Idiopathic hypertrophic pachymeningitis

    Dumont AS, Clark AWm Sevick RJ, Myles ST. Idiopathic hypertrophic pachymeningitis:  A report of two cases and review of the literature.   Background-- Authors note entity was described by Charcot and Joffroy, and that there are three forms:  spinal, intracranial and craniospinal (latter is rarer).    Past cases were often attributed to specific etiologies but most recent cases are idiopathic after investigation.  Authors case 1 underwent 2 surgeries for biopsy/decompression , had persistent pain and numbness, but was non progressive for 15 years after one early relapse.  The second one received steroids after biopsy with resolution of symptoms and MRI changes.  Authors argue based on above that the condition is not autoimmune.    Literature review suggested a worse prognosis for patients with "inflammatory signs" (fever, high sed rate, CRP, or elevated WBC).    Associations include infections: syphilis, TB, HTLV-1, fungi; may be presenting sign of adjacent ear or sinus infection.  Autoimmune associations include RA, orbital pseudotumor, multifocal fibrosclerosis, MCTD, Wegener's granulomatosis.    The 23 reports run the gamut from marked to slight improvement to deterioration and late recurrence or surgical death.    For interest, Charcot's clinical descriptors divided the spinal form into distinct stages: first intermittent r...
Source: neurologyminutiae - Category: Neurology Source Type: blogs