Hereditary vertical nystagmus.
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Biomedical subjects
Publications and source records attributed to M F Marmor.
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1. Current-voltage relations were generated in the Anisodoris giant neurone (G cell) by either current pulses or slow biphasic current ramps.2. Inward-going rectification occurred during hyperpolarization at warm temperatures (10-15 degrees C), but not at cold temperatures (0-5 degrees C) or in the absence of external K.3. Replacing external K with Rb eliminated inward-going rectification in the warm, but produced it in the cold. The removal of external Na, Cl or Ca had no effect upon inward-going rectification.4. At cold temperatures the I-V relation was linear when generated by current pulses, but was non-linear in accordance with the constant field hypothesis when generated by current ramps.5. A high conductance state developed when the membrane was hyperpolarized beyond a critical potential (approximately - 130 mV in the cold, and - 110 mV in the warm) which was dependent upon external Ca, but not upon K, Na or Cl.6. Hysteresis was observed in the ramp-generated I-V relation whenever the cell was polarized into the high conductance state.7. Rectification and the high conductance state appear to involve different mechanisms within the membrane. However, both are dependent upon absolute membrane potential and not the resting potential.8. The axonal-somatic conductance ratio for the G cell was calculated to be between 2 and 10.9. The membrane time constant (200-100 msec) and specific resistance (0.1-1.5 x 10(6) Omega cm(2)) varied with temperature, membrane potential, and external ions in a manner that correlated with changes in the shape of the I-V relation. In addition, the resistance was dependent upon external Ca.10. The K permeability (P(K)), measured during inhibition of inwardgoing rectification, was independent of temperature and membrane potential. However, P(Na) increased with warming.11. The specific capacitance was calculated to be 0.5-1.0 muF/cm(2). The capacitance increased slightly with warming, but was independent of membrane potential and unaffected by reductions in external K or Na.
1. The current-voltage relations of the Anisodoris giant neurone (G cell) were studied in the presence and absence of Na pump activity.2. Inhibition of the electrogenic Na pump with ouabain had no effect on either the presence at warm temperatures (10-15 degrees C), or absence at cold temperatures (0-5 degrees C), of inward-going rectification.3. Abolition of inward-going rectification in the warm, by replacement of external K with Rb, did not affect the electrogenic Na pump.4. The current generated by the electrogenic pump was essentially constant between the membrane potentials of - 30 and - 100 mV.5. The potential produced by the electrogenic pump can be predicted by a modification of the constant field equation.6. It is estimated that the energy required to extrude Na was between 3160 and 3700 cal/g-atom, and that uncoupled Na efflux during pump activity was typically between 0.2 and 4.0 p-mole/cm(2).sec.
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The resting potential of a molluscan neuron can be separated experimentally into two components: one which depends on ionic gradients and permeabilities in accordance with the Goldman equation, and a second which depends on the electrogenicity of active sodium transport.
1. The membrane potential of the gastro-oesophageal giant neurone of the marine mollusc, Anisodoris nobilis, was examined during changes of temperature and of the ionic medium.2. The response of the membrane potential to rapid changes in the external K concentration was prompt, stable, and reversible up to 200 mM-K, and was independent of the external Cl concentration.3. Warming the cell produced a prompt hyperpolarization that was approximately 10 times greater than predicted by the Nernst or constant field equations. Electrogenic activity of the Na-K exchange pump was shown to be responsible for this effect.4. At temperatures below 5 degrees C, the relationship between the membrane potential and the external K concentration could be predicted by a constant field equation.5. At temperatures above 5 degrees C, the membrane potential could not be predicted by the constant field equation except after inhibition of the electrogenic Na pump with ouabain or the reduction of internal Na.6. Inhibition of the electrogenic Na pump by low external K concentrations was dependent upon the external Na concentration.7. It is concluded that the membrane potential is the sum of ionic and metabolic components, and that the behaviour of the ionic component can be predicted by a constant field type equation.
1. The temperature dependence of the membrane potential of a molluscan giant neurone was examined under conditions which block the electrogenic activity of the Na-K exchange pump.2. When the Na pump was blocked by ouabain or the removal of external K, the membrane potential depolarized as temperature was increased.3. This depolarization was prevented by the replacement of external Na with impermeant cations, but was greater when Na was replaced with Li.4. All observed effects of ouabain were attributable to inhibition of the Na pump. The depolarization in response to ouabain at warmer temperatures was completely reversible, and the rate of both onset and reversibility of the ouabain effect was dependent upon temperature.5. Using a modified form of the constant field equation, the internal K concentration and the Na-K permeability ratio, P(Na)/P(K), were calculated from the experimental data.6. P(Na)/P(K) was found to increase from 0.028 at 4 degrees C to 0.068 at 18 degrees C. It is suggested that this increase is due primarily to a change in P(Na).
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The pathophysiology of central serous chorioretinopathy is incompletely understood but appears to involve the retinal pigment epithelium. We recorded consecutively the fast oscillation, hyperosmolarity response, acetazolamide response, and light peak from four patients with active central serous chorioretinopathy and three normal subjects to determine whether the affected eyes showed any electrophysical abnormalities. We found essentially no differences in any of the four responses between the active and the inactive eyes of the patients or between patients and normal subjects. Whatever retinal pigment epithelial dysfunction exists in central serous chorioretinopathy is unassociated with clinically evident changes in these retinal pigment epithelial electrophysiologic responses.
This study examines whether electroretinogram recovery after short-term (15- or 20-min) ischemia is enhanced by agents (catalase and dextromethorphan) that are known to enhance recovery after longer (60-75 min) periods of ischemia. Under both light-adapted and dark-adapted conditions, Dutch rabbits were exposed to two sequential sets of short-term ischemia, each followed by 60 min of reperfusion during which the electroretinogram was monitored. Catalase or dextromethorphan was administered intravenously before the second reperfusion period. Control experiments showed that electroretinogram recovery curves from sequential ischemic episodes were similar, and neither intravenous catalase nor dextromethorphan increased the rate or magnitude of electroretinogram recovery. This negative result suggests that the mechanisms of injury or recovery after short-term retinal ischemia may be different from those operating after 60-75 min of ischemia.
The pattern electroretinogram is a retinal response that can be evoked by viewing an alternating grating or checkerboard. It is receiving increasing clinical and research attention because it can provide information about inner retinal cells and the macula. However, clinicians may have trouble choosing between different techniques for recording the Pattern electroretinogram that have been described in the literature. The International Society for Clinical Electrophysiology of Vision has prepared guidelines for a basic pattern electroretinogram recording procedure to aid new users in obtaining reliable responses and to encourage more uniformity among existing users.
One--hundred years have passed since Franz Boll and Willy Kühne first characterized rhodopsin and discovered the visual pigment cycle. Some of the events and individuals of that period are described, and color photographs are presented to show the appearance of rhodopsin in the living retina.
No "major" painter is known to be color deficient. Are there truly no color deficient artists, or have they not been recognized? The historical literature cites criteria for recognizing color deficiency in artists, but they are hard to apply without knowing the intentions of an artist. The work and commentary of a color-deficient artist who works currently in Paris are presented as an example. He uses a limited palette of colors, based on advice from colleagues as much as his own perceptions, and he uses colors in ways that do not always fit with expectations for color deficiency. Biographies of earlier painters suggest that there were a few whose color sense was poor, but these painters used assistants to help. The color sense of others, such as the English landscape painter John Constable (1776-1837), has been questioned because of a preponderance of suspicious color, such as murky green. However, there are good reasons to doubt that Constable was color deficient. It is instructive to know how proven color deficiency has influenced an artist's style. When medical information is unavailable, the best advice for the diagnostically-inclined observer is just to enjoy the art.
Sildenafil citrate (Viagra) is a new oral medication that inhibits phosphodiesterase-5 (PDE5) in the corpus cavernosum to facilitate penile erection for the treatment of male impotence. The drug also has a mild inhibitory effect on PDE6, which controls the level of cyclic guanosine monophosphate in the retina, and it may cause a perception of bluish haze or increased light sensitivity in some patients. Long-term retinal damage has not been reported, but long-term electroretinographic studies have not been performed. Sildenafil causes a mild lowering of blood pressure and is absolutely contraindicated in patients taking any form of nitrate medication. A number of cardiovascular deaths and retinal vascular events in patients taking sildenafil have been reported, but so far the rate of these complications does not exceed expectation for an elderly population. Ophthalmologists should alert patients to the ocular side effects and potential risks of this new drug until further clinical experience has been obtained.
Metamorphopsia is a symptom of retinal distortion from intrinsic retinal disease. It has undoubtedly been experienced for millennia, but its clinical significance has been appreciated only in modern times. The Norwegian painter Edvard Munch recognized scotomas and metamorphopsia after suffering an intraocular hemorrhage in his 60th year. Drawings made during this illness show his changing perceptions, and also his attempts to document them with a grid of lines. The Scottish philosopher Thomas Reid may have been the first to write about metamorphopsia. He described distortion of his vision in 1764, after an episode of sungazing, and recognized that the problem was probably of retinal origin. Lines or grids to document metamorphopsia have appeared in ophthalmology textbooks for more than 100 years, but testing for macular degeneration did not become routine until the dissemination of Amsler's grids in the middle of the 20th century. This is in large measure a result of developments in ophthalmology that made therapy for macular disease possible.
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South East (SE) Asians accounted for a disproportionate percentage of the functional visual loss (FVL) seen in our area between 1983 and 1987. Moreover, 94% of the SE Asian FVL patients were Cambodian, although Cambodians only represented 20-30% of the local SE Asian patient population. Cambodian refugees are, at present, generally from lower socio-economic classes than the Vietnamese, and a much larger percentage of them have a history of camp incarceration and previous trauma. The Cambodians presenting with FVL may have conversion hysteria influenced by their wartime experience and cultural background. This study demonstrates that the SE Asian refugees in California are not a homogenous group with respect to visual problems, and that an awareness of cultural or historical factors can be important to the management of ophthalmic symptoms in our multi-cultural societies.