Hypotonia

Major areas of difficulty for children w/ hypotonia ( based on MOVEMENTS )

	Presentation
Motion	↓ → poor antigravity
Orientation	↓ → poor antigravity + stability + orientation/ poor protection
Variety	↓ → small repertoire of movements → movements may be possible but seldom used
Ease	↓ → poor endurance + effort + generally unrestricted movement
Modulation	↓ → low muscle recruitment
Effectiveness	↓ → variable in relation to effort + endurance
Normality	Delayed  Antigravity skill Quality of movements
Timing	↓  Reaction time  Movement time
Safety	↓ Equilibirum  Protection

Non motor impairments for hypotonia ( cognitive + CV + pain )

Aspect	Presentation	Mx
Cognitive	Intellectual impairment due to genetic condition causing concomitant hypomobility
Cardiorespiratory	Cardiac problems due to specific genetic condition  Muscles of rib cage for breathing also affected by hypomobility	Monitor + control physical demands of therapy
Pain	Lack of joint stability → pain after physical activity

Non motor impairments for hypotonia ( hypermobility + GI + communication )

Aspect	Presentation	Mx
Hypermobility	Higher risk of joint degeneration + pain  → hypermobility is associated w/ hypotonia	Be aware that resting positions  → avoid damaging positions
Gastrointestinal	Hypotonia + associated hypermobility  → generalised connective tissue impairments  → prolaspe	Be aware of tasks involving exertion
Communication + feeding	Oral + facial hypotonia → eating + speaking + swallowing affected	Speech + language pathologist to improve posture + movement

Expected development of children w/ hypotonia

Delayed 

Esp 

1. Antigravity skills e.g. learning to sit/ crawl/ stand/ walk 

2. Quality of fine motor + object control skills

Aetiology for down syndrome

Presence of extra copy of chromosome 

Commonly: 

1. Extra copy of chromosome 21 

Less commonly: 

1. Combination of normal chromosomes + trisomic cells → milder presentation ? 

2. Chromosome translocation

Signs + symptoms for Down syndrome

Height as children + adults 

1. Shorter

Facial + cervical: 

1. Almond shaped eyes slanting up

2. Low nasal bridge

3. Small ears

4. Tongue sticking out of mouth

5. Flattened occiput

6. Shorter neck 

MSK: 

1. Low muscle tone 

2. Loose joints

Extremities: 

1. Small hands + feet 

2. Small pinky fingers curving towards thumb

Signs + symptoms for CV + respiratory + digestive + metabolic + immunological + sensory

	Presentation
Sensory	Impairments:  Visual: myopia + refractive errors + cataracts  Hearing: otitis media
CV	Congenital heart disease  Ventricular septal defect  Pulmonary hypertension  Atherosclerosis
Respiratory	Recurrent respiratory infection  Aspiration  Sleep related breathing disorders
Digestive	Atresia of oesophagus/ duodenum/ small bowel  Small bowel obstruction  Imperforate anus  Hirschprung disease
Immunological	Leukemia  Respiratory infections
Metabolic	Hypothroidism  Low basal metabolic rate

Prader Willi syndrome aetiology

Genetic conditions

Presentation of Prader Willi syndrome

Consistently: 

1. Hypothalamic pituitary dysfunction 

→ presentation: 

1. Involuntary urge to eat 

2. Low caloric needs 

3. Low metabolism

→ rapid weight gain + obesity 

MSK:

1. Low bone density 

2. Severe hypotonia 

Impairments of other body systems of Prader Willi syndrome

1. Hypogonadism 

2. Daytime sleepiness 

3. Temp regulation difficulties 

4. ↓ pulmonary function 

5. Susceptibility to infection 

6. Strabismus 

7. Scoliosis

Mx strategies for hypotonia

1. More hands off approach 

→ conformity to support surface 

→ accept as much physical support as you provide

→ remove support to see what they are capable of + encourage towards achieving goals

Aspect of facilitation strategies + purpose

Aspect of facilitation strategies	Explanation
Arousal	↑ →  ↑ active tone
Approximation	Proprioceptive facilitation of muscle activation
Activity	↑ weight bearing ↑  muscle activation
Automatic	Apply automatic responses to drive ↑ tone
Augmentation	Light + intermittent handling to augment movement
Antigravity	Encourage antigravity movement for participation → can be very tiring bc of ↑ amount of voluntary muscle activation

Aspect of facilitation strategies ( arousal )

Aspect of facilitation strategies	Explanation
Arousal	Auditory:  Louder  Higher pitched voice Faster pace  Visual:  Brighter  ↑ Colours ↑ Movement + activity Vestibular:  Spinning  Jumping  Swinging  Running  Tactile:  Interesting contrasting textures

Approximation + activity strategies for hypotonia facilitation

Approximation + compression → drives muscle contraction →  improves joint stability + mobility 

Approximation: force through long bones into joint 

Compression: force through support surface when weight bearing 

But: 

1. ↑ fatigue after practicing antigravity activities 

→ plan session for “ just right “ challenge 

→ continue to pitch difficulty of activity for antigravity control + endurance w/ fatigue balance

Automatic + augmentation strategies for hypotonia facilitation

Aspect of facilitation strategies	Explanation
Automatic	Provide positive  Vestibular  Visual stimuli  → activate automatic righting reactions to drive active tone
Augmentation	Least amount of support offered within safety limits  Reduction of use of long term replacement strategies e.g. AFOs Provide opportunities for independent muscle activation

Antigravity strategies for hypotonia facilitation

Causes of difficulties w/ antigravity movements: 

1. Low arousal 

2. Fatigue 

3. Too much support given

Facilitate via

1. Engaging activities 

2. Min handling

Positions + environmental facilitation for hypotonia( supine + prone + head control )

	Body position facilitation	Environment/ equipment
Activity in supine	Encourage hand to knee  Hip position?  Moving out of supine
Head control	Start in positions w/ head control  Methods of holding + positioning of child
Activity in prone	Arm + leg positioning considerations	Wedge/ roll/ parent’s body for positioning

Body position facilitation + environment for movement exploration/ sitting independently/ getting out of sitting

	Body position facilitation	Environment/ equipment
Movement exploration	All forms of mobility  Rolling  Pivoting  Pushing	More stimulation environment  Mobility devices
Sitting independently	BoS + posture considerations  Allow all functional sitting styles	External support + ways to carry/ position child
Getting out of sitting	Leg position considerations  → encourage child to go to side instead of forwards	Parental assist

Body position facilitation + environment for getting into sitting/ standing/ moving while standing/ walking independently

	Body position facilitation	Environment/ equipment
Getting into sitting	Encourage trunk rotation  4 point kneeling → sit ?  Leg position considerations  Allow diff ways to move
Getting into standing	4 point kneel + half kneel → standing  Foot position considerations Allow diff ways to move	Height of surface ?
Move while standing	Encourage standing  Encourage trunk rotation  Foot position considerations
Walking independently	Encourage more time in standing	Mobility device