Get a grip!

roast_chicken
Not a HaNDL but a bavarian HeNDL

When a disease is discovered nowadays, it needs to be assigned a proper acronym – see CLIPPERS, MELAS, CADASIL. If we were living in the good ole eponymic days of, say, Steele-Richardson-Olszewski, the HaNDL syndrome would be named Swanson-Bartleson-Whisnant – the authors of a 1980s Neurology paper on the condition we covered in our rounds today. Or better: by Stigler’s law it should be named Berg-Williams-Syndrome after the 1995 Neurology article that coined the acronym HaNDL.

By all we know about the inflammatory pathophysiology of migraine, an increase in frequency and intensity of migraine auras should be able to produce a pleocytosis, but this is not the explanation for HaNDL, for it should enjoy all the epidemiologic characteristics of migraine then. And it doesn’t.

It is men of middle age (40-70, not young women) with only rarely (slightly more than chance predicts) any migraine in their history, who – sometimes after a preceding viral illness – suffer a series of episodes over days to weeks that resemble aura (except: less visual symptoms, more aphasia, more sensorimotor disturbances) in their development and migratory nature, but take way too long (a few hours rather than 10-60 minutes), accompanied or followed by headaches that resemble migraine with slightly less phobias (osmo-, kineto-, photo-, phonophobia). The usual workup (CT, MRI, labs) is negative, but there is a lymphocytotic pleocytosis of > 15/µl and often a raised CSF opening pressure.

Of course, as in any case of focal symptoms plus pleocytosis (aka encephalitis), we send off the standard microbiology tests (HIV, TPHA, borreliosis, PCR for HSV, VZV), start acyclovir and maybe ceftriaxone at once and wait until everything comes back negative. Then we are left with the hopes of a spontaneously resolving syndrome – by definition it should take weeks or months to clear.

In my experience, the usual migraine therapy (iv high dose NSAIDs = metamizol or ASS, plus antiemetics – MCP or dimenhydrinate) covers each episode but does not prevent the next. Steroids might, but don’t last long enough. I usually treat with spreading depression drugs (valproate, topiramate) and hope for the best.

The disease certainly is underrecognized, being replaced by “a minor encephalitis”. There should be some studies of  autoimmune mechanisms and antibodies, but I know of no proper results yet. So, if you are faced with your next HaNDL why not send of an experimental panel?

References

Migraine in the Emergency Room

We are currently writing new SOPs for some of the standard neurological emergency room problems – one of them being classical migraine. So don’t worry about the differential, sinus vein thrombosis etc. but assume a classical migraine presentation – how do you treat this? Luckily, there is a good reference for this situation in a 3-part series in Headache 2012 (available on our local neuro drive). Here is my personal view on this:

  • Start with Novalgin (Metamizol) 1g + Vomex (Dimenhydrinat which is roughly Diphenhydramin) iv
  • After 20 min add Mg 1 ampule

Next choose among the following options:

  • Promethazin 50 mg or Haloperidol 5 mg (ECG!) iv
  • Valproate 300-300 mg iv
  • Dexamethasone 10 mg iv
  • Propofol 30-20-20-20-20 mg iv until pt is sleeping (my favorite, ambu bag!, monitor)
  • Sumatriptan 6 mg sc or 10 mg intranasally.

Remarks:

  • Quite surprisingly, triptans don’t work very well in the ER unless parenterally – which is quite expensive. Don’t give up on it, though, if you are lucky to have the 40€ one hit costs.
  • I don’t like opiates for migraine – it might lead to hypersensitivity.
  • I don’t like Paracetamol ~ Acetaminophen, because there are no iv studies for it.
  • I like ASS 1g iv but again there is no proper iv study for it.
  • Dihydroergotamin is not available for iv in Germany