Objective: To determine prevalence, clinical patterns and outcomes of neurological involvement in a cohort of primary sjogren’s syndrome (PSS) patients presenting to a tertiary care hospital.
Study Design: Observational retrospective cross-sectional case-control study.
Place and Duration of Study: This study was carried out at Neurology department of Pak Emirates Military Hospital, Rawalpindi, from May 2015 to Jun 2016.
Patients and Methods: All patients fulfilling American College of Rheumatology (ACR) criteria of PSS and having neurological involvement, who were admitted in Neurology wards from May 2015 to June 2016, were included in the study. Demographic, clinical and seroimmunological data of the patients was documented.
Results: A total of 26 patients with PSS had some degree of neurological involvement. Mean age was 40.50 years. (SD 14.803, min 22, max 78). Fifteen patients were female and 11 were male. Sicca symptoms (ocular and oral dryness) were present in 38.5%. Serological marker anti Ro and La were present in 76.9% and 42.3% respectively while both Ro and La were present in 34.6%. Lip biopsy was diagnostic in 80.8% and schirmer test was positive in 46.2%. Refractory headache was present in 84.6%. Seizures occurred in 34.6%, which were focal in 23.1% and generalized in 11.5%. Trigeminal neuralgia was present in 26.9%, multiple cranial nerve palsies in 15.4% and recurrent facial nerve palsies in 11.5%. Optic neuritis was seen in 19.2%. Clinical presentation mimicking relapsing and remitting multiple sclerosis was seen in 30.8% of patients among whom 61.5% also met revised McDonald criteria for dissemination in space (DIS) on MRI and 23.1% met criteria for dissemination in time. MRI brain showed cortical lesions in 42.3%. Longitudinally extensive transverse myelitis involving cervical and upper thoracic cords was present in 26.9% of patients.
Conclusion: The diagnosis of neuro-sjogren’s syndrome (NSS) can be difficult in the absence of sicca symptoms especially when neurological manifestations precede sicca symptoms by many years. This requires a high index of clinical suspicion and low threshold for investigations like lip biopsy and autoantibody profile for the diagnosis. This study highlights the need to revise the overemphasis of sicca symptoms in various current diagnostic criteria in order to improve early recognition and initiation of treatment.
Keywords : Central Nervous System (CNS), Intracranial Hypertension (ICH), Neuro-Sjogren’s syndrome (NSS), Peripheral Nervous System (PNS).