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.

Myasthenia gravis with Holmes-Adie syndrome:a case report / 中华神经科杂志

Holmes-Adie syndrome (HAS) is a clinical syndrome mainly characterized by tonic pupil and disappearance of tendon reflex. It is mostly idiopathic and can also be seen in cerebral diseases, such as trauma, infection and tumors. However, it is rarely reported to be accompanied with myasthenia gravis (MG). We report a case of MG and HAS, whose clinical manifestations were fluctuation of limb weakness, breathing difficulties, right ptosis. Her pupils were unequal: the left pupil was 3 mm, the right pupil was 2 mm, direct and indirect light reflex was slow in left pupil, and right pupil was sensitive to light reflex. The left eye pupil shrank to 2 mm after dripped pilocarpine diluent for 10 minutes, while the right pupil was still 2 mm. Chest CT examination revealed thymoma. After treatment with thymectomy, glucocorticoid, immunoglobulins and tacrolimus, her symptoms of MG were improved, but the left pupil diameter and light reflex were not changed. Combined with the patient's symptoms, physical signs and examinations, this patient was diagnosed as MG accompanied with HAS.

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.

Adie-Holmes Syndrome / Síndrome de Adie-Holmes

The Holmes-Adie syndrome is characterized by the presence of tonic pupil associated with absence or diminution of deep tendon reflexes. In some cases there may be autonomous nerve dysfunction. The mechanism that causes the disorder is not fully known, but is believed to be caused by denervation of the postganglionic supply to the sphincter of the pupil and the ciliary muscle which can occur following viral disease. Typically it affects young adults and is unilateral in 80% of cases, although it may develop in the contralateral eye in months or years. We report a case of a woman presenting typical signs of this syndrome, in which pharmacological test was essential for diagnosis.

A Síndrome de Holmes-Adie É caracterizada pela presença de pupila tônica associada à diminuição ou ausência dos reflexos tendíneos profundos. Em alguns casos pode haver disfunção nervosa autônoma. O mecanismo que causa a desordem não é totalmente conhecido, mas acredita-se que seja causada pela desnervação do suprimento pós-ganglionar para o esfíncter da pupila e para o músculo ciliar, que pode ocorrer após doença viral. Tipicamente afeta adultos jovens e é unilateral em 80% dos casos, embora possa se desenvolver no olho contralateral em meses ou anos. Nós relatamos o caso de uma mulher apresentando sinais típicos desta síndrome, em que o teste farmacológico foi fundamental para o diagnóstico.

Fixed Pupillary Light Reflex due to Peripheral Neuropathy after Liver Transplantation / 대한구급학회지

A 46-year-old female patient was admitted to the intensive care unit (ICU) after liver transplantation. About an hour later after the ICU admission, she had no pupillary light reflex. Both pupils were also fixed at 5 mm. Patients who undergo liver transplantation are susceptible to neurologic disorders including hepatic encephalopathy, thromboembolism and intracranial hemorrhage. Abnormal pupillary light reflex usually indicates a serious neurologic emergency in these patients; however, benign neurologic disorders such as peripheral autonomic neuropathy or Holmes-Adie syndrome should also be considered. We experienced a case of fixed pupillary light reflex after liver transplantation diagnosed as peripheral autonomic neuropathy.

Fixed Pupillary Light Reflex due to Peripheral Neuropathy after Liver Transplantation / 대한중환자의학회지

A 46-year-old female patient was admitted to the intensive care unit (ICU) after liver transplantation. About an hour later after the ICU admission, she had no pupillary light reflex. Both pupils were also fixed at 5 mm. Patients who undergo liver transplantation are susceptible to neurologic disorders including hepatic encephalopathy, thromboembolism and intracranial hemorrhage. Abnormal pupillary light reflex usually indicates a serious neurologic emergency in these patients; however, benign neurologic disorders such as peripheral autonomic neuropathy or Holmes-Adie syndrome should also be considered. We experienced a case of fixed pupillary light reflex after liver transplantation diagnosed as peripheral autonomic neuropathy.

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.

Síndrome disautonómico de Ross: variaciones de un tema / Disautonomic Ross syndrome: variations about one theme

El síndrome de Ross fue descrito en 1958 como una afección degenerativa del sistema nervioso autónomo definido por la tríada de anhidrosis generalizada, disminución de los reflejos tendinosos y pupila tónica. Desde su descripción inicial se han descrito cerca de cuarenta casos. Comunicamos tres pacientes con variantes de interés que incluyen la presencia de espasmos cíclicos espontáneos del esfínter de iris, el desarrollo conjunto de síndrome de Holmes-Adie en un lado y síndrome Horner posganglionar en el otro, trastornos del desarrollo piloso en el lado de la anhidrosis, alteraciones de la motilidad intestinal, lengua sin papilas gustativas y disfunción sexual.

Ross Syndrome was described in 1958 as a degenerative condition of the autonomic nervous system defined by a triad of generalized anhidrosis, reduction of tendon reflexes and tonic pupil. Since its initial description about 40 cases have been described. We communicate three cases with variants of interest involving the presence of the simultaneous development of syndrome of Holmes-Adie on one side and Horner syndrome in the other, disorders of pilous follicle development on the side of anhidrosis, spontaneous disturbances of intestinal motility, tonque without papillae and sexual dysfunction.

Probable Paroxysmal Hemicrania Associated with Holmes-Adie Syndrome

No abstract available.

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.

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.

Two Cases of Holmes-Adie Syndrome

BACKGROUND: Adie's tonic pupil consists of mydriasis, accommodation defect, decreased light reflex, and tonic near reflex. When tonic pupil is combined with hypo- or areflexia, it is called Holmes-Adie syndrome. CASE: Fifty-two-year-old and 37-year-old female patients showed unilateral dilated irregular pupil, decreased light reflex, and decreased or absent DTR. When 0.125% pilocarpine was dropped on the dilated pupil, the pupil was constricted after pilocarpine application. Somatosensory evoked potentials were within normal ranges, and H-reflex responses were absent or delayed.

Agenciamentos sociais, subjetividade e sintoma: a seleção hierarquizada da clientela em um ambulatório público de saúde mental / Social arrangements, subjectivity and sympton: the hierarchized selection of patients in a public mental health ambulatory / Agencements sociales, subjectivité et symptôme: la sélection hiérarchisé de la clientèle dans un ambulatoire public de santé mentale

A pesquisa apresentada neste artigo teve por objetivo o estudo da lógica implícita no padrão de seleção dos pacientes de um ambulatório público pelos Serviços de Psicologia e Psiquiatria. Os pacientes que afluem a esses dois serviços foram classificados em três grupos, com base nos enunciados produzidos para tematização do sofrimento: o dos sujeitos psicológicos, o dos doentes dos nervos e o dos loucos. No processo de investigação, constatou-se que os pacientes do primeiro grupo tendem a ser encaminhados para o Serviço de Psicologia, no qual têm acesso ao dispositivo de escuta psicoterápica, podendo, assim, emitir um discurso sobre seu próprio sofrimento, enquanto os que se situam nos outros dois grupos são encaminhados para o Serviço de Psiquiatria, onde são subme­tidos ao tratamento medicamentoso. Conclui-se que tal padrão de seleção se caracteriza por uma hierarquização da clientela,iegundo uma lógica que implica a desqualificação da diferença, a normalização da loucura, a garantia da produtividade e a realização do mínimo.

The research presented in this paper had as aim the study of implicit Iogic underlying the pattems of patients seIection by psychoIogy and psychiatry services in a public ambuIatory. The patients that flow to these two services were cIassificated in three groups, take into consideration the statements produced about theirs own suffering: the psychoIogical subjects, the nervous and the mads. The patients of the first group are, generally, Ied to the psychoIogy service, where they can speak about theirs suffering in a psychoterapy setting, while the others are Ied to the psychiatry service. where they are subjected to pharmacoIogical treatment. The research conc1uded that one can find a hierarchized care in that pattem of seIection. whose Iogic is based on disqualification of the difference. normalization of the madness, assurance of the productivity and actualization of the minimum.

La recherche présenté dans cet articIe eut pour but étudier Ia IQgique subjacent au modéIe de seIection des patients pour Ies services de psychoIogie et psychiatrie d'un ambuIatoire publico Les patients qui affluent à ces deux services on été divisé dans trois groups, selon des énoncés utilisés pour décrire Ia souffrance: Ies sujets psychologiques, Ies malades des nerfs et Ies fous. On a pu vérifier que Ies patients de Ie prernier group. en général, sont conduits vers Ie Service de PsychoIogie. oú ils peuvent parIer sur sa souffrance. pendant que ceux situés dans Ies autres groups sont conduits vers Ie Service de Psychiatrie, oú ils demande traitrnent médicamenteux. On a pu conc1ure que ce modéIe de séléction est caracterizé par une hierarchisation de Ia clientéIe, dont Iogique implique Ia disqualification de Ia différence, Ia normalisation de Ia folie. Ia garantie de Ia productibilité et Ia réalisation du minimum.

A Case of Primary Sjogren's Syndrome Presenting as Adie's Syndrome / 대한류마티스학회지

Sjogren's syndrome is a hererogenous autoimmune disease characterized by progressive destruction of the exocrine glands and accompanied by a variety of autoimmune phenomena. Sjogren's syndrome patients can develop symptoms of ocular and oral dryness as well as extraglandular complications including central and peripheral nervous system disease. Sometimes neuropathy precedes the diagnosis of Sjogren's syndrome. Adiets syndrome is characterized by tonic pupil and the absence of tendon reflex. Sweating abnormality and chronic peripheral polyneuropathy can also be present. We report a case of primary Sjogren's syndrome preceded by Adie's syndrome with peripheral neuropathy. A 26-year-old woman was admitted for photophobia and paresthesia. On examination, her pupils were anisocoric and did not react to light but constricted promptly to pilocarpin. Sensation decreased on her left side of body and deep' tendon reflexes were absent. Biopsy of minor salivary gland demonstrated infiltration by lymphocyte consistent with Sjogren's syndrome, but Schirmer test was negative. So she was diagnosed as Adie's syndrome with peripheral neuropathy. Five month later she complained of dry eye and dizziness. Rose bengal staining was positive. Sjogren's syndrome was diagnosed and she was discharged with local therapy for the sicca symptoms.

Holmes-Adie syndrome with sweating abnormality (report of 1 case) / 临床神经病学杂志

Objective To investigate the clinical manifestation, pathological features and pathogenesis of Holmes-Adie syndrome (HAS). Methods The clinical data of one HAS patient with sweating abnormality and combined with literatures were analysed. Results The patient had the typical characteristics of HAS, including Adie pupil, areflexia of lower limb and the symptoms of autonomic nerve dysfunction. The characteristic pupil of HAS was proved by slit lamp of ophthalmology. Cranial MRI showed lacunar infarction. A partial or total loss of neurons of the ciliary ganglion was the main pathological characteristic of HAS. Conclusions The etiology of HAS remains unclear, and has characteristic pupil finding, which was associated with autonomic nerve dysfunction to a certain extent. HAS still need to differentiate from Ross syndrome and Harlequin syndrome.