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Episodic ataxia type 8(EA8)

MedGen UID:
863545
Concept ID:
C4015108
Disease or Syndrome
Synonyms: EA8; EPISODIC ATAXIA, TYPE 8
SNOMED CT: Episodic ataxia with slurred speech (773495009); Episodic ataxia type 8 (773495009)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Monarch Initiative: MONDO:0014476
OMIM®: 616055
Orphanet: ORPHA401953

Definition

A rare hereditary ataxia characterized by recurrent episodes of ataxia with variable frequency and duration, associated with slurred speech, generalized muscle weakness and balance disturbance. Other symptoms may occur between episodes, including intention tremor, gait ataxia, mild dysarthria, myokymia, migraine and nystagmus. [from SNOMEDCT_US]

Clinical features

From HPO
Cerebellar ataxia
MedGen UID:
849
Concept ID:
C0007758
Disease or Syndrome
Cerebellar ataxia refers to ataxia due to dysfunction of the cerebellum. This causes a variety of elementary neurological deficits including asynergy (lack of coordination between muscles, limbs and joints), dysmetria (lack of ability to judge distances that can lead to under- or overshoot in grasping movements), and dysdiadochokinesia (inability to perform rapid movements requiring antagonizing muscle groups to be switched on and off repeatedly).
Dysarthria
MedGen UID:
8510
Concept ID:
C0013362
Mental or Behavioral Dysfunction
Dysarthric speech is a general description referring to a neurological speech disorder characterized by poor articulation. Depending on the involved neurological structures, dysarthria may be further classified as spastic, flaccid, ataxic, hyperkinetic and hypokinetic, or mixed.
Slurred speech
MedGen UID:
65885
Concept ID:
C0234518
Finding
Abnormal coordination of muscles involved in speech.
Myokymia
MedGen UID:
146882
Concept ID:
C0684219
Sign or Symptom
Myokymia consists of involuntary, fine, continuous, undulating contractions that spread across the affected striated muscle.
Hereditary episodic ataxia
MedGen UID:
314033
Concept ID:
C1720189
Disease or Syndrome
Periodic spells of incoordination and imbalance, that is, episodes of ataxia typically lasting from 10 minutes to several hours or days.
Intention tremor
MedGen UID:
1642960
Concept ID:
C4551520
Sign or Symptom
A type of kinetic tremor that occurs during target directed movement is called intention tremor. That is, an oscillatory cerebellar ataxia that tends to be absent when the limbs are inactive and during the first part of voluntary movement but worsening as the movement continues and greater precision is required (e.g., in touching a target such as the patient's nose or a physician's finger).
Muscle weakness
MedGen UID:
57735
Concept ID:
C0151786
Finding
Reduced strength of muscles.
Nystagmus
MedGen UID:
45166
Concept ID:
C0028738
Disease or Syndrome
Rhythmic, involuntary oscillations of one or both eyes related to abnormality in fixation, conjugate gaze, or vestibular mechanisms.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEpisodic ataxia type 8

Professional guidelines

PubMed

Garces P, Antoniades CA, Sobanska A, Kovacs N, Ying SH, Gupta AS, Perlman S, Szmulewicz DJ, Pane C, Németh AH, Jardim LB, Coarelli G, Dankova M, Traschütz A, Tarnutzer AA
Cerebellum 2024 Feb;23(1):121-135. Epub 2023 Jan 14 doi: 10.1007/s12311-023-01514-8. PMID: 36640220Free PMC Article
Zesiewicz TA, Wilmot G, Kuo SH, Perlman S, Greenstein PE, Ying SH, Ashizawa T, Subramony SH, Schmahmann JD, Figueroa KP, Mizusawa H, Schöls L, Shaw JD, Dubinsky RM, Armstrong MJ, Gronseth GS, Sullivan KL
Neurology 2018 Mar 6;90(10):464-471. Epub 2018 Feb 9 doi: 10.1212/WNL.0000000000005055. PMID: 29440566Free PMC Article

Recent clinical studies

Etiology

Hassan A
Tremor Other Hyperkinet Mov (N Y) 2023;13:9. Epub 2023 Mar 28 doi: 10.5334/tohm.747. PMID: 37008993Free PMC Article
Paulhus K, Ammerman L, Glasscock E
Int J Mol Sci 2020 Apr 17;21(8) doi: 10.3390/ijms21082802. PMID: 32316562Free PMC Article
Hu Y, Jiang H, Wang Q, Xie Z, Pan S
PLoS One 2013;8(2):e56362. Epub 2013 Feb 18 doi: 10.1371/journal.pone.0056362. PMID: 23441182Free PMC Article
Laasonen M, Kauppinen J, Leppämäki S, Tani P, Harno H, Hokkanen L, Wikgren J
Exp Brain Res 2012 Nov;223(1):19-32. Epub 2012 Sep 5 doi: 10.1007/s00221-012-3237-y. PMID: 22948736
Ophoff RA, Terwindt GM, Vergouwe MN, Frants RR, Ferrari MD
Headache 1997 Sep;37(8):479-85. doi: 10.1046/j.1526-4610.1997.3708479.x. PMID: 9329229

Diagnosis

Hassan A
Tremor Other Hyperkinet Mov (N Y) 2023;13:9. Epub 2023 Mar 28 doi: 10.5334/tohm.747. PMID: 37008993Free PMC Article
Nielsen EN, Ásbjörnsdóttir B, Møller LB, Nielsen JE, Lindquist SG
Cold Spring Harb Mol Case Stud 2022 Oct;8(6) Epub 2022 Oct 28 doi: 10.1101/mcs.a006236. PMID: 36307210Free PMC Article
Choi JH, Oh EH, Choi SY, Kim HJ, Lee SK, Choi JY, Kim JS, Choi KD
J Neurol 2022 May;269(5):2687-2695. Epub 2021 Oct 28 doi: 10.1007/s00415-021-10856-4. PMID: 34709445
D'Adamo MC, Liantonio A, Rolland JF, Pessia M, Imbrici P
Int J Mol Sci 2020 Apr 22;21(8) doi: 10.3390/ijms21082935. PMID: 32331416Free PMC Article
Paulhus K, Ammerman L, Glasscock E
Int J Mol Sci 2020 Apr 17;21(8) doi: 10.3390/ijms21082802. PMID: 32316562Free PMC Article

Therapy

Garces P, Antoniades CA, Sobanska A, Kovacs N, Ying SH, Gupta AS, Perlman S, Szmulewicz DJ, Pane C, Németh AH, Jardim LB, Coarelli G, Dankova M, Traschütz A, Tarnutzer AA
Cerebellum 2024 Feb;23(1):121-135. Epub 2023 Jan 14 doi: 10.1007/s12311-023-01514-8. PMID: 36640220Free PMC Article
Dogra D, Meza-Santoscoy PL, Gavrilovici C, Rehak R, de la Hoz CLR, Ibhazehiebo K, Rho JM, Kurrasch DM
Epilepsia 2023 Aug;64(8):2186-2199. Epub 2023 Jun 4 doi: 10.1111/epi.17659. PMID: 37209379
Hassan A
Tremor Other Hyperkinet Mov (N Y) 2023;13:9. Epub 2023 Mar 28 doi: 10.5334/tohm.747. PMID: 37008993Free PMC Article
González-Mingot C, López-Ortega R, Brieva-Ruiz L
Neurol Sci 2022 Aug;43(8):5099-5101. Epub 2022 May 20 doi: 10.1007/s10072-022-06144-2. PMID: 35595874
Löhle M, Schrempf W, Wolz M, Reichmann H, Storch A
Mov Disord 2008 Jul 15;23(9):1314-6. doi: 10.1002/mds.22071. PMID: 18442126

Prognosis

González-Mingot C, López-Ortega R, Brieva-Ruiz L
Neurol Sci 2022 Aug;43(8):5099-5101. Epub 2022 May 20 doi: 10.1007/s10072-022-06144-2. PMID: 35595874
Ovsepian SV, LeBerre M, Steuber V, O'Leary VB, Leibold C, Oliver Dolly J
Pharmacol Ther 2016 Mar;159:93-101. Epub 2016 Jan 26 doi: 10.1016/j.pharmthera.2016.01.005. PMID: 26825872

Clinical prediction guides

González-Mingot C, López-Ortega R, Brieva-Ruiz L
Neurol Sci 2022 Aug;43(8):5099-5101. Epub 2022 May 20 doi: 10.1007/s10072-022-06144-2. PMID: 35595874
Coin JT, Vance JM
Mov Disord 2021 May;36(5):1264-1267. Epub 2021 Jan 16 doi: 10.1002/mds.28491. PMID: 33452831
Yuan H, Yuan H, Wang Q, Ye W, Yao R, Xu W, Liu Y
Mol Genet Genomic Med 2020 Oct;8(10):e1434. Epub 2020 Jul 23 doi: 10.1002/mgg3.1434. PMID: 32705822Free PMC Article
Ovsepian SV, LeBerre M, Steuber V, O'Leary VB, Leibold C, Oliver Dolly J
Pharmacol Ther 2016 Mar;159:93-101. Epub 2016 Jan 26 doi: 10.1016/j.pharmthera.2016.01.005. PMID: 26825872
Saito H, Okada M, Miki T, Wakamori M, Futatsugi A, Mori Y, Mikoshiba K, Suzuki N
Biochem Biophys Res Commun 2009 Dec 18;390(3):1029-33. Epub 2009 Oct 23 doi: 10.1016/j.bbrc.2009.10.102. PMID: 19854154

Recent systematic reviews

Hassan A
Tremor Other Hyperkinet Mov (N Y) 2023;13:9. Epub 2023 Mar 28 doi: 10.5334/tohm.747. PMID: 37008993Free PMC Article
Zesiewicz TA, Wilmot G, Kuo SH, Perlman S, Greenstein PE, Ying SH, Ashizawa T, Subramony SH, Schmahmann JD, Figueroa KP, Mizusawa H, Schöls L, Shaw JD, Dubinsky RM, Armstrong MJ, Gronseth GS, Sullivan KL
Neurology 2018 Mar 6;90(10):464-471. Epub 2018 Feb 9 doi: 10.1212/WNL.0000000000005055. PMID: 29440566Free PMC Article

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