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In most instances the patient with pupillotonia presents with under accommodation in the affected eye. An unusual case of Adie's pupil with ciliary spasm is discussed. As a result of the unpredictable accommodative response, the necessity of evaluating and prescribing for binocular balance is underscored. Periodic reevaluation of accommodation in relation to symptomatology is emphasized.
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A 45-year-old woman presented with a 10 year history of asymmetrical facial flushing and sweating after exertion or in hot weather. During these episodes the right side of her face remained dry and white, while the left side normally flushed. Sweating was impaired on the left side in the limbs and trunk. She also had areflexia in the lower limbs and slow pupillary reactions to light and darkness, as seen in Adie's syndrome. The topography of the sweating disorder suggested that the lesion involved the sympathetic pathways at the level of spinal cord. The relationship with the harlequin syndrome and related disorders is discussed.
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The authors present a case of bilateral tonic pupil and discuss clinical and physiopathological aspects of this syndrome. They discuss some elements necessary for the understanding of the etiopathogenesis of the disease.
A patient with pre-existing bilateral tonic pupils presented with simultaneous bilateral glaucomatocyclitic crisis. Deep tendon reflexes were absent although they were documented to be present 6 years ago. A possibility of a progressive autonomic dysfunction in both these conditions is discussed.
Ross syndrome is characterized by a triad of tonic pupil, areflexia and segmental hypohidrosis. Hypohidrosis may be accompanied by contralateral hyperhidrosis, probably due to a compensatory mechanism. We report a case of Ross syndrome with socially disabling left-sided hyperhidrosis. Sympathectomy of the second and third thoracic ganglia was performed with satisfactory results. With excellent results in primary hyperhidrosis and very low morbidity, thoracic sympathectomy is the definitive treatment for hyperhidrosis.
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Pupil is richly innervated by autonomic nerves. Therefore, its size or movement well reflects the autonomic nervous function. By analyzing dynamics of pupil movement for light stimulus followed by infrared video-pupillography, the abnormality of autonomic nervous system can be expressed in a quantitative manner. Clinical applications of pupillography and pharmacological examination to Horner syndrome, Adie syndrome, diabetes mellitus, pupillary involvement of Behçet disease, pupil of brain death and patients with chemical sensitivity have been described. Segmental spasm of iris in Horner and Aide syndrome, especially, nasal side of iridoplegia was seen in early stage of Aide syndrome from pupillary dynamics to light stimulus. An involvement of sympathetic nerve subserving iris dilator muscle was confirmed from pupillography, drug reaction to topically applied l-epinephrine in the diabetics. An involvement of cholinergic nerve which subserve the iris sphincter muscle was seen in Behçet disease when there was no manifest eye involvement. Mild mydriasis in brain death patients was seen under denervation of central nervous system. Dysfunction of sympathetic nerve from pupillary light reflex in chemically sensitive patients for cholinated hydrocarbon pesticides.
In general, Adie's syndrome does not represent a serious diagnostic problem. In cases of cerebrospinal syphilis the Argyll-Robertson phenomenon or even absolute pupillary rigidity will be observed. Positive syphilis reactions will clarify these cases. The erroneous diagnosis of brain tumour may arise because of anisocoria. However, if pupillotonia can be ascertained by means of conjunctival instillation of drugs no further invasive diagnostics will be necessary. In some cases of Adie's syndrome concomitant emotional instability may be present. This dysautonomic reaction type of personality will hardly ever be confounded with the psychological disturbances encountered in brain tumours. Therapy is restricted to symptomatic measures. Pupillotonia can be influenced by strychnine and thymoxamine. Headache and facial neuralgia respond to the usual therapeutic schemes. Sweating disorder cannot be influenced. Neuropharmacological treatment may become necessary in periods of acute emotional disturbance.