Primary Autonomic Failure: Three Clinical Presentations of One Disease?

Editorials5 September 2000Primary Autonomic Failure: Three Clinical Presentations of One Disease?Horacio Kaufmann, MDHoracio Kaufmann, MDFrom Mount Sinai School of Medicine; New York, NY 10029Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-133-5-200009050-00014 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Three neurodegenerative diseases of unknown cause involve primary autonomic failure. These diseases are pure autonomic failure, in which autonomic impairment (that is, orthostatic hypotension and bladder and sexual dysfunction) occurs alone; Parkinson disease, in which autonomic failure is combined with an extrapyramidal movement disorder; and multiple-system atrophy (also called Shy–Drager syndrome), in which autonomic failure is combined with an extrapyramidal or cerebellar movement disorder or both (1).During the early stages of multiple-system atrophy, autonomic deficits may be the sole clinical manifestation; therefore, the disease may resemble pure autonomic failure. However, after a variable period that can be as long ...References1. . Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple-system atrophy. J Neurol Sci. 1996;144:218-9. MedlineGoogle Scholar2. Kaufmann H, Oribe E, Miller M, Knott P, Wiltshire-Clement M, Yahr MD. Hypotension-induced vasopressin release distinguishes between pure autonomic failure and multiple system atrophy with autonomic failure. Neurology. 1992;42(3 Pt 1):590-3. CrossrefGoogle Scholar3. Kimber JR, Watson L, Mathias CJ. Distinction of idiopathic Parkinson's disease from multiple-system atrophy by stimulation of growth-hormone release with clonidine. Lancet. 1997;349:1877-81. CrossrefMedlineGoogle Scholar4. Goldstein DS, Polinsky RJ, Garty M, Robertson D, Brown RT, Biaggioni I, et al . Patterns of plasma levels of catechols in neurogenic orthostatic hypotension. Ann Neurol. 1989;26:558-63. CrossrefMedlineGoogle Scholar5. Pramstaller PP, Wenning GK, Smith SJ, Beck RO, Quinn NP, Fowler CJ. Nerve conduction studies, skeletal muscle EMG, and sphincter EMG in multiple-system atrophy. J Neurol Neurosurg Psychiatry. 1995;58:618-21. CrossrefMedlineGoogle Scholar6. Konagaya M, Konagaya Y, Honda H, Iida M. A clinico-MRI study of extrapyramidal symptoms in multiple-system atrophy–linear hyperintensity in the outer margin of the putamen. No To Shinkei. 1993;45:509-13. MedlineGoogle Scholar7. Goldstein DS, Holmes C, Li S, Bruce S, Verhagen Metman L, Cannon RO. Cardiac sympathetic denervation in Parkinson disease. Ann Intern Med. 2000;133:338-47. LinkGoogle Scholar8. Hirayama M, Hakusui S, Koike Y, Ito K, Kato T, Ikeda M, et al . A scintigraphical qualitative analysis of peripheral vascular sympathetic function with meta-[123I]iodobenzylguanidine in neurological patients with autonomic failure. J Auton Nerv Syst. 1995;53:230-4. CrossrefMedlineGoogle Scholar9. Braune S, Reinhardt M, Schnitzer R, Riedel A, Lucking CH. Cardiac uptake of [123I]MIBG separates Parkinson's disease from multiple-system atrophy. Neurology. 1999;53:1020-5. CrossrefMedlineGoogle Scholar10. Orimo S, Ozawa E, Nakade S, Sugimoto T, Mizusawa H. (123)I-metaiodobenzylguanidine myocardial scintigraphy in Parkinson's disease. J Neurol Neurosurg Psychiatry. 1999;67:189-94. CrossrefMedlineGoogle Scholar11. Goldstein DS, Holmes C, Cannon RO, Eisenhofer G, Kopin IJ. Sympathetic cardioneuropathy in dysautonomias. N Engl J Med. 1997;336:696-702. CrossrefMedlineGoogle Scholar12. Papp MI, Lantos PL. Accumulation of tubular structures in oligodendroglial and neuronal cells as the basic alteration in multiple-system atrophy. J Neurol Sci. 1992;107:172-82. CrossrefMedlineGoogle Scholar13. Hague K, Lento P, Morgello S, Caro S, Kaufmann H. The distribution of Lewy bodies in pure autonomic failure: autopsy findings and review of the literature. Acta Neuropathol Berl. 1997;94:192-6. CrossrefMedlineGoogle Scholar14. Den Hartog Jager WA, Bethlem J. The distribution of Lewy bodies in the central and autonomic nervous systems in idiopathic paralysis agitans. J Neurol Neurosurg Psychiat. 1960;23:283-9. CrossrefMedlineGoogle Scholar15. Wakabayashi K, Takahashi H, Ohama E, Takeda S, Ikuta F. Lewy bodies in the visceral autonomic nervous system in Parkinson's disease. Adv Neurol. 1993;60:609-12. MedlineGoogle Scholar16. Polymeropoulos MH, Lavedan C, Leroy E, Ide SE, Dehejia A, Dutra A, et al . Mutation in the α-synuclein gene identified in families with Parkinson's disease. Science. 1997;276:2045-7. CrossrefMedlineGoogle Scholar17. Spillantini MG, Schmidt ML, Lee VM, Trojanowski JQ, Jakes R, Goedert M. α-Synuclein in Lewy bodies [Letter]. Nature. 1997;388:839-40. CrossrefMedlineGoogle Scholar18. Gai WP, Power JH, Blumbergs PC, Blessing WW. Multiple-system atrophy: a new α-synuclein disease? [Letter]. Lancet. 1998;352:547-8. CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAffiliations: From Mount Sinai School of Medicine; New York, NY 10029Corresponding Author: Horacio Kaufmann, MD, Mount Sinai School of Medicine, Box 1052, New York, NY 10029; e-mail, Horacio.[email protected]edu. 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