Pediatric Neuromuscular Disorders Lecture Notes
Key Topics
- Anatomy and physiology of the neuromusculoskeletal system in children
- Diagnostic and laboratory tests for neuromusculoskeletal disorders
- Pathophysiology of selected neuromusculoskeletal disorders
- Nursing process for infants and children
- Pharmacological therapies
- Role of healthcare team
Neuromuscular Anatomy and Physiology
- Neuro Development: Brain and spinal cord
- Primitive Reflexes:
- Rooting reflex (3 months)
- Suck reflex (2-5 months)
- Moro reflex (4 months)
- Tonic neck reflex (4 months)
- Palmar grasp (4-6 months)
- Stepping reflex (2 months)
Child vs Adult Differences
- Neuro Development:
- Myelination
- Primitive reflexes
- Muscular Development:
- Spontaneous vs purposeful movement
- Normal range of motion and muscle tone
- Brisk deep tendon reflexes in newborns
- 25% muscle mass (40% in adults)
- Rapid muscle development in adolescence
Neuromuscular Health History
- Past Medical History: Includes prenatal/pregnancy history
- Family History
- History of Present Illness
- Home Treatments
Neuromuscular Physical Assessment
- Rule out spinal injury before moving
- Vital Signs
- Inspection: LOC, symmetry, alignment, deformity, etc.
- Cranial Nerve, DTR, Primitive Reflexes, Sensory Function
- Auscultation
- Palpation: Tone and strength
Muscle Tone versus Strength
- Tone: Muscles at rest, muscle tension
- Hypotonia common in NM disorders
- Strength: Muscles not at rest, muscle contraction
Pediatric Coma Scale
- Assesses LOC (eye, motor, verbal responses)
- Scores:
- 3: Deep coma or death
- 15: Full awake and aware
- 13-15: None to mild dysfunction
- 9-12: Moderate dysfunction
- 3-8: Severe dysfunction
- Modified scale for intubated patients
Cranial Nerve Assessment
- Olfactory: Smell
- Optic: Visual acuity
- Oculomotor: Eye movement, pupil dilation
- Trochlear: Vertical eye movement
- Trigeminal: Facial expression, sensation, chewing, facial pain
- Abducens: Lateral eye movement
- Facial: Taste, facial expression
- Auditory: Hearing, balance
- Glossopharyngeal: Taste, swallowing
- Vagus: Gag reflex, parasympathetic regulation
- Accessory (spinal): Muscles in head, neck, shoulders
- Hypoglossal: Serves tongue muscle
Signs and Symptoms of Neuromuscular Disorders
- Weakness, poor cough, retained airway secretions
- Inability to lift extremities
- Muscle wasting, hypotonia
- Poor feeding/swallowing, failure to thrive
- Tachypnea, use of accessory muscles for respiration
- Recurrent infections, night sweats
Diagnostic Testing
- Lumbar Puncture
- Radiography: X-rays, fluoroscopy, myelography, CT
- Ultrasound, MRI Imaging
- Electroencephalography (EEG) & Electromyography (EMG)
- Nerve Conduction Velocity, Muscle Biopsy
- Metabolic and Genetic Testing
- Labs: CBC, CK, CRP, ESR
Specific Disorders
Cerebral Palsy (CP)
- Most common motor disability; non-progressive
- Types: Spastic, Dyskinetic, Ataxic, Mixed
- Screening & Diagnosis: Developmental monitoring/screening/evaluations
- Treatment: PT/OT/Speech, Assistive devices, Medication (Baclofen, Benzos, Botulinum toxin)
Muscular Dystrophy
- Inherited conditions causing muscle weakness/wasting
- Progressive; no cure
- 9 types, most common is Duchenne Muscular Dystrophy
- X-linked recessive disorder
- Treatment: Pharmacology (corticosteroids, antidepressants), PT, Assistive devices, Surgical intervention
Spina Bifida
- Neural tube defect
- Types: Spina bifida occulta, Meningocele, Myelomeningocele
Reye Syndrome
- Rare, serious condition affecting brain/liver
- No aspirin recommended
- Diagnosis: LFTs, ammonia, LP, liver biopsy, CT
- Treatment: Early recognition and supportive care
Brain Tumors
- 2nd most common cancer in children; cause often unknown
- Complications: Hydrocephalus, increased ICP
- Diagnosis: CT, MRI, PET scan, LP
- Treatment: Surgery, chemo, radiation
- Nursing Management: Pre-op/post-op care, managing adverse effects
Neuromuscular Pharmacology
- Anticonvulsants: Phenobarbital, Topiramate, Phenytoin, etc.
- Baclofen (Lioresal), Botulinum Toxin
- Corticosteroids: Dexamethasone, Prednisone
- NSAIDs: Ibuprofen, Ketorlac
Interdisciplinary Care
- Maximize potential, promote growth/development
- Promote mobility, maintain nutrition
- Prevent further injury
- Educate patient/family on ongoing conditions
- Encourage support groups
This summary captures the main points and topics covered in the lecture on Pediatric Neuromuscular Disorders, offering a comprehensive outline for further study and review.