G40.42

Cyclin-Dependent Kinase-Like 5 Deficiency Disorder

FY2027Chapter 6: Diseases of the nervous system/G40–G47 Episodic and paroxysmal disorders

BillableComplete code, valid to report

G40.42 (also written G4042) is a billable ICD-10-CM diagnosis code for cyclin-Dependent Kinase-Like 5 Deficiency Disorder. It is part of the FY2027 code set, effective October 1, 2026. It is one of the 58 codes in category G40 (Epilepsy and recurrent seizures). The Alphabetic Index lists it under 2 entries. Its coding notes include a Use additional code instruction, 5 Excludes1 entries and 12 Excludes2 entries.

Use additional code

Add the listed code as a secondary code, if known.

This code
  • code, if known, to identify associated manifestations, such as:
    • cortical blindness (H47.61-)
    • global developmental delay (F88)

Excludes1

Not coded here. The listed conditions generally should not be reported together.

Inherited from G40 Epilepsy and recurrent seizures
  • conversion disorder with seizures (F44.5)
  • convulsions NOS (R56.9)
  • post traumatic seizures (R56.1)
  • seizure (convulsive) NOS (R56.9)
  • seizure of newborn (P90)

Excludes2

Not included here. Both codes may be reported if the patient has both conditions.

Inherited from G40 Epilepsy and recurrent seizures
Also from chapter/block level (8 notes)
Inherited from Chapter 6 Diseases of the nervous system
  • certain conditions originating in the perinatal period (P04-P96)
  • certain infectious and parasitic diseases (A00-B99)
  • complications of pregnancy, childbirth and the puerperium (O00-O9A)
  • congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
  • endocrine, nutritional and metabolic diseases (E00-E88)
  • injury, poisoning and certain other consequences of external causes (S00-T88)
  • neoplasms (C00-D49)
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)

Care settings

How CMS programs treat this code. These are CMS claim edits and list entries, not coverage or eligibility rules.

Home Health Primary

Accepted as the primary (principal) diagnosis on a home health claim. The Home Health Grouper assigns it to the Neuro Rehabilitation clinical group.

Source: CMS Home Health Grouper Software v07.2.26, effective October 1, 2026

Inclusion terms

Conditions classified to this code.

This code
  • CDKL5

Notes

Inherited from G40 Epilepsy and recurrent seizures
  • the following terms are to be considered equivalent to intractable: pharmacoresistant (pharmacologically resistant), treatment resistant, refractory (medically) and poorly controlled

Alphabetic Index terms

Terms the official Alphabetic Index lists for this code. 2 entries