RESUMO
BACKGROUND: The frequency and type of central nervous system involvement in primary Sjögren's syndrome (pSS) remain controversial. Brain magnetic resonance imaging (MRI) abnormalities in pSS are usually discrete hyperintense areas in the white matter. Tumefactive brain lesions have been rarely reported. CASE REPORT: We describe a 31-year-old woman who exhibited transcortical motor aphasia, hemiparesis and partial motor seizures as the initial manifestation of pSS. Brain MRI revealed a large frontoparietal lesion extending into the corpus callosum. The patient had spontaneous recovery and developed sicca symptoms 6 months after onset. Primary SS was diagnosed on the basis of clinical features, abnormal Schirmer test findings, high levels of anti-La/SSB antibodies and positive salivary gland biopsy results. CONCLUSION: The present case suggests that a pseudotumoral brain lesion can occur as an initial symptom of pSS.
Assuntos
Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/diagnóstico , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Adulto , Diagnóstico Diferencial , Feminino , HumanosRESUMO
Microscopic polyangiitis is a systemic necrotizing vasculitis of the small vessels. Its typical clinical manifestations are rapidly progressive glomerulonephritis and alveolar hemorrhage. We describe a 30-year-old woman with rapidly progressive glomerulonephritis. Seven years later, she presented with partial loss of motor and sensory function in both lower limbs with sphincter dysfunction. This is the first reported case of epidural inflammation ascribed to microscopic polyangiitis. Possible mechanisms include auto-immune disease.
Assuntos
Glomerulonefrite/etiologia , Extremidade Inferior/inervação , Poliangiite Microscópica/complicações , Doenças do Sistema Nervoso Periférico/etiologia , Corticosteroides/uso terapêutico , Adulto , Artralgia/etiologia , Artralgia/fisiopatologia , Feminino , Glomerulonefrite/tratamento farmacológico , Glomerulonefrite/fisiopatologia , Humanos , Imunossupressores/uso terapêutico , Dor Lombar/etiologia , Dor Lombar/fisiopatologia , Imageamento por Ressonância Magnética , Poliangiite Microscópica/tratamento farmacológico , Atividade Motora , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/tratamento farmacológico , Doenças do Sistema Nervoso Periférico/fisiopatologia , Gravidez , Sensação , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Incontinência Urinária/etiologia , Incontinência Urinária/fisiopatologiaRESUMO
INTRODUCTION: Langerhans cell histiocytosis is a rare entity. Involvement of the pituitary region is frequently delayed and rarely studied. CASES: We report some clinical and paraclinical particularities of the disease in four women. Diabetes insipidus was the initial symptom in all cases, accompanied by panhypopituitarism at initial presentation in one woman. Hypopituitarism was diagnosed from 6 months to 4 years after the diabetes insipidus in the other cases, and systemic lesions appeared 3 to 6 years thereafter. Magnetic resonance imaging of the hypothalamic-pituitary axis found tumors in two patients and infundibular thickening in the other two. One patient also had a partially empty sella. DISCUSSION: In adults, pituitary involvement seems to occur predominantly in women, and panhypopituitarism is possible at initial presentation. The clinical polymorphism of Langerhans cell histiocytosis makes this disease difficult and often slow to diagnose.
