Abstract Details

Title Hashimoto's Encephalopathy as a Treatable Adult-Onset Cerebellar Ataxia Mimicking Spinocerebellar Degeneration

Topic Neurotoxicology

Presentation(s) P01 - (-)

Poster/Presentation
Number 056

Objective

Background HE presents with a variety of neurologic and neuropsychiatric features. We recently reported on a patient with HE who presented with a sporadic adult-onset cerebellar ataxia mimicking spinocerebellar degeneration (SCD) and showed an excellent response to corticosteroid therapy. Moreover, we have discovered serum anti-NH2-terminal of ?-enolase autoantibodies (anti-NAE Abs), which demonstrated high specificity for HE and a useful diagnostic marker for HE.

Design/Methods The clinical features, treatments, laboratory features, brain imaging, and anti-NAE autoantibodies were investigated in 13 patients who presented with sporadic adult-onset cerebellar ataxia and fulfilled the HE diagnostic criteria (anti-thyroid antibodies and responsiveness to immunotherapy). Other causes of cerebellar degeneration were excluded.

Results All of the patients presented with truncal ataxia, but nystagmus was uncommon (17%). Eight patients had an insidious onset that mimicked SCD, but brain imaging showed little or no cerebellar atrophy in all of the patients. Patients with serum anti-NAE Abs (n = 8) did not have nystagmus and tended to respond better to immunotherapy than the anti-NAE Abs negative patients. Eleven of 12 patients were euthyroid. Elevation of protein in CSF was observed in only 1 patient (8%), and the EEG abnormality was detected in 4 patients (36%).

Conclusions The present study suggests that insidious adult-onset and truncal ataxia are common in the cerebellar ataxic form of HE, which mimics SCD, but that nystagmus and severe cerebellar atrophy are uncommon. Anti-thyroid and anti-NAE Abs may be useful for diagnosing the cerebellar ataxic HE.

Authors/Disclosures
PRESENTER	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Masamichi Ikawa (University of Fukui)	No disclosure on file
	No disclosure on file
Makoto Yoneda (Univ of Fukui)	No disclosure on file

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