<<snip>>
severe nerve fiber degeneration of the sciatic nerve, decreased
number of neuromuscular junctions and degeneration of skeletal muscle
fibers. Most probably, the developing demyelinating neuropathy resulted

in muscular degeneration demonstrated in the extensor digitorum longus
muscle.
<<snip>>

Am J Physiol Regul Integr Comp Physiol. 2006 May 11; [Epub ahead of
print] Related Articles, Links

Excessive erythrocytosis in adult mice overexpressing erythropoietin
leads to hepatic, renal, neuronal and muscular degeneration.

Heinicke K, Baum O, Ogunshola OO, Vogel J, Stallmach T, Wolfer DP,
Keller S, Weber K, Wagner PD, Gassmann M, Djonov V.

Vetsuisse Faculty and Zurich Center for Integrative Human Physiology
(ZIHP Institute of Veterinary Physiology, Zurich, Switzerland.

To investigate the consequences of inborn excessive erythrocytosis we
made use of our transgenic mouse line (tg6) that constitutively
overexpresses erythropoietin (Epo) in a hypoxia-independent manner,
thereby reaching hematocrit levels of up to 0.89. We detected
expression of human Epo in the brain and, to a lesser extent, in the
lung but not in the heart, kidney or liver of tg6 mice. Although no
acute cardiovascular complications are observed, tg6 animals have a
reduced lifespan. Decreased swim performance was observed in five month

old tg6 mice. At about seven months, several tg6 animals developed
spastic contractions of the hind limbs followed by paralysis.
Morphological analysis by light and electron microscopy showed
degenerative processes in liver and kidney characterized by increased
vascular permeability, chronic progressive inflammation, hemosiderin
deposition and general vasodilatation. Moreover, most of the animals
showed severe nerve fiber degeneration of the sciatic nerve, decreased
number of neuromuscular junctions and degeneration of skeletal muscle
fibers. Most probably, the developing demyelinating neuropathy resulted

in muscular degeneration demonstrated in the extensor digitorum longus
muscle. Taken together, chronically increased Epo levels inducing
excessive erythrocytosis leads to multiple organ degeneration and
reduced life expectancy. This model allows investigation of the impact
of excessive erythrocytosis in individuals suffering from polycythemia
vera, chronic mountain sickness or in subjects tempted to abuse Epo by
means of gene doping.

PMID: 16690772 [PubMed - as supplied by publisher]
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<<snip>>
indistinguishable from multiple sclerosis
<<snip>>

Childhood chronic inflammatory demyelinating polyneuropathy with
central nervous system demyelination resembling multiple sclerosis

Neuromuscul Disord 2003 Feb;13(2):158-61
Rodriguez-Casero MV, Shield LK, Coleman LT, Kornberg AJ.
Department of Neurology, Royal Children's Hospital, Flemington Road,
Parkville, Vic. 3052, Australia

Central nervous system demyelination has been described in adults but
not in children with chronic inflammatory demyelinating polyneuropathy.

We describe a patient with clinical and electrophysiological features
consistent with chronic inflammatory demyelinating polyneuropathy who
presented at age 5 with an intramedullary spinal cord tumor-like lesion
and at age 8, represented with cerebral and spinal demyelinating
lesions.

Her clinical course and magnetic resonance imaging features were
atypical for multiphasic disseminated encephalomyelitis and
indistinguishable from multiple sclerosis.

To our knowledge, this association has not been previously described in
the English literature in childhood.

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