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Cerebellar ataxia associated with quadrupedal gait

MedGen UID:
869746
Concept ID:
C4024175
Finding
HPO: HP:0009878

Definition

The presence of cerebellar signs and symptoms such as lack of balance associated with quadrupedal gait (locomotion on all four extremities with a 'bear-like' gait with the legs held straight). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCerebellar ataxia associated with quadrupedal gait

Conditions with this feature

Cerebellar ataxia, intellectual disability, and dysequilibrium syndrome 3
MedGen UID:
442496
Concept ID:
C2750509
Disease or Syndrome
Cerebellar ataxia, impaired intellectual development, and dysequilibrium syndrome (CAMRQ) is a genetically heterogeneous disorder characterized by congenital cerebellar ataxia and mental retardation (summary by Gulsuner et al., 2011). For a discussion of genetic heterogeneity of CAMRQ, see CAMRQ1 (224050).
Cerebellar ataxia, intellectual disability, and dysequilibrium syndrome 4
MedGen UID:
815307
Concept ID:
C3808977
Disease or Syndrome
Cerebellar ataxia, impaired intellectual development, and dysequilibrium syndrome (CAMRQ) is a genetically heterogeneous disorder characterized by congenital cerebellar ataxia and impaired intellectual development (summary by Gulsuner et al., 2011). For a discussion of genetic heterogeneity of CAMRQ, see CAMRQ1 (224050).
Cerebellar ataxia, intellectual disability, and dysequilibrium syndrome 1
MedGen UID:
1639436
Concept ID:
C4551552
Disease or Syndrome
VLDLR cerebellar hypoplasia (VLDLR-CH) is characterized by non-progressive congenital ataxia that is predominantly truncal and results in delayed ambulation, moderate-to-profound intellectual disability, dysarthria, strabismus, and seizures. Children either learn to walk very late (often after age 6 years) or never achieve independent ambulation. Brain MRI findings include hypoplasia of the inferior portion of the cerebellar vermis and hemispheres, simplified gyration of the cerebral hemispheres, and small brain stem – particularly the pons.

Recent clinical studies

Etiology

Kolb LE, Arlier Z, Yalcinkaya C, Ozturk AK, Moliterno JA, Erturk O, Bayrakli F, Korkmaz B, DiLuna ML, Yasuno K, Bilguvar K, Ozcelik T, Tuysuz B, State MW, Gunel M
Neurogenetics 2010 Jul;11(3):319-25. Epub 2010 Jan 15 doi: 10.1007/s10048-009-0232-y. PMID: 20082205

Diagnosis

Kaya N, Aldhalaan H, Al-Younes B, Colak D, Shuaib T, Al-Mohaileb F, Al-Sugair A, Nester M, Al-Yamani S, Al-Bakheet A, Al-Hashmi N, Al-Sayed M, Meyer B, Jungbluth H, Al-Owain M
Am J Med Genet B Neuropsychiatr Genet 2011 Dec;156B(7):826-34. Epub 2011 Aug 2 doi: 10.1002/ajmg.b.31227. PMID: 21812104
Kolb LE, Arlier Z, Yalcinkaya C, Ozturk AK, Moliterno JA, Erturk O, Bayrakli F, Korkmaz B, DiLuna ML, Yasuno K, Bilguvar K, Ozcelik T, Tuysuz B, State MW, Gunel M
Neurogenetics 2010 Jul;11(3):319-25. Epub 2010 Jan 15 doi: 10.1007/s10048-009-0232-y. PMID: 20082205

Prognosis

Richmond CM, Leventer R, Ryan MM, Delatycki MB
Clin Genet 2020 Mar;97(3):516-520. Epub 2019 Nov 14 doi: 10.1111/cge.13666. PMID: 31693170
Onat OE, Gulsuner S, Bilguvar K, Nazli Basak A, Topaloglu H, Tan M, Tan U, Gunel M, Ozcelik T
Eur J Hum Genet 2013 Mar;21(3):281-5. Epub 2012 Aug 15 doi: 10.1038/ejhg.2012.170. PMID: 22892528Free PMC Article

Clinical prediction guides

Paternoster L, Soblet J, Aeby A, De Tiège X, Goldman S, Yue WW, Coppens S, Smits G, Vilain C, Deconinck N
Am J Med Genet A 2020 Nov;182(11):2685-2693. Epub 2020 Aug 18 doi: 10.1002/ajmg.a.61805. PMID: 32808436
Richmond CM, Leventer R, Ryan MM, Delatycki MB
Clin Genet 2020 Mar;97(3):516-520. Epub 2019 Nov 14 doi: 10.1111/cge.13666. PMID: 31693170
Onat OE, Gulsuner S, Bilguvar K, Nazli Basak A, Topaloglu H, Tan M, Tan U, Gunel M, Ozcelik T
Eur J Hum Genet 2013 Mar;21(3):281-5. Epub 2012 Aug 15 doi: 10.1038/ejhg.2012.170. PMID: 22892528Free PMC Article
Kaya N, Aldhalaan H, Al-Younes B, Colak D, Shuaib T, Al-Mohaileb F, Al-Sugair A, Nester M, Al-Yamani S, Al-Bakheet A, Al-Hashmi N, Al-Sayed M, Meyer B, Jungbluth H, Al-Owain M
Am J Med Genet B Neuropsychiatr Genet 2011 Dec;156B(7):826-34. Epub 2011 Aug 2 doi: 10.1002/ajmg.b.31227. PMID: 21812104

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