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Disease of Central
Nervous System
Rahul Dhaker
Asst. Professor
Ramsheni College of Nursing, Bhilwara-
311001, Rajasthan
1R Dhaker, Asst. Professor, RCN
Introduction
• Disease of nervous system are fairly common in
children.
• Almost 20 to 30 percent of children are victims of
neurological illnesses.
• There are major contributors to childhood morbidity
and disability.
• Neurological disorder of infancy and childhood are
different than in adults.
2R Dhaker, Asst. Professor, RCN
Cont… Introduction
• Neurological symptoms are also found in
association with various systemic disease.
• Common disease condition in children
involvement central nervous system-
• congenital malformation
• Prenatal problem
• Developmental disability
• CNS infection
• Craniocerebral trauma
• Brain tumor.
3R Dhaker, Asst. Professor, RCN
• Diagnostic test –
• CT Scan
• MRI
• PET Scan
• SPET scan (Single-photon emission computed
tomography)
• Cerebral angiography
• Myelography
• Neuropsychological testing.
• EEG
• Ultrasonography etc.
Cont… Introduction
4R Dhaker, Asst. Professor, RCN
PET Scan
5R Dhaker, Asst. Professor, RCN
• PET scan to inspect the blood flow, oxygen
intake, and metabolism of your organs and
tissues. PET scans are most commonly used to
detect:
• Cancer
• Heart Problems
• Brain Disorders
• Problems With The Central Nervous System
6R Dhaker, Asst. Professor, RCN
7R Dhaker, Asst. Professor, RCN
Neonatal Convulsion
• Convulsion is the involuntary contraction or
series of contraction of the voluntary muscles.
• It occurs due to disturbance of the brain function
resulting from-
– abnormal excessive electrical discharge from
brain.
8R Dh@ker, Asst. Professor
Cont…
• It is manifested by involuntary, motor, sensory,
autonomic or psychic phenomenon, alone or in
combination.
• It may be associated with alteration of level of
consciousness.
• Convulsion is also term as seizure.
• Convulsion are more commonly found in infants
and children.
9R Dh@ker, Asst. Professor
• It is more commonly found alone with
– cerebral palsy (35%) and
– Mental retardation (20%).
• 57.5/1,000 in infants with birth weights <1,500 g
• 2.8/1,000 in infants weighing between 2,500 and
3,999 g have seizures.
Cont…
10R Dh@ker, Asst. Professor
• Neonatal convulsion are common life
threatening emergency in the new born due to
cerebral or biochemical abnormality.
• Preterm and LBW babies are more prone to
this problem.
• Newborn babies do not manifest febrile
convulsion.
R Dh@ker, Asst. Professor 11
Cont…
Etiology:
(1-4)
days
1.Hypoxic-ischemic encephalopathy
2.Drug withdrawal or toxicity
3.Intraventricular hemorrhage
4.Acute metabolic disorders:
•hypocalcemia,
•hypoglycemia,
•hypomagnesemia and
•hypo or hyper natremia)
5.Inborn errors of metabolism (ex.
Galactosemia)
6.Pyridoxine deficiency. 12
R Dh@ker, Asst. Professor
(4-14) days
1. Infection ( meningitis ,
encephalitis)
2. Metabolic ( hypocalcemia,
persistent hypoglycemia)
3. Benign neonatal convulsion.
4.Kernectirus
5. Drug withdrawal
6. Developmental delay, epilepsy,
neonatal diabetes.
Cont…Etiology:
13R Dh@ker, Asst. Professor
Cont…Etiology:
(2-8) wks
1. Infection ( meningitis , encephalitis)
2. Head injury( subdural hemorrhage,
child abuse)
3. Inherited disorders of metabolism
(ex. Aminoaciduria).
4. Malformation of cortical
development (ex lissencephaly)
5. Tuberous sclerosis.
6. Sturge weber syndrome. 14R Dh@ker, Asst. Professor
Put them all together..
1. Hypoxic ischemic encephalopathy (50-60)%..
Most common cause of neonatal seizure.
2. Vascular events (10 -20) %
3. Intracranial infection (5-10)%
4. Brain malformation (5-10)%
5. Metabolic disturbances (↓glycemia, ↓Ca, ↓Mg,
↓↑Na)
6. Drugs ( withrawal or toxicity)
7. Neonatal seizure syndromes : rare
8. Preinatal complication
9. Developmental neurological problem
15
16
Febrile Convulsion
 Febrile convulsions, the most common seizure
disorder during childhood.
 Occurring between 6 months and 6 years.
 Precipitated by fever from:
 infection/inflammation/metabolic disorders .
 It is not a form of epilepsy because brain is normal.
R Dh@ker, Asst. Professor
Type of Febrile Convulsion
• Typical febrile convulsion
• Atypical febrile convulsion
17R Dh@ker, Asst. Professor
Typical febrile convulsion
• These are generalized rather than focal and last
less than 10 minutes.
• It is usually found in children between 6 month to
5 year of age.
• The fits occur within 24 hours of the onset of
fever and usually single/ febrile episode.
• There is no recurrence before 12 to 18 hours of
attack.
• No paralysis of limb.
R Dh@ker, Asst. Professor 18
Cont…Typical febrile convulsion
• CSF and EEG are normal after the attack.
• Family history of convulsion is frequently present.
• The condition may have genetic predisposition or
may be due to immature neuronal membrane
response to rise of body temperature.
R Dh@ker, Asst. Professor 19
Atypical febrile convulsion
• They predispose to idiopathic epilepsy.
• The children may have focal convulsion of more
than 20 minute duration even without significant
fever.
• They may have abnormal EEG for two weeks after
attack.
R Dh@ker, Asst. Professor 20
Diagnostic evaluation
• Family History of convulsion
• History of Maternal drug addiction and infections
• Time of onset of convulsion
• Blood Examination for
– Calcium
– Sugar
– phosphorus
• Lumper puncture – CSF Examination
• EEG
• CT Scan
• MRI
• ECG
• Serology for STORCH
R Dh@ker, Asst. Professor 21
R Dh@ker, Asst. Professor 22
Management
• Management of febrile convulsion should be
done-
– To control convulsion
– To reduce increased body temperature
– To treat the cause of fever, usually ARI
R Dh@ker, Asst. Professor 23
Cont…Management
• Anticonvulsive drug are indicated in prolonged
convulsion.
– Diazepam 0.3mg/kg IV day
– Phenoberbital 5mg/kg IM/day can be administered.
– Sodium valporate 10 to 20mg.kg/day
• Antipyretic
– Prarcetamol
– Mefanamic acid
– Tepid sponge should be given to treat fever.
• Hydration and nutrition status to be maintained.
• clearing of airway
R Dh@ker, Asst. Professor 24
• Oxygen therapy may be needed for some children
• Rest
• Comfortable position
• Hygienic measure to be provided.
• Explanation and emotional support to the parent
are important and along with necessary health
education.
• Duration of therapy can be 1 to 2 year or upto 5
years.
R Dh@ker, Asst. Professor 25
Cont…Management
Prognosis
• In typical febrile convulsion prognosis is good.
• In atypical type, there is chance of development of
complication like-
– Intellectual impairment
– Behavioral problem
– Epilepsy
• Chance of recurrence is about 30 to 80%.
R Dh@ker, Asst. Professor 26
R Dh@ker, Asst. Professor 27
NEONATAL SEIZURES
R Dh@ker, Asst. Professor 28
Meningitis
29R Dhaker, Asst. Professor, RCN
30R Dhaker, Asst. Professor, RCN
Causes of Meningitis
- Bacterial
- Viral
- Fungal
- Ricketsial (Rocky mountain spotted fever)
- Parasitic/ protozoal
- Physical injury
- Cancer
- Certain drugs ( mainly, NSAID’S)
 Severity/treatment of illnesses differ depending on the
cause. Thus, it is important to know the specific cause of
meningitis.
31R Dhaker, Asst. Professor, RCN
32R Dhaker, Asst. Professor, RCN
33R Dhaker, Asst. Professor, RCN
Clinical Manifestation
• The 3 classic symptoms (less likely in younger
children):
• Fever
• Headache
• Meningeal signs
34R Dhaker, Asst. Professor, RCN
Symptoms in neonates:
• Poor feeding
• Lethargy
• Irritability
• Apnea
• Listlessness
• Apathy
• Fever
• Hypothermia
• Seizures
• Jaundice
• Bulging fontanelle
• Pallor
• Shock
• Hypotonia
• Shrill cry
• Hypoglycemia
• Intractable metabolic
acidosis
35R Dhaker, Asst. Professor, RCN
Symptoms in infants and children:
• Nuchal rigidity
• Opisthotonos
• Bulging fontanelle
• Convulsions
• Photophobia
• Headache
• Alterations of the
sensorium
• Irritability
• Lethargy
• Anorexia
• Nausea
• Vomiting
• Coma
• Fever (generally present,
although some severely ill
children present with
hypothermia)
36R Dhaker, Asst. Professor, RCN
37R Dhaker, Asst. Professor, RCN
38R Dhaker, Asst. Professor, RCN
Diagnosis
• History of illness
• Definitive diagnosis is based on the following:
– lumbar puncture-CSF Examination
– Meningeal inflammation demonstrated by increased
pleocytosis, elevated protein level, and low glucose
level in the CSF
39R Dhaker, Asst. Professor, RCN
CSF findings in different forms of meningitis
Type of
meningitis
Glucose Protein Cells
Acute bacterial low high
PMNs
often > 300/mm³
Acute viral normal normal or high
mononuclear
< 300/mm³
Tuberculous low high
mononuclear and
PMNs, < 300/mm³
Fungal low high < 300/mm³
Malignant low high
usually
mononuclear
40R Dhaker, Asst. Professor, RCN
Cont… Diagnosis
• Bacterial meningitis score
• Components of the bacterial meningitis score are
as follows:
– Positive CSF Gram stain
– CSF absolute neutrophil count 1000/µL or higher
– CSF protein level 80 mg/dL or higher
– Peripheral blood absolute neutrophil count
10,000/µL or higher
– History of seizure before or at the time of
presentation
41R Dhaker, Asst. Professor, RCN
• Specific
• Hematologic
– Blood culture
• radiographic
– CT Scan
– MRI
Cont… Diagnosis
42R Dhaker, Asst. Professor, RCN
Management
• IV antibiotics are required; if cause is unknown,
agents can be based on child’s age, as follows:
– < 30 days, ampicillin and an aminoglycoside or a
cephalosporin
– 30-60 days, ampicillin and a cephalosporin; because
Streptococcus pneumoniae may occur in this age range,
consider vancomycin instead of ampicillin
– In older children, a cephalosporin or ampicillin plus
chloramphenicol with vancomycin (needs to be added
secondary to the possibility of S pneumoniae).
43R Dhaker, Asst. Professor, RCN
• Guidelines and recommendations
• Vancomycin plus either ceftriaxone or cefotaxime
• Duration of therapy:
– Neisseria meningitidis - 7 days
– Haemophilus influenzae - 7 days
– Streptococcus pneumoniae - 10-14 days
– S agalactiae (GBS) - 14-21 days
– Aerobic gram-negative bacilli - 21 days or 2 weeks
beyond the first sterile culture (whichever is longer)
– Listeria monocytogenes - 21 days or longer
44R Dhaker, Asst. Professor, RCN
• Duration of therapy should not be shorter than 5
days for meningococcus, 10 days for H
influenzae, and 14 days for S pneumoniae.
45R Dhaker, Asst. Professor, RCN
Treatment
• Initial till results of
C/S are known
• Probable/Proved
Meningococci
• Ampicillin
300mg/kg/day+
• Chloramphenicol
75-100mg.kg/day
• Penicillins
2-5 lac units /kg/day
46R Dhaker, Asst. Professor, RCN
Treatment
• Probable H.Influenzae
• Probable E.Coli
• Ampicillin +
chloramphenicol or
3rd generation
cephalosporin
(cefotaxime
200mg/kg/day)
• Ampicillin +
gentamycin
200mg/kg+2.5-4 mg/kg IV
12hrly
47R Dhaker, Asst. Professor, RCN
Treatment
• Probable group B
streptococci
• Penicillin
50,000i.u/kgI.V/4
hourly.
48R Dhaker, Asst. Professor, RCN
Other Drugs available
Anti-microbials
• Ceftriaxone
• Cefotaxime
• Penicillin G
• Vancomycin
• Ampicillin
• Gentamicin
Anti-Virals
• Acyclovir
• Ganciclovir (>3mths)
Anti-fungals
Amphotericin B
Fluconazole
49R Dhaker, Asst. Professor, RCN
50R Dhaker, Asst. Professor, RCN
Epilepsy
51R Dhaker, Asst. Professor, RCN
Introduction
• Epilepsy is the most common childhood brain
disorder.
• About two-thirds of all children with epilepsy
outgrow their seizures by the time they are
teenagers.
• If you have a child with epilepsy, you're not alone
— 3 million children have this disorder.
52R Dhaker, Asst. Professor, RCN
• Epilepsy is a disease of the central nervous system
in which electrical signals of the Brain misfire.
• These disruptions cause temporary communication
problems between nerve cells, leading to seizures.
• Epilepsy knows no geographical, racial or
social boundaries. About 50 million people in
World have Epilepsy.
53R Dhaker, Asst. Professor, RCN
Epilepsy
Epilepsy is a brain disorder in which clusters of
nerve cells, or neurons, in the brain sometimes
signal abnormally.
It produces changes
in a person's
• movement,
• behaviour or
• consciousness
54R Dhaker, Asst. Professor, RCN
Causes of Epilepsy
• Some people have a specific problem in the brain that
causes the seizures. These include:
• infectious illness (such as meningitis or encephalitis)
• brain malformation during pregnancy
• trauma to the brain (including lack of oxygen) due to an
accident before, during, or after birth or later in childhood
• metabolic disorders (chemical imbalances in the brain)
• brain tumors
• blood vessel malformation
• strokes
• chromosome disorders 56R Dhaker, Asst. Professor, RCN
• Seizures in children have many causes. Common
causes of childhood seizures or epilepsy include
• fever (these are called febrile seizures)
• genetic causes
• head injury
• infections of the brain and its coverings
• lack of oxygen to the brain
• hydrocephalus (excess water in the brain cavities)
• disorders of brain development
57R Dhaker, Asst. Professor, RCN
• The causes of epilepsy in childhood vary.
In about ⅔ of cases, it is unknown.
• Unknown 67.6%
• Congenital 20%
• Trauma 4.7%
• Infection 4%
• Stroke 1.5%
• Tumor 1.5%
• Degenerative .7%
58R Dhaker, Asst. Professor, RCN
Classification of Seizures
ILAE Classification (1981)
I. Partial (Focal)seizures
A. Simple partial
seizures
B. Complex Partial
Seizures
C. Partial Seizures
evolving to secondary
generalized seizures
(tonic-clonic, tonic or
clonic)
II. Generalized seizures
(Convulsive and non-convulsive)
A. Absence seizures
i) Typical ii) Atypical
B. Myoclonic seizures
C. Clonic seizures
D. Tonic seizures
E. Tonic-Clonic seizures
F. Atonic seizures
(Combinations may occur:
myoclonic and atonic or
myoclonic and tonic)
III. Unclassified epileptic seizures
59R Dhaker, Asst. Professor, RCN
Diagnosis of Epilepsy
Thorough History taking :
From patients
From reliable valid informants
From observer (who observed seizures)
Physical Examination:
Specially neurological system
Higher Psychic function
Laboratory Investigation:
S. Electrolytes, S. Prolactin, Blood sugar, CBC, LFT,
RFT, CSF study
Imaging:
EEG, CT Scan of Brain, MRI of Brain, PET, SPECT.
Polysomnography
60R Dhaker, Asst. Professor, RCN
Management
• Epilepsy affects every child differently
depending on:
• Age
• Types of seizure
• Response to treatment
• Having other health issues, etc.
62R Dhaker, Asst. Professor, RCN
Cont… Management
• Commonly drugs are used-
– Phenoberbital- 3 to 5 mg/kg/d or 1 or 2 divided dose
– Diphenylhydantion- 5 to 8 mg/kg/d in 2 divided dose.
– Carbamazepin – 10 to 20 mg/kg/d in 2 to 3 divided
dose.
– Diazepan- 0.2 mg/kg/d IV
– Sodium Valporate- 15 to 20 mg/kg/d in 3 to 4 divided
dose.
• Usually single drug is used but if fails to relieve
seizure than addition of secondary drug is needed.
63R Dhaker, Asst. Professor, RCN
Surgical Management
• Neurosurgical is indicated in some cases of
convulsion disorder, especially anatomical lesion
like – Brain tumor, hematoma etc.
64R Dhaker, Asst. Professor, RCN
65R Dhaker, Asst. Professor, RCN
Encephalitis
R Dhaker, Asst. Professor, RCN 66
Term
• Encephalitis: Infectious process & inflammatory
response limited to brain parenchyma.
• Meningoencephalitis:
Meninges + brain
• Encephalomyelitis:
Brain + spinal cord
• Encephalomyeloradicilitis:
Brain + spinal cord + nerve root
R Dhaker, Asst. Professor, RCN 67
Introduction
• In encephalitis, there is inflammation in the brain
tissues.
• In most cases, this inflammation is caused by a
virus
• In which children become more sleepy or drowsy
than usual.
• This can sometimes be subtle and noticed only
when there is a change in behavior.
R Dhaker, Asst. Professor, RCN 68
Definition
R Dhaker, Asst. Professor, RCN 69
R Dhaker, Asst. Professor, RCN 70
Two Components:
1. Inflammation of brain, and
2. Dysfunction of brain.
Definition
• Encephalitis is an acute inflammation (swelling) of
the brain usually resulting from either a viral
infection or due to the body's own immune system
mistakenly attacking brain tissue.
• In medicine, "acute" means it comes on abruptly
and develops rapidly; it usually requires urgent
care.
R Dhaker, Asst. Professor, RCN 71
Causes of Encephalitis
• Primary (infectious) encephalitis
can be split into three main
categories of viruses:
• Common viruses - including HSV
(herpes simplex virus) and EBV
(Epstein-Barr virus)
• Arboviruses (spread by mosquitoes,
ticks, and other insects) - including
Japanese encephalitis, West Nile
encephalitis, and tick-borne
encephalitis
R Dhaker, Asst. Professor, RCN
72
R Dhaker, Asst. Professor, RCN 73
Cont… causes
• HIV (human immunodeficiency virus), the virus
that causes AIDS (acquired immunodeficiency
syndrome) and is transmitted when an infected
person's blood or b fluids are introduced into the
bloodstream of a healthy person
• Childhood viruses - including measles and
mumps, chicken pox, rubella (German measles),
polio, and other viral illnesses,
• Enteroviruses
R Dhaker, Asst. Professor, RCN 74
Clinical Manifestation
• Child’s symptoms may depend on her situation—
– the part of the brain that is inflamed,
– the cause of the inflammation,
– the degree of inflammation, her age and other medical
problems she may have.
– But even children in the same situation may show
symptoms differently. Some of the most common
symptoms of encephalitis may include:
• fever
• Severe headache
• Bulging fontanel
R Dhaker, Asst. Professor, RCN 75
Cont… Clinical Manifestation
• Sensitivity to light
• Neck stiffness (nuchal rigidity)
– There may be stiffness of the limbs, slow movements, and
clumsiness
• Skin rashes
• Nausea/vomiting
• Loss of energy/appetite
• Changes in alertness (sleepiness)
• Confusion or hallucinations
• Disorientation ,
• Memory loss,
• Speech problems
• Hearing problems
• Problems walking
• Seizures
R Dhaker, Asst. Professor, RCN 76
• Emergency symptoms:
– Loss of consciousness
– Poor responsiveness, stupor, coma
– Muscle weakness or paralysis
– Seizures
– Severe headache
– Sudden change in mental functions, such as a flat
mood, impaired judgment, memory loss, or a lack of
interest in daily activities
R Dhaker, Asst. Professor, RCN 77
Cont… Clinical Manifestation
Warning signs of encephalitis in
children
• Fever with any of the following symptoms:
– Excessive drowsiness and sleepiness, out of
proportion to the fever
– Inconsolable, persistent irritability in an infant, out
of proportion to the fever
– Marked change in behaviour and personality
– Neck pain or stiffness, Seizures, focal neurological
deficits
R Dhaker, Asst. Professor, RCN 78
Diagnostic Evaluation
• The diagnosis of encephalitis is made after the
sudden or gradual onset of specific symptoms and
after diagnostic testing.
• obtains a complete medical history of child
– including his or her immunization history
– If child has recently had a cold or other respiratory
illness
– gastrointestinal illness and
– if the child has recently had a tick bite, has been around
pets or other animals.
R Dhaker, Asst. Professor, RCN 79
Cont… Diagnostic Evaluation
• Diagnostic test include:-
– X- Ray
– MRI
– CT Scan
– Blood tests
– Urine and stool tests
– Sputum culture
– EEG
– CSF Examination
– Brain biopsy. In rare cases, a biopsy of affected brain
tissue may be removed for diagnosis.
R Dhaker, Asst. Professor, RCN 80
Treatment
• Some kids with very mild encephalitis can be
monitored at home, but most will need care in a
hospital, usually in an intensive care unit (ICU).
• Carefully monitor their
–blood pressure,
–heart rate, and
–breathing,
–as well as their body fluids, to prevent further
swelling of the brain.
R Dhaker, Asst. Professor, RCN 81
Cont… Treatment
• Medication may include:-
– Antiviral medication
– Antibiotic medication
– Anti – Seizure medication
– Steroids
– Sedative to treat irritability.
– Acetaminophen for fever and headache.
R Dhaker, Asst. Professor, RCN 82
Prognosis
• The outcome varies
• Some case are mild and short and person fully
recovers.
• Other case are severe, and permanent impairment
or death is possible.
R Dhaker, Asst. Professor, RCN 83
Complication
• Permanent brain damage may occur in severe
case. It may affect-
– Hearing
– Memory
– Muscle control
– Sensation
– Speech
– Vision
R Dhaker, Asst. Professor, RCN 84
R Dhaker, Asst. Professor, RCN 85
Cerebral Palsy
R Dhaker, Asst. Professor, RCN 86
Introduction
• Cerebral palsy is a condition that affects
thousands of babies and children each year.
• It is not contagious, which means you can't catch
it from anyone who has it.
• The word cerebral means having to do with the
brain.
• The word palsy means a weakness or problem in
the way a person moves or positions his or her
body.
R Dhaker, Asst. Professor, RCN 87
• • Cerebral-
• “Latin Cerebrum”;
- Affected part of brain
R Dhaker, Asst. Professor, RCN 88
• “Palsy " –
Gr. para- beyond,
lysis - loosening
- Lack of muscle control
• In 1860s, known as
• "Cerebral Paralysis” or
“Little’s Disease”
• After an English surgeon
wrote the 1st medical
descriptions
R Dhaker, Asst. Professor, RCN 89
R Dhaker, Asst. Professor, RCN 90
Cont… Introduction
• Cerebral palsy (CP) is a disorder that affects muscle
tone, movement, and motor skills (the ability to move
in a coordinated and purposeful way).
R Dhaker, Asst. Professor, RCN 91
The word “cerebral” refers to the brain’s
cerebrum, which is the part of the brain that
regulates motor function. “Palsy” describes the
paralysis of voluntary movement in certain
parts of the body.
• Muscles are
unaffected
• Brain is unable
to send the
appropriate
signals necessary
to instruct
muscles when to
contract and relax
R Dhaker, Asst. Professor, RCN
92
Etiology
• Prenatal –
mother
• Iron def., poor –
nutrition
• Inf, UTI, high fever
• Chorioamniotis
• HTN, DM
• Teratogens
• Poor ANC
• Rh ?
• Twins
• Fetal vasculopathy
• Maternal
drugs/smoking(>30
) R Dhaker, Asst. Professor, RCN 93
•Perinatal
•Birth asphyxia
•Breach/vacuum/forc
•Premature /
LBW(>60/1000)
•IUGR
•Hyperbilirubenemia
•Intraventricular
hemorrrhage
•Sepsis, pneumonia,
meningitis
•Develop.
Malformation,
•abruptio
Postnatal
•CNS infections
•Head injuries
•Seizures
•Hypoxic
damage
•Hyperpyrexi a
damage
•Stroke
CHIEF CAUSE
• Severe deprivation of
oxygen or blood flow
to the brain
– Hypoxic-ischemic
encephalopathy
or
– intrapartal asphyxia
R Dhaker, Asst. Professor, RCN 94
R Dhaker, Asst. Professor, RCN 95
Incidence
• Time (% of cases)
• Prenatal (44%)
– First trimester
– Second trimester
• Labor and delivery (19%)
• Perinatal (8%)
• Childhood (5%)
• Not obvious (24%)
R Dhaker, Asst. Professor, RCN 96
• An incidence of cerebral palsy is
– 0 .6 to 4 per 1000 live birth and but high incidence
(27 time more) in low birth weight (<1.5 kg) and
– pre term born babies (< 7 month of pregnancy).
• Worldwide about 15 million and in India about 3
million are affected with cerebral palsy.
R Dhaker, Asst. Professor, RCN 97
Classification of CP
1. Neurologic deficits
2. Type of movement involved
3. Area of affected limbs
R Dhaker, Asst. Professor, RCN 98
Neurologic deficits
• Based on the
–extent of the damage
–area of brain damage
• Each type involves
the way a person
moves
R Dhaker, Asst. Professor, RCN 99
Cont…Neurologic deficits
1. PYRAMIDAL
• originates from the
motor areas of the
cerebral cortex
2. EXTAPYRAMIDAL
• basal ganglia and
cerebellum
3. MIXED
R Dhaker, Asst. Professor, RCN 100
Type of movement involved
R Dhaker, Asst. Professor, RCN 101
Cont… Type of movement involved
1. Spastic CP
2. Athethoid CP
3. Ataxic CP
4. Spastic &
Athethoid CP
R Dhaker, Asst. Professor, RCN 102
R Dhaker, Asst. Professor, RCN 103
R Dhaker, Asst. Professor, RCN 104
Area of affected limbs
• Paraplegia
• Diplegia
• Hemiplegia
• Quadriplegia
• Monoplegia -one limb (extremely rare)
• Triplegia -three limbs (extremely rare)
R Dhaker, Asst. Professor, RCN 105
R Dhaker, Asst. Professor, RCN
106
R Dhaker, Asst. Professor, RCN 107
Clinical Manifestation
R Dhaker, Asst. Professor, RCN 108
R Dhaker, Asst. Professor, RCN 109
Late infancy
• Inability to perform motor skills as indicated:
– Control hand grasp by 3 months
– Rolling over by 5 months
– Independent sitting by 7 months
• Abnormal Developmental Patterns:
– Hand preference by 12 months
– Excessive arching of back
– Log rolling
– Abnormal or prolonged parachute response
R Dhaker, Asst. Professor, RCN 110
Associated Problems Of
Cerebral Palsy
• Hearing and visual problems
• Sensory integration problems
• Failure-to-thrive, Feeding problems
• Behavioral/emotional difficulties,
• Communication disorders
R Dhaker, Asst. Professor, RCN 111
Cont… Associated Problems Of Cerebral Palsy
• Bladder and bowel control problems, digestive
problems (gastroesophageal reflux)
• Skeletal deformities, dental problems
• Mental retardation and learning disabilities in
some
• Seizures/ epilepsy
R Dhaker, Asst. Professor, RCN 112
Diagnostic Evaluation
• Physical evaluation, Interview
• MRI, CT Scan EEG
• Laboratory and radiologic work up
• Assessment tools
–i.e. Development Motor Skills,
–Denver Test II
R Dhaker, Asst. Professor, RCN 113
• History Taking
– Include all that may predispose an infant to brain
damage or CP
– Risk factors
– Psychosocial factors
– Family adaptation
• Child's Health History
– Often admitted to hospitals for corrective surgeries and
other complications.
– Respiratory status
– Motor function
– Presence of fever
– Feeding and weight loss
– Any changes in physical state -Medical regimen
R Dhaker, Asst. Professor, RCN 114
• Physical Examination
• P osturing / Poor muscle control and strength
• O ropharyngeal problems
• S trabismus/ Squint
• T one (hyper-, hypotonia)
• E volutional maldevelopment
• R eflexes (e.g. increaseddeep tendon)
*Abnormalities 4/6 strongly point to CP
R Dhaker, Asst. Professor, RCN 115
Treatment
• No treatment to cure cerebral palsy.
• Brain damage cannot be corrected.
• Crucial for children with CP:
– Early Identification;
– Multidisciplinary Care; and
– Support
R Dhaker, Asst. Professor, RCN 116
• General management
– Proper nutrition and personal care
• Pharmacologic
– Botox, Intrathecal, Baclofen
• control muscle spasms and seizures,
– Glycopyrrolate -control drooling
– Pamidronate -may help with osteoporosis.
• Surgery
– To loosen joints,
– Relieve muscle tightness,
– Straightening of different twists or unusual curvatures
of leg muscles
– Improve the ability to sit, stand, and walk.
R Dhaker, Asst. Professor, RCN 117
• Physical Aids
– Orthosis, braces and splints
– Positioning devices
– Walkers, special scooters, wheelchairs
• Special Education
• Rehabilitation Services- Speech and
occupational therapies
• Family Services -Professional support
• Other Treatment
– Therapeutic electrical stimulation, -Acupuncture,
– Hyperbaric therapy
– Massage Therapy might help
R Dhaker, Asst. Professor, RCN 118
• Physical Therapy
• Sitting
– Vertical head control and control of head and trunk.
• Standing and walking
– Establish an equal distribution of weight on each foot,
train to use steps or inclines
• Prone Development
• Supine Development
– Head control on supine and positions
R Dhaker, Asst. Professor, RCN 119
Neural Tube
Defects
120R Dhaker, Asst. Professor, RCN
Introduction
• Neural tube defects are birth defects of the
– brain,
– spine, or
– spinal cord.
• NTDs are one of the most common birth defects,
affecting over 300,000 births each year worldwide.
121R Dhaker, Asst. Professor, RCN
• The neural tube forms by the 28th day after
conception
R Dhaker, Asst. Professor, RCN 122
• In the 3rd week of pregnancy called gastrulating,
specialized cells on the dorsal side of the embryo
begin to change shape and form the neural tube.
When the neural tube does not close completely,
an NTD develops.
123R Dhaker, Asst. Professor, RCN
124R Dhaker, Asst. Professor, RCN
Classification
• NTDs can be classified, based on embryological
considerations and the presence or absence of
exposed neural tissue:-
• Open NTD
• Close NTD
125R Dhaker, Asst. Professor, RCN
• Open NTDs frequently involve the entire CNS
• (eg, associated hydrocephalus, Chiari II
malformation) and are due to failure of primary
neurulation. Neural tissue is exposed with
associated cerebrospinal fluid (CSF) leakage.
126R Dhaker, Asst. Professor, RCN
• Closed NTDs are localized and confined to the
spine (brain rarely affected) and result from a
defect in secondary neurulation. Neural tissue is
not exposed and the defect is fully epithelialized,
although the skin covering the defect may be
dysplastic.
127R Dhaker, Asst. Professor, RCN
• Cranial presentations include the following
• Anencephaly
• Encephalocele (meningocele or
meningomyelocele)
• Craniorachischisis totalis
• Congenital dermal sinus
128R Dhaker, Asst. Professor, RCN
• Spinal presentations include the following
• Spina bifida aperta (cystica)
• Myelomeningocele (see following images)
• Meningocele
• Myeloschisis
• Congenital dermal sinus
• Lipomatous malformations (lipomyelomeningoceles)
• Split-cord malformations
• Diastematomyelia
• Diplomyelia
• Caudal agenesis
129
R Dhaker, Asst. Professor, RCN
Etiology
• The chance that a pregnancy will be affected by a
neural tube defect is less than one in 1000.
• However, there are a number of factors that will
increase this risk. The main one is a close family
history of neural tube defects.
R Dhaker, Asst. Professor, RCN 130
Cont… etiology
• The majority of NTDs are etiologically complex.
• Vitamin B9 and vitamin B12 are very important in
reducing the occurrences of NTDs.
• Genetic factor
• Environmental factor
– folic acid deficiency,
– anti-seizure medications
– uncontrolled diabetes,
– alcohol, obesity, and
– increased body temperature
R Dhaker, Asst. Professor, RCN 131
Spina bifida
R Dhaker, Asst. Professor, RCN 132
• Spina bifida is a birth defect where there is
incomplete closing of the backbone and
membranes around the spinal cord.
• The most common location is the
– lower back, but
– in rare cases it may be the middle back or neck
R Dhaker, Asst. Professor, RCN 133
• Spina bifida is one of the most common birth
defects, with an average worldwide incidence of
one to two cases per 1000 births, but certain
populations have a significantly greater risk.
R Dhaker, Asst. Professor, RCN 134
CAUSES
• Maternal diabetes
• Family history
• Obesity
• Increased body temperature from fever or external
sources such as hot tubs and electric blankets may
increase the chances of delivery of a baby with a
spina bifida.
• Medications such as some anticonvulsants.
• Pregnant women taking Valproic acid have an
increased risk of having children with spina bifida
• Genetic basis.
• Folic acid deficiency
135R Dhaker, Asst. Professor, RCN
TYPES:
• Spina bifida malformations fall into three
categories:
spina bifida occulta
 spina bifida cystica with meningocele
spina bifida cystica with myelomeningocele.
(The most common location of the malformations
is the lumbar and sacral areas)
136R Dhaker, Asst. Professor, RCN
R Dhaker, Asst. Professor, RCN 137
Spina bifida occulta
• Occulta is Latin for "hidden".
• This is the mildest form of spina
bifida.
• In occulta, the outer part of some
of the vertebrae is not
completely closed.
• The splits in the vertebrae are so
small that the spinal cord does
not protrude.
138R Dhaker, Asst. Professor, RCN
Cont… Spina bifida occulta
• The skin at the site of the lesion may be normal, or it
may have some hair growing from it; there may be a
dimple in the skin, or a birthmark.
• The incidence of spina bifida occulta is approximately
10% of the population, and most people are diagnosed
incidentally from spinal X-rays
R Dhaker, Asst. Professor, RCN 139
Meningocele:
• The least common form of
spina bifida is a posterior
meningocele
(or meningeal cyst).
• In this form, the vertebrae
develop normally, but
the meninges are forced
into the gaps between the
vertebrae.
140R Dhaker, Asst. Professor, RCN
Myelomeningocele
• This type of spina bifida often results in the most
severe complications.
• In individuals with myelomeningocele, the
unfused portion of the spinal column allows the
spinal cord to protrude through an opening.
• The meningeal membranes that cover the spinal
cord form a sac enclosing the spinal elements.
141R Dhaker, Asst. Professor, RCN
• Spina bifida with myeloschisis is the most severe
form of myelomeningocele. In this type, the
involved area is represented by a flattened, plate-
like mass of nervous tissue with no overlying
membrane.
• The exposure of these nerves and tissues make the
baby more prone to life-threatening infections
such as meningitis.
R Dhaker, Asst. Professor, RCN 142
R Dhaker, Asst. Professor, RCN 143

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Disease of central nervous system...asst

  • 1. Disease of Central Nervous System Rahul Dhaker Asst. Professor Ramsheni College of Nursing, Bhilwara- 311001, Rajasthan 1R Dhaker, Asst. Professor, RCN
  • 2. Introduction • Disease of nervous system are fairly common in children. • Almost 20 to 30 percent of children are victims of neurological illnesses. • There are major contributors to childhood morbidity and disability. • Neurological disorder of infancy and childhood are different than in adults. 2R Dhaker, Asst. Professor, RCN
  • 3. Cont… Introduction • Neurological symptoms are also found in association with various systemic disease. • Common disease condition in children involvement central nervous system- • congenital malformation • Prenatal problem • Developmental disability • CNS infection • Craniocerebral trauma • Brain tumor. 3R Dhaker, Asst. Professor, RCN
  • 4. • Diagnostic test – • CT Scan • MRI • PET Scan • SPET scan (Single-photon emission computed tomography) • Cerebral angiography • Myelography • Neuropsychological testing. • EEG • Ultrasonography etc. Cont… Introduction 4R Dhaker, Asst. Professor, RCN
  • 5. PET Scan 5R Dhaker, Asst. Professor, RCN
  • 6. • PET scan to inspect the blood flow, oxygen intake, and metabolism of your organs and tissues. PET scans are most commonly used to detect: • Cancer • Heart Problems • Brain Disorders • Problems With The Central Nervous System 6R Dhaker, Asst. Professor, RCN
  • 7. 7R Dhaker, Asst. Professor, RCN
  • 8. Neonatal Convulsion • Convulsion is the involuntary contraction or series of contraction of the voluntary muscles. • It occurs due to disturbance of the brain function resulting from- – abnormal excessive electrical discharge from brain. 8R Dh@ker, Asst. Professor
  • 9. Cont… • It is manifested by involuntary, motor, sensory, autonomic or psychic phenomenon, alone or in combination. • It may be associated with alteration of level of consciousness. • Convulsion is also term as seizure. • Convulsion are more commonly found in infants and children. 9R Dh@ker, Asst. Professor
  • 10. • It is more commonly found alone with – cerebral palsy (35%) and – Mental retardation (20%). • 57.5/1,000 in infants with birth weights <1,500 g • 2.8/1,000 in infants weighing between 2,500 and 3,999 g have seizures. Cont… 10R Dh@ker, Asst. Professor
  • 11. • Neonatal convulsion are common life threatening emergency in the new born due to cerebral or biochemical abnormality. • Preterm and LBW babies are more prone to this problem. • Newborn babies do not manifest febrile convulsion. R Dh@ker, Asst. Professor 11 Cont…
  • 12. Etiology: (1-4) days 1.Hypoxic-ischemic encephalopathy 2.Drug withdrawal or toxicity 3.Intraventricular hemorrhage 4.Acute metabolic disorders: •hypocalcemia, •hypoglycemia, •hypomagnesemia and •hypo or hyper natremia) 5.Inborn errors of metabolism (ex. Galactosemia) 6.Pyridoxine deficiency. 12 R Dh@ker, Asst. Professor
  • 13. (4-14) days 1. Infection ( meningitis , encephalitis) 2. Metabolic ( hypocalcemia, persistent hypoglycemia) 3. Benign neonatal convulsion. 4.Kernectirus 5. Drug withdrawal 6. Developmental delay, epilepsy, neonatal diabetes. Cont…Etiology: 13R Dh@ker, Asst. Professor
  • 14. Cont…Etiology: (2-8) wks 1. Infection ( meningitis , encephalitis) 2. Head injury( subdural hemorrhage, child abuse) 3. Inherited disorders of metabolism (ex. Aminoaciduria). 4. Malformation of cortical development (ex lissencephaly) 5. Tuberous sclerosis. 6. Sturge weber syndrome. 14R Dh@ker, Asst. Professor
  • 15. Put them all together.. 1. Hypoxic ischemic encephalopathy (50-60)%.. Most common cause of neonatal seizure. 2. Vascular events (10 -20) % 3. Intracranial infection (5-10)% 4. Brain malformation (5-10)% 5. Metabolic disturbances (↓glycemia, ↓Ca, ↓Mg, ↓↑Na) 6. Drugs ( withrawal or toxicity) 7. Neonatal seizure syndromes : rare 8. Preinatal complication 9. Developmental neurological problem 15
  • 16. 16 Febrile Convulsion  Febrile convulsions, the most common seizure disorder during childhood.  Occurring between 6 months and 6 years.  Precipitated by fever from:  infection/inflammation/metabolic disorders .  It is not a form of epilepsy because brain is normal. R Dh@ker, Asst. Professor
  • 17. Type of Febrile Convulsion • Typical febrile convulsion • Atypical febrile convulsion 17R Dh@ker, Asst. Professor
  • 18. Typical febrile convulsion • These are generalized rather than focal and last less than 10 minutes. • It is usually found in children between 6 month to 5 year of age. • The fits occur within 24 hours of the onset of fever and usually single/ febrile episode. • There is no recurrence before 12 to 18 hours of attack. • No paralysis of limb. R Dh@ker, Asst. Professor 18
  • 19. Cont…Typical febrile convulsion • CSF and EEG are normal after the attack. • Family history of convulsion is frequently present. • The condition may have genetic predisposition or may be due to immature neuronal membrane response to rise of body temperature. R Dh@ker, Asst. Professor 19
  • 20. Atypical febrile convulsion • They predispose to idiopathic epilepsy. • The children may have focal convulsion of more than 20 minute duration even without significant fever. • They may have abnormal EEG for two weeks after attack. R Dh@ker, Asst. Professor 20
  • 21. Diagnostic evaluation • Family History of convulsion • History of Maternal drug addiction and infections • Time of onset of convulsion • Blood Examination for – Calcium – Sugar – phosphorus • Lumper puncture – CSF Examination • EEG • CT Scan • MRI • ECG • Serology for STORCH R Dh@ker, Asst. Professor 21
  • 22. R Dh@ker, Asst. Professor 22
  • 23. Management • Management of febrile convulsion should be done- – To control convulsion – To reduce increased body temperature – To treat the cause of fever, usually ARI R Dh@ker, Asst. Professor 23
  • 24. Cont…Management • Anticonvulsive drug are indicated in prolonged convulsion. – Diazepam 0.3mg/kg IV day – Phenoberbital 5mg/kg IM/day can be administered. – Sodium valporate 10 to 20mg.kg/day • Antipyretic – Prarcetamol – Mefanamic acid – Tepid sponge should be given to treat fever. • Hydration and nutrition status to be maintained. • clearing of airway R Dh@ker, Asst. Professor 24
  • 25. • Oxygen therapy may be needed for some children • Rest • Comfortable position • Hygienic measure to be provided. • Explanation and emotional support to the parent are important and along with necessary health education. • Duration of therapy can be 1 to 2 year or upto 5 years. R Dh@ker, Asst. Professor 25 Cont…Management
  • 26. Prognosis • In typical febrile convulsion prognosis is good. • In atypical type, there is chance of development of complication like- – Intellectual impairment – Behavioral problem – Epilepsy • Chance of recurrence is about 30 to 80%. R Dh@ker, Asst. Professor 26
  • 27. R Dh@ker, Asst. Professor 27
  • 28. NEONATAL SEIZURES R Dh@ker, Asst. Professor 28
  • 30. 30R Dhaker, Asst. Professor, RCN
  • 31. Causes of Meningitis - Bacterial - Viral - Fungal - Ricketsial (Rocky mountain spotted fever) - Parasitic/ protozoal - Physical injury - Cancer - Certain drugs ( mainly, NSAID’S)  Severity/treatment of illnesses differ depending on the cause. Thus, it is important to know the specific cause of meningitis. 31R Dhaker, Asst. Professor, RCN
  • 32. 32R Dhaker, Asst. Professor, RCN
  • 33. 33R Dhaker, Asst. Professor, RCN
  • 34. Clinical Manifestation • The 3 classic symptoms (less likely in younger children): • Fever • Headache • Meningeal signs 34R Dhaker, Asst. Professor, RCN
  • 35. Symptoms in neonates: • Poor feeding • Lethargy • Irritability • Apnea • Listlessness • Apathy • Fever • Hypothermia • Seizures • Jaundice • Bulging fontanelle • Pallor • Shock • Hypotonia • Shrill cry • Hypoglycemia • Intractable metabolic acidosis 35R Dhaker, Asst. Professor, RCN
  • 36. Symptoms in infants and children: • Nuchal rigidity • Opisthotonos • Bulging fontanelle • Convulsions • Photophobia • Headache • Alterations of the sensorium • Irritability • Lethargy • Anorexia • Nausea • Vomiting • Coma • Fever (generally present, although some severely ill children present with hypothermia) 36R Dhaker, Asst. Professor, RCN
  • 37. 37R Dhaker, Asst. Professor, RCN
  • 38. 38R Dhaker, Asst. Professor, RCN
  • 39. Diagnosis • History of illness • Definitive diagnosis is based on the following: – lumbar puncture-CSF Examination – Meningeal inflammation demonstrated by increased pleocytosis, elevated protein level, and low glucose level in the CSF 39R Dhaker, Asst. Professor, RCN
  • 40. CSF findings in different forms of meningitis Type of meningitis Glucose Protein Cells Acute bacterial low high PMNs often > 300/mm³ Acute viral normal normal or high mononuclear < 300/mm³ Tuberculous low high mononuclear and PMNs, < 300/mm³ Fungal low high < 300/mm³ Malignant low high usually mononuclear 40R Dhaker, Asst. Professor, RCN
  • 41. Cont… Diagnosis • Bacterial meningitis score • Components of the bacterial meningitis score are as follows: – Positive CSF Gram stain – CSF absolute neutrophil count 1000/µL or higher – CSF protein level 80 mg/dL or higher – Peripheral blood absolute neutrophil count 10,000/µL or higher – History of seizure before or at the time of presentation 41R Dhaker, Asst. Professor, RCN
  • 42. • Specific • Hematologic – Blood culture • radiographic – CT Scan – MRI Cont… Diagnosis 42R Dhaker, Asst. Professor, RCN
  • 43. Management • IV antibiotics are required; if cause is unknown, agents can be based on child’s age, as follows: – < 30 days, ampicillin and an aminoglycoside or a cephalosporin – 30-60 days, ampicillin and a cephalosporin; because Streptococcus pneumoniae may occur in this age range, consider vancomycin instead of ampicillin – In older children, a cephalosporin or ampicillin plus chloramphenicol with vancomycin (needs to be added secondary to the possibility of S pneumoniae). 43R Dhaker, Asst. Professor, RCN
  • 44. • Guidelines and recommendations • Vancomycin plus either ceftriaxone or cefotaxime • Duration of therapy: – Neisseria meningitidis - 7 days – Haemophilus influenzae - 7 days – Streptococcus pneumoniae - 10-14 days – S agalactiae (GBS) - 14-21 days – Aerobic gram-negative bacilli - 21 days or 2 weeks beyond the first sterile culture (whichever is longer) – Listeria monocytogenes - 21 days or longer 44R Dhaker, Asst. Professor, RCN
  • 45. • Duration of therapy should not be shorter than 5 days for meningococcus, 10 days for H influenzae, and 14 days for S pneumoniae. 45R Dhaker, Asst. Professor, RCN
  • 46. Treatment • Initial till results of C/S are known • Probable/Proved Meningococci • Ampicillin 300mg/kg/day+ • Chloramphenicol 75-100mg.kg/day • Penicillins 2-5 lac units /kg/day 46R Dhaker, Asst. Professor, RCN
  • 47. Treatment • Probable H.Influenzae • Probable E.Coli • Ampicillin + chloramphenicol or 3rd generation cephalosporin (cefotaxime 200mg/kg/day) • Ampicillin + gentamycin 200mg/kg+2.5-4 mg/kg IV 12hrly 47R Dhaker, Asst. Professor, RCN
  • 48. Treatment • Probable group B streptococci • Penicillin 50,000i.u/kgI.V/4 hourly. 48R Dhaker, Asst. Professor, RCN
  • 49. Other Drugs available Anti-microbials • Ceftriaxone • Cefotaxime • Penicillin G • Vancomycin • Ampicillin • Gentamicin Anti-Virals • Acyclovir • Ganciclovir (>3mths) Anti-fungals Amphotericin B Fluconazole 49R Dhaker, Asst. Professor, RCN
  • 50. 50R Dhaker, Asst. Professor, RCN
  • 51. Epilepsy 51R Dhaker, Asst. Professor, RCN
  • 52. Introduction • Epilepsy is the most common childhood brain disorder. • About two-thirds of all children with epilepsy outgrow their seizures by the time they are teenagers. • If you have a child with epilepsy, you're not alone — 3 million children have this disorder. 52R Dhaker, Asst. Professor, RCN
  • 53. • Epilepsy is a disease of the central nervous system in which electrical signals of the Brain misfire. • These disruptions cause temporary communication problems between nerve cells, leading to seizures. • Epilepsy knows no geographical, racial or social boundaries. About 50 million people in World have Epilepsy. 53R Dhaker, Asst. Professor, RCN
  • 54. Epilepsy Epilepsy is a brain disorder in which clusters of nerve cells, or neurons, in the brain sometimes signal abnormally. It produces changes in a person's • movement, • behaviour or • consciousness 54R Dhaker, Asst. Professor, RCN
  • 55. Causes of Epilepsy • Some people have a specific problem in the brain that causes the seizures. These include: • infectious illness (such as meningitis or encephalitis) • brain malformation during pregnancy • trauma to the brain (including lack of oxygen) due to an accident before, during, or after birth or later in childhood • metabolic disorders (chemical imbalances in the brain) • brain tumors • blood vessel malformation • strokes • chromosome disorders 56R Dhaker, Asst. Professor, RCN
  • 56. • Seizures in children have many causes. Common causes of childhood seizures or epilepsy include • fever (these are called febrile seizures) • genetic causes • head injury • infections of the brain and its coverings • lack of oxygen to the brain • hydrocephalus (excess water in the brain cavities) • disorders of brain development 57R Dhaker, Asst. Professor, RCN
  • 57. • The causes of epilepsy in childhood vary. In about ⅔ of cases, it is unknown. • Unknown 67.6% • Congenital 20% • Trauma 4.7% • Infection 4% • Stroke 1.5% • Tumor 1.5% • Degenerative .7% 58R Dhaker, Asst. Professor, RCN
  • 58. Classification of Seizures ILAE Classification (1981) I. Partial (Focal)seizures A. Simple partial seizures B. Complex Partial Seizures C. Partial Seizures evolving to secondary generalized seizures (tonic-clonic, tonic or clonic) II. Generalized seizures (Convulsive and non-convulsive) A. Absence seizures i) Typical ii) Atypical B. Myoclonic seizures C. Clonic seizures D. Tonic seizures E. Tonic-Clonic seizures F. Atonic seizures (Combinations may occur: myoclonic and atonic or myoclonic and tonic) III. Unclassified epileptic seizures 59R Dhaker, Asst. Professor, RCN
  • 59. Diagnosis of Epilepsy Thorough History taking : From patients From reliable valid informants From observer (who observed seizures) Physical Examination: Specially neurological system Higher Psychic function Laboratory Investigation: S. Electrolytes, S. Prolactin, Blood sugar, CBC, LFT, RFT, CSF study Imaging: EEG, CT Scan of Brain, MRI of Brain, PET, SPECT. Polysomnography 60R Dhaker, Asst. Professor, RCN
  • 60. Management • Epilepsy affects every child differently depending on: • Age • Types of seizure • Response to treatment • Having other health issues, etc. 62R Dhaker, Asst. Professor, RCN
  • 61. Cont… Management • Commonly drugs are used- – Phenoberbital- 3 to 5 mg/kg/d or 1 or 2 divided dose – Diphenylhydantion- 5 to 8 mg/kg/d in 2 divided dose. – Carbamazepin – 10 to 20 mg/kg/d in 2 to 3 divided dose. – Diazepan- 0.2 mg/kg/d IV – Sodium Valporate- 15 to 20 mg/kg/d in 3 to 4 divided dose. • Usually single drug is used but if fails to relieve seizure than addition of secondary drug is needed. 63R Dhaker, Asst. Professor, RCN
  • 62. Surgical Management • Neurosurgical is indicated in some cases of convulsion disorder, especially anatomical lesion like – Brain tumor, hematoma etc. 64R Dhaker, Asst. Professor, RCN
  • 63. 65R Dhaker, Asst. Professor, RCN
  • 64. Encephalitis R Dhaker, Asst. Professor, RCN 66
  • 65. Term • Encephalitis: Infectious process & inflammatory response limited to brain parenchyma. • Meningoencephalitis: Meninges + brain • Encephalomyelitis: Brain + spinal cord • Encephalomyeloradicilitis: Brain + spinal cord + nerve root R Dhaker, Asst. Professor, RCN 67
  • 66. Introduction • In encephalitis, there is inflammation in the brain tissues. • In most cases, this inflammation is caused by a virus • In which children become more sleepy or drowsy than usual. • This can sometimes be subtle and noticed only when there is a change in behavior. R Dhaker, Asst. Professor, RCN 68
  • 67. Definition R Dhaker, Asst. Professor, RCN 69
  • 68. R Dhaker, Asst. Professor, RCN 70 Two Components: 1. Inflammation of brain, and 2. Dysfunction of brain.
  • 69. Definition • Encephalitis is an acute inflammation (swelling) of the brain usually resulting from either a viral infection or due to the body's own immune system mistakenly attacking brain tissue. • In medicine, "acute" means it comes on abruptly and develops rapidly; it usually requires urgent care. R Dhaker, Asst. Professor, RCN 71
  • 70. Causes of Encephalitis • Primary (infectious) encephalitis can be split into three main categories of viruses: • Common viruses - including HSV (herpes simplex virus) and EBV (Epstein-Barr virus) • Arboviruses (spread by mosquitoes, ticks, and other insects) - including Japanese encephalitis, West Nile encephalitis, and tick-borne encephalitis R Dhaker, Asst. Professor, RCN 72
  • 71. R Dhaker, Asst. Professor, RCN 73
  • 72. Cont… causes • HIV (human immunodeficiency virus), the virus that causes AIDS (acquired immunodeficiency syndrome) and is transmitted when an infected person's blood or b fluids are introduced into the bloodstream of a healthy person • Childhood viruses - including measles and mumps, chicken pox, rubella (German measles), polio, and other viral illnesses, • Enteroviruses R Dhaker, Asst. Professor, RCN 74
  • 73. Clinical Manifestation • Child’s symptoms may depend on her situation— – the part of the brain that is inflamed, – the cause of the inflammation, – the degree of inflammation, her age and other medical problems she may have. – But even children in the same situation may show symptoms differently. Some of the most common symptoms of encephalitis may include: • fever • Severe headache • Bulging fontanel R Dhaker, Asst. Professor, RCN 75
  • 74. Cont… Clinical Manifestation • Sensitivity to light • Neck stiffness (nuchal rigidity) – There may be stiffness of the limbs, slow movements, and clumsiness • Skin rashes • Nausea/vomiting • Loss of energy/appetite • Changes in alertness (sleepiness) • Confusion or hallucinations • Disorientation , • Memory loss, • Speech problems • Hearing problems • Problems walking • Seizures R Dhaker, Asst. Professor, RCN 76
  • 75. • Emergency symptoms: – Loss of consciousness – Poor responsiveness, stupor, coma – Muscle weakness or paralysis – Seizures – Severe headache – Sudden change in mental functions, such as a flat mood, impaired judgment, memory loss, or a lack of interest in daily activities R Dhaker, Asst. Professor, RCN 77 Cont… Clinical Manifestation
  • 76. Warning signs of encephalitis in children • Fever with any of the following symptoms: – Excessive drowsiness and sleepiness, out of proportion to the fever – Inconsolable, persistent irritability in an infant, out of proportion to the fever – Marked change in behaviour and personality – Neck pain or stiffness, Seizures, focal neurological deficits R Dhaker, Asst. Professor, RCN 78
  • 77. Diagnostic Evaluation • The diagnosis of encephalitis is made after the sudden or gradual onset of specific symptoms and after diagnostic testing. • obtains a complete medical history of child – including his or her immunization history – If child has recently had a cold or other respiratory illness – gastrointestinal illness and – if the child has recently had a tick bite, has been around pets or other animals. R Dhaker, Asst. Professor, RCN 79
  • 78. Cont… Diagnostic Evaluation • Diagnostic test include:- – X- Ray – MRI – CT Scan – Blood tests – Urine and stool tests – Sputum culture – EEG – CSF Examination – Brain biopsy. In rare cases, a biopsy of affected brain tissue may be removed for diagnosis. R Dhaker, Asst. Professor, RCN 80
  • 79. Treatment • Some kids with very mild encephalitis can be monitored at home, but most will need care in a hospital, usually in an intensive care unit (ICU). • Carefully monitor their –blood pressure, –heart rate, and –breathing, –as well as their body fluids, to prevent further swelling of the brain. R Dhaker, Asst. Professor, RCN 81
  • 80. Cont… Treatment • Medication may include:- – Antiviral medication – Antibiotic medication – Anti – Seizure medication – Steroids – Sedative to treat irritability. – Acetaminophen for fever and headache. R Dhaker, Asst. Professor, RCN 82
  • 81. Prognosis • The outcome varies • Some case are mild and short and person fully recovers. • Other case are severe, and permanent impairment or death is possible. R Dhaker, Asst. Professor, RCN 83
  • 82. Complication • Permanent brain damage may occur in severe case. It may affect- – Hearing – Memory – Muscle control – Sensation – Speech – Vision R Dhaker, Asst. Professor, RCN 84
  • 83. R Dhaker, Asst. Professor, RCN 85
  • 84. Cerebral Palsy R Dhaker, Asst. Professor, RCN 86
  • 85. Introduction • Cerebral palsy is a condition that affects thousands of babies and children each year. • It is not contagious, which means you can't catch it from anyone who has it. • The word cerebral means having to do with the brain. • The word palsy means a weakness or problem in the way a person moves or positions his or her body. R Dhaker, Asst. Professor, RCN 87
  • 86. • • Cerebral- • “Latin Cerebrum”; - Affected part of brain R Dhaker, Asst. Professor, RCN 88 • “Palsy " – Gr. para- beyond, lysis - loosening - Lack of muscle control
  • 87. • In 1860s, known as • "Cerebral Paralysis” or “Little’s Disease” • After an English surgeon wrote the 1st medical descriptions R Dhaker, Asst. Professor, RCN 89
  • 88. R Dhaker, Asst. Professor, RCN 90
  • 89. Cont… Introduction • Cerebral palsy (CP) is a disorder that affects muscle tone, movement, and motor skills (the ability to move in a coordinated and purposeful way). R Dhaker, Asst. Professor, RCN 91 The word “cerebral” refers to the brain’s cerebrum, which is the part of the brain that regulates motor function. “Palsy” describes the paralysis of voluntary movement in certain parts of the body.
  • 90. • Muscles are unaffected • Brain is unable to send the appropriate signals necessary to instruct muscles when to contract and relax R Dhaker, Asst. Professor, RCN 92
  • 91. Etiology • Prenatal – mother • Iron def., poor – nutrition • Inf, UTI, high fever • Chorioamniotis • HTN, DM • Teratogens • Poor ANC • Rh ? • Twins • Fetal vasculopathy • Maternal drugs/smoking(>30 ) R Dhaker, Asst. Professor, RCN 93 •Perinatal •Birth asphyxia •Breach/vacuum/forc •Premature / LBW(>60/1000) •IUGR •Hyperbilirubenemia •Intraventricular hemorrrhage •Sepsis, pneumonia, meningitis •Develop. Malformation, •abruptio Postnatal •CNS infections •Head injuries •Seizures •Hypoxic damage •Hyperpyrexi a damage •Stroke
  • 92. CHIEF CAUSE • Severe deprivation of oxygen or blood flow to the brain – Hypoxic-ischemic encephalopathy or – intrapartal asphyxia R Dhaker, Asst. Professor, RCN 94
  • 93. R Dhaker, Asst. Professor, RCN 95
  • 94. Incidence • Time (% of cases) • Prenatal (44%) – First trimester – Second trimester • Labor and delivery (19%) • Perinatal (8%) • Childhood (5%) • Not obvious (24%) R Dhaker, Asst. Professor, RCN 96
  • 95. • An incidence of cerebral palsy is – 0 .6 to 4 per 1000 live birth and but high incidence (27 time more) in low birth weight (<1.5 kg) and – pre term born babies (< 7 month of pregnancy). • Worldwide about 15 million and in India about 3 million are affected with cerebral palsy. R Dhaker, Asst. Professor, RCN 97
  • 96. Classification of CP 1. Neurologic deficits 2. Type of movement involved 3. Area of affected limbs R Dhaker, Asst. Professor, RCN 98
  • 97. Neurologic deficits • Based on the –extent of the damage –area of brain damage • Each type involves the way a person moves R Dhaker, Asst. Professor, RCN 99
  • 98. Cont…Neurologic deficits 1. PYRAMIDAL • originates from the motor areas of the cerebral cortex 2. EXTAPYRAMIDAL • basal ganglia and cerebellum 3. MIXED R Dhaker, Asst. Professor, RCN 100
  • 99. Type of movement involved R Dhaker, Asst. Professor, RCN 101
  • 100. Cont… Type of movement involved 1. Spastic CP 2. Athethoid CP 3. Ataxic CP 4. Spastic & Athethoid CP R Dhaker, Asst. Professor, RCN 102
  • 101. R Dhaker, Asst. Professor, RCN 103
  • 102. R Dhaker, Asst. Professor, RCN 104
  • 103. Area of affected limbs • Paraplegia • Diplegia • Hemiplegia • Quadriplegia • Monoplegia -one limb (extremely rare) • Triplegia -three limbs (extremely rare) R Dhaker, Asst. Professor, RCN 105
  • 104. R Dhaker, Asst. Professor, RCN 106
  • 105. R Dhaker, Asst. Professor, RCN 107
  • 106. Clinical Manifestation R Dhaker, Asst. Professor, RCN 108
  • 107. R Dhaker, Asst. Professor, RCN 109
  • 108. Late infancy • Inability to perform motor skills as indicated: – Control hand grasp by 3 months – Rolling over by 5 months – Independent sitting by 7 months • Abnormal Developmental Patterns: – Hand preference by 12 months – Excessive arching of back – Log rolling – Abnormal or prolonged parachute response R Dhaker, Asst. Professor, RCN 110
  • 109. Associated Problems Of Cerebral Palsy • Hearing and visual problems • Sensory integration problems • Failure-to-thrive, Feeding problems • Behavioral/emotional difficulties, • Communication disorders R Dhaker, Asst. Professor, RCN 111
  • 110. Cont… Associated Problems Of Cerebral Palsy • Bladder and bowel control problems, digestive problems (gastroesophageal reflux) • Skeletal deformities, dental problems • Mental retardation and learning disabilities in some • Seizures/ epilepsy R Dhaker, Asst. Professor, RCN 112
  • 111. Diagnostic Evaluation • Physical evaluation, Interview • MRI, CT Scan EEG • Laboratory and radiologic work up • Assessment tools –i.e. Development Motor Skills, –Denver Test II R Dhaker, Asst. Professor, RCN 113
  • 112. • History Taking – Include all that may predispose an infant to brain damage or CP – Risk factors – Psychosocial factors – Family adaptation • Child's Health History – Often admitted to hospitals for corrective surgeries and other complications. – Respiratory status – Motor function – Presence of fever – Feeding and weight loss – Any changes in physical state -Medical regimen R Dhaker, Asst. Professor, RCN 114
  • 113. • Physical Examination • P osturing / Poor muscle control and strength • O ropharyngeal problems • S trabismus/ Squint • T one (hyper-, hypotonia) • E volutional maldevelopment • R eflexes (e.g. increaseddeep tendon) *Abnormalities 4/6 strongly point to CP R Dhaker, Asst. Professor, RCN 115
  • 114. Treatment • No treatment to cure cerebral palsy. • Brain damage cannot be corrected. • Crucial for children with CP: – Early Identification; – Multidisciplinary Care; and – Support R Dhaker, Asst. Professor, RCN 116
  • 115. • General management – Proper nutrition and personal care • Pharmacologic – Botox, Intrathecal, Baclofen • control muscle spasms and seizures, – Glycopyrrolate -control drooling – Pamidronate -may help with osteoporosis. • Surgery – To loosen joints, – Relieve muscle tightness, – Straightening of different twists or unusual curvatures of leg muscles – Improve the ability to sit, stand, and walk. R Dhaker, Asst. Professor, RCN 117
  • 116. • Physical Aids – Orthosis, braces and splints – Positioning devices – Walkers, special scooters, wheelchairs • Special Education • Rehabilitation Services- Speech and occupational therapies • Family Services -Professional support • Other Treatment – Therapeutic electrical stimulation, -Acupuncture, – Hyperbaric therapy – Massage Therapy might help R Dhaker, Asst. Professor, RCN 118
  • 117. • Physical Therapy • Sitting – Vertical head control and control of head and trunk. • Standing and walking – Establish an equal distribution of weight on each foot, train to use steps or inclines • Prone Development • Supine Development – Head control on supine and positions R Dhaker, Asst. Professor, RCN 119
  • 118. Neural Tube Defects 120R Dhaker, Asst. Professor, RCN
  • 119. Introduction • Neural tube defects are birth defects of the – brain, – spine, or – spinal cord. • NTDs are one of the most common birth defects, affecting over 300,000 births each year worldwide. 121R Dhaker, Asst. Professor, RCN
  • 120. • The neural tube forms by the 28th day after conception R Dhaker, Asst. Professor, RCN 122
  • 121. • In the 3rd week of pregnancy called gastrulating, specialized cells on the dorsal side of the embryo begin to change shape and form the neural tube. When the neural tube does not close completely, an NTD develops. 123R Dhaker, Asst. Professor, RCN
  • 122. 124R Dhaker, Asst. Professor, RCN
  • 123. Classification • NTDs can be classified, based on embryological considerations and the presence or absence of exposed neural tissue:- • Open NTD • Close NTD 125R Dhaker, Asst. Professor, RCN
  • 124. • Open NTDs frequently involve the entire CNS • (eg, associated hydrocephalus, Chiari II malformation) and are due to failure of primary neurulation. Neural tissue is exposed with associated cerebrospinal fluid (CSF) leakage. 126R Dhaker, Asst. Professor, RCN
  • 125. • Closed NTDs are localized and confined to the spine (brain rarely affected) and result from a defect in secondary neurulation. Neural tissue is not exposed and the defect is fully epithelialized, although the skin covering the defect may be dysplastic. 127R Dhaker, Asst. Professor, RCN
  • 126. • Cranial presentations include the following • Anencephaly • Encephalocele (meningocele or meningomyelocele) • Craniorachischisis totalis • Congenital dermal sinus 128R Dhaker, Asst. Professor, RCN
  • 127. • Spinal presentations include the following • Spina bifida aperta (cystica) • Myelomeningocele (see following images) • Meningocele • Myeloschisis • Congenital dermal sinus • Lipomatous malformations (lipomyelomeningoceles) • Split-cord malformations • Diastematomyelia • Diplomyelia • Caudal agenesis 129 R Dhaker, Asst. Professor, RCN
  • 128. Etiology • The chance that a pregnancy will be affected by a neural tube defect is less than one in 1000. • However, there are a number of factors that will increase this risk. The main one is a close family history of neural tube defects. R Dhaker, Asst. Professor, RCN 130
  • 129. Cont… etiology • The majority of NTDs are etiologically complex. • Vitamin B9 and vitamin B12 are very important in reducing the occurrences of NTDs. • Genetic factor • Environmental factor – folic acid deficiency, – anti-seizure medications – uncontrolled diabetes, – alcohol, obesity, and – increased body temperature R Dhaker, Asst. Professor, RCN 131
  • 130. Spina bifida R Dhaker, Asst. Professor, RCN 132
  • 131. • Spina bifida is a birth defect where there is incomplete closing of the backbone and membranes around the spinal cord. • The most common location is the – lower back, but – in rare cases it may be the middle back or neck R Dhaker, Asst. Professor, RCN 133
  • 132. • Spina bifida is one of the most common birth defects, with an average worldwide incidence of one to two cases per 1000 births, but certain populations have a significantly greater risk. R Dhaker, Asst. Professor, RCN 134
  • 133. CAUSES • Maternal diabetes • Family history • Obesity • Increased body temperature from fever or external sources such as hot tubs and electric blankets may increase the chances of delivery of a baby with a spina bifida. • Medications such as some anticonvulsants. • Pregnant women taking Valproic acid have an increased risk of having children with spina bifida • Genetic basis. • Folic acid deficiency 135R Dhaker, Asst. Professor, RCN
  • 134. TYPES: • Spina bifida malformations fall into three categories: spina bifida occulta  spina bifida cystica with meningocele spina bifida cystica with myelomeningocele. (The most common location of the malformations is the lumbar and sacral areas) 136R Dhaker, Asst. Professor, RCN
  • 135. R Dhaker, Asst. Professor, RCN 137
  • 136. Spina bifida occulta • Occulta is Latin for "hidden". • This is the mildest form of spina bifida. • In occulta, the outer part of some of the vertebrae is not completely closed. • The splits in the vertebrae are so small that the spinal cord does not protrude. 138R Dhaker, Asst. Professor, RCN
  • 137. Cont… Spina bifida occulta • The skin at the site of the lesion may be normal, or it may have some hair growing from it; there may be a dimple in the skin, or a birthmark. • The incidence of spina bifida occulta is approximately 10% of the population, and most people are diagnosed incidentally from spinal X-rays R Dhaker, Asst. Professor, RCN 139
  • 138. Meningocele: • The least common form of spina bifida is a posterior meningocele (or meningeal cyst). • In this form, the vertebrae develop normally, but the meninges are forced into the gaps between the vertebrae. 140R Dhaker, Asst. Professor, RCN
  • 139. Myelomeningocele • This type of spina bifida often results in the most severe complications. • In individuals with myelomeningocele, the unfused portion of the spinal column allows the spinal cord to protrude through an opening. • The meningeal membranes that cover the spinal cord form a sac enclosing the spinal elements. 141R Dhaker, Asst. Professor, RCN
  • 140. • Spina bifida with myeloschisis is the most severe form of myelomeningocele. In this type, the involved area is represented by a flattened, plate- like mass of nervous tissue with no overlying membrane. • The exposure of these nerves and tissues make the baby more prone to life-threatening infections such as meningitis. R Dhaker, Asst. Professor, RCN 142
  • 141. R Dhaker, Asst. Professor, RCN 143