Table 5 Proposed diagnostic criteria for the new category of headache attributed to genetic disorder

15.1 Headache attributed to CADASIL

 A. Attacks of headache with or without neurological symptoms

 B. Typical white matter changes on MRI (T₂W)

 C. Diagnostic confirmation from skin biopsy evidence or genetic testing (Notch 3 mutations)

15.1.1 Migraine without aura attributed to CADASIL

 A. Attacks of headache fulfilling criteria A–D for 1.1 Migraine without aura

 B. Attributed to CADASIL

15.1.2 Migraine with aura attributed to CADASIL

 A. Attacks of headache fulfilling criteria A and B for 1.2 Migraine with aura

 B. Attributed to CADASIL

15.1.2.1 Typical aura with migraine headache attributed to CADASIL

 A. Attacks of headache fulfilling criteria A–D for 1.2.1 Typical aura with migraine headache

 B. Attributed to CADASIL

15.1.2.2 Typical aura without headache attributed to CADASIL

 A. Attacks of headache fulfilling criteria A–D for 1.2.3 Typical aura without headache

 B. Attributed to CADASIL

15.1.2.3 Hemiplegic migraine attributed to CADASIL

 A. Attacks of headache fulfilling criteria A–C for 1.2.5 Sporadic hemiplegic migraine

 B. Attributed to CADASIL

15.1.2.4 Basilar-type migraine attributed to CADASIL

 A. Attacks of headache fulfilling criteria A–D for 1.2.6 Basilar-type migraine

 B. Attributed to CADASIL

15.1.3.1 Status migrainosus attributed to CADASIL

 A. Attacks of headache fulfilling criteria A and B for 1.5.2 Status migrainosus

 B. Attributed to CADASIL

15.1.3.2 Persistent aura without infarction attributed to CADASIL

 A. Attacks of headache fulfilling criterion A for 1.5.3 Persistent aura without infarction

 B. Attributed to CADASIL

15.1.5 Unspecified headache

 A. Headache is or was present

 B. Not enough information to classify the headache in a patient otherwise diagnosed with CADASIL