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Poorly formed metencephalon

MedGen UID:
870504
Concept ID:
C4024951
Anatomical Abnormality
HPO: HP:0007027

Definition

A morphological abnormality of the metencephalon. [from HPO]

Term Hierarchy

Conditions with this feature

Aprosencephaly cerebellar dysgenesis
MedGen UID:
330459
Concept ID:
C1832412
Disease or Syndrome
A rare genetic non-syndromic central nervous system malformation with characteristics of absence of the telencephalon and absent or abnormal diencephalic structures, combined with severe abnormalities of the mesencephalon and cerebellum. Further malformations, for example of the hands and feet, have been described in addition.

Recent clinical studies

Etiology

de León GA, Grant JA, Darling CF
J Neurosurg 1996 Jul;85(1):157-62. doi: 10.3171/jns.1996.85.1.0157. PMID: 8683267
Ferrer I, Ribalta T, Fabregues I, Pineda M, Cusi V
Clin Neuropathol 1986 Mar-Apr;5(2):53-9. PMID: 2423283

Diagnosis

Caksen H, Tuncer O, Kirimi E, Fryns JP, Uner A, Unal O, Cinal A, Odabaş D
Genet Couns 2004;15(1):9-17. PMID: 15083694
de León GA, Grant JA, Darling CF
J Neurosurg 1996 Jul;85(1):157-62. doi: 10.3171/jns.1996.85.1.0157. PMID: 8683267

Therapy

Chung C, Elrick MJ, Dell'Orco JM, Qin ZS, Kalyana-Sundaram S, Chinnaiyan AM, Shakkottai VG, Lieberman AP
PLoS Genet 2016 May;12(5):e1006042. Epub 2016 May 6 doi: 10.1371/journal.pgen.1006042. PMID: 27152617Free PMC Article

Prognosis

Butcher PA, Ivry RB, Kuo SH, Rydz D, Krakauer JW, Taylor JA
J Neurophysiol 2017 Sep 1;118(3):1622-1636. Epub 2017 Jun 21 doi: 10.1152/jn.00451.2017. PMID: 28637818Free PMC Article
de León GA, Grant JA, Darling CF
J Neurosurg 1996 Jul;85(1):157-62. doi: 10.3171/jns.1996.85.1.0157. PMID: 8683267

Clinical prediction guides

Butcher PA, Ivry RB, Kuo SH, Rydz D, Krakauer JW, Taylor JA
J Neurophysiol 2017 Sep 1;118(3):1622-1636. Epub 2017 Jun 21 doi: 10.1152/jn.00451.2017. PMID: 28637818Free PMC Article
Jain M, Armstrong RJ, Tyers P, Barker RA, Rosser AE
Exp Neurol 2003 Jul;182(1):113-23. doi: 10.1016/s0014-4886(03)00055-4. PMID: 12821381

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