giant axonal neuropathy


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.

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.