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Upbeat nystagmus

MedGen UID:
107943
•Concept ID:
C0585545
•
Finding; Finding
Synonyms: Upbeat central nystagmus; Upbeat central vestibular nystagmus
SNOMED CT: Upbeat nystagmus (307677002); Upbeat central nystagmus (307677002); Upbeat central vestibular nystagmus (307677002)
 
HPO: HP:0011477

Definition

In primary position, the eyes drift slowly downward and then spontaneously beat upward. Upward gaze accentuates the nystagmus. The associated oscillopsias are often very irritating, but the symptoms are usually transient. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUpbeat nystagmus

Conditions with this feature

Spinocerebellar ataxia type 27
MedGen UID:
373075
•Concept ID:
C1836383
•
Disease or Syndrome
Disease with characteristics of early-onset tremor, dyskinesia and slowly progressive cerebellar ataxia. Fewer than 30 cases have been reported to date. This disease is caused by a mutation in the fibroblast growth factor 14 FGF14 gene (13q34). Prognosis is relatively good. Life-threatening status epilepticus and intractable seizure or severe dysphagia is rare.
See: Condition Record
Spinocerebellar ataxia type 27

Professional guidelines

PubMed

The pharmacological treatment of acquired nystagmus.
Mehta AR, Kennard C
Pract Neurol 2012 Jun;12(3):147-53. doi: 10.1136/practneurol-2011-000181. PMID: 22661344
Nystagmus and oscillopsia.
Straube A, Bronstein A, Straumann D; European Federation of Neurologic Societies
Eur J Neurol 2012 Jan;19(1):6-14. Epub 2011 Sep 12 doi: 10.1111/j.1468-1331.2011.03503.x. PMID: 21906211
Medical treatment of vestibular disorders.
Brandt T, Zwergal A, Strupp M
Expert Opin Pharmacother 2009 Jul;10(10):1537-48. doi: 10.1517/14656560902976879. PMID: 19527184

Recent clinical studies

Etiology

Pharmacotherapy of vestibular disorders and nystagmus.
Strupp M, Kremmyda O, Brandt T
Semin Neurol 2013 Jul;33(3):286-96. Epub 2013 Sep 21 doi: 10.1055/s-0033-1354594. PMID: 24057832
Pharmacotherapy of vestibular and ocular motor disorders, including nystagmus.
Strupp M, Thurtell MJ, Shaikh AG, Brandt T, Zee DS, Leigh RJ
J Neurol 2011 Jul;258(7):1207-22. Epub 2011 Apr 2 doi: 10.1007/s00415-011-5999-8. PMID: 21461686Free PMC Article
Effect of gravity on vertical eye position.
Pierrot-Deseilligny C
Ann N Y Acad Sci 2009 May;1164:155-65. doi: 10.1111/j.1749-6632.2009.03864.x. PMID: 19645894
The Chiari I malformation and the neurotologist.
Kumar A, Patni AH, Charbel F
Otol Neurotol 2002 Sep;23(5):727-35. doi: 10.1097/00129492-200209000-00021. PMID: 12218627
Nystagmus after medulloblastoma.
Elliott AJ, Simpson EM, Oakhill A, Decock R
Dev Med Child Neurol 1989 Feb;31(1):43-6. doi: 10.1111/j.1469-8749.1989.tb08410.x. PMID: 2920872

Diagnosis

Generalized vestibular hyporeflexia and chronic upbeat nystagmus due to thiamine deficiency.
Zhu W, Steenerson KK, Kattah JC
J Neurol 2023 Mar;270(3):1713-1720. Epub 2022 Dec 15 doi: 10.1007/s00415-022-11514-z. PMID: 36520241
Nystagmus: Diagnosis, Topographic Anatomical Localization and Therapy.
Strupp ML, Straumann D, Helmchen C
Klin Monbl Augenheilkd 2021 Nov;238(11):1186-1195. Epub 2021 Nov 16 doi: 10.1055/a-1525-0030. PMID: 34784642
Central oculomotor disturbances and nystagmus: a window into the brainstem and cerebellum.
Strupp M, Hüfner K, Sandmann R, Zwergal A, Dieterich M, Jahn K, Brandt T
Dtsch Arztebl Int 2011 Mar;108(12):197-204. Epub 2011 Mar 25 doi: 10.3238/arztebl.2011.0197. PMID: 21505601Free PMC Article
Vestibular falls.
Brandt T, Dieterich M
J Vestib Res 1993 Spring;3(1):3-14. PMID: 8275242
Otoneurology.
Brandt T, Strupp M
Curr Opin Neurol Neurosurg 1992 Oct;5(5):727-32. PMID: 1356523

Therapy

Generalized vestibular hyporeflexia and chronic upbeat nystagmus due to thiamine deficiency.
Zhu W, Steenerson KK, Kattah JC
J Neurol 2023 Mar;270(3):1713-1720. Epub 2022 Dec 15 doi: 10.1007/s00415-022-11514-z. PMID: 36520241
Update on the pharmacotherapy of cerebellar and central vestibular disorders.
Kalla R, Teufel J, Feil K, Muth C, Strupp M
J Neurol 2016 Apr;263 Suppl 1:S24-9. Epub 2016 Apr 15 doi: 10.1007/s00415-015-7987-x. PMID: 27083881Free PMC Article
The pharmacological treatment of acquired nystagmus.
Mehta AR, Kennard C
Pract Neurol 2012 Jun;12(3):147-53. doi: 10.1136/practneurol-2011-000181. PMID: 22661344
Medical treatment of vestibular disorders.
Brandt T, Zwergal A, Strupp M
Expert Opin Pharmacother 2009 Jul;10(10):1537-48. doi: 10.1517/14656560902976879. PMID: 19527184
EFNS task force--therapy of nystagmus and oscillopsia.
Straube A, Leigh RJ, Bronstein A, Heide W, Riordan-Eva P, Tijssen CC, Dehaene I, Straumann D
Eur J Neurol 2004 Feb;11(2):83-9. doi: 10.1046/j.1468-1331.2003.00754.x. PMID: 14748767

Prognosis

Central ocular motor disorders, including gaze palsy and nystagmus.
Strupp M, Kremmyda O, Adamczyk C, Böttcher N, Muth C, Yip CW, Bremova T
J Neurol 2014 Sep;261 Suppl 2(Suppl 2):S542-58. doi: 10.1007/s00415-014-7385-9. PMID: 25145891Free PMC Article
Medical treatment of vestibular disorders.
Brandt T, Zwergal A, Strupp M
Expert Opin Pharmacother 2009 Jul;10(10):1537-48. doi: 10.1517/14656560902976879. PMID: 19527184
Upbeat and downbeat nystagmus occurring successively in a patient with posterior medullary haemorrhage.
Rousseaux M, Dupard T, Lesoin F, Barbaste P, Hache JC
J Neurol Neurosurg Psychiatry 1991 Apr;54(4):367-9. doi: 10.1136/jnnp.54.4.367. PMID: 2056325Free PMC Article
Nystagmus after medulloblastoma.
Elliott AJ, Simpson EM, Oakhill A, Decock R
Dev Med Child Neurol 1989 Feb;31(1):43-6. doi: 10.1111/j.1469-8749.1989.tb08410.x. PMID: 2920872
Upbeat nystagmus and internuclear ophthalmoplegia with brainstem glioma.
Troost BT, Martinez J, Abel LA, Heros RC
Arch Neurol 1980 Jul;37(7):453-6. doi: 10.1001/archneur.1980.00500560083015. PMID: 7387494

Clinical prediction guides

Pearls & Oy-sters: Vibration-Induced Downbeat Nystagmus: A New Cerebellar Sign Observed in Paraneoplastic Syndrome.
Kim HJ, Kim HJ, Lee JH, Kim JS
Neurology 2023 Jan 3;100(1):43-46. Epub 2022 Oct 3 doi: 10.1212/WNL.0000000000201466. PMID: 36192172
Analysis of Dix-Hallpike maneuver induced nystagmus based on virtual simulation.
Zheng Y, Wu S, Yang X
Acta Otolaryngol 2021 May;141(5):433-439. Epub 2021 Feb 9 doi: 10.1080/00016489.2021.1876247. PMID: 33557660
Functional positional eye and eyelid movements.
Mustafa MM, Akram H, Oliva-Domínguez M, Kaski D
J Neurol 2020 Nov;267(11):3425-3428. Epub 2020 Jul 15 doi: 10.1007/s00415-020-10071-7. PMID: 32671528Free PMC Article
Effect of gravity on vertical eye position.
Pierrot-Deseilligny C
Ann N Y Acad Sci 2009 May;1164:155-65. doi: 10.1111/j.1749-6632.2009.03864.x. PMID: 19645894
Vestibular falls.
Brandt T, Dieterich M
J Vestib Res 1993 Spring;3(1):3-14. PMID: 8275242

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