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Muscle Weakness in Children

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Muscle Weakness in Children


Ak a: Muscle Weak ness in Children, Motor Delay in Children, Pediatric Neuromuscular Disorder, Congenital
Neuromuscular Disorder, Gross Motor Delay, Motor Developmental Delay

See Also

Page Contents

Developmental Milestone
Developmental Coordination Disorder
Clumsiness in Children
Muscular Dystrophy

II. Epidemiology
A. Neuromuscular disorders are rare compared to other developmental disorders
1. Neuromuscular disorders occur in early childhood in only 1 in 1000
2. Contrast with Learning Disorders (1 in 15), Autism (1 in 88), Cerebral Palsy (1 in
303)

III. Precautions
A. Early diagnosis is preferred
1. Improved outcomes
2. Improved access to services and reduction in caregiver stress
3. Prompts genetic counseling
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IV. Screening
A. See Developmental Milestone
B. See Developmental Evaluation
C. Using standardized tests is critical to avoid missing cases
D. Bright Futures Forms
1. http://brightfutures.aap.org/tool_and_resource_kit.html
E. CDC Muscular Dystrophy
1. http://www.cdc.gov/ncbddd/musculardystrophy/

V. History
A. Birth history
1. Perinatal Trauma
2. Newborn disorders or signs
B. Family History
1. Weakness
2. Developmental delay

VI. Exam
A. See Developmental Evaluation
B. See Gross Motor Milestone
C. See Developmental Red Flags
D. Infants age 4-6 months
1. Assess for head lag with Pull-to-sit
2. Initiate early intervention if not meeting milestones
a. Head lag persists at 4 months or older
3. Reassess at one month after intervention, and if not meeting Gross Motor
Milestones
a. Obtain Creatinine phosphokinase (CPK)
b. Refer to pediatric neurology
E. Infants age 6-9 months
1. Assess for ability to achieve and maintain sitting position
2. Obtain Creatinine phosphokinase (CPK) and initiate early intervention if not meeting
milestones
a. Not sitting independently by 7 months
b. Not getting into a sitting position by 9 months
3. Reassess at one month after intervention, and if not meeting Gross Motor
Milestones
a. Refer to pediatric neurology
F. Children >12 months
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1. Assess gait and rise to standing


2. Initiate early intervention or physical therapy if not meeting milestones
a. Not walking by age 15 months
b. Not standing from supine without support by 18 months
3. Reassess at 18 months of age and if not meeting Gross Motor Milestones (or
regression)
a. Obtain Creatinine phosphokinase (CPK)
b. Refer to pediatric neurology
4. Other assessments or concerns at 18-24 months of age (and later) to prompt CPK
and referral
a. Walking or Running difficulties
b. Frequent falling
c. Difficulty standing from supine
i. Unable to stand when pulled upward
ii. Hands braced on knee for support when standing (Gowers Maneuver)

VII. Symptoms
A. Muscle cramps

VIII. Signs: General


A. Gross Motor Delay
1. Delayed crawling or walking
2. Decreased Muscle Strength
3. Decreased neck strength and ability to hold head upright (head lag)
4. Decreased muscle tone (floppiness)
5. Frequent falls
B. Other findings variably present
1. Fine motor delay
2. Cognitive delay
3. Incoordination (see Clumsiness in Children)

IX. Signs: Distinguishing central from peripheral cause


A. Central Nervous System cause (e.g. Cerebral Palsy)
1. Typically fixed deficits, with signs in neonatal period
2. Increased Deep Tendon Reflexes and Clonus
3. Spastic gait
4. Variable muscle tone
5. Toe walking (as with peripheral causes)
B. Peripheral Nervous System or neuromuscular cause (anterior horn of spinal cord, nerve,
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muscle)
1. Typically progressive worsening (often with regression from milestones)
2. Altered chest appearance may be present (bell-shaped)
3. Weak, blunted facial expressions
4. High arched Palate
5. Tongue fasciculations (esp. spinal muscular atrophy)
6. Decreased muscle tone
7. Decreased or absent Deep Tendon Reflexes
8. Hyperlordotic, Waddling Gait
9. Toe walking (as with central causes)

X. Differential Diagnosis
A. Central cause (e.g. Cerebral Palsy)
B. Neuromuscular disorder (e.g. Muscular Dystrophy)
C. Hypothyroidism
D. Congenital Heart Disease
E. Nutritional deficiency

XI. Labs: General


A. See CPK below
B. Thyroid Stimulating Hormone
C. Liver Function Tests
1. Serum Aspartate Aminotransferase increased (from muscle breakdown)
2. Serum Alanine Aminotransferasencreased (from muscle breakdown)

XII. Labs: Serum Creatinine Phosphokinase (CPK)


A. Normal CPK (24 to 180 U/L)
1. Primary Central Nervous System Disorder
2. Normal CPK does not exclude Muscular Dystrophy
B. Mildly increased CPK (180 to 3000 U/L)
1. Neuromuscular Causes
a. Spinal muscular atrophy
b. Neuropathy
c. Congenital Myopathy
2. Other causes
a. See Serum Creatinine Phosphokinase (CPK)
b. Recent Immunizations
c. Muscle Trauma
d. Viral infection
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3. Plan if in this range


a. Repeat in 2-3 weeks
b. Refer to pediatric neurology if still increased (esp. more than three times
normal)
C. Grossly increased CPK (3000 to 50,000 U/L)
1. Causes
a. Duchenne Muscular Dystrophy
b. Becker Muscular Dystrophy
2. Plan if in this range
a. Repeat in 1 week
i. Refer to pediatric neurology if still in same range
ii. Repeat in 2-3 weeks if <3000 U/L on recheck to exclude muscle
Trauma cause
D. Severely increased CPK (>50,000 U/L)
1. Admit for Acute Rhabdomyolysis
E. References
1. National Task Force for the early identification of childhood neuromuscular disorders
a. http://www.childmuscleweakness.org/index.php/clinical-evaluation

XIII. Imaging
A. Not typically indicated in isolated weakness
B. Indications for brain imaging: Suspected central nervous system cause
1. Increased muscle tone
2. Suspected Cerebral Palsy
3. Microencephaly
4. Macroencephaly
5. Neurocognitive delay or regression

XIV. Evaluation: Gross Motor Delay


A. Acutely ill patients (e.g. muscle pain, Dark Urine, respiratory distress or CPK>50,000)
1. Hospitalization
B. Central cause suspected (e.g. Cerebral Palsy)
1. Refer to pediatric neurology
2. Refer to early intervention
3. Consider imaging (consider discussing with pediatric neurology)
C. Peripheral Cause evaluation
1. See Developmental Evaluation
2. Perform history as above
3. Perform exam as above to direct initial approach
a. Refer to early intervention if indicated
b. Refer to pediatric neurology if indicated
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4. Obtain CPK if indicated


a. Interpret and manage as described above

XV. Resources
A. Neuromuscular Disorder videos (ChildMuscleWeakness.org)
1. http://www.childmuscleweakness.org/index.php/videos
B. National Task Force for the early identification of childhood neuromuscular disorders
1. http://www.childmuscleweakness.org/index.php/clinical-evaluation

XVI. References
A. Lurio (2015) Am Fam Physician 91(1): 38-44 [PubMed]

Images: Related links to external sites (from Bing)

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Related Studies (from Trip Database)


Developmental Medicine and Child Neurology

Open in New Window

23 Apr 2015

Muscle weakness in children with neurofibromatosis type 1.


(http://www.ncbi.nlm.nih.gov/pubmed/25913846)
FP Notebook

24 Jul 2015

Muscle Weakness in Children


http://www.fpnotebook.com/peds/Sx/MsclWknsInChldrn.htm

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Muscle Weakness in Children


(http://fpnotebook.com/Peds/Sx/MsclWknsInChldrn.htm)
PROSPERO

01 Jan 2016

Risk factors for weakness of the pelvic floor muscles: a systematic review
(http://www.crd.york.ac.uk/prospero/display_record.asp?src=trip&ID=CRD42016039891)
American Journal of Human Genetics

29 Jun 2016

Mutations in Complex I Assembly Factor TMEM126B Result in Muscle Weakness and


Isolated Complex I Deficiency.
(http://www.ncbi.nlm.nih.gov/pubmed/27374773)
British Thoracic Society

01 Jul 2012

Guidelines for the respiratory management of children with neuromuscular

Ontology: congenital neuromuscular disorder (C0596371)


Definition
(CSP)

diseases existing at birth and often before birth, which effect the motor neuron, peripheral
nervous system and certain muscular diseases; manifestations include muscle weakness,
fasciculation, atrophy, spasm, myokymia, hypertonic, hypotonia and myalgias.

Concepts

Congenital Abnormality (T019) , Disease or Syndrome (T047)

English

congenital neuromuscular disorder

Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Motor developmental delay (C1852607)


Concepts

Finding (T033)

English

Motor developmental delay

Spanish

Retraso en el desarrollo motor

Portuguese

Atraso de desenvolvimento motor

French

Retard du dveloppement moteur

Dutch

motorische ontwikkeling achterstand

Czech

Opodn motorick vvoj

Italian

Ritardo nello sviluppo motorio

German

Motorische Entwicklungsverzoegerung

Japanese

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Hungarian

Motoros fejlds kslekedse

Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Gross motor delay (C1855566)


Concepts

Finding (T033)

English

Gross motor delay

Spanish

Retraso en desarrollo motor grosero

Portuguese

Atraso do desenvolvimento do motor grosso

Dutch

grove motoriek vertraging

Italian

Ritardo motorio grossolano

French

Ralentissement moteur grossier

German

Grobmotorik verzoegert

Czech

Hrub motorick zpodn

Japanese

Hungarian

Nagy motoros kslekeds

Derived from the NIH UMLS (Unified Medical Language System)

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This page was written by Scott Moses, MD, last revised on 1/5/2015 and last published on 9/5/2016.

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