What are the neurologic complications of hemochromatosis?
Hemochromatosis is a disorder of iron overload resulting in multiorgan fibrosis and dysfunction.
Acquired causes result from excess total body iron due to multiple blood transfusions.
Hereditary hemochromatosis is due to mutations in the HFE gene, which encodes for a protein involved in the regulation of GI iron absorption and uptake.
Encephalopathy, truncal ataxia, and rigidity may all complicate hemochromatosis and invariably are due to liver disease (liver cirrhosis and failure) resulting from massive iron deposition in the liver.
Neuritis is either a complication of the diabetes mellitus (DM) that accompanies most cases of hemochromatosis or is a result of local iron deposition.
Treatment requires serial phlebotomies four to six times per year.
Lifetime treatment with phlebotomies is currently the treatment of choice, although newer therapies using growth factor control over red blood cell production are being tested.
