title: Neurological and adrenal dysfunction in the adrenal insufficiency/alacrima/achalasia (3A) syndrome.
abstract: Review of 20 patients with glucocorticoid deficiency (three cases also with salt loss) associated with absent tear secretion (19 cases) and achalasia of the cardia (15 cases) revealed neurological abnormalities in 17 including hyper-reflexia, muscle weakness, dysarthria, and ataxia together with impaired intelligence and abnormal autonomic function, particularly postural hypotension. These findings indicate that significant neurological problems are common in this multisystem disorder.
authors.affiliation: Hospital for Sick Children, London.
authors.forename: D B
authors.initials: DB
authors.lastname: Grant
authors.forename: N D
authors.initials: ND
authors.lastname: Barnes
authors.forename: M
authors.initials: M
authors.lastname: Dumic
authors.forename: M
authors.initials: M
authors.lastname: Ginalska-Malinowska
authors.forename: P J
authors.initials: PJ
authors.lastname: Milla
authors.forename: W
authors.initials: W
authors.lastname: von Petrykowski
authors.forename: R J
authors.initials: RJ
authors.lastname: Rowlatt
authors.forename: R
authors.initials: R
authors.lastname: Steendijk
authors.forename: J H
authors.initials: JH
authors.lastname: Wales
authors.forename: E
authors.initials: E
authors.lastname: Werder
mesh_terms: D000224:Addison Disease / Q000150:complications / Q000503:physiopathology*; D000293:Adolescent; D000302:Adrenal Cortex / Q000503:physiopathology; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004931:Esophageal Achalasia / Q000150:complications / Q000503:physiopathology*; D005192:Family Health; D005260:Female; D006801:Humans; D007360:Intelligence; D008297:Male; D009420:Nervous System / Q000503:physiopathology; D009422:Nervous System Diseases / Q000150:complications / Q000503:physiopathology*; D013577:Syndrome; D013666:Tears / Q000378:metabolism*