Landau-Kleffner Syndrome (LKS)
The interplay of epileptic activity and the disruption to the language network makes therapeutic development for Landau-Kleffner syndrome (LKS) quite difficult. Protheragen is focused on providing the most advanced diagnostic and therapeutic development solutions for the difficult problems of LKS management. With our enduring unwavering support in the LKS therapeutic research, we offer complete and unparalleled solutions to any of your scientific research problems.
Overview of Landau-Kleffner Syndrome (LKS)
Landau-Kleffner syndrome (LKS), or acquired epileptic aphasia, is a rare neurobiological disorder associated with the loss of language skills in conjunction with the emergence of epileptiform phenomena. This disorder usually develops in children who were previously developing normally between the ages of 3 to 7 years. LKS is estimated to occur in 1 in 1,000,000 children, which poses a distinctive treatment concern because of the disorder's combined neurobiological and developmental pathology.
Fig.1 The epilepsy-aphasia spectrum (EAS). (Antonio Benítez-Burraco, et al., 2023)
Pathogenesis of Landau-Kleffner Syndrome (LKS)
Landau-Kleffner syndrome (LKS) is characterized by dysfunctional glutamatergic signaling within the language networks of the cortex, particularly involving GRIN2A mutations, which weaken synaptic plasticity through NMDA receptors, along with epileptiform activity (continuous spike-and-wave during slow-wave sleep) that disrupts the thalamocortical circuits during crucial periods of language development which culminates in an acquired aphasia and preserve non-verbal cognition.
Therapeutic Development for Landau-Kleffner Syndrome (LKS)
| Drug Names | Mechanism of Action | Targets | Research Phase |
| Diazepam | Positive allosteric modulator of GABAA receptors; enhanced chloride influx | GABAA receptor | Approved |
| Acetazolamide | Carbonic anhydrase inhibition; reduced neuronal excitability via pH modulation | Carbonic anhydrase II/IV | Early research |
| Valproic Acid | HDAC inhibition + GABAergic enhancement; broad-spectrum anti-seizure effects | GABA transaminase/HDAC enzymes | Approved |
Disclaimer: Protheragen focuses on providing preclinical research services. This table is for information exchange purposes only. This table is not a treatment plan recommendation. For guidance on treatment options, please visit a regular hospital.
Our Services
At Protheragen, we specialize in preclinical research in Landau-Kleffner syndrome (LKS) and strive to offer all-inclusive biomarker identifying and therapeutics development services. Our capabilities in disease modeling includes patient-derived iPSCs, genetically engineered models, and sophisticated blood-brain barrier (BBB) models assessing drug efficacy and neuroprotection. We offer our collaborators target validation, optimization of lead candidates, and complete preclinical research services.
Therapeutic Development Services
By Mechanism of Action
Disease Model Development Services
In Vitro Model Development
- GRIN2A Knockout Model: GRIN2A mutations are linked to ~20% of LKS cases, affecting NMDA receptor function.
- Tetrodotoxin (TTX) Induced Model: Temporarily silences auditory/language cortex, mimicking functional aphasia.
- Other Models
Specializing in comprehensive preclinical assessment, Protheragen offers professional pharmacodynamic (PD), pharmacokinetic (PK) and toxicology research services to accelerate the therapeutic development of Landau-Kleffner syndrome (LKS). If you are interested in our services, please feel free to contact us for more details and quotation information of related services.
Reference
- Antonio Benítez-Burraco, et al. "Language deficits in GRIN2A mutations and Landau–Kleffner syndrome as neural dysrhythmias." Journal of Neurolinguistics (2023).