Neurology & Pain Management Coding Alert

Question: What is the correct diagnosis for CADASIL?

Minnesota Subscriber

Answer: The best primary diagnosis is 758.5 (Other conditions due to autosomal anomalies). The ICD-9-CM coding guidelines require that you include additional codes to describe the manifestations of a syndrome when you can't submit a specific code for the condition. In this case you might choose a diagnosis representing present complications of the disorder in the patient, such as 323.9 (Unspecified causes of encephalitis, myelitis, and encephalomyelitis) for leukoencephalopathy or 435.9 (Unspecified transient cerebral ischemia) for TIA.

Definition: CADASIL is the acronym for Cerebral Autosomal Dominant Arteriopathy with Subcortical Infacts and Leukoencephalopathy. CADASIL is a genetic condition, inherited from one affected parent, and is the most common form of hereditary stroke disorder. The most common clinical signs of CADASIL are migraine headaches and transient ischemic attacks (TIA) or strokes with confirmation by MRI. Symptom onset usually occurs between ages 40 and 50.