Abstract
A 61-year-old woman with a history of untreated low-grade B-cell lymphoma presented with blurry vision, unsteadiness, and worsening pain on touching skin of the upper trunk was enrolled. Blurry vision was attributed to oscillopsia from downbeat nystagmus, which later evolved into macrosaccadic oscillations. MRI brain and spine showed mild, longitudinally extensive T2 hyperintensity in the central gray matter of the spinal cord extending from the medulla to T11 level. Serum paraneoplastic panel was negative; however, she had very high titers of anti-Ma2 antibodies in cerebrospinal fluid. The diagnosis of paraneoplastic neurological syndrome was made. Empiric treatment with high dose of intravenous steroids followed by intravenous immunoglobulin infusions did not improve her symptoms. An extensive search for an underlying tumor commenced and was initially unrevealing. However, two-month follow-up positron emission tomography scan showed increased uptake in a right pulmonary nodule, which when biopsied confirmed diagnosis of extranodal marginal zone lymphoma. The final diagnosis was anti-Ma2 antibody-mediated paraneoplastic cerebellar degeneration and myeloneuropathy secondary to lymphoma.
Overview
- The study focuses on a 61-year-old woman with a history of untreated low-grade B-cell lymphoma, presenting with blurry vision, unsteadiness, and skin pain, which was attributed to oscillopsia from downbeat nystagmus and later macrosaccadic oscillations.
- The patient's symptoms were investigated using MRI brain and spine, serum paraneoplastic panel, and cerebrospinal fluid analysis, which revealed anti-Ma2 antibodies.
- The primary objective of the study is to describe a case of anti-Ma2 antibody-mediated paraneoplastic cerebellar degeneration and myeloneuropathy secondary to lymphoma, and to identify the underlying tumor after an extensive search.
Comparative Analysis & Findings
- The study found that the patient's symptoms, including blurry vision, unsteadiness, and skin pain, were attributed to oscillpsia from downbeat nystagmus and later macrosaccadic oscillations, which were not improved by empiric treatment with high-dose steroids and intravenous immunoglobulin infusions.
- The patient's high titers of anti-Ma2 antibodies in cerebrospinal fluid supported the diagnosis of paraneoplastic neurological syndrome, and the MRI brain and spine showed mild, longitudinally extensive T2 hyperintensity in the central gray matter of the spinal cord.
- The diagnosis was confirmed by biopsy, which revealed an extranodal marginal zone lymphoma in a right pulmonary nodule, which was not detected initially.
Implications and Future Directions
- The study highlights the importance of thorough investigation and search for underlying tumors in patients presenting with paraneoplastic neurological symptoms, as delayed diagnosis can lead to poor outcomes.
- Future studies should focus on identifying reliable biomarkers for detecting anti-Ma2 antibodies and improving treatment outcomes for patients with paraneoplastic cerebellar degeneration and myeloneuropathy.
- Clinicians should consider the possibility of paraneoplastic neurological syndrome in patients with unexplained neurological symptoms, especially those with a history of lymphoma, and perform relevant tests to identify the underlying tumors.