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Idiopathic hypothalamic dysfunction with dilated unresponsive pupils: report of two cases.

Idiopathic hypothalamic dysfunction is a rare but well-defined entity in childhood characterized by adipsia-hypernatremia, obesity, poor thermoregulation, and disturbance of pituitary function. Two cases of idiopathic hypothalamic dysfunction are described. There are 10 previously reported cases in the literature, and the clinical features are compared. The present cases are unique in that the patients also had bilaterally dilated unresponsive pupils. In the first case, there was no demonstrable pathology at autopsy; in the second case, lymphocytic infiltration of the hypothalamus and midbrain associated with neuronal loss was present at autopsy. Possible etiologies are discussed.

Autopsy↗

Retinal ischaemia and relative afferent pupil defects in central retinal vein occlusion.

A consecutive series of 40 patients were assessed for relative afferent pupil defects and retinal ischaemia by fluorescein angiography. A strong correlation was found between density of afferent defect and area of retinal non-perfusion. There was an increasing incidence of rubeosis iridis with more severe grades of afferent defects or ischaemia.

Afferent Pathways↗

Pupil studies in depressed patients: an investigation of the mechanism of action of desipramine.

Six depressed patients were treated routinely with desipramine, a relatively selective noradrenergic uptake blocking drug. After 0, 1 and 3 weeks' treatment, pupillary responses to tyramine, phenylephrine and pilocarpine were measured using a photographic techniques. Both 1 and 3 weeks' treatment significantly inhibited tyramine and phenylephrine-induced mydriasis, but did not inhibit pilocarpine-induced miosis; in fact the longer treatment enhanced miosis due to pilocarpine. Resting pupil size was significantly increased after 1 and 3 weeks' treatment. The findings can be explained by the known ability of desipramine to block noradrenaline uptake and alpha adrenoceptors: they provide no evidence of muscarinic receptor blockade or of a slowly developing adaptation at alpha adrenoceptors.

Adult↗

The pupil in familial dysautonomia.

We performed infrared pupillography on 10 patients with familial dysautonomia. Pupillary constriction to light and accommodation was normal. There was no evidence for light-near dissociation, and tonic responses were not observed. Dilatation in darkness was normal. Ocular application of dilute pilocarpine produced miosis in all patients. Supersensitivity of the pupil to muscarinic agents in familial dysautonomia is unlikely to be explained by parasympathetic denervation. Possible explanations for this phenomenon include diminished lacrimation and corneal ulcerations.

Adolescent↗

Relative pupil-sparing third nerve palsy: etiology and clinical variables predictive of a mass.

The causes of ophthalmoplegia in 24 consecutive patients with neurologically isolated, relative pupil-sparing third nerve palsy included infarction in 10 patients, compression by tumors or aneurysms in 10 patients, and miscellaneous disorders in four patients. There was no significant difference in the proportion of patients with pain, degree of external ophthalmoplegia, or degree of internal ophthalmoplegia between the groups with infarction or mass lesions. Therefore, screening for mass lesions using neuroimaging is indicated in patients with this presentation of third nerve palsy.

Aged↗

Higher order ocular aberrations after cycloplegic and non-cycloplegic pupil dilation.

PURPOSE: Clinical aberrometry is commonly undertaken with the use of mydriatic agents, however there is no literature available on whether aberrometry results obtained under cycloplegia differ from those obtained without cycloplegia. METHODS: Higher order aberrations were measured over a 6-mm pupil with a Bausch and Lomb Technolas Zywave Aberrometer on the right eyes of 31 young subjects (average age 19.7 +/- 1.7 years; 5 females, 16 males). Two measurement conditions were used for each subject: 1) topical installation of 3 drops 1% cyclopentolate hydrochloride; and 2) topical installation of 1 drop 2.5% phenylephrine hydrochloride, prior to aberrometry measurements. RESULTS: For higher order aberrations (3rd to 5th order), average root mean square (RMS) after phenylephrine measurement (0.3852 microm) was significantly lower than after cyclopentolate (0.4259 microm). A small but statistically significant difference was found between the two conditions for average vertical and horizontal coma and, to a lesser extent, horizontal 5th order aberrations. Repeatability RMS, a measure of test-retest measurement repeatability, was similar for the two conditions at 0.15 microm, and significantly lower than the average RMS for the difference between the two conditions (residual RMS) of 0.22 microm. CONCLUSIONS: The difference between cycloplegic and non-cycloplegic aberration measurements has implications for surgical correction of higher order aberrations.

Administration, Topical↗

New iris retractor for pupil dilatation during anterior vitrectomy: double-hook iris retractor.

To report a new iris retractor as a tool for pupillary dilatation in eyes with a small pupil. This instrument was designed to prevent the retractor from dropping out of corneal incision, especially during anterior peripheral vitrectomy requiring a scleral indentation. A new iris retractor was designed with the top in the form of a double-hook, based on the conventional flexible iris retractor. The new instrument was used in 16 eyes, and the conventional flexible iris retractor was used in 14 eyes, with proliferative retinopathy or proliferative diabetic retinopathy in cases in which anterior vitrectomy was performed. The number of times that the instrument has been replaced were recorded. The conventional flexible iris retractor was replaced at mean number of 4.2 times, while the new iris retractor was replaced a mean number of 0.3 times during vitreous surgery. The difference was statistically significant (P = 0.009, Mann-Whitney test). The new iris retractor can easily hook onto the iris and is useful in anterior vitrectomy.

Eye Diseases↗

Effects of mydriatics and a miotic on ocular discomfort and pupil responses.

Mydriatics produced two different effects on the discomfort threshold following administration of the drugs depending upon whether the mydriasis was produced by paralysis of the sphincter muscle or by activation of the dilator. When the sphincter was paralyzed by tropicamide, the discomfort threshold was elevated during the period of time that the pupillary light reflex was significantly reduced to the flashing stimulus. Initially, phenylephrine-induced mydriasis had no effect on the discomfort threshold, but as the pupil responses returned to normal the threshold rapidly increased and then gradually returned to a pre-drug level. The return of the threshold back to normal seemed to parallel the decline of action of phenylphrine on the dilator muscle. Significant impairment of the light reflex by pilocarpine-produced miosis was also related to an increase of the discomfort threshold.

Humans↗

Disorders of the pupil.

The pupil is one objective marker of vision and autonomic pathways. A good understanding of its anatomy and careful examination techniques are the essential tools for proper clinical diagnosis of pupillary disorders.

Diagnostic Techniques, Ophthalmological↗

[Lasermicrosurgery in glaucoma (Lasertrabeculopuncture I1LTP], tangential irisbasecoagulation [TLC], removal of miosis and shifting of pupils) (author's transl)].

The long-term results of Lasertrabeculopuncture (LTP) and of tangential irisbasecoagulation (TIC) according to experience of 4 years are presented and critically discussed in comparison with the reports of other authors. The removal of miosis and the shifting of displaced pupils in glaucomatous and cataract eyes is demonstrated. The long-term results of other interventions in glaucoma by lasermicrosurgery are announced.

Glaucoma↗

Optic discoscopy through a miotic pupil.

A technique is described whereby visualization of the posterior pole of the retina is accomplished through a very miotic pupil. The technique is simple and quick to perform. A wide field of view is maintained as well as stereopsis.

Constriction↗

[Age effects of topical tyramine and cocaine on the pupil].

In order to obtain the age distribution of normal values of the effects of topical tyramine or cocaine on the pupil, we measured the pupillary light reflexes of 50 normal volunteers before and 45 minutes (tyramine) or 90 minutes (cocaine) after the topical instillation in right eyes. Left eyes were used as controls. An infrared videopupillogram (HTV-C301) was used. Topical tyramine as well as cocaine caused mydriasis, increase of maximum %-velocity of dilatation (%-VDmax), reduction of %-amplitude of constriction (%-A) and decrease of maximum %-velocity of constriction (%-VCmax). These alternations of pupillary parameters coincided with the changes induced by topical epinephrine. The effects of tyramine or cocaine significantly increased as the age of the subjects increased, though the increase of %-VDmax caused by cocaine was only related to age. Age-related augmentation of pupillary changes were also been observed in a previous study using topical epinephrine or topical pilocarpine which concluded that the effects of any topical autonomics were increased by aging. No physiological senile Horner's syndrome was detected. It was concluded that the age-related augmentation of pupillary changes caused by topical autonomics probably resulted from the increment of corneal permeability in the aged which was confirmed in the previous fluorophotometric study by the authors following topical fluorescein administration. It is possible to assess the pupillary sensitivity to topical adrenergics of the patients with bilateral Horner's syndrome by comparing the age-related normal values obtained in this study.

Adolescent↗

[Bilateral ptosis and upper gaze palsy with pupil sparing caused by midbrain hemorrhage].

The arrangement within the midbrain oculomotor nerve complex and crossed innervation of the superior rectus muscle are not elucidated in humans. A 65-year-old woman visited our hospital complaining of difficulty opening her eyes. Neurological examination revealed bilateral ptosis and impaired supraduction. The pupils prompty constricted to light. Results of CT and MRI demonstrated that there was a hematoma located in the dorsal midbrain tegmentum that spared the rostral mid-brain. It was presumed that the nerves of the superior rectus and levator palpebrae lie in the midbrain tegmentum more dorsally and medially than the others. Furthermore, crossed innervation of the superior rectus muscle can explain that lesions located in the dorsal midbrain tegmentum cause more often impaired supraduction.

Aged↗

Tonic pupils and human parvovirus (B19) infection.

We document the case of a young woman with bilateral internal ophthalmoplegia and subsequently tonic (Adie's) pupils, corneal epitheliopathy, and uveitis due to human parvovirus B19 infection. To our knowledge, this is the first reported case of ocular complications of this virus.

Adolescent↗

Atonic pupil: an unusual complication of cataract surgery.

Eight cases of atonic pupil, an unusual complication following cataract surgery, are presented. Six of the eyes involved had intracapsular cataract extraction and anterior chamber intraocular lens implantation; one case underwent extracapsular cataract extraction with a posterior chamber intraocular lens implantation; the other had an extracapsular procedure with no implant. The etiology is unknown, but we believe the most likely one is that the sodium hyaluronate used in all these patients caused a temporary reduction of the iris blood supply by mechanical factors or by a vasoconstriction induced by some toxicity of the material that produced a lesion on the iris sphincter.

Adult↗

Effects of tetramethylpyrazine on heart rate and pupil diameter in rats.

The alpha-adrenergic nature of tetramethylpyrazine (TMPZ), a commonly used cardiovascular drug in China, was studied with a combined bradycardia and mydriasis model in the pentobarbital anesthesized rat. Intravenous injections of TMPZ at 1-30 mg/kg failed to change heart rate or pupil size, whereas iv injections of both clonidine (alpha 2-adrenoceptor agonist, 1-30 micrograms/kg) and methoxamine (alpha 1-adrenoceptor agonist, 10-300 micrograms/kg) caused dose-dependent bradycardia and mydriasis. Pretreatment of rats with TMPZ (30 mg/kg) did not significantly change clonidine- or methoxamine-induced bradycardia or mydriasis. However, when TMPZ was injected 2 min after clonidine administration (30 micrograms/kg), while the mydriasis reached a maximum, it slightly but significantly reversed the clonidine-induced mydriasis for 15-20 s. In contrast, the alpha 2-adrenoceptor antagonist idazoxan (6 micrograms/kg) caused the same degree of reduction of the clonidine-induced mydriasis for greater than 2 min. Our results suggested that TMPZ at the doses studied did not have alpha-adrenoceptor agonistic activities, but may have slight alpha 2-adrenoceptor antagonistic activities.

Animals↗