Weakness and numbness of extremities with bowel and bladder dysfunction for four years

Abstract

The patient was a 53-year-old female who was admitted for “numbness and weakness of lower extremities, urinary and defecation dysfunction for 4 years” in September 2016.  Four years ago, the patient felt numbness and weakness of lower extremities, with chest zonesthesia and back pain. The weakness of left lower extremity got severer next morning and she cannot feel her left foot on the pedal when riding bicycle. At night, she presented numbness under umbilicus level, feet scuff and urine retention. She was also unable to squat. No fever, headache, vomiting or visual defect was found. She was treated as stroke which showed no effect. Two days later, the numbness extended to thoracic level with no sweating below, dyspnea, deep voice, bucking, dysphagia and weakness of both upper and lower extremities. Spinal MRI showed a long T₁ signal and long T₂ signal extended cervical cord lesion from C₄-T₅ segments and spinal cord swelling. She was suspected to have multiple sclerosis (MS) or neuromyelitis optica (NMO), thus prednisone was given that resulted in the improvement other than numbness of lower extremities, stagger and bladder dysfunction. In the following 4 years, alike symptoms relapsed every year. Though the glucocorticoid treatment was helpful for symptoms remission, sequelae including extremities numbness, weakness, stagger, urinary incontinence and intermittent constipation were left. The predisposing and relieving factors were unclear for relapse. Six months ago, the patient stopped the glucocorticoid treatment by herself, followed by the increase of attack frequencies. One month ago, she got diplopia in the right eye. The patient was admitted for further diagnosis and treatment. Since the onset of symptoms, the patient has been conscious. The appetite and defecation have been normal. No dryness of mouth and eye, shin rash, Reynolds Syndrome, photosensitization and joint pain was noticed. She has a weight loss of 5kg, during which time the weight increased apparently once because of the intake of glucocorticoid.

Past history, personal history and family history: Her left eye lost sight because of cataracts secondary to repeated episodes of uveitis 30 years ago. She received thyroid hormone therapy for 6 years because of hypothyroidism and withdrew one year ago without the guidance of a doctor. Three years ago she was diagnosed with cataracts in the right eye and treated with pupilplasty+phaco+intraocular lens (IOL) implantation surgery. She also had severe vertebral compression fractures 2 years ago.

Physical examination: T 36.7℃, R 19/min, BP 90/51mmHg (1mmHg=0.133kPa), SaO₂ 99%; The patient was thin and had a thoracic kyphosis malformation. No edema, ulcer or rash was found on her skin. She was awake, alert, and able to respond to commands. The left eye was blind and showed enophthalmos. Visual partially loss was detected in the right eye with normal visual field. Eye movements were normal without nystagmus. Muscle capacity decreased partially. The muscle strength was 4  of right upper limb, 5 of left upper limb, 3 of right lower limb and 4 of left lower limb. Muscle tone of right upper limb was high. The heel-to-knee tests of right side and Romberg test were not stable. She walked with spastic gait. The tendon reflexes of both upper and lower limbs were active. The Babinski and Chaddock signs of both sides were positive. Hypoesthesia was present in the right cheek. The pinprick, vibration and touch sensation lost partially below T₄ level while only vibration sensation lost below T₁₂ level.

Investigations: The blood, urine and stool routine tests, biochemical tests, coagulation tests, HIV, hepatitis B and hepatitis C tests were all normal. The erythrocyte sedimentation rate (ESR) was 38mm/h (0-20mm/h); IgG 24.16g/L (7-17g/L), C₃ 0.62g/L (0.73-1.46g/L), C₄ 0.08g/L (0.1-0.4g/L), rheumatoid factor (RF) 158.20IU/ml (0-20IU/ml). The immunofixation electrophoresis was negative. Antinuclear antibody (ANA) 1:640 (＜1：40), antineutrophilic cytoplasmic antibody (ANCA), antiphospholipid antibody were negative. DNA-immunofluorescence (IF) 1:10 (＜1：5), DNS-enzyme-linked immunosorbent assay (ELISA) 296IU/ml (＜100IU/ml); Tumor biomarkers screening showed no positive finding. Serum iron 8.243umol/L (8.59-30.43 umol/L), transferrin 18.06umol/L (22.72-40.90 umol/L), total iron binding capacity 41.35umol/L (44.75-80.55 umol/L), transferrin saturation 16.70% (25-50%). The cerebrospinal fluid (CSF) was clear. The routine, biochemistry and lactic acid of CSF were within normal range. Cytological examination was negative. AQP-4 antibody (also named NMO-IgG) in CSF was 1:100. No abnormal finding showed in the chest CT and cranial MRI. Thoracic spinal cord MRI showed a longitudinally extensive high intensity lesion on spinal cord of C₇-T₇ segments in sagittal T₂WI and compression fractures of T_7-10, especially T_7-8. Labial salivary gland biopsy showed lymphocytes infiltration.

Diagnosis and treatment: After admission, she was treated with vitamin B (vitamin B1 10mg 3 times/d and mecobalamine 0.5mg 3 times/d), ginkgo capsule 400mg 3 times/d and baclofen 5mg 3 times/d orally. She was diagnosed as neuromyelitis optica spectrum disorder (NMOSD), connective tissue disease (CTD), systemic lupus erythematosus (SLE) , secondary Sjögren’s Syndrome (SS). Thyroxine replacement therapy (levothyroxine sodium 50μg/d) and calcium supplements (calcium carbonate 500mg/d, calcitriol 0.3μg 2 times/d, alendronate sodium 70mg/week) and orthosis were taken according to the endocrinologists and orthopedists. She was treated with azathioprine (50mg/d) and methyprednisolone pulse therapy (500mg/d, intravenous drip) followed by oral methylprednisolone (50mg/d). The dose of methylprednisolone decreases by 5mg/d every two weeks. Two months later, the patients showed improvement in walking while no obvious change was noticed of other symptoms and signs. 

DOI: 10.3969/j.issn.1672-6731.2017.03.014