giant axonal neuropathy


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gi·ant ax·o·nal neu·rop·a·thy

a rare disorder beginning at or after the third year of life, and presenting clinically with kinky hair, progressive painless clumsiness, muscle weakness and atrophy, sensory loss, and areflexia. Pathologically, both myelinated and unmyelinated nerve fibers contain axonal spheroids packed with neurofilaments; sporadic in nature.

giant axonal neuropathy

an autosomal-recessive neuropathy of childhood characterized by enlarged axons made up of masses of tightly woven neurofilaments.

gi·ant ax·o·nal neu·rop·a·thy

(jī'ănt ak'sŏ-năl nūr-op'ă-thē)
A rare disorder beginning at or after the third year of life, and presenting clinically with kinky hair, progressive clumsiness, muscle weakness and atrophy, sensory loss, and areflexia.

axonal

pertaining to or arising from an axon.

axonal degeneration
an axon dies and cannot be replaced if its cell body is destroyed. A damaged axon in the central nervous system similarly cannot undergo regeneration, but a peripheral nerve with an intact nerve cell can regenerate. See also wallerian degeneration.
axonal dystrophy
specific diseases characterized by nutritional abnormalities of axons include in sheep, Suffolk, Coopworth, Merino axonal dystrophies, in dogs, Rottweiler, Chiahuahua dystrophies, in horses Haflinger, Morgan, in cats a dystrophy in lilac coat color domestic shorthaired.
giant axonal neuropathy
see giant axonal neuropathy.
axonal migration
the movement of axoplasm from the proximal segment of a severed nerve fiber to the distal portion, following Schwann cell extensions, in the process of peripheral nerve regeneration.
axonal reaction
central chromatolysis of the axon characterized by eccentric relocation of the nucleus, greater prominence of the nucleolus and a basophilic cap of RNA on its cytoplasmic aspect, dispersal of the Nissl substance to the periphery of the cell, and an increase in the number of neurofilaments.

neuropathy

a general term denoting functional disturbances and pathological changes in the peripheral nervous system. The etiology may be known (e.g. poidoning by arsenicals, ischemic or traumatic neuropathy) or unknown. Encephalopathy and myelopathy are corresponding terms relating to involvement of the brain and spinal cord, respectively. The term is also used to designate noninflammatory lesions in the peripheral nervous system, in contrast to inflammatory lesions (neuritis).

central peripheral neuropathy
see Boxer progressive axonopathy.
diabetic neuropathy
a chronic symmetrical sensory polyneuropathy associated with diabetes mellitus in humans, which occurs uncommonly in dogs and cats.
entrapment neuropathy
a neuropathy due to mechanical pressure on a peripheral nerve.
giant axonal neuropathy
a familial disease of German shepherd dogs, characterized by ataxia, hypotonia, reduced pain sensation, and loss of reflexes and proprioception in the hindlegs, which develops from a young age. Vomiting, associated with esophageal dilatation, also occurs.
hereditary neuropathy
recorded in Tibetan mastiff as an inherited defect in myelin production. Weakness, loss of reflexes and quadriplegia develop quickly and at an early age.
hereditary sensory neuropathy
an inherited abnormality in which affected dogs have impaired perception of pain in the feet and lower limbs from a young age; causes extensive self-mutilation of toes and footpads. It occurs in German shorthaired pointers, English pointers and English springer spaniels. Called also acral mutilation syndrome.
infiltrative splanchnic neuropathy
see macaw wasting disease.
progressive neuropathy
disease of young Cairn terriers with many similarities to globoid cell leukodystrophy. Affected dogs show quadriparesis, ataxia and head tremors.
retrobulbar neuropathy
trigeminal neuropathy
see mandibular neurapraxia.