IgG4: related disease presenting with headache and papilloedema

• Autor(a) principal: Lourenço, Eva Patrícia
• Data de Publicação: 2016
• Outros Autores: Nzwalo, Hipólito, Sampaio, Mário Rui, Afonso, Luís
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: http://hdl.handle.net/10400.1/9168

A previously healthy man aged 57years presented with a 4-week duration of predominant decubitus daily holocranial headache after minor head injury. Apart from unintentional weight loss of 8 pounds, there were no associated constitutional or systemic manifestations.The generalandneurologicalexamination was unremarkable. The funduscopy showed bilateral grade 2 papilloedema. Brain magnetic resonance (MRI) findings are shown in figure 1A–E. The brain MRI showed the presence of diffuse dural thickening (figure 1B–E) hypointense on unenhanced T1 imaging (figure 1B–F), with dural enhancement on T1-weighed imaging (figure 1B–F). The opening cerebrospinal fluid (CSF) pressure was 380mm Hg. With the exception of serum-elevated IgG4 immunoglobulin (IgG4=302 mg/dL) and elevated CSF protein level (68 mg/dL), the wide and extensive complementary study including body positron emission tomography scan, laboratory blood/CSF work-up was negative, excluding neoplastic, infectious, autoimmune and collagen vascular disorders. Meningeal biopsy showed fibrous thickening with moderate and predominant B and T cells inflammatory infiltrate. The patient improved with spinal taps evacuation and methylprednisolone treatment. Intracranial hypertrophic pachymeningitis is a severe and rare manifestation in the spectrum of IgG4-related diseases.12Biopsy of meninges is the gold standard for the diagnosis.3 Exclusion of systemic inflammatory disorders and demonstration elevated serum IgG4 can support the diagnosis in patients with typical diffuse dural thickening.13The disease respondsto immunosuppressive drugs such as corticosteroids, methotrexate, azathioprine, mycophenolate or cyclophosphamide.12Mass effect due to dural thickening can cause progressive neurological debilitation,blindness and cerebellarataxia.In the case of symptomatic hydrocephalus, surgical exploration for decompression maybe required