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Intellectual developmental disorder with poor growth and with or without seizures or ataxia(IDPOGSA)

MedGen UID:
1711370
Concept ID:
C5394135
Disease or Syndrome
Synonyms: IDPOGSA; INTELLECTUAL DEVELOPMENTAL DISORDER WITH POOR GROWTH AND WITH OR WITHOUT SEIZURES OR ATAXIA
 
Gene (location): ABCA2 (9q34.3)
 
Monarch Initiative: MONDO:0032930
OMIM®: 618808

Definition

Intellectual developmental disorder with poor growth and with or without seizures or ataxia (IDPOGSA) is an autosomal recessive neurologic disorder characterized by global developmental delay apparent from infancy, hypotonia, and poor overall growth, sometimes with borderline microcephaly. The phenotype is highly variable: some patients may show ataxia and some may have seizures (summary by Hu et al., 2019). [from OMIM]

Clinical features

From HPO
Aggressive behavior
MedGen UID:
1375
Concept ID:
C0001807
Individual Behavior
Behavior or an act aimed at harming a person, animal, or physical property (e.g., acts of physical violence; shouting, swearing, and using harsh language; slashing someone's tires).
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.
Intellectual disability, mild
MedGen UID:
10044
Concept ID:
C0026106
Mental or Behavioral Dysfunction
Mild intellectual disability is defined as an intelligence quotient (IQ) in the range of 50-69.
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.
Delayed ability to walk
MedGen UID:
66034
Concept ID:
C0241726
Finding
A failure to achieve the ability to walk at an appropriate developmental stage. Most children learn to walk in a series of stages, and learn to walk short distances independently between 12 and 15 months.
Incoordination
MedGen UID:
141714
Concept ID:
C0520966
Finding
Gait ataxia
MedGen UID:
155642
Concept ID:
C0751837
Sign or Symptom
A type of ataxia characterized by the impairment of the ability to coordinate the movements required for normal walking. Gait ataxia is characteirzed by a wide-based staggering gait with a tendency to fall.
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.
Mild global developmental delay
MedGen UID:
861405
Concept ID:
C4012968
Finding
A mild delay in the achievement of motor or mental milestones in the domains of development of a child.
Muscle weakness
MedGen UID:
57735
Concept ID:
C0151786
Finding
Reduced strength of muscles.
Microcephaly
MedGen UID:
1644158
Concept ID:
C4551563
Finding
Head circumference below 2 standard deviations below the mean for age and gender.
Increased LDL cholesterol concentration
MedGen UID:
154289
Concept ID:
C0549399
Finding
An elevated concentration of low-density lipoprotein cholesterol in the blood.
Fair hair
MedGen UID:
336542
Concept ID:
C1849221
Finding
A lesser degree of hair pigmentation than would otherwise be expected.
Developmental cataract
MedGen UID:
3202
Concept ID:
C0009691
Congenital Abnormality
A cataract that occurs congenitally as the result of a developmental defect, in contrast to the majority of cataracts that occur in adulthood as the result of degenerative changes of the lens.

Professional guidelines

PubMed

Charalambous M, Muñana K, Patterson EE, Platt SR, Volk HA
J Vet Intern Med 2024 Jan-Feb;38(1):19-40. Epub 2023 Nov 3 doi: 10.1111/jvim.16928. PMID: 37921621Free PMC Article
Zuberi SM, Wirrell E, Yozawitz E, Wilmshurst JM, Specchio N, Riney K, Pressler R, Auvin S, Samia P, Hirsch E, Galicchio S, Triki C, Snead OC, Wiebe S, Cross JH, Tinuper P, Scheffer IE, Perucca E, Moshé SL, Nabbout R
Epilepsia 2022 Jun;63(6):1349-1397. Epub 2022 May 3 doi: 10.1111/epi.17239. PMID: 35503712
Pedroso JL, Vale TC, Braga-Neto P, Dutra LA, França MC Jr, Teive HAG, Barsottini OGP
Arq Neuropsiquiatr 2019 Mar;77(3):184-193. doi: 10.1590/0004-282X20190020. PMID: 30970132

Recent clinical studies

Etiology

Cabaraux P, Agrawal SK, Cai H, Calabro RS, Casali C, Damm L, Doss S, Habas C, Horn AKE, Ilg W, Louis ED, Mitoma H, Monaco V, Petracca M, Ranavolo A, Rao AK, Ruggieri S, Schirinzi T, Serrao M, Summa S, Strupp M, Surgent O, Synofzik M, Tao S, Terasi H, Torres-Russotto D, Travers B, Roper JA, Manto M
Cerebellum 2023 Jun;22(3):394-430. Epub 2022 Apr 12 doi: 10.1007/s12311-022-01373-9. PMID: 35414041
Ziobro J, Shellhaas RA
Semin Neurol 2020 Apr;40(2):246-256. Epub 2020 Mar 6 doi: 10.1055/s-0040-1702943. PMID: 32143234
Shellhaas RA
Handb Clin Neurol 2019;162:347-361. doi: 10.1016/B978-0-444-64029-1.00017-5. PMID: 31324320
Pedroso JL, Vale TC, Braga-Neto P, Dutra LA, França MC Jr, Teive HAG, Barsottini OGP
Arq Neuropsiquiatr 2019 Mar;77(3):184-193. doi: 10.1590/0004-282X20190020. PMID: 30970132
Sullivan R, Yau WY, O'Connor E, Houlden H
J Neurol 2019 Feb;266(2):533-544. Epub 2018 Oct 3 doi: 10.1007/s00415-018-9076-4. PMID: 30284037Free PMC Article

Diagnosis

Kuo SH
Continuum (Minneap Minn) 2019 Aug;25(4):1036-1054. doi: 10.1212/CON.0000000000000753. PMID: 31356292Free PMC Article
de Silva RN, Vallortigara J, Greenfield J, Hunt B, Giunti P, Hadjivassiliou M
Pract Neurol 2019 Jun;19(3):196-207. Epub 2019 May 2 doi: 10.1136/practneurol-2018-002096. PMID: 31048364Free PMC Article
Pedroso JL, Vale TC, Braga-Neto P, Dutra LA, França MC Jr, Teive HAG, Barsottini OGP
Arq Neuropsiquiatr 2019 Mar;77(3):184-193. doi: 10.1590/0004-282X20190020. PMID: 30970132
Sullivan R, Yau WY, O'Connor E, Houlden H
J Neurol 2019 Feb;266(2):533-544. Epub 2018 Oct 3 doi: 10.1007/s00415-018-9076-4. PMID: 30284037Free PMC Article
Mitoma H, Manto M, Hampe CS
Curr Neuropharmacol 2019;17(1):33-58. doi: 10.2174/1570159X16666180917105033. PMID: 30221603Free PMC Article

Therapy

Hassan A
Tremor Other Hyperkinet Mov (N Y) 2023;13:9. Epub 2023 Mar 28 doi: 10.5334/tohm.747. PMID: 37008993Free PMC Article
Jain P, Aneja S, Cunningham J, Arya R, Sharma S
Seizure 2022 Nov;102:74-82. Epub 2022 Sep 26 doi: 10.1016/j.seizure.2022.09.017. PMID: 36209676
Ziobro JM, Eschbach K, Shellhaas RA
Neurotherapeutics 2021 Jul;18(3):1564-1581. Epub 2021 Aug 12 doi: 10.1007/s13311-021-01085-8. PMID: 34386906Free PMC Article
Kuo SH
Continuum (Minneap Minn) 2019 Aug;25(4):1036-1054. doi: 10.1212/CON.0000000000000753. PMID: 31356292Free PMC Article
Trinka E, Kälviäinen R
Seizure 2017 Jan;44:65-73. Epub 2016 Nov 14 doi: 10.1016/j.seizure.2016.11.001. PMID: 27890484

Prognosis

Salanova V, Sperling MR, Gross RE, Irwin CP, Vollhaber JA, Giftakis JE, Fisher RS; SANTÉ Study Group
Epilepsia 2021 Jun;62(6):1306-1317. Epub 2021 Apr 8 doi: 10.1111/epi.16895. PMID: 33830503
Kuo SH
Continuum (Minneap Minn) 2019 Aug;25(4):1036-1054. doi: 10.1212/CON.0000000000000753. PMID: 31356292Free PMC Article
Bertini E, Zanni G, Boltshauser E
Handb Clin Neurol 2018;155:91-103. doi: 10.1016/B978-0-444-64189-2.00006-8. PMID: 29891079
Patterson JL, Carapetian SA, Hageman JR, Kelley KR
Pediatr Ann 2013 Dec;42(12):249-54. doi: 10.3928/00904481-20131122-09. PMID: 24295158
Winchester S, Singh PK, Mikati MA
Handb Clin Neurol 2013;112:1213-7. doi: 10.1016/B978-0-444-52910-7.00043-X. PMID: 23622331

Clinical prediction guides

Coarelli G, Wirth T, Tranchant C, Koenig M, Durr A, Anheim M
J Neurol 2023 Jan;270(1):208-222. Epub 2022 Sep 24 doi: 10.1007/s00415-022-11383-6. PMID: 36152050Free PMC Article
Baud MO, Proix T, Rao VR, Schindler K
Curr Opin Neurol 2020 Apr;33(2):163-172. doi: 10.1097/WCO.0000000000000798. PMID: 32049738
Xu MY
Stroke Vasc Neurol 2019 Mar;4(1):48-56. Epub 2018 Dec 9 doi: 10.1136/svn-2018-000175. PMID: 31105979Free PMC Article
Acharya UR, Hagiwara Y, Adeli H
Epilepsy Behav 2018 Nov;88:251-261. Epub 2018 Oct 11 doi: 10.1016/j.yebeh.2018.09.030. PMID: 30317059
Krogias C, Postert T, Eyding J
Int Rev Neurobiol 2010;90:217-35. doi: 10.1016/S0074-7742(10)90016-0. PMID: 20692505

Recent systematic reviews

Pressler RM, Abend NS, Auvin S, Boylan G, Brigo F, Cilio MR, De Vries LS, Elia M, Espeche A, Hahn CD, Inder T, Jette N, Kakooza-Mwesige A, Mader S, Mizrahi EM, Moshé SL, Nagarajan L, Noyman I, Nunes ML, Samia P, Shany E, Shellhaas RA, Subota A, Triki CC, Tsuchida T, Vinayan KP, Wilmshurst JM, Yozawitz EG, Hartmann H
Epilepsia 2023 Oct;64(10):2550-2570. Epub 2023 Sep 1 doi: 10.1111/epi.17745. PMID: 37655702
Jain P, Aneja S, Cunningham J, Arya R, Sharma S
Seizure 2022 Nov;102:74-82. Epub 2022 Sep 26 doi: 10.1016/j.seizure.2022.09.017. PMID: 36209676
Lamberink HJ, Otte WM, Geerts AT, Pavlovic M, Ramos-Lizana J, Marson AG, Overweg J, Sauma L, Specchio LM, Tennison M, Cardoso TMO, Shinnar S, Schmidt D, Geleijns K, Braun KPJ
Lancet Neurol 2017 Jul;16(7):523-531. Epub 2017 May 5 doi: 10.1016/S1474-4422(17)30114-X. PMID: 28483337
Marquer A, Barbieri G, Pérennou D
Ann Phys Rehabil Med 2014 Mar;57(2):67-78. Epub 2014 Feb 6 doi: 10.1016/j.rehab.2014.01.002. PMID: 24582474
Mewasingh LD
BMJ Clin Evid 2014 Jan 31;2014 PMID: 24484859Free PMC Article

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