A Case Report of Plasmapheresis in Paraneoplastic Cerebellar Ataxia Associated With Anti‐Tr Antibody

Abstract

A 53-year-old-male patient was admitted to the Hiroshima University Hospital in August 2001, with a history of progressive cerebellar ataxia, notable by standing and gait disturbances. Brain computed tomography (CT) and magnetic resonance imaging (MRI) showed no signs of ischemic damage. Two weeks later, left axillary lymphadenopathy developed. The pathological finding was Hodgkin's disease (HD; a classical malignant lymphoma in an early stage). Serum antibodies against Purkinje's cells (anti-Tr antibody) were detected by immunohistochemical study. Thus, we diagnosed this as paraneoplastic cerebellar degeneration (PCD) associated HD. We immediately carried out chemotherapy (ABVD therapy), radiotherapy with 20-30 Gy, steroid pulse and intravenous immunoglobulin (IVIg) without neurologic improvement. The patient was subsequently treated by double filtration plasmapheresis (DFPP), leading to a marked improvement of standing and gait disturbances and to a reduction of autoantibodies, which became undetectable. We conclude that DFPP might be useful in treating patients with cerebellar ataxia, and should be considered as a therapeutic choice for it.

Paraneoplastic cerebellar degeneration associated with squamous cell carcinoma of the lung

“…1 Immunosuppressive treatments generally do not work well, despite the autoimmune nature of this condition. 7 Treatment of PCD with plasmapheresis, 8 intravenous immunoglobulin, 9 steroids and cyclophosphamide 10 has been reported, however few patients show significant or prolonged neurological improvement.…”

Hodgkin’s Lymphoma and Paraneoplastic Phenomena in the Central Nervous System: A Case Report and Review of the Literature

“…The differential diagnosis included PDC typically presenting with dizziness, nausea and vomiting, usually with acute onset and followed several days later by gait instability, oscillopsia, diplopia, ataxia, dysarthria and dysphagia [6, 7]. Possible causes of PDC are circulating anti-Tr and anti-mGluR1 antibodies destroying Purkinje cells in the cerebellum and leading to severe symptoms in particular ataxia [6, 7]. Our patient had several of these symptoms such as nausea, vomiting and vertigo, but no focal loss of neurological function was found on clinical examination.…”

“…anti-Tr or anti-mGluR1) against antigens in Purkinje cells, are well known paraneoplastic manifestations of lymphomas [6, 7]. Suggested therapies are steroids, intravenous immunoglobulin and plasmapheresis [6]. Clinical outcomes differ but are usually poor with permanent neurological sequel.…”

Treatment and management issues in ataxic diseases

“…The gluten-free diet may be therapeutic and has undergone study both in Europe and at the NIH with variable results (Hadjivassiliou et al, 2003b;Briani et al, 2005;Volta et al, 2006). Immunomodulating therapies that have been used for both paraneoplastic and non-paraneoplastic cerebellar syndromes include corticosteroids (Bataller et al, 2001;Birand et al, 2006), plasmapheresis (McCrystal et al, 1995;Yeh et al, 1999;Miyamoto et al, 2002;Meloni * Taniguchi et al, 2006), intravenous immunoglobulin (Daaboul et al, 1998;Abele et al, 1999;Go, 2003;Dalakas, 2005;Phuphanich and Brock, 2007), and standard chemotherapy agents that target B-cells (mycophenolate mofetil). Results with these modalities seem better for the non-paraneoplastic conditions, than for those associated with a neoplasm (which may respond to treatment of the underlying cancer).…”