Section I: Evidence Summary |
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Hypotonia can be defined as abnormally low muscle tone, or reduced resistance to passive, relatively rapid movement. The imprecision of the definition reflects the lack of psychometric properties and reliability of assessments for hypotonia, therefore only clinical definitions currently in use by neurology specialists will be used in this pathway. Other terms for hypotonia include, but are not limited to, central hypotonia, floppy baby syndrome, benign congenital hypotonia, and neonatal hypotonia.
Hypotonia may originate from disturbances in the physiology of central or peripheral nervous systems or of the end organs themselves (muscles and muscle groups). The current pathway will only address children whose hypotonia is centrally-mediated and will exclude those whose hypotonia can be attributed conclusively to peripheral causes. Spinal Muscular Atrophy (SMA) is not included within the definition of centrally mediated hypotonia. Centrally-mediated hypotonia will be further referred to in this document as “central hypotonia” in the interest of simplicity. Specific etiologies of central hypotonia include brain insults and malformations, as well as genetic, metabolic, traumatic, anatomical, or idiopathic causes of central neural dysfunction.
Central hypotonia may be generalized and affect the limbs, trunk and neck or may be localized such that specific areas of the body are predominantly hypotonic with others having normal or hypertonic characteristics. Hypotonia is often seen in combination with muscle weakness. In the case of perinatal insults to white matter tracts, such as in encephalopathy of prematurity and neonatal encephalopathy, central hypotonia can evolve over the course of the first few years of life and progress to hypertonia. In addition, central hypotonia can co-exist with abnormalities of movement (such as ataxia or dyskinesia) or sensation (dysethesias, paresthesias).
Infants and young children with diagnoses of Down syndrome (DS), Cerebral Palsy (CP), and/or developmental delay (DD) often present with low muscle tone that can influence their gross motor development. Other children presenting to therapists may have no established diagnoses.
Children from birth to six years-of-age with central hypotonia and gross motor delays that limit activity and participation.
Therapists/Early Interventionists caring for children from birth to six years-of-age with central hypotonia and their families.
If the child has not already been seen for a diagnostic work-up, referral to a neurologist, geneticist and/or developmental medical specialist is always recommended. During the therapy assessment, determine the impact of the hypotonia on function, activity limitations and participation restrictions, pain/comfort (including sleep), care-giving and whether management is required. Assess whether the clinical presentation is consistent with infants at “high risk of CP” (see Early Detection Guidelines, JAMA Pediatrics Novak et al. 2017).
Therapy Assessment: Children with hypotonia may have delays in motor development. It is recommended that therapists use valid and reliable measures of motor abilities. As there are no established evidence-based approaches to measure hypotonia, and as the focus of therapy should be on improved functioning, we recommend the following: use motor function assessments with good psychometric properties for infants at high risk for motor delays and neuromotor problems such as: the Hammersmith Infant Neurological Examination (HINE, 3 to 24 months), the Test of Infant Motor Performance (TIMP, term to 3 months), Peabody Developmental Motor Scales (PDMS), Development Assessment of Young Children (DAYC-2) Motor Scale, the Alberta Infant Motor Scale (AIMS, 0-18 months), Brigance III, etc. (See Early Detection Guidelines for CP, JAMA Pediatrics Novak et al., 2017). A broader perspective on promoting child development is offered by the ‘F words in childhood disability’ (Rosenbaum & Gorter, 2012)
Goal Setting: Use valid and reliable outcome measures outcomes such as Canadian Occupational Performance Measure (COPM), Goal Attainment Scaling (GAS) (see Section III for further details).
Most of the interventions in the Central Hypotonia Care Pathway have low or very low levels of evidence (based on GRADE ratings).
Developmental Strategies: strategies used by physiotherapists (PTs), occupational therapists (OTs) and early interventionists are considered cornerstones in the management of central hypotonia. General principles include:
(for details on each intervention including potential risks, see the evidence summary in Section II):
Central Hypotonia Evidence for Interventions - GRADE ratings
Click on the links for free full text.
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A. Hypotonia Wheel and Guide |
B. Clinical Assessment of Hypotonia Tools:
C. Goal Setting Tools:
D. Diagnostic Algorithms:
Stakeholder Consultation:
Three families with children aged between 3 months and 6 years-of-age with varying degrees of hypotonia reviewed the pathway and provided written feedback that was incorporated into the care pathway.
Expert Consensus Team:
| Name | Affiliation(s) and specialty | Location | Role |
|---|---|---|---|
| Ginny Paleg, PT, MPT, DScPT | Montgomery County Infants and Toddlers Program Physical Therapist |
Rockville, Maryland, USA | Team Leader, Author and Consensus Expert Author of Systematic Review |
| Roslyn Livingstone, MSc(RS) OT | Sunny Hill Health Centre for Children Occupational Therapist |
Vancouver, British Columbia, Canada | Author and Consensus Expert Author of Systematic Review |
| Elisabet Rodby-Bousquet, PT, PhD | Centre for Clinical Research, Uppsala University Associate Professor Lund University and Physical Therapist |
Västerås, Sweden | Author and Consensus Expert |
| Maureen Story, BSR PT/OT | Sunny Hill Health Centre for Children Occupational and Physical Therapist |
Vancouver, British Columbia, Canada | Co-Author of Hypotonia Tool Kit and Wheel and Consensus Expert |
| Nathalie L Maitre, MD, PhD | Nationwide Children’s Hospital Associate Professor of Pediatrics and Neonatologist | Columbus, Ohio, USA | Author and Consensus expert |
| Pragashne (Naidoo) Govender, PhD | School of Health Sciences, University of KwaZulu-Natal Associate Professor and Occupational Therapist |
Durban, South Africa | Consensus Expert |
| Mark Romness, MD | Department of Orthopedic Surgery University of Virginia Associate Professor and Pediatric Orthopedic Surgeon |
Charlottesville, VA, USA | Consensus Expert Author of Systematic Review |
| Christopher Lunsford, MD | Department of Orthopedic Surgery and Department of Pediatrics Duke University Pediatric Physiatrist |
Durham, NC, USA | Consensus Expert |
| Garey Noritz, MD | Nationwide Children’s Hospital Division Chief of the Complex Health Care Program Professor of Pediatrics and Ohio State University Developmental Pediatrician and Internist |
Columbus, OH, USA | Consensus Expert |
| Julia Looper, PT, PhD | University of Puget Sound Associate Professor of Physical Therapy and Physical Therapist |
Tacoma, WA, USA | Consensus Expert |
| Kathy Martin, PT, DHSc | University of Indianapolis Krannert School of Physical Therapy Professor, DPT Program Director and Physical Therapist |
Indianapolis, IN, USA | Consensus Expert |
| Lourdes Macias, PT, PhD, MSc | Universitat Internacional de Catalunya Early Intervention Public Department, President of the Spanish Pediatric Physical Therapy Association (SEFIP), Professor and Physical Therapist |
Barcelona, Spain | Consensus Expert |
| Sharon Eylon, MD | ALYN Rehabilitation Hospital for Children and Adolescents Head of Orthopedic Services Pediatric Orthopedic Surgeon |
Jerusalem, Israel | Consensus Expert |
The American Academy for Cerebral Palsy and Developmental Medicine has developed care pathways to assist the busy clinician. Please submit any advice or constructive feedback to make this pathway more useful.
NOTE: Feedback will be directed to the AACPDM Care Pathway Taskforce to review and consider on a queue 6-month basis.
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