Abstract
Unique Presentation of Neurosarcoidosis: Case Report and Systematic Review
Author(s): Humberto Foyaca-Sibat*Background: Sarcoidosis (Sa) is an uncommon and poorly known inflammatory multisystem disease of uncertain aetiology, histopathological characterized by noncaseating giant-cell granulomas affecting the lungs, the heart, peripheral nervous system, and the eyes mainly. Neurologic involvement of Sa (NS) can involve the central nervous system or peripheral nervous system or both simultaneously, causing remarkable morbidity and mortality if NS remains untreated. Up to date, there is no worldwide agreed diagnostic program for patients with Sa.
Case presentation: A 37-year-old female patient complained of chronic headache, upper back pain, and progressive decreasing visual acuity since 2012. In May 2021, she was admitted to the ophthalmology ward because her visual acuity deteriorated, and she lost vision on the right eye three weeks after admission. She also complained of generalized intermittent headache and dizziness, not associated with other symptoms or signs, without aggravating or realizing factors but responding to routinary pain killers. HIV non-reactive with PCR for COVID-19 negative. The diagnosis of ON in the right eyes and optic peri-neuritis in the left eyes was made. Neuro-ophthalmologic examination confirmed ON and OP. Investigations confirmed EST, ON, OP and IDEM granulomas.
Results: Only one male patient presented NS/ON/OP was identified through this searching research. Therefore, our case is the first one affected by ON/OP/EST/IDEM/NS to be reported to the international medical community.
Discussion: After a powerful searching of the literature, we did not find a case of NS combining ON/OP with empty sellar turcica syndrome and spinal tumour. Therefore, this patient is the first reported to the medical literature with such presentation from our knowledge. We discuss on the Histopathology and differential diagnosis of NS, Disorder of the sella turcica, ocular sarcoidosis, treatment, and mortality rates. Finally, we delivered one hypothesis on pathogenesis of Sa granuloma formation.
Conclusion: We delivered a hypothesis on the pathogenesis of the NS based on the role played by TNF-α, IFN-β/γ, and IL-2, dysbiosis, and other elements the mechanism of Sa granuloma formation. Finally, we concluded that this literature search might serve other investigators to comprehend better the NS, the importance of making an early diagnosis with therapy and performing a regular assessment looking for other frequently associated disorders and their management. Notwithstanding, large, and well-designed cross-section studies and RCT will be necessary to provide the best information about all this matter.