disease of central nervous system...asst

Disease of Central Nervous System

Rahul DhakerAsst. 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.



PET Scan


• 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


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…


• 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:


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


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.


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.


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.


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


Management

• Management of febrile convulsion should be

done-

– To control convulsion

– To reduce increased body temperature

– To treat the cause of fever, usually ARI


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


• 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.


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%.


NEONATAL SEIZURES


Meningitis


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.


Clinical Manifestation

• The 3 classic symptoms (less likely in younger

children):

• Fever

• Headache

• Meningeal signs


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


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)


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


CSF findings in different forms of meningitis

Type of

meningitisGlucose Protein Cells

Acute bacterial low highPMNs

often > 300/mm³

Acute viral normal normal or highmononuclear

< 300/mm³

Tuberculous low highmononuclear and

PMNs, < 300/mm³

Fungal low high < 300/mm³

Malignant low highusually

mononuclear


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


• Specific

• Hematologic

– Blood culture

• radiographic

– CT Scan

– MRI

Cont… Diagnosis


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).


• 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


• Duration of therapy should not be shorter than 5

days for meningococcus, 10 days for H

influenzae, and 14 days for S pneumoniae.


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


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


Treatment

• Probable group B

streptococci

• Penicillin

50,000i.u/kgI.V/4

hourly.


Other Drugs available

Anti-microbials

• Ceftriaxone

• Cefotaxime

• Penicillin G

• Vancomycin

• Ampicillin

• Gentamicin

Anti-Virals

• Acyclovir

• Ganciclovir (>3mths)

Anti-fungals

Amphotericin B

Fluconazole


Epilepsy


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.


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


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

http://kidshealth.org/en/parents/meningitis.html

http://kidshealth.org/en/parents/encephalitis.html

• 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


• 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%


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 seizures59R Dhaker, Asst. Professor, RCN

Diagnosis of Epilepsy

Thorough History taking :From patientsFrom reliable valid informantsFrom observer (who observed seizures)

Physical Examination:Specially neurological systemHigher 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


Management

• Epilepsy affects every child differently

depending on:

• Age

• Types of seizure

• Response to treatment

• Having other health issues, etc.


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.


Surgical Management

• Neurosurgical is indicated in some cases of

convulsion disorder, especially anatomical lesion

like – Brain tumor, hematoma etc.


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 rootR 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.


Definition



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.


http://www.nhs.uk/Conditions/encephalitis/Pages/introduction.aspx

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, RCN72

https://www.medicalnewstoday.com/articles/151739.php

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


https://www.medicalnewstoday.com/articles/37135.php

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


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


• 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


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


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.


Cont… Treatment

• Medication may include:-

– Antiviral medication

– Antibiotic medication

– Anti – Seizure medication

– Steroids

– Sedative to treat irritability.

– Acetaminophen for fever and headache.


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.


Complication

• Permanent brain damage may occur in severe

case. It may affect-

– Hearing

– Memory

– Muscle control

– Sensation

– Speech

– Vision


Cerebral Palsy


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.


http://kidshealth.org/en/kids/brain.html

• • Cerebral-

• “Latin Cerebrum”;

- Affected part of brain


• “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



• Cerebral palsy (CP) is a disorder that affects muscle

tone, movement, and motor skills (the ability to move

in a coordinated and purposeful way).


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


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


Incidence• Time (% of cases)

• Prenatal (44%)

– First trimester

– Second trimester

• Labor and delivery (19%)

• Perinatal (8%)

• Childhood (5%)

• Not obvious (24%)


• 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.


Classification of CP

1. Neurologic deficits

2. Type of movement involved

3. Area of affected limbs


Neurologic deficits

• Based on the– extent of the damage

–area of brain damage

• Each type involves the way a person moves


Cont…Neurologic deficits

1. PYRAMIDAL

• originates from the motor areas of the cerebral cortex

2. EXTAPYRAMIDAL

• basal ganglia and cerebellum

3. MIXED


Type of movement involved


Cont… Type of movement involved

1. Spastic CP

2. Athethoid CP

3. Ataxic CP

4. Spastic &

Athethoid CP


Area of affected limbs• Paraplegia

• Diplegia

• Hemiplegia

• Quadriplegia

• Monoplegia -one limb (extremely rare)

• Triplegia -three limbs (extremely rare)


Clinical Manifestation


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


Associated Problems Of Cerebral Palsy

• Hearing and visual problems

• Sensory integration problems

• Failure-to-thrive, Feeding problems

• Behavioral/emotional difficulties,

• Communication disorders


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


Diagnostic Evaluation• Physical evaluation, Interview

• MRI, CT Scan EEG

• Laboratory and radiologic work up

• Assessment tools

– i.e. Development Motor Skills,

–Denver Test II


• 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


• 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


Treatment • No treatment to cure cerebral palsy.

• Brain damage cannot be corrected.

• Crucial for children with CP:

– Early Identification;

– Multidisciplinary Care; and

– Support


• 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.


• 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


• 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


Neural Tube Defects


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.


• The neural tube forms by the 28th day after

conception


• 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.


Classification

• NTDs can be classified, based on embryological

considerations and the presence or absence of

exposed neural tissue:-

• Open NTD

• Close NTD


• 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.


• 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.


• Cranial presentations include the following

• Anencephaly

• Encephalocele (meningocele or

meningomyelocele)

• Craniorachischisis totalis

• Congenital dermal sinus


• 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 agenesis129

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.


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


Spina bifida


• 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


https://en.wikipedia.org/wiki/Back

• 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.


CAUSES• Maternal diabetes

• Family history

• Obesity

• Increased body temperature from fever or externalsources such as hot tubs and electric blankets mayincrease the chances of delivery of a baby with aspina bifida.

• Medications such as some anticonvulsants.

• Pregnant women taking Valproic acid have anincreased risk of having children with spina bifida

• Genetic basis.

• Folic acid deficiency


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)


http://en.wikipedia.org/wiki/Lumbar

http://en.wikipedia.org/wiki/Sacrum

http://www.lpch.org/media/images/conditions/es_2716.gif

http://www.lpch.org/media/images/conditions/es_2716.gif

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.


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


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.


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.


• 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.
