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5. HEADACHE ATTRIBUTED TO HEAD AND/OR NECK TRAUMA 6. HEADACHE ATTRIBUTED TO CRANIAL OR CERVICAL VASCULAR DISORDER 6.1. Headache attributed to ischaemic stroke or transient ischaemic attack6.2. Headache attributed to non-traumatic intracranial haemorrhage [I62] 6.3. Headache attributed to unruptured vascular malformation [Q28] 6.4. Headache attributed to arteritis [M31]6.5. Carotid or vertebral artery pain [I63.0, I63.2, I65.0, I65.2 or I67.0]6.6. Headache attributed to cerebral venous thrombosis (CVT) [I63.6] 6.7. Headache attributed to other intracranial vascular disorder6.7.1. Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) [I67.8] 6.7.2. Mitochondrial Encephalopathy, Lactic Acidosis and Stroke-like episodes (MELAS) [G31.81] 6.7.3. Headache attributed to benign (or reversible) angiopathy of the central nervous system [I99] 6.7.4. Headache attributed to pituitary apoplexy [E23.6] Bibliography7. HEADACHE ATTRIBUTED TO NON-VASCULAR INTRACRANIAL DISORDER 8. HEADACHE ATTRIBUTED TO A SUBSTANCE OR ITS WITHDRAWAL 9. HEADACHE ATTRIBUTED TO INFECTION 10. HEADACHE ATTRIBUTED TO DISORDER OF HOMOEOSTASIS 11. HEADACHE OR FACIAL PAIN ATTRIBUTED TO DISORDER OF CRANIUM, NECK, EYES, EARS, NOSE, SINUSES, TEETH, MOUTH OR OTHER FACIAL OR CRANIAL STRUCTURES 12. HEADACHE ATTRIBUTED TO PSYCHIATRIC DISORDER
| IHS |
Diagnosis |
ICD-10 |
| 6.7.1 |
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) [I67.8] |
G44.81
|
Diagnostic criteria:
- Attacks of migraine with aura, with or without other neurological signs
- Typical white matter changes on MRI T2WI
- 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.
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IHS vs. ICD-10
To facilitate headache diagnosis in daily practice, the classification provides the corresponding WHO ICD-10NA codes for each IHS code.
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IHS Subcommittee
The Classification Subcommittee prepares and revises the International Classification of Headache Disorders.
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Cephalalgia
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