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Adie syndrome: evidence for refractive error and accomodative asymmetry as the cause of amblyopia.

PURPOSE: To report objective changes in accommodation in a child with Adie syndrome. METHODS: A child aged 2 years 10 months when initially examined was found to have good visual acuity in both eyes, a low degree of hypermetropia (isometropic), and Adie pupil presumed to be caused by chicken pox that had occurred 2 months earlier. Amblyopia developed but responded well to treatment, which involved correction of the refractive error and occlusion therapy. Objective changes in the refraction of the eye were measured on the Canon R1 autorefractor at 3.8 m and 33 cm. RESULT: The degree of accommodation in the affected eye when both eyes were open was markedly reduced. CONCLUSION: The presence of isometropic hypermetropia, which remains uncorrected when Adie syndrome is present, can lead to the development of amblyopia in a child.

Accommodation, Ocular↗

Holmes-Adie syndrome, autoimmune hepatitis and celiac disease: a case report.

A 35-year-old female patient presented with the following symptoms of Holmes-Adie syndrome: photophobia,enlargement of the left pupil unresponsive to light, Achilles areflexia. The pilocarpine test was positive. No tumor or other neurological abnormality was found. She had a 19-year history of autoimmune hepatitis. Flares up were observed following each 3 deliveries. At age of 31 she presented with diarrhea and weight loss. Abdominal tumor was detected by ultrasound. The surgically removed tumor was histologically a benign mesenteric multicystic lymphangioma.Simultaneously, celiac disease was diagnosed.Gluten-free diet resulted in a significant improvement of celiac disease,but not of autoimmune hepatitis. Autonomic neuropathy was proven by standard cardiovascular tests. The patient was a homozygous carrier for HLA DQ2 antigen characteristic for celiac disease and heterozygous for HLA DR3 B8 frequent in autoimmune liver diseases. Our novel observation on association of Holmes-Adie syndrome with autoimmune hepatitis and celiac disease is suggestive for a common immunological background for all three entities present in a patient with mesenteric multicystic lymphangioma.

Adie Syndrome↗

Idiopathic unilateral hyperhidrosis with Holmes-Adie syndrome: case report.

Hyperhidrosis is a disease that is characterized by excessive sweating due to hyperactivation of eccrine sweat glands. It may be localized or generalized form. Holmes-Adie syndrome is an idiopathic disease with unilateral pupil dilatation and loss of deep tendon reflexes. We present a 37-year-old female patient diagnosed with unilateral hyperhidrosis coincident with Holmes-Adie syndrome because of this unusual presentation.

Adie Syndrome↗

[Adie syndrome].

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Adie Syndrome↗

On the cause of hyporeflexia in the Holmes-Adie syndrome.

Electrophysiologic studies were carried out on 11 patients with Holmes-Adie syndrome, 8 of whom had reduced or absent ankle jerks. Conduction velocities and evoked nerve and muscle compound action potentials in the peroneal, posterior tibial, and sural nerves were normal. The H reflex was absent (or virtually absent) in the patients with depressed reflexes. The amplitude of the composite Ia EPSP in single soleus motoneurons was estimated from changes in firing probability of voluntarily activated soleus motor units in response to stimulation of low threshold afferents in the tibial nerve. These amplitudes were used to test the afferent side of the reflex pathway. Composite group Ia EPSPs in Holmes-Aide patients with hyporeflexia were smaller than normal or absent, indicating that the areflexia in the Holmes-Aide syndrome is due to loss of large spindle afferents or reduced effectiveness of their monosynaptic connections to motoneurons.

Action Potentials↗

[Physiopathology of the Holmes-Adie syndrome. Neurophysiological examinations of the autoreflex disorder].

Areflexia of 8 patients with Holmes-Adie-syndrome was investigated. The voluntary movements, electromyograms, motor and sensory nerve conduction velocities and the nerve action potentials were normal. The mechanically elicited afferents of mechanoreceptors of the soleus muscle (recorded with semimicro-electrodes) were not different in Holmes-Adie-patients with areflexia and normals.

Adie Syndrome↗

Tonic vibration reflex in Holmes-Adie syndrome: an electrophysiological study.

The pathophysiological mechanisms underlying benign areflexia were studied in six patients with Holmes-Adie syndrome. No impairment of sensory conduction velocity of sural nerve was found. A normal tonic vibration reflex was obtained in all patients. H reflex was absent in five patients, but responses like F waves were recorded in three subjects. These findings suggest that muscle spindles are not affected and that spinal motoneurone excitability is normal.

Adie Syndrome↗

Orthostatic hypotension and the Holmes-Adie syndrome. A study of two patients with afferent baroreceptor block.

Two patients who presented with symptoms due to orthostatic hypotension were found on examination to have the Holmes-Adie syndrome. Physiological investigation suggested that they both had an afferent block from baroreceptors in contrast to the efferent autonomic block found in most other cases of idiopathic orthostatic hypotension, including the cases of multisystem disease, now often called the Shy-Drager syndrome.

Adie Syndrome↗