There has been a "Reynolds phenomenon", is now suddenly numbing the lower limbs, urination difficulties, this is the final decision?

36-year-old male, sudden numbness in the lower limbs, urination difficulties, ask edging the medical history 6 months ago had the phenomenon of Reynolds, rash, there is no potential connection between them?casethe patient was a 36-year-old male who had sudden numbness in his lower lower limbs two weeks earlier and had difficulty urinatingthe medical history:6 months ago suffering from "large leaf pneumonia." After the pneumonia is cured, the patient develops weakness in the lower extremities, the renocosity of both hands, and the whole body is scattered in the rash, no pain or itchingSince then, the patient's fatigue gradually increases, the rash increases, and the finger swelling and finger ulcersDuring the course of the disease, the patient's temperature fluctuated between 37 and 38 degrees C, accompanied by hair loss, poor ness, difficulty swallowing, the recent weight loss of 10 kgThere is no special personal history or family historyadmitted to the hospital:body temperature 37.9 degrees C, double upper limb muscle strength 4/5 level, left lower limb muscle strength 3/5 level, right lower limb muscle strength 0/5 levelBabinski and Chaddock are positiveNervous system examination found that the T10 level below the pain temperature perception and the right lower limb body sensation disappearedHands are swollen, multiple fingertip ulcers, and the cheeks, back and limbs on both sides are visible with a patchy brown rash accompanied by pigmentation (Figure 1)Pressure sores can be seen at the tail of the shin and the nodules of the sciaticaThe conjunctiva and the bed paleFigure 1 can be seen in the swelling of the hands, fingertip ulcers, double lower limb patchy brown rash and pigmentation 3 days after admission, the patient's condition gradually deteriorated, muscle strength dropped to 0/5 level, the left lower limb body sensation disappeared, and the appearance of stool incontinence assisted examination: chest MRI and visually induced potential were not abnormal laboratory indicators are as follows: , folic acid, vitamin B12 and tumor markers are normal; cerebrospinal fluid test: protein 1.33g/L, glucose 1.83mmol/L, white blood cell count 2 x 106/L, IgG 745.00 mg/L given the patient's renoetic symptoms, finger swelling and rash symptoms, the level of IgG in cerebrospinal fluid is elevated and autoimmune diseases should be highly suspected recommended that patients further improve the relevant examination: electromyogram suggests local myogenesis injury, multiple mononeuroitis head MRI suggests multiple cavity cerebral infarction rash biopsy suggests connective tissue disease (Figure 2) Figure 2 the immunology indicators of the of the rash were as follows: answer questions correct answer: mixed connective tissue disease (MCTD) combined with the above information, the patient was diagnosed with MCTD, transverse spinal itisis, multiple mononeuroitis, multiple cavity cerebral infarction Patients received small doses of methicillin (40mg/d) intravenous injection and hydroxychloroquine (400mg/d) treatment, after which the above-mentioned serological indicators improved significantly, symptoms improved, and glucocorticoids gradually decreased After 1 year follow-up, the patient recovered well, lower limb muscle strength 4/5 level, the sequelae for urinary incontinence mcTD diagnostic criteria are as follows: should be noted that some patients tend to BE diagnosed with MCTD when they develop, and further develop clinical manifestations more in line with systemic lupus erythematosus or rheumatoid arthritis." In long-term follow-up, more than 50% of patients still meet MCTD diagnostic criteria : McTD has a variety of neurological lesions that are extremely rare, leading to challenging diagnosis, and patient history and clinical manifestations (Reynolds, finger swelling, and rashes) provide important clues to diagnosis combined with this case, together to understand the relevant knowledge of MCTD - MCTD was originally reported by Sharp in 1972, is a serum with high titration of spot-type antinuclear anti-nuclear antibodies and anti-u1RNP antibodies, clinically have the phenomenon of Reno, double swelling, multi-joint pain or arthritis, limb palaty sclerosis, myositis, esophageal motor dysfunction, pulmonary arterial hypertensive syndrome patients can exhibit clinical symptoms of a variety of connective tissue diseases (systemic lupus erythematosus, systemic scleroderma, dermatitis/polymyosis, rheumatoid arthritis) Many clinical manifestations of MCTD do not occur at the same time, overlapping characteristics can appear one after another, and different patient performance is not the same Common clinical manifestations of early MCTD associated with anti-u1RNP antibodies are double-hand swelling, arthritis, renophenomenonation, inflammatory myopathy, and end-of-finger sclerosis central nervous system lesions are not the most significant clinical features of MCTD, and the mechanism of their occurrence is not clear Several studies have found that vasculitis and thrombosis may be a potential cause of neurological lesions of MCTD The most common form of MCTD neuro-affected lesions is trigeminalism, which also includes epilepsy-like seizures, metaphysical psychosyndrome, multiple peripheral neuropathy, cerebral embolism and cerebral hemorrhage transverse myelitis is an extremely rare and serious complication of MCTD, mainly affecting the chest spinal cord, MRI is an important test However, about 30% of patients with systemic lupus with transverse myelitis have normal MRI performance in addition, cerebrospinal fluid testing can also provide a basis for MCTD-related central nervous system lesions Transverse elitis needs to be treated in a timely manner, MCTD combined with transverse myelitis there is no standard treatment plan, systemic lupus combined with transverse myelitis treatment plan: intravenous methotrexate (1g/d) combined with cyclophosphamide (1g/m2) for 3 days, followed by oral glucocorticoid prosaccharide sequence therapy In addition, previous studies have shown that hydroxychloroquine can effectively reduce the risk of recurrence of systemic lupus combined with transverse myelitis given the patient's current situation, this case did not use glucocorticoid shock therapy, but chose small doses of glucocorticoids and hydroxychloroquine, in which the patient responded well to small doses of glucocorticoids The prognosis of MCTD combined transmyelitis is not clear, and the prognosis is good in this case, but long-term follow-up treatment and observation are still required author: Jindi Wang Yuwei Source: