Journal of Neuro-Ophthalmology, December 1984, Volume 4, Issue 4

Neuro-ophthalmology in the recent European literature. Part III. Heredodegenerations, stroke, pain, and diagnostic techniques.

J. Clill. Neuro-ophtha/11lo/. 4: 275-284, 1984. Neuro-ophthalmology in the Recent European Literature Part III. Heredodegenerations, Stroke, Pain, and Diagnostic Techniques AVINOAM B. SAFRAN, M.D. In Part III, the final section in the series of articles reviewing neuro-ophthalmology in the recent European literature (January to June 1983), the following topics will be discussed: heredode­generative disorders; retinal and cerebral stroke; pain; and diagnostic techniques. Heredodegenerative Disorders A survey of 97 cases of tuberous sclerosis, covering seizures, handicap, physical findings, and family aspects, was provided by Hunt.! T.he author emphasized the importance of dep!g­mented spots as a diagnostic clue to tuberous sclerosis. Depigmented skin macules were the most common skin lesion, reported in 92 cases (95%). Occurrence of adenoma sebaceum w~s noted in 78 cases (80%), and shagreen patches In 47 (48%). Depigmented spots and shagreen patches were observed at birth, whereas the pres­ence of adenoma sebaceum was not apparent at birth. Initially, adenoma sebaceum appeared as small red spots, which in some cases were tran­sitory. Hunt stressed the importance of ~~e s.ys­tematic use of ultraviolet light for IdentIflcatIon of depigmented spots when examining f.amily members of affected children. Although It has been claimed that, in diseases with dominant inheritance pattern, the symptomatology of the affected subjects may vary according to the sex of the carrier parent, no such correlation could be established in this study. Profound handicap was mainly noted in those children who had an early onset of seizures. Of greatest concern for the parents were behavioral problems including. l~ck of communication, screaming, and hyperactIvity. They occurred in 58 children. Twenty parents said their major problem was the management of seizures. From Clinique d'ophtalmolgie, Hopital Cantonal Universi­taire,. Geneva, Switzerland. December 1984 Patients with tuberous sclerosis can develop an unusual form of benign subependymal glioma characteristically located at the foramen of Monro. These patients should, therefore, be checked regularly for clinical evidence of i~creas­ing intracranial pressure. Because of resultIng hy­drocephalus, these tumors are much small:r tha~ most other supratentorial gliomas when flrst di­agnosed. Of eight patients with intracranial m~n­ifestations of tuberous sclerosis examined by Wm­ter," four also presented with glioma at the fora­men of Monro. The value of computerized to­mography in diagnosis of intracranial tumo~s ver­sus tubers in patients with tuberous scleroSIS was discussed. Although both contain calcifications, glioma are differentiated from tubers by contrast enhancement in computerized tomography scan­ning. The importance of diagnosing bilateral ob­struction of the foramen of Monro when the tumor arises bilaterally or when a large unilateral tumor encroaches on both foramina of Monro was also stressed by Winter. This condition ne­cessitates bilateral shunting, a requirement re­portedly easily overlooked in neurosurgical prac­tice because of its rare occurrence. Ataxia-telangiectasia heterozygotes are predis­posed to die from malignant neoplasm. This was suggested by Swift et al. 3 in 1976. The assertion was recently confirmed by Swift and Chase4 in a survey of causes of death in 16 deceased parents of affected patients. In addition to higher than usual rates of cancer death, a high incidence of ischemic heart disease was found. This was ap­parently the first study of causes of death in obligatory ataxia-telangiectasia heterozygotes, i.e., in parents of affected patients. Cancer in ataxia-telangiectasia could be treated with reduced radiotherapy and chemotherapy doses. This is an important fact when considering the elevated incidence of cancer and leukemia among these patients. Severe reaction to ionizing irradiation in patients with ataxia-telangiectasia has been known of for several years.s In addition, 275 Recent Literature: Part III Abadir and Hakami6 recently observed unusually high chemosensitivity in a case of ataxia-telangi­ectasia. Remarkably, in this patient, control of a nasopharyngeal undifferentiated lymphoma was achieved for 5 years with a therapy which is usually considered inadequate. A variety of patients showing atypical patterns of hereditary degenerative disorders were re­ported. Tuck and McLeod? observed four patients presenting with clinical features resembling Ref­sum's disease, but lacking both detectable bio­chemical abnormality and "onion bulb" forma­tions on sural nerve biopsy. Occurrence of occa­sionally minimal peripheral nerve involvement in Refsum's disease was confirmed by Barbieri et al. 8 Salisachs9 reviewed his personal experience with Charcot-Marie-Tooth's and Refsum's dis­eases, and summarized new findings on the dis­eases. Variations in the motor and sensory neu­ropathy in these two conditions were discussed. Prominent bilateral papilloedema was ob­served by Beck10 in a patient with Hunter's syn­drome (type II mucopolysaccharidosis). When he was a 23-year-old, the patient was first examined by an ophthalmologist for intermittent blurring of vision. Gross bilateral papilloedema was found, as well as pigmentary retinopathy. Lum­bar puncture showed normal cerebrospinal fluid pressure, and cerebral computerized tomography scanning was unremarkable. Myelogram dem­onstrated an almost complete block at the C7-Tl level. Beck gave credit to McKusik11 for having previously described papilloedema in two pa­tients with Hunter's syndrome. Bilateral fixed miosis was noted by Dick et al. 12 in four patients with hereditary spastic ataxia, aged between 17 and 44 years. Pupils were de­scribed as being 2 mm in diameter in two patients, and all four patients were unable to respond to light, accommodation, or reduction in ambient illumination. It was felt that the patients had congenital miosis, with absent or hypoplastic di­lator muscle. Previous cases of congenital myosis were reviewed. The condition may be inherited, generally as an autosomal-dominant trait. It has been described in conjunction with arachnodac­tyly, congenital rubella and Lowe's oculocerebral syndrome. Retinal and Cerebral Stroke Monocular visual changes due to temporary reduction in blood flow to the ophthalmic arterial system may differ from those resulting from em­bolism. Ross Russell and Page13 described four patients with extensive occlusive disease of the extracranial arteries who complained of intermit­tent alteration in vision related to standing or mild exertion, or precipitated by heat (e.g., enter- 276 ing a hot room). In one patient, visual symptoms were reported to develop over minutes, and re­covery of vision similarly took place gradually. Attacks consisted of a bright dazzling sensation, bright objects appeared lighter whereas dark ob­jects became difficult to see. The overall effect was one of "overexposure." Another patient de­scribed the appearance as "a negative of a pho­tograph." Sight becoming gradually patchy and fragmented was reported by two patients. A strik­ing feature experienced by the patients was the occurrence of visual phenomena without any symptoms of cerebral ischemia. This led the au­thors to emphasize that the eye was a more sensitive index of hypotension than the brain. The risk of eventually developing contralateral occipital infarction following a unilateral occipital stroke was evaluated by Bogousslavsky et al. 14 Thirteen out of 58 patients studied (22.4%) pre­sented with a contralateral occipital involvement, over a mean follow-up period of 39.6 months. The contralateral occipital stroke occurred after a delay of 2 days to 12 months with an average of 4.2 months. Absence of improvement of initial visual fields and border zone extension of the initial infarct towards the Sylvian area were sig­nificantly correlated with occurrence of contralat­eral involvement. During this follow-up period, there were only three delayed Sylvian strokes, versus 13 controlateral occipital infarction. The so-called atheromatous pseudo-occlusion is an extreme type of stenosing lesion which is easily misdiagnosed as complete occlusion with noninvasive evaluation and conventional angiog­raphy. However, in contrast with asymptomatic or oligosymptomatic complete occlusion of the internal carotid artery, pseudo-occlusion repre­sents a major danger for the patient because, as emphasized by Ringelstein et al, 15 occurrence of infarction as a result of hemodynamic changes is frequent during the stage of subtotal stenosis. Therefore, pseudo-occlusion requires fast and ap­propriate therapeutic measures. Recent data in­dicate that pseudo-occlusion of the internal ca­rotid artery is a frequent diagnostic trap. Recog­nition necessitates appropriate angiographic pro­cedure. The serial imaging run must be prolonged to allow the dye to pass through the stenotic lesion during the radiological procedure. Further­more, subtraction of the images is required. Rin­gelstein et al. reported nine subjects presenting with such pseudo-occlusion, and, apparently for the first time, described the way this condition was diagnosed by means of Doppler ultrasound. When typical sonographic features of internal carotid artery occlusion were observed (i.e., retro­grade blood flow through the ophthalmic artery, altered blood flow through the ophthalmic artery, altered blood velocity pattern in the common Journal of Clinical Neuro-ophthalmology carotid artery, and lack of signal from the internal carotid artery), special effort was required in eval­uating the area of the carotid bulb for a faint, sharp, hissing sound. Its recognition was report­edly difficult. Ringelstein et al. indicated that it was so faint that documentation on both the "scope" and the recording chart was generally not possible. However, the tone was said to be so typical that it was diagnostic. In seven patients, ultrasound diagnosis of pseudo-occlusion was confirmed by appropriate angiography, and in one patient without angiography the diagnosis was confirmed during endarterectomy. In the re­maining patient, diagnosis was missed with Dop­pler evaluation, but was eventually established by means of cerebral angiography. Aspirin was shown to be effective in the sec­ondary prevention of atherothrombotic cerebral ischemia in the apparently largest controlled trial ever conducted on the matter. Six hundred four patients were studied by Bousser et al. 10 over a follow-up period of 3 years. They were divided into three groups: 1) patients treated with aspirin 1 g a day; 2) patients treated with aspirin 1 g a day and dipyridamole 225 mg a day; and 3) patients receiving a placebo. The cumulative rate of cerebral infarction was 10% in the two treated groups, and 18.5% in the placebo group. The difference between the two treated groups con­sidered together and the placebo group was found to be significant (p < 0.02). Analysis of results failed to show either a significant differ­ence between the two treated groups or a differ­ence in the efficacy of aspirin for patients of different sexes. Also of interest was the significant reduction in occurrence of myocardial infarction in the two treated groups (p < 0.05). The overall incidence of cerebral and myocardial infarction showed a strong negative correlation with treat­ment (p < 0.004). Clinical value of phosphorus nuclear magnetic resonance spectroscopy for investigation of cere­bral metabolism was demonstrated by Cady et al. 17 in newborn infants with cerebral hypoxia. The technique allowed metabolic consequences of cellular hypoxia to be detected rapidly. As compared to values measured in a presumably normal infant, phosphocreatinine inorganic or­thophosphate ratio was shown to be reduced in three infants who had suffered birth asphyxia, but increased as clinical condition improved. In­fusions of mannitol solution were followed by a rapid ratio increase on four occasions in two of the infants. Demonstration that large porence­phalic cysts developed in two infants some time after grossly abnormal phosphorus spectra had been demonstrated also emphasized the prognos­tic value of nuclear magnetic resonance. Nuclear magnetic resonance imaging of revers- December 1984 Safran ible cerebral ischemia in man was also described by Sipponen et al. 18 One reported patient pre­sented with a ruptured intracranial aneurysm, and showed temporary aphasia and slight hemi­paresis. Huber and Yasargil 19 summarized their expe­rience with 40 carotid-ophthalmic artery aneu­rysms (in 33 patients) with regard to symptoma­tology, modern microsurgical treatment, and postoperative results. Following surgery, wors­ening of visual defects was noted in only four patients. The uncommon association of Wallenberg's syndrome with "en plaque" craniopharynir0ma was reported by van der Bergh and Dom2 in a 71-year-old man. The tumor was spreading along the pons, involving the posterior inferior cerebel­lar artery, and the basilar artery and its branches. Van der Bergh and Dom reviewed the reported etiologies of Wallenberg's syndrome, which in­clude arteriosclerotic thrombotic occlusions, em­bolic occlusions, syphilitic vascular disease, brain stem tumors, metastatic carcinoma, metastatic brainstem encephalitis caused by infectious foci in Lang's herpetic brainstem encephalitis, demye­linating disease, whiplash trauma, and mechani­cal trauma to the vertebral artery in the neck. Pain Although the endorphin hypothesis has been accepted by many as the best explanation for the pain control with acupunctural stimulation or placebo administration, controversies about the real importance of endorphin-mediated analgesia are far from being resolved. Chapman et al.,:'1 in a recent study on acu­puncture analgesia following injection of nalox­one (a narcotic antagonist), were unable to dem­onstrate reversal of analgesia when naloxone was given. These findings did not support the asser­tion of endorphin as the main factor of acupunc­ture analgesia. Chapman et a!. suggested that there may be two kinds of acupunctural analge­sia. One mechanism would be linked to the stress resulting from the acupuncture procedure itself, that induces secretion of beta-endorphin and ad­renocorticotropin (ACTH), while a second mech­anism would involve an acupuncture-specific nonopioid-mediated phenomenon. Relative im­portance of each of these mechanisms could be related to the circumstances of the acupuncture procedure. A significant stress response is less likely to occur in a human subject of laboratory research than in clinical patients. Chapman et aI., therefore, hypothesized that this may account for inconsistencies of naloxone reversal of acupunc­ture analgesia among the various experimental sets. 277 Recent Literature: Part III The hypothesis of endorphin-mediated anal­gesia in placebo treatment was partiallx sup­ported by the study of Grevert et al., 2 who showed that administration of naloxone dimin­ished the analgesic effectiveness of the placebo. However, naloxone did not prevent significant placebo-induced analgesia from occurring. Thermal biofeedback emerged as the treatment of choice of migraine headaches, as compared with frontalis electromyography biofeedback and relaxation technique, in a study conducted by Lacroix et al 23 with 27 patients. Although reduc­tion in headache was observed with each method, it was most prominent with thermal biofeedback. The latter technique led to immediate and sub­stantial improvement that lasted as long as 6 months after the end of the treatment. In contrast with the immediate effects of thermal biofeed­back, those of relaxation treatment took months to develop. However, by the 6 months of follow­up, these patients enjoyed an improvement com­parable to that of patients treated with thermal biofeedback. Headache reduction resulting from electromyography biofeedback was much smaller than that obtained by either of the other two techniques. Interestingly, reduction in migraine headache symptomatology could be correlated with changes of skin temperature in all three groups of patients. To compare the prophylactic effect of acetyl­salicylic acid with that of propranolol in the treat­ment of common migraine, Baldrati et al. 24 con­ducted a double-blind crossover study in 18 pa­tients. In comparison with the pretreatment pe­riod, both drugs reduced migraine index, fre­quency, duration, and severity of attacks. No correlation was found either between plasma con­centration and effects of the drug, or between results in acetylsalicylic acid and propranolol­treated groups. However, the number of patients involved in the study was limited. Placebo com­parison was not used. The increasing number of known side-effects of propranolol is a matter of some concern. Inhi­bition of phagocytic activity of cultured retinal pigment epithelial cells when incubated with pro­pranolol was recently added by Matsuda et al. 25 to the previously reported alterations in ocular metabolism induced by beta-blockers. In a review in The Lancet, Breckenridge26 addressed the prob­lem of heterogeneity of the side effects of beta­blockers and stressed the importance of newly observed drug-induced effects that may consti­tute major risk factors. Among these are propran­olol- induced increase of plasma triglycerides, de­creased concentration of high-density lipopro­teins, and increased concentration of low-density lipoproteins. As indicated by Breckenridge, clini­cal importance of these induced changes in lipid 278 metabolism is difficult to assess when overall effects of beta-blockers may be to decrease the incidence of ischemic heart disease by means of other mechanisms. In patients with atypical periodic headache, Geraud et al. 27 suggested the assessment of su­persensitivity to dopamine agonist for differen­tiating migraine from chronic nonmigrainous headache. In a study involving 150 patients, they showed that 30 minutes after intravenous admin­istration of 0.1 mg/kg of piribediL 94% of patients with migraine exhibited nausea, vomiting, and rapid fall in blood pressure, whereas no effect was seen in 59% of patients presenting with nonmigrainous headache. Chronic paroxysmal hemicrania has been dif­ferentiated from cluster headache by the follow­ing clinical features: 1) predominant occurrence in females, 2) frequent short attacks (15 or more per day), and 3) full relief with indomethacin. So far, a limited number of patients with chronic paroxysmal hemicrania have been described. The first British case was recently reported by Petty and Clifford-Rose. 28 "If possible the investigator engaged in research on pain in animals should try the pain stimulus on himself; this principle applies for most non­invasive stimuli causing acute pain." This sugges­tion was at least included in the "Ethical Guide­lines of the Committee for Research and Ethical Issues of the International Association for the Study of Pain."29 Diagnostic Techniques Automated Perimetry The Peritest is a new computer-assisted perim­eter, first described by Greve.30 Its efficiency was assessed recently by Greve et al.,31 Greve and Dannhein,32 and Etienne et al.,33 mainly in glau­comatous subjects. The Peritest is based on light­emitting diode (LED) technology. Two hundred six positions can be examined, using a threshold­related gradient adapted suprathreshold strategy. It makes possible the performance of both auto­matic and semiautomatic (manual) visual field examination with the same instrument. The sem­iautomatic procedure allows a quick control eval­uation of inconsistent results. In addition, by us­ing multiple rather than single stimuli, semiau­tomatic examination is performed more quickly than With the completely automatic procedure. Reliability of the patient is measured. In glaucom­atous patients, the Peritest was found to be su­perior to routine kinetic examination, but less sensitive than careful static perimetry. An automated static perimeter/adaptometer using light-emitting diodes was described by Ernst et al. 34 It measures thresholds with lights of Journal of Clinical Neuro-ophthalmology two wavelengths (530 and 660 nm). The proce­dure allows assessment of rod and cone mecha­nisms either during dark adaptation or with full dark adaptation. The instrument has been de­vised primarily for the diagnosis and manage­ment of retinal disorders, but it may have appli­cations in other clinical fields. The Competer is a computerized perimeter that was modified by Bynke and Krakau in 198235 to make it better suited to neuro-ophthalmological examination. Exploration of the central visual field (2.5° to 20° excentricity) is performed by means of 68 testpoints, and midperipheral field 20° to 35° excentricity with 44 testpoints. The sums of the threshold values in the 68 and 44 testpoints, respectively reflect the overall function of the central and midperipheral fields. Differ­ence between the performance values in the tem­poral and the nasal hemifields indicates extension and depth of a defect restricted to the hemifield. In a recent evaluation, Bynke36 found that these parameters were suitable for quantitative studies in cooperative patients. Pe'er et al.37 described a method for converting visual field isopters plotted with Goldmann pe­rimetry into digitized data for computer evalua­tion. The contours of the visual field isopters are traced with a stylus generating sparks at its end. Two linear microphone sensors located on adja­cent sides of the table measure the time required for the sound waves to reach them. The infor­mation is fed into a computer for further process­ing. This method allows area calculations, graphic display and storage of patients' data, and seems relatively inexpensive and easy to perform. Contrast Sensitivity Measurements Contrast sensitivity was found by Lund et al. 38 to be greater in the lower than in the upper field. These observations correlate with the better vis­ual acuity that has been reported in the lower part of the visual field,39 as well as with electro­physiological findings indicating a greater sensi­tivity in the lower visual field. 40 In normal subjects, better acuities were shown by Owsley et al. 41 to be associated with a gener­alized decline in sensitivity for low and interme­diate spatial frequencies. These findings do not support previous observations stating that sub­ject's acuity is not predictive of sensitivity to low and intermediate frequencies. In their analysis of changes in contrast sensitivity throughout adult­hood, Owsley et al. also noted progressively de­creased perception of intermediate and high spa­tial frequencies starting in the 20s. In addition, older adults demonstrated impairment in the processing of temporaly modulated targets. It was hypothesized that elderly subject's sensitivity loss December 1984 Safran for intermediate and high frequency bands can be attributed partially to changes in retinal illu­minance, but neural involvement was not pre­cluded. In optic neuritis, loss of sensitivity for low spatial fre~uency stimulus was found by Hess and Plant4 to be diminished with increase in temporal frequency of stimulus presentation. In contrast, loss of sensitivity for higher spatial fre­quency was independent of the temporal rate of stimulation. Importance of grating field size for evaluation of contrast sensitivit~ threshold was emphasized by Hyvarinen et al.4 A number of patients with optic nerve or macula disorders retained reason­ably good contrast sensitivity for low and inter­mediate spatial frequencies, in spite of markedly reduced visual acuity. Relative preservation of contrast sensitivity can be overlooked if sensitiv­ity is assessed by means of grating fields as small as 2° to 5° in diameter. Measuring contrast sen­sitivity thresholds with various grating field sizes is still another way of evaluating visual function. Even at suprathreshold levels, contrast percep­tion in normals differs from that of amblyopes. Loshin and Levi44 provided further evidence that at all contrast levels the amblyopic eyes show prolonged reaction times as compared to non­amblyopic eyes. Following amblyopia therapy, poor correlation was found between the effects on contrast sen­sitivity and those on visual acuity. After stripe treatment, Lundh and Lennerstrand45 observed that some subjects displayed increase contrast sensitivity while no improvement was noted in visual acuity. Since assessment of contrast sensitivity thresh­olds is now an essential part of clinical evaluation of visual function, it is important to devise easy clinical tests to be used in children. They should be fast and simple for the child to understand. Maione et al. 46 proposed to fill outlines of horses with gratings of various contrast. When the grat­ing is resolved by the eye, the picture looks like a zebra. Visual-Evoked Potentials Multiple sclerosis is probably the neurological disorder in which visual evoked potential record­ings have been the most useful. This examination technique made possible a reclassification of the clinical diagnosis of multiple sclerosis in 30% of the 500 patients studied by Lowitsch. 47 Usefulness of visual-evoked potentials in the diagnosis of multiple sclerosis condition was com­pared to that of other electrophysiological exam­inations. According to Kjaer's48 personal experi­ence with 160 patients, if multiple sclerosis pa­tients were to be examined by only one evoked 279 Recent Literature: Part III potential technique, visual-evoked response re­cording should be preferred. Indeed, in compari­son with brain stem auditory-evoked potentials and somatosensory evoked potentials, pattern re­versal visual-evoked potentials were demonstra­ted to be the most sensitive technique, as well as the easiest to perform and evaluate. Brain stem auditory-evoked potentials were found some­what less sensitive, but revealed many subclinical lesions. Somatosensory evoked potentials were as sensitive as brain stem auditory evoked poten­tials, but in most cases they only confirmed clin­ical findings. Blink reflex evaluation was a less sensitive method than evoked potential testing. Delplace and Guillaumat49 reevaluated Uthoffs phenomenon in multiple sclerosis by means of pattern visual evoked potentials. A number of diabetic subjects with no evidence of ocular changes showed altered pattern visual­evoked responses. In 10 (62%) of 16 patients studied by Puvanendran et al.,50 latency was in­creased by more than three standard deviations, often in conjunction with severe reduction in amplitude. All cases with altered visual-evoked potentials also showed delayed sensory conduc­tion in the median nerve. In a study of pattern visual-evoked potentials in patients with neurosyphilis, Conrad et al. 51 found altered responses in 16 (20%) out of 79 affected patients. A higher percentage of altered visual evoked potentials was noted in patients with tabes dorsalis than in patients with general paresis or meningovascular forms. Increase in latencies noted with neurosyphilis (up to 30 ms) was less marked than that reported in multiple sclerosis (up to about 100 ms). Sokol52 observed that in amblyopic eyes, pat­tern reversal visual evoked potentials demonstra­ted longer latency of the first and shorter latency of the second major positive waves. Since shorter latency of the second positive wave is normally elicited by low spatial frequency stimuli as a reflection of local luminance activity, Sokol hy­pothesized that the pattern of changes in latencies reported in amblyopic eyes indicated a loss of contrast-specific mechanism and activation of that of local luminance. Lithium treatment was reported by Fenwick and Robertson53 to induce a significant increase in amplitude of pattern visual-evoked potentials. In hyperactive children, stimulants speed reaction time, but without shortening visual-evoked re­sponse latency. However, as demonstrated by Halliday et aI. 54 with methylphenidate adminis­tration, minimal drug-induced changes in ampli­tude can occur. The nature of these effects varied according to age, so that opposite effects could occur in children under the age of 10 and older children. 280 Alteration in late occipital positive components in human visual-evoked potentials (about 270 ms after the stimulus onset) were interpreted by Ki­tajima et al. 55 as being due to amplitude changes of the overlapping occipital negative component occurring with latency of 250 ms, and reflecting task-specific perceptual activities. It was suggested by Whittaker and Siefried56 that low amplitude high frequency wavelets that were observed superimposed upon large slow waves reflected the earliest cortical visual proc­essing recorded in man. They usually began 35 to 40 ms after the onset of the flash stimulus and reached a maximum at the O2 electrode locus (2.5 cm above the inion), and wavelet polarities in­verted when adjacent bipolar electrode pairs were moved across the occipital region. Evaluation of Anterior Visual Pathway Function in Patients with Cataract In patients suffering from cataract, assessment of the "neural" visual acuity is important in neuro­ophthalmological evaluation. Recent papers dealt with assessment of visual function in these pa­tients by various methods, all of which, however, have been found to have limitations. Blue field entoscopy is based on perception of one's own leucocytes in the parafoveolar capil­laries upon diffuse illumination by light of 430 nm. Miris and Missotten57 investigated the extent to which blue field entoscopy was useful for prediction of postoperative visual acuity in pa­tients with cataract. They noted that all 27 pa­tients who preoperatively were able to perceive the entopic phenomenon demonstrated good postoperative visual acuity, i.e., 20/30 or better. However, the authors noted that a negative result from the test did not allow a prediction of post­operative visual acuity. The test required some cooperation from the patient. Before cataract surgery, photopic flash electro­retinogram and visual-evoked response were found by Vrijland and van Lith58 to be good indications for estimating postoperative vision. When flash-evoked potential was larger than 21 /lV, 75% of 16 eyes demonstrated postoperative visual acuity of 20/25 or better. With evoked responses of 14 /lV to 21 /lV in amplitude, 52% of 98 eyes showed good postoperative acuity. Gratings of various spatial frequencies can be generated on the retina using interference franges, independently of the optics of the eye. Two commercially available instruments, the Haag-Streit visometer and the Rodenstock retinometer, were assessed by Halliday and ROSS 59 in cataractous eyes, for comparison with postoperative Snellen acuity. Results with both instruments were essentially the same. Of the 50 Journal of Clinical Neuro-ophthalmology studied eyes, 22 achieved acuity approximately equal (±25%) to the predicted value. Twenty patients saw better and eight worse than pre­dicted. Eight patients with mature cataract failed to see the grating, a fhenomenon that had al­ready been reported.6 Conversely, patients with amblyopia had markedly higher gratin? than Snellen acuity, as also shown previously.6 Conventional Radiology The oblique lateral projection of the orbital floor used in conjunction with usual radiological examination was advocated by Laurin and Johansen62 to permit a reliable diagnosis of blow­out fractures to be reached in the majority of cases without tomography. However, it report­edly required experience for evaluation because anatomy was presented in a somewhat unusual way and the fracture was sometimes difficult to observe. Oblique lateral projection was not rec­ommended for identifying destructive changes of the orbital floor. Minor radiological sellar changes, e.g., slight irregularity of the lamina dura, asymmetry of the sellar floor, or thinning of the dorsum sellae, could not be correlated with histopathological abnormalities in a study by Sanna et al. 63 These conclusions were reached following a multidirec­tional thin section tomography performed on 62 excised sphenoidal bones, and histological eval­uation of the pituitary glands. Nuclear Magnetic Resonance Early results from examination of human orbit and brain with nuclear magnetic resonance im­aging are promising. Mosley et al. 64 reported on their preliminary experience with orbital imaging by means of nuclear magnetic resonance. Char­acteristics of various orbital tissues were pro­vided, according to imaging parameters. The lens could be clearly seen, whereas the ciliary body was poorly visualized. Aqueous, vitreous, sclera, and conjunctiva showed considerable changes in relative intensity with variation of imaging pa­rameters. Retina, choroid and sclera were remark­ably demonstrated. The optic nerve was shown on both sagittal and axial imaging, without move­ment artifacts in spite of the minimum time of 4.3 minutes for the scanning sequence. The su­perior ophthalmic vein and the optic nerve showed summation of their shadow on axial sec­tions, as with computerized tomography scan­ning. Mosley et al. emphasized that this summa­tion should not be mistaken for a lesion within the nerve. The four recti muscles and the levator palpebrae were clearly visible. With the strong signal of orbital fat, contrast was high and mus­cles were easily identified. The superior December 1984 Safran ophthalmic vein was clearly seen, showing a com­plex phenomenon called ·paradoxical enhance­ment." Mosley et al. stressed both the considera­ble superiority in many respects of nuclear mag­netic resonance imaging over computerized to­mography scanning and its limitations with re­gard to bone or calcified tissue definition. Nuclear magnetic resonance tomograms of the eye were also provided by Wollensak and Seiler.65 Wooden intraocular foreign body and ocular mel­anoma were noticeably delineated. Simmonds et al. 66 reported on the use of nu­clear magnetic resonance imaging for human brain anatomy demonstration. Sharp gray-white matter contrast is invaluable for delineating brain structures. Moreover, it allows demonstration of myelination during childhood. The same tech­nique was used by Sydder and Whitelaw67 for evaluating myelination of brain structures in chil­dren. Corston et al. 68 studied a variety of intracranial lesions by means of nuclear magnetic resonance multiplanar imaging. The technique permitted a precise assessment of extrasellar extension of pi­tuitary tumors, and their relationship to the chiasm. Empty sella could be clearly differen­tiated from pituitary tumors. References 1. Hunt, A.: Tuberous sclerosis: A survey of 97 cases. Dev. Med. Child Neural. 25: 346-357, 1983. Re­prints: Ann Hung, B.A., Research Worker, Human Development Research Unit, Park Hospital for Children, Old Road, Headington, Oxford OX3 7LQ, England 2. Winter, J.: Computed tomography in diagnosis of intracranial tumors versus tubers in tuberous scle­rosis. Acta Radial. 23: 337-344, 1982. Reprints: Dr. James Winter, Department of Radiological Sci­ences, Neuroradiology Section, UCLA School of Medicine, University of California, Los Angeles, California 90024 3. 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