Sjögren’s syndrome and subacute demyelinating polyradiculopathy: an unusual association
Introduction. Sjögren’s syndrome is a chronic inflammatory condition of unknown aetiology and autoimmune pathology. The defining feature is the dry syndrome, expressed as xerophthalmia and xerostomia. Extra-glandular involvement at many other levels may also occur. Neurological involvement is not unusual. The peripheral nervous system is most frequently involved, and a predominantly sensitive symmetrical distal polyneuropathy may be the first sign of the condition. Other patterns of peripheral involvement are also associated with the syndrome. We present a case of subacute demyelinating polyradiculopathy associated with primary Sjögren’s syndrome. Clinical case. A 28 year old woman with dry syndrome presented with paraesthesia in her hands and feet, distal weakness, which had progressed proximally in the muscles of her arms and legs, and bilateral facial weakness. The condition progressed for eight weeks. When complementary tests were done, alterations typical of this condition (FR, ANA, anti-Ro and anti-La) were seen and also others typical of the dry syndrome (Schirmer’s test). Therefore, in view of these findings and the clinical features, after other conditions had been ruled out, a diagnosis of primary Sjögren’s syndrome was made. The type of neuropathy was determined by the clinical features, electromyography and CSF findings. Treatment with corticosteroids gave good results. Conclusions. Demyelinating polyradiculopathy is a form of peripheral nervous system involvement which is rarely seen in this disorder. In the differential diagnosis Sjögren’s syndrome should be considered, an orientative history taken, autoantibodies determined and an ophthalmological examination made
Caso clínico Mujer de 28 años, con un síndrome seco, que presentó cuadro de parestesias en manos y pies, así como debilidad distal que fue ascendiendo hasta musculatura proximal de brazos y piernas, con afectación facial bilateral. El cuadro progresó durante ocho semanas. Entre las pruebas complementarias, se detectaron alteraciones analíticas típicas de esta enfermedad (FR, ANA, anti-Ro y anti-La), así como otras que objetivaron el síndrome seco (test de Schirmer), lo que junto a la clínica, y una vez descartadas otras enfermedades, establecieron el diagnóstico de síndrome de Sjögren primario. El patrón clínico, electromiográfico y de LCR determinaron el tipo de neuropatía. Hubo una buena respuesta a los corticosteroides.
Conclusiones La polirradiculopatía desmielinizante es una forma de afectación del sistema nervioso periférico rara en esta enfermedad. En su diagnóstico diferencial se debe contemplar el síndrome de Sjögren, incluyendo anamnesis dirigida, determinación de autoanticuerpos y examen oftalmológico
