Bilateral tonic pupils secondary to anti-GD2 antibody therapy for neuroblastoma.

We report the case of a 12-year-old girl with stage 4 neuroblastoma who developed tonic pupils secondary to immunotherapy with dinutuximab, an anti-GD2 antibody, based on the timeline provided by her mother with regard to onset of symptoms. The patient presented with difficulty reading and chronic dilated pupils bilaterally, according to her mother's observations over 6 months prior to presentation. Testing with dilute pilocarpine supported our presumption of tonic pupils.

Bilateral tonic pupil during a migraine attack: a case report / Pupila tônica bilateral durante crise de enxaqueca: relato de caso

INTRODUCTION:Tonic pupil or Adie's pupil occurs due to parasympathetic denervation, and it is characterized by mydriasis with little or no response to light, with pupillary contraction to accommodation. It is caused by eye pathologies, such as infections, trauma, neoplasms, inflammatory diseases, and systemic diseases with autonomic dysfunction. Few cases have been reported of bilateral tonic pupils associated with migraine attacks. CASE REPORT: Our aimed to describe the case of a young female patient with a history of chronic migraine without aura, who presented acutely with bilateral pupillary mydriasis during a migraine attack, characterized as tonic pupil, and to discuss the possible causes of mydriasis during a migraine attack.

INTRODUÇÃO: A pupila tônica ou pupila de Adie ocorre devido à denervação parassimpática e é caracterizada por midríase com pouca ou nenhuma resposta à luz, com contração pupilar à acomodação. É causada por patologias oculares, como infecções, traumas, neoplasias, doenças inflamatórias e doenças sistêmicas com disfunção autonômica. Poucos casos foram relatados de pupilas tônicas bilaterais associadas a crises de enxaqueca. RELATO DE CASO: Nosso objetivo foi descrever o caso de uma paciente jovem, com história de enxaqueca crônica sem aura, que apresentou agudamente midríase pupilar bilateral durante uma crise de enxaqueca, caracterizada como pupila tônica, e discutir as possíveis causas da midríase durante uma crise de enxaqueca. ataque de enxaqueca.

Tonic pupil in child after chicken pox.

This is a case report of a 3-year-old boy who presented with unilateral anterior uveitis and tonic pupil following varicella-zoster virus (VZV) Infection. The patient had red and irritated eyes and photophobia. Ophthalmological findings included anterior uveitis and tonic pupil accompanied by reduced vision and accommodation. An MRI of the cerebrum was normal. To ease the symptoms the patient was prescribed photophobia glasses with correction of hyperopia. Tonic pupil due to VZV infection is a rare complication, but may have long-term consequences, why patients with eye-involving VZV infection need to be examined by an ophthalmologist.

Ross syndrome with chronic cough and RF positivity: a case report.

Ross syndrome is a rare disorder of unknown etiology, characterized by the triad of segmental anhidrosis, tonic pupil, and areflexia/hyporeflexia. Ross syndrome is thought to be a limited and selective ganglioneuropathy. Its etiology has not been fully elucidated. Autonomic findings may also accompany. We wanted to present our 25-year-old patient who was diagnosed with Ross syndrome and presented with complaints of inability to sweat, heat intolerance, headache, diarrhea and chronic cough. Keyword: cough, tonic pupil, anhidrosis, compensatory.

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.

A pupillometric investigation of state regulation in adults scoring high versus low on ADHD symptomatology.

According to the state regulation deficit (SRD) account, ADHD is associated with difficulties regulating tonic arousal levels, which may be due to inefficient effort allocation. We aimed to test the SRD account by using a target detection task with three different event rates (ER; 700 ms, 1800 ms, 6000 ms), in order to manipulate the tonic arousal state and its effects on performance and pupil indices in adults with high (n = 40) versus low (n = 36) ADHD symptom levels. In an additional condition, a fast ER (700 ms) was accompanied by auditory white noise (WN), to further increase tonic arousal level. The ER manipulation had a clear effect on RT and variability of RT. These effects were more pronounced for the high-ADHD group, especially for variability of RT with decreasing ER, suggestive of deficient upregulation of a tonic arousal state in that group, in line with their self-reported SRDs in daily life. Adding WN to the fast condition led to more errors, however similarly for both groups. Contrary to our predictions, the ER manipulation had no effect on tonic pupil size (as a measure of tonic arousal). Phasic pupil amplitude (as a measure of cognitive effort) linearly increased with decreasing ER, suggesting more effort allocation during slower ERs. WN decreased phasic pupil amplitude, but had no impact on tonic pupil size. Importantly, however, no ADHD-related differences were present for the pupil indices. In conclusion, adults with elevated levels of ADHD symptoms reported more SRDs in daily life and showed a performance pattern that suggests difficulties in upregulating but not downregulating the tonic arousal state. Surprisingly, these findings were not accompanied by group differences in pupillometric indices. This casts some doubts on the relationship between these measures of autonomic nervous system activity and state regulation, in particular in the context of ADHD symptomatology.

Zoom behavior during visual search modulates pupil diameter and reflects adaptive control states.

Adaptive gain theory proposes that the dynamic shifts between exploration and exploitation control states are modulated by the locus coeruleus-norepinephrine system and reflected in tonic and phasic pupil diameter. This study tested predictions of this theory in the context of a societally important visual search task: the review and interpretation of digital whole slide images of breast biopsies by physicians (pathologists). As these medical images are searched, pathologists encounter difficult visual features and intermittently zoom in to examine features of interest. We propose that tonic and phasic pupil diameter changes during image review may correspond to perceived difficulty and dynamic shifts between exploration and exploitation control states. To examine this possibility, we monitored visual search behavior and tonic and phasic pupil diameter while pathologists (N = 89) interpreted 14 digital images of breast biopsy tissue (1,246 total images reviewed). After viewing the images, pathologists provided a diagnosis and rated the level of difficulty of the image. Analyses of tonic pupil diameter examined whether pupil dilation was associated with pathologists' difficulty ratings, diagnostic accuracy, and experience level. To examine phasic pupil diameter, we parsed continuous visual search data into discrete zoom-in and zoom-out events, including shifts from low to high magnification (e.g., 1× to 10×) and the reverse. Analyses examined whether zoom-in and zoom-out events were associated with phasic pupil diameter change. Results demonstrated that tonic pupil diameter was associated with image difficulty ratings and zoom level, and phasic pupil diameter showed constriction upon zoom-in events, and dilation immediately preceding a zoom-out event. Results are interpreted in the context of adaptive gain theory, information gain theory, and the monitoring and assessment of physicians' diagnostic interpretive processes.

Pupillary Abnormalities After Excision of Orbital Cavernous Venous Malformations.

AIMS: The aim of this study was to determine the incidence and recovery rate for pupillary abnormalities after excision of orbital cavernous venous malformations (OCVMs). METHODS: Retrospective case-note and imaging review for patients with OCVMs, with an estimation of odds ratios (ORs) in relation to the position of the mass and surgical approach. RESULTS: The 287 included patients (176 female; 61%) presented at a mean age of 46.5 years: 243/287 (85%) OCVMs were intraconal, 253/287 (88%) located in the posterior two-thirds of the orbit, and 29/287 (11%) wedged tightly in the apex. Pupillary changes-varying from minor sectoral paresis (only evident on slit-lamp examination) to a marked mydriasis-were noted in 21% (60/287) of patients and were strongly associated with removal of intraconal (60/243 [25%]; p < 0.001), apical (14/29 [48%]; OR, 4.3; p < 0.001), inferior or inferotemporal (41/105 [39%]; OR, 5.5; p < 0.001) masses. Lateral approaches-either with (23/129; 18%) or without (30/71; 42%) osteotomy-were associated with 88% (53/60) of all abnormalities (OR, 4.1; p < 0.001). Where known, the abnormalities improved in 43/50 (86%), although 15/43 (35%) had a persistent tonic pupil. CONCLUSION: Pupillary abnormalities after excision of OCVMs can occur in up to a quarter of intraconal lesions-particularly those located inferotemporally or inferiorly-and in about a half of apical lesions. Lateral or inferolateral orbital approaches that disrupt the inferior intraconal fat appear to be associated with a higher risk. Most changes resolve or improve markedly, although a third of those affected may have a persistent tonic pupil.

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.

Photophobia as a Presenting Feature in Adie's Tonic Pupil.

A 34 years old female presented with complains of photophobia since 6-7 months. On examination, she had anisocoria of 4 mm in room light which increased in bright light. The left pupil was dilated and unresponsive to direct and indirect light stimuli. It did however, constrict slowly on near fixation followed by slow redilatation on distance fixation. A diagnosis of Adie's tonic pupil was made since left pupil constricted with instillation of dilute pilocarpine 0.1%. Her symptoms of photophobia and blurred vision immediately resolved. Photochromatic glasses and dilute pilocarpine 0.1% three times a day were prescribed. Prompt symptomatic relief of photophobia and blurred vision was observed.

Fatigue and Arousal Modulations Revealed by Saccade and Pupil Dynamics.

Saccadic eye movements are directed to the objects of interests and enable high-resolution visual images in the exploration of the visual world. There is a trial-to-trial variation in saccade dynamics even in a simple task, possibly attributed to arousal fluctuations. Previous studies have showed that an increase of fatigue level over time, also known as time-on-task, can be revealed by saccade peak velocity. In addition, pupil size, controlled by the autonomic nervous system, has long been used as an arousal index. However, limited research has been done with regards to the relation between pupil size and saccade behavior in the context of trial-to-trial variation. To investigate fatigue and arousal effects on saccadic and pupillary responses, we used bright and emotional stimuli to evoke pupillary responses in tasks requiring reactive and voluntary saccade generation. Decreased voluntary saccade peak velocities, reduced tonic pupil size and phasic pupillary responses were observed as time-on-task increased. Moreover, tonic pupil size affected saccade latency and dynamics, with steeper saccade main sequence slope as tonic pupil size increased. In summary, saccade dynamics and tonic pupil size were sensitive to fatigue and arousal level, together providing valuable information for the understanding of human behavior.

Acute Oropharyngeal Palsy Following Bilateral Adie's Tonic Pupils Associated with Anti-GT1a and GQ1b IgG Antibodies.

A 36-year-old man was admitted to our hospital with complaints of dysphagia and photophobia. A neurological examination showed oropharyngeal palsy and bilateral mydriasis with loss of light reflexes in the absence of external ophthalmoplegia. Bilateral pupils were supersensitive to pilocarpine 0.1%, which was compatible with Adie's tonic pupils. Serum IgG reacted with GQ1b, GT1a, GalNAc-GD1a, and GD3. Intravenous high-dose immunoglobulin therapy improved his neurological symptoms within three weeks. To our knowledge, there is no medical literature describing acute oropharyngeal palsy with Adie's tonic pupils. We recommend evaluating antiganglioside antibodies to clarify the cause of oropharyngeal palsy and Adie's tonic pupils.

Adie's Pupil: A Diagnostic Challenge for the Physician.

Adie's pupil, also called tonic pupil, is mainly seen in young women. Most patients have unilateral eye involvement. The pupil of the affected side is significantly larger than that on the healthy side. The direct and indirect light reflection from the pupil on the affected side disappears. The pupil on the affected side is sensitive to low concentrations of pilocarpine. The pathogeneses of Adie's pupil are complex, some of which are insidious and lack corresponding specific diseases. Through a literature review, we found that Adie's pupil is mainly associated with infectious diseases, most commonly syphilis, followed by immune diseases and paraneoplastic syndromes. The ophthalmological symptoms and pupil abnormalities can disappear after active treatment of the primary disease. Pilocarpine can be used to treat ophthalmologic symptoms, such as blurred vision, for which patients might visit an ophthalmologist or neurologist. It is essential for clinicians to improve their understanding of the disease to avoid misdiagnosis. Differential diagnosis between Adie's pupil, oculomotor nerve palsy, anticholinergic drug overdose, Argyll-Robertson pupil, and congenital mydriasis need to be identified by the physician. Here, the clinical manifestations, pathogenesis, relationship between Adie's pupil and diseases, and differential diagnosis of Adie's pupil are reviewed.