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F. Congenital Neurologic Disorders

Ateneo de Zamboanga University
College of Nursing
Congenital Neurologic Disorders
Febrile Seizure disorders:
May occur after an immunization
seizures show an active tonic-clonic pattern; lasts 15 – 20 secs.
Sudden loss of consciousness (may or may not have an aura) followed by:
Rigidity and sustained contraction of muscles (tonic phase)
Violent jerking or intermittent muscular contraction (clonic phase)
Relaxation or deep sleep
Usually occur due to sudden spike of temperature
Exact cause unknown for most seizures after 3 years
Perinatal hypoxia, birth trauma
Infections (meningitis/encephalitis)
Head trauma
Toxins: drugs, lead
Metabolic disorders: hypo- or hyperglycemia
Predisposing factors:
Electrolyte imbalance
Hypo- or hyperglycemia
Cellular dehydration
Endocrine changes
Most common in the 1st 2 years of life
hx of other family members having similar seizures
Signs and Symptoms:
Vary w/ types of seizures
Aura – sensation the precedes seizure/convulsive episode
Loss of consciousness
Eye movement changes, staring in infants
Tonic-clonic movements unilaterally or bilaterally
Post-ictal signs: sleep, weakness and inactivity
Diagnostic Tests/ Procedures:
Health hx, physical examination
Clinical signs
EEG, skull x-ray, CT scan, MRI
Lumbar puncture (to rule out meningitis)
Blood glucose
Serum electrolytes
Anti-seizure drugs until 2 years, after cessation of seizures
Commonly used anti-convulsant drugs:
phenobarbital, phenytoin (Dilantin)
Antipyretic drugs
Appropriate antibiotics
Diet: High fat diet (ketogenic diet) for intractable or myoclonic seizures
Nursing Care:
Assist in thorough assessment: hx-taking, keen observation of signs of seizures
Onset, time, duration; behavior before and during seizures; loss of consciousness
Movements during seizures
Facial changes, eye movements
Postictal activities: duration of sleep, state of consciousness, motor activities
Provide care during seizures:
Ensure patent airway @ all times (No. 1 priority)
Promote child’s safety during seizures:
Stay w/ the child. NO tongue depressor. NO restraints
Loosen restrictive clothing
Place child flat on padded floor to protect head
Remove unsafe objects away from the child
Prevent seizures: Avoid strong stimuli.
Administer anticonvulsant drugs as ordered.
Provide parental teaching on the proper administration of maintenance drugs
Provide psychological support and opportunities for child/parents to express feelings, fears and
Encourage parents to promote child’s appropriate development:
Activities, play, socialization, education
Reassure parents that febrile seizures do not lead to brain damage and child is almost always
completely well afterward
Neurologic Infections
Inflammation of membranes surrounding the brain and spinal cord
infection of the CNS that may be acquired as a primary disease or as a result of local infection
(e.g. otitis media, sinusitis), systemic infections, trauma and neurosurgery
Etiology: Infection by bacteria, viruses, protozoa and fungi:
Bacterial Meningitis
Most commonly caused by Haemophilus influenzae in young children (streptococcus
pneumonia in adults)
Meningococcal meningitis
Transferred thru droplets or by direct contact
Viral meningitis
Associated w/ viruses such as the mumps, enterovirus and herpes viruses
Predisposing/precipitating factors:
Head trauma
Respiratory infection
Neurosurgical procedures
Hemoglobinopathies (sickle cell anemia)
Higher during cold months (winter and spring)
Higher in people living in crowded places
Signs and symptoms:
Fever (40 – 40.5°C) and chills
Nausea, projectile vomiting
Poor feeding/sucking/ anorexia
High-pitched shrill cry, bulging anterior fontanel in infants
Altered level of consciousness.
Varying levels:
Comatose: various levels
Lethargic: obtunded
Confused: disoriented
Alert: oriented
Signs of meningeal irritation:
Opisthotonus position
Stiff neck and nuchal rigidity
Signs of meningeal irritation:
Kernig’s sign = supine, w/ thigh flexed on abdomen, child cannot completely extend his
- Brudzinski = lying flat, and neck is flexed towards chest = flexion of knees and hips;
passive flexion of one lower extremity produces similar movement on the opposite
(contralateral) extremity
Diagnostic Test/ Procedures:
Lumbar puncture: testing of CSF that shows:
cloudy CSF,
 ICP,
 protein,
 glucose
Smear and culture of CSF and blood demonstrate organism
Antibiotics therapy/ IV:
Penicillin G (drug of choice)
Neurologic sequelae: paresis, plegia
Pericarditis, heart failure
Nursing Care:
Isolate infant: 1st nsg implementation on admission
Ensure patent airway (top priority)
Position: Side-lying; turn Q2h
Suction prn, oxygen as needed
Monitor breathing during and after seizure
Promote safety:
Stay with the child during seizures
Place child in side-lying w/ side rails up
Do not restrain during seizure
Pad crib or bed to protect soft tissues
Remove sharp or rough objects/toys from bed
Loosen clothing around neck
Observe and monitor VS, skin color, signs of respiratory distress, signs of seizures, and levels of
Monitor and control temperature
Administer cool sponge bath, hypothermia blanket
Antipyretics p.o./ per rectum
Maintain a cool temperature
Administer antimicrobials, as ordered
Provide for rapid replacement of F&E; child becomes readily dehydrated from fever
Prevent convulsions/observe seizure precaution
Maintain a quiet environment; avoid strong stimuli (noise)
Have airway ready @ bedside
Suction as necessary
Pad side rails
Administer anticonvulsants as prescribed: phenytoin (Dilantin)
Provide appropriate nutrition
As tolerated; given in small amounts and frequently
Increase calories, carbs
Oral fluids as tolerated; IV fluids, if necessary
Strict I&O
Be on constant alert for complications:
Heart failure
Neurologic sequelae
Disseminated intravascular coagulation (DIC)
Nursing care:
Provide parental teaching and support
Explain all procedures and tx regimen to family
Instruct parents in the administration of anticonvulsants, including side effects and
adverse effects
Communicate child’s condition/status
Include prevention of spread:
use of mask,
prophylactic antimicrobials for the family prn or as prescribed
Trisomy 21 (Down’s Syndrome):
Chromosomal aberration characterized by trisomy 21 or the rippling of chromosome number 21
Presence of an extra copy of chromosome 21 = total number of 47 rather than 46
Usually the result of non-disjunction during gamete division
Predisposing factors:
Although no age group is immuned to the development of Down’s syndrome
Found to be more common among children of mothers w/ increased or advanced age
Associated disorders and problems:
Congenital heart defects
Ventricular septal defects (common)
Hirschsprung’s disease
Diagnostic Tests/ Procedures:
Physical examination for associated disorders and problems
Test for intelligence quotient (IQ)
In-utero diagnostic tests/procedures:
Nuchal translucency test
Measures amount of fluid @ the back of fetus’ head
11 – 14 weeks of pregnancy
Blood tests
Goal: for the child to reach his optimum development potential and be able to cope as
effectively as possible to this mental handicap
Based on child’s developmental age rather than chronological age
Behavioral modification therapy
Mental feeding/multisensory stimulation
Diagnosis and tx of any associated cong. disorders and health problems
Nursing Care:
Assess for physical anomalies and mental capacity
Refer parents for genetic counselling
Follow discharge from hospital:
Refer to community resources for continued counselling and child guidance
Educate parents on:
Care of a mentally retarded child:
Meet physical needs
Protect from injuries and infection
Provide consistent care
Give simple discipline
Means to provide multisensory stimulation/mental feeding
Means to prevent infection (highly susceptible)
Educate parents on:
Identifying developmental milestones such as sitting, self-feeding, toilet training, etc –
indicative of neurologic development
Emphasize importance of giving consistent care
to favor the development of sense of trust (foundation of personality) and
feeling of security
Provide care to possible associated problems such as heart defects, intestinal defects and
Provide psychological support to parents
Cognitive or Mental Health Disorders:
AUTISTIC Disorder:
A pervasive development disorder characterized by withdrawal and impaired interpersonal
There is often lack of responsiveness to other people, gross impairment in communication skills,
and bizarre responses to various aspects of the environment – all developing w/in the 1st 30
months of age (may be as early as 18 mos.)
Occurs in 16 – 40 per 10,000 children (Hagman & Dech, 2008)
Boys; may have genetic cause (Volkmar, 2008)
50% of children affected are also cognitively challenged (APA, 2000)
20% may have coexistent mental hlth diagnoses (Mouridsen et al., 2008)
Concern that immunization may precede or cause the disorder has now been r/o as a cause
(Clifton, 2007)
Signs and Symptoms:
Strong desire to be alone: spends hours in solitary play and becomes attached to objects
Withdraws from social interaction, including parents; becomes attached to inanimate
Minimal eye contact – does not look one in the eye; looks past a person
With repetitive, ritualistic behavior and restrictive body movement
Adheres to routines – likes things to stay the same
Head banging, whirling, rocking and teeth grinding
Language/speech delays and deficits:
Pronominal reversal: “HE” to refer to “SELF”; “YOU” for “I” and vice-versa
Echolalia: repetition of words/phrases heard
Labile mood
Long term memory and “savant” skills
IQ < 70
Better in a therapeutic residential setting w/ consistent caregiver(s) rather than hospital setting
Behavior therapy and operant conditioning for speech training, controlling of
destructive behavior and non-functional repetitive behavior
Tranquilizers for aggressive and self-mutilating outbursts
Antipsychotic drugs – haloperidol, triflouperazine
For social withdrawal and stereotypic behavior
More than 2/3 of cases remain severely handicapped and dependent on caregivers (Gelman,
Nursing care:
Provide high-calorie nutrition, ample vitamins and minerals and fluids.
Provide quiet eating environment
Provide consistent care as much as possible
Encourage persistent eye-to-eye contact when talking to the child
Attention Deficit Disorder (ADD)/Attention Deficit Hyperactivity Disorder (ADHD)
Behaviors rel. to inattention that are maladaptive and inconsistent w/ the child’s developmental
Characterized by 3 major behaviors:
Unable to complete tasks effectively; easily distracted
Act before thinking = difficulty waiting for turn
Hyperactivity (Sykora, 2008)
Shift excessively from one activity to the other; excessive or exaggerated
muscular activity
Etiology: exact cause UNKNOWN:
Predisposing/Precipitating factors:
Prenatal factors: lead poisoning, prematurity, head trauma, anoxia
Genetic factor: temperament
Child-rearing practices: child neglect
Allergic reactions
Disturbances in CNS neurochemistry or neurophysiology
Disturbances w/ metabolites of brain catecholamines dopamine and norepinephrine
ADHD common in school-age children
More common among boys: 4-6 times than in girls
Onset usually before 7 years old; duration of @ least months
Diagnosable by 36 mos. – tho’ parents excuse this as “active” or “always on the go”
Signs and Symptoms:
Consistent problems:
Difficulty maintaining attention (short attention span)
Impulsivity and distractibility
Tends to act w/o considering consequences
Hyperactivity may be present (but not always, and tends to disappear as child grows older);
restlessness is common
Inability to wait and complete tasks
Low tolerance for frustrations
Aggressive behavior w/ little expression of guilt
Unpopularity w/ peers
Poor concentration poor school performance
Emotional and behavior difficulties
Social withdrawal
Chronic poor performance in academics
Lack of coordination and self-control = poor sports performance
Other S/Sx:
Difficulty w/ concepts: right and left, before and after, yesterday and tomorrow
Call for sequencing (relating things to one another in time or space)
Difficulty forming common letters such as b and d
Difficulty reading; prepositions, conjunctions
Turning door knobs, keys, tying shoe laces, faucets = becomes complex tasks
Diagnostic Tests:
Initial identification of the “hyperactive” child as he enters school
Physical examination and lab studies do not establish the dx
Psychologic and psycho-educational testing
Therapy must incorporate the following:
Construction of stable learning environment
May include special instructions, free from distractions of an entire class or home
Child’s academic and psychosocial needs
Stimulant: METHYLPHENIDATE HCL (Ritalin, Concerta)
Stimulates dopamine receptors to calm rather than stimulate activity
Short-acting (2 – 4 hours); fewer side effects
Used when primary sx are manifested in school
Less reliable if used by pre-schoolers and adolescents
Short-term SE: sleep disturbances and decreased appetite
Antidepressant: IMIPAMINE (Tofranil)
Immediate response w/ ADHD
Given OD – for improved monitoring of compliance and toxicity using plasma levels
Monitor cardiacstatus and signs of overdose
Short-term anticholinergic SE: dry mouth, constipation, blurred vision and insomnia
Sometimes used in combination w/ stimulants for symptomatic relief
Short-term SE: drowsiness, dry mouth, nasal congestion, blurred vision, postural
hypotension and weight gain
Effective in working w/ clients w/ limited cognitive ability such as children and the
mentally retarded
Nursing Care:
Child’s response to illness and nsg interventions w/c are meant to:
Modify child’s coping skills and
Strengthen his competency skills
Establish trusting rel. and develop therapeutic alliance w/ child
Adapt nsg interventions appropriate to the child’s devtal age; promote progression of the child’s
Monitor, educate and evaluate medication effects w/ children and their families
Use age-appropriate materials (e.g. puppets, audo-visual materials, etc) to teach and
prepare the child for medication and tx procedures
Promoting age-appropriate knowledge of medication and tx procedures
can help improve the child’s self-esteem and feelings of control and self-worth
Encourage participation of the significant person(s) in the tx
Provide supportive environment
Give clear and simple rules and discipline coupled w/ firm limits
Observe principles of behavioral therapy as appropriate:
Provide consistency in nsg care
Promote daily routine the child is expected to accomplish and give reward
(praise) for successful accomplishment
Provide balance of stimulation and relaxation; avoid excessive fatigue and overstimulation
Quiet , non-stimulating environment/activity around bedtime; no TV and rough games
before bedtime