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.

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.

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.