Landau Kleffner Variant: What's In A Name?

Alex has Landau Kleffner Variant. You won't find Landau Kleffner Variant in the medical diagnostic manuals though. What you will see is Landau Kleffner Syndrome.

With LKS overnight seizures in the temporal lobes rob children of their speech. It also blindsides them with Autistic-like symptoms and severe aggression. Though Landau Kleffner Variant is triggered by abnormal electrical activity in those same areas of the brain and not full blown seizure spikes, it inflicts similar patterns of devastating regressions in children.

There is no consensus among Neurologists on Landau Kleffner Variant. While some doctors informally call this speech robbing cousin to LKS Landau Kleffner Variant, others loosely refer to it as Temporal Lobe Epilepsy. I prefer the term Landau Kleffner Variant because it is more precise. After all, plenty of people with Temporal Lobe Epilepsy have no loss of speech whatsoever. And, while Temporal Lobe Epilepsy can be responsive to anti-convulsants, Landau Kleffner Variant is not. LKV, like LKS, often requires aggressive treatment with Prednisone or Corticosteroids.

We encountered this diagnostic confusion within Alex's Neurology practice. After Alex's 24 hour EEG the staff neurologist at the hospital said our son was a candidate for Prednisone and instructed us to have A's neurologist to write the prescription. But Alex's neurologist was reluctant to write the order and did so only because his colleague at the hospital had offered it as an option. Despite Alex having made dramatic strides in speech, and academics, and behavior on Prednisone his Neurologist eventually refused to continue the treatment, claiming Prednisone is not considered "Best Practice" for Temporal Lobe Epilepsy. When we asked his partner for a second opinion that neurologist said Alex clearly has Landau Kleffner Variant and returned him to Prednisone Protocol immediately.

Though Landau Kleffner Syndrome, which onsets between ages three and seven, is rare, Landau Kleffner Variant may not be, particularly among those who developed Regressive Autism as babies. From the website of Boston Children's Hospital, the premier experts on LKS:

"If we strictly limit the definition of LKS to those children who loose language and develop autistic characteristics after a seizure or seizures, LKS would remain a rare entity. However, we soon came to note a pattern where a child whose development was normal or nearly normal would, after some sort of insult (usually between ages 12 and 24 months), loose language and demonstrate autistic characteristics. The insult could be a seizure but could involve many major to minor illnesses or traumas. Thus epilepsy may be just one of many precipitants of our syndrome of interest."

A normal four year old regresses into Landau Kleffner Syndrome. It is so obvious because sudddenly this typical, verbal preschooler is unable to speak or comprehend language, practically overnight. Doctors get right on it and with prompt treatment the child might make a full recovery. An Autistic child of the same age develops LKS or LKV and it gets missed because his parents are told by doctors that the regressive symptoms are "just the Autism". Years go by before an EEG is given, if at all. By this time the LKS/LKV symptoms have gone untreated for so long that it is unlikely that child will ever fully recover speech.

That's what happened to Alex.

He first regressed into Autism at 6 months of age. Even so, Alex was quite high functioning. In fact, the Clinical Psychologist who diagnosed him came back three times because she wasn't sure he was even Autistic. And there was talk among his therapists that he might have been a savant because his intelligence was well beyond that of a three year old. Then at age four Alex was pummeled by a second regression. We expressed concern to his pediatricians and were assured, "That's just Autism. There's not much you can do." No neurologist referral. Nothing. Four years passed before the full extent of Alex's epilepsy was diagnosed. By that time he was severely Autistic, barely verbal, and back in diapers. His IQ test was 51 that year.

Had Alex been accurately diagnosed at four he would likely have high functioning Asperger's right now.

I imagine those less familiar with Autism might assume our children are marched to a neurologist upon being diagnosed. Wrong. Most neurologists don't treat Autism. 'Tis true. They treat Autistic people who have other neurological conditions such as epilepsy but they do not treat Autism. Unfortunately many pediatricians do not recognize when an Autistic child needs a neurology consult. This is how Petite Mal epilepsy in this population gets missed for years to such tragic result.

Regressive Autism speaks. It's time to listen. It is time to start listening to parents and Boston Children's Hospital. Every child with Regressive Autism deserves an EEG. Period.



1. "Boy, Interrupted" - Discover Magazine

2. Study Provides New Insight Into The Implications of Autism Onset Patterns - Johns Hopkins Kennedy Krieger Institute

3.Brain Waves A Key To Autism language Woes - CBS News

4.Magnetoencephalographic patterns of epileptiform activity in children with regressive autism spectrum disorders. Lewine JD, Andrews R, Chez M, Patil AA, Devinsky O, Smith M, Kanner A, Davis JT, Funke M, Jones G, Chong B, Provencal S, Weisend M, Lee RR, Orrison WW Jr. Department of Radiology, University of Utah

5.Pediatrics. 1997 Apr;99(4):560-6. Regression in pervasive developmental disorders: seizures and epileptiform electroencephalogram correlates. Tuchman RF, Rapin I. Department of Neurology, Miami Children's Hospital, Florida 33155, USA J Child Neurol. 2004 Aug;19 Suppl 1:S49-57 . 6. Seizures and epilepsy among children with language regression and autistic spectrum disorders.

7. Boston Children's Hospital - Landau Kleffner Clinic