U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Episodic ataxia type 6(EA6)

MedGen UID:
390739
Concept ID:
C2675211
Disease or Syndrome
Synonym: Episodic Ataxia, Type 6
SNOMED CT: Episodic ataxia type 6 (718753002)
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.
 
Gene (location): SLC1A3 (5p13.2)
 
Monarch Initiative: MONDO:0012982
OMIM®: 612656
Orphanet: ORPHA209967

Definition

An exceedingly rare form of hereditary episodic ataxia with varying degrees of ataxia and associated findings including slurred speech, headache, confusion and hemiplegia. [from SNOMEDCT_US]

Clinical features

From HPO
Nausea
MedGen UID:
10196
Concept ID:
C0027497
Sign or Symptom
A sensation of unease in the stomach together with an urge to vomit.
Vomiting
MedGen UID:
12124
Concept ID:
C0042963
Sign or Symptom
Forceful ejection of the contents of the stomach through the mouth by means of a series of involuntary spasmic contractions.
Vertigo
MedGen UID:
53006
Concept ID:
C0042571
Sign or Symptom
An abnormal sensation of spinning while the body is actually stationary.
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.
Hemiparesis
MedGen UID:
6783
Concept ID:
C0018989
Finding
Loss of strength in the arm, leg, and sometimes face on one side of the body. Hemiplegia refers to a complete loss of strength, whereas hemiparesis refers to an incomplete loss of strength.
Hemiplegia
MedGen UID:
9196
Concept ID:
C0018991
Sign or Symptom
Paralysis (complete loss of muscle function) in the arm, leg, and in some cases the face on one side of the body.
Seizure
MedGen UID:
20693
Concept ID:
C0036572
Sign or Symptom
A seizure is an intermittent abnormality of nervous system physiology characterized by a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.
Photophobia
MedGen UID:
43220
Concept ID:
C0085636
Sign or Symptom
Excessive sensitivity to light with the sensation of discomfort or pain in the eyes due to exposure to bright light.
Migraine
MedGen UID:
57451
Concept ID:
C0149931
Disease or Syndrome
Migraine is a chronic neurological disorder characterized by episodic attacks of headache and associated symptoms.
Slurred speech
MedGen UID:
65885
Concept ID:
C0234518
Finding
Abnormal coordination of muscles involved in speech.
Cerebellar hypoplasia
MedGen UID:
120578
Concept ID:
C0266470
Congenital Abnormality
Cerebellar hypoplasia is a descriptive term implying a cerebellum with a reduced volume, but a normal shape and is stable over time.
Truncal ataxia
MedGen UID:
96535
Concept ID:
C0427190
Sign or Symptom
Truncal ataxia is a sign of ataxia characterized by instability of the trunk. It usually occurs during sitting.
Cerebellar atrophy
MedGen UID:
196624
Concept ID:
C0740279
Disease or Syndrome
Cerebellar atrophy is defined as a cerebellum with initially normal structures, in a posterior fossa with normal size, which displays enlarged fissures (interfolial spaces) in comparison to the foliae secondary to loss of tissue. Cerebellar atrophy implies irreversible loss of tissue and result from an ongoing progressive disease until a final stage is reached or a single injury, e.g. an intoxication or infectious event.
Phonophobia
MedGen UID:
155864
Concept ID:
C0751466
Mental or Behavioral Dysfunction
An abnormally heightened sensitivity to loud sounds.
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.
Motor delay
MedGen UID:
381392
Concept ID:
C1854301
Finding
A type of Developmental delay characterized by a delay in acquiring motor skills.
Episodic generalized hypotonia
MedGen UID:
870528
Concept ID:
C4024976
Anatomical Abnormality
The occurrence of repeated episodes of generalized muscular hypotonia.
Diplopia
MedGen UID:
41600
Concept ID:
C0012569
Disease or Syndrome
Diplopia is a condition in which a single object is perceived as two images, it is also known as double vision.
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.
Hypometric saccades
MedGen UID:
98065
Concept ID:
C0423082
Finding
Saccadic undershoot, i.e., a saccadic eye movement that has less than the magnitude that would be required to gain fixation of the object.

Recent clinical studies

Etiology

Chivukula AS, Suslova M, Kortzak D, Kovermann P, Fahlke C
Hum Mutat 2020 Nov;41(11):1892-1905. Epub 2020 Sep 9 doi: 10.1002/humu.24089. PMID: 32741053

Diagnosis

Chivukula AS, Suslova M, Kortzak D, Kovermann P, Fahlke C
Hum Mutat 2020 Nov;41(11):1892-1905. Epub 2020 Sep 9 doi: 10.1002/humu.24089. PMID: 32741053
Choi KD, Jen JC, Choi SY, Shin JH, Kim HS, Kim HJ, Kim JS, Choi JH
J Hum Genet 2017 Mar;62(3):443-446. Epub 2016 Nov 10 doi: 10.1038/jhg.2016.137. PMID: 27829685
Gosalakkal JA, Swamy PM
Pediatr Neurol 2006 Apr;34(4):301-2. doi: 10.1016/j.pediatrneurol.2005.08.022. PMID: 16638506

Therapy

Choi KD, Jen JC, Choi SY, Shin JH, Kim HS, Kim HJ, Kim JS, Choi JH
J Hum Genet 2017 Mar;62(3):443-446. Epub 2016 Nov 10 doi: 10.1038/jhg.2016.137. PMID: 27829685
Gosalakkal JA, Swamy PM
Pediatr Neurol 2006 Apr;34(4):301-2. doi: 10.1016/j.pediatrneurol.2005.08.022. PMID: 16638506

Prognosis

Choi KD, Jen JC, Choi SY, Shin JH, Kim HS, Kim HJ, Kim JS, Choi JH
J Hum Genet 2017 Mar;62(3):443-446. Epub 2016 Nov 10 doi: 10.1038/jhg.2016.137. PMID: 27829685
Hotzy J, Schneider N, Kovermann P, Fahlke C
J Biol Chem 2013 Dec 20;288(51):36492-501. Epub 2013 Nov 8 doi: 10.1074/jbc.M113.489385. PMID: 24214974Free PMC Article

Clinical prediction guides

Chivukula AS, Suslova M, Kortzak D, Kovermann P, Fahlke C
Hum Mutat 2020 Nov;41(11):1892-1905. Epub 2020 Sep 9 doi: 10.1002/humu.24089. PMID: 32741053
Choi KD, Jen JC, Choi SY, Shin JH, Kim HS, Kim HJ, Kim JS, Choi JH
J Hum Genet 2017 Mar;62(3):443-446. Epub 2016 Nov 10 doi: 10.1038/jhg.2016.137. PMID: 27829685
Hotzy J, Schneider N, Kovermann P, Fahlke C
J Biol Chem 2013 Dec 20;288(51):36492-501. Epub 2013 Nov 8 doi: 10.1074/jbc.M113.489385. PMID: 24214974Free PMC Article

Supplemental Content

Recent activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...