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As speech-related symptoms of Landau–Kleffner syndrome (LKS) are often refractory to pharmacotherapy, and resective surgery is rarely available due to the involvement of the vital cortex, multiple subpial transection (MST) was suggested to improve patient outcome and preserve cortical functions. Here, we analyze the reports about MST use in LKS, regarding its impact on seizures, language, behavior, EEG, cognition, and reported adverse effects. In conditions like LKS, surgery is not a popular treatment option and presumably should be considered sooner. Candidates for MST should be selected carefully, optimally with the unilateral onset of epileptic activity. Laterality can be assessed using a methohexital suppression test (MHXT), electrical intracarotid amobarbital test, or magnetoencephalography. After MST, a significant percentage of LKS patients present seizure-free status, normalization of EEG patterns, and rapid behavior improvement. Data comprising language outcomes are mixed, with improvement reported in 23.8–100% of cases, and no superiority was found in the only study comparing MST with a non-surgical group. Cognitive outcomes are not well described. The risk linked to MST is described as low, with cerebral edema and transient neurological deficits being the most common complications. MST successfully improves seizure, EEG, and behavioral outcomes in LKS patients. However, its beneficial impact on language and cognition is not well proven. It is generally a safe neurological operation. [ABSTRACT FROM AUTHOR]

Background and Objectives: Developmental and epileptic encephalopathy refers to a group of conditions where patients experience abnormal development due to various causes as well as frequent epileptiform discharges that ultimately contribute, in an independent and additive fashion, to cognitive and linguistic impairments. The language and cognition outcome of these patients in adulthood has been understudied. This paper aims to present a scoping review of linguistic abilities in adults with developmental and epileptic encephalopathy to determine the extent to which language outcomes in adulthood and their relation to cognitive outcomes have been studied. Design: Two online databases were searched and the methodological framework by Arksey & O'Malley (2005) was adopted. Results: Out of the 27 selected studies, only 13 exclusively examined adults, 15 were group studies, 5 were case studies and 7 were case series. A total of 9 out of the 15 group studies provided individual results for adults. Twenty-two studies included a follow-up examination. Twenty-three studies addressed the relationship between language and cognition. The selected studies indicate the presence of language impairments, which are nevertheless differentially manifested in the syndromes under investigation, whereas individual variability is also reported. Aspects of cognition seem to correlate with linguistic abilities. Conclusions: In sum, despite variability in linguistic abilities, language deficits constitute a significant aspect of the clinical profile of many adults with developmental and epileptic encephalopathy, a finding that should be taken into account for the treatment protocols of these individuals. [ABSTRACT FROM AUTHOR]

Landau–Kleffner syndrome (LKS), also known as acquired epileptic aphasia, is a childhood disease associated with acquired aphasia and epileptiform abnormalities on the electroencephalographic. We report the uneventful anesthesia management of a 17-year-old female patient with LKS syndrome. Her vital signs and laboratory results were normal, and plasma levels of valproic acid (73.6 µg/mL) were within therapeutic limits. She had mild micrognathia, short thyromental distance, and normal neck movements and mouth opening. An intravenous induction was performed with 100 mg lidocaine, 100 µg fentanyl, 200 mg propofol, and 60 mg rocuronium intravenously. After preoxygenation with 80% O2 for 3 min, she was intubated without complications with a number 7.5 mm endotracheal tube connected to a ventilator. Anesthesia maintenance was provided with 2.2% sevoflurane and 50% oxygen–50% air mixture, 0.01–0.05 μg/kg/min infusion of remifentanil. The target partial pressure of carbon dioxide was 30–35 mm Hg. Care was taken to ensure that intra-abdominal pressure did not exceed 14 mm Hg. Spontaneous breathing returned in 5 min after discontinuation of sevoflurane. She was extubated after following commands and transferred to the post-anesthesia care unit. It is unknown whether general anesthesia has a short- or long-term effect on patients with LKS. Since epileptic seizures are essential in patients with LKS, the correct selection of anesthetic agents is significant. In addition, hypoxemia, hypercarbia, hypocapnia, high positive end-expiratory pressure, and acidosis should all be avoided as they may trigger seizure activity.

In the context of childhood epilepsy, the concept of continuous spike–waves during slow sleep (CSWS) includes several childhood-onset heterogeneous conditions that share electroencephalograms (EEGs) characterized by a high frequency of paroxysmal abnormalities during sleep, which have negative effects on the cognitive development and behavior of the child. These negative effects may have the characteristics of a clear regression or of a slowdown in development. Seizures are very often present, but not constantly. The above makes it clear why CSWS have been included in epileptic encephalopathies, in which, by definition, frequent EEG paroxysmal abnormalities have an unfavorable impact on cognitive functions, including socio-communicative skills, causing autistic features, even regardless of the presence of clinically overt seizures. Although several decades have passed since the original descriptions of the electroclinical condition of CSWS, there are still many areas that are little-known and deserve to be further studied, including the EEG diagnostic criteria, the most effective electrophysiological parameter for monitoring the role of the thalamus in CSWS pathogenesis, its long-term evolution, the nosographic location of Landau–Kleffner syndrome, standardized neuropsychological and behavioral assessments, and pharmacological and non-pharmacological therapies. [ABSTRACT FROM AUTHOR]

Background and Objectives: Developmental and epileptic encephalopathy refers to a group of conditions where patients experience abnormal development due to various causes as well as frequent epileptiform discharges that ultimately contribute, in an independent and additive fashion, to cognitive and linguistic impairments. The language and cognition outcome of these patients in adulthood has been understudied. This paper aims to present a scoping review of linguistic abilities in adults with developmental and epileptic encephalopathy to determine the extent to which language outcomes in adulthood and their relation to cognitive outcomes have been studied. Design: Two online databases were searched and the methodological framework by Arksey & O’Malley (2005) was adopted. Results: Out of the 27 selected studies, only 13 exclusively examined adults, 15 were group studies, 5 were case studies and 7 were case series. A total of 9 out of the 15 group studies provided individual results for adults. Twenty-two studies included a follow-up examination. Twenty-three studies addressed the relationship between language and cognition. The selected studies indicate the presence of language impairments, which are nevertheless differentially manifested in the syndromes under investigation, whereas individual variability is also reported. Aspects of cognition seem to correlate with linguistic abilities. Conclusions: In sum, despite variability in linguistic abilities, language deficits constitute a significant aspect of the clinical profile of many adults with developmental and epileptic encephalopathy, a finding that should be taken into account for the treatment protocols of these individuals.

OBJECTIVES: To determine how continuous spike and wave during slow wave sleep (CSWS) is currently managed and to compare the effectiveness of current treatment strategies using a database from 11 pediatric epilepsy centers in the US. STUDY DESIGN: This retrospective study gathered information on baseline clinical characteristics, CSWS etiology, and treatment(s) in consecutive patients seen between 2014 and 2016 at 11 epilepsy referral centers. Treatments were categorized as benzodiazepines, steroids, other antiseizure medications (ASMs), or other therapies. Two measures of treatment response (clinical improvement as noted by the treating physician; and electroencephalography improvement) were compared across therapies, controlling for baseline variables. RESULTS: Eighty-one children underwent 153 treatment trials during the study period (68 trials of benzodiazepines, 25 of steroids, 45 of ASMs, 14 of other therapies). Children most frequently received benzodiazepines (62%) or ASMs (27%) as first line therapy. Treatment choice did not differ based on baseline clinical variables, nor did these variables correlate with outcome. After adjusting for baseline variables, children had a greater odds of clinical improvement with benzodiazepines (OR 3.32, 95%CI 1.57-7.04, P = .002) or steroids (OR 4.04, 95%CI 1.41-11.59, P = .01) than with ASMs and a greater odds of electroencephalography improvement after steroids (OR 3.36, 95% CI 1.09-10.33, P = .03) than after ASMs. CONCLUSIONS: Benzodiazepines and ASMs are the most frequent initial therapy prescribed for CSWS in the US. Our data suggests that ASMs are inferior to benzodiazepines and steroids and support earlier use of these therapies. Multicenter prospective studies that rigorously assess treatment protocols and outcomes are needed.

Landau-Kleffner syndrome (LKS) is an epilepsy syndrome of childhood, characterized by auditory verbal agnosia and severe electroencephalographic abnormalities. It happens between 3-7 years of age, 20% without seizures. A 6-years-old boy, with normal psychomotor development, who begins to experience difficulties in understanding the meaning of words, sometimes with incoherent speech and writing difficulties (irregular handwriting, spelling mistakes). Neurological evaluation showed loss of comprehensive language with auditory verbal agnosia, difficulties in fine motor skills and in graphomotor coordination. After clinical suspicion, the diagnosis of SLK was confirmed by night video-electroencephalogram (EEG): abundant paroxysmal activity of bilateral and independent fronto-center-temporal spikes and spike-wave activity, with an almost continuous exacerbation during sleep. Initially treated with valproate and clobazam, without improvement, he started prednisolone, with improvement in language, writing and normalization of the EEG. Knowledge of this syndrome is important for differential diagnosis with other neurological disorders, especially learning difficulties.

In the context of childhood epilepsy, the concept of continuous spike–waves during slow sleep (CSWS) includes several childhood-onset heterogeneous conditions that share electroencephalograms (EEGs) characterized by a high frequency of paroxysmal abnormalities during sleep, which have negative effects on the cognitive development and behavior of the child. These negative effects may have the characteristics of a clear regression or of a slowdown in development. Seizures are very often present, but not constantly. The above makes it clear why CSWS have been included in epileptic encephalopathies, in which, by definition, frequent EEG paroxysmal abnormalities have an unfavorable impact on cognitive functions, including socio-communicative skills, causing autistic features, even regardless of the presence of clinically overt seizures. Although several decades have passed since the original descriptions of the electroclinical condition of CSWS, there are still many areas that are little-known and deserve to be further studied, including the EEG diagnostic criteria, the most effective electrophysiological parameter for monitoring the role of the thalamus in CSWS pathogenesis, its long-term evolution, the nosographic location of Landau–Kleffner syndrome, standardized neuropsychological and behavioral assessments, and pharmacological and non-pharmacological therapies.

It is essential to be more vigilant in understanding impact of COVID‐19 on children's speech and language skills. As studies in these lines are very sparse, it is imperative to profile these children and derive accurate diagnosis. Accurate diagnosis aids Speech‐Language Pathologists (SLPs) to render speech and Language therapy systematically. The ongoing COVID‐19 is a global pandemic. Though fatality has reduced, its devastating circumstances have continued to prevail. The current study highlights the profiling, assessment, and management of 2‐year‐old child, who showed regression following COVID‐19. The study unveils the relationship between COVID‐19, acquired childhood aphasia, and Japanese‐Encephalitis. [ABSTRACT FROM AUTHOR]

Abstract Background Landau-Kleffner syndrome (LKS) is an acquired aphasia and electroencephalogram (EEG) abnormalities mainly in temporoparietal areas. SLC26A4 mutations can cause hearing loss associated with enlarged vestibular aqueduct (EVA). Case presentations We report a case of LKS in a 5-year-old boy with non-syndromic EVA due to homozygous mutations of c.919-2A>G (IVS7-2A>G) in SLC26A4. He had normal language development before 2 years old. At the age of 2.5 years, he was admitted to the hospital due to remarkable language delay, and diagnosed with hearing loss with EVA. The seizures started at 4.4 years of age and EEG recording showed electrical status epilepticus during sleep (ESES) with a posterior-temporal predominance. He received cochlear implantation in the right ear at 4.7 years of age, which improved his hearing and language skills. The nocturnal focal motor seizures recurred at 4.9 years of age. Then a remarkable inability to respond to calls and reduction in spontaneous speech were noticed. He was treated with methylprednisolone at 5 years old, which controlled the seizures, suppressed ESES, and remarkably improved the language ability. The absence of seizures maintained until the last follow-up at 5.3 years of age, with further improvements in EEG recording and language ability. Conclusions The co-existence of LKS and hearing loss caused by SLC26A4 mutations increases the difficulty of LKS diagnosis, especially in the presence of hearing loss and impaired language skills. EEG discharges predominantly in temporoparietal areas, the occurrence of ESES, and language improvement after antiepileptic medications are potential indicators for LKS diagnosis.

Presumably, patients with early childhood onset LKS also require this right-hemisphere function during the recovery process of lost language. Keywords: Landau-Kleffner syndrome; aphasia; dual-stream model; epileptogenic zone EN Landau-Kleffner syndrome aphasia dual-stream model epileptogenic zone 295 298 4 09/07/22 20220701 NES 220701 Conflict of Interest None declared. Examination by SP 99 m sp Tc-ethylcysteine dimer single-photon emission computed tomography (ECD-SPECT) at relapse showed hyperperfusion in the bilateral anterior temporoparietal area with right-hemisphere dominance (Fig. [Extracted from the article]

The 2017 International League Against Epilepsy classification has defined a three‐tier system with epilepsy syndrome identification at the third level. Although a syndrome cannot be determined in all children with epilepsy, identification of a specific syndrome provides guidance on management and prognosis. In this paper, we describe the childhood onset epilepsy syndromes, most of which have both mandatory seizure type(s) and interictal electroencephalographic (EEG) features. Based on the 2017 Classification of Seizures and Epilepsies, some syndrome names have been updated using terms directly describing the seizure semiology. Epilepsy syndromes beginning in childhood have been divided into three categories: (1) self‐limited focal epilepsies, comprising four syndromes: self‐limited epilepsy with centrotemporal spikes, self‐limited epilepsy with autonomic seizures, childhood occipital visual epilepsy, and photosensitive occipital lobe epilepsy; (2) generalized epilepsies, comprising three syndromes: childhood absence epilepsy, epilepsy with myoclonic absence, and epilepsy with eyelid myoclonia; and (3) developmental and/or epileptic encephalopathies, comprising five syndromes: epilepsy with myoclonic–atonic seizures, Lennox–Gastaut syndrome, developmental and/or epileptic encephalopathy with spike‐and‐wave activation in sleep, hemiconvulsion–hemiplegia–epilepsy syndrome, and febrile infection‐related epilepsy syndrome. We define each, highlighting the mandatory seizure(s), EEG features, phenotypic variations, and findings from key investigations. [ABSTRACT FROM AUTHOR]

Landau-Kleffner syndrome (LKS) should be considered in the differential diagnosis in a child with acquired aphasia. Early recognition and intervention might help in prevention of further complications, such as speech impairment, cognitive impairment and seizure. Landau-Kleffner syndrome (LKS) should be considered in the differential diagnosis in a child with acquired aphasia. Early recognition and intervention might help in prevention of further complications, such as speech impairment, cognitive impairment and seizure.

Epileptic encephalopathies represent a group of devastating epileptic disorders that occur early in life and are often characterized by pharmaco-resistant epilepsy, persistent severe electroencephalographic abnormalities, and cognitive dysfunction or decline. Next generation sequencing technologies have increased the speed of gene discovery tremendously. Whereas ion channel genes were long considered to be the only significant group of genes implicated in the genetic epilepsies, a growing number of non-ion-channel genes are now being identified. As a subgroup of the genetically mediated epilepsies, epileptic encephalopathies are complex and heterogeneous disorders, making diagnosis and treatment decisions difficult. Recent exome sequencing data suggest that mutations causing epileptic encephalopathies are often sporadic, typically resulting from de novo dominant mutations in a single autosomal gene, although inherited autosomal recessive and X-linked forms also exist. In this review we provide a summary of the key features of several early- and mid-childhood onset epileptic encephalopathies including Ohtahara syndrome, Dravet syndrome, Infantile spasms and Lennox Gastaut syndrome. We review the recent next generation sequencing findings that may impact treatment choices. We also describe the use of conventional and newer anti-epileptic and hormonal medications in the various syndromes based on their genetic profile. At a biological level, developments in cellular reprogramming and genome editing represent a new direction in modeling these pediatric epilepsies and could be used in the development of novel and repurposed therapies.

Resumen: Introducción: La punta-onda continua durante el sueño lento (POCS) es un trazado electroencefalográfico característico, que aparece en la infancia y que en ocasiones condiciona un deterioro cognitivo. Este patrón electroencefalográfico puede aparecer tanto en determinados síndromes epilépticos como en la evolución de epilepsias idiopáticas y sintomáticas. El objetivo de nuestro estudio es analizar las características epidemiológicas y clínicas de pacientes que presentan en algún momento de su evolución POCS, describir la respuesta a distintos tratamientos y estudiar factores predictores de su evolución. Métodos: Estudio observacional retrospectivo de pacientes pediátricos con POCS seguidos en un hospital terciario en el periodo de noviembre 1997 a noviembre 2017. Resultados: Obtuvimos una muestra de 25 pacientes (68% varones). El 76% presentaba de base alteraciones en pruebas de neuroimagen o retraso psicomotor (POCS secundaria). El 24% restante eran niños sanos o con epilepsias benignas (POCS primaria). La edad media al inicio de la POCS fue de 6,7 años, siendo menor en los casos secundarios. Durante el periodo de POCS, el 72% presentó alguna manifestación clínica añadida. Todos recibieron combinaciones de antiepilépticos, siendo eficaces en el 36%. La POCS cesó en el 72%, siendo más probable el cese cuanto más tarde se hubiera instaurado. Un tercio tuvo alguna secuela, principalmente alteraciones cognitivas y del comportamiento, más frecuentes en POCS secundaria y en los pacientes en que duró más tiempo. Conclusiones: El trazado electroencefalográfico de POCS, aunque infrecuente, supone un reto terapéutico. Es importante seguir a los pacientes con epilepsia, especialmente si asocia deterioro neurológico, para detectar la presencia POCS e iniciar tratamiento precoz. Abstract: Introduction: Continuous spikes and waves during slow sleep (CSWS) is an EEG pattern that appears during childhood, and is often associated with cognitive impairment. It can appear in the course of epileptic syndromes, as well as in benign epilepsy. The aim of this study is to analyse epidemiological and clinical characteristic of patients with CSWS, in order to describe possible predictive factors in their outcome. Methods: A retrospective study was conducted on paediatric patients with CSWS treated in a third-level hospital from November 1997 to November 2017. Results: The study included 25 patients (68% male), of whom 76% had abnormalities in the neuroimaging or suffered from psychomotor development disorder (secondary CSWS). The rest were healthy, or diagnosed with idiopathic epilepsy. The mean age of onset of CSWS was 6.7 years, but earlier in the secondary CSWS cases. Symptoms were present during the CSWS episode in 72% of cases. All of them were treated with antiepileptic drugs, which were effective in 36%. CSWS stopped in 72%, and remission was longer if the CSWS onset occurred at an older age. One-third (33%) presented with sequelae, mostly cognitive and behavioural alterations. Outcome was poorer in those with secondary CSWS and, in those whose CSWS started at an earlier age and lasted longer. Conclusion: The CSWS pattern, although rare, is still a therapeutic challenge. A close follow-up of the patients with epilepsy is important, especially if associated with cognitive impairment, in order to establish an early diagnosis and treatment.

Purpose Acquired epileptic aphasia (AEA) accompanied by electroencephalogram (EEG) abnormality is a rare disease; therefore, there are few studies investigating the prognostic factors and treatment efficacy. We aimed to determine the therapeutic effects and prognostic factors for clinical seizure and neuropsychological function in acquired aphasia patients. Methods We retrospectively studied cases of AEA diagnosed at Severance Children's Hospital from January 2013 to October 2017. We evaluated the efficacy of antiepileptic drugs, steroids, and ketogenic diets (KD) in treating acquired aphasia. The EEG patterns and prognostic factors were predicted by the background EEG and frequency of spike and wave during sleep (SWS). Results The study analyzed 20 patients, 11 male and 9 female, with AEA. Aphasia most commonly occurred at 4 years of age, and clinical seizure was most likely to occur between 2 and 4 years of age and focal seizures were the most common seizure type. KD was shown to be the best treatment for clinical seizure in AEA patients. Patients with normal EEG background showed better responses to clinical seizure treatment and improvements in neuropsychological function. Conclusion KD and steroids generate the best therapeutic effects for clinical seizure in AEA patients. Improvements in neuropsychological function in AEA patients may be related to the EEG background and the SWS patterns. Additionally, the results suggest that the response of clinical seizure to antiepileptic drugs may also be related to the EEG background. However, the current study had some limitations and further research is needed.

Landau–Kleffner syndrome (LKS) is a rare epilepsy occurring in children usually between the ages of three and nine years, characterised by dramatic loss of language skills in association with epileptiform activity over the language centres during sleep. It may be associated with convulsive seizures and additional difficulties with behaviour, social interaction, motor skills and cognition. Early treatment to suppress the EEG abnormalities can at least partially reverse this regression in 60–70% but usually needs to be continued for some years. Management focuses on restoring communication so that the child continues to engage and learn. Therapy and education support are essential and need to be very flexible as the child's profile can fluctuate dramatically and may include signing when they cannot access language and may require considerable opportunities for consolidation and overlearning. The epileptiform activity usually resolves by puberty, often with continuing recovery in skills for some years that will benefit from ongoing support. Many have persisting deficits as adults which are more severe with earlier age of onset and a longer epileptiform period. It is important to recognise LKS as early multi-disciplinary management may improve outcomes. [ABSTRACT FROM AUTHOR]

We report a case of a 48-year-old woman who was diagnosed with Landau-Kleffner syndrome (LKS) at the age of 4 and reassessed by the same neurologist four decades later. While her seizures abated by the time she was 12 years old, she was left with chronic aphasia, despite receiving optimal care. Although she graduated from high school, started her own family, and was gainfully employed, she was vulnerable in situations that required clear communication. This case reflects successful management of an otherwise debilitating condition and reminds us of the vulnerability of adults with LKS and their need for a life-long support.

Mucopolysaccharidosis type III (MPS III; Sanfilippo syndrome) is a metabolic disorder characterized by a lysosomal enzyme deficiency in the catabolic pathway of heparan sulfate. The patients with mucopolysaccharidosis type III usually present with declined neurocognitive functions such as speech and hearing loss. Subtle somatic features of patients with mucopolysaccharidosis type III can lead to diagnostic delay and consequently, a greater neurocognitive deterioration may happen. Herein, we report a 9-yr-old boy referred to Loghman Hospital, Tehran, Iran, in 2018. He had developed normally up to four yr of age when his symptoms initiated with behavioral disturbances such as auditory agnosia and decreased verbal communication. Progression of his symptoms to seizure and ataxia, brain perfusion scan and electroencephalography features strongly suggested landau-Kleffner syndrome. However, results of gene sequencing analysis and high urinary glycosaminoglycan excretion confirmed mucopolysaccharidosis type III as his final diagnosis. This case strongly recommends screening for metabolic disorders such as mucopolysaccharidosis type III in the patients diagnosed as having landau-Kleffner syndrome. [ABSTRACT FROM AUTHOR]

We overview here the new data about the epileptic spectrum disorders within the frame of perisylvian epileptic network since our first trial to synthetize knowledge about this system epilepsy (Halász et al., 2005). We found evidences for a continual features relating together syndromes constituting this spectrum disorder in several fields: in sharing genetic origin, in common perisylvian human communication circuitry, in NREM sleep related potentiation of interictal epileptiform discharges of the centro-temporal spike phenomenon and in the discharge related cognitive impairment, reflecting functional deficits in human communication abilities. The transformation of a part of the children to develop into a malignant course with different degree of residual cognitive loss, through compromising sleep plastic functions, by the epileptic discharges during sleep, beside pure genetic origin, is still under research. Both factual data and new conceptual approaches helps understand better the developmental childhood epilepsies. [ABSTRACT FROM AUTHOR]

Landau–Kleffner syndrome (LKS) is a disease from the group of age-dependent epileptic encephalopathies with the phenomenon of prolonged spike–wave activity in slow-wave sleep, which is manifested by acquired sensorimotor aphasia, impaired higher mental functions concurrent with various epileptic seizures and diffuse electroencephalographic (EEG) epileptiform changes in the absence of cerebral structural changes. In 1957, W. M. Landau, a pediatric neurologist, and F. R. Kleffner, a logopedist, first described the clinical presentation of the disease in 6 patients. The author associated the occurrence of aphasia with epileptiform EEG activity rather than with epileptic seizures, i. e. they per se formulated the modern concept of epileptic encephalopathy. There has been currently evidence that prolonged diffuse epileptiform activity has a damaging effect on a child’s developing brain, which is seen in impairments of speech and other higher mental functions. The age of patients with manifestations of LKS (3–5 years) is a critical period for the development of speech function. According to the concept of H. Doose (2003), congenital brain maturation disorder underlies LKS. The paper considers ideas on the origin of the LKS, including the possible genetic bases of diseases, epidemiological aspects, clinical and EEG manifestations with a detailed description ictal and interictal EEG changes, and treatment approaches, including the author’s data. The disease is characterized by an electroclinical triad: seizures (may be absent or may be single in a history), impaired higher mental functions (with a predominance of aphasic disorders), epileptiform EEG activity (a combination of regional and diffuse patterns with an increase during sleep).

The article describes the phenomenon of dual pathology – a combination of structural changes in the brain and benign epileptiform discharge of childhood on electroencephalogram. The uniqueness lies in the observation that the child, since birth suffering from spastic form of cerebral palsy and severe epilepsy, demonstrated the development of Stevens–Johnson syndrome due to intolerance of one of the antiepileptic drugs. Therapeutic approaches to overcome a whole range of violations are discussed in the article.

Acquired epileptic aphasia, otherwise known as Landau–Kleffner syndrome, is a childhood epilepsy syndrome, characterized by acquired aphasia, seizures, behavioral abnormalities, and abnormal electroencephalography (EEG), in various combinations. Sometimes, some intracranial pathologies, such as brain tumor, head injury, demyelination, and neurocysticercosis, have been found in association. Here, we report a case of a 5-year-old male child presenting with gradual loss of speech, behavioral abnormalities, such as hyperactivity, inattentiveness, and abnormal EEG findings, but without the classical seizures seen with this syndrome.

Some of childhood epileptic syndromes reminds immunological etiologies with their good response to immuno-therapy and histopathological findings. These syndromes, such as Rasmussen encephalitis, FIRES, West syndrome, and Landau-Kleffner syndrome each of which are proceeding with severe epileptic encephalopathy are discussed with their physiopathological, clinical, EEG, laboratory, treatment and prognostic characteristics by the help of current literatüre.

In this case, we discuss the difficulties and challenges faced when diagnosing and treating a six-year-old boy presenting with abnormal behaviors and difficulty in concentration and inattentiveness, followed by regression of expressive language. These symptoms were then followed by hyperactivity, bouts of anger, and difficulty sleeping. The patient was seen by a psychiatrist, and he was diagnosed with attention-deficit/hyperactivity disorder (ADHD) initially and treated with little to no improvement. He was then recommended to see a neurologist by his psychiatrist and underwent a series of investigations, which included the following: brain MRI with contrast, magnetic resonance (MR) spectroscopy, cerebrospinal fluid (CSF) routine, CSF N-methyl-D-aspartate (NMDA) receptor antibody and glutamate decarboxylase (GAD) antibody analysis, and a CSF meningitis multiplex polymerase chain reaction, thyroid function, ammonia, lactate, creatine kinase, liver function, and metabolic screening of urine organic acids (UOAs), all of which revealed no abnormalities. Vitamin D was low at 38 ng/ml (>50). An electroencephalogram (EEG) done under standard conditions and provocative stimulation was abnormal with bilateral central and frontal discharge and more activity on the right side, revealing a background activity of moderately organized alpha waves (8-13 Hz) and bursts of sharp and slow wave activities that were accentuated by photic stimulation. Polysomnography showed poor sleep efficiency of 84.7%, and rapid eye movement (REM) stage was not reached due to interrupted sleep. He was then diagnosed with epileptic encephalopathy and Landau-Kleffner syndrome (LKS). The patient was prescribed sodium valproate, intravenous immunoglobulin (IVIG), and pulse steroids, with no major improvement, risperidone was added but was poorly tolerated, and the dose tapered off and eventually discontinued. Methylphenidate was the prescribed starting at 5 mg, and the dose was gradually increased to 20 mg/day given separately as 10 mg twice a day. A week later, melatonin 2 mg was added. Three months, later the EEG was repeated and was normal, and sodium valproate was tapered off and eventually discontinued. Later on that year, he was diagnosed with COVID-19 and developed acute myositis as a complication, methylphenidate was stopped, and only sertraline and melatonin were continued. MRI was repeated only this time, showing evidence of viral/autoimmune encephalitis (AIE) sequela, and IV corticosteroids were given alongside IVIG. He was discharged on prednisolone, and a month later, major improvements were seen in all aspects., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Althagafi et al.)

Highlights • Developmental regression is associated with a number of developmental disabilities. • We reviewed 38 intervention studies addressing regression in cases of developmental disability. • Twelve studies with 44 participants provided a convincing demonstration of a positive intervention effect. • Positive results using behavioral interventions were noted for challenging behavior, communication, motor, and play skills. • Future research should target the causes of regression and treatments for regression in autism spectrum disorder. Abstract Developmental regression is characteristic of Rett syndrome and it also occurs in a number of other developmental disabilities. To assist clinicians in identifying promising therapeutic approaches, we identified 38 studies that sought to improve adaptive behavior functioning in cases where developmental regression had either already occurred or was likely to occur. Studies were summarized in terms of (a) participants, (b) intervention, (c) dependent variables, (d) outcomes, (e) study design, and (f) certainty of evidence. The available literature included 136 participants from preschoolers to adults. Most participants (n = 132) had Rett syndrome. Interventions targeted a range of dependent variables (e.g., challenging behavior, communication, motor, and play skills). Multi-component interventions derived from behavior analytic principles were the norm, suggesting the need for clinical expertise in the application of such principles. However, only 12 studies (with 44 participants) were rated as providing conclusive evidence of a positive intervention effect. Future research on the mechanisms underlying developmental regression might lead to new and more effective interventions. [ABSTRACT FROM AUTHOR]

The purpose of this review is to discuss rare neurological disorders with respect to communication difficulties typical of children. Firstly, communication disorders with special focus on rare communication neurological disorders are discussed. Secondly, on the basis of literature review, the authors explore clinical studies on the most typical rare children's communication neurological disorders. Thirdly, on the basis of the findings from the clinical studies, they set a few recommendations for their medical therapies and management. The methodology was based on the literature review of research studies exploring the research issue. The findings show that the intervention strategies appear to have positive effects on the improvement of speech and language production among children suffering from Landau- Kleffner syndrome and childhood apraxia of speech. Nevertheless, randomized control trials are needed in order to accelerate and facilitate an early and relevant diagnosis and treatment management. In addition, a multidisciplinary approach seems to be the most appropriate for the accurate diagnosis and comprehensive treatment. [ABSTRACT FROM AUTHOR]

We review epilepsy-related aphasias in connection with GRIN2A mutations, focusing on acquired childhood epileptic aphasias such as Landau-Kleffner syndrome (LKS). The spontaneous speech of children with LKS exhibits syntactically simplified utterances, severe word finding difficulties, and severe phonological paraphasias. Characterizing LKS as a neural dysrhythmia, we review how EEG abnormalities typically manifested during non-rapid eye movement (NREM) sleep as electrical status epilepticus are related to sleeping disorders partly caused by GRIN2A mutations. Expanding on speculations originally put forward by Landau & Kleffner, 1957, we explore how neural processes such as sharp-wave ripples, sleep spindles, slow oscillations, and their cross-frequency couplings are necessary for language-related processes which are perturbed in LKS, accounting in part for the linguistic profile of epileptic aphasias. • Specific genes (GRIN2A) help understand the etiology of childhood epileptic aphasias. • Examining cross-frequency (de)couplings is key for understanding language deficits. • These conditions can be construed as neural dysrhythmias or oscillopathies [ABSTRACT FROM AUTHOR]

Objective Electrical status epilepticus in sleep (ESES) syndrome is characterized by near-continuous sleep-induced epileptiform activity and acquired cognitive deficits. Treatment is assumed mandatory to improve cognitive outcome. We aimed to compare EEG characteristics, subjective evaluation and objective neuropsychological assessment as measures to evaluate treatment efficacy, and to analyze possible predictors. Methods We retrospectively included patients with ESES syndrome treated in our center. Treatment effect was analyzed on sleep EEG spike wave index (SWI) and cognitive functioning. Results 47 patients had 147 (43 steroid and 104 non-steroid) treatments. Cognitive improvement was reported after 36% of treatments at first follow-up and 45% of treatments at last follow-up. The median SWI change for treatments resulting in subjective cognitive improvement was −44%, and 0% for those not resulting in subjective cognitive improvement at first follow-up (p = 0.008) and −50% vs. +5% at last follow-up (p = 0.002). No clear association between subjective cognitive improvement and IQ change, and between SWI and IQ change was found. By means of logistic regression we found that steroid treatment, as compared to non-steroid treatment, was associated with cognitive improvement at first follow-up (multivariate OR after multiple imputation 2.5, 95% CI 1.1–5.7), while at last follow-up, higher age at diagnosis was related to cognitive improvement only in univariate analysis (OR 1.02, 95% CI 1.01–1.04). Conclusions We found that in children with ESES, cognitive improvement after treatment was strongly associated with SWI decrease, while it was not reflected by a significant IQ increase. Steroid treatment was most successful in improving cognitive performance. [ABSTRACT FROM AUTHOR]

OBJETIVO: Descrever os resultados obtidos nas avaliações audiológica e eletrofisiológica da audição, verificando a ocorrência de alterações auditivas periféricas e/ou centrais, de indivíduos com síndrome de Landau-Kleffner. MÉTODOS: Foram submetidos à avaliação audiológica (inspeção do meato acústico externo, medidas de imitância acústica, audiometrias tonal e vocal) e eletrofisiológica da audição (potenciais evocados auditivos de curta, média e longa latência), quatro indivíduos com diagnóstico de síndrome de Landau-Kleffner, na faixa etária de nove a 19 anos, encaminhados ao Laboratório de Investigação Fonoaudiológica em Potenciais Evocados Auditivos do Curso de Fonoaudiologia da Universidade de São Paulo. RESULTADOS: Os resultados mostraram que 100% dos indivíduos apresentaram alteração em pelo menos uma das avaliações realizadas, sendo que houve uma maior ocorrência de alterações no potencial evocado auditivo de média latência (100% dos indivíduos apresentaram alterações). CONCLUSÕES: Observou-se uma grande ocorrência de alterações nos resultados das avaliações audiológicas e eletrofisiológicas da audição em indivíduos com síndrome de Landau-Kleffner. Enfatiza-se a importância da investigação da função auditiva destes indivíduos a fim de verificar possíveis relações entre os déficits da comunicação e alterações auditivas que possam estar presentes nessa população.PURPOSE: To describe the audiological and electrophysiological results of individuals with Landau-Kleffner syndrome, verifying the occurrence of peripheral and/or central auditory disorders. METHODS: Four individuals with Landau-Kleffner syndrome with ages ranging from nine to 19 years old, referred to the Auditory Evoked Potentials Laboratory of the Speech and Language Pathology and Audiology Course of the University of São Paulo, were submitted to audiologic (otoscopy, immitance measurements, pure tone and speech audiometry) and electrophysiological evaluation (short, middle and long-latency auditory evoked potentials). RESULTS: All individuals showed alterations in at least one of the evaluations performed, and it was observed a higher rate of alterations in the middle latency response (100% of the individuals showed alterations). CONCLUSIONS: A great occurrence of auditory disorders in audiological and electrophysiological evaluation of individuals with Landau-Kleffner syndrome was observed. It is emphasized the importance of the audiological investigation of these individuals, in order to verify possible relations between communication and auditory disorders in this population.

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ABSTRACT The categorisation of the childhood epilepsies into a number of different syndromes has allowed greater insight into the prognosis, not only with regard to seizure control but also in relation to cognitive and behavioural outcome. The role of genetics in determining both the syndrome and the behavioural outcome remains promising, although the promise is still largely unfulfilled. The behavioural/psychiatric outcome of a selection of the large number of childhood epilepsy syndromes is presented. The rate of autism in West syndrome, particularly in children who have tuberous sclerosis with temporal tubers, is high. In Dravet syndrome there is a loss of skills, with an associated increase in behavioural problems. The frequency of both subtle and overt seizures in the Lennox-Gastaut syndrome almost certainly accounts for the apparent poor motivation; however, a marked improvement in seizure control with treatment can also result in behavioural problems, probably as a result of the 'release phenomenon'. A number of cognitive problems can arise in the so-called 'benign' syndrome of epilepsy with centrotemporal spikes (BECTS) and the rate of ADHD is high. Autistic features and ADHD have been described in the Landau-Kleffner syndrome and other syndromes associated with electrical status epilepticus of slow-wave sleep (ESES). Early effective treatment may reverse some of these features. There is clear evidence fora behavioural syndrome in relation to juvenile myoclonic epilepsy (JME), in which both clinical descriptions and functional neuroimaging indicate frontal lobe deficits. [ABSTRACT FROM AUTHOR]