Clinical and laboratory findings in a cohort of patients with Bickerstaff brainstem encephalitis or Fisher syndrome
| Bickerstaff brainstem encephalitis | Fisher syndrome | |
|---|---|---|
| Number of patients | 53 | 466 |
| Age (years): median (range) | 40 (0–78) | 44 (2–86) |
| Sex (male/female) | 37/16 | 281/185 |
| Antecedent illness: n (%) | ||
| Upper respiratory infectious symptoms | 32 (60%) | 354 (76%) |
| Diarrhoea | 15 (29%) | 117 (25%) |
| Neurological signs: n (%) | ||
| Consciousness disturbance | 53 (100%) | 0 (0%) |
| Blepharoptosis | 18 (34%) | 172 (37%) |
| External ophthalmoplegia | 53 (100%) | 466 (100%) |
| Internal ophthalmoplegia | 29 (55%) | 163 (35%) |
| Facial weakness | 22 (42%) | 103 (22%) |
| Bulbar palsy | 18 (34%) | 79 (17%) |
| Mild limb weakness | 26 (49%) | 117 (25%) |
| Deep tendon reflexes | ||
| Absent or decreased | 16 (60%) | 466 (100%) |
| Normal or brisk | 10 (40%) | 0 (0%) |
| Extensor plantars | 20 (38%) | 9 (2%) |
| Ataxia | 53 (100%) | 466 (100%) |
| Sensory disturbance | 22 (42%) | 242 (52%) |
| Assisted ventilation: n (%) | 18 (34%) | 5 (1%) |
| Serum IgG anti-GQ1b antibodies | 36 (68%) | 387 (83%) |
| CSF in the first week | ||
| Number of patients | 44 | 375 |
| Albuminocytological dissociation: n (%) | 11 (25%) | 139 (37%) |
| Cell count (cells/μl): median (range) | 4 (0–668) | 1 (0–105) |
| Pleocytosis: n (%) | 14 (32%) | 15 (4%) |
| MRI | ||
| Number of patients | 47 | 353 |
| Abnormal findings: n (%) | 5 (11%) | 4 (1%) |
| EEG | ||
| Number of patients | 30 | 32 |
| Abnormal findings: n (%) | 17 (57%) | 8 (25%) |
Modified from reference15 with permission.
CSF, cerebrospinal fluid; IgG, immunoglobulin G.