AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2020 Issue 4; New/Revised ICD-10-CM Codes

Cyclin-Dependent Kinase-Like 5 Deficiency Disorder

Code G40.42, Cyclin-Dependent Kinase-Like 5 Deficiency Disorder, was created to identify a developmental epileptic encephalopathy that is caused by mutations in the cyclin-dependent kinase-like 5 (CDKL5) gene. The CDKL5 gene provides instructions for making a protein that is essential for normal brain and neuron development. A deficiency of CDKL5 reduces the amount of functional CDKL5 protein or alters its activity in neurons. The hallmark of CDKL5 deficiency disorder is the onset of seizures at a very young age that are severe and difficult to control with medication. There are neurodevelopmental impairments that cause delays in cognitive, motor...

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