Clinical analysis of hypertrophic cranial pachymeningitis associated with anti- myeloperoxidase and anti-neutrophil cytoplasmic antibody with optic nerve dysfunction / 中华神经科杂志

ABSTRACT

Objective:To investigate the clinical characteristics, diagnosis, treatment and prognosis of patients with hypertrophic cranial pachymeningitis （HCP） associated with myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) with optic nerve dysfunction.Methods:Clinical data of patients with MPO-ANCA associated HCP admitted to Beijing Tongren Hospital, Capital Medical University from 2008 to 2019 were collected and analyzed.Results:A total of 55 HCP patients were diagnosed, including five patients with MPO-ANCA associated HCP, two males and three females, with the onset ages ranging from 61 to 80 years.The main clinical manifestations were successively five cases of multiple cranial neuropathies, four cases of headache, four cases of chronic sinusitis, three cases of otitis media or mastoiditis，one case of cerebral infarction, while two cases of mild kidney involvement, all without lower airways involvement. Peripheral blood MPO-ANCA concentration and erythrocyte sedimentation rate were elevated in all patients, and protease 3-ANCA was negative. Cerebrospinal fluid protein content was elevated in three patients and leukocyte was increased in one patient. The abnormal frequency of gadolinium-enhanced MRI of brain scan was dura mater (five cases), optic nerve sheath (five cases), sinus mucosa (four cases), inner auditory tract meninges (three cases), orbital wall periosteum (two cases), and brain parenchyma (one case). All patients received glucocorticoid therapy, meanwhile two patients received cyclophosphamide treatment. Multiple cranial neuropathies and headache were improved，but the improvement of visual function damage was not obvious.Conclusions:MPO-ANCA associated HCP is a special type of HCP. Among the dural region involved, the optic nerve sheath is a common site of this disease, and the sinus mucosa and inner auditory canal meninges are also frequently involved. MPO-ANCA associated HCP should be distinguished, if patients with dural lesions, especially those accompanied by optic nerve sheath lesions, sinusitis, and middle ear mastoiditis.