Table 1.
IgG-specificity and endpoint titres | |||||||||
---|---|---|---|---|---|---|---|---|---|
Patient age (y) and gender | Clinical features and CNS syndrome | Serum | CSF | CSF WBC (cells/uL) | CSF: serum glucose ratio | CSF protein (mg/dL) | Identified infection | Outcome | Meeting criteria for possible autoimmune encephalitis?19 |
A. 22M | Prodromal fever then acute confusion, neck stiffness, agitation, delirium, hypersomnolence/insomnia, catatonia; meningoencephalitis | NMDAR 1:200 |
NMDAR 1:200 |
15 | 0.50 | 55 | None | Discharged, not improved - diagnosis catatonic schizophrenia | YES |
B. 45F | Prodromal fever, headache, neck stiffness then acute confusion, drowsiness, agitation, delirium; admitted initially to psychiatry; meningoencephalitis | NMDAR 1:500 | NMDAR 1:2 | 50 | 0.81 | 28 | None | Discharged moribund | YES |
C. 35M | 7-d history of fever and headache followed by acute confusion, drowsiness; meningoencephalitis | NMDAR 1:500 | Neg. | 85 | 0.35 | 55 | Pus in ears, culture negative | Deceased | NO |
D. 53M | 10-d history of fever, headache, neck stiffness, cough, vomiting, confusion, right leg weakness; meningoencephalitis | NMDAR 1:400 | Neg. | 610 | CSF 2.0 mmol/L* | 276 | TB culture CSF positive | Deceased, diagnosis TB meningitis | NO |
E. 45M | Acute onset of fever, headache, neck stiffness, agitation, drowsiness, confusion, with single convulsion; meningoencephalitis | NMDAR 1:200 | Neg. | 10 | 0.43 | 38 | Serum murine typhus IgM+, Rickettsia typhi PCR negative | Discharged well | NO |
F. 73M | Acute onset of fever, neck stiffness, delirium, agitation and altered consciousness; admitted initially to psychiatry; meningoencephalitis | NMDAR 1:200 | Neg. | 5 | CSF 5.2 mmol/L* | 30 | None | Discharged moribund | NO |
G. 51M | 1-mo weight loss preceding acute onset fever, headache and neck stiffness; meningitis; suspected underlying malignancy | CASPR2 1:800 | Neg. | 0 | 1.09 | 72 | N. meningitidis PCR positive on CSF (culture negative) | Discharged AMA, no improvement | NO |
H. 57M | 7-d fever, headache, cough and rash without seizures or confusion | GABAAR 1:160 | GABAAR 1:8 | 18 | 0.34 | 20 | None | Unknown | NO |
Abbreviations: AMA, against medical advice; CASPR2, contactin-associated protein-like 2; CSF, cerebrospinal fluid; GABAAR, gamma-amino butyric acid A receptor; NMDAR, N-methyl-D-aspartate receptor; WBC, white blood cells.
The following cut-offs were used for positive serum neuronal autoantibody titres: NMDAR antibodies >1:100, CASPR2 antibodies >1:500, GABAAR antibodies >1:50. In CSF, any antigen-specific reactivity is considered positive.
*no concurrent serum glucose available for these patients.
See Dubot-Pérès et al. (2019)11 for diagnostic techniques for the pathogens given in the Identified infection column.
The criteria for possible autoimmune encephalitis used are = diagnosis can be made when all three of the following criteria have been met: (a) subacute onset (rapid progression of less than 3 mo) of working memory deficits (short-term memory loss), altered mental status or psychiatric symptoms; (b) at least one of the following: new focal CNS findings, seizures not explained by a previously known seizure disorder, CSF pleocytosis (white blood cell count of more than five cells per mm3), MRI features suggestive of encephalitis; and (c) reasonable exclusion of alternative causes.19