Concurrence of Hyperhidrosis and Hypohidrosis in Ross Syndrome.

Ross Syndrome is a rare disorder characterized by tonic pupils, hyporeflexia, and abnormal segmental sweating. The pathophysiology of the disease remains unclear, with either hypohidrosis or hyperhidrosis reported in individual patients. We present the case of a man, aged 57 years, who presented with hyperhidrosis in his right extremities, anhidrosis in the left extremities, and changes in his pupils. The disease was not associated with markers of autoimmune disease, which supports recent research findings on the role of neurodegeneration. The patient's son was exhibiting similar symptoms, which implicates genetic inheritance in the process. A multidisciplinary approach is crucial for the diagnosis and ultimate management of patients with Ross Syndrome.

Pupillotonia after endolaser retinopexy during vitrectomy for retinal detachment: a prospective cohort study comparing circumferential and focal retinopexy.

PURPOSE: To determine differences in postoperative pupil diameter in eyes that undergo pars plana vitrectomy (PPV) for rhegmatogenous retinal detachment (RRD) with endolaser retinopexy (ELR), comparing 360° vs focal ELR. METHODS: Patients with uncomplicated RRD who underwent PPV were prospectively analysed regarding the postoperative pupil diameter difference (PDD) between the affected eye and the partner eye. Group 1 underwent 360° ELR and group 2 received focal ELR. Postoperative vision and complications, including redetachment rate, macular oedema and epiretinal membrane formation, were also compared. RESULTS: A total of 72 patients, 42 in group 1 and 30 in group 2, were analysed. PDD, as observed at 6 weeks, was significantly greater than the preoperative values in both groups 1 and 2. It increased by a mean of 1±1.11 mm in group 1 and by 0.5±0.78 in group 2. This initial increase in PDD receded over time, but remained statistically significant in both groups, even at 6 months. The top 20% of patients with the largest PDD change comprised 13 out of 15 eyes from group 1, which was a statistically significant overrepresentation (p=0.0435). CONCLUSIONS: Moderate pupillotonia was induced post-ELR in vitrectomy and correlated to the extent of ELR. The pupillotonia effect of ELR was significantly less marked in pseudophakic eyes.

Is Ross Syndrome an Autoimmune Entity? A Case Series of 11 Patients.

BACKGROUND: Ross syndrome is diagnosed by the presence of segmental anhidrosis, areflexia, and tonic pupils. Fewer than 60 cases have been described in literature so far. There have been reports of presence of antibodies in such patients, suggesting an autoimmune pathogenesis. METHODS: We describe the clinical profile in this case series of 11 patients with Ross syndrome and discuss the current status of autoimmunity in its pathogenesis and the management. RESULTS: Of the 11 patients with Ross syndrome there was an almost equal sex distribution (male:female ratio was 1.17:1) and the mean age of onset of symptoms was 26 years. Patients took an average of 6 years to present to a tertiary center. Sixty-three percent of the patients presented with complaints of excessive sweating, whereas only 27% had complaints of decreased sweating over a particular area of the body. Only 45% of the patients had the complete triad of Ross syndrome, which included segmental anhidrosis, tonic pupil, and absent reflexes. Eighty-nine percent of the patients had documented absent sympathetic skin response on electromyography. The various markers of autoimmunity were negative in all patients who were investigated for the same in this series. Ninety percent of the patients were managed conservatively. CONCLUSIONS: These findings suggest that, in Ross syndrome, generalized injury to ganglion cells or their projections are not purely autoimmune-mediated.

An approach to anisocoria.

PURPOSE OF REVIEW: Anisocoria is a finding seen on a daily basis in nearly every eye clinic. Although often benign, it can also represent the sole sign of a life-threatening disease making an up-to-date understanding of pathophysiology and diagnosis essential for anyone practicing medicine. RECENT FINDINGS: Many aspects of the traditional approach to anisocoria still hold true today, but advancements in imaging technology and changing trends in pharmacologic diagnosis and localization have led many to rethink that approach. In addition, the differential diagnosis for anisocoria continuously expands with identification and improved understanding of causal disease processes. SUMMARY: The present article discusses an approach to the classic anisocoria diagnostic algorithm modified by current knowledge from the most recent literature.

[Cessation of anesthesia after newly occurring anisocoria without light reflexes]. / Abbruch der Anästhesie bei neu aufgetretener Anisokorie mit fehlenden Lichtreflexen.

Anisocoria during general anaesthesia is rare. The combination of anisocoria and absence of any reaction to light is highly suspicious of an intracerebral lesion. This article reports the case of a patient with known Adie's syndrome where anisocoria led to an unnecessary immediate interruption of anesthesia. A preoperative examination, including inspection of the pupils, frequent perioperative check ups and documentation of diagnostic findings, even if negative, has to be standard for every anesthesia.

[Adie's tonic pupil and migraine: a chance association?]. / Pupille tonique d'Adie et migraine: une association fortuite?

INTRODUCTION: Adie s tonic pupil is defined as a dilated pupil, constricting poorly to light and exhibiting light-near dissociation. CASE REPORT: The authors report an 18-year-old woman diagnosed with transient bilateral Adie's tonic pupil during a migraine attack. Work-up including neuroiaeaging disclosed no underlying detectable abnormalities. DISCUSSION: The association between tonic pupil and migraine is rarely reported. This type of transient mydriasis could result from postganglionic parasympathetic dysfunction, affecting the iris sphincter. This situation is different from the heterogeneous group with benign episodic unilateral mydriasis. CONCLUSION: Transient Adie's tonic pupil may be associated with migraine attacks. This association should be known to avoid unnecessary examinations.

[Segmental anhidrosis associated with Adie's pupil: an incomplete case of Ross syndrome]. / Anhidrosis segmentaria asociada a pupila de Adie: un caso de síndrome de Ross incompleto.

We present the case of a 43-year-old woman who presented with a localized area of anhidrosis on the upper left trunk and ipsilateral upper limb. It was associated with Adie's tonic pupil and an area of compensatory hyperhidrosis on the contralateral side. After 9 years of follow up, the alterations have remained stable. These alterations of the autonomous nervous system correspond to an incompletely expressed Ross syndrome.

A case of angle closure glaucoma caused by plateau iris and adie's pupil.

PURPOSE: To describe a case of plateau iris associated with bilateral Adie's pupil. DESIGN: Interventional case report. METHODS: A 54-year-old woman presented with pain in her right eye and headache. Intraocular pressure was 34 mm Hg in the right eye. Light reflex was defective bilaterally. RESULTS: Ultrasound biomicroscopic imaging revealed normal anterior chamber depth and narrow angle. The ciliary processes were situated anteriorly. The eyes showed supersensitivity to 0.125% pilocarpine. The patient was diagnosed as having bilateral angle-closure glaucoma induced by Adie's pupil and plateau iris. CONCLUSION: Mild pupillary dilation caused by Adie's pupil may have played a role in the development of angle closure in the plateau iris configuration of our patient.

Adie's tonic pupil-induced angle-closure glaucoma.

A 52-year-old woman was diagnosed as having Adie's tonic pupil in her right eye. She reported few episodes of blurring of vision in her right eye in the recent few months. On one of the routine follow-up visits, right intraocular pressure (IOP) was 70 mm Hg and on gonioscopy the angle was closed 360 degrees. Medical treatment resulted in IOP reduction and laser iridotomy was then performed. This is, to our knowledge, the first description of intermittent angle-closure glaucoma attacks induced by a tonic pupil. The possibility of angle-closure glaucoma should be considered in patients with a tonic pupil, especially with symptoms of blurred vision or ocular pain.

Asymmetric pigment dispersion in a patient with the unilateral Adie pupil.

PURPOSE: Pigmentary glaucoma is a bilateral disorder. When it occurs asymmetrically or unilaterally, a cause should be sought because it may help us to understand the pathophysiology of this condition better. We describe a patient with unilateral pigmentary glaucoma and the Adie pupil in the same eye and discuss the possible role of the Adie pupil in the development of the pigmentary glaucoma. METHODS: A case report. A 37-year-old woman presented with asymmetric pigment dispersion resulting in pigmentary glaucoma in the right eye and an unilateral Adie pupil in the same eye. RESULTS: We believe that the dilated Adie pupil in the right eye might have resulted in a decreased amount of relative pupillary block. This, then, might have led to an increased contact between the posterior iris and the zonules, resulting in greater pigment dispersion and trabecular obstruction in the right eye. CONCLUSION: It has been proposed that the pigment is liberated from the iris pigment epithelium because of the mechanical rubbing of the posterior iris against the anterior zonular packets. Our case supports this proposed mechanism of pigment release.

Tonic pupil associated with congenital neuroblastoma, Hirschsprung disease, and central hypoventilation syndrome.

PURPOSE: To report the association of tonic pupil, congenital neuroblastoma, Hirschsprung disease, and central hypoventilation syndrome. METHODS: Case reports. RESULTS: Two infants with central hypoventilation syndrome and Hirschsprung disease were evaluated for dilated, nonreactive pupils present since birth. In both cases, pharmacologic testing with dilute pilocarpine confirmed denervation supersensitivity consistent with tonic pupil. The diagnosis of congenital neuroblastoma was subsequently established in both infants. CONCLUSIONS: The close association of these conditions in these two young children supports the concept of a common neural crest abnormality being present. A careful systemic evaluation to rule out congenital neuroblastoma should be performed in any young child who presents with tonic pupil in the setting of central hypoventilation syndrome and Hirschsprung disease.

Transient tonic pupils in botulism type B.

We report a 29-year-old woman who developed unilateral unreactive mydriasis and cycloplegia after 5 days of persistent constipation. During the next hours the patient complained of dry mouth and difficulties in swallowing food; iris and ciliary muscle palsies spread over the second eye. Ocular motility was normal and there were no clinical signs of neuromuscular involvement. Conventional electromyography and evoked muscle action potentials following repetitive nerve stimulation were normal; single-fiber electromyography showed normal jitter and absence of blocking. The diagnosis of botulism was considered as most likely, and the patient was given botulinum antitoxin. The post-treatment course was characterized by bilateral tonic pupillary reaction to near, sectoral iris contractions to light and pupillary constriction to 2 mm in 40 min following topical instillation of 0.1% pilocarpine. Ocular manifestations completely disappeared within 5 weeks. Botulism type B toxin was demonstrated in the pretreatment stool of the patient but not the serum.