Disclosures: Milan Ristic, DO: No financial relationships or conflicts of interest

Case Description: A 19-year-old female presented multiple times to the emergency department with complaints of headache. She was discharged with suspected viral syndrome after an unremarkable non-contrast head CT. A week after discharge she developed bilateral lower extremity weakness with difficulty ambulating, tremors of her legs and hands, in addition to vision changes.

Setting: Acute Inpatient Rehabilitation

Patient: A 19-year-old female with acute onset of headaches, initially negative on imaging, found to have a rare autoimmune neurological disorder. Assessment/

Results: An MRI of the brain and spine showed diffuse meningoencephalitis of the brain and possible cord edema or lesion in T2-12. Additional neurology workup included CSF and serum autoimmune antibodies and encephalitis panels. Her hospital course was further complicated by hypothermia, hypertension, urinary retention and constipation. Send out labs were significant for positive antibody Anti-Neuronal acetylcholine receptor Ganglionic Alpha-3, consistent with autoimmune dysautonomia. The patient received intravenous immunoglobulin (IVIG) and intravenous methylprednisolone followed by improvement in her autonomic dysfunction. The patient improved functionally and cognitively during acute inpatient rehabilitation and was discharged with outpatient services on an oral steroid taper.

Discussion: Autoimmune Autonomic Ganglionopathy (AAG) is very rare and often associated with high titers of ganglionic acetylcholine receptor antibody found in the autonomic ganglia. Symptoms can include severe orthostasis, constipation, urinary retention, fixed and dilated pupils, xerostomia, and dry eyes. Treatment may include, IVIG, corticosteroids, total plasmapheresis, plasma exchange, and immunosuppressive medicines. Limited case reports show a variable course with spontaneous improvements occurring in roughly one third of patients with often times incomplete recovery.

Conclusion: AAG is a very rare disorder with a variable course and at times spontaneous improvement. Due to its rarity, conventional treatments have not been agreed upon. In this case study, the patient recovered most of her function and cognition with a short course of IVIG and oral steroids.

Level of Evidence: Level V

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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/rare-neurologic-disorder-in-high-functioning-young-female-a-case-report/