Landau-Kleffner Syndrome

May 28, 2009

Who am I kidding? I’m a fricking nerd.

From Epilepsy Currents:

Landau-Kleffner syndrome is characterized by acquired aphasia [first manifestation: “Parents report a child no longer responds to their commands, even with raised voices”] and paroxysmal, sleep-activated EEG paroxysms [“highly correlated with the occurrence of clinical seizures“] predominating over the temporal or parieto-occipital regions. Secondary symptoms include psychomotor or behavioral disturbances and epilepsy with a favorable outcome for seizure control. The prevalence is unclear. A male predominance exists, with an approximately 2:1 ratio. This regressive syndrome affects children after having achieved early developmental milestones, with 3–9 years being the usual age of presentation.

LKS “has commonalities with autism spectrum disorder.”

Communication deficits in autism include abnormal development of spoken language and impaired ability to initiate or sustain conversation. The autistic child’s language is often stereotyped, repetitive, and idiosyncratic, with echolalia and neologisms 11. Confusing the picture is the fact that seizures may occur in autism, and EEG abnormalities are common. Furthermore, at least a third of autistic toddlers demonstrate neurodevelopmental regression, involving language, sociability, play, and cognition. LKS represents selective loss of language in association with an abnormally paroxysmal EEG, eventually characterized by electrographic status epilepticus of slow-wave sleep (ESES).

McCarthy’s son started seizing when he was two, so if he has LKS, it seems like he presented earlier than usual.

Only 10% of children with LKS regress before three years [versus “the great majority of children with autism who undergo language regression”]. As regression in autism occurs early, it usually entails the loss of single words, versus more drastic changes in LKS children who are typically older and have more developed vocabulary and language. LKS does not feature the behavioral profile that encompasses the core deficits of autism, i.e., abnormalities of reciprocal social relatedness and restricted stereotypical patterns of interests and behaviors. There is an intricate relationship between LKS, autism, ESES, and developmental dysphasias and the interaction between epileptiform discharges and cognitive dysfunction remains enigmatic.

Autism is so interesting: Why would there be multiple autism-related disorders?


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