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Abstract Details

Immunoglobulin for Co-existing Chronic Autoimmune Autonomic Ganglionopathy and Neuropathy
Autoimmune Neurology
Autoimmune Neurology Posters (7:00 AM-5:00 PM)
081
Immune mediated disorders of the nervous system are related to autoantibodies directed against localized areas of nervous system tissue and are associated with typical clinical syndromes. An unusual case of co-existing symptomatic autoantibodies to both the autonomic sensory ganglia and the somatic peripheral nerve ganglioside is presented. 

A 38 year old wheelchair bound female self referred to neurology for a multisystem complex. About age 23, she began suffering from numerous clinical syndromes and symptoms including postural orthostatic tachycardia syndrome, adrenal insufficiency and Graves', gastrointestinal and skin conditions with severe intermittent erythema patches, severe fatigue, fibromyalgia, chronic pain on opioids, migraine and other symptoms which lead to a homebound state for many years. On exam her vital signs were compensated by glucocorticoids, she had sensory ataxia with severe gait impairment. Laboratories targeting the neurologic findings revealed high titers of autoantibodies against both the nicotinic ganglionic acetylcholine receptor (nAChR) and ganglioside GD1b.

Following three months of IVIG therapy, she ambulated and shopped outside her home for the first time in 15 years.  Her skin condition resolved.  After several more months, she was able to reduce her opioids and cortisone doses, and her gastrointestinal issues resolved.  Repeat laboratories showed very decreased GD1b titers, but less so the nAChR titers.  She continues with IVIG treatments. 

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This case demonstrates that autoimmune nervous system disorders with different pathologic localizations may co-exist. There are no randomized treatment trials for autoimmune autonomic ganglionopathy with anti-nChR antibody; case studies suggest treatment with IVIG, plasma exchange and immunomodulatory drugs are effective.  These same treatments are considered effective for the somatic peripheral neuropathy of sensory ataxia associated with anti-ganglioside antibody GD1b  Careful integration of the patient's medical history and characterization of the neurological syndromes on exam are critical since treatments are effective despite years of severe chronicity.
Authors/Disclosures
Marian P. LaMonte, MD, MSN, FAAN (Ascension St. Agnes Hospital)
PRESENTER
Dr. LaMonte has nothing to disclose.