Rare Disease Day Part 2: Landau Kleffner Syndrome


Artwork by Calvin “Sonny” Clarke


We are continuing the series highlighting rare diseases. Additionally, March is Developmental Disabilities month, so this is a perfect opportunity to offer some education on a disease that is both rare and developmental in nature.


Summary


Landau Kleffner Syndrome (LKS) or acquired epileptiform aphasia is a rare, neurological, childhood disorder characterized by language regression and seizures. Symptoms typically start between 3-7 years old. It’s more typical in males than females. The causes are unknown but currently genetic and autoimmune mechanisms are suspected. LKS causes loss of previously acquired receptive and expressive language and seizures. Standard treatment includes antiepileptic medication and introducing AAC.


Relevance to SLP/AUD

  • Remember to include LKS in your differential diagnosis for autism because LKS is often initially misdiagnosed as Autism which can lead to delays in treatment and management interventions.

  • Auditory verbal agnosia or “word deafness” is a prominent early sign.

  • Impaired language comprehension, among other signs, is often one of the first overt symptoms of LKS, thus audiologists and speech-language pathologists can play an important role in identifying the disorder and helping to prevent misdiagnosis (ASHA).

  • Having a basic understanding of rare diseases is important for recognizing them in clinical practice to expedite correct diagnosis which means sooner access to proper treatment and management.

  • Speech and language therapy for LKS should focus on functionally relative words.

Noah's story

The following story was originally published on Action Medical Research


Previously a happy, healthy baby and toddler, Noah started to experience seizures aged three. After many tests, he was diagnosed with a childhood epilepsy disorder. Then Noah started to stutter and slur his words.


To his family’s distress, within just two weeks, Noah’s ability to talk disappeared altogether. He was able to pick up British Sign Language quickly and music therapy helped him to express himself. But, desperately worried, Madeka and Ryan opted for a private consultation and Noah, who was experiencing up to 40 seizures a day, was diagnosed with LKS.


Noah, who is now 10, takes seven different medicines each day including steroids. Thankfully he no longer has seizures and, following intensive speech and language therapy, he is able to talk again.


Citations:

Alpern, C. S.,. Identification and treatment of Landau-Kleffner syndrome. The ASHA Leader. Retrieved February 28, 2022, from

https://leader.pubs.asha.org/doi/10.1044/leader.FTR3.15112010.34


Noah's story. Action Medical Research. (2021, February 5). Retrieved February 28, 2022, from https://action.org.uk/research/family-stories/noahs-story


Cognitive linguistic treatment. (n.d.). Retrieved February 28, 2022, from https://journals.sagepub.com/doi/full/10.1177/2329048X211022196

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