Clinical Analysis Of Diagnosis And Therapy With Hypertrophic Cranial Pachymeningitis

Objective:Combined with relevant literature to explore hypertrophic cranial pachymeningitis (HCP) epidemiology, pathogenesis, etiology and classification, clinical manifestations, cerebrospinal fluid features, blood biochemical features, imaging features, pathological features, diagnosis and differential diagnosis, treatment and prognosis, etc. the purpose of this paper is to improve the acquaintance of hypertrophic cranial pachymeningitis, to decrease the rate of missed diagnosis and misdiagnosis and improve clinical outcomes.Methods:The clinical data of 6 patients with HCP admitted in the Third People’s Hospital of Chengdu from January 2011 to December 2014 were analyzed retrospectively. Systematically review the relevant literature and discuss the epidemiology, pathogenesis, etiology and classification, clinical manifestations, cerebrospinal fluid features, blood biochemical features, imaging features, pathological features, diagnosis and differential diagnosis, treatment and prognosis, etc.Results:It has a chronic course in all of 6 patients, the first symptom of all the patients was headache, headache usually associated with dura hypertrophy parts and presented persistent pain or needle-like pain.3 cases (50%) of them presented cranial nerve paralysis,1 case of them presented spinal dura mater impairment, it manifested decreased vision, diplopia, hearing loss, dysarthria, Central facial paralysis, limb paralysis, etc; Cerebrospinal fluid examination:5 cases (83.3%) of them showed cerebrospinal fluid protein increased, amplitude:(503.2-1741.9) mg/L and only one of them was more than 1000mg/L,1 case of them showed cerebrospinal fluid white blood cell was 18x10^6/L,1 case of them showed cerebrospinal fluid immunoglobulin G was 44.1mg/L; Blood examination: 2 cases (33.3%) of them showed the percent of neutrophile granulocyte increased, it was the augument of C-reactin protein, erythrocyte sedimentation rate, rheumatoid factors, antistreptolysin o in 1 patient respectively. The enhanced magnetic resonance imaging(MRI) revealed localized or diffuse dural enhancement,3 cases showed diffuse dural enhancement, the other revealed localized dural enhancement,1 case of them also revealed enhancement in thoracic segments of spinal dura mater; 3 patients (50%) were misdiagnosed early; Cortieostemid therapy improved the condition in 3 cases.Conclusions:Hypertrophic cranial pachymeningitis is a rare clinical disease, mostly case reports literature-based, clinical manifestation, the lack of specific symptoms, esay to misdiagnosis. the disease is more common in middle-aged to elderly men, HCP typically causes chronic headache and paralysis of multiple cranial nerves, other manifestations of ataxia, epilepsy, limb paralysis, etc. The pathogenesis and etiology remains unclear, it includes secondary hypertrophic cranial pachymeningitis(SHCP) and idiopathic hypertrophic cranial pachymeningitis(IHCP), SHCP usually associate with infection, surgery and trauma, IHCP usually associate with autoimmunity. Laboratory tests are nonspecific, increased CSF protein and white blood cell, increased the percent of neutrophile granulocyte, increased C-reactin protein, increased erythrocyte sedimentation rate, increased rheumatoid factors, increased perinuclear anti-neutrophil cytoplasmic antibodies are more common. The enhanced magnetic resonance imaging(MRI) shows characteristic manifestations with localized or diffuse dural enhancement, the middle-aged to elderly men who with chronic headache and paralysis of multiple cranial nerves is necessary to make a enhanced MRI. Pathological examination is the gold standard for diagnosis, it shows a visible dural fibrous hyperplasia, and hyaline degeneration or caseous necrosis, also visible lymphocytes, neutrophils, plasma cells, fibroblast chronic inflammatory cell infiltration. In addition, pathological examination may find fungi, bacteria, tuberculum bacillus, pseudomonas aeruginosa, virus, and provide the evidence for the etiological diagnosis. SHCP should actively control the primary disease, IHCP should choose corticosteroid therapy first, it may combine with immunosuppressant therapy, radiotherapy or surgery to refractory HCP. Most patients receive relieved after treatment with drugs or surgery, few patients spontaneously relieved without any treatment, a very small number of patients were exacerbated, ultimately death.