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]
Entire Body System
- Camping
ネルソン Nelson syndrome ネルソン症候群 Nicolas-Favre ニコラ・ファーブル Nicolas-Favre disease ニコラ・ファーブル病 Niemann-Pick ニーマン・ピック Niemann-Pick disease ニーマン・ピック病 AlbertNiemann (1880-1921) German pediatrician and Ludwig Pick (1868-1944) Germanpathologist died in concentrate camp [jams.med.or.jp]
- Heat Intolerance
Although Holmes-Adie syndrome is considered to be benign and of unknown aetiology, troublesome symptoms may result from autonomic dysfunction; these include hyperhydrosis, heat intolerance, and syncope. [jnnp.bmj.com]
Respiratoric
- Chronic Cough
Chronic cough may be an accompaniment in the Holmes-Adie syndrome, like other forms of autonomic dysfunction. [ncbi.nlm.nih.gov]
However, in all five patients, chronic cough produced appreciable morbidity. [jnnp.bmj.com]
Ford PA, Barnes PJ, Usmani OS (2007) Holmes-Adie syndrome chronic cough. Lancet 369:342 CrossRef PubMed Google Scholar 6. [link.springer.com]
Chronic cough and Holmes-Adie syndrome. The Lancet. January 2007 369:342. ↑ Kimber J. Mitchell D, Mathias CJ. Chronic cough in the Holmes-Adie syndrome: association in five cases with autonomic dysfunction. [eyewiki.org]
Causes of this denervation include viral infection, trauma, vasospasm due to migraine, ocular surgery, tumors, and possibly chronic cough, among other potential causes. [3] Differential diagnosis Adie’s Tonic Pupil is a benign idiopathic syndrome. [eyewiki.aao.org]
- Postnasal Drip
Discussion Chronic cough is a common symptom and may be caused by various stimuli which range from neoplasms, inflammatory lung disease, and postnasal drip to increased cough sensitivity resulting from respiratory tract infections, asthma, gastro-oesophageal [jnnp.bmj.com]
Fever, nasal discharge, postnasal drip, and hyponosmia or anosmia, are helpful diagnostic characteristics. [intranet.tdmu.edu.ua]
- Dry Cough
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]
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]
Gastrointestinal
- Chronic Diarrhea
diarrhea. [7] Our patient had HAS without any feature of autonomic involvement. [amhsjournal.org]
Musculoskeletal
- 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]
Psychiatrical
- Withdrawn
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]
Neurologic
- Throbbing Headache
She used to have a chronic throbbing headache of moderate intensity particularly on winter seasons suggestive of migraine since last 8 years, but she never took any medication. [amhsjournal.org]
On presentation at the age of 26 she had a 2 year history of posture induced syncope together with paroxysmal throbbing headaches and epistaxis provoked by prolonged recumbency. [jnnp.bmj.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]
- Hyporeflexia
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 [ncbi.nlm.nih.gov]
Showing of 3 | Percent of people who have these symptoms is not available through HPO Autosomal dominant inheritance 0000006 Hyporeflexia Decreased reflex response Decreased reflexes [ more ] 0001265 Tonic pupil 0012074 Showing of 3 | Last updated: 7/ [rarediseases.info.nih.gov]
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]
Serum
- 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]
Pulmonary Function Test
- 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 Type
- 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. × Treatment Doctors may prescribe reading glasses to compensate for impaired vision in the affected eye, and pilocarpine drops to [ninds.nih.gov]
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]
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]
[ edit ] 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. [1] Thoracic sympathectomy [en.wikipedia.org]
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]
What is the prognosis? What research is being done? Clinical Trials What is Holmes-Adie syndrome ? Holmes-Adie syndrome (HAS) is a neurological disorder affecting the pupil of the eye and the autonomic nervous system. [sweyeinstitute.com]
With appropriate treatment, the prognosis of Adie Syndrome is good Who gets Adie Syndrome? (Age and Sex Distribution) Women between the ages of 20 and 40 years are most susceptible to Adie Syndrome. [dovemed.com]
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]
It is thought to result form damage to the ciliary ganglion and the dorsal root ganglion by viral or bacterial infections, however there are idiopathic etiologies 1,3. [radiopaedia.org]
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]
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]
Pathophysiology Pupil size depends on the effects of the autonomic nervous system and the iris muscle, and numerous pathophysiological processes can cause anisocoria. [emedicine.medscape.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]
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]
Prevention
We can hope that Holmes-Adie Syndrome can be prevented and treated better in the days ahead. [weheartvision.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. [sweyeinstitute.com]
Prevention of hip fracture in elderly people with use of a hip protector. [books.google.com]