Chorioretinitis, bilateral optic neuritis and pachymeningitis as manifestation paraneoplastic probable of esophageal squamous cell carcinoma

Case presentation: Man, 61 years old, smoker, started in February 2024 progressive visual impairment of the left eye and holocranial headache in “pressure”. Evolutionarily, he presented a left clavicle fracture. On physical examination, the patient didn’t have neurological deficits, except for the left eye with visual acuity 0.3 and fundoscopy with optical disc edema. Bone scintigraphy detected diffuse radiopharmaceutical uptake, including the skullcap, left clavicle, and costal arches, and the neuroimaging study showed hypersignal on T2/FLAIR sequences, and contrast uptake in the retina, optic nerve on the left, and meningeal enhancement at the base of the brainstem, and anterior cranial fossa. The visual evoked potential test demonstrated bilateral optic neuropathy, worse on the left, and rachicentesis, pleocytosis with lymphomonocytic predominance and hyperproteinorrachia, latex for bacterial or fungal agents, and culture negative. Neoplastic cells on cytopathological examination absent, serology for HTLV, HIV, CMV, HBV , HCV and syphilis negative. Pulse therapy was performed with methylprednisolone 1 g/day for 5 days, with improvement in the condition. Esophagogastroduodenoscopy with histopathology positive for esophagogastric transitional squamous cell carcinoma, palliative chemotherapy with carboplatin and paclitaxel is initiated. Later, the worsening conditions, associated with aggressiveness and convulsive crises of focal onset, the new cytopathological examination of cerebrospinal fluid detects atypical cells, suggesting meningeal carcinomatosis. Admitted to a palliative care ward, he developed a headache that was refractory to optimized analgesia and worsened his behavioral condition, resulting in death. Discussion: Paraneoplastic neurological syndromes represent 1 in every 300 cancer patients, and result from the production of antibodies with target organ dysfunction. The diagnosis is based on the understanding of clinical, oncological, and immunological heterogeneity, with the need to exclude other causes, such as meningeal carcinomatosis or brain metastasis, but its treatment is still uncertain. The diagnostic criteria are supported by a panel called PNS-Care that includes high-risk phenotypes, such as limbic encephalitis, and intermediate-risk phenotypes, when the onset is rapidly progressive, with inflammatory findings on cerebrospinal fluid or neuroimaging. Although uncommon in tumors of the gastrointestinal tract, a literature review carried out between 1970 and 2021 about esophageal cancer described phenotypes for neurological paraneoplastic syndromes. Final comments: Paraneoplastic neurological manifestations are heterogeneous, we report a case of choreoretinitis, bilateral optic neuritis and pachymeningitis as the initial manifestation of esophageal squamous cell carcinoma, signaling it for further studies, and alerting clinical reasoning in the face of similar conditions that occur in others centers.