{
  "abstract": "We report three cases describing ITPR-1 antibody-associated disease, detected in approximately 0.015% of patients undergoing paraneoplastic antibody testing. Antibodies to ITPR-1, highly expressed in the central nervous system (particularly the cerebellar purkinje cells) and in the peripheral nervous system, demonstrate heterogeneous clinical presentations.Case 1: 59-year-old female presented with subacute cerebellar ataxia and vertigo, associated with 8kg weight loss. CSF analysis revealed pleocytosis (20x10^6/L mononuclear cells) and elevated protein (670 mg/L). Serum and CSF analysis detected ITPR-1 antibodies, and subsequent investigations revealed stage IIIC lung adenocarcinoma. Despite immunotherapy with methylprednisolone and plasmapheresis, as well as palliative radiotherapy, she experienced rapid functional deterioration.Case 2: Serum ITPR-1 antibodies were detected in a 73-year-old male as workup for a 20-year history of episodic ataxia, oscillopsia and vertigo. He was lost to follow-up and immunotherapy was never instigated. Seven year later, he developed small lymphocytic lymphoma. His neurological symptoms remained stable despite successful lymphoma treatment.Case 3: 44-year-old male presented with recurrent left brachial plexopathy post influenza vaccination, with unremarkable brachial plexus/cervical spine imaging. An inflammatory neuropathy screen identified ITPR-1 antibodies in serum. Malignancy screening was negative. He has shown limited response to intravenous immunoglobulin therapy at 3 months follow-up.ITPR-1 antibody-associated disease, primarily involving cerebellar ataxia and peripheral neuropathy, is also described in encephalopathy, seizures, myelopathy and spastic paraparesis. Screening for underlying malignancy is crucial, as is reported in approximately 45% of cases. Treatment responses may be poor, with limited repose to immunotherapy or treatment of the primary tumour.",
  "authors": [
    {
      "affiliations": [
        "Princess Alexandra Hospital, Woolloongabba, QLD, Australia"
      ],
      "name": "Sandra Elias"
    },
    {
      "affiliations": [
        "Immunology Department, Pathology Queensland, Brisbane, QLD, Australia"
      ],
      "name": "Kerri Prain"
    },
    {
      "affiliations": [
        "Princess Alexandra Hospital, Woolloongabba, QLD, Australia"
      ],
      "name": "Alex Lehn"
    },
    {
      "affiliations": [
        "Princess Alexandra Hospital, Woolloongabba, QLD, Australia"
      ],
      "name": "Nabeel Sheikh"
    },
    {
      "affiliations": [
        "Princess Alexandra Hospital, Woolloongabba, QLD, Australia"
      ],
      "name": "Po Yang"
    },
    {
      "affiliations": [
        "Princess Alexandra Hospital, Woolloongabba, QLD, Australia"
      ],
      "name": "Cullen O’Gorman"
    }
  ],
  "title": "3446 Anti-inositol 1,4,5 triphosphate receptor-1 (ITPR-1) associated neurological syndromes: case series",
  "uid": "3827bc88-4ec9-5040-a829-9e315cda6e5f"
}