History and clinical findings: A 30-year-old previously healthy man su
ddenly developed double vision, unsteady gait and some difficulty in s
peech articulation. Within 4 weeks he had become markedly ataxic, unab
le to walk, stand or sit down unaided. Neurological examination indica
ted a severe cerebellar syndrome. There were no other abnormal finding
s on physical examination. Investigations: There was no pleocytosis an
d no oligoclonal bands in cerebrospinal fluid (CSF). A test for anti-P
urkinje cell antibodies was negative in both CSF and serum. Computed t
omography and nuclear magnetic imaging (NMI) of the brain were normal.
Treatment and course: As a para-or postinfectious or paraneoplastic p
rocess was suspected. i.v. immunoglobulin and oral corticosteroids wer
e administered, but without improvement. 13 month later, a mediastinal
mass was noted on a chest radiogram. This led to the diagnosis of a s
tage IA Hodgkin's disease. Retrospectively the cerebellar degeneration
was most likely a paraneoplastic change related to the Hodgkin's dise
ase. However, an independent second disease cannot be excluded. While
the treatment of Hodgkin's disease was successful, the neurological sy
mptoms remained unchanged. Severe cerebellar atrophy was demonstrated
on NMI. Conclusion: In case of cerebellar atrophy of undetermined aeti
ology a paraneoplastic cause should be considered and an underlying ma
lignant disease looked for.