XXXX : clinicopathological characteristics and a potential therapy.

@entity1 @entity32 @entity82 @entity5 @entity6437 @entity425 @entity4216 @entity158 @entity230 @entity393 @entity528 @entity9
We sought to determine the clinical, electrophysiological, neuroimaging, and pathological features of @entity6437 . Five @entity1 were identified. All cases presented with a gradually progressive @entity425 , @entity393 , and prominent @entity9 . The median duration of symptoms was 7 months (range 3-36 months). Electrophysiological results were in keeping with chronic @entity4216 with variable findings of active denervation and reinnervation. MRI demonstrated irregular, large masses involving and surrounding nerve with heterogenous signal characteristics on T1- and T2-weighted and post-contrast sequences. Histopathological features of the nerve slightly varied but shared commonalities including chronic inflammatory infiltrates, increased collagen, and increased numbers of microvessels. @entity528 and decreased density of myelinated fibers were also noted. Three @entity1 were treated with weekly courses of intravenous @entity230 for 3 months. All reported improvement in @entity158 and @entity425 . @entity6437 is not a @entity5 and has reactive features of @entity32 , increased vascularity, and marked @entity82 . It presents as a progressive @entity4216 with @entity425 , @entity393 , and @entity158 that may be episodic. The primary pathophysiology is unknown but the @entity32 and response to treatment suggests that there may be an immune component.
