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Holmes-Adie Syndrome

Adie's Syndrome


Presentation

  • We present a 37-year-old female patient diagnosed with unilateral hyperhidrosis coincident with Holmes-Adie syndrome because of this unusual presentation.[ncbi.nlm.nih.gov]
Dry Cough
  • Five such patients are reported on who also had a troublesome chronic dry cough, which was of unknown aetiology and was resistant to a range of treatments. The cough may be related to involvement of afferent or efferent pathways in the vagus.[ncbi.nlm.nih.gov]
  • She continued to have an unremitting dry cough. Patient 5 was a 66 year white woman who was a non-smoker. She had presented 12 years previously with a troublesome dry cough.[jnnp.bmj.com]
Paroxysmal Cough
  • Two years later she developed asymmetric facial sweating and paroxysmal coughing attacks, particularly while eating and during prolonged talking.[jnnp.bmj.com]
Aspiration
  • In the three who underwent cine-video fluoroscopy, there was no evidence of laryngeal aspiration. In three, capsaicin challenge indicated increased cough sensitivity.[jnnp.bmj.com]
Short Arm
  • Analysis of 1 1 kindreds and linkage to the short arm of chromosome 2. J Clin Invest 1996:97: 699-705. ‏[books.google.com]
Cranial Neuropathy
  • We report a case of a Holmes-Adie syndrome variant with hitherto unreported cranial neuropathy. A 41 year old woman developed insidious onset of sensory symptoms related to her left trigeminal and chorda tympani nerves over a few months.[ncbi.nlm.nih.gov]
  • Hereditary motor and sensory neuropathy type I (HMNS I) associated with cranial neuropathy: an autopsy case report . Acta Neurol Scand, 1990; 82: 368–373. PubMed Google Scholar [15] Ulrich J . Morphological basis of Adie's syndrome .[link.springer.com]

Workup

  • Ophthalmologic examination confirmed Holmes-Adie syndrome (HAS), and peripheral distal polyneuropathy, orthostatic hypotension and leg hyperhidrosis were detected on further workup.[ncbi.nlm.nih.gov]
  • “Their deep tendon reflexes were also tested clinically and a full autonomic and neurological workup was performed to look for an underlying cause for their Harlequin syndrome.”[eyeworld.org]
  • Can be dangerous if a manifestation Horner's syndrome (e.g. carotid dissection) or from damage to the third nerve (e.g. aneurysmal expansion) Consider further workup such as imaging if anisocoria is suspected to be from a pathologic process Relative Afferent[stanfordmedicine25.stanford.edu]
  • Given that malignancy is a prominent feature of recently diagnosed preganglionic Horner syndrome, patients may avoid an unnecessary extensive workup for carcinoma if the lesion can be shown to be old or long-standing.[emedicine.medscape.com]
Hypercapnia
  • Bernardi L, Hilz M, Stemper B, Passino C, Welsch G, Axelrod FB (2003) Respiratory and cerebrovascular responses to hypoxia and hypercapnia in familial dysautonomia.[link.springer.com]
Reversible Airway Obstruction
  • Investigations for cough indicated mild reversible airway obstruction. Treatment with inhaled bronchodilators and steroids (salbutamol, salmeterol, beclomethasone) produced a partial reduction in coughing.[jnnp.bmj.com]
HLA-DR3
  • 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.[ncbi.nlm.nih.gov]
HLA-DQ2
  • 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.[ncbi.nlm.nih.gov]

Treatment

  • Thoracic sympathectomy, which severs the involved sympathetic nerve, is the definitive treatment for excessive sweating.[ninds.nih.gov]
  • Treatments of Adie’s Syndrome The usual treatment of a standardised Adie syndrome is to prescribe reading glasses to correct for impairment of the eye(s).[whereincity.com]
  • The usual treatment of a standardised Adie syndrome is to prescribe reading glasses to correct for impairment of the eye(s).[1] Pilocarpine drops may be administered as a treatment as well as a diagnostic measure, and should be applied three times daily[eyecare-information-service.org.uk]
  • Five such patients are reported on who also had a troublesome chronic dry cough, which was of unknown aetiology and was resistant to a range of treatments. The cough may be related to involvement of afferent or efferent pathways in the vagus.[ncbi.nlm.nih.gov]
  • Although bronchodilator treatment improved the dyspnoea it did not prevent coughing. She had been hypertensive and noted that treatment with an angiotensin converting enzyme inhibitor exacerbated her cough and the drug was withdrawn.[jnnp.bmj.com]

Prognosis

  • Prognosis Holmes-Adie syndrome is not life-threatening or disabling. The loss of deep tendon reflexes is permanent. Some symptoms of the disorder may progress.[ninds.nih.gov]
  • This has a number of implications, including those relating to aetiology and prognosis. The authors recommend regular clinical and laboratory follow-up, especially of cardiovascular autonomic function, in patients with HAS.[ncbi.nlm.nih.gov]
  • Pilocarpine drops may be administered as a treatment as well as a diagnostic measure, and should be applied three times daily.[1] Thoracic sympathectomy is the definitive treatment of diaphoresis, if the condition is not treatable by drug therapy.[1] Prognosis[eyecare-information-service.org.uk]
  • […] tonic pupils: Holmes Adie syndrome or generalized neuropathy Adie syndrome - GARD Center Diagram of the Eye Eye Disease Simulations Adie Syndrome Holmes-Adie syndrome – definition Adie's syndrome Alternative eponyms Adie syndrome - Symptoms, Diagnosis, Prognosis[weheartvision.com]
  • What is the prognosis? Holmes-Adie syndrome is not life-threatening or disabling. The loss of deep tendon reflexes is permanent. Some symptoms of the disorder may progress. For most individuals, pilocarpine drops and glasses will improve vision.[web.archive.org]

Etiology

  • The HAS can be either idiopathic or associated with neuropathy of various etiology (autoimmune, paraneoplastic and infectious). In our patient, the pupillotonia was the first and early symptom of hitherto unrecognized HIV neuropathy.[ncbi.nlm.nih.gov]
  • A syndrome of unknown etiology and pathology characterized by tonic pupillary reactions with tendon reflexes possibly absent or diminished.[medical-dictionary.thefreedictionary.com]
  • The book describes the varied etiology of the disease, but emphasizes clinical protocols and step-by-step procedures for diagnosis and treatment planning.[books.google.com]
  • The etiology is still unknown. Bibliography J. Ware: Observations relative to the near and distant sight of different persons. Philosophical Transactions of the Royal Society of London, 1813: 31-50.[whonamedit.com]

Epidemiology

  • […] treatable by drug therapy. [1] Prognosis [ edit ] Adie's syndrome is not life-threatening or disabling. [1] As such, there is no mortality rate relating to the condition; however, loss of deep tendon reflexes is permanent and may progress over time. [1] Epidemiology[en.wikipedia.org]
  • Th e epidemiology of Sjogren’s syndrome. Clin Epidemiol 2014;6:247-55. Gabriel SE, Michaud K. Epidemiological studies in incidence, prevalence, mortality, and comorbidity of the rheumatic diseases. Arthritis Res Th er 2009;11:229.[scopublicaciones.socoftal.com]
  • Epidemiology Frequency United States Anisocoria is common, although no overall prevalence statistics are available. The incidence and prevalence data for anisocoria depend on the specific pathophysiology and population.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • Abstract 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.[ncbi.nlm.nih.gov]
  • Disponible en ligne depuis le mercredi 20 septembre 2017 Doi : 10.1016/j.neucli.2017.08.001 Received : 20 June 2017 ; accepted : 25 August 2017 Areflexia in Holmes-Adie syndrome: A sign in search of its pathophysiology Aréflexie dans le syndrome de Holmes-Adie[em-consulte.com]
  • Bilateral tonic pupil and polyneuropathy in Sjögren's syndrome: a common pathophysiological mechanism? Eur Neurol, 1991; 31: 114–116.[link.springer.com]
  • […] loss of deep tendon reflexes, and abnormalities of sweating. [1] Other signs may include hyperopia due to accommodative paresis, photophobia and difficulty reading. [3] Some individual with Adie syndrome may also have cardiovascular abnormalities. [4] Pathophysiology[en.wikipedia.org]

Prevention

  • We can hope that Holmes-Adie Syndrome can be prevented and treated better in the days ahead.[weheartvision.com]
  • .: Prevention of hip fracture in elderly people with use of a hip protector. ‏[books.google.com]
  • Much of this research focuses on finding better ways to prevent, treat, and ultimately cure disorders, such as HAS. Select this link to view a list of studies currently seeking patients.[web.archive.org]
  • Much of this research focuses on finding better ways to prevent, treat, and ultimately cure disorders, such as HAS. Information from the National Library of Medicine’s MedlinePlus Autonomic Nervous System Disorders Eye Diseases[ninds.nih.gov]

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