Lai-fung Li1, Philip Yat-hang Tse1, Frederick Chun-pong Tsang1, Regina Cheuk-lam Lo2, Wai-man Lui1, Gilberto Ka-kit Leung3. 1. Division of Neurosurgery, Department of Surgery, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong. 2. Department of Pathology, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong. 3. Division of Neurosurgery, Department of Surgery, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong. Electronic address: gilberto@hkucc.hku.hk.
Abstract
BACKGROUND: Hypertrophic pachymeningitis has been described as a manifestation of a number of conditions, like infection and neoplasms such as dural carcinomatosis or lymphomas. IgG4-related hypertrophic pachymeningitis is a new entity identified during the past decade and most reports described pachymeningeal involvement only. CASE DESCRIPTION: We present a 58-year-old woman with isolated IgG4 hypertrophic pachymeningitis at the falx cerebrii that exhibited cerebral parenchymal invasion. She initially presented with left lower limb weakness. Magnetic resonance imaging of brain showed a contrast enhancing lesion located at the falx and that eroded into the right frontal lobe. Subsequent excisional biopsy confirmed the lesion with brain parenchyma invasion. The pathologic diagnosis was IgG4-related disease. She had no evidence of extracranial involvement. CONCLUSIONS: Overall, this is an uncommon condition that may exhibit parenchymal invasion. Surgical biopsy would serve to establish a definitive diagnosis, and prompt comprehensive management of what is essentially a systemic and treatable condition.
BACKGROUND:Hypertrophic pachymeningitis has been described as a manifestation of a number of conditions, like infection and neoplasms such as dural carcinomatosis or lymphomas. IgG4-related hypertrophic pachymeningitis is a new entity identified during the past decade and most reports described pachymeningeal involvement only. CASE DESCRIPTION: We present a 58-year-old woman with isolated IgG4 hypertrophic pachymeningitis at the falx cerebrii that exhibited cerebral parenchymal invasion. She initially presented with left lower limb weakness. Magnetic resonance imaging of brain showed a contrast enhancing lesion located at the falx and that eroded into the right frontal lobe. Subsequent excisional biopsy confirmed the lesion with brain parenchyma invasion. The pathologic diagnosis was IgG4-related disease. She had no evidence of extracranial involvement. CONCLUSIONS: Overall, this is an uncommon condition that may exhibit parenchymal invasion. Surgical biopsy would serve to establish a definitive diagnosis, and prompt comprehensive management of what is essentially a systemic and treatable condition.