1 case of pregnancy combined neurospinalitis

Optic neurospinalitis (neuromyelitis optica, NMO) is an immunomediated central nervous system inflammatory demyelinator disease, mainly manifested in the visual nerve and spinal cord fatigueBecause of the disease is characterized by the necrosis of neuronal cells, the clinical manifestations are more serious and the prognosis is often poorPregnancy-combined neurospinalitis is rare in clinical practiceThe First People's Hospital of Hangzhou University, affiliated with Zhejiang University Medical College, recently admitted a related case, and the case is reported as follows in the context of literature review1 Medical Record Summarypatient 23 years old, due to "pregnancy 31 to 3 weeks, right limb numbness 3 d, neck pain 2d" on May 9, 2018 emergency treatment in Zhejiang University Medical College affiliated Hangzhou First People's HospitalThe patient's last menstruation is October 1, 2017, and the early pregnancy is checked for pregnancyThere are no special cases of regular birth tests at local hospitals during pregnancyPatients with 3 d before admission experienced a noticeable numbness at the end of the right finger after sleep, followed by gradual lysing on the right limb2 d before admission to the hospital after neck pain discomfort, neck movement is limited, no dizziness headache, no limb paraplegia, no nausea and vomiting, no size incontinence and so onPast history: pregnancy 2 birth 0, the past abortion 1 timeDeny the history of other diseasesadmitted to the hospital for physical examination: body temperature 37 degrees C, pulse 86 times/min, breathing 20 times/min, blood pressure 134/79mmHg, height 160cm, weight 57kg, skin no yellowing, no spots, neck resistance, normal muscle tension, heart and lung unheard and abnormal, normal limb muscle, lower llecan body body sensation, right-hand positive, left-handObstetric examination: uterine height 29cm, abdominal circumference 90cm, uterine contraction, fetal heart rate 140 times / minadmitted to the hospital after the emergency cervical vertebral MRI show: neck 2 to 6 horizontal vertebral spinal cord thickening with abnormal signal (see Figure 1), skull MRI shows: two sides of the frontal lobe cortex, the side of the ventricle and the two sides of the semi-egg circle center area multi-abnormal signal lesions, combined with neck MRI, consider the possibility of neurospinalitisAfter admission to the a strong dragon static drop and C globulin impact treatment 4 d, the patient's right limb numbness is not relieved, neck pain intensified, and the left limb numbness After admission, the relevant examination return shows: anti-nuclear antibody (ANA) 1:320, anti-SS-A antibody: positive, anti-SS-B antibody: negative, anti-Ro-52 antibody: positive, anti-mitochondrial antibody (AMA-2): weak positive Lumbar puncture examination shows: cerebrospinal fluid routine and biochemical examination is normal, acid staining, bacteria, fungi and other cultures are all (-) Serum and cerebrospinal fluid water channel protein (AQP4)-IgG were positive There is no special examination of the bottom of the eye and THE MRI examination of the optic nerve After multidisciplinary consultation, pregnancy combined with neurospinalitis diagnosis is basically clear Taking into account the progression of the disease, after completing 1 course of treatment to promote the maturation of the fetal lungs, the 5th day of pregnancy 32 weeks of pregnancy in the lower stage of the whole hemp lower uterine section of the caesarean section, gave birth to a baby girl, weight 1750g, Apgar score: 1min 7 points, 5min 6 points, 10min 7 points, newborn to NICU, maternal transfer ICU further treatment After the mother into the ICU, to 5 plasma replacement, limb numbness and other symptoms gradually alleviated after the transition to rheumatism immunology department, continue to the meta-strong dragon, the esterofophenol tablets, sulphate chlorpyrifos tablets and other treatment, the condition was stable on June 8 after discharge Newborn into the NICU after check: ANA 1:100, anti-SS-A antibody positive, anti-Ro-52 antibody-positive, residual negative No water channel protein 4 antibody (AQP4-IgG) check, no neurological symptoms during hospitalization, 13 d after hospitalization to return to normal, discharged 2 Discussion NMO was proposed by Devic in 1894, also known as Devic disease The incidence of NMO is mainly related to AQP4-IgG The positive rate of AQP4-IgG in NMO patients was 68% to 91% AQP4 is a water channel protein of the central nervous system, which is mainly expressed in the foot protrusions of astrocytes When AQP4-IgG binds to AQP4 on astral glial cell pneutrusions, it eventually leads to neuronal cell necrosis by activating the complement currently internationally using the diagnostic criteria developed by the 2015 NMO Diagnostic Team The previous standard refers to lesions limited to the optic nerve and spinal cord The new diagnostic criteria also incorporate a number of non-visual nerve and spinal cord lesions, commonly known as psydiatrisis spectrum disease (NMO spectrum disorders, NMOSD) In 2016, China also formulated guidelines for the diagnosis and treatment of psylococtic spinal cord inflammation in China in accordance with this standard Layered according to serological AQP4-IgG results: (1) Serological AQP4-IgG positive: with at least 1 clinical core characteristics: optic neuritis, myelitis, final syndrome (seizure, nausea, vomiting), exclusion of other causes, and other brain stem syndrome (2) Serology AQP4-IgG negative or unknown state: at least two core clinical characteristics, at least 1 core characteristic synod, acute myelitis or final syndrome, and require strict MRI conditions: the long t2 signal or T1 enhanced signal, 1/2 visual nerve length; for early or clinical characteristics, MRI performance of atypical cases, need to carry out relevant differential diagnosis, including: other inflammatory demyelinating diseases, other autoimmune diseases: such as systemic lupus erythematosus, dry syndrome and other neurological disorders, infectious and metabolic diseases: such as tuberculosis, ischemic hypoxia encephalopathy, Wernicke NMOSD is better in women, female patients are about 8 to 9 times more than male patients, the median incidence of the disease is between 35 and 37 years old, many NMOSD patients are still in the reproductive age The study found that NMOSD patients had a significantly higher risk of spontaneous abortion and pre-eclampsia during pregnancy AQP4 in addition to expression in the central nervous system, in humans and mice's placental conjoined nourishing cells also have expression Infusion of AQP4-IgG and tonics in the abdominal cavity of mice can lead to inflammatory immersion and necrosis of placental tissue, which can lead to embryo abortion Nour et al reviewed 54 pregnancies in 40 patients with Serological AQP4-IgG-positive NMOSD and found that the pre-eclampsia incidence rate was 11.5%, especially in patients with combined with other multi-system autoimmune diseases or a recent history of natural abortion The rate of natural abortion in pregnancy after NMOSD also increased significantly (43 percent vs 7 percent) compared with before the onset of the disease, especially when NMOSD disease is still active and has a significantly higher risk of miscarriage Studies such as Delgado-Garc?a found that nearly half (42%) of pre-nMOSD pregnancies had at least one pregnancy loss, which may have been associated with subclinical levels of AQP4-IgG in the patient's body prior to the onset of clinical symptoms in NMOSD more than a number of scholars have found that 1 to 3 months after birth is the peak period of NMOSD recurrence , suggesting that childbirth may be a trigger for NMOSD recurrence Kim et al found that the rate of NMOSD recurrence increased significantly after 1 to 3 months and 4 to 6 months after childbirth (5.3 times and 3.7 times before pregnancy, respectively), and the incidence of postpartum recurrence was as high as 77% Klawiter et al also noted the highest recurrence rate in the three months after birth, noting in particular an increased risk of postpartum recurrence in AQP4-IgG-positive NMOSD Huang and others conducted a multi-center study of four hospitals in southern China, and found that the recurrence rate of NMOSD in the six months after birth was significantly higher than before pregnancy The above studies suggest that it is very important for us to continue postnatal treatment in NMOSD maternal AQP4-IgG can reach the fetus through the placental barrier, the level of AQP4-IgG in the serum increased in the newborn 1 to 3 months after birth, but the newborn did not show NMOSD-related symptoms, may be related to the integrity of the blood-brain barrier limiting the serum AQP4-IgG into the central nervous system Chang and others reported a case of NMOSD pregnant women in 24 weeks ultrasound found fetal defect finger (toe) malformation induced delivery, may be the only case of NMOSD and fetal malformation reported In this case, the fetus has obvious finger defects (toe) deformities in both hands and right foot The pregnant woman has no bad pregnancy history, no radioactive, toxic and other contact history during pregnancy, the pregnancy stopped taking thiopental, only take a small dose of metastasis, fetal induced post-natal chromosomal examination did not see abnormal, placental disease detection prompt moderate inflammation changes It is known that AQP4-IgG can reach fetal blood circulation through the placenta, whether it will affect fetal growth and development, resulting in fewer cases of fetal malformation, but still worthy of our attention NMOSD often coexists with some autoimmune diseases such as systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), and dry syndrome (pSS) (pSS) About half of NMOSD patients simultaneously combined other autoimmune markers such as antinuclear antibodies, anti-SS-A antibodies, anti-SS-B antibodies, anti-double-stranded DNA antibodies positive In this case, the patient was admitted to the hospital with positive serum antinuclear antibodies and anti-SSA antibodies Because anti-SS-A and/or anti-SS-B antibodies can reach the fetus through the placenta, causing an increased risk of congenital heart conduction blockage in the fetus or newborn, and even causing fetal cardiac arrest, it is also necessary to strengthen the detection of fetal heart ultrasound and electrocardiogram The acute stage of nMOSD's clinical treatment mainly includes the treatment of glucocorticoids, plasma replacement, immunoglobulin, and the treatment of immunosuppressants and monoclonal antibodies The choice and safety of NMOSD during pregnancy has also led to a number of studies acute illness in patients in this case, combined with clinical symptoms, neck MRI, head MRI and AQP4-IgG testing, the diagnosis was quickly and clearly clarified After the treatment of glucocorticoids and a large number of cropin shock treatment, the symptoms of the patient did not ease and further aggravate, combined with the consideration of pregnancy week, complete dying fetal lung maturation, decisive caesarean section to terminate the pregnancy After the operation, plasma replacement treatment was given, and the patient's condition was gradually stabilized Patients after the regular rheumatism immunology department follow-up, six months after the birth of the disease did not recur However, the recurrence of the disease in the 7th month after the birth of the patient manifests itself as optic neuritis, vision loss in both eyes, and sexual aggravation, after a number of rheumatism immunology department hospitalization, after the treatment of litoxil monoantigen, the current vision gradually restored to stability Newborns are regularly re-diagnosed after discharge from the hospital, normal growth and development, normal vision checks, no symptoms of nervous system impairment , patients with nMOSD history, especially those with a history of Serology AQP4-IgG positive, to avoid pregnancy when the activity of the disease, serum AQP4-IgG level is high, the pregnancy needs rheumatology immunology, neurology and obstetricians to jointly manage, after the postpartof still need to be in the rheumatology immunology department for standardized treatment, which is to prevent the recurrence of the disease, re-birth when the prevention of natural abortion, sub-pregnancy and other pre-complications are very important For the clinical once pregnant women appear neurological symptoms, such as drowsiness, limb numbness, vomiting, incontinence, vision loss, etc , in addition to considering more common pregnancy combined SLE, pSS caused by the nervous system, but also pay attention to AQP4-IgG detection, early diagnosis, early treatment Obstetricians need to improve the identification of pregnancy-combined medical conditions, promote multidisciplinary cooperation, and strengthen the management of high-risk maternal care, thereby ensuring maternal and child safety references