A 26-year-old female was admitted to a local hospital with symptoms of acute fever, headache, phonophobia, photophobia, nausea, vomiting, dizziness, appetite loss, and fatigue. She was administered oral cefuroxime axetil and 0.9% intravenous saline for two days, with a diagnosis of dehydration and acute sinusitis. She took the antibiotic for two days. She was then evaluated by another clinician due to suspicion of meningitis based on marked neck stiffness and a positive Kernig's sign, and was administered Procaine penicillin for one day and cefazolin for four days. Because of persistent fever, she was referred to our hospital. On admission, her complaints were only headache and fatigue, and she presented a fever of 39.5 C. Her physical examination revealed no distinct findings or any definite signs of meningeal irritation or mucocutaneous lesions. She was alert, fully conscious, and was well oriented. Her sensation, including the perineal area, was normal. She demonstrated no signs of encephalopathy. Her neurological examination showed only right truncal and gait ataxia. She presented no seizure or head trauma, and she was not taking any antiepileptic drugs.
Laboratory examination revealed normal C-reactive protein, with a normal white blood cell count but a high erythrocyte sedimentation rate (40 mm/h). The only laboratory test performed before she was accepted to our hospital was urinalysis, which revealed a density of 1030. The blood chemistry, urinalysis, and immunoglobulin concentrations (G, A, and M) were normal. The cerebrospinal fluid (CSF) examination showed a mononuclear leukocytosis of 408/mm3, increased protein content of 165 mg/dl, and a mildly decreased glucose level of 38 mg/dl (34% of serum glucose). Bacterial smears and cultures, including tuberculosis, were negative. No increase in oligoclonal bands and a mild increase in the IgG index (0.8) were observed in the CSF. The CSF enzyme immunoassay demonstrated negative IgM antibodies against herpes simplex virus type-1 (HSV-1) and herpes varicella zoster (VZV) viruses. Neither tuberculosis nor HSV was detected in her CSF sample by polymerase chain reaction (PCR). Furthermore, brucella agglutination was negative in the CSF.
Serological tests on the blood samples -- brucella agglutination, salmonella agglutination, brucella Coombs, and VDRL -- were negative. Western blot examination revealed that the samples were negative for IgM and IgG antibodies against Borrelia burgdorferi and IgM antibodies against HSV-1. Anti-ds DNA, Ena Jo-1, Ena SCL70, Ena Sm, Ena Sm-RNP, Ena SsA, Ena SsB, anti-cardiolipin, and anti-phospholipid antibodies were also negative. The lipid profile, fibrinogen, homocysteine, antithrombin III, and protein C and S levels were all within normal ranges.
Electroencephalography (EEG), echocardiography, and abdominal and urinary ultrasonography were normal. Cranial magnetic resonance images (MRI) on admission demonstrated an isolated small lesion in the SCC that was markedly hyperintense on diffusion-weighted images (DWI), fluid-attenuated inversion recovery (FLAIR) images, and T2-weighted images (T2WI); hypointense on the apparent diffusion coefficient (ADC) and slightly hypointense on T1-weighted images (T1WI) (Figure 1). Some increase was observed only in the enhancement of the leptomeninges (Figure 1A).
Four days later, the patient developed lower abdominal pain and one episode of fecal incontinence. The urinary bladder was distended and palpable above the symphysis pubis. Catheterization of the urinary bladder yielded some clear urine. An urodynamic study revealed an acontractile neurogenic bladder, but bladder sensation was spared with a first urge to void after 129 ml of 0,9% intravesically saline was given. No other neurological abnormalities were detected. Spinal cord MRI revealed that the cervical and thoracic spinal cord was swollen. T2WI showed diffuse high-signal intensity inside the cervical and thoracic spinal cord (Figure 2A). The pia mater over the cervical spinal cord, the thoracic spinal cord, and the parenchyma were enhanced heterogeneously, whereas diffuse meningeal enhancement was present over the conus medullaris (Figure 2B, 2C).
Based on the CSF findings, ceftriaxone and acyclovir treatment were started for an initial diagnosis of aseptic meningitis or poorly treated bacterial meningitis. After five days of treatment, the patient was still febrile. Ampicillin was added to the treatment for suspected meningitis due to Listeria monocytogenes. After three days of ampicillin treatment, her fever resolved. Ceftriaxone and acyclovir treatment was continued for fourteen days, and ampicillin treatment for twenty-one days.
In order to ameliorate the voiding difficulty, a permanent internal catheter was implanted for six weeks. Ten days later, her fever had diminished. On follow-up five weeks after the initial examination, gait ataxia and cranial and spinal MRI findings had resolved (Figures 3 and 4); at seven weeks, the urodynamic study was normal.