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.
Objectives-To assess the extent of loss of myelinated nerve fibres and spinal motor neuron loss in chronic inflammatory demyelinating polyneuropathy (CIDP), a clinicopathological study was conducted on biopsied sural nerves and necropsied spinal cords from patients with CIDP. Methods-The myelinated fibre pathology of 71 biopsied sural nerves and motor neuron pathology of nine necropsied spinal cords at L4 levels in patients with CIDP were quantitatively and immunohistochemically assessed. The pathological hallmark of chronic inflammatory demyelinating polyneuropathy (CIDP) is segmental demyelination and mononuclear inflammatory cell infiltration in the peripheral nerves, accompanied by varying degrees of axonal degeneration, myelinated fibre loss, and endoneurial oedema. Results-Myelinated1 Regarding the pathology of the CNS in CIDP, central chromatolysis in spinal motor neurons has often been reported, [2][3][4][5][6] and sporadic cases with a slight loss of spinal motor neurons have occasionally been documented.3 6-9 Spinal motor neuron loss as well as nerve myelinated fibre loss could be important factors influencing functional recovery. In this study, we assessed the degree of involvement of spinal motor neurons and peripheral nerve axons in CIDP. Methods SPECIMENSAfter informed consent was given, sural nerve biopsy specimens from 71 patients with CIDP (50 males and 21 females) were obtained at the Nagoya University School of Medicine and its aYliated hospitals over 11 years. Age at biopsy ranged from 2 to 81 years; mean (SD) age 48.5 (21.9) years. The duration of illness before biopsy ranged from 2 months to 28 years; mean (SD) 2.9 (5.8) years. The spinal cords were obtained at necropsy from nine patients with CIDP. Three of these patients were necropsied at the Nagoya University Hospital and aYliated hospitals, and others were necropsied in hospitals located throughout Japan during the past 11 years. These patients consisted of six men and three women, aged 49 to 73 years; mean (SD) 62.4 (8.9) years. Their duration of illness ranged from 4 months to 8 years. Clinical profiles of necropsy cases are summarised in table 1. 10The diagnosis of CIDP in our study was assessed using the criteria of Barohn et al 11 or the ad hoc subcommittee of the American Academy of Neurology. 12ASSESSMENT OF SURAL NERVE BIOPSY Sural nerve biopsy specimens were fixed in glutaraldehyde in 0.025 M cacodylate buVer (pH 7.4) and embedded in epoxy resin. Semithin sections were stained with toluidine blue, and the density of myelinated fibres was analysed quantitatively using a computer assisted imaging system (Luzex FS, Nireco, Tokyo, Japan). The extent of subperineurial oedema was assessed as an increase in the subperineurial space by comparing the subperineurial area to the total endoneurial area using the same imaging system. A part of the nerve specimen was processed for teased fibre analysis, and the condition of each fibre was assessed according to our previously indicated criteria. 13A portion of the nerve specimen was ...
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