NEUROLOGIC DISORDER
lait ("coffee with cream") spots appear that
NEURAL TUBE DISORDERS
-The neural tube is the embryonic structure that
matures to form the CNS.
NEUROCUTANEOUS SYNDROMES - are
characterized by the presence of skin or
pigment disorders with CNS dysfunction.
2 types of neurocutaneous syndromes:
-STURGE-WEBER SYNDROME
(ENCEPHALOFACIAL ANGIOMATOSIS)
-NEUROFIBROMATOSIS (VON RECKLINGHAUSEN
DISEASE)
1. STURGE-WEBER SYNDROME
(ENCEPHALOFACIAL ANGIOMATOSIS)
- Has a congenital port-wine birthmark on the
skin of the upper part of the face that follows
the distribution of the first division of the fifth
cranial nerve (trigeminal nerve).
- A CT scan or an MRI of the skull demonstrates
calcification of the involved cerebral cortex
which appears as a "railroad track" or double-
groove pattern.
-An EEG usually shows decreased voltage in the
affected areas.
-When this syndrome is first diagnosed, parents
may ask to have the skin lesion surgically
removed in the belief that this will correct their
child's condition. Unfortunately, because the
lesion is not just a surface phenomenon, it's
important that parents understand the need for
long-term follow-up particularly if the child has
accompanying seizures that require long-term
anti-seizure therapy.
2. NEUROFIBROMATOSIS (VON
RECKLINGHAUSEN DISEASE)
- Neurofibromatosis is the unexplained
development of subcutaneous tumors.
- The disorder can occur as a mutation or it can
be inherited as an autosomal dominant trait
carried on the long arm of chromosome 17. It
occurs in approximately 1 of every 4,000 live
births and may be diagnosed prenatally.
- As an infant: the child typically shows irregular
but excessive skin pigmentation.
- Later in childhood: pigmented nevi or café-au-
tend follow the paths of cutaneous nerves (six
or more spots larger than 1 cm in diameter are
diagnostic).
- By puberty: multiple soft cutaneous tumors
begin to form in the child's skin along nerve
pathways, and the child may develop seizures.
- Young Adulthood: Subcutaneous tumors may
develop
- The acoustic nerve (cranial nerve VIII) is
frequently involved, leading to hearing
impairment.
- Involvement of the optic nerve can lead to
vision loss.
- Girls especially need to be aware of the
disorder not only because of its inheritance
pattern but also because tumor formation can
increase with pregnancy.
- Little therapy is available to halt the tumor
growth. If lesions are causing acoustic or optic
degeneration, surgical removal of the tumors
may be attempted to preserve hearing or sight.
3. CEREBRAL PALSY
is a group of nonprogressive disorders of upper
motor neuron impairment that result in motor
dysfunction.
Signs & Symptoms:
speech or ocular difficulties
seizures
cognitive challenges, or hyperactivity
Muscle spasticity can lead to orthopedic or gait
difficulties.
- Head injury such as from child maltreatment
or automobile accidents also may lead to CP
symptoms.
- Infections such as meningitis or encephalitis
can result in CP symptoms as well.
Four main types of CP:
- pyramidal or spastic type
- extrapyramidal (dyskinetic) type,
- Ataxic
- mixed
Spastic Type
- Spasticity is tone in the voluntary muscles that
results from loss of upper motor neurons.
- Spastic involvement may affect both
extremities on one side (hemiplegia), all four
extremities (quadriplegia), or primarily the
lower extremities (diplegia or paraplegia).
Children with hemiplegia usually have greater
involvement in the arm than the leg.
A CHILD WITH SPASTIC TYPE:
-hypertonic muscles
-abnormal clonus
-exaggeration of deep tendon reflexes
-abnormal reflexes such as a positive Babinski
reflex
-continuation of neonatal reflexes, such as the
tonic neck reflex, well past the age at which
these usually disappear.
Infant:
-If held in ventral suspension position they arch
their backs and extend their arms and legs
abnormally.
-fail to demonstrate a parachute reflex if
lowered suddenly and tend to assume a
"scissors gait"
School age:
-tightening of the heel cord can become so
severe that children walk on their toes
-unable to stretch their heel to touch the
ground
Older children:
-leg involvement may be detected most easily
by examining the child's shoes as, because the
child does not put the heel all the way down on
the involved side, one shoe heel will be much
more worn than the other. On physical
examination, it may be difficult to abduct the
involved hip fully, extend the knee, or dorsiflex
the foot.
Dyskinetic or Athetoid Type
- The athetoid type of CP involves abnormal
involuntary movement (athetoid means
"wormlike").
-Early in life, the child appears limp and flaccid.
-Later, in place of voluntary movement, children
make slow, writhing motions.
-This can involve all four extremities plus the
face, neck, and tongue.
-Under emotional stress, the involuntary
movements may become irregular and jerking
(choreoid) with disordered muscle tone
(dyskinetic).
Ataxic Type
-Children with ataxic involvement have an
awkward, wide based gait.
-On neurologic examination, they are unable to
perform fine coordinated motions, the finger-
to-nose test, or rapid, repetitive movements
(tests of cerebellar function).
Mixed Type
-Some children show symptoms of both
spasticity and atheroid or ataxic and athetoid
movements. This combination obviously results
in a severe degree of physical impairment
ASSESSMENT
 History and physical assessment.
 Any episode of possible anoxia during
prenatal life or at birth should be
documented.
 Determining the extent of involvement in
an infant is difficult, so the full extent of
the disorder may not be recognized until
the child attempts complex motor skills,
such as walking or coloring.
 Sensory alterations such as strabismus,
refractive disorders, visual perception
problems, visual field defects, and speech
disorders such as abnormal rhythm or
articulation.
 Attention deficit disorder or autism
spectrum syndrome.
 Cognitive challenge and recurrent seizures
also frequently accompany all types of the
disorder.
 A skull X-ray or ultrasound may show
cerebral asymmetry.
 An EEG may be abnormal, although the
pattern is highly variable.
LONG-TERM CARE
 Listen to parents during healthcare visits
and encourage them to discuss the
difficulties of daily living
 Offer them support as needed if they
grieve.
INFECTION
Infection of the nervous system is always
potentially serious. It typically ccurs from
illnesses such as meningitis, encephalitis,
Guillain-Barré syndrome, Reye syndrome, and
botulism.
1. BACTERIAL MENINGITIS
 It tends to occur most frequently in
children younger than 24 months of age
and most often in winter.
 The organisms most frequently seen are
Streptococcus pneumoniae or group B
Streptococcus.
 In children younger than 2 months of age,
Escherichia coli is a common cause.
 If children with myelomeningocele
develop meningitis, Pseudomonas
infection may be the causative agent.
Children who have had a splenectomy are
particularly susceptible to pneumococcal
meningitis unless they have received a
pneumococcal vaccine. Haemophilus
influenzae, once a major cause of
meningitis, is now rarely seen because of
routine immunization against this
organism.
 Pathologic organisms usually spread to the
meninges from upper respiratory tract
infections by lymphatic drainage possibly
through the mastoid or frontal sinuses or
by direct introduction through a lumbar
puncture or skull fracture.
 Brain abscess or invasion of the infection
into cranial nerves can result in blindness,
hearing impairment, or facial paralysis. If a
thick exudate accumulates in the narrow
aqueduct of Sylvius, it can cause
obstruction leading to hydrocephalus.
Assessment
 2 or 3 days of upper respiratory tract
infection prior to the development of
meningitis.
 Intense head ache.
 Sharp pain when they bend their head
forward.
 In the newborn, symptoms such as poor
sucking. weak cry, or lethargy develop.
 Children may hold their back arched and
their neck hyperextended (opisthotonos).
 Will not be able to follow a light through
full visual fields.
 If the meningitis is caused by H.
influenzae, the child may develop septic
arthritis.
 If it is caused by Neisseria meningitidis, a
papular or purple petechial skin rash may
occur
 Sudden cardiovascular shock, seizures,
nuchal rigidity, or apnea can occur.
 CSF results indicative of meningitis include:
 increased white blood cell and protein
levels
 increased ICP
 glucose level less than 60% of blood
glucose (because bacteria have fed on the
glucose). In addition to supplying blood for
glucose level, blood is cultured and
examined for increased WBC count.
 tuberculin skin test to rule out
tuberculosis meningitis.
 A CT scan, MRI, or ultrasound study will be
prescribed to examine for brain abscess.
Therapeutic Management
 Antibiotic therapy as indicated by
sensitivity studies is the primary therapy.
 Intrathecal injections (directly into the
CSF) may also be necessary.
 In some children, it takes a month before
the CSF cell count returns to normal. A
corticosteroid such as dexamethasone or
the osmotic diuretic mannitol may be
administered to reduce ICP and help
prevent hearing loss.
 Children with meningitis are placed on
respiratory precautions for 24 hours after
the start of antibiotic therapy to prevent
transmission of the infection to other
family members or healthcare providers.
 Neurologic sequelae, such as learning
problems, seizures, hearing and cognitive
challenges, and inability to concentrate
urine from lessened antidiuretic hormone
secretion, must be assessed in the weeks
to come because these can be long-term
consequences.
2. GROUP B STREPTOCOCCAL INFECTION
 A major cause of meningitis in newborns is
group B streptococci.
 The organism is contracted either in utero
or from B secretions in the birth canal.
 Colonization can result in either an early-
onset onset illness.
 With the early-onset form, symptoms of
pneumonia become apparent in the first
few hours of life.
 The late-onset type leads to meningitis
instead of pneumonia.
 With meningitis, at approximately 2 weeks
of age, the or a late infant gradually
becomes lethargic and develops a fever
and upper respiratory tract symptoms.
 Treatment is with antibiotics that are
effective against the group B
Streptococcus, such as ampicillin and
cephalosporins.
3. ENCEPHALITIS
- Inflammation of brain tissue and, possibly, the
meninges as well.
- It can arise from protozoan, bacterial, fungal,
or viral invasions.
- Enteroviruses are the most frequent cause,
followed by arboviruses.
- Several encephalitis viruses, such as those that
cause St. Louis encephalitis, West Nile
encephalitis, and East emequine encephalitis,
are borne by mosquitoes and thus are seen
most often during the summer months.
- Encephalitis also can result from direct
invasion of the CSF during lumbar puncture.
- Another cause is complication of childhood
diseases such as measles, mumps, or
chickenpox.
Assessment
- headache
- high temperature
- ataxia (loss of usual muscle movements)
- muscle weakness or paralysis
- diplopia
- confusion
- irritability
- nuchal rigidity
- positive Brudzinski or Kernig sign
- A child becomes increasingly lethargic and
eventu ally comatose.
- CSF evaluation will reveal an elevated
leukocyte count and an elevated protein level.
- An EEG will demonstrate wide spread cerebral
involvement.
- A brain biopsy, identifies the virus.
Therapeutic Management
- Treatment for a child with encephalitis is
primarily support.
- An antipyretic is prescribed to control fever.
- Mechanical ventilation may be required to
maintain the child's respirations during the
acute phase.
- A variety of medications, such as acyclovir
(Zovirax), an antiviral agent, and carbamazepine
(Tegretol), an anticonvulsant, may be
prescribed.
- A steroid such as dexamethasone or an
osmotic diuretic such as man nitol may be
needed to decrease brain edema and ICP.
- Encephalitis is always a serious diagnosis
because although child may recover from the
initial attack without further symptoms, there
can be residual neurologic damage, such as a
seizures or learning disabilities. Parents may
find it hard to believe their child is so seriously
ill at first.
4. REYE SYNDROME
- Reye syndrome is acute encephalopathy with
accompanying fatty infiltration of the liver,
heart, lungs, pancreas, and skeletal muscle.
- It occurs in children from 1 to 18 years of age
regardless of gender
- Symptoms such as lethargy, vomiting,
confusion, and combativeness usually occur
after a viral infection such as varicella
(chickenpox) or influenza that was treated with
acetylsalicylic acid (aspirin).
- Treatment is supportive. Untreated, the
condition leads to coma and death. Anticipatory
guidance to parents and children about avoiding
the use of aspirin during viral infections has
almost prevented the syndrome.
5. GUILLAIN-BARRÉ SYNDROME
(inflammatory polyradiculoneuropathy)
- is a perplexing syndrome that occurs in about
1 in every 100,000 children.
- Both motor and sensory portions of peripheral
nerves are affected.
- Boys develop it more often than girls
- With successful widespread polio eradication
efforts, Guillain-Barré syndrome is now the
most common cause of acute and subacute
flaccid paralysis in childhood
- It is suspected that the reaction is immune
mediated, occurring after upper respiratory
tract or gastrointestinal illnesses or, rarely,
immunizations.
- Inflammation of the nerve fibers apparently
causes temporary demyelization of the nerve
sheaths.
Assessment
- Peripheral neuritis several days after the
primary infection.
- Tendon reflexes begin to decrease and then
become absent.
- Muscle paralysis and paresthesia (loss of sen
sation)
- facial weakness and difficulty in swallowing.
- spontaneous respirations are no longer
possible, leading to respiratory involvement
severe enough to warrant mechanical
ventilation.
- elevated CSF protein level.
- An EEG may show denervation and decreased
nerve conduction velocity.
Therapeutic Management
- Treatment of Guillain-Barré syndrome is
supportive until the paralysis peaks at 3 weeks
and then is followed by gradual recovery.
- Plasmapheresis or transfusion of immune
serum globulin may shorten the course of the
illness. - - Cardiac and respiratory function must
be closely monitored.
- All patients should be given subcutaneous
fractionated or unfractionated heparin and
support stockings until they are able to walk
independently to prevent deep vein thrombosis.
- prevention of the effects of extreme
immobility while guarding respiratory fun tion.
- An indwelling urinary catheter is usually
inserted to monitor urine output.
- Enteral or total parenteral nutrition.
- If the child has discomfort from neuritis,
adequate analgesia is necessary.
- To prevent muscle contractures and effects of
immobility, turning and repositioning every 2
hours is important in addition to passive range-
of-motion exercises about every 4 hours.
6. BOTULISM
- Botulism occurs when spores of Clostridium
botulinum colonize and produce toxins in the
intestine.
- Infant botulism is not transmitted from person
to person and occurs predominantly in infants
younger than 6 months of age.
-Symptoms occur within a few hours after
ingestion of the contaminated food. Almost
immediately, there is generalized weakness,
hypotonia, listlessness, a weak cry, and a
diminished gag reflex, followed by a flaccid
paralysis of the bulbar muscles that leads to
diminished respiratory function.
-The organism can be cultured from stools or
serum.
- Electromyography may be helpful to support
the diagnosis.
-Treatment is supportive care. Human-derived
botulinum immune globu lin may stop the
progress of the disease.
INFLAMMATORY DISORDERS
Two neurologic inflammatory disorders are
found frequently in adolescents.
 CARPAL TUNNEL SYNDROME
 FACIAL PALSY (BELL PALSY)
1. CARPAL TUNNEL SYNDROME
- Is nerve compression of the median nerve that
passes through the carpal tunnel at the wrist.
- Compression of the nerve causes numbness
and
sharp pain and burning in the thumb and the
second,
third, and fourth fingers of the hand.
- Word processing, texting, and video games
have
turned this previously adult disorder into a
disorder that occurs in children as well.
- Pain usually occurs at night and is enough to
keep a child awake.
- The usual therapy is application of a splint to
the wrist,
which holds the wrist in a neutral (not flexed
and not
extended) position.
- An oral anti-inflammatory medication and
perhaps
a corticosteroid injection into the inflammed
wrist both
help to relieve pain.
- If these therapies are not succesful, the
stricture at the carpal canal can be relieved
surgically.
2. FACIAL PALSY (BELL PALSY)
 Is facial paralysis of the seventh (facial)
cranial nerve, the nerve that innervates
the muscles of facialexpression.
 The syndrome occurs abrubtly and may be
associated with herpes or Lyme disease
infection or occur as a result of cold air
from skiing or from riding in a convertible.
 Therapy in adults consists of prednisone to
reduce infammation and acyclovir if the
syndrome is herpes related. In children,
prednisone use is variable.
 If the child is unable to close the eye on
the affected side, eye drops three or four
times daily wil be needed. Although 4
months, most children recover without any
permanent disability.

PAROXYSMAL DISORDERS
Seizures, headaches, and breath-holding spells
are the most frequent types seen in childhood.
1. EPILEPSY (RECURRENT SEIZURES)
- Is an involuntary contraction of muscle caused
by abnormal electrical brain discharges.
- Approximately 5% of children will have at least
oneseizure by the time they reach adulthood.
- About 50% of seizures are idiopathic (unknown
cause), they also can be attributed to infection,
trauma, or tumor growth.
- Familial or polygenic inheritance may be
responsible.
- The types and causes of seizures vary with age
and are classified into two major categories:
partial and generalized seizures.
SEIZURES IN THE NEWBORN PERIOD
- Consist only of twitching of the head, arms, or
eyes; smacking of the lips; slight cyanosis; and
perhaps respiratory difficulty or apnea.
SEIZURES IN INFANT AND TODDLER PERIOD
- This age group are infantile spasms, a form of
generalized seizure often called “salaam” or
“jackknife” seizures, or infantile myoclonic
seizures.
- Characterized by very rapid movements of the
trunk with sudden strong contractions of most
of the body, including flexion and adduction of
the limbs, or the infant suddenly slumps
forward from a sitting position or falls from
standing position.
- Approximately 50% of affected children, there
is an identifiable cause such as trauma, a
metabolic disease such as phenylketonuria, or a
viral invasion such as herpes or
cytomegalovirus.
- Approximately 90% of infants with this type of
involvement will be developmentally delayed as
an intellectual development appears to halt and
even regress after the pattern of seizures
begins.
- These seizures occur slighty more often in
males than females.
SEIZURES CAUSED BY POISONING OR DRUGS
- The possibility of poisoning has to be
considered in any child who has a first seizure.
- Likely to appear between 6 months and 3
years of age.
SEIZURES IN CHILDREN OLDER THAN THREE
YEARS OF AGE
(FEBRILE SEIZURES)
- Associated with high fever (102⁰ to
104⁰F[38.9⁰- 40.0⁰C]) most common type seen
in preschool children.
- Generalized tonic-clonic pattern, which lasts
for 15-20 seconds.
THERAPEUTIC MANAGEMENT
- After a febrile seizure subsides, parents should
sponge the child with tepid water to reduce the
fever quickly.
- Caution parents not to apply alcohol or cold
water.
- Parents should not attempt to give oral
medications such as acetaminophen.
- Suppositories may be given at the appropriate
dose.
- At the health care facility, a lumbar puncture
will be performed to rule out meningitis.
COMPLEX PARTIAL (PSYCHOMOTOR OR
TEMPORAL LOBE) SEIZURES
- More than half of children who develop
recurrent seizures during school age have an
idiopathic type or the cause of the seizures
cannot be discovered.
- Complex partial (psychomotor) seizures vary
greatly in extent and symptoms and tend to be
a difficult to control. This type of seizure often
begins with a sudden change in posture, such as
an arm dropping suddenly to the side.
MEDICAL MANAGEMENT
- Carbamazepine (Tegretol), Valproate
(Depakene)
PARTIAL (FOCAL) SEIZURES
- Partial seizures originate from a specific brain
area.
- A typical partial seizure with motor signs
begins in the fingers and spreads to the wrist,
arm, and face in a clonic contraction.
- If the movement remains localized, there will
be no loss of consciousness.
- If the spread is extensive, the seizure can cross
the midline and become generalized and, that
point, is impossible to differentiate from a full
generalized tonic-clonic seizure.
ABSENCE SEIZURES
- One form of generalized seizures, formely
known as petit mal seizures.
- Occur more often in girls than boys, usually
occur in school age children between 4 to 12
years, and consist of a staring spell that lasts for
a few seconds.
- Rythmic blinking and twictching of the mouth
or an extremity may accompany the staring.
- No first aid measures are necessary for
absence seizures, and downplaying the
importance of these episodes helps children
maintain a positive self-image.
MEDICAL MANAGEMENT
- Ethosuximide (Zarontin), Valproate, or “off-
label” lamotrigine (Robotham, 2011).
- Follow-up supervision is necessary during
adolescence because some children’s seizure
pattern changes from absence involvement to
tonic-clonic involvement as they approach
adulthood.
TONIC-CLONIC SEIZURES
- Formely termed grand mal seizures
- Generalized seizures consisting of three stages:
a prodromal period of hours or days or an aura,
or warning, immediately before the seizure that
a seizure is about to occur; a tonic-clonic stage;
and, a postictal stage.
MANAGEMENT
- Therapy includes daily administration of an
antiseizure medication such as valproate
(Depakene) and Carbamazepine (Tegretol).
- Phenobarbital may be administered to young
children.
- Prescribed a ketogenic diet or a diet high in fat
and low in protein and carbohydrate.
STATUS EPILEPTICUS
- Refers to a seizure that lasts continuously for
longer than 30 minutes or a series of seizures
from which the child does not return to the
previous level of consiousness.
MEDICAL MANAGEMENT
- Diazepam must be administered with extreme
caution, however, based on the child’s drug
history because the drug is incompatible with
many other medications, and any accidental
infiltration into subcutaneous tissue causes
extensive tissue sloughing.
ASSESSMENT OF THE CHILD WITH SEIZURES
- A thorough pregnancy history be obtained on
any child with seizures.
- Investigate the child’s overall behavior in the
last few weeks such as bed wetting, or failing
marks in school.
- Complete physical and neurologic examination
and blood studies are necessary to rule out
metabolic or infectious processes.
-Prepare a child for a lumbar puncture to rule
out meningitis or bleeding.
- Caution children during the EEG
2. BREATH HOLDING
- A phenomenon that occurs in young children
when they are stress or angry.
- Brain cells become anoxic, the child appears
cyanotic and slumps to the floor, momentarily
unconscious.
- The child needs no immediate therapy except
reassurance the episode is over.
- It may be associated with iron-deficiency
anemia.
3. HEADACHE
- In children younger than school age used to
be considered rate, but young children can
report “hair hurt” or have fussiness from
allergies and viral inflammation of the upper
respiratory tract that cause headaches.
- It may also occur with a fever because of
increase ICP caused by increased cerebral blood
flow.
- Headache pain results because of meningeal or
vascular irritation.
TENSION OR STRESS HEADACHE
- A type of headache is usually experienced as a
dull, steady pain across the forehead, the
temporal area, or the back of the neck.
- Stress headaches are relieved by simple
analgesics, such as acetaminophen or
ibuprofen, or by sleep or application of a cool
compress.
- Advising children to take frequent “stretches”
while studying can help avoid muscle tension
and stress and reduce the number of headaches
experienced.
SINUS HEADACHE
- Usually accompanies sinusitis or is associated
with inflammation and possible obstruction of
the sinusis.
MIGRAINE HEADACHE
- A specific type of headache that may or may
not begin with an aura or visual disturbance
such as diplopia or a zigzag pattern across the
visual field.
- The cause of migraine headache is not well
understood but probably results from abnormal
constriction of intracranial arteries that
temporarily reduces cerebral blood supply.
THERAPEUTIC MANAGEMENT
- At the time of the headache, sleep or lying
down may be necessary to relieve pain and
vomiting.
-Acetaminophen and nonsteroidal anti-
inflammatory drugs are the first-line medical
treatment for headaches in children, including
migraines.
- Children may need to be reassured that even
though their headaches are intense and even
incapacitating, migraine headaches are benign
and will not lead to any other condition.
- Follow-up visits are necessary to confirm that
they are not growing worse so treatment is
remaining adequate.
4. SPINAL CORD INJURY
- The resilience of their vertebrae, children have
fewer spinal cord injuries than adults.
- Another frequent cause of spinal cord injury is
diving into too shallow water at beaches or
backyard pools.
- Spinal cord injury without radiologic
abnormality (SCIWORA) syndrome may occur,
so any child with a multiple traumatic injury
needs to be assessed for spinal cord damage.
-Stabilizing the neck at the accident scene is the
best protection against further injury in these
children.
RECOVERY PHASES
- Result when the spinal cord becomes
compressed or severed by the vertebrae;
further cord damage can result from
hemorrhage, edema, or inflammation at the
injury site as the blood supply becomes
impeded.
FIRST RECOVERY PHASE
- Immediately after the injury, the child
experiences a spinal shock syndrome or loss of
autonomic nervous system function (loss of
nerve fibers traveling through the anterior horn
of the spinal canal), leading to loss of motor
function, sensation, reflex activity, and the
presence of flaccid paralysis in body areas
below the level of the injury.
SECOND RECOVERY PHASE
- During the second phase of recovery, the
flaccid paralysis of the shock phase is replaced
by spastic paralysis.
- Spasticity in the second phase is caused by the
loss of upper level control or transmission of
meaningful innervation to the anterior horn.
THIRD RECOVERY PHASE
- Learning to live with the final outcome or
permanent limitation of motor and sensory
function.
ASSESSMENT OF SPINAL CORD INJURY
- Cervical and thoracolumbar areas of the spine
are the ones most likely to sustain injury, but
spinal cord injury should be suspected
whenever a child has sustained a forceful
trauma of any kind.
ATAXIC DISORDERS
- Ataxia is failure of muscular coordination or
irregularity of muscle action. Often manifested
by an awkward gait or lack of coordination.
Causes of ataxia differ, but degeneration of
cerebellar or vestibular function is always
involved.
 ATAXIA-TELANGIECTASIA
 FRIEDREICH ATAXIA
1. ATAXIA-TELANGIECTASIA
- Transmitted as an autosomal recessive trait
attributable to a defect of chromosome 11, is a
primary immunodeficiency disorder that results
in progressive cerebellar degeneration.
- A multisystem disease with neurologic and
immunologic aspects.
- Both immunologic and neurologic symptoms
of this disorder vary in severity and onset.
- Neurologic symptoms caused by the
degeneration process can usually be detected in
early infancy when developmental milestones
are not met.
2. FRIEDREICH ATAXIA
- Carried on the short arm of chromosome 9 as
an autosomal recessive trait, involves a variety
of degenerative symptoms.
-Symptoms such as progressive cerebellar and
spinal cord dysfunction occur in late
adolescence.
- Teenagers develop a progressive gait
disturbance, a lack of coordinated arm
movements, a high-arched foot (pes cavus),
hammer toes, and scoliosis.
-Antioxidant therapy may help to delay this
outcome by reducing ventricular hypertrophy.