The median duration of follow-up was 42?weeks (24C65?weeks). features and results were retrospectively analyzed. Results Fourteen of 31 individuals (45.16?%) were seropositive for NMO-IgG. The 71.43?% of anti-AQP4 (+) Abdominal muscles individuals converted to NMO, while only 11.76?% of anti-AQP4 (-) Abdominal muscles individuals progressed to NMO. Anti-AQP4 (+) Abdominal muscles individuals demonstrated a higher IgG index (0.68??0.43 vs 0.42??0.13, test. Survival was estimated according to the Kaplan-Meier method. The primary study endpoint was the time to NMO conversion, as indicated from the Wingerchuks criteria [18]. The log-rank test was used to compare the survival analysis between anti-AQP4 (+) and anti-AQP4 (-) Abdominal muscles individuals. All valueacute brainstem syndrome, neuromyelitis optica, neuromyelitis optica spectrum disorder, multiple Sclerosis, optic neuritis, cerebrospinal fluid, Kurtzke Expanded Disability Status Level, a, EDSS scores at the 1st assault; b, EDSS scores of the last check out of follow-up BML-277 Brainstem symptoms The brainstem symptoms of 31 Abdominal muscles individuals were outlined in Table?2. Five anti-AQP4 (+) individuals (35.71?%) experienced recurrent brainstem symptoms before the attacks of ON or myelitis, while only one anti-AQP4 (-) individuals (5.88?%, valueacute brainstem syndrome MRI examinations Mind BML-277 and spinal cord MRI examinations were conducted for those individuals. Within the sagittal section of mind MRI, the lesions were more frequently occurred in the medulla of anti-AQP4 (+) individuals than those in anti-AQP4 (-) individuals (78.75?% vs 35.29?%, valueacute brainstem syndrome Open in a separate windowpane Fig. 3 Axial T2-weighted FLAIR MRI shows periependymal lesions are involved in the dorsal midbrain (a, b and f, g; value /th th rowspan=”1″ colspan=”1″ em n /em ?=?14 /th th rowspan=”1″ colspan=”1″ em n /em ?=?17 /th th rowspan=”1″ colspan=”1″ em n /em ?=?31 /th /thead Mind MRIsLesions in additional mind regions?Juxtacortical0/14 (0)2/17 (11.76?%)2/31 (6.45?%)0.488?Subcortical1/14 (7.14?%)5/17 (29.41?%)6/31 (19.35?%)0.185?Infratentorial14/14 (100?%)17/17 (100?%)31/31 (100?%)Spinal cord MRI?Segments5 (3C7)2 (0C4)3.5 (0C8)0.001?Cervical10/14 (71.43?%)13/17 (76.47?%)23/31 (74.19?%)0.750?Thoracic7/14 (50?%)5/17 (29.41?%)12/31 (38.71?%)0.242?Cervical and thoracic3/14 (21.43?%)1/17 (5.88?%)4/31 (12.90?%)0.199?LETM9/14 (64.29?%)3/17 (17.65?%)12/31 (38.71?%)0.012Meet Barkhof criteria0/14 (0?%)5/17 (29.41?%)5/31 (16.13?%)0.185 Open in a separate window CSF examinations CSF specimens were from all patients in the acute stage (Table?1). The protein concentrations in CSF of anti-AQP4 (+) individuals (0.30??0.10?mg/dl) were related with those of anti-AQP4 (-) individuals (0.25??0.12?mg/dl). Anti-AQP4 (+) individuals had a slightly higher quantity of white blood cells (WBCs) in CSF (11.29??4.27) than anti-AQP4 (-) patients (8.47??2.07). However, the values of IgG index in anti-AQP4 (+) patients were significantly higher than those of anti-AQP4 (-) patients (0.68??0.07 vs 0.42??0.13, em p /em ? ?0.01). Only one anti-AQP4 (+) ABS patient and one anti-AQP4 (-) ABS patient were positive for OCBs in CSF. Prognosis in ABS patients All the patients were followed up at least 2?years after the first episode of ABS. The median duration of follow-up was 42?months (24C65?months). 7/31 (22.58?%) of patients did not show a second episode during the follow-up period, 12/31(38.70?%) of patients developed to NMO, 17/31(54.84?%) of patients converted to NMOSD, and 7/31(22.58?%) of patients converted to MS. In the follow-up duration, the mean annual relapse rates of anti-AQP4 (+) patients were significantly higher than those of anti-AQP4 (-) patients (1.05??0.40 vs 0.72??0.40, em p /em ?=?0.031) (Table?1). Although the EDSS scores at the first attack were comparable between two groups, anti-AQP4 (+) patients presented a significantly higher EDSS scores than anti-AQP4 (-) patients at the last visit of follow-up [5(3C7) VS 2.5(1.5C4.5), em p /em ? ?0.01]. Kaplan-Meier survival analysis showed that the risk of developing to NMO in anti-AQP4 (+) patients were significantly higher than that in anti-AQP4 (-) patients (log rank 5.23, em p /em ?=?0.012) (Fig.?4). Open in a separate windows Fig. 4 A survival analysis comparison of the risk of developing to NMO between anti-AQP4 (+) ( em n /em ?=?14) and anti-AQP4 (-) ( em n /em ?=?17) patients with ABS ( em p /em ?=?0.012) Discussion NMO frequently begins with an acute or subacute episode of ON or myelitis. However, brainstem symptoms in NMO were not rare [8, 10C12, 17]. According to the previous studies, brainstem symptoms have recently been described in NMO as well as in NMOSD [10, 12, 13, 17, 21]. In 17.05C30.61?% NMO patients, brainstem symptoms even act as the sole manifestation [14, 17]. Brainstem symptoms with anti-AQP4 antibodies has also been recommended in the latest diagnostic criteria for NMOSD [1]. Various brainstem symptoms such as area postrema clinical brainstem symptoms (including intractable hiccups, nausea and vomiting), diplopia, and bulbar dysfunctions attributed to lesions in the dorsal region of the medullar and the pons surrounding the fourth ventricle could be the first manifestation Mouse monoclonal to IHOG of symptoms [9, 10, 12, 14]. Our results demonstrated that area postrema clinical brainstem symptoms in anti-AQP4 (+) ABS patients were more frequent than those in anti-AQP4 (-) ABS patients. Intractable hiccups, nausea, and vomiting have BML-277 been reported as unique symptoms in NMO due to the involvement of the pericanal region in the medulla oblongata, which included the areas postrema, nucleus tractus solitaries where the putative hiccup and vomiting centres.