Year: 2024
Type: article
Abstract: Objective Describe paraneoplastic neurologic syndromes (PNS) related to an underlying lymphoma. Background Although neurologic symptoms in patients with lymphomas are often secondary to direct tumor involvement or chemotherapy, PNS can occur in a minority and needs to be better characterized. Design/Methods We retrospectively identified lymphoma associated PNS patients evaluated at our institution (2005-2020). Results From our Mayo Clinic PNS cohort (n=212), we identified 10 patients with PNS associated with lymphoma (5%), of whom five (50%) were females. Median age of PNS onset was 48 years (range 25-77). Eight (80%) patients had a neuromuscular presentation with polyradiculoneuropathy (n=6, 60%) being the most common phenotype; two (20%) patients had a rapidly progressive cerebellar syndrome. Other presentations included recurrent plexopathy (n=1) and cranial neuropathy with dysautonomia (n=1). Malignancy associations included Hodgkin’s lymphoma in 5 (50%) patients, while others were non-Hodgkin lymphomas that included mantle cell (n=1), marginal zone (n=1), mucosa associated lymphoid tissue (MALT) (n=1), diffuse large B-cell (n=1) and lymphoplasmacytic lymphoma (n=1). Only three patients (30%) had a high-risk autoantibody associated with the PNS, and all were Purkinje cell cytoplasmic antibody type Tr (PCA-Tr). All patients received cancer treatment while immunotherapy was administered in 50% patients. Nine patients achieved hematologic remission, 6 of whom demonstrated improvement or stabilization of the PNS. One patient who had an initial neurological improvement deteriorated later and died of cancer recurrence. Both patients with rapidly progressive cerebellar syndrome showed no improvement. At last follow up, only 50% of patients had mRS≤3. Conclusions In our study polyradiculoneuropathy was the most common presenting PNS phenotype associated with lymphoma. High-risk paraneoplastic antibody (PCA-Tr IgG) association was limited to ataxia/cranial neuropathy phenotype. Most patients improved from hematological perspective (60%), however, even after hematologic remission, debilitating neurologic symptoms often persisted.
Source: Neurology
Language: English
Subfield: Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W4403206785