Hehi Test #2 - Congenital Neurological Disorders, Cerebral Palsy And Down Syndrome

Front 1

Neural Tube

Back 1

1. Closes between 15-28 days post conception
2. Failure of normal closure results in neural tube defects
3. spina bifida and anencephaly make up 90% of NTD
4. 1:5000 live births

Front 2

Anencephaly

Back 2

Both cerebral hemispheres absent - incompatible with life

Front 3

Spina Bifida

Back 3

1. Failure of body spine to close
2. Occurs most often in lumbar and sacral areas

Front 4

Spina Bifida Occulta

Back 4

Failure of vertebrae to close without protrusion of spinal cord or meninges
*cutaneous manifestations may or may not occur
*Neuromuscular disturbances - most often asymptomatic/ no consequences

Front 5

Spina Bifida Cystica

Back 5

Visible defect with saclike protrusion
1. Meningocele- rare, contains meninges and CSF only
2. Myelomeningocele - contains meninges, CSF, spinal cord, and/ or nerve roots

Front 6

Risk factors for having a baby with SB

Back 6

1. Previous pregnancies with NTD
2. Folic acid deficiency
3. girls > boys
4. Caucasian > African American
5. Diabetes in mom (gestational , type I or type II)
6. Obesity
7. Anticonvulsants - Valproic acid
8. Materna heat exposure - hot tubs and baths

Front 7

Folic Acid

Back 7

ALL WOMEN SHOULD TAKE A FOLIC ACID MULTI VITAMIN BECAUSE MANY PREGNANCIES ARE NOT PLANNED!! daily recommendation: 0.4 mg
4 mos. before pregnancy: 4 mg

Front 8

Assessment of Myelomeningocele

Back 8

1. Degree of neuro impairment - usually loss of sensation and complete/ partial paralysis
2. Hydrocephalus - most frequent anomaly associated with SB = increased intracranial pressure

Front 9

Effects of SB

Back 9

1. Bladder - decrease nerve supply - overflow incontinence or constant trickling (at risk for UTI, kidney infection, renal failure)
2. Anal sphincter - lack of bowel control
3. Orthopaedic abnormalities
4. Spinal deformities

Front 10

Management of SB

Back 10

1. Initial care - prevention of infection (sterile moist dressing over sac)
2. Neuro assessment - daily head circumference
3. surgical closure 24-72 hours after delivery
4. Basic needs of infant

Front 11

Initial care of SB

Back 11

1. Radient warmer
2. Prone position
3. Sterile moist dressing over defect - don't want sac to dry out
4. Inspect sac closely
5. Observe for s/s hydrocephalus

Front 12

S/S of increased intracranial pressure (infant)

Back 12

1. bulging fontanels
2. high pitched cry (neuro cry)
3. bulging scalp veins

Front 13

General care of SB

Back 13

1. GU function - in/out cath. q4h
2. Bowel control - diet modification, reg. toilet habits, ACE procedure
3. Tactile stimulation
4. Latex allergy

Front 14

Care plan for SB

Back 14

1. At risk for sensory deficit r/t postitioning s/t MM sac
2. At risk for infection r/t non-epithelial meningeal sac s/t MM
3. Nutrition less than body requirements r/t decreased intake s/t prone positioning or increased ICP
4. At risk for urinary retention/ UTI/ r/t neurogenic bladder s/t MM
5. Falls always! At risk for injury r/t neuromuscular impairment s/t MM

Front 15

Hydrocephalus

Back 15

Increased acumulation of CSF within ventricles of the brain
- congenital
- acquired

Front 16

Communicating Hydrocephalus

Back 16

Impaired absorption

Front 17

Non-communicating

Back 17

Impaired/ obstructed flow of CSF through ventricle system

Front 18

Clinical Manifestation of Hydrocephaly (infant)

Back 18

1. Bulging fontanel
2. Dilated scalp veins
3. Head growth at abnormal rate
4. Frontal bossing - protrusion of frontal bone
5. Setting sun eyes
6. Irritable
7. Lethargic
8. Poor feeding
9. Change in LOC
10. Paradoxical crying - crying when picked up
11. high pitched cry (neuro cry)

Front 19

Clinical Manifestations of Hydrocephaly (child)

Back 19

1. irritable
2. Lethargy
3. Confusion
4. Apathetic
5. Increased BP
6. Decreased pulse
7. Decreased RR
8. Irregular RR
9. Headache
10. Ataxia

Front 20

Diagnosis of Increased intracranial pressure

Back 20

1. Antenatal - fetal ultrasound (14 weeks)
2. Increasd head circumference
3. Associated neurological signs
4. Primary diagnostic tools - CT and MRI

Front 21

Management of Hydrocephaly

Back 21

1. Shunt procedure - provides primary drainage of CSF from ventricles to extracranial compartment usually peritoneum

Front 22

Complications of VP shunt

Back 22

1. Infection - greatest risk 1-2 months after placement, may be sepsis or wound infection
2. Malfunction - usually mechanical obstruction
3. Displacement for growth

Front 23

S/S of VP shunt infection - treated with antibiotics

Back 23

1. Shunt malfunction (increased ICP)
2. Fever
3. Wound/shunt inflammation
4. Abdominal pain

Front 24

S/S shunt malfunction in infants

Back 24

1. Bulging fontanel
2. swelling along shunt tract
3. Prominnt veins on scalp
4. Downward deviation of the eyes
5. Vomiting/ change in appetite
6. Irritability or drowsiness
7. Seizures
8. High pitched cry

Front 25

S/S of shunt malfunction in toddlers

Back 25

1. Headache
2. Vomiting/ Change in appetite
3. Lethargy/ Irritability
4. Swelling along shunt tract
5. Seiaures

Front 26

S/S of malfunction in older child

Back 26

Same as toddler plus:
1. Deterioration in school performance
2. Neck pain/ pain over MM site
3. Personality
4. Decrease in sensory and motor function of motor function

Front 27

Post op care for VP shunt

Back 27

1. Observe for s/s of shunt infection and malfunction
2. Position flat on un-operated side
3. Inspect operative sire frequently
4. Skin care - pressure of head
5. Family support - fear
6. Family/ patient teaching

Front 28

Prognosis of hydrocephaly

Back 28

50-60% mortality in untreated; survivors with high risk of incidence of subnormal intellectual capacity; 90% survival in those who receive surgical treatment; 2/3 are intellectually normal

Front 29

Bacterial Meningitis

Back 29

Epidemiology - HIB vaccine 1990; 1987 - 41 cases in 100K; 1993 - 2 cases per 100K; 1996 near illimination

Front 30

Etiology of Bacterial meningitis in neonates and those over 2 months of age

Back 30

neonates - H flu, strep pneumoniae, nisseria meningitidis (menningococcus)
Over 2 months of age - GBS, e. coli, listeria

Front 31

Pathophysiology of bacterial meningitis

Back 31

Most common route of infection - vascular dissemination from a focus of infection elsewhere
2. direct entry of organisms - post LP or surgical procedures
3. Anatomic abnormalities - spina bifida
4. Foreign body - VP shunt

Front 32

Clinical Manifestation of bacterial meningitis in children/ adolescents

Back 32

*Can be abruspt of gradual onset - fever, chills, headache, vomiting, change in sensorium, irritable, agitated, photophobia, confusion, hallucinations, drowsiness, stupor, coma
* Nuchal rigidity
* Kernig's sign - reflex of leg to body
* Brudzinski sign - positive - flex hips to relieve nuchal rigidity
* Meningococcal - purpuric/ petechial rash

Front 33

Clinical Manifestation of Meningitis in 3 mos. - 2 yrs.

Back 33

1. Bulging fontanel (most significant sign)
2. fever
3. poor feeding
4. vomiting
5. Irritablity
6. high pitched cry

*Nuchal rigidity, Brudzinski, and Kernig's not consistently present in children less than 18 mos.

Front 34

Clinical manifestations of maningitis in neonates

Back 34

1. poot feeding
2. poor sucking
3. poor muscle tone
4. hypothermia/ fever
5. respiratory irregularities
6. Apnea
7. Weight loss
8. irritability
9. Drowsiness
10. Bulging fontanel (may/ may not appear)

*Neck is usually supple

Front 35

Diagnosis of Bacterial meningitis

Back 35

1. Lumbar puncture
a. spinal fluid pressure
b. WBC: elevated
c. Protein concentration: increased
d. Glucose level: decreased
e. Gram stain: poitive (2 days)

Front 36

Complications of bacterial meningtis

Back 36

1. Obstructive hydrocephalus
2. Deafness, blindness, paralysis or facial muscles
3. Cerebral Palsy
4. Mental handicap
5. Learning diorder
6. ADHD
7. Seizures
*Greatest morbidity between birth and 4 yrs.

Front 37

Management of bacterial meningitis

Back 37

1. Isolation
2. Antimicrobial therapy
3. Maintain hydration
4. Ventilation
5. Decrease increased ICP
6. Manage systemic shock
7. Control seizures
8. Control temp.
9. treat complications

Front 38

Nursing care for meningitis

Back 38

1. Environment - sensitivity to light/ sound
2. Position of choice
3. Pain evaluation
4. Safety - seizure precautions
5. Frequent assessment: VS, neuro checks, LOC, I/O's
6. Family support

Front 39

Spinal tap result for viral meningitis

Back 39

1. elevated WBC
2. Protein: WNL or slight increase
3. Glucose: WNL
4. Color: slightly cloudy/ clear
5. Gram stain: negative

Front 40

Treatment for viral meningitis

Back 40

Symptomatic - hydration, pain management, positioning

*clinical manifestations: HA, fever, malaise, GI symptoms

Front 41

AAMR definitionof mental retardation

Back 41

Based on 3 diagnostic criteria:
1. Intellectual functioning
2. Functional strengths/ weaknesses - impairment in 2/10 adaptive skills domains (communication, self care, home living, social, community, self direction, health/ safety, functional academics, leisure, work
3. Onset before 18 years of age

Front 42

Standardized diagnostic tests of Mental Retardation

Back 42

1. Bayley Scale of Infant Development
2. Denver Developmental Screening Test

Front 43

Etiologies of Mental Retardation

Back 43

1. Infection and intoxication
2. Trauma or physical agent
3. Metabolism or nutrition
4. Chromosome abnormalities
5. Gestational disorders
6. Environmental influences

Front 44

Classification of Mental Retardation

Back 44

Mild - not noticed by casual observation (IQ 50-70)
Moderate - Noticeable delays (IQ 40-50)
Severe - Few communication skills (IQ 20-40)
Profound - IQ < 20

Front 45

Nursing Care of Mentally Retarded - Dignity, Respect, Privacy

Back 45

Goal - promote optimum development
1. Education
2. Hospitalization
3. Discipline
4. Social skills
5. PLAY exercises
6. Sexuality (most retarded people have normal sex drives)

Front 46

Cerebral Palsy

Back 46

Group of nonprogressive disorders of movement and posture caused by abnormal development of or damage to motor control centers of brain - May not manifest all at once, but it is not progressive

Front 47

CP - description, prevalence

Back 47

Impaired movement - cerebral cortex, non-progressive
*1 in 500 live births - often low birth weight babies, occurs more often now because medical science can keep babies alive when it once could not
* Leading cause of disability in children

Front 48

CP etiologies (prenatal, perinatal, postnatal)

Back 48

1. Prenatal - known/ unknown
2. Perinatal - 5-10% due to difficult birth
3. Postnatal - meningitis

Front 49

Types of CP

Back 49

1. Spastic - most common (hypertoniscity, impaired motor skills, abnormal posturing)
2. Athnoid - intellectually superior to spastic (dyskinetic, wormlike writhing movements, drooling, dysarthria)
Hyperbilirunbinemia - bilirubin deposits in brain

Front 50

Diagnostic Criteria for Cerbral Palsy

Back 50

1. Neuro exam and history
2. Motor dysfunction
3. Persitence of primitive reflexes (70% have normal intellectual ability)
4. Differential diagnosis

Front 51

Therapeutic agents for CP

Back 51

1. Establish locomotion, communication, self help
2. Gain integration of motor functions
3. Correct associated defects
4. Provide educational opportunities
5. Promote socialization experiences
EVERYBODY IS INVOLVED

Front 52

Pharmacotherapy for CP

Back 52

1. Centrally acting muscle relaxants (baclofen, valium)
2. Skeletal muscle direct action (dantrolene)
3. Local nerve block to reduce spasticity
4. Anticonvulsants (phenobarbital, dilantin)
5. ADD-HD (ritalin)

Front 53

Diagnosis of Down's Syndrome

Back 53

1. Flattened hypoplastic midface with depressed nasal bridge
2. Inner epicanthal folds
3. Upward obliquity or palpebral fissures
4. small external ears
5. Small mouth and narrow palate
6. Prominent palatine ridges

Front 54

Concerns with Down's Syndrome

Back 54

1. Cardiac - septal defects (present in 50%)
2. Respiratory - mucous in upper airways, susceptibility to infection, compromised resp. expansion, acute and chronic obstructions
3. GI - alteration in nutrition - feeding intolerance, vomiting, FTT
4. GU- hypospadias (2%), undecended testes (25-50%)
5. Hematologic concerns 10-30X greater risk of leukemia
6. visual - 50% refractive errors, 60% strabismus
7. Hearing loss - conductive

Front 55

Atlantoaxial instability

Back 55

Occurs in 14% of Down's Syndrome patients; defined as the excessive movement between the junction of the axis and the atlas as a result of either a bony or a ligamentus abnormality, must be ruled out through xray before participating in contact activities

Front 56

Endocrinologic Concerns

Back 56

1. Dificient growth in infancy, adolescence
2. Thyroid dysfunction (hypo)
3. Fertility in males is rare
4. Ovulation present in most females (40-70%)

Front 57

Neurologic Concerns for Down's syndrome

Back 57

1. Deminished muscle tone
2. Seiaures (10%)
3. Alzheimer's (by 51)
4. Cognitive - mild to moderate (IQ 40-70), expressive language disabilities, strength seen in socialability