A 22-year-old Filipino male college student with no prior hospitalizations but with a family exposure to tuberculosis and multiple male sexual partners had a two-week history of undocumented fever, headache, nape pain, progressive leg numbness, and subsequent paraplegia. After one week, the patient developed slowness in mentation, progressive blurring of vision, and had one episode of left focal tonic seizure on the day of admission. On admission, he was normotensive, slightly tachycardic, and afebrile, with drowsiness, orientation to self, and ability to follow commands. He had no light perception, with sluggishly reactive 3mm pupils, cranial nerve 5–7 palsies on the right, hyporeflexia, and paraplegia. The cerebellar exam was noncontributory, but nuchal rigidity was present.
Cranial computed tomography (CT) with contrast was done on admission and revealed leptomeningeal and basal cistern enhancement, hydrocephalus, and a minimally enhancing mass along the right cerebellopontine angle. The neurosurgery team placed an emergency ventriculoperitoneal shunt (VPS) due to clinical signs of raised intracranial pressure on the second day of admission. Cerebrospinal fluid (CSF) analysis was done intraoperatively under sedation 2 hours prior to the surgery, which showed yellow, hazy fluid with red blood cells (RBC) 140 x10^6/L, white blood cells (WBC) 231 x10^6/L (lymphocytic predominant), elevated proteins 11,043 mg/dL, and hypoglycorrhachia. He was managed as tuberculous meningoencephalitis with right cerebellopontine tuberculoma, and received intensive phase of the anti-Koch regimen and received dexamethasone 15mg/day for the 2 months he was admitted.
Complete CSF tests were available on the 3rd week of hospitalization and were negative for acid-fast bacilli smears, Cryptococcal Antigen Latex Agglutination System, Bactigen latex agglutination, TB culture, and aerobic, anaerobic, and fungal cultures. HIV, toxoplasmosis, and syphilis screening tests were also negative. Chest radiograph and sputum studies were sent for TB work-up which were negative. The initial CT imaging raised suspicion of a possible tuberculoma, but the mass's extent and location in the ventral midline pontomedullary area surrounding the ventral brainstem were atypical for tuberculosis. Since CSF culture studies were negative, aseptic meningitis became an alternative diagnosis. A craniocervical magnetic resonance imaging (MRI) with single voxel spectroscopy was scheduled after the CSF studies were negative but was only done on the 5th week of admission due to financial and logistical issues including a prior CT imaging that was deemed diagnostic already of tuberculosis and further imaging was less prioritized by hospital administration. However, his MRI and MRS revealed a well-defined, non-enhancing mass that did not expand the internal auditory canal and located in the right cerebellopontine angle with no restricted diffusion or magnetic susceptibility artifacts. (See Fig. 1) Single voxel spectroscopy indicated the persistence of an N-acetyl aspartate (NAA)- --like peak and the absence of lipid-lactate peaks, consistent with a mucin-containing cyst. Given the conventional MRI findings, the mass was a neurenteric cyst that had ruptured, causing chemical meningitis. Anti-Koch regimen was halted since he developed elevated liver enzymes on serial electrolyte monitoring and was no longer warranted but dexamethasone at 15mg/day was continued and slowly tapered due to prolonged use.
The patient reported initial improvement of mentation and motor strength from total paraplegia to paraparesis after the emergency VPS. He was open to undergo further surgery in hopes to further improve motor strength. Further surgery was in discussion when he experienced multiple bouts of pneumonia as he reported worsening dysphagia. He eventually developed sepsis and septic shock and was not fit for surgery and eventually succumbed to respiratory failure on the 2nd month of hospitalization, exacerbated by further brainstem compression.