Objective: To investigate cognitive deficits in patients with primary Sjögren's syndrome (PSS). Method: Eighteen patients with PSS, aged between 25 and 61 years, were subjected to a short neuropsychological battery and compared with 18 patients with multiple sclerosis and 18 healthy controls. Results: The analysis of variance (ANOVA) revealed that the clinical groups had significantly worse performance than the control group on the Rey Auditory Verbal Learning Test 3; (F 2,53 =3.500, p=0.038) and 7 (F 2,53 =5.068, p=0.010). The clinical groups had elevated levels of depression on the Beck Depression Inventory (BDI); (p=0.003). The analysis of the data from the Trail Making Test B-A revealed a significant difference between the clinical and control groups (p=0.023). The analysis of covariance with BDI score as a covariate, did not change the outcome. Conclusion: Our study revealed cognitive deficits in patients with PSS detectable by a short neuropsychological battery.Keywords: neuropsychological evaluation; Sjögren's syndrome, cognitive impairment, neuropsychology. Primary Sjögren's syndrome (PSS) is an inflammatory autoimmune disease characterized by infiltration of exocrine glands. It occurs without an association with other connective tissue diseases and affects mainly salivary and lachrymal glands, causing xerostomy and keratoconjunctivitis sicca 1,2 . It often afflicts middle-age women, with an estimated prevalence of 2% in the adult population 3 . Moreover, PSS can be associated with impairments of the nervous system. In the central nervous system (CNS), the focal and nonfocal involvement of the brain and spinal cord has been described, resulting in a wide spectrum of neurological, psychiatric, and cognitive symptoms 4 .Cognitive impairments in patients with PSS were reported by Malinow 5 and later confirmed by other studies 6,7 . In Brazil, we have not found any case-series studies that have investigated cognitive impairments in PSS. We found one case report 8 .The results from other countries suggest that patients with PSS display cognitive impairments in attention, memory,
BACKGROUNDThe relationship between primary Sjögren's syndrome (pSS) and demyelinating diseases is still not well understood. These diseases seem to coexist amidst autoimmunity, raising questions about clinical characteristics, relationship with immunomodulatory treatment, and possible common immunological background underlying their pathogenesis. The objective of this work was to evaluate the frequency of dry oral, ocular manifestations and autoantibodies characteristic of pSS in a population of patients with multiple sclerosis.
METHODSA total of 202 patients with multiple sclerosis answered a questionnaire to identify complaints of xerostomia and xerophthalmia, according to diagnostic criteria for pSS; 43 answered positively to at least one question; 27 had comorbidities or used drugs that cause dry symptoms and were excluded; 16 patients were selected for examinations for oral, ocular and serum anti-Ro/SS-A autoantibody evaluation.
RESULTSEleven (68.8%) patients complained of xerostomia; 14 (87.5%) of xerophthalmia. Sialometry < 0.1 mL/min was observed in three (18.8%); 13 patients underwent minor salivary gland biopsy and histopathological examination: focal score > 1 in three (23.1%). Schirmer test was < 5 mm/5 min in four (25%). Lissamine green/fluorescein dye score was > 5 in three (18.8%). Anti-Ro/SS-A > 10 IU/mm in two (12.5%). Three (1.5%) patients met current criteria for pSS.
CONCLUSIONPatients with multiple sclerosis may report xerostomia and/or xerophthalmia even in the absence of comorbidities and use of medications capable of causing these symptoms, which may fulfill the diagnostic criteria for pSS. In this study, the frequency of pSS according to current criteria was within the range observed in the literature with older criteria. But the question remains whether the association between these diseases is fortuitous or whether there is a pathogenic link.
As neoplasias malignas de células linfoides surgem de células do sistema imunológico em diferentes estágios de diferenciação, resultando em amplos achados clínicos, morfológicos e imunológicos.
A distrofia muscular de cintura/Limb Girdle Muscular Distrophy (DMC/LGMD) é causada por mutações do gene CAPN3 que codifica a proteína Calpaína, que desempenha um papel na manutenção da integridade e função muscular.
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