共济失调
医学
皮肤病科
舍格伦综合征
病理
全身性疾病
精神科
免疫病理学
作者
Cristina Saade Jaques,Marianna Pinheiro Moraes de Moraes,Eduardo Antonio Roquim Silva,Antônio R. Coimbra‐Neto,Alberto Martínez,Sarah Camargos,Francisco Cardoso,Marcondes C. França,Anamarli Nucci,José Luiz Pedroso,Orlando Graziani Póvoas Barsottini
标识
DOI:10.1136/jnnp-2019-322373
摘要
Sjogren’s syndrome is a chronic autoimmune disorder characterised by lymphocytic infiltration of exocrine glands that causes dysfunction in salivary and lacrimal glands.1 Clinical features include dry eyes, dry mouth, fatigue, muscle pain and swelling, in association with the production of serum autoantibodies.1
The neurological manifestations of Sjogren’s syndrome reach up to 70% of these patients and include peripheral neuropathy, aseptic meningitis, vasculitis, seizures, myelitis, cognitive dysfunction, optic neuropathies, parkinsonism and others.1 The most common neurological manifestations are characterised by axonal neuropathy, sensory neuronopathy and small fibre neuropathy.1
On the other hand, ataxias are a heterogeneous group of diseases that comprise genetic and non-genetic aetiologies. The non-genetic or sporadic ataxias are frequently related to autoimmune disorders.2 However, cerebellar ataxia is rarely described in Sjogren’s syndrome. It is mandatory to classify ataxia as related to cerebellar damage (cerebellar ataxia) or to peripheral neuropathy (sensory ataxia). Usually, patients with Sjogren’s syndrome may present with sensory ataxia related to a sensory neuronopathy.2
This study aimed to characterise the pattern of ataxia in Sjogren’s syndrome and also to describe cerebellar ataxia and cerebellar atrophy in some of these patients.
Fourteen patients with confirmed Sjogren’s syndrome and ataxia were enrolled on this study. All patients fulfilled criteria for primary Sjogren’s syndrome according to the American-European Consensus Group, 2002 and underwent clinical evaluation. Complementary tests included serum autoantibodies Ro (SSA) and …
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