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Medicina Clinica Aug 2018
Topics: Adie Syndrome; Female; Humans; Miotics; Photography; Pilocarpine; Young Adult
PubMed: 29196034
DOI: 10.1016/j.medcli.2017.10.024 -
Acta Neurologica Belgica Jun 2021Harlequin syndrome is a rare condition, presenting with unilateral facial flushing and hyperhidrosis in response to physical exercise, heat or emotional stressors and... (Review)
Review
Harlequin syndrome is a rare condition, presenting with unilateral facial flushing and hyperhidrosis in response to physical exercise, heat or emotional stressors and has scarcely been reported in pediatric patients. It is caused by a dysfunction of vasomotor and sudomotor sympathetic fiber activity inhibiting the ability to flush on the affected side, causing the neurologically intact side to appear red. We present three pediatric cases of this uncommon syndrome, each of them of different origin and displaying distinct associated (neurological) symptoms, and review medical literature. Insight into the anatomical structure of the thoracocervical and facial sympathetic nervous system is pivotal as it dictates symptomatology. About half of Harlequin syndrome cases are complicated with ocular symptoms and a minority may be part of more extensive partial dysautonomias affecting facial sudomotor, vasomotor and pupillary responses, such as Holmes-Adie syndrome and Ross syndrome. Etiology is generally idiopathic, however, cases secondary to surgery, trauma or infection have been described. Considering its predominantly self-limiting nature, treatment is usually unnecessary and should be restricted to incapacitating cases.
Topics: Autonomic Nervous System; Autonomic Nervous System Diseases; Child, Preschool; Female; Flushing; Humans; Hypohidrosis
PubMed: 33515404
DOI: 10.1007/s13760-021-01593-6 -
Journal of Clinical Neuro-ophthalmology Mar 1989Two young adults, aged 24 and 31, had a long history of small, poorly reactive pupils. There was no history of large pupils, and a review of old photographs confirmed 10...
Two young adults, aged 24 and 31, had a long history of small, poorly reactive pupils. There was no history of large pupils, and a review of old photographs confirmed 10 and 5 years, respectively, of miosis. Both were found to have bilateral tonic pupils that were supersensitive to diluted pilocarpine. Although it is possible that they had an unusually early onset of bilateral Adie's syndrome with dilated pupils that was not noticed, it is suggested that some patients might have primary miotic Adie's pupils without ever passing through a mydriatic phase.
Topics: Adie Syndrome; Adult; Humans; Male; Pupil; Reflex, Pupillary
PubMed: 2522946
DOI: 10.3109/01658108909019506 -
American Journal of Ophthalmology May 1997To report Adie syndrome as the initial sign of primary Sjögren syndrome.
PURPOSE
To report Adie syndrome as the initial sign of primary Sjögren syndrome.
METHODS
Case report.
RESULTS
Adie syndrome was associated with necrotizing gingivitis and xerostomia. Antibodies against Ro (SS-A) were present. Prednisone and antimalarial drugs were ineffective in treating Adie syndrome but improved the necrotizing gingivitis.
CONCLUSION
Search for Sjögren syndrome is mandated in patients with Adie syndrome. The latter condition is likely related to ganglionitis, a mechanism responsible for peripheral nervous system involvement in primary Sjögren syndrome.
Topics: Adie Syndrome; Adult; Anti-Inflammatory Agents; Antibodies, Antinuclear; Antimalarials; Autoantigens; Female; Gingivitis, Necrotizing Ulcerative; Humans; Prednisone; RNA, Small Cytoplasmic; Ribonucleoproteins; Sjogren's Syndrome; Xerostomia
PubMed: 9152077
DOI: 10.1016/s0002-9394(14)71084-0 -
Journal of Neuro-ophthalmology : the... Dec 2023
Topics: Humans; Tonic Pupil; Adie Syndrome; Pupil; Autoantibodies
PubMed: 35439231
DOI: 10.1097/WNO.0000000000001598 -
Neurophysiologie Clinique = Clinical... Dec 2017
Topics: Adie Syndrome; Adult; Female; Humans; Lower Extremity; Reflex; Reflex, Abnormal
PubMed: 28939242
DOI: 10.1016/j.neucli.2017.08.001 -
Molecular and Clinical Oncology Jun 2017Adie's syndrome (AS) and paraneoplastic sensorimotor neuropathy with cerebellar ataxia (PSN CA) are extremely rare, rapidly progressive, autoimmune diseases associated...
Synchronous Adie's syndrome and type 1 antineuronal nuclear antibody (anti-Hu)-related paraneoplastic neurological syndromes as predictors of complete response in limited-stage small-cell lung cancer: A case report.
Adie's syndrome (AS) and paraneoplastic sensorimotor neuropathy with cerebellar ataxia (PSN CA) are extremely rare, rapidly progressive, autoimmune diseases associated with the development of antibodies against neuronal-specific Hu proteins that are abnormally expressed in small-cell lung cancer (SCLC). We herein present the unique case of a 55-year-old obese woman, previous heavy smoker, who, during treatment with standard cisplatin-etoposide chemotherapy for limited-stage SCLC, developed simultaneous AS and worsening symptoms consistent with PSN CA that led to significant neurological disability and severe axonal electrophysiological pattern on nerve conduction studies. Serology confirmed the presence of low-titre type 1 antineuronal nuclear antibodies (ANNA-1), previously referred to as anti-Hu antibodies. Following plasmapheresis, immunosuppressive therapy and physical rehabilitation, the neurological symptoms progressively improved. The tumour completely regressed, with no recurrence detected on subsequent radiological examinations. The aim of this case was to highlight the importance of a multidisciplinary team approach for early recognition and rapid treatment of paraneoplastic neurological syndromes (PNS) as key to achieving significant recovery and marked improvement of the neurological deficit. This report extends the literature by confirming earlier studies showing that the presence of serum ANNA-1 in SCLC, an aggressive type of pulmonary carcinoma that is challenging to treat, may portend a more favourable prognosis and response to chemotherapy. Thus, patients with SCLC and new-onset neurological symptoms should be tested for ANNA-1. The role of a multimodality approach to treating PNS is also emphasized.
PubMed: 28588791
DOI: 10.3892/mco.2017.1248 -
Archives of Ophthalmology (Chicago,... Jan 1970
Review
Topics: Adie Syndrome; Adrenal Rest Tumor; Amblyopia; Animals; Behcet Syndrome; Biopsy; Brain Stem; Craniopharyngioma; Eye Diseases; Female; Humans; Lipidoses; Male; Nervous System Diseases; Neurofibromatosis 1; Nystagmus, Pathologic; Ophthalmoplegia; Optic Atrophy; Optic Chiasm; Optic Neuritis; Papilledema; Retina; Visual Fields; Whipple Disease
PubMed: 4190335
DOI: 10.1001/archopht.1970.00990030105020 -
L' Ospedale Maggiore Jul 1948
Topics: Adie Syndrome; Humans; Reflex; Reflex, Pupillary
PubMed: 18888901
DOI: No ID Found -
Revue D'oto-neuro-ophtalmologie 1954
Topics: Adie Syndrome; Cervical Plexus; Disease; Female; Humans; Uterine Cervical Diseases
PubMed: 14372717
DOI: No ID Found