Fisher Syndrome (FS)

Mr. N is a 23 yo Zerspanungsmechaniker (some kind of mechanic, I couldn’t translate it) and complained about 2 days of progressive sensory symptoms at all extremities, nasal speech and difficulty walking, about a week after an abdominal infection with diarrhea. On initial presentation (after 2 days) he showed bilateral mydriasis (sluggishly reactive), bilateral palatal palsy, hypesthesia at arms and legs and slightly slower reflexes at the upper extremities compared to the lower ones. CSF should slightly elevated protein (550) and 6 cells. MRI of the brainstem and cervical spine was negative. During the first few days, the reflexes at the upper extremity disappeared, while PSR and ASR remained, his speech improved, otherwise he was unchanged.

We discussed the differential and then reviewed Fisher’s syndrome, using the following 2 references:

  • Snyder. The Fisher variant of GBS. J Neuro-Ophthalm 2009
  • Mori. Fisher Syndrome. Curr Treatment Options in Neurology 2011

(these might not be the best references, but they are the most current ones).

Take home messages:

  • Charles Miller Fisher described 3 patients (some with brainstem symptoms) and coined the triad: areflexia, ataxia and augenprobleme (the latter is for germans only – AAA).
  • It makes no sense to call it “Miller Fisher syndrome” unless you also speak of “the Delano Roosevelt administration”
  • There are a lot of abortive GQ1B syndromes such as singular oculomotor nerve palsies, isolated ataxia and more
  • Differential: Botulism (if only motor symptoms), CPM, Wernicke, vascular, brainstem encephalitis, sarcoid, Behcet, autoimmune/paraneoplastic rhombencephalitis
  • Workup: MRI, CSF, neurophys, antibodies
  • Treatment is necessary for FS with GBS and reasonable for FS + Bickerstaff
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