Canavan disease (CD) is a leukodystrophy characterized by the degeneration of white matter that is replaced by fluid, thereby causing vacuolating
myelinopathy (1, 2).
(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.
(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.