Early motor repertoire and developmental function outcomes in infants with West syndrome: a case series.

BACKGROUND West syndrome (WS), also known as infantile spasm, is a rare form of severe epilepsy that begins during early infancy. This case series aimed to describe the early motor repertoire and examine the developmental function outcomes of infants with WS. CASE DESCRIPTIONS Three infants (one female) with WS were assessed for early motor repertoire using the General Movement Assessment (GMA) which determined General Movement Optimality Scores (GMOS) at 4 post-term weeks of age, and Motor Optimality Scores (MOS) at 12 post-term weeks of age. Cognitive, language, and motor development were evaluated with the Bayley Scales of Infant and Toddler Development - Third Edition (Bayley-III) at 3, 6, 12, and 24 months of age. OUTCOMES At 4-weeks post-term, one infant showed poor repertoire movements, while the other two showed cramped-synchronized movements with their GMOS ranging from 6 to 16 (out of 42). All infants showed sporadic/absent fidgety movements at 12 weeks post-term with their MOS ranging from 5 to 9 (out of 28). All sub-domain scores of Bayley-III were <2 SD at all follow-up assessments, that is <70, indicating severe developmental delay. CONCLUSION These infants with WS had less than optimal scores of early motor repertoire, and developmental delay at a later age. Early motor repertoire might be an early sign for developmental function outcome at a later age in this population suggesting the need for additional research.

[1]  B. Vollmer,et al.  Predictive validity of a qualitative and quantitative Prechtl's General Movements Assessment at term age: Comparison between preterm infants and term infants with HIE. , 2021, Early human development.

[2]  C. Ahuja,et al.  Long-term epilepsy control, motor function, cognition, sleep and quality of life in children with West syndrome , 2021, Epilepsy Research.

[3]  J. Shieh,et al.  Electroencephalography complexity in infantile spasms and its association with treatment response , 2020, Clinical Neurophysiology.

[4]  U. Ådén,et al.  Movements and posture in infants born extremely preterm in comparison to term-born controls. , 2020, Early human development.

[5]  A. Mutlu,et al.  General Movements and Developmental Functioning in an Individual with Rhizomelic Chondrodysplasia Punctata within the First Months of the Life: A Case Report , 2020, Physical & occupational therapy in pediatrics.

[6]  A. Polizzi,et al.  West syndrome: a comprehensive review , 2020, Neurological Sciences.

[7]  Matteo Porro,et al.  Early detection of general movements trajectories in very low birth weight infants , 2020, Scientific Reports.

[8]  Giovanni Cioni,et al.  Cerebral Palsy: Early Markers of Clinical Phenotype and Functional Outcome , 2019, Journal of clinical medicine.

[9]  J. Nasiri,et al.  Neurodevelopmental outcomes of the West syndrome in pediatric patients: The first report from the Middle-East. , 2019, Research in developmental disabilities.

[10]  Florian B. Pokorny,et al.  Early motor and pre-linguistic verbal development in Prader-Willi syndrome - A case report. , 2019, Research in developmental disabilities.

[11]  P. Malhi,et al.  Safety, tolerability, and effectiveness of oral zonisamide therapy in comparison with intramuscular adrenocorticotropic hormone therapy in infants with West syndrome. , 2019, European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society.

[12]  Hoon-Chul Kang,et al.  Vigabatrin and high-dose prednisolone therapy for patients with West syndrome , 2018, Epilepsy Research.

[13]  K. Kapur,et al.  Detailed Magnetic Resonance Imaging (MRI) Analysis in Infantile Spasms , 2018, Journal of child neurology.

[14]  M. Hadders‐Algra.  Neural substrate and clinical significance of general movements: an update , 2018, Developmental medicine and child neurology.

[15]  Sarah McIntyre,et al.  Early, Accurate Diagnosis and Early Intervention in Cerebral Palsy: Advances in Diagnosis and Treatment , 2017, JAMA pediatrics.

[16]  P. Marschik,et al.  The motor repertoire in 3- to 5-month old infants with Down syndrome , 2017, Research in developmental disabilities.

[17]  P. Marschik,et al.  The association between the early motor repertoire and language development in term children born after normal pregnancy. , 2017, Early human development.

[18]  C. Einspieler,et al.  Very low birth weight infants in China: the predictive value of the motor repertoire at 3 to 5 months for the motor performance at 12 months , 2016, Early human development.

[19]  Christa Einspieler,et al.  The general movement optimality score: a detailed assessment of general movements during preterm and term age , 2016, Developmental medicine and child neurology.

[20]  N. Badawi,et al.  Sensitivity and specificity of General Movements Assessment for diagnostic accuracy of detecting cerebral palsy early in an Australian context , 2016, Journal of paediatrics and child health.

[21]  P. Striano,et al.  Infantile spasms syndrome, West syndrome and related phenotypes: What we know in 2013 , 2014, Brain and Development.

[22]  Steven P. Miller,et al.  MRI findings in infants with infantile spasms after neonatal hypoxic-ischemic encephalopathy. , 2013, Pediatric neurology.

[23]  M. Ruggieri,et al.  West Syndrome Treatment: New Roads for an Old Syndrome , 2013, Front. Neurol..

[24]  L. Doyle,et al.  General Movements in Very Preterm Children and Neurodevelopment at 2 and 4 Years , 2013, Pediatrics.

[25]  R. Ware,et al.  A systematic review of tests to predict cerebral palsy in young children , 2013, Developmental medicine and child neurology.

[26]  S. Moshé,et al.  Treatment outcomes of West syndrome in infants with Down syndrome. , 2013, Pediatric neurology.

[27]  P. Marschik,et al.  Cerebral palsy in children: Movements and postures during early infancy, dependent on preterm vs. full term birth , 2012, Early human development.

[28]  P. Marschik,et al.  Early behavioural manifestation of Smith-Magenis syndrome (del 17p11.2) in a 4-month-old boy , 2012, Developmental neurorehabilitation.

[29]  William B Dobyns,et al.  Genetic and biologic classification of infantile spasms. , 2011, Pediatric neurology.

[30]  Andrea Ranzi,et al.  General movements in full-term infants with perinatal asphyxia are related to Basal Ganglia and thalamic lesions. , 2011, The Journal of pediatrics.

[31]  R. Scott,et al.  Seizure outcome in infantile spasms—A retrospective study , 2011, Epilepsia.

[32]  P. Marschik,et al.  Movements and postures of infants aged 3 to 5 months: to what extent is their optimality related to perinatal events and to the neurological outcome? , 2011, Early human development.

[33]  L. Lagae,et al.  Treatment and long term outcome in West syndrome: The clinical reality. A multicentre follow up study , 2010, Seizure.

[34]  J. H. Cross,et al.  Revised terminology and concepts for organization of seizures and epilepsies: Report of the ILAE Commission on Classification and Terminology, 2005–2009 , 2010, Epilepsia.

[35]  A. Fois.  Infantile spasms: review of the literature and personal experience , 2010, Italian journal of pediatrics.

[36]  G. Cioni,et al.  Early motor repertoire is related to level of self‐mobility in children with cerebral palsy at school age , 2009, Developmental medicine and child neurology.

[37]  A. Bouma,et al.  The quality of preterm infants' spontaneous movements: an early indicator of intelligence and behaviour at school age. , 2009, Journal of child psychology and psychiatry, and allied disciplines.

[38]  Lars Adde,et al.  Inter-observer reliability of the "Assessment of Motor Repertoire--3 to 5 Months" based on video recordings of infants. , 2009, Early human development.

[39]  C. Einspieler,et al.  Abnormal general movements in girls with Rett disorder: The first four months of life , 2005, Brain and Development.

[40]  Christa Einspieler,et al.  Prechtl's assessment of general movements: a diagnostic tool for the functional assessment of the young nervous system. , 2005, Mental retardation and developmental disabilities research reviews.

[41]  L. Mazzone,et al.  The General Movements in children with Down syndrome. , 2004, Early human development.

[42]  Mijna Hadders-Algra,et al.  General movements: A window for early identification of children at high risk for developmental disorders. , 2004, The Journal of pediatrics.

[43]  R. Riikonen.  Topical Review: Infantile Spasms: Therapy and Outcome , 2004 .

[44]  James D Frost,et al.  Infantile Epileptic Encephalopathy with Hypsarrhythmia (Infantile Spasms/West Syndrome) , 2003, Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society.

[45]  Giovanni Cioni,et al.  Cramped synchronized general movements in preterm infants as an early marker for cerebral palsy. , 2002, Archives of pediatrics & adolescent medicine.

[46]  M. Yeargin-Allsopp,et al.  The Descriptive Epidemiology of Infantile Spasms Among Atlanta Children , 1999, Epilepsia.

[47]  R. Riikonen,et al.  Incidence and Aetiology of Infantile Spasms from 1960 to 1976: a Population Study in Finland , 1979, Developmental medicine and child neurology.