Holmes-Adie syndrome and vitamin B12 - associated peripheral neuropathy: An association or coincidence?

A 30-year-old male patient presented to the eye department with complaints of blurring of vision of right eye at distance and near for a duration of 1.5 months. Ocular examination revealed Anisocoria with enlarged pupil in the right eye. On instillation of 0.1% pilocarpine, there was a pronounced miosis in the dilated pupil seen at 30 min associated with an improvement in distance and near vision. On slit lamp examination, vermiform movements were seen in the affected pupil on shining the slit from temporal aspect. Fundus examination was within normal limits. Systemic examination revealed absent deep tendon reflexes. Based on the clinical features, a diagnosis of Holmes-Adie syndrome was reached and the patient was started on 0.1% pilocarpine eye drops. This case highlights the importance of thorough systemic examination and investigations in all cases of anisocoria.

Concurrent tonic pupil and trochlear nerve palsy in COVID-19.

Since COVID-19 was first reported, different neurological complications have been acknowledged, but their description is constantly evolving. We report a case of concurrent tonic pupil and trochlear nerve palsy in this context. A 62-year-old man reported a 5-day history of binocular vertical diplopia and blurred vision in his left eye, noticing that his left pupil was dilated. He had suffered a flu-like syndrome 2 weeks before. Clinical exam showed a right trochlear nerve palsy and a left mydriatic pupil. MRI, X chest ray, and analytical results were normal. Antibodies for SARS-CoV-2 were positive (low IgM and high IgG titers). Antiganglioside antibodies were negative. A 0.125% pilocarpine test confirmed Adie's pupil diagnosis. The patient was treated with a tapered prednisone dose with resolution of his diplopia but no change in Adie's pupil. This is the first case reporting Adie's pupil as a postinfectious manifestation of COVID-19. An immune-mediated mechanism is presumed.

Tonic pupils: an unusual autonomic involvement in chronic inflammatory demyelinating polyneuropathy (CIDP).

BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neuropathy which affects mainly large myelinated axons and has a typically mild autonomic dysfunction mainly from postganglionic nerve fiber involvement. CASE REPORT: We report here an acute onset CIDP initially diagnosed as Guillain-Barré syndrome (GBS), unresponsive to treatment with intravenous immunoglobulin (IVIg), which later responded to plasmapheresis and corticoids. The patient had a markedly distal demyelination, prominent cranial nerve involvement and, interestingly, bilateral fixed dilated pupils. Despite complete clinical recovery, this neurological sign remained. CONCLUSIONS: Tonic pupils have previously been described in different neurologic conditions, including GBS, but not yet in acute onset CIDP or in variants with predominantly distal demyelination. It differs from the classical Adie's pupil because it lacks the light-near dissociation. This case report expands the range of possible autonomic signs in acute onset CIDP, which could help physicians establish optimal treatment strategies earlier on.

[Adie syndrome]. / Sindrom Éidi.

Disorders and abnormalities of pupil reactions comprise important part in the clinical practice of both ophthalmologists and neurologists. The present article presents a historical perspective on one of such pathologies - Adie syndrome, and discusses its etiology, pathogenesis and clinical symptomatology. The article also describes a clinical case of one patient with comorbidity.

Sir Gordon Morgan Holmes (1876-1965): one of the founders of modern neurology.

Sir Gordon Morgan Holmes (1876-1965) was one of the most important founders of modern neurology and a great teacher and scientist. He was the first scientist to challenge the theory of the unitary function of the cerebellum and described cerebellar disorders. Holmes together with Thomas Grainger Stewart (1877-1957) described 40 cases of the rebound phenomenon in cerebellar disease (Stewart-Holmes maneuver or Stewart-Holmes test). He also described the symptoms of inherited neurodegenerative spinocerebellar ataxia involving the olivary nucleus (Gordon-Holmes syndrome). Independently from the Australian neurologist William John Adie (1886-1935), he described the partial iridoplegia (Holmes-Adie pupil or Holmes-Adie syndrome). His teaching skills became clearly visible in Goulstonian and Croonian lectures dedicated to spinal cord injuries.

Tonic Pupil, a Paraneoplastic Neuro-Ophtalmological Disease Associated with Occult Breast Cancer.

Here, we present a case of tonic pupil associated with occult breast cancer as a paraneoplastic neuro-ophthalmology syndrome. A 45-year-old woman developed progressive photophobia and blurred vision due to unilateral Adie's tonic pupil. Magnetic resonance image of her brain and neurological examination (including deep tendon reflexes) were normal at first visit. Follow-up examinations performed by ophthalmologist every 6 month without any change in her condition. After 2 years, patient discovered a mass in her breast which identified to be malignant after diagnostic procedures. Despite surgical and medical treatment for cancer, no change in the ocular condition was happened.

Bilateral Tonic Pupils as the Initial Manifestation of Sjögren's Syndrome.

Adie's pupil is characterised by pupil dilatation, segmental iris palsy, light-near dissociation, and slow re-dilatation. Most commonly, tonic pupils are unilateral and idiopathic, but can be caused by orbital disorders and autonomic neuropathies. There are only a few case reports of tonic pupils in patients with Sjögren's syndrome, caused by an autoimmune ciliary ganglionitis. The authors report on two cases with bilateral tonic pupils as the initial manifestation of primary Sjögren's syndrome. Both patients presented with blurred vision, bilateral tonic pupils, and sicca symptoms. The findings suggest that Sjögren's syndrome should be considered in patients presenting with bilateral Adie's tonic pupils.

Bilateral Adie's Tonic Pupil and Hyperopic Shift in Idiopathic Sclerochoroidal Calcification.

Sclerochoroidal calcification is a rare but recognised ophthalmic manifestation seen mostly in elderly Caucasian individuals. The lesions, often bilateral, appear as yellow-white irregular subretinal lesions usually found along the mid-peripheral fundus. Though typically asymptomatic, sclerochoroidal calcification has rarely been associated with parafoveal involvement, choroidal neovascularisation, and serous detachment of the calcifications. Visual involvement is typically minimal, and neovascularisation is often visually insignificant. We present a rare case of sclerochoroidal calcification in a 64-year-old Caucasian female who presented with painless progressive bilateral vision loss and a hyperoptic shift with subsequent development of bilateral sequential Adie's tonic pupil. To the best of our knowledge, this is the first such report in the English language literature.

William John Adie: the man behind the syndrome.

William John Adie was an Australian neurologist in the early 20th century responsible for extensively describing the tonically dilated pupil associated with absent deep tendon reflexes - both features of a syndrome that now bears his name. In addition to other neurological syndromes, he was also significant in delineating narcolepsy through his clinical essays and case series. His ophthalmic and neurologic contributions have served the test of time and played an important role in the modern understanding of Adie syndrome and narcolepsy. This report reviews Adie's medical contributions, extensive descriptions of Adie syndrome, and provides a brief biographical account of his life.

W. J. Adie and his "pyknolepsy," a century ago.

On November 8, 1923, William John Adie described an unusual disorder to the Section of Neurology of the Royal Society of Medicine. The condition comprised frequent momentary stereotyped impairments of consciousness that occurred in children, did not respond to antiseizure medications, and did not develop into epilepsy, as that term was then commonly understood, since no convulsive seizures occurred. After some time, the episodes terminated spontaneously, leaving the sufferer unhandicapped and neurologically intact. Almost certainly, Adie had described the present-day entity of childhood absence epilepsy. He termed it "pyknolepsy," knowing that the name "pyknolepsie" had been used for a similar disorder in Germany from 1916 onwards, though not reported elsewhere. Following Adie's account, published in 1924, reports of the disorder appeared in the English and French-language literature and continued to be published in German. It became increasingly accepted that pyknolepsy was a form of epilepsy that was part of Lennox's petit mal triad. The word pyknolepsy itself never became widely used and is now largely forgotten. Adie never took up the topic in print again. However, he had awakened English-language readers to one component in a broadening of the concept of what constituted epilepsy.

Adie's tonic pupil after COVID-19: a case report and literature review.

Objective: Various neurological complications have been reported after COVID-19. The study aimed to document an unusual case of Adie's tonic pupil following COVID-19. Methods: The study was a case report. Results: A 28-year-old female had suffered a flu-like disease about 2 months before and the SARSCoV-2 polymerase chain reaction test at that time was positive. Two weeks after infection she noticed an asymmetry between the pupils. The only pathological finding on examination was anisocoria with a larger left pupil in ambient light. Light reflexes were observed in the right eye, while in the left eye, they were absent. Also, there was no near response in the left pupil. A 0.1% pilocarpine test results validated Adie's pupil diagnosis. After one year of follow-up, the anisocoria decreased but did not completely recover. Discussion: COVID-19 may cause damage to neural structures due to autoimmune ways by activating immune pathways or because of vascular complications that may affect the vasa nervorum. Adie's tonic pupil is often idiopathic, but it may develop following viral infection. Conclusions: Ocular complications that involve pupil abnormalities may manifest following COVID-19. In the cases of Adie's tonic pupil, infectious diseases, including COVID-19, should be questioned. Abbreviations: RT PCR = reverse transcription polymerase chain reaction.

[Dysautonomic syndrome of the face with Harlequin sign and syndrome: Three new cases and a review of the literature]. / Syndromes dysautonomiques du visage avec signe et syndrome d'Harlequin : étude de trois cas et revue de la littérature.

INTRODUCTION: Harlequin phenomenon is characterized by a strictly unilateral erythrosis of the face with flushing and hyperhydrosis, and controlaterally a pale anhydrotic aspect. This syndrome can occur alone or associated to other dysautonomic phenomena such as Horner syndrome, Adie syndrome or Ross syndrome. PATIENTS AND METHODS: We report three cases: two patients presented a Harlequin sign, associated with Horner syndrome for one and Ross syndrome for the second. The etiologic investigation was normal, allowing recognizing the idiopathic nature of the disorder. For the third patient, Harlequin syndrome was observed in a neoplastic context due to breast cancer, metastatic dissemination, and bone metastases involving the right side of the T2 body. DISCUSSION: We reviewed the literature: 108 cases have been described. This syndrome occurred alone in 48 patients and was associated with other dysautonomic syndromes such as Horner syndrome in 38 patients, Holmes Adie syndrome in six, and Ross syndrome in six; both Ross and Holmes Adie syndrome were associated five cases and associations were not reported in five patients. The pathophysiological mechanisms of this autonomic cranial neuropathy, the possible etiologies, and therapeutic management were discussed. CONCLUSION: Harlequin phenomenon with flushing and unilateral hyperhydrosis is rare, occurring alone or in combination with other autonomic syndromes of the face. Idiopathic in two-thirds of cases, Harlequin phenomenon does not require specific treatment; sympathectomy may be discussed in the severe cases with a significant social impact.

A Case of Surgery Cancellation Following the Discovery of Anisocoria After Induction of General Anesthesia.

Anisocoria after induction of general anesthesia may indicate a severe cerebrovascular disorder. We encountered a case in which anisocoria in the left and right eyes was noticed after induction of general anesthesia, and the surgery was canceled. The patient was a 53-year-old woman with a history of hypertension. She received general anesthesia 10 years ago, but the details were unknown. Anesthesia was induced with propofol, remifentanil, and rocuronium, followed by nasal intubation. No significant change was observed in vital signs during the induction of anesthesia. After intubation, the pupils were checked according to the protocol for observing pupil diameter. The pupil size was found to be unequal, measuring 1 mm in the left eye and 4 mm in the right eye. A cerebrovascular disorder was suspected; thus, the surgery was canceled, and the patient was awakened and extubated. Neurological symptoms such as limb movements were checked after awakening, and no disorientation or motor dysfunction was detected in the patient. However, her pupils remained unequal, measuring 2 mm in the left eye and 4 mm in the right eye. Regarding light reflex, the left eye was miotic, but the right eye remained mydriatic. The pupillary symptom persisted even during discharge the next day. Since our hospital is a solely dental hospital, following discharge, after consulting the ophthalmology department of a nearby medical university hospital, the patient was diagnosed with pupillotonia, as she had been experiencing light dazzling in only her right eye for seven years, had no light reflex but near reflex, and was miotic due to the use of pilocarpine hydrochloride eye drops, which promotes miosis. The patient has had these symptoms in the right eye for seven years, and it is possible that she had anisocoria during the preoperative examination at this time. If anisocoria had been detected and examined carefully during the preoperative examination, there would have been no need to cancel the surgery. In this case, we strongly felt that the pupils must be checked during the preoperative examination.

Development of listening competence in business education.

Previous research indicates that listening is the most important type of oral communication in the workplace. Unfortunately, there is little evidence showing that business programs share this view. The goal of this literature review is to close the gap between employer preferences and business school priorities, thereby enhancing the listening competence of business graduates. Research has identified four listening styles. Task-oriented listening and critical listening focus on message content whereas relational listening and analytical listening are relationally oriented. Although competence in all four styles is needed, the most appropriate style to use is contingent on why one is listening. We propose a systems approach utilizing the ADIE (assessment, design, implementation, evaluation) model for developing the listening competence of business students.

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.

Bilateral Adie's Pupil following Laser Treatment of the Ischemic Peripheral Retina for Uveitis: A Case Report.

Adie's pupil is a neurological condition of unknown origin with unusual, asymmetric presentation known as anisocoria with the enlarged pupil failing to react to light. It is believed that this pupillary abnormality results from damage to the ciliary ganglion or postganglionic short ciliary nerves. Affected individuals (usually female) may be symptomatic with photophobia or difficulty reading in the diseased eye. Although most Adie's pupil cases are idiopathic, previous studies have associated photocoagulation and uveitis with symptom onset. To the best of our knowledge, there have been no reports of specific means of preventing Adie's pupil. We describe a patient who experienced varying severities of Adie's pupil after separate laser treatments of the ischemic peripheral retina for uveitis. Fluorescein angiography revealed peripheral retinal nonperfusion in the bilateral eyes of a 37-year-old Japanese female who had been suffering from posterior uveitis. To avoid proliferative changes, 360° laser photocoagulation of the retinal nonperfusion region located in the far periphery was first delivered to the left eye over 2 sessions. Soon after treatment, the patient complained of acute photophobia and blurred vision in the treated eye. Ocular examination revealed left pupil dilation and poor light sensitivity, although the pupil was reactive to a close stimulus. The left pupil also displayed positive denervation sensitivity based on the dilute pilocarpine (0.125%) test. Adie's pupil was diagnosed based on these observations. Three months later, similar, albeit milder, findings were observed in her right eye after 360° peripheral laser photocoagulation that was more conservatively performed over 4 sessions. Four months after the first treatment, her subjective visual function had improved, and the pupil diameter had decreased to a normal size in both eyes without additional treatment. We encountered a patient whose severity of Adie's pupil was apparently reduced by more conservative laser photocoagulation of the ischemic peripheral retina.

Holmes-Adie syndrome as an early manifestation of systemic lupus erythematosus. / Síndrome de Holmes-Adie como primera manifestación de lupus eritematoso sistémico.

Lupus is an autoimmune disease with multiple manifestations and multiorgan damage. Neuro-ophthalmic disorders are the less common ophthalmological manifestations of lupus. Adie's tonic pupil is mostly idiopathic and may rarely be caused by autoimmune disorders. The combination of abnormal pupil size and a decrease or loss of deep tendon reflexes is usually called Holmes-Adie syndrome. A case is reported of Holmes-Adie syndrome as an early manifestation of systemic lupus erythematosus.

PUPILLOTONIA AND ADIE SYNDROME.

The aim of the work is to approach the examination of the pupil with a focus on anisocoria, its characteristics and approach to the diagnosis of pupillotonia and Adie's syndrome and its clinical evaluation. Pupil function is important not only in neurophthalmological examination but also in general ophthalmological examination. First of all, we need to know how the reflex arc works in order to be able to exclude or confirm whether the parasympathetic or sympathetic is affected. It is also necessary to know the exact characteristics of the pupil, such as size, shape, placement, function and reaction to light and at close range. Only on this basis can we distinguish pathological features. We do not often encounter this diagnosis, but it is necessary to keep it in mind, especially in the field of neurophthalmology but also in general ophthalmology. We also present three cases of pupilotonia and Adie's syndrome, which we diagnosed at the Department of Ophthalmology, Faculty of Medicine, Comenius University, after the patient himself came by emergency admission or was sent directly to ophthalmology clinic. In the discussion, we present various other diagnoses, where the reflex arc may not be affected, but the pathological pupil is caused by intraocular tumors, general systemic diseases and, last but not least, local therapy or alkaloids.

Ophthalmic and Neuro-ophthalmic Manifestations of Coronavirus Disease 2019 (COVID-19).

PURPOSE: To describe a case of inflammatory chorioretinopathy and Adie's syndrome possibly associated with COVID-19. METHODS: Observational case report. RESULTS: A 51-year-old woman developed fever, cough, and headache followed by retro-ocular pain and reading impairment. She tested positive for SARS-COV-2 infection by qualitative real-time reverse-transcriptase-polymerase-chain-reaction. The slit-lamp and funduscopic exam revealed abnormal pupillary response and yellowish creamy deep chorioretinal lesions, which were not present in previous examinations. Instillation of pilocarpine demonstrated denervation supersensitivity, and it was suggestive of bilateral Adie tonic pupil. A comprehensive work-up ruled out other systemic, autoimmune, or infectious diseases. CONCLUSIONS: This case illustrates the possible association between multifocal chorioretinitis and Adie's syndrome, and the SARS-COV-2 infection in humans. Further investigation of virus infectivity specifically within ocular tissues has to be conducted.

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.