Idiopathic hypertrophic pachymeningitis

Abstract

Idiopathic hypertrophic pachymeningitis is a rare disorder of unknown origin. It is a fibrosing inflammatory process that involves the dura mater. Herein are described 14 patients with idiopathic hypertrophic pachymeningitis; their clinical, laboratory and radiological findings, as well as their treatment, are analyzed. Neuropathological findings of six cases including two autopsied cases are also presented. The main clinical features were headache and cranial nerve palsies. Many patients had mild to moderate elevation of C-reactive protein, and three patients had perinuclear antineutrophil cytoplasmic antibody. The CSF in most cases showed inflammatory changes. Neuroimaging studies revealed diffuse or localized thickening of the dura, and MRI findings were key to diagnosis of this disorder. The clinical course was chronic. All patients were treated with corticosteroid and improved intially, but half of them experienced relapses. Two patients received surgical intervention. Pathological examination in two autopsied cases revealed diffuse thickening of the dura, especially in the posterior part of the falx cerebri and the tentorium cerebelli. Microscopic examination of the dura showed dense fibrosis with inflammatory cell infiltration composed mainly of lymphocytes. The cell infiltration was marked at the surface of the dura mater. One case had necrotizing vasculitis of the small arteries located in the dura and the cerebral surface. There were no giant cells, caseation necrosis, or epitheloid granuloma. Four patients underwent biopsy of the dura, and the pathological study showed non-specific inflammatory changes. The relationship of idiopathic hypertrophic pachymeningitis with connective tissue disease or vasculitis syndrome is discussed.

Idiopathic hypertrophic pachymeningitis mimicking prolactinoma with recurrent vision loss

“…Demographically, the median age of patients with idiopathic hypertrophic pachymeningitis is 58.3 years (standard deviation, 15.8; range, 37-88 years). [4][5][6] Only a few paediatric patients have been reported, with the youngest age being 2 years and 11 months in India. 7 Our patient had early-onset disease.…”

“…Multiple cranial nerve involvement, ataxia, cortical blindness, psychosis, motor function disturbance, fever, convulsion, and/or loss of consciousness have all been reported. 5,6 Yamada et al 2 thought that the inflammatory thickening of the dura may cause damage to the superior hypophyseal artery resulting in subarachnoid haemorrhage and apoplexy in the anterior lobe of the pituitary gland.…”

Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis

“…Idiopathic hypertrophic pachymeningitis is a chronic, progressive, diffuse, inflammatory fibrosis of the dura mater [13][14][15]22]. Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare inflammatory disorder that leads to spinal cord compression.…”

Paquimeningitis craneal hipertrófica idiopática.

“…La paquimeningitis hipertrófica idiopática es un proceso inflamatorio crónico fibrosante de etiología desconocida infrecuente que causa engrosamiento de la duramadre y que produce déficit neurológico progresivo debido a la compresión de las estructuras adyacentes (1). De acuerdo con la localización anatómica y en orden de frecuencia, los casos de paquimeningitis hipertrófica se pueden subdividir en espinal, craneal y craneoespinal (2).…”

“…La paquimeningitis hiper trófica ha sido frecuentemente atribuida a etiologías específicas como la tuberculosis y la sífilis (3,4). Sin embargo, los casos más recientes parecen ser idiopáticos ya que una intensa búsqueda para definir la etiología fue negativa (1,(5)(6)(7).…”