Glucocorticoid Insufficiency, Achalasia, Alacrima with Autonomic and Motor Neuropathy

Brief Reports1 January 1987Glucocorticoid Insufficiency, Achalasia, Alacrima with Autonomic and Motor NeuropathyB. G. STUCKEY, F. L. MASTAGLIA, W. D. REED, P. T. PULLANB. G. STUCKEY, F. L. MASTAGLIA, W. D. REED, P. T. PULLANAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-106-1-62 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptWe report the case of a patient with adrenal disease with achalasia and alacrima, which appear to be part of a widespread defect in adrenal and nervous system function.A 21-year-old white woman at age 6 had had hypoglycaemia, hyperpigmentation, low plasma cortisol level (30 nmol/L; normal, 200 to 600), and a lack of cortisol response to cosyntropin. Alacrima had been noted since infancy but there were no other abnormal neurologic features. A roentgenogram of the chest at age 9 showed bilateral pulmonary opacities presumed due to aspiration; these have persisted. Nocturnal cough, vomiting of undigested food, and recurrent chest...References1. ALLGROVECLAYDENGRANTMACAULAY JGDJ. Familial glucocorticoid deficiency with achalasia of the cardia and deficient tear production. Lancet. 1978;1:1284-6. CrossrefMedlineGoogle Scholar2. LANESPLOTNICKBYNUM RLT. Glucocorticoid and partial mineralocorticoid deficiency associated with achalasia. J Clin Endocrinol Metab. 1980;50:268-70. CrossrefMedlineGoogle Scholar3. GEFFNERLIPPEKAPLAN MBS. Selective ACTH insensitivity, achalasia, and alacrima: multisystem disorder presenting in childhood. Pediatr Res. 1983;17:532-6. CrossrefMedlineGoogle Scholar4. DEGLI ESPOSTIAMBROSIONIGIARDINA AGA. Sindrome familiare di ipoglicocorticismo da insensibilita all ACTH, acalasia, alacrimia, con associata neuromiopatia distale. Minerva Pediatr. 1985;37:353-9. MedlineGoogle Scholar5. POMBODEVESATABORDA MJA. 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Author, Article, and Disclosure InformationAffiliations: Sir Charles Gairdner Hospital Nedlands Australia PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited ByCardiovascular dysautonomia in Achalasia Patients: Blood pressure and heart rate variability alterationsAchalasiaEpidemiology, Evaluation, and Classification of Achalasia and Other Esophageal Motility DisordersEpidemiology, Diagnosis, and Medical Management of AchalasiaMotility Disorders of the Esophagus"A" motor neuron diseaseEsophageal Neuromuscular Function and Motility DisordersChapter 5 The Molecular Basis of Adrenocorticotrophin Resistance SyndromeA Novel DNA Sequence Variation in the First Genetically Confirmed Allgrove Syndrome in IranAllgrove syndrome in adulthoodOverview of Hyper- and HypocortisolismMotilitätsstörungen von Pharynx und ÖsophagusPrimary Enteric Neuropathies Underlying Gastrointestinal Motor DysfunctionPathophysiology of achalasiaPeriventricular Brain Heterotopias in a Child With Adrenocortical Insufficiency, Achalasia, Alacrima, and Neurologic Abnormalities (Allgrove Syndrome)Adrenocorticotropin Insensitivity SyndromesACTH Resistance SyndromesPATHOPHYSIOLOGY AND ENDOSCOPIC/BALLOON TREATMENT OF ESOPHAGEAL MOTILITY DISORDERSAllgrove syndrome: Documenting cholinergic dysfunction by autonomic testsFour siblings with achalasia, alacrimia and neurological abnormalities in a consanguineous familyThe Enteric Nervous SystemFamilial adrenocortiocotropin unresponsiveness associated With alacrima and achalasia: Biochemical and molecular studies in two siblings with clincial heterogeneityThe "4A" syndrome: Adrenocortical insufficiency associated with achalasia, alacrima, autonomic and other neurological abnormalitiesVariant of the Triple A syndromeFamilial Adrenal Insufficiency, Achalasia, Alacrima, Peripheral Neuropathy, Microcephaly, Normal Plasma Very Long Chain Fatty Acids, and Normal Muscle Mitochondrial Respiratory Chain EnzymesEsophageal Physiology and PathophysiologyNeurological and adrenal dysfunction in the adrenal insufficiency/alacrima/achalasia (3A) syndrome.Epidemiology of hospitalization for achalasia in the United StatesFive year prospective study of the incidence, clinical features, and diagnosis of achalasia in Edinburgh.Achalasia of the cardia in childhood and the syndrome of achalasia alacrima and ACTH insensitivityAdrenocorticotropic hormone insensitivity associated with autonomic nervous system disordersAllgrove syndrome: an autosomal recessive syndrome of ACTH insensitivity, achalasia and alacrimaAchalasiaFamilial Achalasia, Microcephaly, and Mental RetardationEsophageal motility disordersSouthwestern Internal Medicine Conference: Esophageal Achalasia Syndromes 1 January 1987Volume 106, Issue 1Page: 62-64KeywordsBlood plasmaCortisolEndocrinologyFoodHyperpigmentationNervous systemNeuropathyThoraxVomiting Issue Published: 1 January 1987 PDF DownloadLoading ...