Idiopathic Hypertrophic Spinal Pachymeningitis Case Report And Review Of The Literature

Idiopathic hypertrophic spinal pachymeningitis(IHSP)is a rare inflammatory disorder that leads to spinal cord compression which is characterized by acute onset,rapid progress,easy misdiagnosis.To increase awareness of this condition,reduce misdiagnosis rate and improve the therapeutic effect,we report the details of a case here.A 48-year-old man revealed a half-month history of progression shoulder pain and a two-day history of muscle weakness of the lower limbs,hypoesthesia from the T2 level area down to the feet.On MRI,the spinal cord was compressed by an encircled mass,which showed hypointensity on T1-and T2-weighted images with gadolinium enhancement at the T1 to T3 vertebra.Because of the rapid progression of myelopathy,surgical decompression was performed and IHSP was finally diagnosed by immunohistochemistry in two hospitals.IHSP is a diagnosis of exclusion.When all the know etiologies such as infectious,tumor,autoimmune disorders are ruled out and typical pathological findings are displayed,this diagnosis can be established.Early surgical intervention and Steroid therapy demonstrates the positive effects and is essential to prevent irreversible damage to the nervous system.