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Título

Cerebellar ataxia in primary Sjögren's syndrome without cerebellar atrophy

RESUMO

Case presentation: An 86-years-old woman presented with imbalance, hand tremors, and frequent falls for 3 years. She reported systemic hypertension, coronary artery disease, previous hepatitis B and C, and a history of alcoholism interrupted for more than 10 years. The neurological evaluation showed hypometric saccades in the pursuit of the gaze, altered vestibulo-ocular reflex, axial and appendicular signs of cerebellar ataxia more evident on the left side. Sensory examination was normal. She had signs of sicca syndrome, with tongue fissures and thick saliva, as well as ocular biomicroscopy with altered But test. Brain MRI showed no cerebellar atrophy, and there was elevated erythrocyte sedimentation rate and positive anti-RO. Dosages of vitamin E, thiamine, anti-GAD, protein electrophoresis, cryoglobulins, and CSF analysis were normal. Nerve conduction studies were unavailable. In order to exclude occult neoplasm, mammography, thyroid and endovaginal ultrasound, and CT scans of the chest, abdomen and pelvis were unremarkable. Antineuronal antibodies were not available. Discussion: This patient met the criteria for Primary Sjögren's syndrome (PSS) according to the EULAR-2016. PSS is an autoimmune inflammatory disease characterized by lymphocytic infiltration of the exocrine glands. Up to 70% of patients may have neurological manifestations, predominating the involvement of the peripheral nervous system. Cerebellar ataxia is rarely described, especially in the absence of cerebellar atrophy. In a series of cases that included 14 patients and sought to characterize ataxia in patients with PSS, only one 71-year-old woman with dysmetria and associated sensory ataxia did not have cerebellar atrophy. Other study reported a 56-year-old woman with PSS, cerebellar ataxia and limbic encephalitis without MRI alterations. Immunohistochemistry for antineuronal antibodies revealed cerebellar Purkinje cell staining. Final comments: This is the third case of PSS with cerebellar ataxia without cerebellar atrophy described in the literature. Antibodies against Purkinje cells may be involved in the pathogenesis of the condition. It is worth mentioning that due to the latency between the appearance of neurological symptoms and the detection of cancer, a paraneoplastic syndrome in similar cases remains possible, although extensive screening has been carried out.

Palavras Chave

Cerebellar ataxia; primary Sjögren's syndrome; sicca syndrome

Área

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