Hypertrophic Cranial Pachymeningitis: A Case Report And A Review Of The Literature

ObjectionThe aim is to investigate the clinical manifestations, laboratory features, imaging and pathological features of hypertrophic cranial pachymeningitis (HCP) and to improve the acquaintance of this rare disease, to decrease the rate of misdiagnosis and missed diagnosis.MethodsWe retrospectively analyzed the clinical data of one case which is pathologically confirmed and reviewed foreign and domestic literatures. And gave a Systematic exposition of the etiology, pathogenesis, clinical manifestations, laboratory features, imaging and pathological features, diagnosis and treatment of HCP.ResultsThe clinical manifestations of this case were chronic headache, cerebellar ataxia, etc. Headache was persistent gas pains and was not accompanied by nausea, vomiting, photophobia. And the posterior occipital and copular part were the most obvious. Ataxia manifested as standing and walking instability, less steady finger-to-nose test and heel-knee-tibia test, and Romberg sign(+).Laboratory test results:ESR:25 mm/h, slightly faster; Rheumatoid Series:Anti-ssA antibody (+), ANA 1:160 positive; PPD test (++++); cerebrospinal fluid pressure on lumbar puncture showed slightly higher; cerebrospinal fluid protein increased; cerebrospinal fluid cell count increased, mainly lymphocytes; cerebrospinal fluid immunoglobulin increased. Brain magnetic resonance imaging (MR I) gadolinium enhanced scans show:the tentorium and right posterior temporal, occipital, partial dura were significantly strengthened with more significantly enhanced tentorium and pair of maxillary, ethmoid, frontal sinus inflammation. Pathological examination showed chronic dural inflammation, more capacity lymphocytes and plasma cells infiltration, fibrous tissue hyperplasia, hyaline degeneration, non-specific granuloma formation. With empirical anti-tuberculosis treatment and corticosteroid therapy, symptoms had got significantly improvement.ConclutionsHCP is a rare clinical disease, etiology and pathogenesis remains unclear. Chronic headache and multiple cranial nerve palsy are the main clinical manifestations. Laboratory tests are nonspecific; increased ESR and CRP, elevated cerebrospinal fluid protein, increased cell count, increased intracranial pressure, etc was more common. There are specific changes in brain MRI examination. Proliferation of dural hypertrophy, T1-weighted images showed low or equal signal, T2-weighted image shows low signal, enhanced scan showed significantly enhanced lesions are typical performance. Pathological changes are chronic inflammation of the dura, the more typical non-specific granuloma formation is the main basis for diagnosis. Cortisol steroid hormone treatment is first choice. Small number of patients spontaneously relieved without any treatment, most patients receive the clinical and imaging relieves with appropriate treatment; despite the treatment, symptoms of a very small number of patients were exacerbated, ultimately death. View of hypertrophic cranial pachymeningitis is a rare clinical disease and their clinical manifestations are not specific, clinicians do not understand the disease and made more clinically misdiagnosis, which resulted in delays in patient condition. Therefore, clinicians should raise the awareness of the disease and ensure early detection, early diagnosis and early treatment.