From: Philip de Sterke [p.sterke@chello.nl] Sent:
Tuesday, February 20, 2001 7:17 AM To: excessiron@mail-list.com;
FHHF@yahoogroups.com 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.
American
Hemochromatosis Society www.americanhs.org "AHS.....Prevention Through
Genetic Testing...."
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