Landau-Kleffner syndrome: study of four cases

Santos, Lucia Coutinho dos Santos; Antoniuk, Sérgio Antônio; Rodrigues, Marcelo Masruha; Bruno, Silvio; Bruck, Isac

doi:10.1590/s0004-282x2002000200010

Cited by 8 publications

(3 citation statements)

References 20 publications

(12 reference statements)

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“…Santos et al . (2002)[ 12 ] reported four cases with LKS. They had a mean age of presentation around 4 years.…”

Section: Discussionmentioning

confidence: 99%

Clinical profile of patients with rolandic epilepsy at a clinic in rural Maharashtra

Sable¹,

Sable²,

Tamhankar

et al. 2021

J Family Med Prim Care

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Purpose: To describe the seizure pattern, treatment strategies and outcome in a series of children with Rolandic seizures or childhood epilepsy with centrotemporal spikes. Materials and Methods: Patients were defined as Rolandic epilepsy if on electroencephalographic studies high voltage spike and waves were seen in centrotemporal areas, could be followed by slow waves, often activated on sleep and could shift from one side to other or be secondarily generalized. Typical (TRS) or benign were those with normal intellect. Atypical rolandic seizures (ARS) were those associated with neuroregression of language and cognitive milestones. Patients were treated with antiepileptic drugs if more than one episode occurred or the first episode was generalized status epilepticus. Results: Thirty-three patients were included over the period of eight years (2012-2020). There was male preponderance (21 males versus 12 females). Four patients (12.12%) later evolved into Landau Kleffner syndrome (ARS group). The mean age of onset of epilepsy in the TRS group (29 patients) was 7.2 (+/-2.2) with the youngest patient being 4 years and the eldest being 12 years. In the ARS group the mean age of onset was 5 yrs. (+/-1.41). In the TRS group, 23 (79.31%) patients were managed on monotherapy AED. Seventeen patients (58.62%) responded (remission) to carbamazepine monotherapy alone. Six patients (20.68%) could afford oxcarbazepine monotherapy and went in remission with this therapy. In the ARS group all patients required three drugs (valproate, clobazam and levetiracetam). By the end of the study period, 23/33 (75.75%) patients remained seizure free. Conclusions: Most patients with rolandic seizures have excellent prognosis being seizure free around puberty. The neurological outcome in most patients was normal.

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“…Santos et al . (2002)[ 12 ] reported four cases with LKS. They had a mean age of presentation around 4 years.…”

Section: Discussionmentioning

confidence: 99%

Clinical profile of patients with rolandic epilepsy at a clinic in rural Maharashtra

Sable¹,

Sable²,

Tamhankar

et al. 2021

J Family Med Prim Care

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show abstract

“…Kortikotropin ile tedaviye başladıktan 3 hafta sonra epileptik aktivitenin kaybolduğu, takip eden 3 ay içinde afazinin tam remisyonu ile konuşmanın 6 ay içinde geri döndüğü; dil gelişimine ek olarak anlama, dikkat süresi ve hiperkinezide önemli ölçüde iyileşmenin olduğu bildirilmiştir 17 . Oral kortikosteroid ile 2-3 aylık tedaviden sonra konuşmada progresif iyileşmenin olduğu bildirilen vakalar olmakla birlikte 17 ; nöbetlerin ve EEG'nin iyi kontrolünün sağlanmasına rağmen afaziye daha az yanıt alındığını bildiren vaka bildirimleri de vardır 18 . Sunduğumuz vakada olduğu gibi antiepileptik ve oral kortikosteroid ile tedavi edilen beş vakadan oluşan vaka serisinde; 3-6 aylık tedaviden sonra EEG'nin normale döndüğü ve konuşmanın iyileştiği bildirilmiştir 19 .…”

Section: Discussionunclassified

A Case of Landau-Kleffner Syndrome with Autism Spectrum Disorder Symptoms Improved After Corticosteroid Treatment

YILDIRIM¹,

EROĞLU

Gümüş³

2022

Uludağ Üniversitesi Tıp Fakültesi Dergisi

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Introduction: Landau-Kleffner syndrome (LKS), a very rare childhood disease, is acquired aphasia associated with epileptiform activity on the electroencephalogram (EEG). The clinical presentation of LKS may resemble autistic disorders in some children. In this article, a case who came to the Child and Adolescent Psychiatry outpatient clinic with autism symptoms but was diagnosed with LKS as a result of neurological examinations, showed significant improvement in autism symptoms after treatment is presented. Case: The boy aged 4 years 7 months was brought to the Child and Adolescent Psychiatry outpatient clinic with complaints of language regression, decreased interest in the environment, and impaired communication with peers and adults. After the neurological evaluations, the patient was diagnosed with LKS and antiepileptic treatment was started. An substantial improvement in language and social skills was observed within 1-3 months after adding corticosteroid therapy to the patient who did not benefit from antiepileptic treatment. Discussion: In this article, in cases where EEG pathology and autism symptoms did not improve with antiepileptic treatment in LKS; It has been reported that rapid improvement can be achieved in both neurological and autism symptoms after corticosteroid addition treatment. Although there is more information in the literature that known treatments are effective in improvement language regression in LKS, there are few case reports on reducing the accompanying autism symptoms.

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“…Hence, LandauKleffner syndrome is difficult to diagnose and may be misdiagnosed as autism, pervasive developmental disorder, hearing impairment, learning disability, auditory/verbal processing disorder, attention deficit disorder, mental retardation, childhood schizophrenia, or emotional/behavioral problems (National Institute of Neurological Disorders and Stroke, NIH, 2008). Since Landau-Kleffner syndrome was discovered in 1957 (Landau & Kleffner, 1957), there have been some case studies examining language outcomes in children suffering from this disease (Duran, Guimaraes, Medeiros, & Guerreiro, 2009;Grote, Van Slyke, & Hoeppner, 1999;Honbolygo et al, 2006;Lanzi, Veggiotti, Conte, Partesana, & Resi, 1994;Robinson, Baird, Robinson, & Simonoff, 2001;Rossi et al, 1999;Santos, Antoniuk, Rodrigues, Bruno, & Bruck, 2002). But most studies tested vocabulary in the context of short-term phonological memory, sentence comprehension, verbal IQ, and/ordwhen it comes to prosodydword stress.…”

Section: Introductionmentioning

confidence: 99%

A novel prosody assessment test: Findings in three cases of Landau–Kleffner syndrome

Kim-Dufor

Dufor

Astésano

et al. 2012

Journal of Neurolinguistics

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Landau-Kleffner syndrome: study of four cases

Cited by 8 publications

References 20 publications

Clinical profile of patients with rolandic epilepsy at a clinic in rural Maharashtra

Clinical profile of patients with rolandic epilepsy at a clinic in rural Maharashtra

A Case of Landau-Kleffner Syndrome with Autism Spectrum Disorder Symptoms Improved After Corticosteroid Treatment

A novel prosody assessment test: Findings in three cases of Landau–Kleffner syndrome

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