Clinical characteristics and investigation findings of the five cases
| Case | Age at onset | Time to treatment (months) | Duration of follow-up (months) | Symptoms at onset | Symptoms at maximum disability | Systemic features and autoantibodies | Time from onset to brain biopsy (months) | CSF results | Brain MRI features |
| 1 | 65 | 6 | 12 | Gait ataxia | Gait ataxia, dysarthria, R upper limb ataxia, Holmes tremor, diffuse cognitive deficits, pseudobulbar affect | L parotid uptake on gallium scan. Chronic lymphocytic sialadenitis on biopsy. ANA 1/40 speckled ENA +ve (SS-A) | 5 | Protein 0.25 g/l, cells-3 monos/mm3, OCB +ve | Contrast enhancing lesions bilateral brachium pontis, pons, cerebellar white matter. |
| 2 | 51 | 4 | 36 | Gait ataxia | Gait ataxia, dysarthria, R hemiparesis, R appendicular cerebellar ataxia | Nil | 1 | Protein 0.52 g/l, cells-7 monos/mm3, OCB −ve | Contrast enhancing lesions pons, bilateral brachium pontis, cerebellar white matter, cerebral white matter, basal ganglia. |
| 3 | 20 | 2 | 64 | Gait ataxia, dysarthria | Gait and limb ataxia, spastic paraparesis, diplopia, nystagmus, cognitive deficits | Hypercalcaemia with high 1,25-dihydroxy vitamin D (low serum ACE level) | 1 | Protein 1.04 g/l, cells-0, OCB −ve | Contrast enhancing lesions pons, bilateral brachium pontis, cerebellar white matter, midbrain, medulla, cervical spinal cord. |
| 4 | 54 | 4 | 72 | R facial nerve palsy | Gait and limb ataxia, dysarthria, nystagmus, L abducens palsy, L tinnitus and reduced hearing, dysphagia, pseudobulbar affect | R parotid uptake on gallium scan. Lymphocytic conjunctival infiltrate without granulomas. ANA 1/160 (homogenous) | 4 | Protein 0.55 g/l, cells-0, OCB −ve | Contrast enhancing lesions pons, bilateral brachium pontis, cerebellar white matter, thalami. cerebral white matter. |
| 5 | 27 | 9 | 100 | Headaches, fatigue | Ataxia, dysarthria, diplopia, R hemiparesis, pseudobulbar affect, frontal release reflexes | Nil | 50 | Protein 0.65 g/l, cells-19 monos/mm3, OCB −ve | Contrast enhancing lesions pons, bilateral brachium pontis, medulla, midbrain. Small T2 hyperintensities cerebral white matter. |
ACE, angiotensin converting enzyme; ANA, antinuclear antibodies; ENA, extractable nuclear antigen; L, left; monos, monocytes; OCB, oligoclonal bands; R, right; SS-A, Sjögren's Syndrome-A.