Nursing children with neurological concerns
‫الدكتور عبد المهدي عبد الرضا حسن‬
‫ جامعة بابل‬/ ‫كلية التمريض‬
PhD, pediatric & Mental Health Nursing
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Cognitive and sensory impairment
Cerebral dysfunction
: Neuromuscular or muscular dysfunction
COGNITIVE AND SENSORY IMPAIRMENT
Autism spectrum disorders
Genetic disorders (e.g. Down syndrome, fragile X syndrome
Disorders of auditory or visual perception or processing.
`There are many ways of being smart`
Children with cognitive and sensory impairments learn differently
than typical children. The role of those who help them grow up is
to try to understand and support those pathways, and promote
normalization and integration where appropriate. Atraumatically.
Step-by-step processes of learning (how to dress, how to feed
oneself, how to read, how to speak) require task analysis of what
is involved – and assistance with the fraction of the task is
problematic.
e.g. Many visually impaired children CAN learn to read… but there
needs to be a system of substitution for the visual input of letters
on a page. Many children with Down syndrome CAN learn basic
self-care, but perhaps velcro can be used a substitute for buttons.
DOWN SYNDROME
Approx 95% of children with Down Syndrome have an extra
chromosome 21.
A minority have MOSAIC Down, wherein some cells have the
trisomy, and other cells have the typical 46 chromosomes.
Although women aged 40-and-over have a 1:110 chance of having
a baby with Down syndrome, MOST children with Down are born to
parents in their 20s-30s.
Prenatal diagnosis possible – with parents then having to make the
tough choice about termination of pregnancy.
Characteristics of children with Down syndrome:
Head/face:
--rounded, small head (bradycephaly)
--speckling of irides (brushfield’s spots)
--short pinna, small ears
--depressed nasal bridge
--inner epicanthal folds
--high, arched narrow palate
--protruding tongue
--short, broad neck
Body:
--shortened rib cage, short stature
--congenital heart defects common
--hypotonia (“like a rag-doll”)
--wide space b/w big and second toe
--transverse palmar (“simian”) crease
--increase risk of hypothyroidism and leukemia
Intellectual
--average IQ around 50
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Incidence: 1 per 800-1000 live births.
Lifespan: more than 80% survive to age 55 years and beyond.
No treatment exists to change the genetic makeup; but there are
treatments and approaches to the multiple associated health
issues: increased rates of congenital heart disease, hypotonia,
hypothyroidism.
You are working with an Early Intervention service and 5-year-old
Justin has just been referred to you. You want to help him develop
his muscle tone, to encourage expression and to build his
vocabulary. How will you do that.
FRAGILE X SYNDROME
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Caused by an abnormal gene on the long arm of the X
chromosome.
Women can be carriers (the healthy X chromosome dominates) but
men exhibit the trait.
Classic physical findings include a long face with prominent jaw
(prognathism), large protruding ears, and behavioral
manifestations such as outbursts, hyperactivity, short attention
span
Speech and language therapies
Modifications in teaching
 Occupational therapy
 Rx: Serotonin agents such as Carbamazepine (Tegretol) or
Fluoxetine (Prozac) to control outbursts
* Rx: CNS stimulants to improve attention span and decrease
hyperactivity
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AUTISM SPECTRUM DISORDERS
Immune and-or environmental factors interact with a genetic
disorder of prenatal or postnatal brain development.
What we see: some combination of difficulties in regulating motion
and some parts of memory, delayed speech, atypical social
interaction often starting with difficulties establishing eye contact,
persistence of infantile reflexes, epileptic seizures.
Incidence: 1 in 150 children are living with ASD (CDC, 2007).
Screening for normal speech:
12 months… at least—babbling, gesturing
18 months… at least—a few single words
24 months… at least some two-word phrases
A group of diverse disorders including:
autism
Asperger`s
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Pervasive Developmental Disorder Not Otherwise Specified (PDDNOS).
Diagnostic criteria (APA, DSM-4) includes:
marked impairment in social interaction
qualititative impairments in communication
restricted repetitive and stereotyped patterns of behavior,
interests, and activities
(see p. 1200 of Perry et al text)
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The majority – but not all – children with autism have some degree
of intellectual impairment, but also may have atypical abilities…
and may excel in music, puzzle-building, mathematics.
Most children with autism will require lifelong supports. Families
need support.
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Videos of children and families living with autism.
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E.g Autism every day. 7-minute youtube video
PERCEPTUAL DISORDERS
Visual impairment
Hearing impairment
Dyslexia, dyscalculia, dysgraphia
Auditory processing disorders
Visuo-spatial processing disorders (nonverbal learning disabilities)
VISUAL IMPAIRMENTS
Can be caused by
Genetic conditions such as: albinism, TaySachs disease
Prenatal and perinatal infections such as: CMV, toxoplasmosis,
herpes, chlamydia
Ocular trauma
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Retinoblastoma. Unilateral (usually) or bilateral ocular tumors.
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Retinopathy of prematurity
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Amblyopia (strabismus, or e.g., or any cause of unilateral vision
impairment leads the brain to receive images only from the
stronger eye… so that the weaker eye is essentially unused … and
corresponding visual centers in the brain also do not develop.
HEARING IMPAIRMENTS
Common – an estimated 3:1000 infants have some degree of
hearing loss
Types:
 Conductive hearing loss
 Sensorineural hearing loss
 Mixed
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Causes: genetic predisposition, anatomic malformations, perinatal
infection such as CMV or toxoplasmosis, ototoxicity from certain
antibiotics such as gentamycin used in treating pediatric infections,
chronic ear infections, Down syndrome.
Conductive hearing loss can be partially remedied with hearing
aids, that amplify sound.
Sensorineural hearing loss – cochlear implants. Children with
sensorineural hearing loss have damaged the auditory nerves of
the inner ear. The cochlear implants bypass these and stimulate
surviving deeper auditory nerve fibers to send signals to the brain.
Working with visually impaired children:
dependance on auditory and tactile stimuli for orientation to
time and space
educational adaptations
CEREBRAL DYSFUNCTION
Trauma to the head, brain tumors, seizure disorders, and CNS
infections all have neurological effects and sequelae.
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Head trauma remains a significant cause of death and permanent
disability in children.
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Neurological assessment --
pupillary response
eye opening in response to stimulus
best motor response to stimulus
best response to auditory or visual stimulus
hand grip: equal, unequal
LOC: alert; sleepy; irritable; disoriented; combative; lethargic;
agitated…
Muscle tone: normal; arching; spastic; flaccid; weak; decorticate;
decerebrate…
Fontanel: soft; flat; sunken; tense; bulging; closed
Mood: content; quiet; withdrawn; flat; hostile
Eye movement: normal; nystagmus; strabismus…
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Pediatric versions of Glasgow coma scale are available. Similar, but
small children may not typically be oriented to time and place
though verbal children usually can state their first name.
TRAUMAS
Head traumas
acceleration & deceleration traumas
subdural and epidural hematomas
concussion
skull fracture
Post-traumatic syndromes
post-concussion syndrome
cranial nerve palsies
seizure disorders
Infants and young children have soft and not-quite-fused skulls.
This presents a special plasticity to the brain, and overall, children
can survive brain injury better than adults. However, their stilldeveloping brains are especially vulnerable. Also, their
comparatively large head size in rt the rest of their bodies means
the head is particularly vulnerable to trauma.
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Underlying causes of symptoms is often related to not only to the
actual injury or disease process and bruising/bleeding, but to the
swelling that occurs, or blockage in the normal flow of cerebral
spinal fluid through the ventricles of the brain… in the closed bony
cranium.
All of these events result in INCREASED INTRACRANIAL PRESSURE
(IIP)… irritable, high-pitched unconsolable cry; tense and bulging
fontanels; distended scalp veins; vomiting unrelated to feeding;
headache; seizures; bradycardia; drowsiness; flexion or extension
posturing… coma.
Blunt trauma to the head in acceleration/deceleration impact
results in bruising of the brain and tissues at the point of impact
(coup), and directly opposite this (contrecoup).
Trauma may also cause deformation of the head, with destruction
of bone, brain, and other tissue.
Care of children with head injury involves sustaining vital signs,
possibly neurosurgery , possibly corticosteroid medication to
reduce swelling.
Support for families. While extent of tissue damage can be
described, neurological outcomes are often unclear.
Post-traumatic syndromes include hydrocephalus from damage to
structures, cranial nerve palsies, sensory or motor deficits,
emotional lability, behavior disturbances.
CONCUSSION
The most common head injury is concussion, a transient neuronal
dysfunction, with instantaneous loss of awareness and
responsiveness that lasts minutes to hours. May result in loss of
consciousness – but not necessarily.
Concussion is defined as a traumatically induced alteration in
mental status. Confusion and amnesia follow.
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Pathogenesis of concussion is still a bit unclear, but may be a result
of shearing forces that cause stretching, compression, and tearing
of nerve fibers.
POST-CONCUSSION SYNDROME can last for weeks to months.
Involve symptoms of impaired memory, headache, behavioral
disturbances.
NEUROLOGICAL INFECTIONS
Menigitis
Encephalitis
Reye’s syndrome
Rabies
MENINGITIS
An acute inflammation of the meninges and CSF. Most common
among under-fives.
Recent immunizations against Hib (Haemophilus influenza type B)
has successfully reduced the incidence of meningitis. However,
meningitis can be caused by other organisms as well—bacterial or
viral, such as Staphylococccus aureus, Escherichia coli, Neisseria
meningitidis (meningococcus).
The most common route of infection is vascular dissemination from
foci of infection elsewhere.
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Baby with meningococcal infection
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Symptoms: sudden illness; arching; nuchal rigidity; irritability;
vomiting unrelated to feeding; thermo instability; mottling;
alterations in behavior and consciousness.
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Meningitis is a medical emergency.
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Dx: lumbar puncture for CSF
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Treatment:
Sustain vital signs
Isolation precautions
IV access and hydration
IV antibiotics (start with two broad-spectrum antibiotics; once
culture and sensitivity results are back, one may suffice)
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Corticosteroids (e.g. dexamethasone) to reduce swelling
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10 – 15% of bacterial meningitis is fatal.
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Outcomes depend on duration of illness before antibiotic therapy;
type of organism; age of child; and adequacy of treatment.
ENCEPHALITIS
Can involve direct inflammation of the brain by a virus (e.g.
herpes) or postinfectious inflammation after infection.
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Early symptoms are vague… fever, irritability… but these evolve to
headache, dizziness, nuchal rigidity, changing levels of
consciousness and possibly seizures
Mild to severe forms.
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Tx: antiviral medications such as Acyclovir
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Prognosis is highly variable. Some children recover completely.
Others have lifelong neurological sequelae.
SEIZURE DISORDERS
(EPILEPSY)
Seizures are caused by excessive and disorderly neuronal
discharges in the brain.
Many forms…from subtle and transient to severe and continuous.
Multiple possible causes – acute trauma or infection, toxins,
tumors; metabolic disorders such as hypoglycemia or
hypocalcemia; residual damage from past traumas…
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Types:
simple partial seizures with motor signs (tonic/clonic movements
of part of the body)
simple partial seizures with sensory signs (numbness, tingling,
prickling, pain, or paresthesia in part of the body)
complex partial seizures (psychomotor seizures – such as
transient amnesia or confusion; inability to respond to environment
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tonic-clonic seizures (formerly known as grand mal) involving a
tonic phase (stiffening; eyes rolling up; apnea…); a clonic phase
(from a few seconds to a half hour or longer, usually about 30
seconds) of violent jerking, incontinence…), and a post-ictal phase.
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Status epilepticus
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Series of seizures at intervals too brief to allow the child to regain
consciousness between the time one event ends and another
begins.
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A medical emergency as it can lead to respiratory arrest.
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Absence seizures (formerly called petit mal) involving brief loss of
consciousness and may go unrecognized or be confused with
“daydreaming”.
Atonic and akinetic seizures (“drop attacks” with sudden loss of
tone)
Infantile spasms (sudden muscle spasms, possible rolling of eyes
upwards in an infant)
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What happens in children with seizure disorders?
In response to physiological stimuli, such as cellular dehydration,
severe hypoglycemia, electrolyte disturbances, sleep deprivation,
emotional stress, or endocrine changes… hyperexcitable cells
activate normal cells, and the impulse spreads thoughout… causing
changes in sensorimotor perception and level of consciousness.
Caring for a child having a seizure:
Do not move or forcibly restrain him/her
Support airway with positioning
Remain calm, stay with the child, protect from injury.
Isolate from view of curious bystanders
911 for status epilepticus
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ER treatment of status epilepticus involves
support of respirations and vital signs (ABC)
rectal diazepam / valium
IV access
IV antiepileptics: lorazepam/ ativan
or diazepam/ valium
or fosphenytoin/ cerebrex
or phenobarbitol / phenobarb
Treatment:
Usually pharmacological, but it may take numerous drug
adjustments to find the right dose of the right drug to control
seizures while limiting side-effects.
e.g. phenytoin (dilantin)
valproic acid (depakene)
Neuromuscular dysfunctions
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Cerebral palsy
Myelomeningocoele
Congenital hydrocephalus
Anencephaly
Spinal muscular atrophy
Werdnig-Hoffman Disease
Kugelberg-Welander Disease
Duchenne Muscular Dystrophy
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CEREBRAL PALSY (CP)
A group of permanent disorders of the development of movement
and posture, causing activity limitation, that are caused by
nonprogressive disturbances that occurred in the fetal or infant
brain.
In addition to motor disturbances, CP also involves some
combination of differences in sensation, perception,
communication, cognition, secondary musculoskeletal conditions,
and epilepsy
Many children with CP have normal-or-better intellectual abilities.
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Causes: prenatal or perinatal asphyxia; low birth weight and
prematurity; intrauterine infections; meningitis; shaken baby
syndrome…
Improvements in survivorship of very low birth weight babies has
also resulted in a rise in the number of children with CP.
Different types:
spastic or pyramidal (>70%). involving hypertonicity and poor
control of posture, balance, and coordinated movement.
dyskinetic. Writhing and twisting movements
ataxic or extrapyramidal. Wide gait; rapid repetitive movements
mixed.
Goals of care:
To establish locomotion and communication.
To gain optimal appearance
To correct associated defects such as contractures as soon as
possible
To adapt educational opportunities
To provide for social interaction with other affected and
unaffected children.
Orthopedic devices to aid locomotion include:
Physical therapy
Orthopedic surgery to correct contractures
Pharmocotherapy for muscle relaxation
Pharmacotherapy for seizure control
Voice-activated computer technology
Voice synthesizers for children who cannot speak
Dental hygiene
Protective headgear
Recreational activities
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Supporting children and families…
You are a nurse at a children’s hospital Ortho Unit. Bethany, age
6, who has spastic quadriplegic CP, is admitted for ortho surgery to
correct some bony contractures of her lower limbs. Her voice is
dysarthric. She is of normal intellect, and is frustrated when you
cannot make out what she is saying. Her mother understands her
words. No sibs. The dad left the family two years ago to start
another family, and mom quit her job to be a full-time caregiver.
The family receives social assistance.
Plan? For Bethany? For her mom?
NEURAL TUBE DEFECTS
Normally, the spinal cord is covered by meninges and a column of
bone, fusing in early fetal life.
MYELODYSPLAGIA refers to any malformation of the spinal canal
and spinal cord. NEURAL TUBE DEFECTS result from
myelodysplagia and can occur at any point along the spinal tract,
involving some or all of it.
Can involve:
Spina bifida occulta
Meningocoele
Myelomeningocoele
Anencephaly
MYELOMENIGOCOELE
(spina bifida)
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What we see is some degree of motor and sensory loss in areas
innervated at or below the lesion, affecting – to varying degrees –
movement, sensation, and/or continence.
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Depending on the degree of downward-herniation of the spinal
contents, children with spina bifida commonly also have congenital
noncommunicating hydrocephalus.
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The downward traction tends to obstruct the flow of CSF between
the ventricles of the brain.
HYDROCEPHALUS
ANENCEPHALY
DUCHENNE MUSCULAR DYSTROPHY
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Duchenne Muscular Dystrophy is the most severe and most
common form.
An X-linked recessive disorder affecting males almost exclusively…
females are carriers.
Boys with DMD usually have apparently normal development
through infancy and early childhood… with evidence of muscle
weakness appearing b/w ages 3-7.
Early signs: waddling gait, lordosis, frequent falls, inability to ride
a bicycle…
Loss of independent ambulation by age 9-11.
Slow but relentless progressive muscle weakness throughout the
teenage years.
Death occurs from failure of respiratory muscles or infection.
Best lifespan: into early adulthood.
Survival can be extended by respiratory support such as
tracheostomy and suctioning…
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How to maximize independence in teenage boys with DMD?
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Genetic counseling for families affected.
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YOUR feelings about caring for children with serious neurological
disorders?
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Societal stigmas?
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Countering societal stigmas?