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IHS Diagnosis ICD-10
6.7.3 Headache attributed to benign (or reversible) angiopathy of the central nervous system [I99] G44.81  

Diagnostic criteria:

  1. Diffuse, severe headache of abrupt or progressive onset, with or without focal neurological deficits and/or seizures and fulfilling criteria C and D
  2. "Strings and beads" appearance on angiography and subarachnoid haemorrhage ruled out by appropriate investigations
  3. One or both of the following:
    1. headache develops simultaneously with neurological deficits and/or seizures
    2. headache leads to angiography and discovery of "strings and beads" appearance
  4. Headache (and neurological deficits, if present) resolves spontaneously within 2 months

Comments:

This is a poorly understood condition characterised clinically by a severe diffuse headache of variable modes of onset: it can be abrupt, mimicking SAH, or progressive rapidly over hours or more slowly over days. It is one of the identified causes of thunderclap headache. It can be the only symptom of this condition but it is usually associated with fluctuating focal neurological deficits and sometimes seizures. Angiography is, by definition, abnormal, with alternating segments of arterial constriction and dilatation.

A number of causes have been identified: the best defined is post-partum angiopathy which has been related in some cases to use of bromocriptine. The disease is self-limiting in 1-2 months without treatment and with disappearance of the arterial abnormalities but, given the diagnostic difficulty with primary CNS angiitis, a course of steroids is sometimes given.

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