From: Philip de Sterke []
Sent: Tuesday, February 20, 2001 7:17 AM
Subject: FHHF For the eye and brain experts!

      From Barton 2000, p. 211

      Central nervous system and eye

      In the brain, epithelial cells of the choroid plexus almost invariably contain striking hemosiderin deposits (127,146-148). Cells  of area postrema of the medulla oblongata and of the olfactory bulbs usually contain stainable iron also146-148. Extracellular iron deposits are sometimes found scattered troughout the leptomeninges and extracellularly within the pia-arachnoid, but ependymal cells lining the ventricular system, neurons, and neuroglial cells do not appear to contain hemosiderin (148). Stain intensification methods such as diaminobenzidine black are useful to study the small amounts of normal iron in the brains of animals (7, 8), but there appear to be no publisbed studies of the application of such techniques to the analysis of iron deposition in the brain in hemochromatosis. This leaves open the possibility that iron deposition in brain cells is greater or more widespread in hemochromatosis than is presently appreciated. Reports of iron deposition in the spinal cord, peripheral nerves, ganglia, and other peripheral nervous system structures appear to be lacking.

      In the eye, traces of ferric iron can be visualized in the corneal epithelium, sclera, nonpigmented ciliary epithelium, iris pigment epithelium, peripapillary pigment epithelium, and fibroblasts; some iron is extracellular. These correspond to the rust-colored pigmentation of the bulbar conjunctivae (most marked at the inferior limbus and extending onto the cornea), eyelid borders, insertions of the recti muscle, sclerae, and peripapillary regions observed in a minority of patients (149-150).

      146 Sheldon JH. The iron content of the tissues in haemochromatosis with special reference to the brain. Q J Med 1927;21:123-37.

      147 McDougal DB, Adams RD. The neuropathologica1 changes in hemochromatosis. J Neuropath Exp NeuroI1950;9:117-24.

      148 Lewis I, Darja M. The central nervous system in hemochromatosis. Neurology 1963;13:500-4.

      149 Davies G, Dymock L, Harry J, Wllliams R. Deposition of melanin and iron in ocular structures in haemochromatosis. Br J OphthalmoI1972;56:338-42.

      150 Roth AM, Foos RY. Ocular pathologic changes in primary hemochromatosis. Arch OphthalmoI1972;87:507-14.


Philip de Sterke
Hemochromatose Vereniging Nederland

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