E-Pearl of the Week: Neurologic symptoms in relapsing polychondritis

April 9, 2013

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April 9, 2013

Neurologic symptoms in relapsing polychondritis 

Relapsing polychondritis is a rheumatologic condition characterized by inflammation and deterioration of cartilage. Relapsing polychondritis most frequently presents in the 40s to 50s, though children can be affected less commonly.  The condition can affect any cartilage in the body, but often targets the ears, nose, throat, heart valves, joints, rib cage, and sinus cavities.  There have been numerous reports of neurologic involvement in relapsing polychondritis, as high as ten percent of cases in one report (1).  Cranial nerve disorders are common manifestations (2).  Hemiplegia, cerebellar signs, seizures, and headache have also been described, and it has been proposed that an underlying vasculitis may be the cause of the neurologic disorders, but there have been few neuropathologic studies(3).

References

  1. Zeuner M, Straub RH, Rauh G, et.al. Relapsing polychondritis: clinical and immunogenetic analysis of 62 patients. J Rheumatol 1997; 24:96-101.
  2. Sundaram MBM and Rajput AH. Nervous system complications of relapsing polychondritis. Neurology 1983;33: 513.
  3. Stewart SS, Ashizawa T, Dudley AW, et.al. Cerebral vasculitis in relapsing polychondritis. Neurology 1988; 38: 150.

Submitted by Stacey Clardy, M.D. Ph.D. Autoimmune Fellow, Mayo Clinic. 

Disclosure:  Dr. Clardy is a former member of the Resident and Fellow Section of Neurology.

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