The Inconspicuous Cysts

A previously healthy 40-year-old male, with a past medical history significant for latent tuberculosis s/p treatment 15 years ago, presented with blurry vision and progressively worsening headaches for 6 months. He was originally from Guatemala and moved to the US in 2001 (crossed the deserts through Mexico and Texas).

“The Inconspicuous Cysts” Lily Zhang1, Folusakin Ayoade2, Julio Diaz Perez2 1 Bascom Palmer Eye Institute, Miami, Florida, USA, 2University of Miami, Miami, Florida, USA History & Exam A previously healthy 40-year-old male, with a past medical history significant for latent tuberculosis s/p treatment 15 years ago, presented with blurry vision and progressively worsening headaches for 6 months. He was originally from Guatemala and moved to the US in 2001 (crossed the deserts through Mexico and Texas). On exam, his visual acuity was 20/20 OU with dense peripheral field loss and bilateral grade 5 papilledema with hemorrhages. MRI brain/orbits showed bilateral flattening of the posterior sclera with intraocular protrusion of the optic nerve head and prominent leptomeningeal enhancement. The patient was admitted to a multidisciplinary team and an extensive workup was performed. His lumbar puncture (LP) showed an elevated opening pressure (OP >40 CmH2O), severe hypoglycorrhachia, high protein, borderline elevated CSF eosinophils and lymphocytic pleocytosis. His extended CSF/serum/imaging studies were unrevealing. Given the chronicity of the symptoms, epidemiologic factors (history of having crossed the deserts through Mexico and Texas) and CSF findings, he was considered most likely to have chronic fungal meningitis (Coccidiodes). Empiric antifungal treatment was started. A month later, the patient returned with decreased visual acuity of 20/40 OD, worsening visual fields, and papilledema. He was put on acetazolamide and readmitted. Repeated brain MRI revealed similar pachymeningitis. Thoracic MRI showed mildly enhanced mid-right anterolateral cord lesion and pial linings around mid-distal cord and conus. Repeated LP showed elevated OP and CSF signs of meningitis, but his extended CSF/serum studies remained unrevealing. Due to his high risk of vision loss, a VP shunt was placed and antifungal treatment was continued. He returned 2 months later with abdominal pain, urinary retention, and difficulty in ambulating. MRI spine showed diffuse leptomeningeal enhancement that had worsened collections, rim enhancing lesions, and cord compression. A diagnostic procedure was performed. Financial Disclosures: The authors had no disclosures. Grant Support: None. 2022 Annual Meeting Syllabus | 39 “The Inconspicuous Cysts” Answer Final Diagnosis Severe extra-parenchymal neurocysticercosis (NCC) with arachnoiditis and spinal lesions resulting in papilledema, lower extremity weakness, and bladder dysfunction Summary of Case The patient underwent a laminectomy with a biopsy of the spinal lesions with neurosurgery. The intramedullary biopsy was positive for Neurocysticercosis. He was started on albendazole, praziquantel, and prednisone with significant improvement in his symptoms. On follow-up, his visual acuity was 20/20 OU with significantly improved visual field and complete resolution of papilledema. This is an intriguing case of severe papilledema as the initial sign of extra-parenchymal NCC involving the spinal cord. Morevoer, the worsening papilledema after a course of dedicated therapy led to the further exploration of the pathology. Accurate and early diagnosis of NCC is critical but remains challenging due to its variable clinical presentations and neuroimaging findings. Especially for extra-parenchymal NCC, the parasites may not be detected by MRI because they have similar signals as CSF and could not be enhanced by gadolinium due to lack of scolex.[1] Hence, a high clinical suspicion for this occult disease is crucial in managing patients with intractable meningitis. We would recommend considering NCC as one of the differential diagnoses for patients with intractable meningitis, especially when there is established epidemiological link. This will include routinely checking CSF/serum cysticercoid IgG antibody [2], performing an MRI FIESTA (fast imaging employing steady-state acquisition) sequence for better visualization of small cysts in the arachnoid space[1] and even considering the promising quantitative PCR or next-generation sequencing-based pathogen analysis of CSF [3] for improving the chances of early diagnosis. Moreover, spinal NCC is very rare, about 0.7% to 5.8% of all NCC cases. Mainstay treatment is medical but there is no standard of treatment, even from most recent guidelines from the Infectious diseases society of America [4]. Recent reports suggest combined surgical and medical therapy may have the best outcome[5] such as our patient whose neurological symptoms improved with mild residual deficits. Struggle/Dilemma of the Clinical Presentation Description We struggled in diagnosing the cause of his meningitis and related severe papilledema. The patient has no recent travels or recent risk factors for the tapeworm Taenia solium infection even though he is from Central America with high epidemiological link to tapeworm infections. He has been in Miami for 20 years and has lived with his wife without any known exposure to raw meat. Additionally, extensive and repeated neuroimaging did not show typical cystic lesions. Keywords: papilledema, magnetic resonance imaging (MRI), vision field loss, ring-enhancing lesions, lumbar puncture References 1. Carrillo Mezo, R., et al., Relevance of 3D MRI sequences in diagnosing basal subarachnoid neurocysticercosis, Acta Trop, 152, 60-5, 2015. 2. Arroyo, G., et al., Antibody Banding Patterns of the Enzyme-Linked Immunoelectrotransfer Blot and Brain Imaging Findings in Patients With Neurocysticercosis. Clin Infect Dis, 66, 282-88, 2018. 3. O'Connell, E.M., et al., A Novel, Highly Sensitive Quantitative Polymerase Chain Reaction Assay for the Diagnosis of Subarachnoid and Ventricular Neurocysticercosis and for Assessing Responses to Treatment, Clin Infect Dis, 70(9), 8751881, 2020. 4. White, A.C., Jr., et al., Diagnosis and Treatment of Neurocysticercosis: 2017 Clinical Practice Guidelines by the Infectious Diseases Society of America (IDSA) and the American Society of Tropical Medicine and Hygiene (ASTMH),Am J Trop Med Hyg, 98(4), 945-66, 2018. 5. Qi, B., et al., Spinal intramedullary cysticercosis: a case report and literature review, Int J Med Sci, 8(5), 420-3, 2011. Contact Information: None provided. 40 | North American Neuro-Ophthalmology Society