PO50 - “From Chest to Brain”: Three Clinical Presentations of Neurosarcoidosis Spectrum
João Maria Bento (Portugal)1; Mariana Rodrigues de Oliveira (Portugal)2; Leandra Fagundes (Portugal)2; Juliana Barata (Portugal)1; Eunice Magalhães (Portugal)1; Pedro Ferreira (Portugal)3; Maria La Salete Valente (Portugal)1;
1 - Serviço de Pneumologia, ULS da Cova da Beira; 2 - Serviço de Neurologia, ULS de Coimbra; 3 - Serviço de Pneumologia, ULS de Coimbra;
Keywords: Neurosarcoidosis; Phenotypic variability;
Select the theme: Challenges in Sarcoidosis Diagnosis and Staging
Type: Clinical Cases
Presentation: Poster Presentation

Introduction: Neurosarcoidosis is a rare and heterogeneous manifestation of Sarcoidosis that may involve the central or peripheral nervous system at different stages of systemic disease. We present three illustrative cases demonstrating distinct clinical phenotypes.

Clinical Case Description: Case 1: A 63-year-old man with previous uveitis developed progressive lower-limb weakness. Neurological examination revealed symmetrical proximal paraparesis with pyramidal signs. Neuroaxis-MRI showed multiple intramedullary and diencephalic abnormalities suggestive of granulomatous inflammatory disease. He subsequently developed migratory arthritis and restrictive pulmonary disease. Thoraco-abdomino-pelvic CT demonstrated calcified mediastinal-hilar lymphadenopathy, pleural effusion, ground-glass pulmonary changes consistent with sarcoidosis (Scadding 2), and extrathoracic nodal involvement. The overall findings supported possible neurosarcoidosis. He responded well to corticosteroids, with temporary methotrexate use, achieving clinical recovery and radiological improvement (Scadding 1).

Case 2: A 60-year-old woman presented with bilateral parotitis followed by sequential peripheral facial palsies and progressive gait imbalance. Examination showed bilateral vestibular impairment and glove-and-stocking sensory loss. MRI identified a left carotid expansive lesion, while electromyography demonstrated mild sensory axonal polyneuropathy. Further investigations revealed uveitis (Heerfordt syndrome). FDG-PET showed hypermetabolic cervical, mediastinal, and bilateral hilar lymphadenopathy. Lymph node biopsy confirmed sarcoidosis. Diagnosis of probable neurosarcoidosis was established, with good response to corticosteroid and Infliximab.

Case 3: A 35-year-old man initially presented with Löfgren syndrome, including fever, erythema nodosum, arthralgia, bilateral hilar lymphadenopathy, and pulmonary involvement (modified Scadding 2). Initially managed conservatively, he later developed a seizure caused by an expansive periventricular brain lesion. Brain biopsy demonstrated non-necrotizing granulomatous inflammation, establishing definite neurosarcoidosis. Corticosteroid therapy led to neurological stabilization and pulmonary radiographic improvement (Scadding 1).

Conclusions: These clinical cases highlight the phenotypic variability of neurosarcoidosis, with possible, probable and definite diagnosis, encompassing both central and peripheral manifestations. These cases underscore the necessity of a high index of clinical suspicion, the importance of histological confirmation whenever feasible, and the requirement for a multidisciplinary approach.