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Orthostatic hypotension

MedGen UID:
43803
Concept ID:
C0020651
Disease or Syndrome
Synonyms: Postural hypotension
SNOMED CT: Orthostatic hypotension (28651003); Postural hypotension (28651003)
 
OMIM®: 146500
HPO: HP:0001278

Definition

A significant drop in BLOOD PRESSURE after assuming a standing position. Orthostatic hypotension is a finding, and defined as a 20-mm Hg decrease in systolic pressure or a 10-mm Hg decrease in diastolic pressure 3 minutes after the person has risen from supine to standing. Symptoms generally include DIZZINESS, blurred vision, and SYNCOPE. [from MeSH]

Conditions with this feature

Familial dysautonomia
MedGen UID:
41678
Concept ID:
C0013364
Congenital Abnormality
Familial dysautonomia (FD) affects the development and survival of sensory, sympathetic, and parasympathetic neurons. It is a debilitating disease present from birth. Neuronal degeneration progresses throughout life. Affected individuals have gastrointestinal dysfunction, vomiting crises, recurrent pneumonia, altered sensitivity to pain and temperature perception, and cardiovascular instability. About 40% of individuals have autonomic crises. Hypotonia contributes to delay in acquisition of motor milestones. Older individuals often have a broad-based and ataxic gait that deteriorates over time. Life expectancy is decreased.
Sandhoff disease
MedGen UID:
11313
Concept ID:
C0036161
Disease or Syndrome
Sandhoff disease is a progressive neurodegenerative disorder characterized by an accumulation of GM2 gangliosides, particularly in neurons, and is clinically indistinguishable from Tay-Sachs disease (272800).
Cutis laxa, X-linked
MedGen UID:
82793
Concept ID:
C0268353
Congenital Abnormality
Occipital horn syndrome is a rare connective tissue disorder characterized by hyperelastic and bruisable skin, hernias, bladder diverticula, hyperextensible joints, varicosities, and multiple skeletal abnormalities. The disorder is sometimes accompanied by mild neurologic impairment, and bony abnormalities of the occiput are a common feature, giving rise to the name (summary by Das et al., 1995).
Glucocorticoid deficiency with achalasia
MedGen UID:
82889
Concept ID:
C0271742
Disease or Syndrome
Triple A syndrome is an inherited condition characterized by three specific features: achalasia, Addison disease, and alacrima. Achalasia is a disorder that affects the ability to move food through the esophagus, the tube that carries food from the throat to the stomach. It can lead to severe feeding difficulties and low blood sugar (hypoglycemia). Addison disease, also known as primary adrenal insufficiency, is caused by abnormal function of the small hormone-producing glands on top of each kidney (adrenal glands). The main features of Addison disease include fatigue, loss of appetite, weight loss, low blood pressure, and darkening of the skin. The third major feature of triple A syndrome is a reduced or absent ability to secrete tears (alacrima). Most people with triple A syndrome have all three of these features, although some have only two.Many of the features of triple A syndrome are caused by dysfunction of the autonomic nervous system. This part of the nervous system controls involuntary body processes such as digestion, blood pressure, and body temperature. People with triple A syndrome often experience abnormal sweating, difficulty regulating blood pressure, unequal pupil size (anisocoria), and other signs and symptoms of autonomic nervous system dysfunction (dysautonomia).People with this condition may have other neurological abnormalities, such as developmental delay, intellectual disability, speech problems (dysarthria), and a small head size (microcephaly). In addition, affected individuals commonly experience muscle weakness, movement problems, and nerve abnormalities in their extremities (peripheral neuropathy). Some develop optic atrophy, which is the degeneration (atrophy) of the nerves that carry information from the eyes to the brain. Many of the neurological symptoms of triple A syndrome worsen over time.People with triple A syndrome frequently develop a thickening of the outer layer of skin (hyperkeratosis) on the palms of their hands and the soles of their feet. Other skin abnormalities may also be present in people with this condition.Alacrima is usually the first noticeable sign of triple A syndrome, as it becomes apparent early in life that affected children produce little or no tears while crying. They develop Addison disease and achalasia during childhood or adolescence, and most of the neurologic features of triple A syndrome begin during adulthood. The signs and symptoms of this condition vary among affected individuals, even among members of the same family.
Neuropathy, hereditary sensory and autonomic, adult-onset, with anosmia
MedGen UID:
324809
Concept ID:
C1837492
Disease or Syndrome
Orthostatic hypotensive disorder, streeten type
MedGen UID:
327101
Concept ID:
C1840438
Disease or Syndrome
Polyglucosan body disease, adult
MedGen UID:
342338
Concept ID:
C1849722
Disease or Syndrome
Adult polyglucosan body disease (APBD) is characterized by adult-onset progressive neurogenic bladder, gait difficulties (i.e., spasticity and weakness) from mixed upper and lower motor neuron involvement, sensory loss predominantly in the distal lower extremities, and mild cognitive difficulties (often executive dysfunction).
Parkinson disease 4
MedGen UID:
381361
Concept ID:
C1854182
Disease or Syndrome
Parkinson disease is a progressive disorder of the nervous system. The disorder affects several regions of the brain, especially an area called the substantia nigra that controls balance and movement.Often the first symptom of Parkinson disease is trembling or shaking (tremor) of a limb, especially when the body is at rest. Typically, the tremor begins on one side of the body, usually in one hand. Tremors can also affect the arms, legs, feet, and face. Other characteristic symptoms of Parkinson disease include rigidity or stiffness of the limbs and torso, slow movement (bradykinesia) or an inability to move (akinesia), and impaired balance and coordination (postural instability). These symptoms worsen slowly over time.Parkinson disease can also affect emotions and thinking ability (cognition). Some affected individuals develop psychiatric conditions such as depression and visual hallucinations. People with Parkinson disease also have an increased risk of developing dementia, which is a decline in intellectual functions including judgment and memory.Generally, Parkinson disease that begins after age 50 is called late-onset disease. The condition is described as early-onset disease if signs and symptoms begin before age 50. Early-onset cases that begin before age 20 are sometimes referred to as juvenile-onset Parkinson disease.
Motor neuropathy peripheral with dysautonomia
MedGen UID:
381527
Concept ID:
C1854961
Disease or Syndrome
Charcot-Marie-Tooth disease with ptosis and parkinsonism
MedGen UID:
396191
Concept ID:
C1861668
Disease or Syndrome
Corticosterone methyloxidase type 2 deficiency
MedGen UID:
483046
Concept ID:
C3463917
Disease or Syndrome
CMO type II deficiency is an autosomal recessive disorder caused by a defect in the final biochemical step of aldosterone biosynthesis, the 18-hydroxylation of 18-hydroxycorticosterone (18-OHB) to aldosterone. This enzymatic defect results in decreased aldosterone and salt-wasting associated with an increased serum ratio of 18-OHB to aldosterone. In CMO II deficiency, aldosterone can be low or normal, but at the expense of increased secretion of 18-OHB. These patients have a low ratio of corticosterone to 18-OHB (Portrat-Doyen et al., 1998). The CYP11B2 gene product also catalyzes an earlier step in aldosterone biosynthesis: the 18-hydroxylation of corticosterone to 18-OHB. A defect in that enzymatic step results in CMO type I deficiency (204300), an allelic disorder with an overlapping phenotype but distinct biochemical features. In CMO I deficiency, aldosterone is undetectable, whereas its immediate precursor, 18-OHB, is low or normal (Portrat-Doyen et al., 1998).
Shy-Drager syndrome
MedGen UID:
811503
Concept ID:
C3714927
Finding
Multiple system atrophy (MSA) is a distinct clinicopathologic entity that manifests as a progressive adult-onset neurodegenerative disorder causing parkinsonism, cerebellar ataxia, and autonomic, urogenital, and pyramidal dysfunction in various combinations. Two main subtypes are recognized: 'subtype C,' characterized predominantly by cerebellar ataxia, and 'subtype P,' characterized predominantly by parkinsonism. MSA is characterized pathologically by the degeneration of striatonigral and olivopontocerebellar structures and glial cytoplasmic inclusions (GCIs) that consist of abnormally phosphorylated alpha-synuclein (SNCA; 163890) or tau (MAPT; 157140) (Gilman et al., 1998; Gilman et al., 2008; Scholz et al., 2009). 'Subtype C' of MSA has been reported to be more prevalent than 'subtype P' in the Japanese population (65-67% vs 33-35%), whereas 'subtype P' has been reported to be more prevalent than 'subtype C' in Europe (63% vs 34%) and North America (60% vs 13%, with 27% of cases unclassified) (summary by The Multiple-System Atrophy Research Collaboration, 2013). MSA is similar clinically and pathologically to Parkinson disease (PD; 168600) and Lewy body dementia (127750). See also PARK1 (168601), which is specifically caused by mutation in the SNCA gene. Pure autonomic failure manifests as orthostatic hypotension and other autonomic abnormalities without other neurologic involvement. Although there is some phenotypic overlap, the relationship of pure autonomic failure to MSA is unclear (Vanderhaeghen et al., 1970; Schatz, 1996).
Partial lipodystrophy, congenital cataracts, and neurodegeneration syndrome
MedGen UID:
813897
Concept ID:
C3807567
Disease or Syndrome
Lipodystrophies are rare disorders characterized by loss of body fat from various regions and predisposition to metabolic complications of insulin resistance and lipid abnormalities. FPLD7 is an autosomal dominant disorder with a highly variable phenotype. Additional features, including early-onset cataracts and later onset of spasticity of the lower limbs, have been noted in some patients (summary by Garg et al., 2015). For a general phenotypic description and a discussion of genetic heterogeneity of familial partial lipodystrophy (FPLD), see 151660.
Alacrima, achalasia, and mental retardation syndrome
MedGen UID:
816068
Concept ID:
C3809738
Disease or Syndrome
Alacrima, achalasia, and mental retardation syndrome (AAMR) is an autosomal recessive disorder characterized by onset of these 3 main features at birth or in early infancy. More variable features include hypotonia, gait abnormalities, anisocoria, and visual or hearing deficits. The disorder shows similarity to the triple A syndrome (231550), but patients with AAMR do not have adrenal insufficiency (summary by Koehler et al., 2013). See also 300858 for a phenotypically similar disorder that shows X-linked inheritance.
Parkinson disease 22, autosomal dominant
MedGen UID:
907886
Concept ID:
C4225238
Disease or Syndrome
ORTHOSTATIC HYPOTENSION 2
MedGen UID:
1648282
Concept ID:
C4748569
Disease or Syndrome
Orthostatic hypotension-2 is an autosomal recessive disorder characterized by severe orthostatic hypotension, recurrent hypoglycemia, and low norepinephrine levels. The disorder has onset in infancy or early childhood. Some patients may also have renal dysfunction and reduced life expectancy. The disorder results from a defect in the biosynthesis of norepinephrine from dopamine due to a cofactor deficiency. For a discussion of genetic heterogeneity of ORTHYP, see ORTHYP1 (223360).

Recent clinical studies

Etiology

Sforza M, Assogna F, Rinaldi D, Sette G, Tagliente S, Pontieri FE
Neurol Sci 2018 Aug;39(8):1459-1462. Epub 2018 Apr 7 doi: 10.1007/s10072-018-3394-2. PMID: 29627942
Freud T, Punchik B, Kagan E, Barzak A, Press Y
Geriatr Gerontol Int 2018 Jul;18(7):1009-1017. Epub 2018 Mar 2 doi: 10.1111/ggi.13291. PMID: 29498476
Bocti C, Pépin F, Tétreault M, Cossette P, Langlois F, Imbeault H, Duval N, Lacombe G, Fulop T
J Neurol Sci 2017 Nov 15;382:79-83. Epub 2017 Sep 27 doi: 10.1016/j.jns.2017.09.028. PMID: 29111025
Grijalva CG, Biaggioni I, Griffin MR, Shibao CA
J Am Heart Assoc 2017 Oct 12;6(10) doi: 10.1161/JAHA.117.006848. PMID: 29025750Free PMC Article
Torres RV, Elias MF, Crichton GE, Dore GA, Davey A
J Clin Hypertens (Greenwich) 2017 Dec;19(12):1357-1365. Epub 2017 Sep 19 doi: 10.1111/jch.13095. PMID: 28929576Free PMC Article

Diagnosis

van Twist DJL, Dinh T, Bouwmans EME, Kroon AA
Int J Cardiol 2018 Nov 15;271:269-273. doi: 10.1016/j.ijcard.2018.05.043. PMID: 30223355
Sforza M, Assogna F, Rinaldi D, Sette G, Tagliente S, Pontieri FE
Neurol Sci 2018 Aug;39(8):1459-1462. Epub 2018 Apr 7 doi: 10.1007/s10072-018-3394-2. PMID: 29627942
Freud T, Punchik B, Kagan E, Barzak A, Press Y
Geriatr Gerontol Int 2018 Jul;18(7):1009-1017. Epub 2018 Mar 2 doi: 10.1111/ggi.13291. PMID: 29498476
Bocti C, Pépin F, Tétreault M, Cossette P, Langlois F, Imbeault H, Duval N, Lacombe G, Fulop T
J Neurol Sci 2017 Nov 15;382:79-83. Epub 2017 Sep 27 doi: 10.1016/j.jns.2017.09.028. PMID: 29111025
Torres RV, Elias MF, Crichton GE, Dore GA, Davey A
J Clin Hypertens (Greenwich) 2017 Dec;19(12):1357-1365. Epub 2017 Sep 19 doi: 10.1111/jch.13095. PMID: 28929576Free PMC Article

Therapy

Foster-Dingley JC, Moonen JEF, de Ruijter W, van der Mast RC, van der Grond J
J Hypertens 2018 May;36(5):1201-1206. doi: 10.1097/HJH.0000000000001681. PMID: 29373479
Mar PL, Raj SR
Curr Opin Cardiol 2018 Jan;33(1):66-72. doi: 10.1097/HCO.0000000000000467. PMID: 28984649Free PMC Article
Grijalva CG, Biaggioni I, Griffin MR, Shibao CA
J Am Heart Assoc 2017 Oct 12;6(10) doi: 10.1161/JAHA.117.006848. PMID: 29025750Free PMC Article
Joseph A, Wanono R, Flamant M, Vidal-Petiot E
Nephrol Ther 2017 Apr;13 Suppl 1:S55-S67. doi: 10.1016/j.nephro.2017.01.003. PMID: 28577744
Kotagal V, Lineback C, Bohnen NI, Albin RL; CALM-PD Parkinson Study Group Investigators.
Parkinsonism Relat Disord 2016 Nov;32:127-129. Epub 2016 Sep 9 doi: 10.1016/j.parkreldis.2016.09.011. PMID: 27639815Free PMC Article

Prognosis

Sforza M, Assogna F, Rinaldi D, Sette G, Tagliente S, Pontieri FE
Neurol Sci 2018 Aug;39(8):1459-1462. Epub 2018 Apr 7 doi: 10.1007/s10072-018-3394-2. PMID: 29627942
Freud T, Punchik B, Kagan E, Barzak A, Press Y
Geriatr Gerontol Int 2018 Jul;18(7):1009-1017. Epub 2018 Mar 2 doi: 10.1111/ggi.13291. PMID: 29498476
Mar PL, Raj SR
Curr Opin Cardiol 2018 Jan;33(1):66-72. doi: 10.1097/HCO.0000000000000467. PMID: 28984649Free PMC Article
Grijalva CG, Biaggioni I, Griffin MR, Shibao CA
J Am Heart Assoc 2017 Oct 12;6(10) doi: 10.1161/JAHA.117.006848. PMID: 29025750Free PMC Article
Fedorowski A, Hamrefors V, Sutton R, van Dijk JG, Freeman R, Lenders JW, Wieling W
Clin Auton Res 2017 Jun;27(3):167-173. Epub 2017 Feb 27 doi: 10.1007/s10286-017-0409-7. PMID: 28243824Free PMC Article

Clinical prediction guides

Shanbhag A, Awai H, Rej S, Thomas AJ, Puka K, Vasudev A
Int J Geriatr Psychiatry 2018 Oct;33(10):1397-1402. Epub 2018 Jul 24 doi: 10.1002/gps.4951. PMID: 30043432
Bocti C, Pépin F, Tétreault M, Cossette P, Langlois F, Imbeault H, Duval N, Lacombe G, Fulop T
J Neurol Sci 2017 Nov 15;382:79-83. Epub 2017 Sep 27 doi: 10.1016/j.jns.2017.09.028. PMID: 29111025
Torres RV, Elias MF, Crichton GE, Dore GA, Davey A
J Clin Hypertens (Greenwich) 2017 Dec;19(12):1357-1365. Epub 2017 Sep 19 doi: 10.1111/jch.13095. PMID: 28929576Free PMC Article
Kotagal V, Lineback C, Bohnen NI, Albin RL; CALM-PD Parkinson Study Group Investigators.
Parkinsonism Relat Disord 2016 Nov;32:127-129. Epub 2016 Sep 9 doi: 10.1016/j.parkreldis.2016.09.011. PMID: 27639815Free PMC Article
Frith J, Newton JL
Geriatr Gerontol Int 2016 Jul;16(7):785-90. Epub 2015 Jul 14 doi: 10.1111/ggi.12553. PMID: 26179107

Recent systematic reviews

Peters R, Anstey KJ, Booth A, Beckett N, Warwick J, Antikainen R, Rockwood K, Peters J, Bulpitt CJ
Eur Heart J 2018 Sep 1;39(33):3135-3143. doi: 10.1093/eurheartj/ehy418. PMID: 30052878Free PMC Article
Chen JJ, Han Y, Tang J, Portillo I, Hauser RA, Dashtipour K
Ann Pharmacother 2018 Dec;52(12):1182-1194. Epub 2018 Jul 4 doi: 10.1177/1060028018786954. PMID: 29972032
Strassheim V, Newton JL, Tan MP, Frith J
J Hypertens 2016 Oct;34(10):1933-41. doi: 10.1097/HJH.0000000000001043. PMID: 27442791
Angelousi A, Girerd N, Benetos A, Frimat L, Gautier S, Weryha G, Boivin JM
J Hypertens 2014 Aug;32(8):1562-71; discussion 1571. doi: 10.1097/HJH.0000000000000235. PMID: 24879490
Smeenk HE, Koster MJ, Faaij RA, de Geer DB, Hamaker ME
Neth J Med 2014 Feb;72(2):80-5. PMID: 24659590

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