Anti-Purkinje Cell Antibody Autoimmune Cerebellar Ataxia: A pediatric case report
Zachary Cox1, Naeem Mahfooz2
1School of Medicine, 2Neurology, University of Toledo
Objective:
To report a novel case of autoimmune cerebellar ataxia secondary to reactive inflammation.
Background:
Autoimmune cerebellar ataxia (ACA) is a rare condition and is typically a paraneoplastic process. ACA caused by anti-Tr antibody targets Purkinje cells in the cerebellum and is often a devastating condition with poor prognosis that occurs secondary to Hodgkin Lymphoma. Here we present a case of ACA caused by anti-Tr antibody presenting months after extensive second and third degree burns without evidence of Hodgkin Lymphoma.
Results:
An 11 year-old female presented with 6 days of acute onset progressive ataxia. CT and MRI brain did not show acute intracranial processes and MRI spine only showed a small enhancing region in the conus medullaris consistent with a lipoma. Cerebrospinal fluid had mildly elevated oligoclonal banding and resulted positive for Anti-Tr antibodies. CT chest, abdomen, and pelvis showed several enlarged lymph nodes. The largest was biopsied by fine needle aspiration and core biopsies with pathology showing small lymphocytes and flow cytometry showing no monoclonal B cells consistent with reactive inflammation inconsistent with Hodgkin Lymphoma. Serial CTs showed decrease in lymph node size after ACA treatment. Clinical improvement of ataxia and speech were achieved with IVIG and short high-dose steroid treatment.
Conclusions:
This is the first case of anti-Tr autoimmune cerebellar ataxia reported associated with reactive inflammation and burn injury and without Hodgkin Lymphoma. No consensus treatment exists and outcomes are variable, with few examples of cases without Hodgkin Lymphoma. Here, clinical improvement was seen with treatment six weeks after initial presentation with minimal improvement prior to treatment, and continued therapy required for maintenance at more than 18 months following initial presentation.