Week 4 (pt. 2): Neuromuscular Dysfunction

Cerebral Palsy (CP)

Non-progressive disorder of posture and movement caused by brain injury before, during, or after birth; most common permanent physical disability in childhood; associated with sensory, cognitive, and communication impairments

• types: spastic, dyskinetic, ataxic, and mixed

Cerebral Palsy (CP) → Signs

Delayed gross motor milestones, abnormal posture, persistence of primitive reflexes (Moro, tonic neck), poor head control, impaired cognitive ability, epilepsy, difficulty walking

Cerebral Palsy (CP) → Treatment & Nurs Care

physical & speech therapy, occupational therapy, education, recreation, surgery, meds, technical aids, mobilizing devices; early recognition enables early intervention

• nurs care: assess developmental delays, reinforce therapies, maintain skin integrity from immobility, prevent contractures, support feeding, encourage independence, emotional support for families.

Feeding a Child with Cerebral Palsy (CP)

requires special attention to posture, swallowing safety, and nutrition, due to poor oral-motor control, weak tongue and jaw muscles; risk for aspiration

• sit child at 90 degree angle, supporting head, neck and trunk

• stabilize the jaw to assist swallowing; don’t tilt head back

• use small amounts of food at a time

• use soft, blended, or semi-solid foods

Neural Tube Defects

Congenital failure of neural tube closure, leading to spinal or cranial malformations; may involve entire length or small area of neural tube

• types: anencephaly, spina bifida (occulta or cystica), myelodysplasia

Neural Tube Defects → Ancephaly

severe neural tube defect where cerebral structures and the cranial vault are absent; incompatible with life; facial features present but head appears flattened or malformed

Neural Tube Defects → Spina Bifida Occulta

hidden defect with no visible sac; usually asymptomatic but may cause bladder or bowel dysfunction b/c lower body doesn’t work

Neural Tube Defects → Spina Bifida Cystica

visible defect with external saclike protrusion; neuro deficits are minimal or absent

Neural Tube Defects → Myelodysplasia

any malformation of the spinal canal or cord

Myelomeningocele

disorder where neural tube fails to close anywhere along the spinal column; often in the lumbar or lumbosacral area

• diagnosis: prenatal

• manifestations: visible sac-like protrusion, loss of motor function below deficit, paralysis, sensory loss, bladder/bowel dysfunction

Myelomeningocele → Management + Nurs Care

• management: bowel control, management of GU function, musculoskeletal considerations, initial care

• nurs care: assessment, protect the myelomeningocele sac, postop care, family support and home care

• high risk of latex allergy due to repeated exposure via procedures

Spinal Muscular Atrophy (Type 1), Werdnig-Hoffman’s disease

known as Werdnig-Hoffman’s disease; most common paralytic form of floppy infant syndrome (congenital hypotonia) caused by degeneration of motor neurons (autosomal recessive disorder); most die before 2yr 

• manifestations: laying in “frog leg” position, feeding difficulties, hypotonic, absent DTRs, respiratory failure

• nurs care: respiratory support, positioning, and family education

Spinal Muscular Atrophy (Type 3), Kugelberg-Welander Syndrome

also known as Kugelberg-Welander Syndrome; pattern of muscular weakness

• manifestations: proximal muscle weakness (especially lower limbs), muscular atrophy, slow progressive

• nurs care: maintaining mobility, preventing contractures

Guillain-Barré Syndrome (GBS)

Acute autoimmune demyelinating polyneuropathy causing with progressive paralysis; also known as infectious polyneuritis; contains acute, plataeu and recovery phases; fatal when reaches respiratory 

• manifestations: ascending symmetric muscle weakness, sensory changes, respiratory muscle weakness, autonomic dysfunction, cranial nerve/facial involvement

• management: IVIG, supportive care

• nurs care: monitor respiratory function, prevent complications.

Tetanus (Lockjaw)

disorder characterized by muscle rigidity involving the masseter and neck muscles caused by presence of tetanus spores or vegetative forms of bacillus'; can enter via wound

• manifestations: headache, difficulty swallowing, facial muscle spasms, jaw stiffness, sweating, fever, rigid ab/limb muscles, tetany of respiratory muscles

• management: DTaP vaccination, wound care, muscle relaxants

• nurs care: monitor airway and minimize stimuli

Botulism

food poisoning resulting from ingestion of Clostridium botulinum toxin (often in honey or canned foods)

• symptoms: weakness, poor feeding, drooping eyelids, paralysis, drooling, breathing problems, CNS symptoms begin 12-36 hr post ingestion

• management: supportive care, antitoxin therapy

• nurs care: respiratory support, nutrition/hydration, neuro monitoring, prevent complications

Neuromusuclar Physiology → Spinal Nerves

extend from medulla oblongata to lower border of first lumbar vertebra; consist of segments lying within vertebrae

Spinal Cord Injury in Children

Often due to motor vehicle accidents or sports trauma; severity depends on injury level and degree of muscular relaxation at time of injury

• manifestations: loss of motor fucnction, loss of sensation, autonomic dysfunction, etc

• nurs care: respiratory/cardiac care, temp regulation, physical therapy, neurogenic bladder, remobilization, prevent skin breakdown, etc

Muscular Dystrophies

largest group of muscular disease in children consisting of genetic origin leading to increasing disability and deformity with loss of strength

• manifestations: gradual degeneration of muscle fibers, progressive weakness, wasting of skeletal muscles

Duchenne Muscular Dystrophy (DMD)

most severe common muscular dystrophy in children; X-linked inheritance; diagnosed via manifestations or DNA analysis/biopsy

• manifestations: muscle weakness appearing at 3-7yr, difficulty running or riding bike or climbing stairs, Gower sign (child to uses their hands and arms to go from sitting to stand)

• management: no cure

• nurs care: promote mobility & independence, growth & development, prevent complications/isolation, support child/family

Duchenne Muscular Dystrophy (DMD) → Complications

Contractures, scoliosis, infection, obesity, cardiopulmonary complications

Contractures

permanent shortening or tightening of a muscle, tendon, or joint that results in limited movement or deformity of the affected limb or body part; occurs when normal muscle elasticity is lost due to prolonged immobility, spasticity, or muscle imbalance