-year-old boy has been followed up for 11 years because of intractable epilepsy. He is an adopted child and first seizure occurred at the age of 14 months. Seizures were described as tonic, atypical absence and partial motor, occurred daytime and nocturnal, more frequent in the latter. He was retarded in development and had failed to acquire speech. Serial EEG showed moderately developed posterior dominant rhythm during wakefulness, and, however, continuous 2.5 to 3 Hz bilaterally synchronous centro-temporal sharp waves were observed during sleep state. The EEG features were similar, regardless of spontaneous or induced sleep, and remained essentially unchanged during a 12 year follow up period. Brain MRI was not significant. During follow-up period, carbamazepine and vigabatrin worsened his seizures in frequency and intensity, resulting in frequent generalization. On the contrary, there were rare seizures with combination therapy of valproate, clonazepam and lamotrigine and seizures were nearly disappeared after topiramate add-on. This is a case of ESCS with cognitive, behavioral and language disturbances. Clinical and EEG features of related syndromes will be briefly reviewed.
Adolescent ; Brain ; Carbamazepine ; Child ; Clonazepam ; Electroencephalography ; Epilepsy ; Epilepsy, Rolandic ; Follow-Up Studies ; Generalization (Psychology) ; Humans ; Incidence ; Landau-Kleffner Syndrome ; Magnetic Resonance Imaging ; Male ; Seizures ; Status Epilepticus* ; Valproic Acid ; Vigabatrin ; Wakefulness

OBJECTIVETo evaluate the therapeutic effect of methylprednisolone for electrical status epilepticus during sleep (ESES) in children.

METHODThe clinical and EEG data of 82 epilepsy patients with ESES, which included benign childhood epilepsy with centro temporal spikes (BECT) variants, epilepsy with continuous spikes and waves during slow sleep (CSWS) , Landau-Kleffner syndrome (LKS) collected from department of pediatrics, Peking University First Hospital were analyzed from July 2007 to September 2012. During ESES period, all patients received methylprednisolone treatment for three courses, which included methylprednisolone intravenous infusion for three days, followed by oral prednisone for four days every time. After three courses, prednisone [1-2 mg/(kg × d)] were taken by all patients for 6 months. The ESES phenomenon and seizures were observed before and after treatment. The efficacy of corticosteroid on ESES suppression, seizure control of three epilepsy syndrome were analyzed.

RESULTThirty-nine cases were male and 43 cases were female. The epilepsy syndromes included 49 patients diagnosed as benign childhood epilepsy with centrotemporal spike (BECT) variants, 27 patients diagnosed as epilepsy with continuous spikes and waves during slow sleep (CSWS), and 6 patients diagnosed as LKS. Age of onset ranged from 1 year and 4 months to 11 years. The age of ESES newly monitored was from 2 years to 10 years and 8 months. The total effective rate of corticosteroid was 83% (68/82) for ESES, BECT variants was 82% (40/49), CSWS was 81% (22/27), LKS was 100% (6/6). There was no statistically significant difference in effective rates between the front two (χ² = 0.09, P > 0.05). The seizures were improved in the first month after methylprednisolone treatment in 3 epilepsy syndromes. The recurrence rate of BECT variants was 47% (23/49) , CSWS was 59% (16/27) , LKS was 50% (3/6) after 1 year follow up. There was no association between disease parameters, including age at seizure onset, duration of ESES and the treatment effect of ESES examined by Kruskal-Wallis method (χ² = 3.585, 0.932, P > 0.05).

CONCLUSIONMethylprednisolone was effective for improving ESES and seizures in 3 epilepsy syndromes combined with ESES. There was no significant correlation between age at seizure onset, duration of ESES and treatment effect of ESES.

Adrenal Cortex Hormones ; therapeutic use ; Child ; Child, Preschool ; Electroencephalography ; Female ; Humans ; Infant ; Landau-Kleffner Syndrome ; drug therapy ; physiopathology ; Male ; Methylprednisolone ; therapeutic use ; Pediatrics ; Seizures ; prevention & control ; Sleep ; physiology ; Status Epilepticus ; drug therapy ; physiopathology ; Treatment Outcome

OBJECTIVETo investigate the clinical and electroencephalographic (EEG) characteristics, therapeutic response and long-term prognosis of Landau Kleffner syndrome (LKS).

METHODSThe clinical and EEG data of 10 children with LKS were analyzed, and therapeutic response and long-term outcome were followed up.

RESULTSThe age of onset was from 2 to 10.5 years of age. All patients had acquired aphasia, characterized by verbal auditory agnosia. All patients had epileptic seizures. Partial motor seizures during sleep occurred in 8 patients, and other seizure type including atypical absence seizure and generalized tonic-clonic seizure were also observed. Psychological and behavioral abnormalities occurred in 9 patients. There were no abnormalities of hearing and neuro-imaging tests in all patients, and family histories were negative. All the patients had EEG abnormalities. Focal spike and waves of temporal lobe were recorded in 9 patients. Electrical status epilepticus during sleep (ESES) was observed on Video-EEG (VEEG) monitoring in 4 patients. Anti-epileptic drugs (AEDs) showed favorable effects on epileptic seizures, but no effects on aphasia. All patients responded to corticosteroid, and got language improved. Eight patients were followed up for long-term outcome. All patients were seizure free, while the level of language development was abnormal in 5 patients. The VEEG follow-up was conducted in 6 patients. Continuous epileptic discharges in slow sleep recurred in 2 patients after the discontinuation of steroid therapy.

CONCLUSIONSLKS is one of the childhood epileptic encephalopathy, and acquired aphasia and epileptic seizures are two main clinical characteristics. Aphasia is characterized by verbal auditory agnosia. Psychological and behavioral abnormalities are very common in children with LKS. Focal epileptic discharges were often located in temporal area, and usually generalized, and could be continuous during sleep. AEDs could control seizure but had no effects on aphasia. Early use of full dose corticosteroids could improve the language significantly. Long-term follow up showed that language impairments often remained, but the outcome in terms of EEG and epileptic seizure was good.

Adrenal Cortex Hormones ; therapeutic use ; Age of Onset ; Agnosia ; drug therapy ; physiopathology ; Anticonvulsants ; therapeutic use ; Auditory Perceptual Disorders ; drug therapy ; physiopathology ; Brain ; drug effects ; physiopathology ; Child ; Child, Preschool ; Electroencephalography ; Female ; Follow-Up Studies ; Humans ; Landau-Kleffner Syndrome ; drug therapy ; physiopathology ; Male ; Prognosis ; Retrospective Studies ; Seizures ; drug therapy ; physiopathology ; Time Factors