Wobbly hedgehog syndrome- a progressive neurodegenerative disease

Wobbly hedgehog syndrome (WHS) has been long considered to be a myelin disease primarily affecting the four-toed hedgehog. In this study, we have shown for the first time that demyelination is accompanied by extensive remyelination in WHS. However, remyelination is not enough to compensate for the axonal degeneration and neuronal loss, resulting in a progressive neurodegenerative disease reminiscent of progressive forms of multiple sclerosis (MS) in humans. Thus, understanding the pathological features of WHS may shed light on the disease progression in progressive MS and ultimately help to develop therapeutic strategies for both diseases. HighlightsO_LIWobbly hedgehog syndrome (WHS) is a progressive neurodegenerative disease. C_LIO_LISpongy degeneration of the brain and spinal cord is the diagnostic feature of WHS. C_LIO_LIWHS affected brain and spinal cord show extensive demyelination and remyelination. C_LIO_LIAxonal degeneration is accompanied by loss of neurons in WHS. C_LI O_FIG O_LINKSMALLFIG WIDTH=185 HEIGHT=200 SRC="FIGDIR/small/547983v1_figu1.gif" ALT="Figure 1"> View larger version (50K): org.highwire.dtl.DTLVardef@cb6155org.highwire.dtl.DTLVardef@634d4dorg.highwire.dtl.DTLVardef@1681726org.highwire.dtl.DTLVardef@c80123_HPS_FORMAT_FIGEXP M_FIG Graphical Abstract C_FIG