Clinicopathological characteristics of patients with Bing-Neel syndrome
| Demographics . | N = 46 . |
|---|---|
| Age, median (range), y | 66.5 (48-85) |
| Male | 26 (52.5%) |
| WM disease characteristics | |
| Extramedullary disease | 14/46 (30.4%) |
| Bone marrow infiltration, median (range) | 20% (0-80) |
| Bone marrow MYD88L265 | 27/28 (96.4%) |
| Bone marrow CXCR4WHIM | 1/6 (16.7%) |
| Symptoms | |
| Sensory and/or motor deficits | 21 (45.7%) |
| Cognitive change or confusion | 8 (17.4%) |
| Cranial nerve palsy | 5 (10.9%) |
| Headaches | 4 (8.7%) |
| Seizures | 3 (6.5%) |
| Hearing loss | 3 (6.5%) |
| Ocular/orbital | 2 (4.3%) |
| CSF findings | |
| CSF leucocyte count median (range), /mm3 | 15.5 (1-153) |
| CSF protein, median (range), g/L | 1.40 (0.25-4.69) |
| CSF IgM, median (range), g/L | 4.13 (0.147-475) |
| CSF MYD88L265P detected | 29/30 (96.7%) |
| Imaging findings | |
| Parenchymal lesions | 12 (26.1%) |
| Leptomeningeal enhancement | 33 (71.7%) |
| Intracranial involvement | 24 (52.2%) |
| Spinal/cauda equina involvement | 27 (58.7%) |
| No MRI findings but positive CSF studies | 7 (15.2%) |
| Demographics . | N = 46 . |
|---|---|
| Age, median (range), y | 66.5 (48-85) |
| Male | 26 (52.5%) |
| WM disease characteristics | |
| Extramedullary disease | 14/46 (30.4%) |
| Bone marrow infiltration, median (range) | 20% (0-80) |
| Bone marrow MYD88L265 | 27/28 (96.4%) |
| Bone marrow CXCR4WHIM | 1/6 (16.7%) |
| Symptoms | |
| Sensory and/or motor deficits | 21 (45.7%) |
| Cognitive change or confusion | 8 (17.4%) |
| Cranial nerve palsy | 5 (10.9%) |
| Headaches | 4 (8.7%) |
| Seizures | 3 (6.5%) |
| Hearing loss | 3 (6.5%) |
| Ocular/orbital | 2 (4.3%) |
| CSF findings | |
| CSF leucocyte count median (range), /mm3 | 15.5 (1-153) |
| CSF protein, median (range), g/L | 1.40 (0.25-4.69) |
| CSF IgM, median (range), g/L | 4.13 (0.147-475) |
| CSF MYD88L265P detected | 29/30 (96.7%) |
| Imaging findings | |
| Parenchymal lesions | 12 (26.1%) |
| Leptomeningeal enhancement | 33 (71.7%) |
| Intracranial involvement | 24 (52.2%) |
| Spinal/cauda equina involvement | 27 (58.7%) |
| No MRI findings but positive CSF studies | 7 (15.2%) |
MRI, magnetic resonance imaging.