Congenital Neurologic Disorders

Hydrocephalus

Common condition of excess CSF accumulation in the skull

• Ventricles dilate, and the brain and blood vessels become compressed —> severe brain damage

Noncommunicating : obstructive(blocked) CSF flow distribution(can’t drain)

Communicating: CSF is not properly absorbed by blood

Hydrocephalus

• when may it develop

• Risk factors

• may be present at birth or develop later

prematurity, pregnancy complications and congenital defects(esp. NS), NS tumors, CNS infections, cerebral hemorrhage, head injuries, familial link

• fatal if left untreated

Hydrocephalus Manifestations

• infants vs older children and adults

infants:

• unusually large head, rapid increase in head size, bulging fontanelle(anterior closes at 12- 18 months, posterior close at brith)

• vomiting(projectile)

• lethargy, irritability, high pitched cry

• seizures, eyes gaze down

• developmental delay, feeding difficulties

Adults:

• headache followed by N/V

• blurred vision/diplopia, sluggish pupil to light, eyes gaze downwards

• uncoordinated movements

• memory loss; confusion

• urinary incontinence

• irritability and personality changes

• impaired performance at school and work

Spina Bifida

Most common neural tube defects (NTDs) in the United States(NT closes in 4 weeks gestation)

• vary in severity(mild to debilitating)

The posterior spinous processes on the vertebrae fail to fuse, permitting the meninges and spinal cord to herniate

• lumbar area of the vertebrae is most commonly the site

Cause of Spina bifida

• complications

Exact cause unknown; genetics and environmental(folate deficiency) factors

• Most common in caucasian and Hispanic populations

• Additional maternal risk factors: fx of neural defects, folate deficiency, meds(anti-seizure), DM, pre-pregnancy obesity, inc. body temp(hot tub, sauna, tanning beds)

Complications—> physical and neurologic impairments, hydrocephalus, meningitis, and learning problems

Spina Bifida Oculta

mildest form

results in a small gap in one or more vertebrae

• spinal nerves and meninges do not protrude through the opening

• no clinical manifestations or neurologic deficits

• looks like a dimple on top of the butt, or a tuft of hair

Meningocele

rare form that involves the same bony defect as oculta, but the meninges protrude through the vertebral opening

• meninges and CSF form a sac on the back(can’t see spine)

• spinal cord develops normally—>no neurologic impairment or paralysis

The membranes can be removed by surgery with little or no damage to nerve pathways • Infection or rupture of the sac can lead to neurologic impairment

Myelomeningocele (open spina bifida)

most severe form

• spinal canal remains open along several vertebrae in the lower or middle back

  • MENINGES, SPINAL CORD, SPINAL NERVES, AND CSF PROTRUDE THROUGH THE LARGE OPENING AT BIRTH, forming a sac on the back

  • Sometimes skin covers the sac

• Tissues and nerves exposed sometimes—> more vulnerable to infections

Complications: neurologic impairment(often including paralysis), bowel and bladder problems, seizures, and other medical complications

Cerebral Palsy

Non-progressive; appears in infancy or early childhood; permanently affects motor movement and muscle coordination

• Damage to the cerebellum(ballerina-balance) during the prenatal period(childbirth), but can occur any time during the first 3 years of life

• most common in males, African Americans, people of lower SES

Cerebral Palsy Contributing factors

PREMATURE, low birth weight, breech birth, multiple fetuses, HYPOXIA, maternal or fetal hypoglycemia, maternal infections during pregnancy(RUBELLA AND VARICELLA), maternal exposure to toxins(mercury), SEVERE JAUNDICE, cerebral hemorrhage, neurologic infections, head injury

Cerebral Palsy Manifestations

• may or may not see at birth(might not see until they start missing milestones)

• may affect the entire body or one area; one side or both sides

Spasticity(stiffening of muscles), uncontrolled movement, ataxia(issues with balance and coordination), developmental delays

• also: early reflexes, flaccidity, hyperreflexia, asymmetrical walking gait, unusual position of limbs when resting or when held up, excessive drooling, difficulty swallowing, impaired sucking, speech issues, facial grimaces, tremors

CP complications

• balance and coordination issues

• scoliosis

  • atrophy below the waist

• malnutrition

• contracture

• learning or cognitive difficulties

• seizures

• vision and hearing issues

• urinary incontinence, constipation

• osteoporosis

• chronic pain

  • injury

speech therapy, occupational therapy, neurologist, physical therapy