以下内容来源于 NEJM 。

A 50-year-old man presented with a 1-year history of progressive headaches and nausea. On physical examination, neck stiffness and dysmetria in both arms on finger-to-nose testing were noted. Results of a lumbar puncture revealed an opening pressure of 31 cm of water (reference range, 7 to 20), and cerebrospinal fluid studies were notable for elevations in the protein level and immunoglobulin index. Subsequent contrast-enhanced, T1-phase magnetic resonance imaging (MRI) of the head showed thickened dura with central hypointensity and peripheral enhancement at the posterior falx and cerebellar tentorium — a finding known as Eiffel-by-night sign (coronal view). Eiffel-by-night sign is thought to represent active inflammation of chronic pachymeningitis and may be seen in the context of inflammatory, autoimmune, neoplastic, or vascular conditions. A dural biopsy showed a lymphoplasmacytic infiltrate with IgG4-positive plasma cells. The serum IgG4 level was elevated. Whole-body imaging showed no other organ involvement. A diagnosis of IgG4-related hypertrophic pachymeningitis was made. Treatment was initiated with methylprednisolone pulse therapy for 5 days and was then transitioned to a slowly tapering dose of oral glucocorticoids. At a 2-month follow-up visit, a repeat MRI of the head showed improvement, and at 6 months, the patient’s headache had abated. A maintenance dose of a glucocorticoid was continued.