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Neurodevelopmental disorder with hyperkinetic movements and dyskinesia(NEDHYD)

MedGen UID:
1794248
Concept ID:
C5562038
Disease or Syndrome
Synonyms: NEDHYD; NEURODEVELOPMENTAL DISORDER WITH HYPERKINETIC MOVEMENTS AND DYSKINESIA
 
Gene (location): ADCY5 (3q21.1)
 
Monarch Initiative: MONDO:0859211
OMIM®: 619651

Definition

Neurodevelopmental disorder with hyperkinetic movements and dyskinesia (NEDHYD) is an autosomal recessive complex neurologic disorder characterized by severe global developmental delay with axial hypotonia, impaired intellectual development, poor overall growth, and abnormal involuntary hyperkinetic movements, including dystonia, myoclonus, spasticity, and orofacial dyskinesia. It is the most severe manifestation of ADCY5-related dyskinetic disorders (summary by Okamoto et al., 2021 and Kaiyrzhanov et al., 2021). [from OMIM]

Clinical features

From HPO
Cardiomyopathy
MedGen UID:
209232
Concept ID:
C0878544
Disease or Syndrome
A myocardial disorder in which the heart muscle is structurally and functionally abnormal, in the absence of coronary artery disease, hypertension, valvular disease and congenital heart disease sufficient to cause the observed myocardial abnormality.
Failure to thrive
MedGen UID:
746019
Concept ID:
C2315100
Disease or Syndrome
Failure to thrive (FTT) refers to a child whose physical growth is substantially below the norm.
Anxiety
MedGen UID:
1613
Concept ID:
C0003467
Finding
Intense feelings of nervousness, tenseness, or panic, often in reaction to interpersonal stresses; worry about the negative effects of past unpleasant experiences and future negative possibilities; feeling fearful, apprehensive, or threatened by uncertainty; fears of falling apart or losing control.
Dystonic disorder
MedGen UID:
3940
Concept ID:
C0013421
Sign or Symptom
An abnormally increased muscular tone that causes fixed abnormal postures. There is a slow, intermittent twisting motion that leads to exaggerated turning and posture of the extremities and trunk.
Intellectual disability, moderate
MedGen UID:
7680
Concept ID:
C0026351
Mental or Behavioral Dysfunction
Moderate mental retardation is defined as an intelligence quotient (IQ) in the range of 35-49.
Myoclonus
MedGen UID:
10234
Concept ID:
C0027066
Sign or Symptom
Very brief, involuntary random muscular contractions occurring at rest, in response to sensory stimuli, or accompanying voluntary movements.
Intellectual disability, severe
MedGen UID:
48638
Concept ID:
C0036857
Mental or Behavioral Dysfunction
Severe mental retardation is defined as an intelligence quotient (IQ) in the range of 20-34.
Tremor
MedGen UID:
21635
Concept ID:
C0040822
Sign or Symptom
An unintentional, oscillating to-and-fro muscle movement about a joint axis.
Agitation
MedGen UID:
88447
Concept ID:
C0085631
Sign or Symptom
A state of exceeding restlessness and excessive motor activity associated with mental distress or a feeling of inner tension.
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.
Delayed speech and language development
MedGen UID:
105318
Concept ID:
C0454644
Finding
A degree of language development that is significantly below the norm for a child of a specified age.
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.
Compulsive behaviors
MedGen UID:
109373
Concept ID:
C0600104
Mental or Behavioral Dysfunction
Recurrent obsessions or compulsions that are severe enough to be time consuming (i.e., they take more than 1 hour a day) or cause marked distress or significant impairment (DSM-IV).
Reduced eye contact
MedGen UID:
303190
Concept ID:
C1445953
Finding
Difficulty in looking at another person in the eye.
Hypoplasia of the brainstem
MedGen UID:
334226
Concept ID:
C1842688
Finding
Underdevelopment of the brainstem.
Delayed ability to sit
MedGen UID:
1368737
Concept ID:
C4476710
Finding
A failure to achieve the ability to sit at an appropriate developmental stage. Most children sit with support at 6 months of age and sit steadily without support at 9 months of age.
Delayed ability to roll over
MedGen UID:
1718874
Concept ID:
C5397980
Finding
Delayed ahcievement of the ability to roll front to back and back to front.
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).
Poor head control
MedGen UID:
322809
Concept ID:
C1836038
Finding
Difficulty to maintain correct position of the head while standing or sitting.
Axial hypotonia
MedGen UID:
342959
Concept ID:
C1853743
Finding
Muscular hypotonia (abnormally low muscle tone) affecting the musculature of the trunk.

Professional guidelines

PubMed

Berardelli I, Pasquini M, Conte A, Bologna M, Berardelli A, Fabbrini G
Expert Rev Neurother 2019 Jan;19(1):55-65. Epub 2018 Dec 10 doi: 10.1080/14737175.2019.1555475. PMID: 30501439
Niemann N, Jankovic J
Drugs 2018 Apr;78(5):525-541. doi: 10.1007/s40265-018-0874-x. PMID: 29484607
Chen JJ, Ondo WG, Dashtipour K, Swope DM
Clin Ther 2012 Jul;34(7):1487-504. Epub 2012 Jun 28 doi: 10.1016/j.clinthera.2012.06.010. PMID: 22749259

Recent clinical studies

Etiology

Niemann N, Jankovic J
Drugs 2018 Apr;78(5):525-541. doi: 10.1007/s40265-018-0874-x. PMID: 29484607
Hirsch LE, Pringsheim T
Cochrane Database Syst Rev 2016 Jun 26;2016(6):CD009043. doi: 10.1002/14651858.CD009043.pub3. PMID: 27344135Free PMC Article
Pietracupa S, Bruno E, Cavanna AE, Falla M, Zappia M, Colosimo C
J Neurol Sci 2015 Nov 15;358(1-2):9-21. Epub 2015 Sep 2 doi: 10.1016/j.jns.2015.08.1544. PMID: 26428309
Ching H, Pringsheim T
Cochrane Database Syst Rev 2012 May 16;(5):CD009043. doi: 10.1002/14651858.CD009043.pub2. PMID: 22592735
Taylor EA
Br J Psychiatry 1986 Nov;149:562-73. doi: 10.1192/bjp.149.5.562. PMID: 3545357

Diagnosis

Lenka A, Jankovic J
Tremor Other Hyperkinet Mov (N Y) 2023;13:8. Epub 2023 Mar 28 doi: 10.5334/tohm.758. PMID: 37008994Free PMC Article
Srinivasan R, Ben-Pazi H, Dekker M, Cubo E, Bloem B, Moukheiber E, Gonzalez-Santos J, Guttman M
Tremor Other Hyperkinet Mov (N Y) 2020;10 Epub 2020 Feb 17 doi: 10.7916/tohm.v0.698. PMID: 32195039Free PMC Article
Hirsch LE, Pringsheim T
Cochrane Database Syst Rev 2016 Jun 26;2016(6):CD009043. doi: 10.1002/14651858.CD009043.pub3. PMID: 27344135Free PMC Article
Kenney C, Kuo SH, Jimenez-Shahed J
Am Fam Physician 2008 Mar 1;77(5):651-8. PMID: 18350763
Taylor EA
Br J Psychiatry 1986 Nov;149:562-73. doi: 10.1192/bjp.149.5.562. PMID: 3545357

Therapy

Truong DD, Frei K
Parkinsonism Relat Disord 2019 Feb;59:146-150. Epub 2018 Nov 28 doi: 10.1016/j.parkreldis.2018.11.025. PMID: 30528171
Hirsch LE, Pringsheim T
Cochrane Database Syst Rev 2016 Jun 26;2016(6):CD009043. doi: 10.1002/14651858.CD009043.pub3. PMID: 27344135Free PMC Article
Kenney C, Kuo SH, Jimenez-Shahed J
Am Fam Physician 2008 Mar 1;77(5):651-8. PMID: 18350763
Schain RJ
Curr Probl Pediatr 1975 Aug;5(10):3-30. doi: 10.1016/s0045-9380(75)80007-7. PMID: 1101714
Barker P
J Child Psychol Psychiatry 1975 Apr;16(2):169-72. doi: 10.1111/j.1469-7610.1975.tb01266.x. PMID: 1055116

Prognosis

Nilles C, Hartmann A, Roze E, Martino D, Pringsheim T
Handb Clin Neurol 2023;196:457-474. doi: 10.1016/B978-0-323-98817-9.00002-8. PMID: 37620085
Lenka A, Jankovic J
Tremor Other Hyperkinet Mov (N Y) 2023;13:8. Epub 2023 Mar 28 doi: 10.5334/tohm.758. PMID: 37008994Free PMC Article
Plourde V, Boivin M, Brendgen M, Vitaro F, Dionne G
Dev Psychopathol 2017 Oct;29(4):1215-1226. Epub 2016 Dec 29 doi: 10.1017/S0954579416001255. PMID: 28031065
Hacker LE, Park JM, Timpano KR, Cavitt MA, Alvaro JL, Lewin AB, Murphy TK, Storch EA
J Atten Disord 2016 Jul;20(7):617-26. Epub 2012 Aug 24 doi: 10.1177/1087054712455845. PMID: 22923782
Ohba C, Shiina M, Tohyama J, Haginoya K, Lerman-Sagie T, Okamoto N, Blumkin L, Lev D, Mukaida S, Nozaki F, Uematsu M, Onuma A, Kodera H, Nakashima M, Tsurusaki Y, Miyake N, Tanaka F, Kato M, Ogata K, Saitsu H, Matsumoto N
Epilepsia 2015 Jun;56(6):841-8. Epub 2015 Apr 10 doi: 10.1111/epi.12987. PMID: 25864721

Clinical prediction guides

Wong LC, Huang CH, Chou WY, Hsu CJ, Tsai WC, Lee WT
Autism Res 2023 May;16(5):953-966. Epub 2023 Mar 21 doi: 10.1002/aur.2916. PMID: 36942618
Plourde V, Boivin M, Brendgen M, Vitaro F, Dionne G
Dev Psychopathol 2017 Oct;29(4):1215-1226. Epub 2016 Dec 29 doi: 10.1017/S0954579416001255. PMID: 28031065
Hacker LE, Park JM, Timpano KR, Cavitt MA, Alvaro JL, Lewin AB, Murphy TK, Storch EA
J Atten Disord 2016 Jul;20(7):617-26. Epub 2012 Aug 24 doi: 10.1177/1087054712455845. PMID: 22923782
Ohba C, Shiina M, Tohyama J, Haginoya K, Lerman-Sagie T, Okamoto N, Blumkin L, Lev D, Mukaida S, Nozaki F, Uematsu M, Onuma A, Kodera H, Nakashima M, Tsurusaki Y, Miyake N, Tanaka F, Kato M, Ogata K, Saitsu H, Matsumoto N
Epilepsia 2015 Jun;56(6):841-8. Epub 2015 Apr 10 doi: 10.1111/epi.12987. PMID: 25864721
Mulligan A, Anney R, Butler L, O'Regan M, Richardson T, Tulewicz EM, Fitzgerald M, Gill M
Child Care Health Dev 2013 Mar;39(2):202-12. Epub 2011 Dec 14 doi: 10.1111/j.1365-2214.2011.01345.x. PMID: 22168816Free PMC Article

Recent systematic reviews

Hirsch LE, Pringsheim T
Cochrane Database Syst Rev 2016 Jun 26;2016(6):CD009043. doi: 10.1002/14651858.CD009043.pub3. PMID: 27344135Free PMC Article
Evans J, Seri S, Cavanna AE
Eur Child Adolesc Psychiatry 2016 Sep;25(9):939-48. Epub 2016 Feb 15 doi: 10.1007/s00787-016-0823-8. PMID: 26880181Free PMC Article
Pietracupa S, Bruno E, Cavanna AE, Falla M, Zappia M, Colosimo C
J Neurol Sci 2015 Nov 15;358(1-2):9-21. Epub 2015 Sep 2 doi: 10.1016/j.jns.2015.08.1544. PMID: 26428309
Ching H, Pringsheim T
Cochrane Database Syst Rev 2012 May 16;(5):CD009043. doi: 10.1002/14651858.CD009043.pub2. PMID: 22592735
Coulter MK, Dean ME
Cochrane Database Syst Rev 2007 Oct 17;(4):CD005648. doi: 10.1002/14651858.CD005648.pub2. PMID: 17943868

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