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Query: UMLS:C0030552 (paresis)
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Adie's syndrome is a disease of unknown etiology. We known where the damage is, and which nerves are involved. We even know something of how the nerves react after the damage is done, but we don't known what causes the primary injury. The first step in working a jigsaw puzzle is to getall of the pieces right side up and take a good look at them. Some of the jigsaw pieces handled in this paper are listed below. Some of them are new observations; many of them are old concepts, partly modified and partly made secure by new facts. 1. Not all "tonic pupils" are due to "Adie's syndrome"; some are due to local injury and some to a generalized peripheral neuropathy (Table II). 2. All patients should have serologic tests for shyphilis. In this series one in six had positive serology. 3. The incidence of Adie's syndrome in Iowa in the early 1970's was approximately 4.7 per 100,000 population per year. 4. The prevalence of Adie's syndrome, therefore, was approximately 2 per 1000. 5. The mean age of onset of Adie's syndrome was about 32.2 years (Figure 1A). 6. The sex ratio was 2.6 females to each male. 7. Right eyes and left eyes were involved at approximately the same rate (Figure 2). 8. The incidence of second eye involvement in unilateral cases was about 4% per year during the first decade of the disease (Figure 18). 9. If this rate of second eye involvement (4% per year) persists during subsequent decades, then most Adie's pupils will eventually become bilateral. 10. The incidence of Adie's syndrome in a largely caucasian patient group is independent of iris color (Figure 4). 11. Only 10% of patients with Adie's syndrome had completely normal muscle stretch reflexes. 12. The muscle stretch reflexes in the arms were just as frequently imparied as those in th elegs, but the degree of impariment tended to be more severe in the ankles and triceps. 13. When there was any light reaction remaining in an Adie's pupil, a segmental paralysis of the sphincter muscle could be seen. 14. The near reaction of the pupil was often segmental and frequently involved segments which did not respond to light. 15. The segmental paralysis to light was randomly distributed around the sphincter (Figure 6B). 16. There was some tendency for the sphincter palsy to gradually become worse. This progression was also random (Figure 8). 17. Almost all patients with Adie's syndrome had an accommodative paresis at the time of onset. 18. Reading glasses given to a patient with a fresh Adie's pupil were soon discarded as accommodation recovered (Figure 9). 19. Accomodative effort induced an astigmatism in about half of the eyes with Adie's--presumably the result of a segmental palsy of the ciliary muscle. 20. Tonicity of accommodation was present in about 1/2 of the eyes with Adie's, making it difficut for the patient to maintain a steady level of ciliary muscle tone. 21. An occasional patient complained of brow ache from ciliary spasm with near work. 22. In most patients with Adie's syndrome the ciliary muscle was supersensitive to pilocarpine 0...
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PMID:Adie's syndrome: some new observations. 61 31

Ciliary muscle function in patients with Adie's syndrome was studied retrospectively in 122 patients and prospectively in 17 patients. When a careful history was taken, two thirds of the patients had ciliary muscle-related symptoms. Most of the patients with Adie's syndrome had a moderate accomodative paresis, but there was a strong tendency for the ciliary muscle to recover with time. Many patients showed a tonicity of accommodation, especially those who had had the condition for several years. Astigmatism could be induced with accommodation in one third of patients. This may be related to segmental paralysis of the ciliary muscle. Seventy-three percent of the patients showed supersensitivity of the ciliary muscle in the involved eye, when both eyes were treated with a mixture of 0.25% pilocarpine hydrochloride and hydroxypropyl methylcellulose (Isopto Carpine). This may be a clinical aid in diagnosing Adie's syndrome. Two patients were found to have bilateral ciliary muscle dysfunction but only unilateral pupillary abnormalities. These two patients may have had a "pupil-sparing" Adie's syndrome.
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PMID:Ciliary muscle dysfunction in Adie's syndrome. 64 91

In a 12-year-old girl with Horner's syndrome and an ipsilateral accommodative paresis, intravenous administration of edrophonium chloride improved the accommodative defect on several occasions, which suggested a possible relationship to myasthenia gravis. Additional testing of the accommodative paresis revealed some of the characteristics of Adie's syndrome and suggested a relationship to pupil-sparing Adie's syndrome.
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PMID:Horner's syndrome with ipsilateral accommodative paresis. 67 22

Adie's syndrome comprises a tonic pupil, which may be associated with impairment of accommodation, in the presence of diminished or absent deep tendon reflexes. We report a case of a 4-year-old boy with Adie's syndrome in which latent hypermetropia was made manifest by accommodative paresis and resulted in reversible amblyopia.
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PMID:Adie's syndrome as a cause of amblyopia. 366 64

A 46-year-old woman observed transient unilateral mydriasis during a classic migraine attack. One week later she experienced a similar episode after which anisocoria was persistent. Subsequent examination showed the clinical and pharmacologic features of a postganglionic parasympathetic paresis (Adie's tonic pupil). This case confirms the hypothesis that transient mydriasis accompanying migraine is due to interruption of parasympathetic innervation rather than sympathetic overactivity.
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PMID:Adie's tonic pupil secondary to migraine. 778 May 72

Irene E. Loewenfeld, PhD has devoted a long and vigorous professional life to understanding the workings of the pupil of the human eye. Her interest in the pupil began in 1940 when she went to work as a technician in the pupillography laboratory of Professor Otto Lowenstein at New York University. It culminated in her widely admired textbook The Pupil, published in 1993. Among her many contributions, Loewenfeld provided rigorous observations about Adie tonic pupil, anisocoria in optic tract lesions, Argyll Robertson pupil, oculomotor paresis with cyclic spasms, and innovations in electronic recordings of pupil movement.
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PMID:Irene E. Loewenfeld, PhD Physiologist of the pupil. 1684 17

Harlequin syndrome (HS) is a rare disorder of the sympathetic nervous system which presents with unilateral decreased sweating and flushing of the face, neck, and chest in response to heat, exercise, or emotional factors. The contralateral side displays a compensatory overreaction to provide normal heat regulation of the face as a whole. In the literature, most of the cases are primary in nature and no underlying cause could be identified. Harlequin sign is used to denote these symptoms in patients who also exhibit associated oculosympathetic paresis, such as Horner syndrome, Adie syndrome, and Ross syndrome.We report a rare case of a 13-year-old boy who presented with complaints of flushing and sweating of the left side of the face after exertion, while the right side remained dry and maintained its normal color. No structural abnormality was identified on detailed work up. Thus, diagnosis of classic idiopathic HS was made. Despite the rarity of this syndrome, dermatologists should be acquainted with this distinctive entity and should refer the patient for complete ophthalmological and neurological examination.
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PMID:Harlequin syndrome: a mask of rare dysautonomic syndromes. 2593 78