Management of optic pathway tumors of childhood.
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The existence of a mechanism by which the ester- and ether-linked aliphatic chains of the major phospholipids are retailored during their axonal transport and sorted to specific membrane systems along the optic nerve and tract was investigated. A mixture of [1-14C]hexadecanol and [3H]arachidonic acid was injected into the vitreous body of albino rabbits. At 24 h and 8 days later, the distribution (as measured by the 3H/14C ratio) and the positioning (as monitored by hydrolytic procedures) of radioactivity in the various phospholipid classes of retina, purified axons, and myelin of the optic nerve and tract were determined. At the two intervals after labeling, the 3H/14C ratios of each diradyl type of phosphatidylethanolamine and phosphatidylcholine were (a) substantially unchanged all along the axons within the optic nerve and tract and (b) markedly modified in comparison with those found in the retina and axons for molecular species selectively restricted to myelin sheath. Evidence is thus available that intraxonally moving ethanolamine and choline glycerophospholipids, among others, are added to axonal membranes most likely without extensive modifications. In contrast, they are transferred into myelin after retailoring. Through these two processes, the sorting and targeting of newly synthesized phospholipids to their correct membrane domains, such as axoplasmic organelles, axolemma, or periaxonal myelin, could be controlled.
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In multiple sclerosis (MS), an increase in delay in visual evoked responses (VER) may be important, but an earlier change is the behaviour of the wave-shape; amplitude is always lower even in MS patients without subjective visual symptoms, provided not only flash but also pattern stimulation is used. Normal amplitude with pattern stimulation does not necessarily mean normal amplitude with flash stimulation. Combining both stimulation methods, we found lowering of amplitude and change of wave-shape in 100 per cent of cases while latency was increased in only 77 per cent. Flash and pattern VER might be even more pathognomonic in MS than oligoclonal gammaglobulin fractionation on agar, which is positive in 75 per cent of cases. We recommend that subclinical cases of MS should be submitted to full electro-oculographic investigation. Patients with sectoral ischaemic neuropathy and open angle glaucoma (OAG) show the same findings; this emphasizes that the method is not specific for demyelinating diseases, nor selective for papillo-macular bundle involvement, but that VER, considering only its first components, reflects axonal damage somewhere in the visual pathway.
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