Créange A, Laplane D, Habib K, Attal N, Assuérus V
Clinique des maladies du Système Nerveux, Groupe hospitalier Pitié-Salpêtrière, Paris.
Rev Neurol (Paris). 1992;148(5):376-80.
Two cases of primary Sjögren's syndrome revealed by dementia are reported. The patients had progressive or subacute memory dysfunction and psychiatric disorders with depression and delirium. The diagnosis of Sjögren's syndrome was established by biopsy of the minor salivary glands. Both patients were treated with corticosteroids. The neuropsychiatric symptoms improved dramatically in one case and remained unchanged in the other case. Dementia in Sjögren's syndrome seems to be without aphasia, apraxia or agnosia, and associated with psychiatric features, particularly depressive symptoms, thus including some characteristics of subcortical dementia. Diagnosis may be difficult because, as shown in our cases, symptoms of ocular and buccal dryness can be absent. Salivary gland biopsy can be useful in the evaluation of patients with dementia of undetermined etiology.
报告了两例以痴呆为首发表现的原发性干燥综合征病例。患者有进行性或亚急性记忆功能障碍以及伴有抑郁和谵妄的精神障碍。通过小唾液腺活检确诊为干燥综合征。两名患者均接受了皮质类固醇治疗。其中一例患者的神经精神症状显著改善,另一例则无变化。干燥综合征所致痴呆似乎无失语、失用或失认,且伴有精神症状,尤其是抑郁症状,因此具有一些皮质下痴呆的特征。诊断可能存在困难,因为如我们的病例所示,眼部和口腔干燥症状可能不存在。唾液腺活检有助于评估病因不明的痴呆患者。