Paraneoplastic cerebellar degeneration preceding the diagnosis of Hodgkin's lymphoma.
Paraneoplastic cerebellar degeneration (PCD) can present as a severe and (sub)acute cerebellar syndrome. PCD can accompany different kinds of neoplasms including small cell lung cancer, adenocarcinoma of the breast and ovary, and Hodgkin's lymphoma. A 34-year-old patient is described with acute dysarthria, gait ataxia and diplopia. Despite extensive laboratory and radiological evaluations in this patient with rapidly deteriorating cerebellar syndrome, the diagnosis of a paraneoplastic syndrome was only made after several months, when an anti-Tr antibody was detected in his serum. The search for Hodgkin's disease as concomitant disorder was then started and resulted in stage II B disease. The patient was successively treated with six courses of etoposide, bleomycin, vinblastine and dexamethasone and radiotherapy, which resulted in a complete remission of the Hodgkin's disease. After starting therapy the cerebellar degeneration stabilised. The pathogenesis of neuronal damage in central nervous system paraneoplastic disorders such as the one we describe is not completely understood. Antitumour therapy is assumed to be the important cornerstone in stabilising the neurological condition. Improvement of the cerebellar syndrome in anti-Tr autoantibody paraneoplastic disease is a rare achievement. Early recognition of the concomitant disorders (anti-Tr autoantibody disease and Hodgkin's lymphoma) is of crucial importance.
Ypma
P F
PF
Department of Haematology, HagaZiekenhuis, location Leyenburg, The Hague, the Netherlands. p.ypma@hagaziekenhuis.nl
Wijermans
P W
PW
Koppen
H
H
Sillevis Smitt
P A E
PA
D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001761:Bleomycin / Q000008:administration & dosage; D002531:Cerebellum / Q000000981:diagnostic imaging / Q000473:pathology*; D003907:Dexamethasone / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D006689:Hodgkin Disease / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology / Q000473:pathology / Q000532:radiotherapy; D006801:Humans; D008297:Male; D009367:Neoplasm Staging; D020362:Paraneoplastic Cerebellar Degeneration / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D049268:Positron-Emission Tomography; D018714:Radiotherapy, Adjuvant; D012074:Remission Induction; D014057:Tomography, X-Ray Computed; D014747:Vinblastine / Q000008:administration & dosage
D001761:Bleomycin; D014747:Vinblastine; D005047:Etoposide; D003907:Dexamethasone