Cerebellar Degeneration as a Rare Paraneoplastic Syndrome in a Child With Hodgkin Lymphoma

Abstract

We report a rare case of cerebellar degeneration as a paraneoplastic syndrome in an 8-year-old boy with Hodgkin lymphoma that presented during first-line treatment. Antibodies against Purkinje cells (anti-Tr antibodies) were detected in the serum of the patient. After successful treatment of the lymphoma, the cerebellar symptoms resolved partially. Childhood presentation of paraneoplastic cerebellar degeneration is extremely rare, with only a few reports in the literature. For this reason, the description of all such cases contributes to the enrichment of the medical knowledge and will improve the diagnosis and the treatment of this complication.

Cerebellar cortical degeneration associated with feline leukemia virus infection and cerebellar lymphoma in a young cat

“…Intrauterine or perinatal infection of feline panleukopenia virus reportedly causes cerebellar hypoplasia, with the presence of a decreased granular cell layer (Shamir et al, 1999). In humans, CCD is a rare paraneoplastic neurological syndrome associated with Hodgkin lymphoma as well as other tumor types (Avramova et al, 2016;Gungor et al, 2017). Polyclonal B-cell activation in human patients with Hodgkin lymphoma may be responsible for an autoimmune reaction in the cerebellum (Avramova et al, 2016).…”

“…Non-Hodgkin lymphomas are classified into B-cell and T-cell type, with 30 types of lymphoid cell neoplasms reported (Cheson et al, 2014). In humans, CCD is a rare paraneoplastic syndrome associated with Hodgkin lymphoma, small-cell lung cancer, breast cancer, and gynecological malignancies (Avramova et al, 2016;Yan et al, 2018). Paraneoplastic neurologic syndrome caused by FeLV-infected cats is associated with lymphoma and lymphocytic infiltration in the brain and spinal cord (Caemichael et al, 2002).…”

Paraneoplastic Syndromes in Children with Hodgkin Lymphoma

“…He had a complete resolution of symptoms with standard HL treatment. 14 Immunotherapy has not been shown to be particularly effective in the treatment of the neurologic outcomes associated with PCD. 14,17 Anti-N-methyl-D-aspartate receptor encephalitis Anti-NMDA receptor encephalitis is a recently described phenomenon that may occur as a PNS.…”

“…14 Immunotherapy has not been shown to be particularly effective in the treatment of the neurologic outcomes associated with PCD. 14,17 Anti-N-methyl-D-aspartate receptor encephalitis Anti-NMDA receptor encephalitis is a recently described phenomenon that may occur as a PNS. Affected children exhibit neuropsychiatric symptoms such as behavioral and personality disturbances, sleep dysfunction, dystonias, autonomic instability, dyskinesias, and speech dysfunction or regression.…”

“…[12][13][14] Although opsoclonusmyoclonus syndrome is usually associated with neuroblastoma, the other three have been described in pediatric HL and may be associated with onconeural antibodies-anti-metabotropic glutamate receptor 5 (anti-mGluR5) in LE, the anti-Purkinje cell cytoplasmic antibody-Tr (anti-Tr) in cerebellar Review Lymphoma degeneration, and anti-NMDA antibody in anti-NMDA encephalitis. 6,12 Although the pathophysiology remains unclear, it has been speculated that the expression of these onconeural proteins by the lymphoma triggers an immune response that is misdirected to the patient's nervous system, resulting in the associated deficits.…”