U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Developmental and epileptic encephalopathy 108(DEE108)

MedGen UID:
1824026
Concept ID:
C5774253
Disease or Syndrome
Synonym: DEE108
 
Gene (location): MAST3 (19p13.11)
 
Monarch Initiative: MONDO:0859314
OMIM®: 620115

Definition

Developmental and epileptic encephalopathy-108 (DEE108) is characterized by the onset of multiple types of seizures in the first 2 years of life. Affected individuals often have normal early development before the onset of seizures, after which they show developmental regression with loss of skills, particularly language; most are nonverbal or speak only a few words. Other features included mildly delayed walking, unsteady gait, hypotonia, and behavioral abnormalities, such as ADHD or autism (Spinelli et al., 2021). For a general phenotypic description and a discussion of genetic heterogeneity of DEE, see 308350. [from OMIM]

Clinical features

From HPO
Focal impaired awareness seizure
MedGen UID:
543022
Concept ID:
C0270834
Disease or Syndrome
Focal impaired awareness seizure (or focal seizure with impaired or lost awareness) is a type of focal-onset seizure characterized by some degree (which may be partial) of impairment of the person's awareness of themselves or their surroundings at any point during the seizure.
Tonic seizure
MedGen UID:
82855
Concept ID:
C0270844
Disease or Syndrome
A tonic seizure is a type of motor seizure characterized by unilateral or bilateral limb stiffening or elevation, often with neck stiffening.
Atonic seizure
MedGen UID:
78735
Concept ID:
C0270846
Disease or Syndrome
Atonic seizure is a type of motor seizure characterized by a sudden loss or diminution of muscle tone without apparent preceding myoclonic or tonic event lasting about 1 to 2 seconds, involving head, trunk, jaw, or limb musculature.
Convulsive status epilepticus
MedGen UID:
137148
Concept ID:
C0311335
Disease or Syndrome
A type of status epilepticus characterized by a prolonged bilateral tonic-clonic seizure, or repeated bilateral tonic-clonic seizures without recovery between.
Epileptic encephalopathy
MedGen UID:
452596
Concept ID:
C0543888
Disease or Syndrome
A condition in which epileptiform abnormalities are believed to contribute to the progressive disturbance in cerebral function. Epileptic encephalaopathy is characterized by (1) electrographic EEG paroxysmal activity that is often aggressive, (2) seizures that are usually multiform and intractable, (3) cognitive, behavioral and neurological deficits that may be relentless, and (4) sometimes early death.
Bilateral tonic-clonic seizure with focal onset
MedGen UID:
164077
Concept ID:
C0877017
Disease or Syndrome
A bilateral tonic-clonic seizure with focal onset is a focal-onset seizure which progresses into a bilateral tonic-clonic phase.
Focal motor status epilepticus
MedGen UID:
1716066
Concept ID:
C1396824
Disease or Syndrome
Status epilepticus with focal motor signs originating within networks limited to one hemisphere. Involves musculature in any form. The motor event could consist of an increase (positive) or decrease (negative) in muscle contraction to produce a movement.
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.
Small pituitary gland
MedGen UID:
868479
Concept ID:
C4022873
Anatomical Abnormality
An abnormally decreased size of the pituitary gland.
Generalized non-motor (absence) seizure
MedGen UID:
1385688
Concept ID:
C4316903
Disease or Syndrome
A generalized non-motor (absence) seizure is a type of a type of dialeptic seizure that is of electrographically generalized onset. It is a generalized seizure characterized by an interruption of activities, a blank stare, and usually the person will be unresponsive when spoken to. Any ictal motor phenomena are minor in comparison to these non-motor features.
Myoclonic seizure
MedGen UID:
1385980
Concept ID:
C4317123
Sign or Symptom
A myoclonic seizure is a type of motor seizure characterized by sudden, brief (<100 ms) involuntary single or multiple contraction of muscles or muscle groups of variable topography (axial, proximal limb, distal). Myoclonus is less regularly repetitive and less sustained than is clonus.
Bilateral tonic-clonic seizure with generalized onset
MedGen UID:
1368929
Concept ID:
C4476643
Sign or Symptom
A bilateral tonic-clonic seizure with generalized onset is a type of bilateral tonic-clonic seizure characterized by generalized onset; these seizures rapidly engage networks in both hemispheres at the start of the seizure.
Thin corpus callosum
MedGen UID:
1785336
Concept ID:
C5441562
Anatomical Abnormality
An abnormally thin corpus callous, due to atrophy, hypoplasia or agenesis. This term is intended to be used in situations where it is not known if thinning of the corpus callosum (for instance, as visualized by magnetic resonance tomography) is due to abnormal development (e.g. a leukodystrophy) or atrophy following normal development (e.g. neurodegeneration).

Professional guidelines

PubMed

Chourasia N, Yuskaitis CJ, Libenson MH, Bergin AM, Liu S, Zhang B, Poduri A, Harini C
Epilepsia 2022 May;63(5):1164-1176. Epub 2022 Mar 18 doi: 10.1111/epi.17209. PMID: 35211955
Mastrangelo M
Metab Brain Dis 2021 Jan;36(1):29-43. Epub 2020 Oct 23 doi: 10.1007/s11011-020-00635-x. PMID: 33095372
van Wegberg AMJ, MacDonald A, Ahring K, Bélanger-Quintana A, Blau N, Bosch AM, Burlina A, Campistol J, Feillet F, Giżewska M, Huijbregts SC, Kearney S, Leuzzi V, Maillot F, Muntau AC, van Rijn M, Trefz F, Walter JH, van Spronsen FJ
Orphanet J Rare Dis 2017 Oct 12;12(1):162. doi: 10.1186/s13023-017-0685-2. PMID: 29025426Free PMC Article

Recent clinical studies

Etiology

Alharbi HM, Pinchefsky EF, Tran MA, Salazar Cerda CI, Parokaran Varghese J, Kamino D, Widjaja E, Mamak E, Ly L, Nevalainen P, Hahn CD, Tam EWY
Neurology 2023 May 9;100(19):e1976-e1984. Epub 2023 Mar 29 doi: 10.1212/WNL.0000000000207202. PMID: 36990719Free PMC Article
Sullivan KR, Ruggiero SM, Xian J, Thalwitzer KM, Ali R, Stewart S, Cosico M, Steinberg J, Goss J, Pfalzer AC, Horning KJ, Weitzel N, Corey S, Conway L, Rigby CS, Bichell TJ, Helbig I
Epilepsia Open 2023 Jun;8(2):320-333. Epub 2023 Apr 27 doi: 10.1002/epi4.12688. PMID: 36625631Free PMC Article
Robertsson Grossmann K, Eriksson Westblad M, Blennow M, Lindström K
Arch Dis Child Fetal Neonatal Ed 2023 May;108(3):295-301. Epub 2022 Dec 9 doi: 10.1136/archdischild-2022-324418. PMID: 36600485Free PMC Article
Mastrangelo M
Acta Neurol Belg 2019 Dec;119(4):511-521. Epub 2019 Jun 3 doi: 10.1007/s13760-019-01160-0. PMID: 31161467
O'Callaghan FJ, Edwards SW, Alber FD, Hancock E, Johnson AL, Kennedy CR, Likeman M, Lux AL, Mackay M, Mallick AA, Newton RW, Nolan M, Pressler R, Rating D, Schmitt B, Verity CM, Osborne JP; participating investigators
Lancet Neurol 2017 Jan;16(1):33-42. Epub 2016 Nov 10 doi: 10.1016/S1474-4422(16)30294-0. PMID: 27838190

Diagnosis

Chang SC, Eichinger CS, Field P
Eur J Med Res 2024 Mar 18;29(1):181. doi: 10.1186/s40001-024-01771-1. PMID: 38494502Free PMC Article
Vezyroglou A, Akilapa R, Barwick K, Koene S, Brownstein CA, Holder-Espinasse M, Fry AE, Németh AH, Tofaris GK, Hay E, Hughes I, Mansour S, Mordekar SR, Splitt M, Turnpenny PD, Demetriou D, Koopmann TT, Ruivenkamp CAL, Agrawal PB, Carr L, Clowes V, Ghali N, Holder SE, Radley J, Male A, Sisodiya SM, Kurian MA, Cross JH, Balasubramanian M
Neurology 2022 Oct 4;99(14):e1511-e1526. Epub 2022 Jul 18 doi: 10.1212/WNL.0000000000200927. PMID: 36192182Free PMC Article
Mastrangelo M
Acta Neurol Belg 2019 Dec;119(4):511-521. Epub 2019 Jun 3 doi: 10.1007/s13760-019-01160-0. PMID: 31161467
van Wegberg AMJ, MacDonald A, Ahring K, Bélanger-Quintana A, Blau N, Bosch AM, Burlina A, Campistol J, Feillet F, Giżewska M, Huijbregts SC, Kearney S, Leuzzi V, Maillot F, Muntau AC, van Rijn M, Trefz F, Walter JH, van Spronsen FJ
Orphanet J Rare Dis 2017 Oct 12;12(1):162. doi: 10.1186/s13023-017-0685-2. PMID: 29025426Free PMC Article
O'Callaghan FJ, Edwards SW, Alber FD, Hancock E, Johnson AL, Kennedy CR, Likeman M, Lux AL, Mackay M, Mallick AA, Newton RW, Nolan M, Pressler R, Rating D, Schmitt B, Verity CM, Osborne JP; participating investigators
Lancet Neurol 2017 Jan;16(1):33-42. Epub 2016 Nov 10 doi: 10.1016/S1474-4422(16)30294-0. PMID: 27838190

Therapy

Martin M, Hill C, Bewley S, MacLennan AH, Braillon A
Birth Defects Res 2022 Jan 1;114(1):13-16. Epub 2021 Dec 5 doi: 10.1002/bdr2.1967. PMID: 34866359
Caraballo RH, Pociecha J, Reyes G, Espeche A, Galichio S, Fasulo L, Semprino M
Epilepsy Behav 2020 Jul;108:107074. Epub 2020 Apr 23 doi: 10.1016/j.yebeh.2020.107074. PMID: 32334364
O'Callaghan FJ, Edwards SW, Alber FD, Hancock E, Johnson AL, Kennedy CR, Likeman M, Lux AL, Mackay M, Mallick AA, Newton RW, Nolan M, Pressler R, Rating D, Schmitt B, Verity CM, Osborne JP; participating investigators
Lancet Neurol 2017 Jan;16(1):33-42. Epub 2016 Nov 10 doi: 10.1016/S1474-4422(16)30294-0. PMID: 27838190
Bromley R, Weston J, Adab N, Greenhalgh J, Sanniti A, McKay AJ, Tudur Smith C, Marson AG
Cochrane Database Syst Rev 2014 Oct 30;2014(10):CD010236. doi: 10.1002/14651858.CD010236.pub2. PMID: 25354543Free PMC Article
Wheless JW, Gibson PA, Rosbeck KL, Hardin M, O'Dell C, Whittemore V, Pellock JM
BMC Pediatr 2012 Jul 25;12:108. doi: 10.1186/1471-2431-12-108. PMID: 22830456Free PMC Article

Prognosis

Mastrangelo M, Manti F, Ricciardi G, Cinnante EMC, Cameli N, Beatrice A, Tolve M, Pisani F
Eur J Pediatr 2024 Sep;183(9):3665-3678. Epub 2024 Jul 2 doi: 10.1007/s00431-024-05657-6. PMID: 38954008Free PMC Article
Usmani MA, Ahmed ZM, Magini P, Pienkowski VM, Rasmussen KJ, Hernan R, Rasheed F, Hussain M, Shahzad M, Lanpher BC, Niu Z, Lim FY, Pippucci T, Ploski R, Kraus V, Matuszewska K, Palombo F, Kianmahd J; UCLA Clinical Genomics Center, Martinez-Agosto JA, Lee H, Colao E, Motazacker MM, Brigatti KW, Puffenberger EG, Riazuddin SA, Gonzaga-Jauregui C, Chung WK, Wagner M, Schultz MJ, Seri M, Kievit AJA, Perrotti N, Wassink-Ruiter JSK, van Bokhoven H, Riazuddin S, Riazuddin S
Am J Hum Genet 2021 Jul 1;108(7):1330-1341. Epub 2021 Jun 7 doi: 10.1016/j.ajhg.2021.05.007. PMID: 34102099Free PMC Article
Spagnoli C, Fusco C, Percesepe A, Leuzzi V, Pisani F
Int J Mol Sci 2021 Apr 18;22(8) doi: 10.3390/ijms22084202. PMID: 33919646Free PMC Article
O'Callaghan FJ, Edwards SW, Alber FD, Hancock E, Johnson AL, Kennedy CR, Likeman M, Lux AL, Mackay M, Mallick AA, Newton RW, Nolan M, Pressler R, Rating D, Schmitt B, Verity CM, Osborne JP; participating investigators
Lancet Neurol 2017 Jan;16(1):33-42. Epub 2016 Nov 10 doi: 10.1016/S1474-4422(16)30294-0. PMID: 27838190
Wheless JW, Gibson PA, Rosbeck KL, Hardin M, O'Dell C, Whittemore V, Pellock JM
BMC Pediatr 2012 Jul 25;12:108. doi: 10.1186/1471-2431-12-108. PMID: 22830456Free PMC Article

Clinical prediction guides

Alharbi HM, Pinchefsky EF, Tran MA, Salazar Cerda CI, Parokaran Varghese J, Kamino D, Widjaja E, Mamak E, Ly L, Nevalainen P, Hahn CD, Tam EWY
Neurology 2023 May 9;100(19):e1976-e1984. Epub 2023 Mar 29 doi: 10.1212/WNL.0000000000207202. PMID: 36990719Free PMC Article
Robertsson Grossmann K, Eriksson Westblad M, Blennow M, Lindström K
Arch Dis Child Fetal Neonatal Ed 2023 May;108(3):295-301. Epub 2022 Dec 9 doi: 10.1136/archdischild-2022-324418. PMID: 36600485Free PMC Article
Vezyroglou A, Akilapa R, Barwick K, Koene S, Brownstein CA, Holder-Espinasse M, Fry AE, Németh AH, Tofaris GK, Hay E, Hughes I, Mansour S, Mordekar SR, Splitt M, Turnpenny PD, Demetriou D, Koopmann TT, Ruivenkamp CAL, Agrawal PB, Carr L, Clowes V, Ghali N, Holder SE, Radley J, Male A, Sisodiya SM, Kurian MA, Cross JH, Balasubramanian M
Neurology 2022 Oct 4;99(14):e1511-e1526. Epub 2022 Jul 18 doi: 10.1212/WNL.0000000000200927. PMID: 36192182Free PMC Article
Usmani MA, Ahmed ZM, Magini P, Pienkowski VM, Rasmussen KJ, Hernan R, Rasheed F, Hussain M, Shahzad M, Lanpher BC, Niu Z, Lim FY, Pippucci T, Ploski R, Kraus V, Matuszewska K, Palombo F, Kianmahd J; UCLA Clinical Genomics Center, Martinez-Agosto JA, Lee H, Colao E, Motazacker MM, Brigatti KW, Puffenberger EG, Riazuddin SA, Gonzaga-Jauregui C, Chung WK, Wagner M, Schultz MJ, Seri M, Kievit AJA, Perrotti N, Wassink-Ruiter JSK, van Bokhoven H, Riazuddin S, Riazuddin S
Am J Hum Genet 2021 Jul 1;108(7):1330-1341. Epub 2021 Jun 7 doi: 10.1016/j.ajhg.2021.05.007. PMID: 34102099Free PMC Article
Glass HC, Grinspan ZM, Li Y, McNamara NA, Chang T, Chu CJ, Massey SL, Abend NS, Lemmon ME, Thomas C, McCulloch CE, Shellhaas RA; Neonatal Seizure Registry Study Group
Epilepsia 2020 Dec;61(12):2774-2784. Epub 2020 Nov 13 doi: 10.1111/epi.16749. PMID: 33188528Free PMC Article

Recent systematic reviews

Mastrangelo M, Manti F, Ricciardi G, Cinnante EMC, Cameli N, Beatrice A, Tolve M, Pisani F
Eur J Pediatr 2024 Sep;183(9):3665-3678. Epub 2024 Jul 2 doi: 10.1007/s00431-024-05657-6. PMID: 38954008Free PMC Article
Chang SC, Eichinger CS, Field P
Eur J Med Res 2024 Mar 18;29(1):181. doi: 10.1186/s40001-024-01771-1. PMID: 38494502Free PMC Article
Spagnoli C, Fusco C, Percesepe A, Leuzzi V, Pisani F
Int J Mol Sci 2021 Apr 18;22(8) doi: 10.3390/ijms22084202. PMID: 33919646Free PMC Article
Jansson JS, Hallböök T, Reilly C
Epilepsy Behav 2020 Jul;108:107079. Epub 2020 Apr 22 doi: 10.1016/j.yebeh.2020.107079. PMID: 32334365
Bromley R, Weston J, Adab N, Greenhalgh J, Sanniti A, McKay AJ, Tudur Smith C, Marson AG
Cochrane Database Syst Rev 2014 Oct 30;2014(10):CD010236. doi: 10.1002/14651858.CD010236.pub2. PMID: 25354543Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...