Absent cardiac and muscle sympathetic nerve activities involvement in Ross syndrome: A follow-up study.

PURPOSE: Ross syndrome (RS) is characterized by selective involvement of post-ganglionic skin sympathetic nerve fibres. We report a follow-up study in 4 patients to clarify whether in RS autonomic dysfunction spreads affecting also cardiovascular system. METHODS: The patients underwent cardiovascular reflexes (CVR) and microneurography recording of muscle sympathetic nerve activity (MSNA) for a follow-up mean period of 5years. RESULTS: CVR and MSNA were normal at baseline and unchanged over the follow-up. CONCLUSIONS: Cardiovascular autonomic system is spared in RS differently from skin autonomic activity dysfunction which progress over time. However, before drawing any definite conclusion, a large cohort of patients needs to be studied.

Pupila tónica como manifestación inicial de un síndrome de Sjögren / Tonic pupil as an initial manifestation of Sjögren's syndrome

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[A Case of Anti-GQ1b Antibody Syndrome Associated with Pure Bilateral Adie's Pupils].

A 37 year-old Japanese male felt photophobia of both eyes one week following the onset of the common cold. His neurological examination revealed bilateral Adie's tonic pupils, no extraocular movement disorder, normal deep tendon reflexes, and no cerebellar signs. Based on markedly increased blood levels of anti-GQ1b IgG and anti-GT1a IgG antibodies, we diagnosed him as anti-GQ1b antibody syndrome. Bilateral Adie's tonic pupils were improved by IVIg drip infusion and methylprednisolone pulse therapy. This case suggests that we need to investigate anti ganglioside antibody when a patient presents with bilateral Adie's tonic pupils.

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.

[Abnormal pupils' reactions]. / Nieprawidlowe reakcje zreniczne.

Examination of the pupils' light reaction and estimation of the pupils' diameter are the components of the routine physical examination of a patient. Disturbances in pupils' light reaction, unequal size of the pupils indicate damage of the nervous system and require careful diagnosis, both neurological and ophthalmological. Different aspects of anatomy, physiology and the most common pathological syndromes associated with disturbed pupils' light reaction and unequal diameters of the pupils were presented in the article. Early diagnosis and proper treatment can allow to improve prognosis in the analyzed patient group.

Ross syndrome: Unilateral hyperhidrosis, Adie's tonic pupils and diffuse areflexia.

Ross syndrome is a rare disorder first described in 1958 with partial autonomic dysfunction. It has three basic components including unilateral or bilateral segmental anhidrosis, Adie's tonic pupils and areflexia or hyporeflexia of deep tendon reflexes. The most disturbing symptom in the patients is segmental compensatory hyperhidrosis and often the hypohidrosis or anhidrosis is not even noticed. While the pathogenesis of Ross syndrome is unclear, degenerative changes or damage to the peripheral autonomic nerve system or dorsal root ganglia have been suggested as possible causes. About 50 cases have been reported, usually by neurologists and ophthalmologists, and less often by dermatologists. We present a 26-year-old patient who displayed the classic triad of this syndrome, emphasizing that the presenting complaint may be hyperhidrosis and that multidisciplinary evaluation in neurology and ophthalmology is essential.

Bilateral subacute angle-closure glaucoma in association with tonic pupils post-coronary artery bypass graft.

Anterior and posterior visual pathway disturbances have been reported post-coronary artery bypass graft surgery. Acute angle-closure glaucoma after general anaesthesia has also been well described in the literature. To date, however, there are only two cases of angle closure caused by tonic pupils. The case is reported herein of bilateral subacute angle-closure glaucoma that occurred following coronary artery bypass graft surgery in association with tonic pupils.

Adie's tonic pupil-induced angle-closure glaucoma.

A 52-year-old woman was diagnosed as having Adie's tonic pupil in her right eye. She reported few episodes of blurring of vision in her right eye in the recent few months. On one of the routine follow-up visits, right intraocular pressure (IOP) was 70 mm Hg and on gonioscopy the angle was closed 360 degrees. Medical treatment resulted in IOP reduction and laser iridotomy was then performed. This is, to our knowledge, the first description of intermittent angle-closure glaucoma attacks induced by a tonic pupil. The possibility of angle-closure glaucoma should be considered in patients with a tonic pupil, especially with symptoms of blurred vision or ocular pain.

[Tonic pupil]. / Pupilotonia.

Authors evaluated the group of 21 patients (22 eyes) with pupillotonia. There were 2 postraumatic pupillotonias, 1 bilateral pupillotonia with systematic polyneuropathy in alcoholism, 6 Adie's pupillotonias and 9 postviral pupillotonias. In 4 one-sided pupillotonias there were finded no traumatic, viral or neurological cause. 2 patients (2 eyes) with traumatic cause, 2 patients with Adie's pupillotonia and 9 patients with viral cause had indicated the marked subjective difficulties in the eyes with pupillotonia--blurred vision, cephalea. After local application of Pilocarpin in 0.05%-0.1% concentration, these patients were indicating the marked improvement of their subjective troubles of the eyes.