OBJECTIVE: Autoimmune longitudinal extensive transverse myelitis (LETM) is often combined with connective tissue disorders (CTD). The purpose of this study was to compare the clinical characteristics of autoimmune LETM with and without CTD.
METHODS: Ninety-two patients diagnosed with autoimmune LETM were enrolled from our clinical database and divided into two groups depending on whether they had a concomitant diagnosis of CTD. Differences in clinical, serological, and imaging characteristics between the two groups were evaluated and compared.
RESULTS: Fifty-nine LETM patients without CTD and 33 LETM patients with CTD were included. LETM patients with CTD had higher Kurtzke Expanded Disability Status Scale at nadir and more severe sensory dysfunction (p < 0.05) than those without CTD. It was also found that LETM patients with CTD, compared with those without CTD, had elevated levels of immune inflammation markers such as IgG, IgA, and globulins (p < 0.05). These abovementioned characteristics were more prominent in patients with aquaporin-4 antibodies (AQP4-ab) than in those without them. In addition, the most common type of CTD in LETM was Sjögren syndrome (SS), which was usually diagnosed at the time of LETM or later.
CONCLUSIONS: LETM patients with CTD, especially those with AQP4-ab, had greater sensory dysfunction and higher levels of inflammatory markers than did LETM patients without CTD. Multicenter cooperation and long-term follow-up are necessary to further study the inherent implications and prognosis of the disease.

Background: Osteogenesis imperfecta (OI), also known as brittle bone disease, is an inherited disease characterized by increased bone brittleness and decreased bone mass. OI patients may also be associated with exoskeleton manifestations such as hearing loss, articular ligament laxity, and heart valve lesions. Reports of internal carotid and cerebral artery dissection related to OI patients are very rare. We present the first case of acute cerebral infarction caused by the progressive stripping of the carotid artery dissection Chinese patient with Osteogenesis imperfecta.Case: A 48-year-old Chinese male who had no prior medical history or bad habits was to admitted the hospital due to leftside temporal headache, paroxysmal right limb weakness, and prolonged blurred vision experienced for a week. After a series of tests were performed to exclude superficial temporal arteritis, the patient was diagnosed with transient ischemic attack and was given aspirin, clopidogrel and atorvastatin without further headache and blurred vision. However, he was again admitted to the emergency department the following day due to right limb weakness for 7 h that was considered acute cerebral infarction caused by left carotid artery dissection.Conclusion: The findings in this case support that internal carotid and cerebral artery dissection may be one of the complications of Osteogenesis imperfecta.

BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) often coexist with connective tissue disorders (CTD). The aim of this study was to investigate and compare the features of NMOSD with and without CTD.
METHODS: NMOSD patients with (n = 18) and without CTD (n = 39) were enrolled, and the clinical, laboratory, and magnetic resonance imaging (MRI) features of the two groups were assessed.
RESULTS: Most of the demographic and clinical features examined were similar between NMOSD patients with and without CTD. Serum immunoglobulin G (IgG), percentage of γ-globulin and seropositivity for several other autoantibodies were significantly elevated in NMOSD patients with CTD (P < 0.05). NMOSD with CTD was marked by longer spinal cord lesions and a lower frequency of short transverse myelitis (TM) than NMOSD without CTD (P < 0.05). NMOSD with CTD also featured more T1 hypointensity and T2 bright spotty lesions (BSLs) on MRI than NMOSD without CTD (P = 0.001 and 0.011, respectively). There were no other differences in laboratory, MRI and clinical characteristics between different NMOSD subtypes.
CONCLUSIONS: A few characteristics differed between NMOSD with and without CTD. NMOSD patients with CTD had higher serum IgG, longer spinal cord lesions, a lower frequency of short TM and more T1 hypointensity and T2 BSLs on spinal MRI than NMOSD patients without CTD.

The detection of anti-aquaporin-4 autoantibody (AQP-4 Ab) is crucial to detect patients who will develop neuromyelitis optica (NMO); however, there are few studies on the AQP-4 Ab serostatus of patients with neuromyelitis optica spectrum ON. We analyzed the clinical and paraclinical features of neuromyelitis optica spectrum ON patients in China according to the patients\' AQP4-Ab serostatus. 125 patients with recurrent and bilateral ON with simultaneous attacks were divided into AQP-4 Ab-seropositive and -seronegative groups. Demographic, clinical, serum autoantibody data, connective tissue disorders (CTDs), visual performance were compared. A Visual Acuity (VA) of less than 0.1 during acute ON attacks occurred more frequently in the seropositive group (p = 0.023); however, there was not a significant difference between groups on VA recovery after the first attack. The seropositive group experienced the worst outcome during the last attack (p = 0.017). Other co-existing autoimmunity antibodies (p < 0.001) and CTDs (p < 0.001) were more prevalent in seropositive patients. There were no significant differences on VA recovery and RNFLT combined with other autoantibodies or CTDs. The two groups did not differ significantly with regard to time to relapse, annualized relapse rates, time of diagnosis NMO, or RNFL. There were no significant differences on VA recovery and RNFLT combined with other autoantibodies or CTDs. RNFLT was thinner in NMO seropositive patients. Although AQP-4 Ab expression predicted poor visual outcome, positive patients were usually associated with mild symptoms at first onset. Anti-SSA/SSB antibody or Sjögren syndrome may be associated with AQP-4 Ab in neuromyelitis optica spectrum ON.