Successful Treatment with High-Dose Immunoglobulin of a Complicated Patient with Catatonia and Central Nervous System Autoantibodies

Abstract

Ms. X is a 52-year-old Filipino female with no prior psychiatric or medical history who initially presented to an outside hospital for new onset headache, neck pain, and altered mental status. She was admitted inpatient medicine service and was found to have HIV/AIDs and cryptococcal meningoencephalitis. Ms. X had a good response to appropriate antifungal therapies and was able to discharge home with full resolution of symptoms on high dose fluconozaole. Unfortunately, Ms. X developed transaminitis on high dose fluconazole, the dose was reduced and she presented to our institution with recurrent cryptococcal meningoencephalitis as evidenced by elevated titers of cryptococcal antigen in CSF and MRI studies showing T2 and FLAIR hyperintensities in bilateral basal ganglia, thalamus and medial temporal lobes and evidence of intracranial hypertension. Consequently, Ms. X developed prominent neuropsychiatric symptoms including agitation, cognitive impairment, paranoia, visual hallucinations and symptoms of catatonia. Despite adequate treatment of the cryptococcal infection evidenced by decreasing cryptococcal antigen titers in CSF, and appropriate psychiatric treatment, her neuropsychiatric symptoms persisted and her MRI studies continued to show signs of neuroinflammation in the aforementioned areas. Antibody testing for basal ganglia targets was completed to investigate a possible non-infectious cause of neuroinflammation and neuropsychiatric symptoms. Ms. X was found to have elevated titers of anti-D1, anti-GM1 and anti-tubulin receptor antibodies in her CSF. Due to the severity and treatment resistant nature of her neuropsychiatric symptoms, she was treated with 2 g/kg/day IVIG for 4 days. She showed significant improvement in her neuropsychiatric symptoms after 3 days and full resolution of her neuropsychiatric symptoms 1 week following IVIG. Given remarkable clinical response to IVIG a second round of IVIG was administered 2 weeks later in order to sustain the response. After several months of prolonged hospitalization, Ms X was able to discharge to the care of her family and to date her neuropsychiatric symptoms remain in remission. We propose Ms. X’s persistent, treatment resistant neuropsychiatric symptoms were related to a post-infectious inflammatory encephalopathy that responded robustly to IVIG.