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Título

Intracranial hypertension as a rare neurological presentation of Sjögren's syndrome: a case report

RESUMO

Case report: A healthy, young female patient presented a sudden short-term loss of consciousness. In the next day she complained of moderate headache that worsened in supine position and emesis. It evolved with bilateral diplopia and convergent strabismus. Brain MRI showed signs of intracranial hypertension (ICH). Her examination revealed good general condition, gait deviation to the right in the Fukuda’s Test, star gait with eyes closed and bilateral paresis of the VI cranial nerve. Pupils were isocoric, photoreactive, visual acuity was normal, as it was the fundoscopy. Lumbar puncture revealed proteins 41, glucose 40, negative anti-AQP4, anti-MOG andOCB, and intrathecal synthesis of IgA and IgM antibodies, indicating blood-brain barrier dysfunction, negative IgG, and intrathecal production of kappa free light chain (Kappa index 7.23), and neurofilaments = 4852. Laboratory tests showed VHS 34, anti-RO 240, anti-LA 174. She was referred to the Rrheumatology Service for Sjögren's Syndrome (SS) investigation. Because of complaints of dysphagia and xerostomy during this period, salivary gland biopsy was performed, and the findings were compatible with SS. She was treated with corticosteroids, acetazolamide, azathioprine, hydroxychloroquine and lubricating eye drops, and all symptoms resolved. Discussion: ICH is a clinical syndrome characterized by increased intracranial pressure without brain or cerebrospinal fluid lesions that justify it, maybe explained by recent discoveries of the glymphatic and lymphatic systems of the brain. It mainly affects overweight or obese women. Although female, the patient in this case was eutrophic. SS is a rheumathologic disease with 8.5 to 70% of the patients presenting with neurological complains. The diagnosis of SS is based on clinical grounds and laboratory findings with positivity of anti-Ro and anti-La in 52% and 34% of the patients respectively. There are few cases described where ICH was the primary symptom of SS. In our case, the CSF analysis revealed a high Kappa index and neurofilaments, important biomarkers related to important neuroaxonal damage. Kappa index and neurofilaments are promising biomarkers for the diagnosis, prognosis and evaluation of the disease progression in immunomediated compromise of the Central Nervous System. Final comments: Although rare, neurological manifestation of SS may be the first symptom of the disease. Among the neurological manifestations described, ICH is even less frequent.

Palavras Chave

intracranial hypertension, sjogren syndrome, neurological manifestation, immunomediated neurological manifestation