Canavan disease (CD) is a leukodystrophy characterized by the degeneration of white matter that is replaced by fluid, thereby causing vacuolating myelinopathy
(1-3) The electrophysiological examination including both nerve conduction and electromyography studies should identify peripheral nerve dysfunction if present, along with the specific location in the nerve pathway, involvement of sensory and/or motor axons, and the presence of myelinopathy
and/or axonopathy neuropathic process.
Haginoya et al., "Schinzel-Giedion syndrome: A further cause of early myoclonic encephalopathy and vacuolating myelinopathy
," Brain & Development, vol.
(1) The coalescence of vacuoles formed between myelin lamellae in the oligodendroglia results in a pattern termed "vacuolating myelinopathy
" when seen under electron microscopy.
Avian vacuolar myelinopathy
(AVM) is a neurologic disease causing recurrent mortality of bald eagles (Haliaeetus leucocephalus) and American coots (Futica americana) at reservoirs and small impoundments in the southern USA.
Disease Pathology Inheritance Genes/chromosomal name (a) mode loci associated with disease, n CMT1 Abnormal myelin AD (b) 9 CMT2 Axonopathy AD 19 CMT4 Either myelinopathy
AR 10 or axonopathy CMTX Axonopathy with XLD 2 secondary myelin changes Disease Proportion name (a) of all CMT, % CMT1 40-50 CMT2 10-15 CMT4 Rare CMTX 10-15 (a) Intermediate dominant (dominant inheritance with NCV between demylinating and axonopathic) and other rare forms are not included.
(12.) Sadick N, Strenberg PE: Vacuolar myelinopathy
of the brain and lamellar ichthyosis.
(4,12) Triggs and Cros wrote a letter to the editor of Neurology by citing their paper and ours, suggesting that the entire findings in GBS patients were produced by only a primary myelinopathy
. (4,35-38) We responded to their comments by suggesting that an animal model of GBS inoculated with C.
Additionally, hydrilla has been implicated in the spread of an emerging avian disease known as avian vacuolar myelinopathy
that affects herbivorous waterbirds and their predators (11), (12), (13).
But there is the same genetically caused myelin sheath damage of peripheral nerves (myelinopathy
) in pathogenesis in majority of them.
Inherited spinal myelinopathy
in Murray Gray cattle.
Researchers at the University of Georgia are working to determine the cause of Avian Vacuolar Myelinopathy
which has killed eagles and other birds in four states, which is currently unknown.