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Intellectual disability, X-linked 101(XLID101)

MedGen UID:
855517
Concept ID:
C3890168
Disease or Syndrome
Synonym: XLID101
 
Gene (location): MID2 (Xq22.3)
 
Monarch Initiative: MONDO:0010489
OMIM®: 300928

Definition

Any non-syndromic X-linked intellectual disability in which the cause of the disease is a mutation in the MID2 gene. [from MONDO]

Clinical features

From HPO
Clinodactyly
MedGen UID:
1644094
Concept ID:
C4551485
Congenital Abnormality
An angulation of a digit at an interphalangeal joint in the plane of the palm (finger) or sole (toe).
Macrotia
MedGen UID:
488785
Concept ID:
C0152421
Congenital Abnormality
Median longitudinal ear length greater than two standard deviations above the mean and median ear width greater than two standard deviations above the mean (objective); or, apparent increase in length and width of the pinna (subjective).
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.
Hyperactivity
MedGen UID:
98406
Concept ID:
C0424295
Finding
Hyperactivity is a condition characterized by constant and unusually high levels of activity, even in situations where it is deemed inappropriate.
Global developmental delay
MedGen UID:
107838
Concept ID:
C0557874
Finding
A delay in the achievement of motor or mental milestones in the domains of development of a child, including motor skills, speech and language, cognitive skills, and social and emotional skills. This term should only be used to describe children younger than five years of age.
Poor speech
MedGen UID:
341172
Concept ID:
C1848207
Finding
Intellectual disability
MedGen UID:
811461
Concept ID:
C3714756
Mental or Behavioral Dysfunction
Intellectual disability, previously referred to as mental retardation, is characterized by subnormal intellectual functioning that occurs during the developmental period. It is defined by an IQ score below 70.
Long face
MedGen UID:
324419
Concept ID:
C1836047
Finding
Facial height (length) is more than 2 standard deviations above the mean (objective); or, an apparent increase in the height (length) of the face (subjective).
Short philtrum
MedGen UID:
350006
Concept ID:
C1861324
Finding
Distance between nasal base and midline upper lip vermilion border more than 2 SD below the mean. Alternatively, an apparently decreased distance between nasal base and midline upper lip vermilion border.
Optic atrophy
MedGen UID:
18180
Concept ID:
C0029124
Disease or Syndrome
Atrophy of the optic nerve. Optic atrophy results from the death of the retinal ganglion cell axons that comprise the optic nerve and manifesting as a pale optic nerve on fundoscopy.
Strabismus
MedGen UID:
21337
Concept ID:
C0038379
Disease or Syndrome
A misalignment of the eyes so that the visual axes deviate from bifoveal fixation. The classification of strabismus may be based on a number of features including the relative position of the eyes, whether the deviation is latent or manifest, intermittent or constant, concomitant or otherwise and according to the age of onset and the relevance of any associated refractive error.
Unilateral ptosis
MedGen UID:
401085
Concept ID:
C1866806
Finding
A unilateral form of ptosis.

Professional guidelines

PubMed

Agrawal N, Verma G, Saxena D, Kabra M, Gupta N, Mandal K, Moirangthem A, Sheth J, Puri RD, Bijarnia-Mahay S, Kapoor S, Danda S, H SV, Datar CA, Ranganath P, Shukla A, Dalal A, Srivastava P, Devi RR, Phadke SR
Eur J Med Genet 2022 Mar;65(3):104447. Epub 2022 Feb 8 doi: 10.1016/j.ejmg.2022.104447. PMID: 35144014
Hong X, Kumar AB, Ronald Scott C, Gelb MH
Mol Genet Metab 2018 Jun;124(2):101-108. Epub 2018 Mar 29 doi: 10.1016/j.ymgme.2018.03.012. PMID: 29680633Free PMC Article
van de Kamp JM, Betsalel OT, Mercimek-Mahmutoglu S, Abulhoul L, Grünewald S, Anselm I, Azzouz H, Bratkovic D, de Brouwer A, Hamel B, Kleefstra T, Yntema H, Campistol J, Vilaseca MA, Cheillan D, D'Hooghe M, Diogo L, Garcia P, Valongo C, Fonseca M, Frints S, Wilcken B, von der Haar S, Meijers-Heijboer HE, Hofstede F, Johnson D, Kant SG, Lion-Francois L, Pitelet G, Longo N, Maat-Kievit JA, Monteiro JP, Munnich A, Muntau AC, Nassogne MC, Osaka H, Ounap K, Pinard JM, Quijano-Roy S, Poggenburg I, Poplawski N, Abdul-Rahman O, Ribes A, Arias A, Yaplito-Lee J, Schulze A, Schwartz CE, Schwenger S, Soares G, Sznajer Y, Valayannopoulos V, Van Esch H, Waltz S, Wamelink MM, Pouwels PJ, Errami A, van der Knaap MS, Jakobs C, Mancini GM, Salomons GS
J Med Genet 2013 Jul;50(7):463-72. Epub 2013 May 3 doi: 10.1136/jmedgenet-2013-101658. PMID: 23644449

Recent clinical studies

Etiology

Sandal S, Verma IC, Mahay SB, Dubey S, Sabharwal RK, Kulshrestha S, Saxena R, Suman P, Kumar P, Puri RD
Indian J Pediatr 2024 Jul;91(7):682-695. Epub 2023 Oct 7 doi: 10.1007/s12098-023-04820-5. PMID: 37804371
Muenzer J, Burton BK, Amartino HM, Harmatz PR, Gutiérrez-Solana LG, Ruiz-Garcia M, Wu Y, Merberg D, Alexanderian D, Jones SA
Orphanet J Rare Dis 2023 Nov 16;18(1):357. doi: 10.1186/s13023-023-02805-3. PMID: 37974184Free PMC Article
Di Pierro D, Ciaccio C, Sbardella D, Tundo GR, Bernardini R, Curatolo P, Galasso C, Pironi V, Coletta M, Marini S
Mol Cell Biochem 2020 Jan;463(1-2):101-113. Epub 2019 Oct 8 doi: 10.1007/s11010-019-03633-5. PMID: 31595423
Alexander-Bloch AF, McDougle CJ, Ullman Z, Sweetser DA
Psychiatr Genet 2016 Jun;26(3):101-8. doi: 10.1097/YPG.0000000000000128. PMID: 27010919Free PMC Article
van de Kamp JM, Betsalel OT, Mercimek-Mahmutoglu S, Abulhoul L, Grünewald S, Anselm I, Azzouz H, Bratkovic D, de Brouwer A, Hamel B, Kleefstra T, Yntema H, Campistol J, Vilaseca MA, Cheillan D, D'Hooghe M, Diogo L, Garcia P, Valongo C, Fonseca M, Frints S, Wilcken B, von der Haar S, Meijers-Heijboer HE, Hofstede F, Johnson D, Kant SG, Lion-Francois L, Pitelet G, Longo N, Maat-Kievit JA, Monteiro JP, Munnich A, Muntau AC, Nassogne MC, Osaka H, Ounap K, Pinard JM, Quijano-Roy S, Poggenburg I, Poplawski N, Abdul-Rahman O, Ribes A, Arias A, Yaplito-Lee J, Schulze A, Schwartz CE, Schwenger S, Soares G, Sznajer Y, Valayannopoulos V, Van Esch H, Waltz S, Wamelink MM, Pouwels PJ, Errami A, van der Knaap MS, Jakobs C, Mancini GM, Salomons GS
J Med Genet 2013 Jul;50(7):463-72. Epub 2013 May 3 doi: 10.1136/jmedgenet-2013-101658. PMID: 23644449

Diagnosis

Marshall AE, Liang Y, Couse M, McConkey H; Care4Rare Canada Consortium, Sadikovic B, Boycott KM, Dyment DA, Kernohan KD
J Hum Genet 2024 Feb;69(2):101-105. Epub 2023 Oct 31 doi: 10.1038/s10038-023-01203-8. PMID: 37904029
Sandal S, Verma IC, Mahay SB, Dubey S, Sabharwal RK, Kulshrestha S, Saxena R, Suman P, Kumar P, Puri RD
Indian J Pediatr 2024 Jul;91(7):682-695. Epub 2023 Oct 7 doi: 10.1007/s12098-023-04820-5. PMID: 37804371
Agrawal N, Verma G, Saxena D, Kabra M, Gupta N, Mandal K, Moirangthem A, Sheth J, Puri RD, Bijarnia-Mahay S, Kapoor S, Danda S, H SV, Datar CA, Ranganath P, Shukla A, Dalal A, Srivastava P, Devi RR, Phadke SR
Eur J Med Genet 2022 Mar;65(3):104447. Epub 2022 Feb 8 doi: 10.1016/j.ejmg.2022.104447. PMID: 35144014
Bellad A, Bandari AK, Pandey A, Girimaji SC, Muthusamy B
J Mol Neurosci 2020 Sep;70(9):1403-1409. Epub 2020 May 12 doi: 10.1007/s12031-020-01560-5. PMID: 32399860
van de Kamp JM, Betsalel OT, Mercimek-Mahmutoglu S, Abulhoul L, Grünewald S, Anselm I, Azzouz H, Bratkovic D, de Brouwer A, Hamel B, Kleefstra T, Yntema H, Campistol J, Vilaseca MA, Cheillan D, D'Hooghe M, Diogo L, Garcia P, Valongo C, Fonseca M, Frints S, Wilcken B, von der Haar S, Meijers-Heijboer HE, Hofstede F, Johnson D, Kant SG, Lion-Francois L, Pitelet G, Longo N, Maat-Kievit JA, Monteiro JP, Munnich A, Muntau AC, Nassogne MC, Osaka H, Ounap K, Pinard JM, Quijano-Roy S, Poggenburg I, Poplawski N, Abdul-Rahman O, Ribes A, Arias A, Yaplito-Lee J, Schulze A, Schwartz CE, Schwenger S, Soares G, Sznajer Y, Valayannopoulos V, Van Esch H, Waltz S, Wamelink MM, Pouwels PJ, Errami A, van der Knaap MS, Jakobs C, Mancini GM, Salomons GS
J Med Genet 2013 Jul;50(7):463-72. Epub 2013 May 3 doi: 10.1136/jmedgenet-2013-101658. PMID: 23644449

Therapy

Zhao C, Liu Y, Wang Y, Li H, Zhang B, Yue Y, Zhang J
BMC Neurol 2020 Apr 20;20(1):145. doi: 10.1186/s12883-020-01726-z. PMID: 32312236Free PMC Article
Di Pierro D, Ciaccio C, Sbardella D, Tundo GR, Bernardini R, Curatolo P, Galasso C, Pironi V, Coletta M, Marini S
Mol Cell Biochem 2020 Jan;463(1-2):101-113. Epub 2019 Oct 8 doi: 10.1007/s11010-019-03633-5. PMID: 31595423
Mercimek-Mahmutoglu S, Connolly MB, Poskitt KJ, Horvath GA, Lowry N, Salomons GS, Casey B, Sinclair G, Davis C, Jakobs C, Stockler-Ipsiroglu S
Mol Genet Metab 2010 Dec;101(4):409-12. Epub 2010 Aug 26 doi: 10.1016/j.ymgme.2010.08.016. PMID: 20846889
Naito E, Ito M, Yokota I, Saijo T, Ogawa Y, Kuroda Y
J Neurol Sci 2002 Sep 15;201(1-2):33-7. doi: 10.1016/s0022-510x(02)00187-9. PMID: 12163191
Bambauer R, Schiel R, Cordes H, Latza R
Ther Apher 2000 Oct;4(5):338-41. doi: 10.1046/j.1526-0968.2000.004005338.x. PMID: 11111814

Prognosis

Agrawal N, Verma G, Saxena D, Kabra M, Gupta N, Mandal K, Moirangthem A, Sheth J, Puri RD, Bijarnia-Mahay S, Kapoor S, Danda S, H SV, Datar CA, Ranganath P, Shukla A, Dalal A, Srivastava P, Devi RR, Phadke SR
Eur J Med Genet 2022 Mar;65(3):104447. Epub 2022 Feb 8 doi: 10.1016/j.ejmg.2022.104447. PMID: 35144014
Greene D; NIHR BioResource, Richardson S, Turro E
Am J Hum Genet 2017 Jul 6;101(1):104-114. Epub 2017 Jun 29 doi: 10.1016/j.ajhg.2017.05.015. PMID: 28669401Free PMC Article
van de Kamp JM, Betsalel OT, Mercimek-Mahmutoglu S, Abulhoul L, Grünewald S, Anselm I, Azzouz H, Bratkovic D, de Brouwer A, Hamel B, Kleefstra T, Yntema H, Campistol J, Vilaseca MA, Cheillan D, D'Hooghe M, Diogo L, Garcia P, Valongo C, Fonseca M, Frints S, Wilcken B, von der Haar S, Meijers-Heijboer HE, Hofstede F, Johnson D, Kant SG, Lion-Francois L, Pitelet G, Longo N, Maat-Kievit JA, Monteiro JP, Munnich A, Muntau AC, Nassogne MC, Osaka H, Ounap K, Pinard JM, Quijano-Roy S, Poggenburg I, Poplawski N, Abdul-Rahman O, Ribes A, Arias A, Yaplito-Lee J, Schulze A, Schwartz CE, Schwenger S, Soares G, Sznajer Y, Valayannopoulos V, Van Esch H, Waltz S, Wamelink MM, Pouwels PJ, Errami A, van der Knaap MS, Jakobs C, Mancini GM, Salomons GS
J Med Genet 2013 Jul;50(7):463-72. Epub 2013 May 3 doi: 10.1136/jmedgenet-2013-101658. PMID: 23644449
Bambauer R, Schiel R, Cordes H, Latza R
Ther Apher 2000 Oct;4(5):338-41. doi: 10.1046/j.1526-0968.2000.004005338.x. PMID: 11111814

Clinical prediction guides

Sandal S, Verma IC, Mahay SB, Dubey S, Sabharwal RK, Kulshrestha S, Saxena R, Suman P, Kumar P, Puri RD
Indian J Pediatr 2024 Jul;91(7):682-695. Epub 2023 Oct 7 doi: 10.1007/s12098-023-04820-5. PMID: 37804371
Muenzer J, Burton BK, Amartino HM, Harmatz PR, Gutiérrez-Solana LG, Ruiz-Garcia M, Wu Y, Merberg D, Alexanderian D, Jones SA
Orphanet J Rare Dis 2023 Nov 16;18(1):357. doi: 10.1186/s13023-023-02805-3. PMID: 37974184Free PMC Article
Zhao MY, Dahlen A, Ramirez NJ, Moseley M, Van Haren K, Zaharchuk G
J Neurosci Res 2023 Jul;101(7):1086-1097. Epub 2023 Mar 26 doi: 10.1002/jnr.25187. PMID: 36967233
Ak M, Akturk Z, Bowyer K, Mignon L, Pasupuleti S, Glaze DG, Suter B, Pehlivan D
Pediatr Neurol 2022 Aug;133:1-8. Epub 2022 May 25 doi: 10.1016/j.pediatrneurol.2022.05.008. PMID: 35716604
Agrawal N, Verma G, Saxena D, Kabra M, Gupta N, Mandal K, Moirangthem A, Sheth J, Puri RD, Bijarnia-Mahay S, Kapoor S, Danda S, H SV, Datar CA, Ranganath P, Shukla A, Dalal A, Srivastava P, Devi RR, Phadke SR
Eur J Med Genet 2022 Mar;65(3):104447. Epub 2022 Feb 8 doi: 10.1016/j.ejmg.2022.104447. PMID: 35144014

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