NMDAR, this study (n=13) | NMDAR antibody encephalitis (n=32)10 | VGKC-complex, this study (n=7) | VGKC-complex encephalopathy in children (n=20)* | |
---|---|---|---|---|
Age at onset (years) | 9.6 (range 1.83–17) | 14 (range 1.9–18) | 8.9 (range 6–15) | 8 (range 0.8–17) |
Cognitive dysfunction | 13 (100%) | 28/32(88%) Behavioural and personality change | 4 (57%) | 14 (70%) |
Psychiatric problems | 10 (77%) | N/A | 3 (43%) | 9 (45%) |
Seizures | 10 (77%) | 23/30(77%) | 7 (100% | 12 (60%) |
Movement disorders | 7 (54%) | 26/31 (84%) | 0 (0%) | 7 (35%) |
Fever at presentation | 4 (30%) | 48% prodrome noted | 4 (57%) | 8 (50%) |
Neoplasm | 1(8%) | 8 (25%) | 0 (0%) | 1 (1%) |
EEG abnormalities | 12 (92%) | 25/25 (100%) | 7 (100%) | 9/9 (100%) |
MRI abnormalities (early) | 2 (15%) | 10/31(32%) | 4 (57%) | 9 (45%) |
MRI abnormalities (late) | 4 (31%) | N/A | 1 (14%) | N/A |
CSF abnormalities | 6 (46%) | 29/31(94%) | 2 (29%) | 5 (25%) |
Lymphocytosis | 3 (WCC 11–73) | 27/31 (WCC 5–200) | 2 (WCC 8–20) | 4 (WCC 6–25) |
Oligoclonal bands | 3 (23%) | 5/6 (83%) | 0 (0%) | N/A |
PCR positive | 2/13 (15%) | 0(0%) | 0 (0%) | N/A |
Immunotherapy received | 11 (77%) | 30/31 (97%) | 4 (57%) | 8 (50%) |
Immunotherapy response | 10/11 (90%) | 22/30 (73%) | 4/4 (100%) | 9/12 (75%) |
Ongoing problems | 9/13 (69%) | 20/31 (65%) | 5 (71%) | 12 (60%) |
Patients with NMDAR encephalitis were more likely to have cognitive dysfunction and movement disorders. Seizures were seen more commonly in VGKC-complex encephalitis; these patients were less likely to receive immunotherapy. When comparing our cohort's clinical and paraclinical features to those in the reported literature, CSF abnormalities were seen less frequently as were the associated malignancies (not statistically significant).
*20 paediatric patients with encephalopathy associated with VGKC-complex antibodies were reported in seven manuscripts.11–17
CSF, cerebrospinal fluid; NMDAR, N-methyl-D-aspartate receptor; WCC, white cell count.