A Serious Version of Migraine with Aura – CADASIL

An heriditary version of migraine with aura could be cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Early on, nothing is evident even on MRI; but later, damage can be seen on MRI from this vasculopathy. Clinical features include migraine with and without aura, cognitive decline, ischemic events (mini-stroke events), and mood disorders. Imetrix/Sumatriptan – like vasoconstrictor agents potentially contraindicated

Curr Pain Headache Rep. 2017 Apr;21(4):21. doi: 10.1007/s11916-017-0621-0.
Recognizing CADASIL: a Secondary Cause of Migraine with Aura.
Burkett JG et al
https://www.ncbi.nlm.nih.gov/pubmed/28281108

  • notch 3 gene on chromosome 19
  • migraine with aura in 20-46% of CADASIL especially in women
  • women first aura age 25; while in men age 30 – 35 – older than usual migraine case
  • may have stroke like symptoms
    – visual, sensory and/or speech or language symptoms together with motor weakness)
    – brainstem aura (at least two of dysarthria, vertigo, tinnitus, hyperacusis, diplopia, ataxia and a decreased level of consciousness.)
  • confusional migraine aura- disorientation and antero-grade amnesia, with or without a decreased level of consciousness ca occur
  • There is a conditon called “CADASIL coma” – an encephalopathy¬† often diagnosed as a viral condition that occurs in 11% of cases.
  • About 1/2 will develop subcortical lacunar infarcts – Ischemic stroke follow months of increased headaches with aura and would have similar though more lasting features to aura
  • Notch3 receptor mutations can be found on genetic testing
  • Characteristic MRI changes eventually occur

Migraine treatment: as usual, anticonvulsants worked best but other could be tried

  • propranolol 10/22 response
  • calcium channel blockers 3/4 response (rarely used though)
  • pizotifen (Sandimigran) 8/16 respond
  • topiramate or valproate – 4/6 respond
  • amitriptyline – 6/15 response
  • using combos (amitriptyline +)helped a few

Interestingly, “A few studies have also reported a significant improvement of CADASIL migraine frequency and severity with acetazolamide with doses ranging from 125 to 500 mg daily.”

  • painkillers acetominophen/asa or ibuprofen with or without codeine will work 2/3 of time.
  • anticoagulants avoided
  • avoidance of triptans is more theoretical though they found it worked 9/18 times to abort headache
  • after an ischemic event, headaches become better

Comment Рthe migraine with aura occurred later than usual for migraine sufferers. Early MRI imaging cannot be relied on.  At this point there is no treatment to prevent progression of disease. Interesting about acetazolamide Рbrings down the brain fluid blood pressure.

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