A 31-year-old woman in Hebei Province of China initially noticed headache with fever, nausea, diplopia, low back and legs pain in December 1990. Lumbar puncture (LP) revealed increased opening pressure (600 mmH2O), pleocytosis (20 cells/ml), elevated protein (1.1 g/L), and reduced glucose (1.83 mmol/L). The results of ink stain, acid fast stain and Mycobacterium tuberculosis culture of the cerebrospinal fluid (CSF) were negative. She was diagnosed with possible tuberculous meningitis (TBM) and started on empirical anti-tuberculous treatment and adjunctive corticosteroids therapy. The symptoms and CSF findings subsequently improved. The anti-tuberculous treatment lasted for more than one year. In 2004 and 2010 year, she was admitted with the same symptoms. LP revealed increased opening pressure with lymphocytic pleocytosis, elevated protein level, and reduced glucose. She was diagnosed with possible TBM and treated empirically for TBM again. Her symptoms and CSF findings improved significantly after treatment.
The forth attack occurred in September 2016. The patient again developed fever, headache and severe, intolerable back and legs pain. In this attack, the patient developed symptoms of generalized tonic-clonic seizure and lose of conscious. The neurological evaluations indicated neck stiffness, decreased lower limbs muscle strength (MRC 4 degree), weakened bilateral patellar reflex, right plantar and saddle numbness, postive right Babinski sign and Kernig sign. LP results showed that opening pressure 300 mmH2O, WBC 2 cells/ml, protein 0.3 g/L, glucose 9.6 mmol/L and CSF IgG index 2.41 (reference value: 0.26–0.62). The fasting blood glucose was 5.6 mmol/L, glycosylated hemoglobin was 5.8% on the same day. The results of ink stain, acid fast stain and Mycobacterium tuberculosis culture of CSF were still negative. CSF cytology for malignant cells was negative. The whole blood Interferon-Gamma Release Assays (IGRAs) was negative. The results of TB-antibody, Mycoplasma antibody, Chlamydia antibody and Legionella antibody of serum were all negative. Erythrocyte sedimentation rate (ESR) was 40mm/h (reference value: 0–20 mm/h) and C-reactive protein (CRP) was 17 mg/L (reference value: 0–8 mg/L). Antineutrophil cytoplasmic antibodies (ANCA) were negative, Antinuclear antibody (ANA) was 1:320, RNP/Sm antibodies were positive. Ophthalmic examination indicated papilledema, retina hemorrhage and exudation. Brain magnetic resonance imaging (MRI) showed white matter lesion without ventriculomegaly (Fig. 1A). Contrast-enhanced brain MRI showed linear pachymeningeal enhancement (Fig. 1B). Cervical spine MRI showed cervical spondylosis and spinal cord compression. Thoracic spinal meningeal reinforcement was found in contrast-enhanced MRI. Lumbar spine MRI showed a cystlike structure in the lumbosacral sac (Fig. 1C,1D) and the clumping of the nerve roots of the cauda equina (Fig. 1C). Contrast-enhanced lumbar spine MRI showed marked enhancement of the cauda equina and spinal meninges (Fig. 1E). The CT scan of chest, abdomen, and pelvis showed no abnormality. She was treated successively with cefatriaxone, fluconazol, anti-tuberculosis drugs and adjunctive steroids. Repeated LP results showed persistent increased intracranial pressure (ICP). CSF analysis results varied widely, WBC varied from 0–65 cells/ml, total protein varied from 0.06–1.4 g/L and glucose varied from 1.3–14.4 mmol/L. After 1.5 months, the clinical symptoms were relieved and CSF findings were improved. However, ICP remained higher than 300 mmH2O. In the next examination, filling defect in superior sagittal sinus was found in magnetic resonance venography (MRV) of the head (Fig. 1f), In November 2016, after consultation in a higher level hospital, she was diagnosed as local hypertrophic pachymeningitis and was treated with high-dose pulse corticosteroid therapy. After treatment, ICP dropped to 150 mmH2O.
Unfortunately, in July 2018, she was hospitalized for the fifth recurrent episodes and the highest body temperature was 39.1℃. LP showed ICP > 330 mmH2O, WBC 6750 cells/ml (5% mononuclear cells, 95% multinuclear cells), total protein 1.88 g/L and glucose < 0.5 mmol/L. NGS of patients’CSF sample was performed and the result identified T. solium infection and the reads was 27729 (Fig. 2A). Meanwhile, the cysticercosis specific antibody of serum and CSF were positive by enzyme-linked immunosorbent assay (ELISA). Combined with imaging results,she was diagnosed as subarachnoid NCC and was treated with albendazole, prednisone. Her symptoms were relieved and CSF findings were improved significantly after treatment of 38 days. The result of CSF demonstrated reduced leukocytosis (48 cells/ml) with protein 1.14 g/L and glucose 1.0 mmol/L. The solium DNA sequence reads dropped to 814 (Fig. 2B). Antihelminthic therapy was not continued because of drugs-induced liver injuries. On February 2019, the patient developed episodic left upper limb weakness with involuntary paroxysmal shaking. Each attack lasted for about 10 seconds and symptoms could relieve completely. Brain MRI didin’t revealed hyperintense lesions on DWI. She was hospitalized and treated with aspirin for transient ischemic attack (TIA) and albendazole for NCC. The symptoms relieved completely after treatment. Before discharge, LP results showed ICP was 150 mmH2O, WBC 100 cells/ml (80% mononuclear cells, 20% multinuclear cells), total protein 1.92 g/L and glucose 0.9 mmol/L. The CSF solium DNA sequence reads dropped to 528 (Fig. 2C). The clinical timeline is presented in Fig. 3 and the results of LP are summarized in Table.1. She did not have a definite history of epidemiological contact. Follow up indicated that she has not had a severe symptom flare until now.