NSG 3107: Neuromuscular or Muscular Dysfunction

Cerebral palsy (CP)

Group of nonprogressive disorders of movement caused by lesion in various motor centers of developing brain; abnormal muscle tone and coordination; difficulty walking, feeding, swallowing, articulation of speech

Prenatal brain abnormalities, chorioamnionitis in utero, prematurity, hypoxic brain injury, severe hypoglycemia

What are some causes of cerebral palsy?

True

True or false? Cerebral palsy is the most common permanent motor disability of childhood

Spastic CP

Most common clinical type of CP; pyramidal; upper motor neuron muscle weakness; increased stretch reflexes, increased muscle tone, weakness

Dyskinetic CP

Extrapyramidal; involuntary irregular jerking movements; slow, twisting movements that usually involve trunk, neck, facial muscles; pharyngeal, laryngeal, oral muscles cause drooling

Ataxic CP

Extrapyramidal; wide-based gait, rapid & repetitive movements performed poorly, disintegration of movements of upper extremities when child reaches for objects

Mixed CP

Combination of spastic and dyskinetic CP

Diplegia

Classification of spastic CP

All extremities affected; lower more than upper

Quadriplegia

Classification of spastic CP

All 4 extremities involved; legs & trunk, mount, pharynx, tongue

triplegia

Classification of spastic CP

Three limbs involved

Monoplegia

Classification of spastic CP

One limb involved

Hemiplegia

Classification of spastic CP

Motor dysfunction on one side of body; upper extremity more affected than lower

Early signs of CP

Early hand preference (before 18 months), delayed/absent achievement of motor development, persistent primitive reflexes

False

True or false? Cerebral palsy is linked to intellectual impairment

Neural tube defects

Abnormalities that derive from the embryonic neural tube; largest group of congenital anomalies

Cranioschisis

Skull defect through which various tissues protrude

Exencephaly

Brain is totally exposed or extruded through an associated skull defect; fetus usually aborted

Anencephaly

If fetus with exencephaly survives, there is degeneration of the brain to a spongiform mass with no bony covering; incompatible with life usually beyond a few days

Encephalocele

Herniation of brain and meninges through defect in the skull producing a fluid-filled sac

Spina bifida

Aka rachischisis; fissure in spinal column that leaves meninges and spinal cord exposed

Spina bifida occulta

Type of spina bifida which is not visible externally and is seen as a dimple on the sacrum; also can manifest as port-wine angiomatous nevus, tufts of dark hair, soft, subcutaneous lipomas

Spina bifida cystica

Type of spina bifida that is visible; external sac-like protrusion; presents a meningocele or myelomeningocele

Meningocele

Type of spina bifida cystica; hernial protrusion of saclike cyst of meninges filled with spinal fluid

True

True or false? Meningocele is not associated with neurological deficit, which occurs in varying, often serious, degrees in myelomeningocele

Myelomeningocele

Type of spina bifida cystica; hernial protrusion of saclike cyst containing meninges, spinal fluid, and portion of spinal cord with its nerves; various degrees of neurological deficit

False (can occur anywhere along spinal column)

True or false? Myelomeningoceles occur only at the base of the spine

0.4 mg

How many milligrams of folic acid should the pregnant person be instructed to take per day (she does not have a history of fetuses with neural tube defects)?

3 months

How many months preconception should folic acid supplementation begin?

Foods rich in folic acid

Dark leafy greens, legumes, asparagus, citrus, nuts, seeds, eggs, liver

Latex

Children with SB are a high risk of developing a ______ allergy

Banana, avocado, kiwi, chestnuts

Foods that children with latex allergies can have cross-reactions with

Muscular dystrophies

Group of muscular diseases in children; all forms have a genetic origin with gradual degeneration of muscle fibers, progressive weakness, and wasting of skeletal muscles

True

True or false? All forms of muscular dystrophy are characterized by increasing disability and deformity with loss of strength

Duchenne Muscular Dystrophy (DMD)

Most severe and most common type of muscular dystrophy; absence of structural protein dystrophin found in muscle fiber membranes = progressive muscle degeneration and weakness; muscle cells go through cycles of deterioration and regeneration until the repair capacity is no longer sufficient and the fibers undergo irreversible degradation and are replaced by fat and connective tissue

X-linked recessive; 1/3 are fresh mutations

What kind of inheritance pattern does DMD follow?

Clinical manifestations of DMD

Waddling gait, frequent falls, Gower's sign, lordosis, enlarged muscles, profound atrophy in later stages, cognitive impairment, complications

Gower's sign

When asked to get up from sitting on floor, child will move hands on legs as though crawling up to the thighs and then assume a standing position

Pseudohypertrophy

Seen in DMD, muscles in calves, thighs, upper arms become enlarged from fatty infiltration and feel unusually firm/woody/rubbery

Maintain function in unaffected muscles for as long as possible

What is the primary goal of DMD management?