Noradrenergic nervous activity in migraine.

The autonomic nervous function in patients with migraine was studied during headache-free intervals. The following observations were made: (1) a decrease in overshoot in Valsalva's maneuver; (2) orthostatic hypotension; (3) low levels of plasma norepinephrine in the steady state; (4) failure in elevation of the plasma norepinephrine level after head-up tilting; (5) dilatation of the pupils after instillation in the eye of 1.25% epinephrine; and (6) a long recovery time in tests by bolus injection of 0.1 microgram of norepinephrine bitartrate per kilogram. The above findings suggest that patients with migraine show sympathetic hypofunction together with denervation hypersensitivity of the iris and the arteries, and that a defective noradrenergic nervous system may play a role in the pathogenesis of migraine.

Monocular blindness with preserved direct and consensual pupillary reflex in multiple sclerosis.

A 22-year-old woman with definite multiple sclerosis had right-eye blindness with preserved direct and consensual pupillary reflex to light. The anatomy of the fibers inside the optic nerve did not give a satisfactory explanation of this phenomenon. On the other hand, this case appeared to support the idea of biochemical differences between the myelin of visual pathways and that of reflex pathways. Studies are necessary to confirm this hypothesis.

Midbrain corectopia.

Intermittent corectopia was recently observed in a patient with bilateral, rostral midbrain infarction. Results from neuropathologic examination disclosed isolated but intact Edinger-Westphal nuclei. An explanation of midbrain corectopia centered on segmental innervation of the pupil by the Edinger-Westphal nucleus. In the presence of a paralyzed dilator muscle, select, central inhibition of sphincter tone resulted in an oval and eccentric pupil.

Juvenile diabetes mellitus and optic atrophy.

Nine patients with the syndrome of juvenile diabetes mellitus and optic atrophy exemplified the wide range of manifestations of this syndrome. The disease may occur as a recessively inherited or sporadic disorder and tends to have multi-system involvement. Hearing loss, diabetes insipidus, and evidence of cerebellar or central vesitbulo-ocular dysfunction are common.

Quality of survival after out-of-hospital cardiac arrest: predictive value of early neurologic evaluation.

One hundred and seventeen patients were admitted following out-of-hospital cardiac arrest. After initial neurologic evaluation, they were followed prospectively until discharge or death. Seventeen patients were alert when admitted. Of these, four died and 10 of 13 survivors were neurologically normal. One hundred of the patients were unresponsive; of these, 60 died. Of 40 survivors, 15 were neurologically normal, at discharge; 15 could perform some self-care but were confused, and 10 required total care. Absence of pupillary light reaction, oculocephalic reflexes, purposeful response to pain, and spontaneous respirations were associated with high mortality and more severe neurologic deficits. However, some patients with usually unfavorable signs recovered good neurologic function.

Misdiagnosis of spasm of the near reflex.

A bilateral sixth nerve palsy portends serious disease of the central nervous system and precipitates extensive patient studies. Spasm of the near reflex, characterized by intermittent convergence, accommodation, and miosis, is a functional disturbance. Five patients with hysterical spasm of the near reflex erroneously diagnosed as a bilateral sixth nerve palsy are reported. The pupillary sign, intense miosis on attempted lateral gaze, is emphasized as an important clue to the correct diagnosis. Despite extensive investigation, no disease of the central nervous system was found. Neurotic or hysterical features were evident in every patient.

Hereditary sensory neuropathy and tonic pupils.

The clinical features of two siblings with both hereditary sensory neuropathy and bilateral tonic pupils are described. Other neurologic disorders associated with tonic pupils are reviewed. Our patients appear to represent a unique instance of the association of disorders that primarily affect the ciliary and dorsal root ganglia.

Fabry disease: impaired autonomic function.

Previous reports of extensive lipid accumulation within neurons of the autonomic nervous system in Fabry disease suggest an anatomicopathologic basis for the peculiar pain, diminished sweating, and gastrointestinal symptoms experienced in this disorder. To further assess autonomic function in Fabry disease, noninvasive clinical tests were performed on 10 patients. Diminished sweating was found in each; the loss was approximately uniform proximally and distally, suggesting sweat gland dysfunction rather than autonomic neuropathy. Impaired pupillary constriction with pilocarpine, and reduced saliva and tear formation were found in half the patients. Disordered intestinal mobility was demonstrated in the oldest patients. In all cases, the cutaneous flare response to scratch and intradermal histamine was diminished, and pruritus was not experienced. Signs of autonomic dysfunction are present in Fabry disease and correlate with the known lipid deposition in autonomic neurons.

Pupillary measures of binocular luminance summation in infants and stereoblind adults.

Preverbal infants and children with ocular misalignments do not perform well on standard clinical measures of binocular function which require accurate alignment of the eyes. In 1983, Birch and Held described a pupillary measure of binocular luminance summation that could be used to assess binocular function in strabismic infants. They reported a correlation between the onset age of stereopsis and the onset age of binocular luminance summation, with both emerging at the end of the fourth postnatal month. Using a similar pupillary technique, we measured significant levels of binocular luminance summation in infants as young as 2 mo of age, as well as in stereoblind adults. In addition, the infant's pupillary system, in comparison to the adult's, showed reduced magnitudes and increased latencies to luminance increments. The sensitivity of the pupillary system to luminance increments provided a better predictor of binocular luminance summation (Pearson r = 0.88) than did stereoscopic performance (Pearson r = 0.43). These data suggest that developmental changes in the pupillary system itself, rather than factors intrinsic to binocular vision, may have been the source of the correlation between binocular luminance summation and stereopsis reported by Birch and Held.

Paradoxic pupillary constriction in a patient with congenital stationary night blindness.

Paradoxic pupillary constriction to an "off-step" of light was studied in a 35-year-old woman with congenital stationary night blindness. ERG was of the Schubert-Bornschein type (scotopic B-wave absent); fundi were normal for a high myope. Paradoxic constrictions were larger in response to full field and peripheral off-steps from low photopic levels than from higher photopic levels. The patient's "on response" was smaller in magnitude and slower in both latency and dynamics than the on-response of normal subjects. Pupillary hippus was larger in magnitude and more peaked in the patient than in normal subjects. Steady-state (tonic) pupil size increased paradoxically with increased light level over the range 1-2 log fL.

Parasympathetic denervation hypersensitivity of the iris in ocular hypertension.

Seventy-eight ocular hypertensive patients and 47 age-and sex-matched control subjects were assessed for parasympathetic denervation hypersensitivity of the iris using topical application of 2.5% methacholine chloride solution. Constriction of the pupil in response to methacholine stimulation of the sphincter pupillae was significantly greater in the ocular hypertensive patients than the control group (P less than 0.001). The implications are discussed, with particular reference to the association between autonomic neuropathy and the primary glaucomas.

The visual evoked potential in glaucoma and ocular hypertension: effects of check size, field size, and stimulation rate.

In order to determine the optimum stimulus conditions for the detection of optic nerve damage due to glaucoma and ocular hypertension, checkerboard pattern reversal visual evoked potentials (VEPs) were recorded from 20 glaucoma patients, 20 ocular hypertensive patients, and 20 age-matched normals. Two check sizes (12' and 48'), two field sizes (14 degrees and 28 degrees), and two alternation rates (1.9 and 7.5 alt/sec) were used. All subjects had visual acuities of 20/40 or better in each eye and equal pupils of 2 to 5 mm diameter. The largest number of VEP abnormalities were found with large checks (48') reversing at a fast rate (7.5 alt/sec). After correcting for the effects of age, visual acuity, and pupil size, 16 of 30 eyes with glaucomatous visual field defects had abnormally long VEP latencies under this condition (beyond the 99% confidence limit of the normal subjects). Nine of 40 ocular hypertensive eyes also had abnormally long latencies. Increased pattern VEP latency was significantly correlated with both the severity and location of visual field defects and the degree of cupping and pallor of the optic disc. VEP latency was not significantly related to intraocular pressure.

Pupillary light reflex in amblyopia.

The pupillary light reflex of 15 strabismic and anisometropic amblyopes, and eight subjects who had recovered from functional amblyopia was studied by using an infrared electropupillogram. Ten of the fifteen amblyopes had significantly longer latencies of contraction when the amblyopic eyes were stimulated than when the normal eyes were stimulated. However, there was no relationship between the delay in pupillary light reflexes and reduced visual acuity of amblyopic eyes. The amplitudes and maximum velocities of the contraction were not altered significantly in amblyopic and normal eyes. All of the subjects who had recovered showed no significant difference of the latencies of the pupillary responses to stimulation between normal and amblyopic eyes. These findings indicate that a retinal mechanism in amblyopic eyes may be responsible for the abnormally long pupillary light reflex latency.

The development of eye alignment in normal and naturally microstrabismic kittens.

The development of interocular alignment was pupillographically measured from eye opening throughout the first half year of life in normal kittens and in kittens deriving from a naturally strabismic cat colony. Whereas the cyclorotatory component of age dependent changes did not differ between the two groups, the horizontal divergence of the optical axes was increased in the future esotropes during the whole observation period. Surprisingly the divergence angle of the optical axes was correlated with the convergence angle of the visual axes as determined during cortical receptive field analysis at 7-9 months of age. The authors suggest a theory that might explain the microstrabismic misalignment of the visual axes with an intraretinal defect.

Pupillary and visual field evaluation in patients with melanocytoma of the optic disc.

Twenty patients with melanocytoma of the optic disc underwent Goldmann perimetry and pupillary evaluation. Two patients (10%) had normal visual fields. Three (15%) had minimal blind spot enlargement. Fifteen patients (75%) had a greatly enlarged blind spot, of which ten had concomitant nerve fiber bundle field defects. These consisted of a nasal step in two cases (10%), relative nerve fiber bundle defects in four cases (20%), and an absolute arcuate defect in four cases (20%). The blind spot enlargement relates to the pigmented tumor extended beyond the disc boundary, thereby shading the peripapillary retina. The nerve fiber bundle defects result from injury to the visual axons passing through the nerve head, although the mechanism is uncertain. A Marcus Gunn pupillary sign produced by the melanocytoma was found in six patients (30%), all of whom had a substantial relative or absolute nerve fiber bundle defect.

Paradoxic pupillary phenomena. A review of patients with pupillary constriction to darkness.

The presence of pupillary constriction to darkness is said to be a useful indicator of retinal disease. However, it is also associated with optic nerve disease. The phenomenon has been reported in patients with congenital stationary nightblindness, congenital achromatopsia, bilateral optic neuritis, and dominant optic atrophy. We have observed this response in additional disorders, including anomalies of the optic nerve development, congenital nystagmus, and a variety of diseases affecting the retina. Notably, four of our patients with strabismus and amblyopia, but without apparent retinal or optic nerve disease, have also demonstrated this paradoxic pupil response. While the mechanism for pupillary constriction to darkness remains unclear, the finding of this response in patients without retinal or optic nerve abnormalities questions its value as a localizing sign.

Effects of routine pupillary dilation on functional daylight vision.

The visual acuity of 100 patients between the ages of 16 and 66 years, seen for routine ophthalmologic examination, was measured before and after dilation. All patients had a predilation visual acuity of 20/40 or better. Postdilation binocular visual acuity using the patients' usual correction was measured first in the office and then outdoors, both with the patient's back to and the patient facing the sun, with and without the aid of postmydriatic sunglasses. Twelve percent experienced disabling photophobia even with the use of postmydriatic sunglasses, with 3% having significant objective visual loss defined as 20/50 or worse. No objective visual loss was found in 30 controls examined outdoors before dilation, without sunglasses. We recommend that patients who have experienced significant photophobia with dilation in the past, or who have never before undergone dilation, make arrangements for transportation after a dilated examination.

Neuro-ophthalmologic signs and symptoms of cysticercosis.

Fifty-six patients with cysticercosis and neuro-ophthalmic signs were examined by me during an 11-year period. Their symptoms generally reflected increased intracranial pressure caused by hydrocephalus, subacute meningitis, cerebral masses or a combination of these effects. Papilledema (37 patients) was the commonest sign, with pupillary abnormalities (16 patients) and nystagmus (12 patients) frequently encountered. Cysticercosis is a relatively benign, although episodic, disease, as long as secondary obstructive hydrocephalus is promptly diagnosed and treated by CSF shunting procedures.

A survey study of the use of electropupillogram in predicting response to psychostimulants.

To confirm the conclusions from a previous study supporting the usefullness of electropupillogram (E.P.G.) in predicting clinical response, data from three separate studies with hyperkinetic and learning disabled (L.D.) children treated with stimulants were surveyed. Change in extent of pupillary contraction (E.C.) after a test dose of stimulant as measured by E.P.G. did not correlate significantly with actual clinical rating change (with one exception out of 14 correlations calculated). These negative results are reported with a reservation regarding their validity because of technical difficulties in data collection.

[Paradoxical pupillary reaction to light -- a pupillographic study (author's transl)]. / Paradoxe Lichtreaktion der Pupille: Pupillographische Untersuchungen

A report given of a 32-year-old female patient who exhibited a unilateral paradoxical pupillary reaction to light, but was obviously healthy in all other respects. All of the neuroophthalmological findings, including the infrared reflex-pupillographic results registered, are indicated and discussed in detail in the report. An analysis of the findings leads to the conclusion that this disturbance has a central, prenuclear location. Apart from a stenosis at the junction of the arteria vertebralis, which was determined angiographically, further examination did not reveal any pathological findings.