Abstract Details

Title Wet, Wacky, and Wobbly Without Hydrocephalus

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-026

Objective Ataxia, incontinence, and confusion comprise Hakim’s triad of normal pressure hydrocephalus which results from dysfunction of the frontal periventricular white matter tracts. Here, we highlight a case consisting of the cardinal features of Hakim’s triad due to a vastly different etiology.

Background A 48-year-old man with no history of head trauma or significant medical comorbidities presented to the emergency department with a 3-month history of progressive ataxia, urinary incontinence, and confusion.

Design/Methods NA

Results Head CT showed hyperattenuation adjacent to the falx cerebri, interpreted as possible subdural hemorrhage with surrounding cerebral edema. Subsequent MRI revealed hypertrophic pachymeningeal enhancement of the entire falx cerebri, with extensive edema of the surrounding frontal white matter. MRI studies of the cervical and thoracic spine were normal. Serum studies revealed elevated IgG4 titers, myeloperoxidase-ANCA titers, and anti-centromere antibodies. CSF studies revealed elevated protein. Meningeal biopsy was nondiagnostic. The patient was diagnosed with a focal autoimmune hypertrophic pachymeningitis. He was treated with intravenous methylprednisolone followed by a maintenance dose of oral prednisone. Symptoms resolved within 3 weeks. MRI performed at 7 weeks demonstrated interval decrease in nodular enhancement of the falx and resolution of white matter edema.

Conclusions We report a case of combined IgG4- and myeloperoxidase-ANCA-related hypertrophic pachymeningitis confined to the falx cerebri presenting with insidious-onset ataxic gait, incontinence, and confusion, responsive to corticosteroid therapy. Hypertrophic pachymeningitis is a rare inflammatory disorder involving thickening of intracranial or spinal dura mater. Although it most commonly presents as isolated headache, focal neurologic deficits have known to occur depending on the location of meningeal inflammation. This was highlighted in our patient by dysfunction of sacral motor fibers in the parafalcine white matter and the cingulate gyrus. A high index of suspicion is needed to diagnose this underrecognized, potentially life-threatening, but treatable immune-mediated condition.

Authors/Disclosures
Peter Hung PRESENTER	No disclosure on file
	No disclosure on file
Kunal V. Desai, MD (Yale Neurology - Greenwich)	Dr. Desai has nothing to disclose.

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