Aaron Greenblat, DO
Resident Physician
University of Minnesota
Lakeville, Minnesota, United States
Florence John, MD, MPH
Faculty Physician
University of Minnesota
Eden Prairie, Minnesota, United States
Waldenstrom Macroglobulinemia
Case Description: A 56 year old male developed fasciculations, paresthesias and generalized weakness. He was diagnosed with Waldenstrom Macroglobulinemia (WM) six months later, and his neuromuscular symptoms worsened during treatment. Work-up included immunofixation panels, rheumatologic studies, electrodiagnostic studies and magnetic resonance imaging (MRI) of his spine. He presented to clinic with severe functional decline and dependence for mobility and activities of daily living. Examination revealed significant weakness in proximal and distal musculature, diminished reflexes, tongue fasciculations and no upper motor neuron signs. Electromyography (EMG) demonstrated thoracic paraspinal fibrillations in the setting of sensorimotor involvement on nerve conduction studies. His symptoms have been attributed to an immune-mediated mixed demyelinating and axonal sensorimotor polyradiculoneuropathy, however there remains a strong suspicion for motor neuron disease. Rehabilitation approach has included physical therapy, occupational therapy, orthotics and evaluation for home equipment needs.
Discussions: Waldenstrom Macroglobulinemia is a lymphoproliferative malignancy affecting lymphocytes and plasma cells that secrete monoclonal proteins. Lymphoproliferative disorders can cause paraprotein associated polyneuropathies. Studies also show that up to five percent of these patients develop a motor neuron disease such as amyotrophic lateral sclerosis (ALS). Suspicion of ALS without the ability to satisfy diagnostic criteria makes definitive diagnosis difficult. Functional loss is accelerated, and impairments are affected by the complexity of their malignancy and treatment. Early rehabilitation is pivotal to maintaining patient independence as long as possible.
Conclusions: Sensorimotor polyneuropathy caused by Waldenstrom Macroglobulinemia can result in functional deficits. However, it is rare to see debilitating progressive weakness that is suggestive of motor neuron disease. This can make rehabilitation efforts challenging, as without a formal diagnosis of motor neuron disease these patients do not have access to multidisciplinary clinics and resources that are crucial to maintain quality of life. Rehabilitation interventions at the time of notable decline are critical to ensure favorable functional outcomes.