Version 1
: Received: 3 November 2023 / Approved: 6 November 2023 / Online: 6 November 2023 (08:05:25 CET)
How to cite:
De Luca, M.; Russo, M.; Pardeo, O.; Sframeli, M.; Messina, S. Central Nervous System Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Review of the Literature. Preprints2023, 2023110292. https://doi.org/10.20944/preprints202311.0292.v1
De Luca, M.; Russo, M.; Pardeo, O.; Sframeli, M.; Messina, S. Central Nervous System Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Review of the Literature. Preprints 2023, 2023110292. https://doi.org/10.20944/preprints202311.0292.v1
De Luca, M.; Russo, M.; Pardeo, O.; Sframeli, M.; Messina, S. Central Nervous System Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Review of the Literature. Preprints2023, 2023110292. https://doi.org/10.20944/preprints202311.0292.v1
APA Style
De Luca, M., Russo, M., Pardeo, O., Sframeli, M., & Messina, S. (2023). Central Nervous System Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Review of the Literature. Preprints. https://doi.org/10.20944/preprints202311.0292.v1
Chicago/Turabian Style
De Luca, M., Maria Sframeli and Sonia Messina. 2023 "Central Nervous System Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Review of the Literature" Preprints. https://doi.org/10.20944/preprints202311.0292.v1
Abstract
Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a rare form of necrotizing granulomatous inflammation that predominantly affects medium and small size vessels. Generally respiratory tracts and kidneys are the most affected organs. Neurological manifestations of GPA usually involve the peripheral nervous system causing mononeuritis multiplex or and cranial nerve palsies. Central nervous system (CNS) involvement is rare and characterized by cerebral vasculitis, meningeal inflammation, or mass lesions. The CNS involvement is often linked to Ear Nose Throat (ENT) system disease. In this report, we present two cases of patients diagnosed with GPA who exhibit CNS involvement as first symptom. In the first one with an ischemic stroke while in the second with an atypical headache. In the first patient, a positive medical history indicating involvement of the Ear, Nose, and Throat (ENT) facilitated a precise diagnosis through the prompt execution of autoimmune screening. This, in turn, enabled a timely commencement of treatment, resulting in neurological improvement. In the second case, the significance of ENT involvement was initially overlooked in the context of pathological definition, leading to a substantial delay in diagnosis and an ultimately grim prognosis for the patient. The ENT involvement could be an easy marker to suspect GPA in patient with atypical CNS presentation and could help to make a prompt diagnosis.
Keywords
GPA; Stroke; headache
Subject
Medicine and Pharmacology, Neuroscience and Neurology
Copyright:
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
