Tags

Type your tag names separated by a space and hit enter

[Shy-Drager syndrome: a rare cause of orthostatic hypotension].
Dtsch Med Wochenschr. 1999 Jan 08; 124(1-2):8-12.DM

Abstract

HISTORY AND ADMISSION FINDINGS

A 71-year-old man was admitted because of treatment-resistant orthostatic hypotension of unknown aetiology. When aged 64 years he developed some impotence and later urinary incontinence and urinary frequency. At 68 years he noted vertigo on physical activity, and a year later he had signs of reversible cerebral ischaemia. At this point the Schellong test demonstrated vasovagal circulatory dysfunction. After his 70th birthday the unsteadiness on walking and standing got worse and he had recurrent syncopes. He was in a wheel-chair when hospitalized and even the unsteady walk he could maintain for only a few seconds.

INVESTIGATIONS

Plasma and urinary concentrations of catecholamines were at the lower limit of normal but failed to increase during orthostasis. Hormonal, cardiological and infectious causes of the orthostatic hypotension were excluded. Orthostatic tests after Schellong and with the tilting table showed orthostatic hypotension without increased sympathetic activity but hypertensive blood pressure levels during the recumbent period. Intravenous infusion of norepinephrine produced an excess rise in blood pressure (raised norepinephrine sensitivity). The recurrent urinary infection was shown to be due to a hypotonic bladder detrusor muscle. Neurological examination revealed cerebellar dysfunction, signs of pyramidal tract abnormality and sensory polyneuropathy. A Shy-Drager syndrome was diagnosed on the basis of the history, absent blood pressure rise and lack of catecholamine release during orthostasis with increased epinephrine sensitivity and characteristic neurological signs.

TREATMENT AND COURSE

Physiotherapy and elastic stockings with administration of mineralocorticoids as well as of one direct (norfenefrine) and one indirect (amezinium) sympathomimetic drug failed to improve adequately the abnormal orthostatic response. But on additional administration of an alpha 2-receptor antagonist (yohimbine) the patient was able to stand and walk for a few minutes, but the urinary incontinence and the other neurological signs remained treatment-resistant.

CONCLUSION

If orthostatic hypotension occurs together with neurological symptoms, a Shy-Drager syndrome should be taken into account.

Links

Publisher Full Text

Authors+Show Affiliations

Oldenburg O
Abteilung für Kardiologie, Universitätsklinikum Essen.
Karliova M
No affiliation info available
Koeppen S
No affiliation info available
Weber F
No affiliation info available
Erbel R
No affiliation info available
Philipp T
No affiliation info available
Kribben A
No affiliation info available

MeSH

Adrenergic alpha-AntagonistsBandagesDrug Therapy, CombinationHumansHypotension, OrthostaticMaleMineralocorticoidsOctopaminePhysical Therapy ModalitiesPyridazinesShy-Drager SyndromeSympathomimeticsYohimbine

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

ger

PubMed ID

9951452

Citation

Oldenburg, O, et al. "[Shy-Drager Syndrome: a Rare Cause of Orthostatic Hypotension]." Deutsche Medizinische Wochenschrift (1946), vol. 124, no. 1-2, 1999, pp. 8-12.
Oldenburg O, Karliova M, Koeppen S, et al. [Shy-Drager syndrome: a rare cause of orthostatic hypotension]. Dtsch Med Wochenschr. 1999;124(1-2):8-12.
Oldenburg, O., Karliova, M., Koeppen, S., Weber, F., Erbel, R., Philipp, T., & Kribben, A. (1999). [Shy-Drager syndrome: a rare cause of orthostatic hypotension]. Deutsche Medizinische Wochenschrift (1946), 124(1-2), 8-12.
Oldenburg O, et al. [Shy-Drager Syndrome: a Rare Cause of Orthostatic Hypotension]. Dtsch Med Wochenschr. 1999 Jan 8;124(1-2):8-12. PubMed PMID: 9951452.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Shy-Drager syndrome: a rare cause of orthostatic hypotension]. AU - Oldenburg,O, AU - Karliova,M, AU - Koeppen,S, AU - Weber,F, AU - Erbel,R, AU - Philipp,T, AU - Kribben,A, PY - 1999/2/10/pubmed PY - 1999/2/10/medline PY - 1999/2/10/entrez SP - 8 EP - 12 JF - Deutsche medizinische Wochenschrift (1946) JO - Dtsch Med Wochenschr VL - 124 IS - 1-2 N2 - HISTORY AND ADMISSION FINDINGS: A 71-year-old man was admitted because of treatment-resistant orthostatic hypotension of unknown aetiology. When aged 64 years he developed some impotence and later urinary incontinence and urinary frequency. At 68 years he noted vertigo on physical activity, and a year later he had signs of reversible cerebral ischaemia. At this point the Schellong test demonstrated vasovagal circulatory dysfunction. After his 70th birthday the unsteadiness on walking and standing got worse and he had recurrent syncopes. He was in a wheel-chair when hospitalized and even the unsteady walk he could maintain for only a few seconds. INVESTIGATIONS: Plasma and urinary concentrations of catecholamines were at the lower limit of normal but failed to increase during orthostasis. Hormonal, cardiological and infectious causes of the orthostatic hypotension were excluded. Orthostatic tests after Schellong and with the tilting table showed orthostatic hypotension without increased sympathetic activity but hypertensive blood pressure levels during the recumbent period. Intravenous infusion of norepinephrine produced an excess rise in blood pressure (raised norepinephrine sensitivity). The recurrent urinary infection was shown to be due to a hypotonic bladder detrusor muscle. Neurological examination revealed cerebellar dysfunction, signs of pyramidal tract abnormality and sensory polyneuropathy. A Shy-Drager syndrome was diagnosed on the basis of the history, absent blood pressure rise and lack of catecholamine release during orthostasis with increased epinephrine sensitivity and characteristic neurological signs. TREATMENT AND COURSE: Physiotherapy and elastic stockings with administration of mineralocorticoids as well as of one direct (norfenefrine) and one indirect (amezinium) sympathomimetic drug failed to improve adequately the abnormal orthostatic response. But on additional administration of an alpha 2-receptor antagonist (yohimbine) the patient was able to stand and walk for a few minutes, but the urinary incontinence and the other neurological signs remained treatment-resistant. CONCLUSION: If orthostatic hypotension occurs together with neurological symptoms, a Shy-Drager syndrome should be taken into account. SN - 0012-0472 UR - https://www.unboundmedicine.com/medline/citation/9951452/[Shy_Drager_syndrome:_a_rare_cause_of_orthostatic_hypotension]_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-2008-1062602 DB - PRIME DP - Unbound Medicine ER -