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.

Treatment of Bilateral Tonic Pupil Associated with Syphilis

PURPOSE: We report a case of a male with bilateral tonic pupils associated with syphilis, that partially improved after syphilis treatment. CASE SUMMARY: A 27-year-old male presented with a 2-month history of near vision impairment. The right and left pupils were 5.5 mm and 6.5 mm in diameter, respectively, in the dark and 5.3 mm and 6.1 mm, respectively, in the light. Both pupils demonstrated light-near dissociation, slow constriction and redilation when looking at near, and constriction after instillation of 0.0625% pilocarpine. Serological tests were positive for syphilis, while cerebrospinal fluid testing was negative. Two months after treatment with intramuscular injection of benzathine penicillin G, his near vision subjectively improved and the right and left pupils were 5.9 mm and 6.4 mm, respectively, in the dark and 4.8 mm and 5.3 mm, respectively, in the light. The size of both pupils decreased and the pupillary light responses partially improved in both eyes. CONCLUSIONS: Patients with bilateral tonic pupils should have serological tests for syphilis. Recovery of tonic pupils can be expected after early treatment with effective antibiotics.

Approach to pupillary abnormalities via anatomical pathways / 영남의대학술지

The pupillary size and movement are controlled dynamically by the autonomic nervous system; the parasympathetic system constricts the iris, while the sympathetic system dilates the iris. Under normal conditions, these constrictions and dilations occur identically in both eyes. Asymmetry in the pupillomotor neural input or output leads to impaired pupillary movement on one side and an unequal pupil size between both eyes. Anisocoria is one of the most common signs in neuro-ophthalmology, and the neurological disorders that frequently cause anisocoria include serious diseases, such as vascular dissection, fistula, and aneurysm. A careful history and examination can identify and localize pupillary disorders and provide a guide for appropriate evaluations.

Approach to pupillary abnormalities via anatomical pathways / 영남의대학술지

The pupillary size and movement are controlled dynamically by the autonomic nervous system; the parasympathetic system constricts the iris, while the sympathetic system dilates the iris. Under normal conditions, these constrictions and dilations occur identically in both eyes. Asymmetry in the pupillomotor neural input or output leads to impaired pupillary movement on one side and an unequal pupil size between both eyes. Anisocoria is one of the most common signs in neuro-ophthalmology, and the neurological disorders that frequently cause anisocoria include serious diseases, such as vascular dissection, fistula, and aneurysm. A careful history and examination can identify and localize pupillary disorders and provide a guide for appropriate evaluations.

Síndrome de Ross en un paciente pediátrico: reporte de caso / Ross syndrome in a pediatric patient: case report

Se presenta el caso de un paciente de 6 años de edad con hipohidrosis segmentaria en el hemicuerpo derecho, pupila tónica de Adie e hiporreflexia ipsilateral. Con esta tríada se hizo el diagnóstico clínico de la tríada del síndrome de Ross. La importancia de este caso radica en la identificación de la hipohidrosis como una manifestación de un desorden autonómico. Su fisiopatología es la denervación posganglionar de las fibras colinérgicas parasimpáticas entre el ganglio ciliar y el iris (pupila tónica), degeneración presináptica de las fibras de la raíz dorsal que transmiten el impulso al asta ventral (hiporreflexia) y la degeneración de las fibras simpáticas posganglionares de las glándulas sudoríparas, su causa no es clara. El curso es crónico, con extensión variable del área dishidrótica. Los pacientes pueden tener compromiso de la termorregulación y alteración en la calidad de vida, por lo que debe enseñarse al paciente y cuidadores estrategias para evitar el sobrecalentamiento corporal.

This the case of a 6 year old with segmental hypohidrosis in the right side of his body, ipsilateral tonic pupil and hiporreflexia. This triad leads to the clinical diagnosis of the Ross Syndrome. The relevance of this case is the recognition of the hypohidrosis as a sign of an autonomic disorder. The Ross syndrome is a progressive benign autonomic disorder, the pathophysiology is the denervation of parasympathetic postganglionic cholinergic fibers between the ciliary ganglion and iris (pupil tonic), presynaptic degeneration of dorsal root fibers (hyporeflexia) and degeneration of the postganglionic sweat glands fibers. The cause is unclear. The course is chronic, with variable extension of the hypohidrosis. Patients may get compromise of thermoregulation and impaired quality of life, that is why the patients and caregivers should be warning and learn the strategies to avoid body overheating.

Syndrome in question

Abstract Ross syndrome is a rare disease characterized by peripheral nervous system dysautonomia with selective degeneration of cholinergic fibers. It is composed by the triad of unilateral or bilateral segmental anhidrosis, deep hyporeflexia and Holmes-Adie's tonic pupil. The presence of compensatory sweating is frequent, usually the symptom that most afflicts patients. The aspects of the syndrome are put to discussion due to the case of a male patient, caucasian, 47 years old, with clinical onset of 25 years.

Síndrome de Sjögren asociado a neuritis periférica, pupila tónica de Adie y fenómeno de Raynaud / Sjögren syndrome associated with peripheral neuropathy, Adie tonic pupil and Raynaud phenomenon

Objetivo: Describir el caso clínico de una paciente con Síndrome de Sjögren asociado a Neuritis periférica, pupila tónica de Adie y fenómeno de Raynaud. Diseño del estudio: Reporte de caso. Metodología: Reportamos el caso clínico de una paciente con Síndrome de Sjögren y sus asociaciones poco frecuente, que consulto a la clínica Instituto Oftalmológico Fernández Vega, Oviedo ­ España. Se realizó una revisión exhaustiva de la historia clínica, del paciente y sus paraclínicos. Resultados: Paciente femenino con antecedentes de Síndrome de Sjögren acude por presentar cervicalgia y neuralgias, además de sensación de ojo seco y disconfort ocular de predominio en OI. En el examen se evidenció agudeza visual mejor corregida (AVMC) 20/20, fenómeno de Raynaud, pupila tónica de Adie en OI (Test de pilocarpina positiva), Test de Schirmer 6 mm en ambos ojos (AO), estesiometría y Lancaster normal AO. Se manejó con corticoides e inmunomoduladores tópicos sin mejoría. La analítica sanguínea para estudios de causas infecciosas e inmunologicas resultópositiva para ANA. Se diagnosticó síndrome de Sjögren asociado a neuropatía periférica. Se inicia tratamiento a metotrexato sistémico con mejoría notoria de síntomas. Test de Schirmer control 16 mm OD y 20 mm OI. Conclusión: Las neuropatías periféricas son posibles manifestaciones del síndrome de Sjögren primario, y se podrían presentar con más frecuencia cuando se asocian a los anticuerpos y fenómeno de Raynaud. Por su parte las neuropatías periféricas podrían ser la primera manifestación en el síndrome de Sjögren en alrededor del 50% de los pacientes.

Objective: To report the clinical case of a female patient with Sjögren syndrome associated with peripheral neuropathy, Adie tonic pupil and Raynaud phenomenon. Study design: Case report. Methods: We performed a descriptive case report with detailed review of the medical record of a female patient with Sjögren syndrome and its associations. The patient was treated at Fernandez Vega Eye Institute, Oviedo-Spain. Her medical records was reviewed and analyzed. Ancillary tests were taken. Results: Female patient with a previous history of Sjögren's syndrome complained about neck pain, neuralgia, dry eye and ocular discomfort predominantly in OS. Best-corrected visual acuity (BCVA) was 20/20. Raynaud's phenomenon was positive. Slit lamp examination: Adie tonic pupil in OS. Schirmer Test 6 mm OU. We started corticosteroids and topical immunomodulators without improvement. Blood tests for infectious and immunological studies (ANA) were positive. After these results Sjögren syndrome associated with peripheral neuropathy was diagnosed and started methotrexate systemic treatment with improvement. Conclusion: Peripheral neuropathies are manifestations of primary Sjögren's syndrome. These manifestations can be present more often when are associated with antibodies (ANA) and Raynaud's phenomenon. On the other hand peripheral neuropathies may be the first manifestation in Sjögren's syndrome in about 50% of patients.

Tonic pupil in leprosy / Pupila tônica em doença de Hansen

Pupil abnormalities in leprosy usually result from chronic iritis with loss of stroma, iris miosis, a sluggish reaction to light, and poor dilation in response to anticholinergic mydriatics. We report two patients with long-standing lepromatous leprosy who developed tonic pupils characterized by mydriasis, absence of reaction to light and hypersensitivity to weak cholinergic solution. Examination revealed iritis and iris atrophy. In both cases, instillation of dilute 0.1% pilocarpine caused miosis in the affected eyes. Tonic pupil occurs in many conditions, but its association with leprosy had not been previously reported.

Anormalidades da pupila em pacientes com doença de Hansen, ocorrem mais comumente devido a irite crônica com perda do estroma iriano, miose, diminuição da reação à luz, e dificuldade de dilatação em resposta a colírios anticolinérgicos. Relatamos dois pacientes com doença de Hansen na forma lepromatosa que desenvolveram pupilas tônicas, caracterizadas por midríase, ausência de reação a luz e para perto e hipersensibilidade a fraca concentração de solução colinérgica. O exame revelou irite e atrofia iriana. Em ambos os casos a instilação de pilocarpina 0,1% causou miose nos olhos afetados. A pupila tônica tem sido relatada em muitas condições, mas sua associação com doença de Hansen ainda não havia sido descrita.

Syphilitic dementia presenting with Adie's tonic pupil and mesial temporal lobes hyperintensities / Demência sifilítica apresentando pupila tônica de Adie e hiperintensidades temporais mesiais

Syphilis became a rare cause of dementia in the present days. Screeningtests for syphilis are no longer recommended according to 2001.American Academy of Neurology guidelines. On the other hand, as itmay represent a potentially treatable cause in developing countries,the Academia Brasileira de Neurologia recommends laboratory screeningfor syphilis in patients with dementia. The diagnosis of neurosyphilisis established with basis on the clinical setting, along withtreponemal and non-treponemal serum antibodies, and cerebrospinalfluid pattern. Magnetic resonance imaging generally reveals cortical atrophy. Focal signs in the temporal lobes are rarely seen. A case of a young man diagnosed with neurosyphilis is presented, on the basis of neuropsychiatric symptoms, uncommon pupillary changes (Adie's tonic pupil), CSF with positive FTA-abs, and increased IgG index, and additionally mesial temporal lobes hypersignal changes.

Considera-se neurossífilis uma causa rara de demência atualmente.Testes para investigação de sífilis não são mais recomendados deacordo com as orientações da Academia Americana de Neurologia,de 2001. Por outro lado, como pode representar uma causa potencialmente tratável, a Academia Brasileira de Neurologia recomendaa investigação de sífilis em pacientes com demência. O diagnósticode neurossífilis é estabelecido pelo quadro clínico em associaçãocom anticorpos treponêmicos e não treponêmicos, e exame de LCR.Ressonância magnética revela, em geral, atrofia cortical. Presençasde sinais focais em lobos temporais são consideradas raras. É apresentado caso de homem jovem com diagnóstico de neurossífilis combase nas manifestações neuropsiquiátricas, alteração incomum aoexame pupilar (pupila de tônica de Adie), LCR com FTA-abs positivoe índice de IgG elevado, e ainda hipersinal nos lobos temporais mesiais.

Clinical Features of Adie's Tonic Pupil: A Retrospective and Cross-Sectional Study

PURPOSE: To report the clinical features of Adie's tonic pupil. METHODS: The medical records of 22 patients who had been diagnosed with Adie's tonic pupil from February 1998 to February 2009, were retrospectively reviewed. On March 2010, a cross-sectional examination was performed in 16 patients (19 eyes) who underwent a follow-up of more than 1 year. Measurements included pupil size in room light, bright light and in darkness; near point of accommodation; presence of segmental iris palsy; light-near dissociation; denervation supersensitivity; corneal sensitivity; and deep tendon reflex (DTR). RESULTS: Among the patients studied, 16 were women in Adie's tonic pupils. Only 3 of patients had bilateral involvement. The mean age of onset was 38.3 years. The mean size of Adie's tonic pupils was 2.3 mm larger than the fellow eyes. Segmental iris palsy was detected in 93.8% of the patients. Denervation supersensitivity was observed in all patients. Light-near dissociation was present in 88.2% and over 90% of the patients had decreased DTR in the biceps, triceps, knee and ankle jerk. CONCLUSIONS: This cross-sectional study showed Adie's tonic pupil tended to become miotic and recover accommodation power over the years.

Neurosyphilis Presenting With Unilateral Tonic Pupil

No abstract available.

Tonic pupil presenting after surgical removal of an orbital cavernous haemangioma

Orbital trauma and surgery are recognised aetiological factors of tonic pupil. Tonic or Adie's pupil is an efferent pupil defect in which light reactions to one or more segments of the iris sphincter are lost due to the postganglionic parasympathetic nerves damage from ciliary ganglion. There is loss of part or all of the light reflex and decrease in accommodative functions at near. We report a case of tonic pupil in a 42-year-lady after a successful surgical removal of an orbital cavernous haemangioma.

Early Dorsal Midbrain Syndrome Mimicking an Adie's Tonic Pupil

BACKGROUND: While tonic pupils have been attributed to various diseases, including syphilis, herpes zoster, orbital trauma, temporal arteritis, endometriosis, and paraneoplastic syndromes, obstructive hydrocephalus has not been implicated. CASE REPORT: A 36-year-old woman visited a neurology department with a 7-day history of throbbing headache and blurred vision in both eyes. She had early dorsal midbrain syndrome mimicking an Adie's tonic pupil, and cholinergic supersensitivity was demonstrated using topical 0.125% pilocarpine. Brain MRI revealed obstructive hydrocephalus at the level of the aqueduct of Sylvius, and her symptoms resolved 4 days after surgery. CONCLUSIONS: We report a patient with early dorsal midbrain syndrome that was initially believed to represent a tonic pupil on the basis of pharmacologic testing. The findings in our patient suggested that early dorsal midbrain syndrome mimicking an Adie's tonic pupil can be caused by obstructive hydrocephalus compressing the Edinger-Westphal nucleus.

A case of rheumatoid arthritis with Sjogren's syndrome presenting as a tonic pupil / 대한내과학회지

Sjogren's syndrome is an autoimmune disease that presents mainly as dry eyes and mouth, and occasionally with extra-glandular symptoms. A peripheral neuropathy is present in 10~30% of the cases with extra-glandular symptoms, although a tonic pupil caused by destruction of the ciliary ganglion is rare. We report a case of rheumatoid arthritis with Sjogren's syndrome presenting as a tonic pupil. A 29-year-old woman was admitted for evaluation of polyarthralgia and a tonic pupil. On physical examination, she had polyarthritis involving the jaws, shoulders, wrists, and hands. Her pupils were anisocoric and did not react to light, but constricted promptly to pilocarpine. Biopsy of the minor salivary gland showed lymphocyte infiltration. Rose-Bengal stain was positive. She was diagnosed with rheumatoid arthritis with Sjogren's syndrome and treated with prednisolone and hydroxychloroquine. Three months later, her polyarthritis had improved markedly, but she still had a tonic pupil.

A Case of Adie's Tonic Pupil Before Presentation of Denervation Hypersensitivity

PURPOSE: Denervation supersensitivity to 0.125% pilocarpine is an important factor in making a diagnosis of Adie's tonic pupil. However, it generally takes several weeks for denervation supersensitivity to manifest after an injury to the nerve ganglion. We report a case of 'acute' Adie's tonic pupil before the manifestation of denervation supersensitivity. CASE SUMMARY: A 53-year-old man with no significant past medical history visited our clinic, reporting mydriasis of his left eye. Pupil size was 8 mm in the right eye, 3 mm in the left. Loss of both direct/indirect light reflex and light-near reflex were observed. The left pupil did not react to 0.125% Pilocarpine, but constricted in response to 1% pilocarpine. After 5 months, the pupil size did not change, but the pupil constricted in response to 0.125% pilocarpine. CONCLUSIONS: In an isolated case of dilated pupil, though the pupil did not react to 0.125% pilocarpine, with respect to the Adie's tonic pupil, to check the manifestation of denervation supersensitivity should be needed at regular intervals.

A Case of Adie's Tonic Pupil Before Presentation of Denervation Hypersensitivity

PURPOSE: Denervation supersensitivity to 0.125% pilocarpine is an important factor in making a diagnosis of Adie's tonic pupil. However, it generally takes several weeks for denervation supersensitivity to manifest after an injury to the nerve ganglion. We report a case of 'acute' Adie's tonic pupil before the manifestation of denervation supersensitivity. CASE SUMMARY: A 53-year-old man with no significant past medical history visited our clinic, reporting mydriasis of his left eye. Pupil size was 8 mm in the right eye, 3 mm in the left. Loss of both direct/indirect light reflex and light-near reflex were observed. The left pupil did not react to 0.125% Pilocarpine, but constricted in response to 1% pilocarpine. After 5 months, the pupil size did not change, but the pupil constricted in response to 0.125% pilocarpine. CONCLUSIONS: In an isolated case of dilated pupil, though the pupil did not react to 0.125% pilocarpine, with respect to the Adie's tonic pupil, to check the manifestation of denervation supersensitivity should be needed at regular intervals.

Vogt-Koyanagi-Harada Disease Associated with Bilateral Tonic Pupils in a Pregnant Patient

PURPOSE: We report a case of bilateral tonic pupils and decreased corneal sensitivity in a patient with Vogt-Koyanagi-Harada (VKH) disease during pregnancy. CASE SUMMARY: A 33-year-old Korean woman in the sixth month of pregnancy presented with visual impairment in both eyes. Her best corrected visual acuity was 0.2 in the right eye and 0.125 in the left. Slit-lamp examination revealed cells in the anterior chamber and serous retinal detachments in both eyes. The patient was treated with topical corticosteroid in both eyes and posterior subtenon injection of triamcinolone acetonide in the left eye. The serous retinal detachment resolved completely at 2 months in the left eye and at 3 months in the right. As pigmentation of the retina developed, VKH disease was confirmed. The patient complained of photophobia, and both pupils were found to be enlarged to 8 mm without dilation. Light reflex was absent and near reflex was suppressed and slow. After instillation of 0.125% pilocarpine, the pupils were constricted to 3.5 mm and were diagnosed as tonic pupils. Though the patient's corrected visual acuity improved to 0.8 in the right eye and 1.0 in the left at 6 months, her pupils remained unchanged. Corneal sensitivity was decreased. CONCLUSIONS: Tonic pupils may be complicated with VKH disease even during pregnancy. We recommend examinations of the pupils and corneal sensitivity in patients with VKH disease, especially those complaining of photophobia.

Tonic Pupil in Neuropathy

BACKGROUND: The clinical and laboratory findings of five patients with tonic pupil (TP) and neuropathy were reviewed for the comprehension of pathogenesis of TP in neuropathy. METHODS: Immunological and nerve conduction studies (NCS) were performed in three patients with Sjogren's syndrome (SS), Miller-Fisher syndrome (MFS), and Adie's syndrome. RESULTS: Upon initial examination, there were no definite sicca syndromes in patients of SS, despite intolerable sensory symptoms. The TP in MFS was improved after intravenous immunoglobulin. Of the cranial neu-ropathies, trigeminal sensory neuropathy was frequent finding. Deep tendon reflexes were absent in all five patients.Absent sensory nerve action potentials and prolonged R1 and R2 of the blink reflex were detected in two SS patients with syncope and asymmetric sensory loss. CONCLUSIONS: These findings in SS patients implicated the possibility of a selective lesion at the level of the dorsal root- or trigeminal- or autonomic- ganglions complicating the TP. In view of the sensory ataxia, opthalmoplegia, areflexia, slow and decreased sensory NCS in the extremity and prolonged R1 and R2, a demyelinating process of postganglionic parasympathetic nerves were suspected to be the cause of the tonic pupil in MFS. Adie's syndrome along with flushing of the left side of the face and chest after exercise, suggested segmental postganglionic lesions of the sympathetic and parasympathetic peripheral nervous systems. In patients with complicat-ing TP and asymmetric progressive sensory neuropathy, the SS has to be considered even if the patient denies the pres-ence of sicca symptoms at first and SS-A/SS-B autoantibody is negative.