Abstract
Objective: We seek to characterize the clinical symptoms and describe the treatment paradigm of a rare case of autoimmune encephalitis secondary to neuronal ganglionic acetylcholine receptor autoimmunity.
Background: Autoimmune encephalopathy can be used to describe a range of neuropsychiatric disorders in which autoantibodies are directed against central nervous system targets. While nicotinic ganglionic acetylcholine receptor autoimmunity has been commonly reported in dysautonomia and peripheral neuropathy, this type of autoimmunity has rarely been shown to cause encephalopathy. Currently, there is limited data regarding the cognitive and behavioral changes which occur in this syndrome, and the efficacy of immunomodulatory therapy.
Design/Methods: A case report.
Results: A 54 year-old previously healthy, right-handed male presented to clinic for evaluation of one year of cognitive impairment which was simultaneous with the onset of severe eczema. He reported weekly cognitive fluctuations and brief involuntary jerks. Symptoms involved difficulty with executive function, working memory, word finding, and calculations; there were no symptoms suggestive of dysautonomia. Cognitive exam revealed poor orientation, impaired comprehension and repetition, dysfunction of working memory and response inhibition, phonemic errors, paucity of functional words, ideomotor apraxia, acalculia, apraxic agraphia, and right-left confusion. Serologies indicated a high antibody titer to alpha-3 neuronal ganglionic acetylcholine receptor antibodies. MRI showed nonspecific T2 lesions. During IVIG treatment, jerking movements ceased and symptoms rapidly improved, particularly in language and short-term memory. His EEG, which was consistent with metabolic encephalopathy pretreatment, normalized after his IVIG course.
Conclusions: Encephalopathy associated with neuronal ganglionic acetylcholine receptor antibodies has been rarely reported, and tends to occur with dysautonomia and peripheral neuropathy. In this case, we describe the spectrum of cognitive findings our patient presented with along with his clinical and electrographic improvement following treatment with IVIG.
Disclosure: Dr. Parker has nothing to disclose. Dr. Rehl has nothing to disclose. Dr. Saadatpour has nothing to disclose. Dr. Tariq has nothing to disclose. Dr. Doty has nothing to disclose. Dr. Seshadri has nothing to disclose. Dr Heilman has nothing to disclose.
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