CHAPTER 5:5 Flashcards
NERVOUS SYSTEM DISORDERS
What is secreted by the Choroid plexus. It is clear and colorless. It bathes the surface of the brain in the subarachnoid space between pia & arachnoid matter
Cerebrospinal fluid (CSF)
What are the NERVOUS SYSTEM DISORDERS
A. HYDROCEPHALUS
B. SPINA BIFIDA (RACHISCHISIS/ SCHISTORRHACHISI)
C. CEREBRAL PALSY
D. DOWN’S SYNDROME/TRISOMY 21/MONGOLISM
Placement of Shunt:
→ Used in older children and in children with abdominal problems
→ CSF drains from the ventricles to the RA bypassing the internal jugular vein→drains into
circulating blood
AV shunt (Atrioventricular Shunt)
Its function is to take food to the brain and spinal cord and axcretes metabolites and act as a shock absorber.
Cerebrospinal fluid (CSF)
A condition caused by an imbalance in the production and absorption of CSF in the ventricular
system
HYDROCEPHALUS
Placement of Shunt:
→ Lateral ventricle/lumbar subarachnoid space to ureter→bladder (CSF is not reabsorbed
=additional intake of water and sodium)
→ Older children
Ventriculoureteral shunt
Placement of the shunt:
→ Necessitates the removal of the kidney
→ Last resort if other types have failed
→ Necessitates prompt treatment of UTI to prevent ascending infection
Ventriculoureteral shunt
Characterized by increased amount of CSF within ventricles and subarachnoid space resulting to enlargement of the head.
HYDROCEPHALUS
predominant sign of hydrocephalus in infancy
Head enlargement
what is used to confirm diagnosis and to compare the ration of lateral ventricle to cortex
Echoencephalography (neonates-infants whose fontanels are open)
ratio as to how many are affected with hydrocephalus
4:1,000 live births
causes of hydrocephalus
- Congenital=infants
- Acquired (complication of illness/trauma) =older children
It is a lifelong problem that needs evaluation regularly=realistic goals to achieve optimum potential , don’t be overprotective
hydrocephalus
Placement of Shunt in hydrocephalus
- Ventriculoperitoneal shunt (VP shunt)
- AV shunt (Atrioventricular Shunt)
- Ventriculoureteral shunt
Placement of Shunt:
→ Necessitates the removal of the kidney
→ Last resort if other types have failed
→ Necessitates prompt treatment of UTI to prevent ascending infection
Ventriculoureteral shunt
Placement of Shunt:
→ Most commonly used type
→ CSF drains from the ventricles to peritoneal cavity – fluid is absorbed across the peritoneal
membrane
Ventriculoperitoneal shunt (VP shunt)
Complication of Shunt Placement:
What Complications of Shunt Placement causes blockage, kinking, breakdown, migration
Shunt failure
Complications of Shunt Placement:
- Shunt failure
- Infection
What Complications of Shunt Placement is:
→ Most serious
→ Most prevalent in the first 2 months after placement
→ Post op ileus and peritonitis in VP shunt
Infection
how do you Provide pre-operative care for those with hydrocephalus
a. Measure head circumference daily
b. Monitor for s/s of ↑ICP
c. positioning
d. reduce chances of skin breakdown
e. measures to minimize ↑ICP
f. nutrition
Point of greatest measurement
occipitofrontal
just above brow, above pinna of the ears, around occipital prominence
infants with HYDROCEPHALUS:
→ early sign: _____
→ late: _____
→ early sign: tense bulging fontanel
→ late: high pitched, shrill cry, seizures
toddlers with HYDROCEPHALUS:
▪ early sign= ____
▪ early sign=headache, loss of appetite
Comfort measures for:
- mild pain
- severe pain
- acetaminophen for mild;
- opioids for severe pain
What is the reason for providing emotional support to parents about their child
Due to fear of retardation or brain damage
▪ literally means “cleft spine”
▪ “divided spine”
SPINA BIFIDA (RACHISCHISIS/ SCHISTORRHACHISIS)
What is any malformation of spinal canal and cord called
Myelodysplasia
A congenital defect in which the spinal column is imperfectly closed so that part of the meninges or spinal cord protrudes, often resulting in hydrocephalus and other neurological disorders.
SPINA BIFIDA (RACHISCHISIS/ SCHISTORRHACHISIS)
Failure of the posterior vertebral arches to fuse during the embryologic development.
SPINA BIFIDA (RACHISCHISIS/ SCHISTORRHACHISIS)
in SPINA BIFIDA (RACHISCHISIS/ SCHISTORRHACHISIS):
Failure of the posterior vertebral arches to fuse (or neural tubes to close) during embryologic development, which is usually between what weeks of fetal life.
2-4 weeks
in SPINA BIFIDA (RACHISCHISIS/ SCHISTORRHACHISIS):
Normally, spinal cord and cauda equina are enclose where?
protective sheath of bone and meninges
Splitting of the already closed neural tube as a result of an abnormal CSF pressure in the first trimester
SPINA BIFIDA (RACHISCHISIS/ SCHISTORRHACHISIS)
→ May involve entire length/restricted to a small area
→ The most common defect of the CNS
→ The higher the defect, the greater the neurologic dysfunction
SPINA BIFIDA (RACHISCHISIS/ SCHISTORRHACHISIS)
Causes of SPINA BIFIDA (RACHISCHISIS/ SCHISTORRHACHISIS)
- unknown (multifactorial inheritance)
- genes
- environment- poor maternal nutrition
All women of childbearing age should at eat foods rich in ________(fortified breads and cereals, fruits, dark green leafy vegetables and egg yolk)
folic acid
TYPES OF SPINA BIFIDA
- Spina bifida occulta
- Spina bifida cystica
2.a. Meningocele | 2.b. Myelomenigocele
Type of Spina Bifida:
* Failure of the posterior arches to fuse
* Mildest form
SPINA BIFIDA OCCULTA
Type of Spina Bifida:
* Not visible; may be identified by a dimple or the pressure of tuft of hair at the point of defect
* Child asymptomatic/with slight deficit-no treatment necessary
SPINA BIFIDA OCCULTA
Type of Spina Bifida:
* Defect in closure of posterior vertebral arch with protrusion through bony tissue
* Visible with an external saclike protrusion
SPINA BIFIDA CYSTICA
Clinically, what does the term spina bifida means
myelomeningocele
Type of Spina Bifida Cystica:
Encases meninges, spinal fluid but with no neural elements. Sac is covered with a skin
MENINGOCELE
Type of Spina Bifida Cystica:
Not associated with neurologic deficits
MENINGOCELE
What does the sac covering of those with meningocele look like?
translucent/membranous
Type of Spina Bifida Cystica:
Contains meninges, spinal fluid and nerves; fluid leakage may occur
MYELOMENIGOCELE
Type of Spina Bifida Cystica:
Parallel to dysfunction=loss of muscle function to lower extremities, bowel and bladder
MYELOMENIGOCELE
Type of Spina Bifida Cystica:
* Lesion poorly covered with imperfect tissue
* In 90-95% of cases, hydrocephalus is frequently associated anomaly
MYELOMENIGOCELE
Amniocentesis
* _____ =anencephaly, myelomenigocele; omphalocele; SCI
* _____ =chromosomal disorder
- Elevated AFP =(+) NTD
- Decreased
when is amniocentesis Best done because after this time, AFP concentrations normally
diminish + can do therapeutic abortion just in case
16-18 weeks
what diagnostic procedure is considered for patients with family history of SB
Chorionic villi sampling (CVS)
When infants are detected as having meningocele / myelomenigocele, they may be born by what mode of delivery to avoid pressure and injury to the spinal cord.
caesarean section
as management to spina bifida, Early surgery of how many hour/s is done after birth to replace the contents and to close the skin (skin graft)
24-72 hours
It is one of the most common causes of neurogenic/neuropathic bladder dysfunction→frequent cause of UTI, ureterohydronephrosis, vesiculourethral reflux)
spina bifida
Goals to those with spina bifida:
Infants= ???
Older children= ???
- to preserve renal function
- preserve renal function and achieve optimum urinary continence
what Surgical procedure for Bladder elimination:
→ stoma created on the abdominal wall for urinary drainage
Vesicostomy
what is it called when head is short but wide; sagittal sutures may separate
Brachycephaly
what Surgical procedure for Bladder elimination:
→ increases bladder capacity and reduces high bladder pressure
Augmentation enterocystoplasty
what Surgical procedure for Bladder elimination:
→ continent neobladder constructed from bowel/stomach +CIC
→ use when severe bladder dysfunction compromising renal function not relieved by other means
Urinary diversion
The most common chromosomal abnormality; 1.66: 1,000 live births
DOWN’S SYNDROME/TRISOMY 21/MONGOLISM
WHat occurs due to hypotonicity of chest and abdominal muscles and dysfunction of the immune system predispose to the development of respiratory infection
prone to upper respiratory infection (URI)
A chromosomal abnormality involving an extra chromosome (21) characterized by a typical
appearance and mental retardation
DOWN’S SYNDROME/TRISOMY 21/MONGOLISM
other terms for:
- nearsightedness
- involuntrary rapid eye movement
- myopia
- nystagmus
what diagnosis for DOWN’S SYNDROME/TRISOMY 21/MONGOLISM uses:
Sample of peripheral venous blood/a sample of cells from the buccal membrane is taken;
cells are allowed to grow (2 weeks) then observed though a microscope
Karyotyping
what diagnosis for DOWN’S SYNDROME/TRISOMY 21/MONGOLISM:
Shows the 3rd chromosome 21 that is attached to another in translocation
Karyotyping
what diagnosis for DOWN’S SYNDROME/TRISOMY 21/MONGOLISM uses:
Visual presentation of a chromosome pattern of an individual
Karyotype
diagnosis for DOWN’S SYNDROME/TRISOMY 21/MONGOLISM:
▪ Most commonly done at 8-10th week
▪ Retrieval and analysis of chorionic villi for chromosome analysis
Chorionic Villi Sampling CVS
diagnosis for DOWN’S SYNDROME/TRISOMY 21/MONGOLISM:
▪ Done at 14-18th week
Amniocentecis
diagnosis for DOWN’S SYNDROME/TRISOMY 21/MONGOLISM:
▪ Done at 18-22th week
▪ Greatest risk of miscarriage
▪ Most accurate to confirm CVS and amniocentecis
Percutaneous Umbilical Blood Sampling (PUBS)
Regular medical care for DOWN’S SYNDROME/TRISOMY 21/MONGOLISM:
▪ For children participating in sports that may involve stress on back and neck
▪ Monitor for neck pain, weakness and torticollis
Atlantoaxial instability
Regular medical care for DOWN’S SYNDROME/TRISOMY 21/MONGOLISM:
▪ At risk for spinal cord compression (s/s persistent neck pain, loss of established
motor skills and bladder and bowel control, changes in sensation)
Atlantoaxial instability
4 Regular medical care for DOWN’S SYNDROME/TRISOMY 21/MONGOLISM:
- eyesight
- hearing
- thyroid function
- atlantoaxial instability
What is setting sun also known as
Sunken Eyeballs
HALLMARK SIGN
It means without coordination. People with this lose muscle control in their arms and legs
ataxia
2 types of HYDROCEPHALUS
- Non-communicating (Intraventricular, Obstructive)
- Communicating (Extraventricular, Non-obstructive)
type of HYDROCEPHALUS:
- Obstruction to the flow of CSF within the ventricles
- Obstruction of CSF flow to subarachnoid space
- Non-communicating (Intraventricular, Obstructive)
type of HYDROCEPHALUS:
Reduced absorption of CSF within the
subarachnoid space
- Communicating (Extraventricular, Non-obstructive)
HYDROCEPHALUS:
What is most effective when disorder is recognized early because once ↑ ICP becomes so acute, the brain tissue is damaged and motor/mental deterioration results
Treatment
last resort for managing HYDROCEPHALUS:
Ventriculoureteral shunt
Why is there a need to monitor if:
v/s=↑RR, BT, and BP (↑SBP); ↓PR
Possibility of increased intracranial pressure
What must be done: if ↑ICP to prevent rapid CSF
drainage
Gradually elevated not greater than 30 degrees
Best position: FLAT | >30’ causeover draining of fluid
SPINA BIFIDA (RACHISCHISIS/ SCHISTORRHACHISIS)
What is the reason why neural tubes are to close
due to mother nottaking folic acid during pregnancy
2 types of SPINA BIFIDA CYSTICA
MENINGOCELE & MYELOMENIGOCELE
a group of non progressive neurodevelopmental disabilities mainly involving muscle control and other sensory and/or intellectual function to some degree
cerebral palsy
Most common CHRONIC disorder of childhood
characterized by impaired movement and posture resulting from an abnormality in the EPS/PS
cerebral palsy
Most common CHRONIC disorder of childhood
Types of Cerebral Palsy according to topography
- Hemiplegia
- Diplegia
- Quadriplegia
Type of Cerebral Palsy according to topography:
one side of body with upper extremities being dysfunctional rather than the lower extremities
Hemiplegia
Type of Cerebral Palsy according to topography:
involvement of the lower extremities, usually spastic
Diplegia
Type of Cerebral Palsy according to topography:
involvement of all extremities with the arm in flexion and leg in extension
Quadriplegia
Types of Cerebral Palsy according to Neuromuscular Dysfunction:
- Spastic
- Athetoid (dyskinetic)
- Ataxic
- Mixed (dystonic)
Type of Cerebral Palsy according to Neuromuscular Dysfunction:
- may involve one/both sides
- the most common form
- hypertonicity with poor control of posture, balance and coordinated motion
spastic
Type of Cerebral Palsy according to Neuromuscular Dysfunction:
- impaired fine and gross motor skill
- active attempts at motion increases abnormal posture and overflow of movements to othre pars of the body
spastic
Type of Cerebral Palsy according to Neuromuscular Dysfunction:
Manifestations:
1. scissor gait when held upright
2. walk on toes
spastic
Type of Cerebral Palsy according to Neuromuscular Dysfunction:
- abnormal involuntary movement
- slow wormlike writhing movements that usually involve the extremities, trunk, facial muscle and tongue
Athetoid (dyskinetic)
Type of Cerebral Palsy according to Neuromuscular Dysfunction:
- involuntary muscles may take on choreoid and dystonic manifestations
- involvement of a pharyngeal, laryngeal, and oral muscles cause drooling and dysarthia (imperfect speech articulation)
Athetoid (dyskinetic)
Type of Cerebral Palsy according to Neuromuscular Dysfunction:
- irregularity and muscle coordination or action
- awkward wide-based gate
Ataxic
Type of Cerebral Palsy according to Neuromuscular Dysfunction:
- rapid repetitive movements performed poorly
- disintegration of movements of the upper extremities when the child reaches for objects
Ataxic
Type of Cerebral Palsy according to Neuromuscular Dysfunction:
- combination of spasticity and athetosis
mixed (dystonic)
It is a condition that affects the bones in the upper spine or neck under the base of the skull.
Atlanto-axial instability (AAI)
