This report describes the neuropathology of progressive axonopathy (PA), an @entity74 of Boxer @entity235 , which affects CNS and PNS. The nerve roots contain numerous myelin bubbles and proximal paranodal axonal swellings containing vesicles, vesiculo-tubular profiles and disorganized neurofilaments. The myelin sheath overlying such swellings is often attenuated. As the disease develops there are progressive changes in the myelin sheath with thinning at paranodal and internodal locations, loss of myelin from lengths of axon and the formation of short internodes with disproportionately thin sheaths. The abnormalities show a very definite selectivity for nerve roots and proximal nerves. Conversely, the frequency of degeneration and regeneration is greater distally except in the cervical ventral roots which contain numerous regenerating clusters. In the CNS numerous axonal spheroids are found in the lateral and ventral columns of the spinal cord and in various brain stem nuclei, particularly the superior olives, accessory cuneate nuclei and lateral lemniscus and its nucleus. @entity528 which occurs mainly in the cord shows no obvious tract or proximal/distal selectivity. The optic pathways are also involved, predominantly adjacent to the chiasma. The autonomic nervous system is affected and distal limb muscles show varying, but usually minor, degrees of @entity194 . The condition, which has no obvious direct parallel in @entity1 or veterinary medicine, shows gross disturbances of axon-glial inter-relationships in both CNS and PNS.

Progressive axonopathy: an XXXX of boxer @entity235 . 2. The nature and distribution of the pathological changes.

@entity1 :: ('9606', 'Species') :: ['human'] @entity235 :: ('9615', 'Species') :: ['dogs'] @entity194 :: ('MESH:D001284', 'Disease') :: ['neurogenic atrophy'] @entity74 :: ('MESH:D030342', 'Disease') :: ['autosomal recessive inherited neuropathy'] @entity528 :: ('MESH:D009410', 'Disease') :: ['Axonal degeneration']
@entity74 :: (MESH:D030342,Disease) :: ['inherited neuropathy']
