TABLE 2.
Previously published GRAF1–IgG positive cases
| Peripheral Neuropathy | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Patient No/Sex/Age/Reference | Cbl Ataxia | Small Fiber | Large Fiber | Other Symptoms/Signs | Evidence of Cancer | MRI Brain | CSF | GRAF1–IgG Serum (s) or CSF IFA Titer | Immunotherapy | Follow‐Up (months) | Outcome |
| 1/F/33/ 1 | + | NA | NA | Diplopia, depression, hyperekplexia | NA | Cerebellar atrophy | Pleocytosis, OCBs positive | S: 1:6000 CSF: 1:200 | IVMP, IVIG, RTX | 16 months | Cerebellar signs and hyperekplexia, stable |
| 2/F/68/ 2 | + | NA | NA | Dizziness, nausea and vomiting | Ovarian cancer | Empty sella, cerebellar atrophy | NA | S:1:32000 | RTX, IVIG, cyclophosphamide | 24 months | Progressive decline |
| 3/M/38/ 2 | + | NA | NA | Weight loss, nausea and vomiting | NA | Cerebellar atrophy | 5 cells, OCBs positive | S:1:3200 | NA | NA | NA |
| 4/M/24/ 3 | + | NA | NA | Oscillopsia, flattened affect, cognitive impairments, weight loss, headache, memory disturbances | NA | Cerebellar atrophy | OCBs positive | S:1:20000 CSF: 1:240 | IVIG, PLEX | 4 years | Severe gait ataxia and cognitive symptoms. Using a walking stroller at 3 years |
| 5/F/34/ 4 | − | NA | NA | Recurrent psychotic symptoms, suicidal thoughts, headache | NA | Normal | Normal | S: 1:1000 CSF: positive | IVIG | NA | NA |
| 6/F/57/ 6 | + | NA | NA | Urticaria, dizziness, abnormal eye movements | History of breast cancer, melanoma | Normal | Normal | S: 1:32 | IVMP | 12 months | Mild ataxia and dizziness, stable |
| 7/F/37/ 6 | + | NA | NA | Hyperekplexia, myoclonic jerks, depression, falls, dysphagia, deficits in verbal fluency | NA | Normal | OCBs positive | S: 1:100 | IVMP | 19 years | Wheelchair bound at 19 years |
| 8/M/84/ 5 | + | NA | NA | Emotional instability, cognitive impairment, hyperekplexia, myoclonic jerks, loss of appetite, weightloss, hyponatraemia | B cell lymphoma | Generalized atrophy | OCBs positive | S:1:1000 | IVMP, PLEX | 3 months | Initial mild improvement in short term memory, agitation, and depression at last follow‐up |
| 9/M/73/ 5 | + | NA | NA | Poor memory | Prostate cancer | NA | NA | S:1:10000 | No | NA | Death from prostate cancer |
| 10/M/77/ 5 | NA | NA | NA | Cognitive impairment | Gastric adenocarcinoma | NA | NA | S: 1:100 | No | NA | NA |
GRA, GTPase Regulator Associated with Focal Adhesion Kinase 1; IgG, immunoglobulin‐G; Cbl, Cerebellar; MRI, magnetic resonance imaging; CSF, cerebrospinal fluid; IFA, immunofluorescence assay; M, male; F, female; NA, not available; OCB, oligoclonal bands; S, serum; IVMP, intravenous methylprednisolone; IVIG, intravenous immunoglobulin; RTX, rituximab; PLEX, plasma exchange.