Objective: To report a case of Bing-Neel Syndrome presenting with parkinsonism.

Background: A 68 year old man presented with an 18 month history of stooped posture and gait difficulties. He had a shuffling gait with general slowness and fatigue but no falls or handwriting difficulties. He had no significant past medical history. His mother had parkinsonism in her 50s and a brother died of Non-Hodgkin Lymphoma. He had been initially diagnosed with Parkinson’s disease and treated with rasagiline with no benefit.

Methods: Examination revealed a shuffling gait with inability to tandem walk with no freezing or cerebellar signs. He had mild hypomimia, a postural (but not rest) tremor of the upper limbs and bradykinesia on the left side.

Results: MRI Brain without contrast showed disproportionate ventricular increase in size in comparison to the cortex raising the possibility of Normal Pressure Hydrocephalus (NPH). A DATSCAN showed suggestion of reduced striatal uptake of the tracer symmetrically. Contrast-enhanced MRI, 3 months later showed diffuse leptomeningeal enhancement with involvement of multiple cranial nerves. Lumbar puncture with 10 metre walk test showed marked improvement which lasted for a few hours. Analysis of the cerebrospinal fluid (CSF), however, revealed an increased white cell count of 155/ml with predominance of lymphocytes (135/ml) and increased protein concentration (1.06 g/L). Molecular studies confirmed B cell clonality of the lymphocytes in keeping with central nervous system (CNS) involvement by lymphoma. There was IgM Kappa paraprotein of 10 g/L in the serum and a MYD88 positive lymphoproliferative population (10%), most in keeping with bone marrow lymphoplasmacytic lymphoma. Bing-Neel syndrome was diagnosed and treatment started with chemotherapy. After the first cycle of chemotherapy he developed hydrocephalus requiring an emergency VP shunt, after which he gradually improved. His improvement continued over the next 4 cycles of chemotherapy.

Conclusions: Bing-Neel syndrome is the infiltration of the CNS by Waldenstrom’s Macroglobulinaemia. It can be the first presentation in 36% of patients and can occur without systemic progression of the macroglobulinanemia (Simon et al, 2015). Although unusual, rest and postural tremor have been reported with this condition (Castillo et al, 2015). Bing-Neel syndrome should be considered in the differential diagnosis of NPH and atypical parkinsonism and CSF analysis is vital.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/bing-neel-syndrome-presenting-with-parkinsonian-features-a-case-report/