Hypertrophic Cranial Pachymeningitis With E-B Virus Positive Of Cerebrospinal Fluid:a Case Report And Literature Review

Objection Combined with relevant literature to explore hypertrophic cranial pachymeningitis (HCP) epidemiology, etiology and classification, clinical manifestations, laboratory features, imaging features, pathological features, diagnosis and differential diagnosis, treatment and prognosis, etc.. Hypertrophic pachymeningitis clinical rare disease, a rare disease, the purpose of this paper is to improve the level of knowledge about the disease clinicians, systematic understanding of the relevant characteristics of the disease and reduce the rate of misdiagnosis and missed diagnosis rate and improve clinical outcomes.Methods We reported the clinical data of one case of HCP, retrospective analysis of the pathogenesis, clinical manifestations, imaging features, treatments, disease outcome of the relevant circumstances. And systematically review the relevant literature, the epidemiological characteristics of HCP, etiology and classification, clinical manifestations, laboratory features, imaging features, pathological features, diagnosis and differential diagnosis, treatment and prognosis, etc. systematic discussion.Results In this case the patient complained of "no obvious incentive to six years paroxysmal headache, diplopia January admission. Patients began six years ago no obvious incentive to intermittent headaches, intermittent time from several months to several years, and each episode was persistent pain after occipital, top heavy, there are several times during the " grand mal seizures." patients in January before again no obvious incentive headache, diplopia, swallowing, poor, without the onset of epileptic seizures. Over the right eye left eye examination weak (right eye1.0, left0.6); left positive direction depending on the material before diplopia right direction depending on the material without diplopia; on the right eye fissure facial skin feel a little less dull; gag reflex slow. Laboratory test results:ESR:33mm/h (2-20), cerebrospinal fluid white blood cell count27×106/L (0-8), Pan’s experiment (+), cerebrospinal fluid protein0.74g/L (0.00-0.40), cerebrospinal fluid virus-DNA: 3140IU/ml (>50positive), C-reactive protein12.60mg/L (0.00-7.00). Brain CT showed tentorium area and the longitudinal high signal, brain MRI shows abnormal proliferation and strengthen local dura after neurotrophic drugs to control the disease.Conclutions Hypertrophic pachymeningitis clinical rare disease, mostly case reports literature-based, clinical manifestation, the lack of specific symptoms, easy to misdiagnosis. The disease is more common in men, at the age of40-60years old. Their etiology of:idiopathic HCP (idiopathic HCP, IHCP) and secondary HCP (secondary HCP, SHCP). Often the first symptom is headache, other manifestations of brain damage symptoms, ataxia, intracranial arterial occlusion, venous sinus thrombosis, hydrocephalus, epilepsy, mental symptoms. Laboratory tests increased white blood cell count and percentage, erythrocyte sedimentation rate (ESR) increased slightly faster, myeloperoxidase neutrophil cytoplasmic antibody (MPO-ANCA)-positive rheumatoid factor (RF) positive, C-reactive protein (CRP) increased slightly more common. CT scan may show hypertrophy of the dura high density, MRI scan shows diffuse thickening of the dura mater, thickening of the dura in T1WI showed equal or low signal, T2WI showed low signal, which can be seen around the high signal intensity. Pathological examination showed a visible dural fibrous hyperplasia, and hyaline degeneration, also visible neutrophils, lymphocytes, plasma cells and other inflammatory cell infiltration. In addition, granuloma seen by the multinucleated giant cells, fibroblasts, epithelial cells formed. Pathological examination may also find specific substances and specific structures associated metabolic activity of bacteria, viruses, fungi, etc. produced. The incidence of the disease in some patients can be presented-ease-onset-and then ease the law, but the overall trend of the disease often has increased, in most cases after medical or surgical treatment may be in remission, and the follow-up displays can often last longer, but still clinical intervention ineffective in some cases, the condition progressive increase in a short time, and even death.