480 - Macro Level Overview of Rehabilitation Relevant Impairments Caused by Waldenstrom Macroglobulinemia

Case Diagnosis: Bing-Neel Syndrome (BNS) in a Patient with Altered Mental Status and Worsening Ataxia Associated with Waldenstrom Macroglobulinemia (WM)

Case Description: A 68-year-old male with WM presented with sudden onset gait abnormalities, left hemiparesis, aphasia, altered mental status, and seizures. MRI revealed right temporal and left parietal lobe meningeal enhancement, indicating a recurrence of BNS. His treatment involved managing seizures, initiating steroids, chemotherapy, and acute inpatient rehabilitation. Unfortunately, cognitive changes prompted a transfer to inpatient oncology for chemotherapy management.

Discussions: WM is a rare lymphoproliferative disorder, occurring annually at three cases per million. This condition can lead to BNS, affecting 0.3-1% of WM cases. BNS arises from the infiltration of malignant lymphoplasmacytic cells within the central nervous system, resulting in a diverse clinical presentation that evolves over several weeks to months. Remarkably, no documented instances of BNS cases undergoing acute rehabilitation exist.

Brain biopsies are the gold standard for precise diagnosis, but cerebrospinal fluid analysis can be performed. The infiltration of macroglobulins into the meninges, cerebral parenchyma, and spinal cord is often detectable through MRI.

The overall survival rate after BNS diagnosis was 71% at 5 years and 59% at 10 years. The response rate after first-line treatment is 70%. Emerging treatments, such as ibrutinib, have extended the 5-year survival rate to 86%. The Eastern Cooperative Oncology Group (ECOG) Performance Status found a significant rate of progression-free survival (PFS).

Conclusions: BNS remains a rare complication of WM, a unique non-Hodgkin’s lymphoma. Considering the high survival rates with PFS, we should set goals beyond the current level of function with support and equipment to improve their long-term quality of life in the rehabilitation setting. Future research is essential to identify prognostic markers and optimize rehabilitation goals.