RESUMO
There are only six cases in literature that describe development of dystonia with Sjogren's syndrome (SS). We describe a case of a 43-year-old woman who presented with symptoms including movement disorder, sensory neurogenic bladder, sensory loss and neuropathic pain, migraine like headaches, musculoskeletal pain, Raynaud's phenomenon and dysautonomia. Symptoms started in 2000, with weakness that progressed to dystonia in 2003. Diagnostic work-up was inconclusive with negative inflammatory serologies, cerebrospinal fluid and MRI for many years. After patient developed sicca syndrome with dry eyes and mouth in 2009, her rheumatoid factor titre was elevated (550 IU/mL), erythrocyte sedimentation rate, anti-Sjogrens syndrome-related antigen A (anti-Ro/SSA) and anti-SSB/La: anti-Sjogrens syndrome-related antigen B (anti-La/SSB) became positive. Lip biopsy confirmed diagnosis of SS. She was diagnosed with primary SS with neurological involvement. Her symptoms responded well to intravenous methylprednisolone. Symptoms stabilised with trials of immune-suppressive therapy. This is a case that demonstrates the delay of diagnosing SS with preceding unique neurological association.
Assuntos
Distonia/diagnóstico , Síndrome de Sjogren/diagnóstico , Adulto , Anticorpos Antinucleares/imunologia , Distonia/etiologia , Distonia/imunologia , Feminino , Humanos , Imunossupressores/uso terapêutico , Transtornos de Enxaqueca/diagnóstico , Transtornos de Enxaqueca/etiologia , Transtornos de Enxaqueca/imunologia , Neuralgia/diagnóstico , Neuralgia/etiologia , Neuralgia/imunologia , Disautonomias Primárias/diagnóstico , Disautonomias Primárias/etiologia , Disautonomias Primárias/imunologia , Doença de Raynaud/diagnóstico , Doença de Raynaud/etiologia , Doença de Raynaud/imunologia , Glândulas Salivares Menores/patologia , Transtornos de Sensação/diagnóstico , Transtornos de Sensação/etiologia , Transtornos de Sensação/imunologia , Síndrome de Sjogren/complicações , Síndrome de Sjogren/tratamento farmacológico , Síndrome de Sjogren/imunologia , Síncope/diagnóstico , Síncope/etiologia , Síncope/imunologia , Bexiga Urinaria Neurogênica/diagnóstico , Bexiga Urinaria Neurogênica/etiologia , Bexiga Urinaria Neurogênica/imunologiaRESUMO
We report on 26 patients with a distinct phenotype of complex regional pain syndrome that progressed toward a multifocal or generalized tonic dystonia. The dystonia initiated distally, involved mainly flexor muscles, and was associated with sensory and autonomic symptoms. Dryness of the eyes or mouth and bladder and bowel disturbances were frequently reported. There was no increase in the familial prevalence of autoimmune-mediated diseases. Compared with controls, a significant elevation of HLA-DR13 was found in the patients. Thus, HLA-DR13 may be a factor indicating susceptibility to this distinct phenotype of complex regional pain syndrome.