In severe carpal tunnel syndrome a continuum of neural changes takes place depending on the degree and duration of the compression, beginning with breakdown of the blood-nerve barrier, followed by endoneurial oedema and, subsequently, perineurial thickening and ischemia. Persisting chronic compression will eventually result in axonal degeneration. We report a case of longstanding carpal tunnel syndrome with amyloid deposits and the unusual intraoperative 'Austrian flag' sign.
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