Paraneoplastic Cerebellar Degeneration in Pediatric Hodgkin Disease

Abstract

Paraneoplastic cerebellar degeneration (PCD) is a rare neurological complication in adults with extracerebral neoplasms. It is characterized by a diffuse cerebellar dysfunction, usually leading to severe neurological sequelae. In childhood, this complication is extremely rare. We report on PCD as primary manifestation of Hodgkin disease (HD) in a thirteen-year old boy. On magnetic resonance imaging, irreversible atrophy of the cerebellum developed within three months. Antibodies against Purkinje cells were detectable at diagnosis and normalised after successful treatment of the lymphoma. Cerebellar symptoms, however, only partially resolved. The necessity of a search for a malignant tumour is emphasised in the presence of an otherwise unexplained, subacutely developing, diffuse cerebellar dysfunction.

Rituximab as potential therapy for paraneoplastic cerebellar degeneration in pediatric Hodgkin disease

“…None reported the use of rituximab or other immunomodulatory therapy for treatment of PCD 7–10. Of these four pediatric patients, two reported complete remission of lymphoma but only partial remission of cerebellar symptoms following treatment 9, 10. Hahn et al 9 reported that while their patient had reduced symptoms following treatment, he never regained the ability to walk.…”

“…Topcu et al 8 reported that only one patient had chronic cerebellar ataxia prior to HD diagnosis in the follow up of 735 children diagnosed with HD in their center. Hahn et al 9 reported that in six consecutive German pediatric HD treatment studies involving approximately 1,200 pediatric patients, only one case of PCD was reported. Our literature search revealed only four reported cases of PCD in children diagnosed with HD 7–10.…”

“…Hahn et al 9 reported that in six consecutive German pediatric HD treatment studies involving approximately 1,200 pediatric patients, only one case of PCD was reported. Our literature search revealed only four reported cases of PCD in children diagnosed with HD 7–10.…”

“…The pediatric patients mentioned above all received multiple cycles of chemotherapy and radiation therapy for HD. None reported the use of rituximab or other immunomodulatory therapy for treatment of PCD 7–10. Of these four pediatric patients, two reported complete remission of lymphoma but only partial remission of cerebellar symptoms following treatment 9, 10.…”

Acute Ataxia in Children: Approach to Clinical Presentation and Role of Additional Investigations

“…Only a few single observations of paraneoplastic cerebellar ataxia associated with Hodgkin disease in children have been published. 56,57 Cerebellar signs including ataxia, dysarthria, and nystagmus have an acute onset and are often associated with neuroimaging evidence of cerebellar atrophy, but initial MRI may be normal.…”