Meningitis as early manifestation of anti-NMDAR encephalitis

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a recently discovered immune mediated encephalitis. The syndrome typically occurs in children and young adult with initial presentations of psychiatric symptoms, seizures, followed by abnormal movement and dysautonomia. There is association with ovarian teratoma. We report here a 16-year-old girl with anti-NMDAR encephalitis, who present with meningitis as initial symptom, confirmed by pleocytosis in the cerebrospinal fluid and leptomeningeal enhancement in MRI. She subsequently manifested the more typical manifestations of oro-facial dyskinesia and choreoathetosis. The diagnosis was confirmed by the presence of antiNMDAR antibody. She was treated with immunotherapy with clinical improvement and drop in the level of autoantibody. However the patient died due to septisemia.

The use of AQP4-antibody testing in diagnosis Thai patients with neuromyelitis optica

Background: There are several methods to detect AQP4-antibody which is essential for diagnosis neuromyelitis optica (NMO). Objective: To evaluate an accuracy of the commercially available kit compared with other available tests. Methods: One hundred and twelve patients who visited the multiple sclerosis (MS) clinic at Siriraj Hospital were tested for AQP4-antibody by cell-based assay with Sendai method (Postfix-CBA), a commercial kit (Prefix-CBA) and an indirect immunofluorescence tissue-based assay (IIF-TBA). The patients were classified to NMO, seropositive NMOSD (AQP4-pos NMOSD), seronegative NMOSD (AQP4-neg NMOSD), classic MS (CMS), atypical MS and clinical isolated syndrome (CIS). Results: Based on postfix-CBA, there were 26 NMO, 25 AQP4-pos NMOSD, 19 AQP4-neg NMOSD, 34 CMS, 4 atypical MS and 14 CIS. There were 5 (1 NMO, 2 AQP4-neg NMOSD, 2 CMS), 7 (1 NMO, 6 AQP4-pos NMOSD) and 2 patients (1 AQP4-neg NMOSD, 1 CIS) were seropositive only by CBA-kit, CBA-Sendai and IIF-TBA respectively. Sixteen patients were seropositive by both CBA but negative by IIF-TBA. Both CBA showed strong correlation. Conclusions: CBA-kit is a relatively sensitive, comparable assay to detect anti-AQP4 antibody in Thai NMO patients. Since the kit may have a few false-negative and false-positive results, a more sensitive assay is necessary for a much more proper diagnosis in the future.

Isolated bulbar palsy with anti-GM3 and GT1b antibodies

Isolated acute bulbar palsy has been described as one of the more rare variants of Guillain-Barré syndrome. IgG anti-ganglioside antibodies are associated with axonal subtypes of Guillain-Barré syndrome as well as Fisher syndrome. However, IgG against GM3 and GT1b in relation to bulbar palsy is uncommon. In this case report, we describe a 64 year-old male patient presenting with isolated bulbar weakness and generalized hyporeflexia without limb weakness. Serological testing for antiganglioside antibodies was positive for IgG anti-GM3 and -GT1b, suggesting the association of these antibodies with isolated bulbar palsy.