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At least 19 recordsLinked to original sources

Disordered pupil reactivity in Parkinson's disease.

Different varieties of pupillary abnormalities have been described in Parkinson's disease but it is not clear if this is due to the disease itself or to the pharmacological treatment. In this study, pupil reactivity to various stimuli (dark/light adaptation, light reflex, near vision reaction and electrical sural stimulation) was investigated in 23 de novo parkinsonian patients (not on drugs) to assess pupillary abnormalities and investigate their pathophysiological mechanisms. Parkinsonian patients exhibited larger pupil diameter after light adaptation, as well as a reduced amplitude of contraction and a prolonged contraction time at light reflex. The data obtained confirmed the existence in Parkinson's disease of a pupillary imbalance mainly involving the parasympathetic system.

Adaptation, Ocular

Pierson syndrome with numerous dilated tubules masquerading as autosomal recessive polycystic kidney disease: a case report.

Pierson syndrome, characterized by congenital nephrotic syndrome, ocular abnormalities, and neurological defects, is caused by biallelic pathogenic variants in LAMB2. LAMB2 encodes laminin β2, a key component of basement membranes that is predominantly expressed in the glomeruli, eyes, and neuromuscular junctions. The renal histopathology of Pierson syndrome typically shows diffuse mesangial sclerosis (DMS), with occasional tubulointerstitial atrophy and fibrosis. We report a case of Pierson syndrome characterized by DMS and prominent tubular dilatation. A fetal ultrasound at 23 weeks of gestation revealed hyperechoic kidneys, which gradually enlarged, accompanied by the onset of anhydramnios from 31 weeks. The patient was delivered at 39 weeks of gestation, weighing 3,132 g, without placentomegaly. Postnatal respiratory failure due to pulmonary hypoplasia required extracorporeal membrane oxygenation, and hemodialysis was initiated for anuria. Left nephrectomy was performed on day 8 of life, revealing replacement of the renal parenchyma by numerous irregularly dilated tubules with eosinophilic casts. The right kidney reached maximal enlargement by 1 month of age and subsequently began to shrink. Ocular findings included bilateral microcoria and cataracts. Whole-exome sequencing identified compound heterozygous truncating variants in LAMB2 (p.Gln1507Ter and p.Gln1622Ter). This case highlights the need to consider Pierson syndrome in the differential diagnosis of prenatally detected hyperechoic and enlarged kidneys, in addition to polycystic kidney disease.

Female

Review of Horner's syndrome and a case report.

Sympathetic denervation of the eye [Horner's Syndrome (HS)] usually presents as ptosis, miosis, and facial anhydrosis. HS presents a challenge to the clinician because the causative lesion may involve a first, second, or third-order neuron. This paper reviews the literature regarding HS, the anatomy of the sympathetic pathway to the eye, the diagnosis, and the localization of the lesion. Our patient developed reversible HS after a migrainous episode which presumably caused "bruising" of the sympathetic plexus within the carotid canal.

Female

[Value of the discover mental defects for the pathogenesis of psychosocial disorders by normal pupils--a study about patients of a neuropsychiatric station for children (author's transl)].

200 normal pupils were tested, to discover mental defects by HAWIK IQ less than or equal to 84. All tested pupils were treated stationary for psychosocial disorders. 37 mental defects were demonstrable. The mental defect gets the third rank in the quantity of pathological causes for psychosocial disorders. The atmosphere reactive disorders head the list followed by partial psychological disorders of performance. The early recognition of these defects is of necessity in medical, psychological and pedagogic view.

Child

Parkinson's syndrome induced in cats by the use of 6-hydroxydopamine. Observations of behavior and motor disorders.

The possibility of inducing Parkinson's syndrome in cats was investigated in three kinds of lesions: by microinjection of 6-hydroxy dopamine (6-OHDA) into the pars compacta of substantia nigra (SNC), bilateral injection into the SNC and globus pallidus (GP) and into the SNC and caput nuclei caudati (NC). In all three kinds of lesions of the dopaminergic system disturbances of behavior involving specially the motor system were obtained, corresponding to the parkinsonism syndrome--in the form of bradykinesia-akinesia, increased muscle tonus of plastic type, vegetative disorders (sialorrhea, pupils) and psychic disorders such as lack of interest in the surroundings and food. The character of the enhanced muscle tonus typical for extrapyramidal disturbances was confirmed by EMG examination. The parkinsonism-like syndrome induced in the cats was transient and receded after several weeks.

Animals

Methylphenidate v. placebo--a randomised double-blind crossover study in children with the attention deficit disorder.

Fourteen pupils of the New Hope School in Pretoria who were considered to be responsive to methylphenidate were randomised to receive either the active drug or placebo in a double-blind trial for each of 4 weeks. Their behaviour was monitored by parents and teachers, who were required to complete a questionnaire (Conner's Abbreviated Teachers' Rating Scale) three times per week. After analysis of the data, only 2 children were identified as methylphenidate responders and 1 as a probable responder. One child showed significant deterioration on the drug while another showed deterioration that approached significance. The remaining 9 exhibited no significant response to methylphenidate.

Attention Deficit Disorder with Hyperactivity

Autonomic disorders in idiopathic parkinsonism.

Clinical evidences and experimental data on various signs and symptoms of autonomic dysfunction in idiopathic parkinsonism are reported and their characteristics related to the effects of the drugs usually administered for the therapy of the extrapyramidal symptoms. The use of instrumental diagnostic criteria may sometimes help to detect alterations of autonomic functions (micturition disturbances, disorders of pupil reactivity, orthostatic and postprandial hypotension, temperature dysregulation), otherwise not easily identifiable. The existence of a large involvement of autonomic structures both at central and peripheral levels underlines the concept of Parkinson's disease being part of a wide spectrum of degenerative disorders where multiple nervous structures are at risk.

Antiparkinson Agents

Neuro-ophthalmology.

The anatomy, neurophysiology, semiology, and pathology of the pupillary reflexes are reviewed. Recent advances in the demonstration of midbrain pathways projecting to and from the Edinger-Westphal (EW) nucleus are discussed. Observations of the pupillary diameter and reflexes in premature infants can be helpful in the diagnosis of neurological disorders. A relative afferent pupillary defect (RAPD) without visual disturbances can be suggestive of midbrain lesions. Automated pupil perimetry is proposed as an objective method for the evaluation of the visual field. Tonic pupil, the pupil in diabetics, and blue-cone monochromatism are also discussed.

Accommodation, Ocular

Associated neurologic and ophthalmologic findings in congenital oculomotor nerve palsy.

Unlike acquired oculomotor nerve palsy in childhood, the congenital form is usually considered to be an isolated disorder, not associated with other neurologic or systemic disease. In a series of 14 patients with this diagnosis, 10 had associated neurologic disorders. Clinical-neuroradiologic correlation identified the brainstem as the site of the lesion in some patients. The ipsilateral pupil was involved in 12 patients and was miotic with a trace or no reaction to light on the side of the palsy in 8 of the 12 patients. In two of these eight patients, the pupil was documented to be dilated and fixed in early infancy and subsequently became miotic at several months of age, concurrent with emergence of other signs of aberrant reinnervation. Pupillary miosis as a result of aberrant reinnervation appears to be much more frequent after congenital than acquired pupillary-involving oculomotor palsy. Four patients showed better visual acuity in the paretic eye, an unexpected finding. The paretic eye appeared to have a fixational advantage if the patient had nystagmus, presumably due to asymmetric dampening of the nystagmus. Significant anisometropia and astigmatic errors in the paretic eye were present in essentially all patients.

Adolescent

Pupillary and electroretinographic abnormalities in a family with neuronal intranuclear hyaline inclusion disease.

Abnormal pupillary function and a severely depressed electroretinogram were found in four members of a family with neuronal intranuclear hyaline inclusion disease, an idiopathic degenerative disorder that involves the central and peripheral nervous systems. Symptoms were limited to the gastrointestinal system and consisted principally of abdominal pain, constipation, and severe weight loss. The discovery of light-fixed pupils in the propositus led to the first antemortem diagnosis by rectal biopsy in two generations of this family. Abnormalities of gastrointestinal motility and pupillary reactions constituted the only objective evidence of autonomic dysfunction; the abnormal electroretinogram was the only evidence of central nervous system dysfunction.

Adult

Anisocoria in the pigmentary dispersion syndrome.

We studied four patients with anisocoria and the pigmentary dispersion syndrome. In all patients, the larger pupil was on the side of the greater iris transillumination. The amount of anisocoria was between 0.5 and 1.5 mm and was the same in both the light and dark. There was no blepharoptosis, and all pupils dilated normally with cocaine. All of the patients were young men with myopia, and one patient had increased intraocular pressure.

Adult