NEUROLOGICAL MANIFESTATIONS OF PRIMARY SJOGREN’S SYNDROME (PSS)

Primary Sjogren’s Syndrome

Abstract

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.