Ateneo de Zamboanga University College of Nursing 2020-2021 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 Etiology: 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 Stress Endocrine changes Incidence: 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 Incontinence 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 Treatment: Pharmacologic: 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 Respirations 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 concerns 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 Meningitis: 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) INCIDENCE: 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 legs - 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 Treatment: Antibiotics therapy/ IV: Penicillin G (drug of choice) COMPLICATIONS: Seizures Neurologic sequelae: paresis, plegia Pericarditis, heart failure Deafness Shock 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 consciousness 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: Shock Pericarditis Heart failure Pneumonia 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: Handwashing, 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 Etiology: 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 Leukemia 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 Amniocentesis Blood tests Treatment: 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 leukemia Provide psychological support to parents Cognitive or Mental Health Disorders: AUTISTIC Disorder: A pervasive development disorder characterized by withdrawal and impaired interpersonal relationships 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.) Incidence: 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 objects/toys 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 Treatment: 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 Prognosis: More than 2/3 of cases remain severely handicapped and dependent on caregivers (Gelman, 2000) 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 level Characterized by 3 major behaviors: Inattention Unable to complete tasks effectively; easily distracted Impulsiveness 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 Incidence: 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 ADD: Anxiety 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 Treatment: Therapy must incorporate the following: ENVIRONMENT Construction of stable learning environment May include special instructions, free from distractions of an entire class or home environment Child’s academic and psychosocial needs Treatment: MEDICATIONS: 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 MEDICATIONS Antipsychotic Sometimes used in combination w/ stimulants for symptomatic relief Short-term SE: drowsiness, dry mouth, nasal congestion, blurred vision, postural hypotension and weight gain BEHAVIORAL THERAPY Effective in working w/ clients w/ limited cognitive ability such as children and the mentally retarded FAMILY SUPPORT Nursing Care: Focus: 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 G&D 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