A 68‐year‐old man with history of remote ischemic stroke, diabetes, and tobacco abuse presented to a community hospital with a complaint of ataxia. He had fallen to the floor after arising from sleep on the morning of admission. He had poorly described visual complaints of 1–2 days' duration and had felt unsteady on his feet for a similar period. He reported recent URI symptoms. On initial exam, strength was intact and gait was ataxic, and reflexes were reported as hypoactive. Head CT was negative for acute changes. Patient was admitted for possible posterior circulation stroke; however, subsequent MRI showed underlying diffuse white matter disease and old basal ganglia lacunae, but no acute changes. The following day neurology consult was obtained, and reexam revealed ophthalmoplegia, areflexia, and persistent ataxia. Lumbar puncture was obtained and showed albuminocytologic dissociation, with elevated protein of 86, few white cells in normal ratio with red cells seen only in tube 1, and CSF glucose consistent with serum glucose. These findings were consistent with the Miller Fisher syndrome (MFS) variant of Guillain‐Barré syndrome (GBS). Patient was therefore treated with 2 g/kg of IVIG over 5 days. The patient's ataxia slowly improved, and there was early resolution of his ophthalmoplegia. He remained areflexic. He was transferred to acute inpatient rehab with goals of further improvement of ataxia and eventual discharge to home.
MFS is an uncommon variant of GBS comprising only 5% of cases in the United States. This form of polyneuropathy presents with areflexia, ophthalmoplegia, and ataxia. Pathophysiology of MFS involves immune‐mediated inflammation of nerves, with the presence of anti‐GQ1b antibodies occurring in 90% of patients. Triggers to MFS may include GI and respiratory illnesses, surgery, and rarely vaccination. Though less commonly, MFS can progress to quadriparesis and respiratory compromise and thus warrants hospitalization. Diagnosis is made by history, exam, CSF analysis, and EMG study (showing abnormal sensory conduction). Treatment is with IVIG or plasma exchange. Course is usually to complete recovery over 2–4 weeks.
Prior history of stroke appropriately leads to concern for recurrent stroke in patients presenting with ataxia. History for recent illness and careful examination of reflexes and extraocular movements should be performed in these patients. Negative neuroimaging for acute event and characteristic findings should lead to consideration of the Miller Fisher syndrome variant of Guillain‐Barré syndrome.
To cite this abstract:Grohman M, Geyer C, Bobker D. Acute Ataxia with Negative Neuroimaging: Consider an Uncommon Polyneuropathy. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 259. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/acute-ataxia-with-negative-neuroimaging-consider-an-uncommon-polyneuropathy/. Accessed November 22, 2019.