More IHS Resources

The International Headache Society (IHS) is the world's membership organisation for all whose professional commitment, whatever their discipline, is to helping people whose lives are affected by headache disorders.Visit the IHS website

univadis
IHS Diagnosis ICD-10
6.7.1 Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) [I67.8] G44.81  

Diagnostic criteria:

  1. Attacks of migraine with aura, with or without other neurological signs
  2. Typical white matter changes on MRI T2WI
  3. Diagnostic confirmation from skin biopsy evidence or genetic testing (Notch 3 mutations)

Comment:

CADASIL is a recently identified autosomal dominant (with some sporadic cases) small artery disease of the brain characterised clinically by recurrent small deep infarcts, subcortical dementia, mood disturbances and migraine with aura.

Migraine with aura is present in one third of cases and, in such cases, is usually the first symptom of the disease, appearing at a mean age of 30, some 15 years before ischaemic strokes and 20-30 years before death. Attacks are typical of 1.2 Migraine with aura except for an unusual frequency of prolonged aura.

MRI is always abnormal with striking white matter changes on T2WI. The disease involves the smooth muscle cells in the media of small arteries and it is due to mutations of Notch 3 gene. The diagnosis is made on a simple skin biopsy with immunostaining of Notch 3 antibodies.

CADASIL is an excellent model to study the pathophysiology of migraine with aura and the relationships between it and ischaemic stroke.

Back

Top

 

Print

Recommend