There have been "Reynolds phenomenon", is sudden lower limb numbness, urination difficulties, this is the dragonfly?

Don't want to miss Jiemei's push? Poke the blue word "medical rheumatism and nephropathy channel" to pay attention to us, and click the "··" menu in the upper right corner, and select "set as star sign" to finalize the wind list, waiting for you to decide! A 36 year old male had a sudden numbness of lower limbs and dysuria. He asked his medical history and learned that he had Raynaud's phenomenon and rash six months ago. Is there any potential connection between them? Let's challenge the final decision of today's Fengbang ~ the case is a 36 year old male who suffered from bilateral lower limb numbness and dysuria 2 weeks ago.▎ follow up medical history: he had lobar pneumonia 6 months ago.after the pneumonia was cured, the patient developed weakness of lower limbs, Raynaud's phenomenon of hands, and scattered rash all over the body, without pain or pruritus.after that, the patient's fatigue gradually increased, the rash increased, and the finger swelling and finger tip ulcer appeared.during the course of the disease, the body temperature fluctuated between 37 ℃ and 38 ℃, accompanied by alopecia, anorexia, dysphagia, and weight loss of 10kg.personal history and family history are not special.▎ admission physical examination: body temperature 37.9 ℃, muscle strength of both upper limbs 4 / 5, left lower limb muscle strength 3 / 5, right lower limb muscle strength 0 / 5.Babinski sign and Chaddock sign were positive.physical examination of nervous system showed that pain and temperature sensation below T10 level and proprioception of right lower limb disappeared.swelling of hands, multiple ulcer of fingertips, patchy brown rash with pigmentation on both sides of cheek, back and limbs (Fig. 1).pressure sores can be seen at sacrococcygeal and ischial tubercles.the conjunctiva and nail bed are pale.Figure 1 physical examination showed swelling of hands, ulcer of fingertips, patchy brown rash and pigmentation on both lower limbs. After 3 days of admission, the patient's condition deteriorated gradually, muscle strength decreased to 0 / 5 grade, proprioception of left lower limb disappeared, and fecal incontinence occurred.▎ auxiliary examination: no abnormality was found in chest MRI and VEP.▎ laboratory indicators were as follows: in addition, folate, vitamin B12 and tumor markers were normal; anti AQP4 antibody and NMO IgG antibody were normal; hepatitis virus, cytomegalovirus, EBV, HIV, tuberculosis, syphilis and other related indicators were negative.cerebrospinal fluid detection: protein 1.33 g / L, glucose 1.83 mmol / L, white blood cell count 2 × 106 / L, IgG 745.00 mg / L.considering the presence of Raynaud's phenomenon, finger swelling and rash, and the increase of IgG level in cerebrospinal fluid, autoimmune diseases should be highly suspected.it is suggested that patients should further improve the relevant examination: EMG indicates local myogenic injury and multiple mononeuritis.multiple lacunar infarctions were found on cranial MRI.biopsy of the rash revealed connective tissue disease (Figure 2).Fig. 2 pathological biopsy of skin rash ▎ immunological indexes are as follows: correct answer: mixed connective tissue disease (MCTD).combined with the above information, the patient was diagnosed as MCTD, transverse myelitis, multiple mononeuritis, multiple lacunar infarction.the patients received low-dose methylprednisolone (40mg / D) intravenous injection and hydroxychloroquine (400mg / D) treatment. After treatment, the above serological indicators were significantly improved, symptoms improved, and glucocorticoid gradually decreased.after 1 year follow-up, the patient recovered well, the muscle strength of lower limbs was 4 / 5 grade, and the sequelae was urinary incontinence.▎ the diagnostic criteria of MCTD are as follows: it should be noted that some patients tend to be diagnosed with MCTD at the onset of disease, and the further clinical manifestations are more consistent with systemic lupus erythematosus or rheumatoid arthritis.in the long-term follow-up, more than 50% of patients still met the diagnostic criteria of MCTD.conclusion: it is extremely rare for MCTD to have multiple nervous system diseases, which makes the diagnosis challenging. The past history and clinical manifestations (Raynaud phenomenon, finger swelling and rash) provide important clues for the diagnosis.combined with this case, let's learn about MCTD. ~ MCTD was first reported by sharp in 1972. It is a clinical syndrome characterized by Raynaud's phenomenon, swelling of hands, polyarthritis or arthritis, limb end sclerosis, myositis, esophageal motor dysfunction, pulmonary hypertension, etc.patients can show clinical symptoms of various connective tissue diseases (systemic lupus erythematosus, systemic scleroderma, dermatomyositis / polymyositis, rheumatoid arthritis).multiple clinical manifestations of MCTD do not appear at the same time, overlapping features can appear successively, and different patients have different manifestations.in the early stage of MCTD, the common clinical manifestations associated with anti U1RNP antibody were swelling of hands, arthritis, Raynaud's phenomenon, inflammatory myopathy and finger tip sclerosis.central nervous system disease is not the most significant clinical feature of MCTD, and its mechanism is still unclear.a number of studies have found that vasculitis and thrombosis may be the potential causes of neurological lesions in MCTD. trigeminal neuropathy is the most common neuropathy in MCTD, including epileptic seizures, organic psychiatric syndrome, multiple peripheral neuropathy, cerebral embolism and cerebral hemorrhage. transverse myelitis is a rare and serious MCTD complication, mainly involving the thoracic spinal cord. MRI is an important examination method. however, about 30% of patients with systemic lupus erythematosus complicated with transverse myelitis have normal MRI findings. in addition, cerebrospinal fluid examination can also provide evidence for central nervous system lesions related to MCTD. transverse myelitis needs to be treated in time. There is no standard treatment for MCTD complicated with transverse myelitis. The treatment plan for SLE complicated with transverse myelitis is: intravenous methylprednisolone (1g / D) combined with cyclophosphamide (1g / m2) for 3 days, followed by oral glucocorticoid sequential treatment. in addition, previous studies have shown that hydroxychloroquine can effectively reduce the recurrence risk of systemic lupus erythematosus complicated with transverse myelitis. considering the current situation of the patient, this case did not use glucocorticoid pulse therapy, but chose low-dose glucocorticoid and hydroxychloroquine. In this case, the patient responded well to low-dose glucocorticoid. the prognosis of MCTD combined with transverse myelitis is not clear. This patient has a good prognosis, but it still needs long-term follow-up treatment and observation. References: [1]. Yulei, Hao, Liangshu, et al. Management of multiple neurologic complications in mixed connective tissue disease: a case report. [J]. Medicine, 2018. [2]. Guidelines for diagnosis and treatment of mixed connective tissue disease [J]. Chinese Journal of Rheumatology, 2011, 015 (001): 42-45. [3]. Ciang, Natalia C. o, Pereira, n í dia, Isenberg, David A. Mixed connective tissue disease—enigma variations?[J]. Rheumatology, 2016:kew265.