Sunday, March 31, 2013
AcePGMed: NEET 2013 Practice Question Paper
AcePGMed: NEET 2013 Practice Question Paper: Ace PG Med Starts new venture from Apr- 01 -2013. Online practice for NEET PG Medical Entrance 2013. One Exam per day. Tim...
NEET 2013 Practice Question Paper
Ace PG Med
Starts new venture from Apr- 01 -2013.
Online practice for NEET PG Medical Entrance 2013.
- One Exam per day.
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Saturday, March 23, 2013
CONGENITAL HEART DISEASE
CONGENITAL HEART DISEASE
Etiology
Environmental
genetic
multifactorial
causes
Chromosomal
abnormalities
trisomy 21, 13 or 18.
40% of those with Down syndrome can have a cardiac defect,
with atrioventricular septal defect and ventricular septal defect accounting
for approximately 80% of lesions.
Cardiac defects are common in trisomy 18 (Edwards’ syndrome)
and 13 (Patau’s syndrome).
Turner’s syndrome, 45XO, left heart lesions with Coarctation
of the aorta in 10% of cases.
autosomal abnormalities = low birthweight, mental
retardation, small stature and
Single gene
defects
Pompe’s disease = causes cardiomyopathy,
Marfan’s syndrome = aorta root dilation
Noonan’s syndrome, = pulmonary valve or artery stenosis,
CATCH 22 syndrome: cardiac defects, abnormal facies, thymic
aplasia, cleft palate, hypocalcemia.
DiGeorge syndrome
Teratogens
maternal infection
illness
ingestion of certain drugs
congenital rubella syndrome = peripheral pulmonary stenosis
or an arterial duct.
offspring of diabetic mothers,
uncontrolled maternal phenylketonuria
Maternal ingestion
of therapeutic drugs
lithium =Ebstein’s anomaly
phenytoin =semilunar valve stenosis, coarctation, arterial
duct
isotretinoin
fetal alcohol syndrome
Syndromes
de Lange= VSD
Williams = supravalvar aortic stenosis,
Friedreich’s ataxia =hypertrophic cardiomyopathy
Jervell and Lange-Nielsen =prolonged QT
Holt–Oram = atrial septal defect,
VATERL=VSD
Recurrence Risk
risk for another pregnancy rises to about 2% if one previous
child is affected
If two previous children are affected the risk rises to 6–8%
if the mother is affected there is an even higher risk
(5–15%)
Fetal echocardiography provides an accurate means of
diagnosing fetal cardiac abnormalities from about 18 weeks’ gestation
Nomenclature Of
Congenital Heart Disease
heart has three parts –
Atrial
chambers
Ventricular
chambers
Arterial trunks
Connections and relationships of these can be determined
Determine of how each of the three basic components or
segments of the heart are connected.
Determine the arrangement and connections of the atrial
chambers
Then to analyze the atrioventricular and ventriculoarterial
junction.
Then position of the heart.
Atrial arrangement
atrial arrangement or situs has first to be determined.
This does not always follow the situs of the abdominal
viscera
but usually that of the thoracic viscera and
thus the bronchial morphology.
analyse of the bronchial anatomy on X-ray, - with a
penetrated film.
The right main bronchus is more vertical and shorter than
the left,
branching above the lower lobe pulmonary artery while the
left branches below it.
The right atrium lies on the same side as the right
bronchus.
The usual atrial arrangement is described as solitus
its mirror image as inversus.
When the atrial situs is uncertain it is frequently called
ambiguous
Careful analysis may show bilateral manifestations of right
or left atrial morphology, which can then be described as right or left atrial
isomerism.
In the
former, (right atrial isomerism) asplenia is the usual association
in the
latter, polysplenia.
Atrial arrangement or situs is summarized as follows:
solitus:
right atrium on right, left atrium on left
inversus:
left atrium on right, right atrium on left
isomerism –
right: bilateral right atria
isomerism –
left: bilateral left atria
ambiguous:
used if arrangement cannot be identified.
Atrioventricular
connection
The
atrioventricular connection then describes the way the atria communicate with
the ventricles at the atrio-ventricular junction.
If the
connections follow the normal pattern they are said to be concordant, e.g.
right atrium to right ventricle and left atrium to left ventricle,
Discordant
atria connect with the contra-lateral ventricle.
When both
atrio-ventricular valves enter one ventricular chamber, the connection is
described as double inlet
if one or
other atria is not directly connected to a ventricle, then that atrio-ventricular
connection is said to be absent.
it may not
be possible to state exactly the atrio-ventricular connection which is then
described as ambiguous.
These are summarized as follows:
concordant:
right atrium to right ventricle, left atrium to left ventricle
discordant:
right atrium to left ventricle, left atrium to right ventricle
ambiguous:
with atrial isomerism and one atrium entering each ventricle
double
inlet: both atria connect to the same ventricle
absent
right or left: no true or potential connection from the right or left atrium to
a ventricle.
Ventriculoarterial
connection
This
describes the means by which the great arteries take origin from the
ventricular chambers.
If an
artery overrides the septum, and thus arises from both ventricles, it is
assigned to that from which more than half takes origin.
Connections can thus be:
concordant:
pulmonary trunk from right ventricle, aorta from left
discordant:
aorta from right ventricle, pulmonary trunk from left
double
outlet: both great arteries from one ventricle
single
outlet: single great artery.
Three further
steps
are then
necessary to complete the analysis: a statement of the relationship of structures; tabulation of associated lesions; and description of the cardiac position within
the chest.
Relationships
These are described in simple terms,
such as
right/left,
anterior/posterior,
superior/inferior,
side by
side.
These relationships neither imply nor give any information
on morphology or connections.
Additional abnormalities
These will include factors such as
venous
drainage,
Septal
defects,
Stenosis or
atresia of valves, and great artery anomalies such as coarctation.
Cardiac position
When the
heart is on the left side this is not usually stated if there is situs solitus,
should be
described as levocardia where there is an abnormal situs.
Dextrocardia
describes the situation in which more than half of the cardiac shadow on X-ray
is in the right side of the chest
it makes no
assumptions as to the atrial situs or intracardiac anatomy.
Mesocardia
is used when the heart appears to be in the center of the thorax.
Comment
nomenclature may seem complicated
not required in the majority of patients who have normal
chamber connections, morphology and
relations.
it simplifies assessment and description of complex defects
prevents any ambiguity in communication between different
cardiologists and surgeons.
use the terms which have been in use for some time
Thus atrial situs solitus, atrioventricular concordance and
ventriculoarterial discordance is simply to say transposition of the great arteries.
Medical Care Of
Congenital Heart Disease
cardiologist use interventional catheterization techniques
to undertake corrective procedures for some less complex lesions
role of the physician is to provide general medical care
use the appropriate investigations to make an accurate
diagnosis
refer the patient to the surgeon at the appropriate time.
The Newborn Infant
With Congenital Heart Disease
The patient with congenital heart disease who survives
beyond infancy has a good outlook,
Nowadays surgery generally carry a low risk.
With modern surgery, up to 15–20% of live-born children in
whom a defect is recognized in infancy can die in the first year of life,
Infant with congenital heart disease may show rapid
progression to severe cardiac failure or cyanosis with hypoxia and acidosis
Heart disease in the newborn is usually recognized by the
presence of cyanosis or heart failure.
Early detection of cyanotic heart disease is difficult
hyperoxic test is useful where there is uncertainty
in response to hyperoxia (80–100% oxygen) - a PO2
of over 150 mmHg (21 kPa) from the upper body excludes a major right to left
shunt and a failure to rise suggests a cardiac defect.
important early signs of heart failure are
tachycardia
>160/min
tachypnea >50/min
hepatomegaly
Palpation of the pulses
compare not only the right arm and the leg pulse but also
the pulses in both arms.
With coarctation of the aorta or hypoplastic left heart
syndrome the femoral pulses may feel normal initially when the ductus is open.
Low volume pulses occur with obstruction to left ventricular
output such as hypoplastic left heart syndrome or severe aortic stenosis.
A parasternal or subxiphoid heave may indicate the presence
of a significant defect in a patient where there is no abnormality on
auscultation.
A single second heart sound should be considered abnormal
after the first day of life.
Gallop rhythm indicates cardiac failure.
Many infants with significant heart disease have no murmur
When a murmur is heard it is not diagnostic but suggests the
presence of an underlying defect.
ECG
Difficult to interpret in the first few days of life
an infant
with severe congenital heart disease can have a normal ECG.
chest X-ray is useful.
contour of
the heart and the great arteries and the effect of the anatomical abnormality
on the pulmonary vascularity
typical
appearances are not always found.
A large
thymus may cause difficulty in interpretation of the cardiac silhouette
increased
pulmonary blood flow is not always reflected in the X-ray appearances.
Echocardiography may be more accurate
Catheterization is usually only required for interventional
procedures such as atrial septostomy.
This is easily performed through the umbilical vein within
the first 2 days of life.
Transfer to the cardiac centers should be as rapid as
possible in a suitable transport incubator.
General care of temperature, acidosis and electrolyte
imbalance is essential.
Prostaglandin
therapy
The use of E-type prostaglandins to dilate the ductus
arteriosus is an essential part in the management of the newborn infant with a
ductus dependent circulation. T
his occurs either when there is
marked
obstruction to pulmonary blood flow (such as pulmonary atresia)
in aortic
arch abnormality (such as critical aortic stenosis or coarctation).
Prostaglandin E2, which is readily available in most
obstetric hospitals, is cheaper than prostaglandin E1, and equally effective
Prostaglandins should be administered as a peripheral venous
infusion
a suitable initial rate being 0.02 mg/kg/min
the dose being increased up to 0.05–0.10 mg/kg/min
depending on the clinical response.
serious complication of prostaglandin therapy is respiratory
depression,
normal respiration rapidly returns when the infusion is
stopped.
It should then be restarted at a reduced dosage.
Other side-effects include fever, tachycardia and jitteriness.
Channelopathies (Myotonias and Periodic Paralysis)
Channelopathies (Myotonias and Periodic Paralysis)
Channelopathies= disorders of ion channels that result in altered excitability of cellular membranes.
Most of channelopathies are disorders of muscle membrane ion channels.
Results in muscle membrane hyper excitability leading to sustained contraction = myotonia
May result in muscle membrane hypoexcitability leading to weakness seen in periodic paralysis
Muscle channelopathies are sodium, calcium, and chloride channel disorders
May be Inherited channelopathies OR
Acquired channelopathies (Acquired channelopathies are autoimmune)
myotonias
dystrophic
nondystrophic disorders.
In dystrophic myotonia, myotonia is one of several muscle symptoms
with muscle atrophy and weakness being most prominent.
These include
dystrophia myotonica
proximal myotonic myopathy
in nondystrophic myotonias the most prominent symptom is myotonia
periodic paralyses - divided into those associated
with a high or normal serum potassium concentration (i.e., hyperkalemic periodic paralysis)
those associated with a low serum potassium concentration (i.e., hypokalemic periodic paralysis).
the abnormal serum potassium concentration is the consequence rather than the cause of the periodic paralysis.
Skeletal Muscle Channelopathies
Channel and disease are -
SODIUM
Hyperkalemic penodic paralysis
With myotonia
Without myotonia
With paramyotonia congenita
Paramyotonia congenita
Sodium channel myotonia
Myotonia fluctuans
Myotonia permanens
Acetazolamide-responsive myotonia
CALCIUM
Skeletal muscle calcium channel alpha-1 subunit
Hypokalemic periodic paralysis
CHLORIDE
Skeletal muscle chloride channel
AD myotonia congenita (Thomsen's)
AR myotonia congenita (Beeker's)
Pathogenesis and Pathophysiology of Sodium Channelopathies
sodium channelopathies result from point mutations in a gene, situated on the long arm of chromosome 17.
reduced inactivation of the sodium channel, followed by
either increased muscle excitability with myotonia
or increased muscle inexcitability with hyperkalemic periodic paralysis.
Pathogenesis and Pathophysiology of Chloride Channelopathies.
reduced muscle membrane chloride conductance ( i.e.rate of flow of chloride is decreased) resulting in muscle membrane hyper excitability à repetitive firing, à leads to the myotonia.
Eg -Thomsen's and Becker's diseases
Pathogenesis and Pathophysiology of Calcium Channelopathies.
In hypokalemic periodic paralysis, the weakness is related to the calcium channel.
There is an influx of potassium into the muscle fiber with an accompanying influx of extracellular water.
influx of potassium may account for the precipitation of hypokalemic periodic paralysis with large carbohydrate meals.
influx of potassium in hypokalemic periodic paralysis causes the muscle fibers to become depolarized and inexcitable.
Clinical Features and Associated Disorders of Sodium Channelopathies.
paramyotonia congenita
hyperkalemic periodic paralysis
sodium channel myotonias.
Paramyotonia Congenita.
The predominant symptom is paradoxical myotonia, which is present from birth and persists throughout life.
The myotonia is paradoxical because unlike classic myotonia, it increases with repetitive movements.
It is exacerbated by cold temperatures, which cause weakness.
In warm environment, patients may have no symptoms
attacks are precipitated by potassium ingestion
Hyperkalemic Periodic Paralysis.
appears in infancy or early childhood
paresis - brief and mild
lasting 15 minutes to 4 hours
precipitated by rest following exercise
by ingestion of potassium-rich foods
by administration of potassium compounds
attacks commonly start in the morning before breakfast
stress provokes them more easily
Weakness is mainly proximal
no ocular or
respiratory muscle weakness
flaccid quadriplegia
with absent reflexes and normal sensory examination.
The
potassium level may rise during the attack
May cause
cardiac dysrhythmias.
between
attacks- patient has normal strength of
muscles
Sodium Channel Myotonias. myotonia becomes worse with cold,
not associated with weakness
responds to acetazolamide (acetazolamide-responsive myotonia
Clinical Features and Associated Disorders of Chloride Channelopathies.
two forms
autosomal dominant disease (Thomsen's disease)
autosomal recessive disease (Becker's disease).
Autosomal Dominant Myotonia Congenita (Thomsen's Disease).
painless generalized myotonia,
looks like muscle stiffness.
first and second decades of life
provoked by exertion following rest.
ask the patient to rise from a chair after a period of quiet sitting.
improves with exercise
well-developed muscles with particular hypertrophy of the lower limbs, giving them an athletic appearance.
Muscle strength may be normal, or even stronger than normal.
normal reflexes,
eyelid, grip, and percussion-induced myotonia can be demonstrated.
Autosomal Recessive Myotonia Congenita (Becker's Disease).
similar to Thomsen's disease except that myotonia appears later in the first decade.
Becker's disease -muscles are initially weak
a period of activity is required before full strength returns.
may have muscle hypertrophy, of the legs and buttocks,
Hypokalemic Periodic Paralysis.
autosomal dominant disorder
common in males
begin at adolescence
occur at night,
the patient awakens with weakness.
episodes may be precipitated by
carbohydrate or alcohol intake,
rest after exercise,
emotional stress.
attacks 1 to 4 hours, may persist for up to 3 days.
Prodromal symptoms of muscle stiffness, heavy limbs, or sweating
followed by proximal lower limb weakness,
spreads to become a tetraparesis.
Ocular or bulbar involvement is rare.
Fatalities are rare = injudicious treatment or hypokalemia-induced cardiac dysrhythmias.
D uring severe attacks patients are flaccid and areflexic.
Differential Diagnosis Myotonias.
The principal symptom of myotonia is
muscle stiffness
inability to relax contracted muscle
sodium channel myotonia is not painful
Stiffness may be confused with spasticity or rigidity.
Muscle cramps, is a feature of a peripheral nerve disorder
Dystonia results in abnormal postures
Painless contractures may be a feature of metabolic myopathy such as McArdle's disease
withdrawl of levodopa = muscle rigidity or stiffness with fever, an elevated creatine kinase (CK) level, and a high white blood cell count.
pseudomyotonia = impaired relaxation without electrical evidence of myotonia
= acid maltase deficiency and Brody's disease
Differential diagnosis of Periodic Paralysis.
causes of a flaccid, areflexic tetraparesis without sensory signs like
Hypercalcemia
Hypocalcemia
Hypophosphatemia
Hypomagnesemia
rhabdomyolysis
Guillain-Barre syndrome
myasthenic syndrome
acute poliomyelitis
Secondary hypokalemic periodic paralysis
intracellular potassium depletion from either renal, endocrine, gastrointestinal, or drug-induced mechanisms
Thyrotoxic periodic paralysis
hyperthyroidism.
Evaluation Myotonias.
Laboratory evaluations
Serum CK level, - elevated in Thomsen's and Becker's diseases
EMG - spontaneous myotonic discharges
Periodic Paralysis.
blood tests for potassium, calcium, magnesium, phosphate, and CK should be obtained during an episode of weakness.
electrocardiogram (ECG) may show changes consistent with hypokalemia or hyperkalemia
EMG
Nerve conduction studies are normal.
Muscle biopsy
Management Myotonias.
anesthesia should be planned
potassium administration can exacerbate myotonia, potassium supplements should be given only when necessary
myotonia congenita = membrane-stabilizing drugs such as procainamide and quinine
Phenytoin is useful for chronic administration
Periodic Paralysis.
Hypokalemic -
prevented by a low-carbohydrate, low-sodium diet. A
cetazolamide prevents paralytic attacks
ECG for cardiac dysrhythmias.
hyperkalemic periodic paralysis,
thiazide diuretics
Carbohydrate-containing foods and fluid may aggravate the weakness,
Inhaled beta-adrenergic agonists such as salbutamol are effective treatments in acute situations
a� 8 c o � � psychiatrist.
Intrathecal
baclofen - used - in selected children with severe spasticity.
Botulinum
toxin - management of spasticity in specific muscle groups, - positive response in - patients studied.
Patients
with incapacitating athetosis occasionally respond to levodopa, and children with dystonia may benefit from
carbamazepine or trihexyphenidyl.
Palpation
Applying the palm of the hand to the chest
Thrills
increased precordial pulsation (apical in left
ventricular hypertrophy and basal and right sided in right ventricular
hypertrophy)
diastolic shock (in the pulmonary area in pulmonary
hypertension)
The apex beat, normally in the fourth or fifth
intercostal space within the mid-clavicular line
pulse wrist
(radial) or inguinal region (femoral).
Sinus arrhythmia (increase
in rate on inspiration with decrease
on expiration)
bounding pulse
weak pulse
collapsing (
femoral pulses may be absent, or delayed
Percussion
right cardiac border does not extend beyond the right
sternal edge
the upper border is at the level of the second
intercostal space
determine cardiac size
Diminished or absent cardiac dullness is found in
emphysema and pneumothorax.
Auscultaition
The ranges for heart rate in infancy and
childhood are:
Newborn 70/120
Infant 80/160
Preschool child
75/120
School child 70/110
Auscultate areas -
Mitral
Tricuspid
Pulmonary
Aortic
3rd & 4th left intercostal
spaces,below left clavicle.
Auscultatory assessment
cardiac rhythm
heart sounds
murmurs.
Third heart sound
ejection click
intensity of heart sounds
Description of murmurs should include
1) site,
2) intensity (graded
0—6) with point of maximum intensity,
3) timing (systolic:
pan, early or late; or diastolic: early diastolic, mid-diastolic or
presystolic,
4) propagation (mitral
systolic murmurs radiate to the left axilla, aortic systolic to the neck,
aortic regurgitant down the left sternal edge) and
5) variation with position. Coarctation of the aorta may produce a murmur audible
over the back.
6) Variation
with respiration
venous hum
pericardial friction
rub
to his ear.
other systems, e.g. by hepatic enlargement in cardiac
failure.
Cervical Lymphadenopathy
Cervical
Lymphadenopathy
most common neck
mass in children.
If - anterior to
the sternocleidomastoid muscle.
Infection is the
usual cause of enlargement; viral etiology and persist for months.
Acute suppurative
submandibular adenitis occur in early childhood (6 mo-3 yrs), is preceded by
pharyngitis or URI, the child develops erythema, swelling and cellulitis, and
management is antibiotics and drainage.
Chronic adenitis: -
persistent node
(> 3 wk., tonsillar),
solitary,
non-tender, mobile and soft.
Generally no treatment
if < 1 cm,
nodes above 2 cm
sizes with rapid growth, clustered, hard or matted do biopsy.
Other causes are:
(1) Mycobacterial adenitis- atypical (MAIS complex), swollen, non-tender,
nor-inflamed, positive skin test, excision is curative,
(2) Cat-Scratch
adenitis- caused by A. Fellis, transmitted by kittens, positive complement
fixation test, minimally tender, fluctuant regional nodes, spontaneous
resolution.
(3) Hodgkin's
disease - teenage and young adults, , non-tender node, associated to weight
loss, biopsy is diagnostic.
Cerebrospinal Fluid Examination
Cerebrospinal Fluid Examination
BASIC CEREBROSPINAL FLUID TESTS
Pressure
Normal cerebrospinal fluid (CSF) pressure = 100-200 mm H2 O.
Attach the end of drip set to LP needle. Do LP with this needle. Keep the drip
set in vertical position. CSF clims though the set. Measure the height of the
CSF column
Elevation is due to increased intracranial pressure.
most common causes of elevated CSF pressure
meningitis and subarachnoid hemorrhage.
Brain tumor and brain abscess cause increased intracranial
pressure after a period of days or weeks.
The CSF pressure varies directly with venous pressure
has no relationship to arterial pressure.
The Queckenstedt sign = increased venous pressure via
jugular vein compression increases CSF pressure at the lumbar region,
a subarachnoid obstruction above the lumbar area prevents
this effect.
Appearance
Normal CSF is clear and colorless.
may be pink or red if red blood cells (RBCs) are present
white and cloudy if there are white blood cells (WBCs) or
high protein content.
there must be more than 400 WBCs/mm3 before the
CSF becomes cloudy.
When blood is present in the CSF for more than 4 hours =
xanthochromia (yellow color) occusr due to hemoglobin pigment from lysed RBCs.
Protein levels of more than 150 mg/100 ml (1.5g/L) may
produce a faint yellowish color -can simulate xanthochromia of RBC origin.
Severe jaundice may also simulate xanthochromia.
Glucose
45 mg/100 ml or higher
in normal persons it is rare to find values below 45 mg/100
ml.
The CSF glucose level is 60% of the serum glucose value
In newborns, the CSF level is about 80% of the serum glucose
level.
It takes 30 min to 2 hours for a change to occur in CSF
values after a change in serum glucose.
CSF glucose level fall –
meningitis due to bacteria, tuberculosis, and fungi.
very early infection the initial CSF glucose value may be
normal,
later it begins to decrease.
only 60%-80% of children with acute bacterial meningitis
have CSF glucose levels below normal
elevated blood glucose levels may mask a decrease in CSF
values
So determine the blood glucose level at the same time that
the CSF specimen is obtained, if intravenous (IV) glucose therapy is being
given.
a low CSF glucose level may be due to peripheral blood
hypoglycemia,
Other causes of hypoglycorrhachia
metastatic carcinoma
Subarachnoid hemorrhage,
leptospiral meningitis
primary amebic meningoencephalitis,
aseptic meningitis or in meningoencephalitis due to mumps,
enteroviruses, lymphocytic choriomeningitis
viral meningitis, encephalitis, brain abscess, syphilis, and
brain tumor, CSF glucose levels typically are normal
Protein
The normal protein concentration of CSF is considered to be
15-45 mg/100 ml
Newborn values are different
From birth to day 30, the range is 75-150 mg/100 ml
From day 90 to 6 months of age, the range is 15-50 mg/100 ml
Values reach adult levels by 6 months of age.
increased protein concentration is proportional to the
degree of leukocytosis in the CSF.
protein concentration is increased by the presence of blood.
mild to moderate protein concentration increase seen with
slight leukocytosis; in
cerebral trauma
brain or spinal cord tumor
brain abscess
cerebral infarct or hemorrhage (CVA
systemic lupus,
uremia, myxedema, multiple sclerosis (MS),
hereditary neuropathy,
chronic CNS infections
Blood in the CSF introduces 1 mg of protein/1,000 RBCs.
when the RBCs begin to lyse, the protein level may appear
disproportionate to the number of RBCs.
In acute bacterial meningitis, the CSF protein is elevated
in about 94% of cases
A marked protein elevation without a corresponding CSF cell
increase is known as "albuminocytologic dissociation."
This has been associated with the Guillain-Barre syndrome
(acute idiopathic polyneuritis) or with temporal (giant cell) arteritis
20% of Guillain-Barre syndrome have normal CSF protein
levels,
Pandy's test, CSF is added to a few drops of saturated
phenol agent. This agent reacts with all protein, more with globulin. Chronic
infections such as (tertiary) syphilis or MS tend to accentuate globulin
elevation and thus may give positive Pandy test results even though the total
CSF protein level may not be greatly increased.
increased CSF gamma-globulin levels occur in – MS
Cell count
Normal CSF contains up to five cells/mm3 , all are
lymphocytes.
In newborns 0-30 cells/cu mm, majority being neutrophils.
conditions that affect the meninges will cause CSF
leukocytosis
degree of leukocytosis depend on the type of irritation, its
duration, and its intensity.
high WBC counts are found in acute meningeal infections.
very early stage, leukocytosis may be minimal
100% of patients with acute bacterial infection have
elevated cell counts
normal count may be misleading.
in bacterial infections, polymorphonuclear neutrophils are
the predominating cell
in viral infections,
chronic nervous system diseases, =, lymphocytes or mononuclears predominate.
tuberculous meningitis, is a bacterial and a chronic type of
infection. - the cells are lymphocytes
coxsackie virus and echovirus infections may have a of
neutrophils
Uremia produce a lymphocytosis
Partial treatment of bacterial meningitis may cause
lymphocytosis.
After therapy is started, WBC values decrease.
with Haemophilus influenzae infection and pneumococcal
infection may take 2 to 14 days for count to become normal
Fungal infections =
elevated neutrophils
Nocardia meningitis or brain abscess, show persistent
neutrophilia
subarachnoid hemorrhage or traumatic spinal fluid taps,
approximately 1 WBC is added to every 700 RBCs
Neonatal CSF
Neonates have higher CSF ranges for protein, glucose, and
cell count than adults
Cell counts 1-7 days after birth average about 5-20/mm3 -
60% neutrophils.
Glucose is about 75%-80% of the blood glucose level.
Culture
The diagnosis of acute bacterial meningitis often depends on
the isolation of the
New borns=
streptococci
Escherichia coli.
Listeria monocytogenes
enteric gram-negative bacteria
age 3 months to 6 years
H. influenzae
Meningococcus
Pneumococcus
In adults, Meningococcus and Pneumococcus
Staphylococci in CNS
operations (e.g., shunt procedures), septicemia, or endocarditis.
if any particular organism is suspected, the laboratory
should be informed so that special media can be used if necessary. =
meningococci grow best in a high carbon dioxide atmosphere, and H. influenzae
should be planted on media provided with a Staphylococcus streak. Culture
detects about 85%
Cell : Glucose
POLYMORPHONUCLEAR with LOW GLUCOSE
Acute bacterial meningitis
POLYMORPHONUCLEAR with
LOW OR NORMAL GLUCOSE
Some cases of early phase acute bacterial meningitis
Primary amebic (Naegleria species) meningoencephalitis
Early phase Leptospira meningitis
POLYMORPHONUCLEAR: with NORMAL
GLUCOSE
Brain abscess
Early phase coxsackievirus and echovirus meningitis
Acute bacterial meningitis with IV glucose therapy
Listeria (about 20% of cases)
LYMPHOCYTIC with LOW GLUCOSE
Tuberculosis meningitis
Cryptococcal (Torula) meningitis
Mumps meningoencephalitis (some cases)
Meningeal carcinomatosis (some cases)
Meningeal sarcoidosis (some cases)
Listeria (about 15% of cases)
LYMPHOCYTIC with NORMAL
GLUCOSE
Viral meningitis
Viral encephalitis
Postinfectious encephalitis
Lead encephalopathy
CNS syphilis (majority of patients)
Brain tumor (occasionally)
Leptospira meningitis (after the early phase)
Listeria (about 15% of cases)
Gram stain
70% positive results in culture-proved acute bacterial
meningitis cases.
a negative Gram stain result does not rule out acute
bacterial meningitis
Latex agglutination tests for bacterial antigens
slide latex agglutination (LA) tests have become available
for detection of pneumococcal, meningococcal,H. influenzae type B, and
streptococcal group B bacterial antigen in CSF
several different strains of meningococci may produce
infection, and it is necessary to have an antibody against each one that it is
desired to detect.
the LA kits are expensive per patient
Cerebrospinal fluid lactate
acute bacterial, tuberculous, and fungal meningitis have
elevated CSF lactate values,
normal persons and in viral ("aseptic") meningitis
do not.
CSF lactate may remain elevated 2-3 days after the start of
antibiotic therapy.
Xanthochromia increases CSF lactate levels
CSF lactate is not specific for bacterial infection,
it is not elevated in all cases of bacterial meningitis,
CSF lactate assay is useful in
patients with symptoms of meningitis +
CSF Gram stain results are negative +
LA test results are also negative.
Such patients could have tuberculous
fungal meningitis
partially treated bacterial meningitis,
meningitis due to other organisms.
Increased CSF lactic acid levels, especially if more than
twice upper limit, suggest that further investigation is essential.
A normal lactate level is not reliable in excluding bacterial
meningitis.
MYCOBACTERIAL MENINGITIS
Mycobacterial meningitis is most common in children between
the ages of 6 months and 5 years
Chest x-ray film = show hilar adenopathy in 50%-90%
normal chest findings are more
Purified protein derivative skin test result is negative in
5%-50%
moderate anemia
erythrocyte sedimentation rate is elevated in 80%
CSF findings typically show
moderate WBC elevation (usually <500/mm3 and almost
always <1,000),
the majority being lymphocytes.
in the early stages, a majority of neutrophil
Protein level is mildly or moderately elevated
Glucose level is decreased in 50%-85%
acid-fast smear
culture
exclusion of other etiologies
evidence of tuberculosis elsewhere,
clinical suspicion.
Acid-fast smears on CSF are positive in about 20%-40%
When findings are atypical, a nucleic acid probe with
polymerase chain reaction (PCR) amplification on CSF can be helpful if it is
available.
- Communication
skills may be enhanced by the use of Bliss symbols, talking typewriters,
and specially adapted computers including artificial intelligence
computers to augment motor and language function.
- Behavior problems interfere with the development of a child with CP; assistance of a psychologist or psychiatrist.
- Learning and attention deficit disorders and mental retardation managed by a psychologist and educator.
- Strabismus, nystagmus, and optic atrophy –consult with ophthalmologist - in the initial assessment.
- Lower urinary tract dysfunction should receive prompt assessment and treatment.
- Several drugs have been used to treat Spasticity, including dantrolene sodium, the benzodiazepines, and baclofen.
Intrathecal
baclofen - used - in selected children with severe spasticity.
Botulinum
toxin - management of spasticity in specific muscle groups, - positive response in - patients studied.
Patients
with incapacitating athetosis occasionally respond to levodopa, and children with dystonia may benefit from
carbamazepine or trihexyphenidyl.
Palpation
Applying the palm of the hand to the chest
Thrills
increased precordial pulsation (apical in left
ventricular hypertrophy and basal and right sided in right ventricular
hypertrophy)
diastolic shock (in the pulmonary area in pulmonary
hypertension)
The apex beat, normally in the fourth or fifth
intercostal space within the mid-clavicular line
pulse wrist
(radial) or inguinal region (femoral).
Sinus arrhythmia (increase
in rate on inspiration with decrease
on expiration)
bounding pulse
weak pulse
collapsing (
femoral pulses may be absent, or delayed
Percussion
right cardiac border does not extend beyond the right
sternal edge
the upper border is at the level of the second
intercostal space
determine cardiac size
Diminished or absent cardiac dullness is found in
emphysema and pneumothorax.
Auscultaition
The ranges for heart rate in infancy and
childhood are:
Newborn 70/120
Infant 80/160
Preschool child
75/120
School child 70/110
Auscultate areas -
Mitral
Tricuspid
Pulmonary
Aortic
3rd & 4th left intercostal
spaces,below left clavicle.
Auscultatory assessment
cardiac rhythm
heart sounds
murmurs.
Third heart sound
ejection click
intensity of heart sounds
Description of murmurs should include
1) site,
2) intensity (graded
0—6) with point of maximum intensity,
3) timing (systolic:
pan, early or late; or diastolic: early diastolic, mid-diastolic or
presystolic,
4) propagation (mitral
systolic murmurs radiate to the left axilla, aortic systolic to the neck,
aortic regurgitant down the left sternal edge) and
5) variation with position. Coarctation of the aorta may produce a murmur audible
over the back.
6) Variation
with respiration
venous hum
pericardial friction
rub
to his ear.
other systems, e.g. by hepatic enlargement in cardiac
failure.
Cerebral Palsy
Cerebral Palsy
Cerebral palsy (CP) is a static encephalopathy
Definition - a nonprogressive disorder of posture and
movement often associated with epilepsy and abnormalities of speech, vision,
and intellect resulting from a defect or lesion of the developing brain.
prevalence of 2/1,000 population.
The condition was first described almost 150yr ago by
Little, an orthopedic surgeon. The primary causes included birth trauma,
asphyxia, and prematurity. During the past 2–3 decades, considerable advances
have been made in obstetric and neonatal care, but there has been no change in
the incidence of CP.
EPIDEMIOLOGY AND
ETIOLOGY.
Prevalence of CP is 4/1,000 live births.
High in babies with increased risk for developing asphyxia
during the perinatal period. One reason is intrapartum asphyxia.
Other - Intrauterine exposure to maternal infection (e.g.,
chorioamnionitis, inflammation of placental membranes, umbilical cord
inflammation, foul-smelling amniotic fluid, maternal sepsis, temperature
greater than 38°C during labor, and urinary tract infection) is associated with
a significant increase in the risk of CP in normal birthweight infants.
High in low birth weight infants, particularly those
weighing less than 1,000g at birth, primarily because of intracerebral
hemorrhage and periventricular leukomalacia.
CLINICAL
MANIFESTATIONS.
CP may be classified into
- physiologic,
- topographic,
- etiologic categories
- functional capacity
physiologic classification = the major motor abnormality,
topographic taxonomy = the involved extremities.
CP is also associated with developmental disabilities, =
- mental retardation,
- epilepsy,
- visual,
- hearing,
- speech,
- cognitive,
- behavioral abnormalities.
Types of CP
Spastic hemiplegia
- Decreased spontaneous movements on the affected side and show hand preference at a very early age.
- The arm is often more involved than the leg and difficulty in hand manipulation is obvious by 1yr of age.
- Walking is usually delayed until 18–24 mo, and a circumductive gait is apparent.
- Examination of the extremities may show growth arrest, particularly in the hand and thumbnail, especially if the contra lateral parietal lobe is abnormal, because this area of the brain influences extremity growth.
- Spasticity is apparent in the affected extremities, particularly the ankle, causing an equinovarus deformity of the foot. An affected child often walks on tiptoes because of the increased tone, and the affected upper extremity assumes a dystonic posture when the child runs.
- Ankle clonus and a Babinski sign may be present, the deep tendon reflexes are increased, and weakness of the hand and foot dorsiflexors is evident. About one third of patients with spastic hemiplegia have a seizure disorder that usually develops during the first year or two, and approximately 25% have cognitive abnormalities including mental retardation.
Spastic diplegia
- Bilateral Spasticity of the legs. The first indication of spastic diplegia is often noted when an affected infant begins to crawl. The child uses the arms in a normal reciprocal fashion but tends to drag the legs behind more as a rudder (commando crawl) rather than using the normal four-limbed crawling movement.
- If the Spasticity is severe, application of a diaper is difficult owing to excessive adduction of the hips.
- Examination of the child reveals Spasticity in the legs with brisk reflexes, ankle clonus, and a bilateral Babinski sign. When the child is suspended by the axillae, a scissoring posture of the lower extremities is maintained.
- Walking is significantly delayed; the feet are held in a position of equinovarus; and the child walks on tiptoes.
- Severe spastic diplegia is characterized by disuse atrophy and impaired growth of the lower extremities and by disproportionate growth with normal development of the upper torso.
- The prognosis for normal intellectual development is excellent for these patients, and the likelihood of seizures is minimal.
- The most common neuropathologic finding is periventricular leukomalacia, particularly in the area where fibers innervating the legs course through the internal capsule.
Spastic
quadriplegia
- The most severe form of CP because of marked motor impairment of all extremities and the high association with mental retardation and seizures.
- Swallowing difficulties are common owing to supranuclear bulbar palsies, and they often lead to aspiration pneumonia.
- At autopsy, the central white matter is disrupted by areas of necrotic degeneration that may coalesce into cystic cavities.
- Neurologic examination shows increased tone and spasticity in all extremities, decreased spontaneous movements, brisk reflexes, and plantar extensor responses.
- Flexion contractures of the knees and elbows are often present by late childhood.
- Associated developmental disabilities, including speech and visual abnormalities, are particularly prevalent in this group of children.
- Children with spastic quadriparesis often have evidence of athetosis and may be classified as having mixed CP.
Athetoid CP
- Rare, especially since the advent of aggressive management of hyperbilirubinemia and the prevention of kernicterus.
- Affected infants are characteristically hypotonic and have poor head control and marked head lag.
- Feeding may be difficult, and tongue thrust and drooling may be prominent. The athetoid movements may not become evident until 1yr of age and tend to coincide with hypermyelination of the basal ganglia, a phenomenon called status marmoratus. Speech is typically affected owing to involvement of the oropharyngeal muscles.
- Sentences are slurred, and voice modulation is impaired. Generally, upper motor neuron signs are not present, seizures are uncommon, and intellect is preserved in most patients.
DIAGNOSIS.
- history
- physical examination
- Exclude progressive disorder of the CNS, including degenerative diseases, spinal cord tumor, or muscular dystrophy.
- Electroencephalogram (EEG) and CT scan - to determine the location and extent of structural lesions or associated congenital malformations.
- Tests of hearing and visual function.
- Multidisciplinary approach is most helpful in the assessment and treatment of such children.
TREATMENT.
A team of
- physicians from specialties
- occupational and physical therapists,
- speech pathologist,
- social worker,
- educator,
- developmental psychologist are needed.
Measures to be taken – 1.handling, 2.exercise, 3.equipments,
4. Soft tissue surgery,
5. Spinal cord surgery, 6. Motorized wheel, 7. Communication, 8.behavior, 9.mental retardation, 10.eye, 11.UTI, 12.drugs
5. Spinal cord surgery, 6. Motorized wheel, 7. Communication, 8.behavior, 9.mental retardation, 10.eye, 11.UTI, 12.drugs
- Parents
should be taught how to handle their child in daily activities such as
feeding, carrying, dressing, bathing, and playing in ways that limit the
effects of abnormal muscle tone.
- They also need to be instructed in the supervision of a series of exercises designed to prevent the development of contractures, especially a tight Achilles tendon. There is no proof that physical or occupational therapy prevents development of CP in infants at risk or that it corrects the neurologic deficit, but ample evidence shows that therapy optimizes the development of an abnormal child.
- Children
with spastic diplegia are treated with equipment, such as walkers, poles,
and standing frames.
- If a
patient has marked spasticity of the lower extremities or evidence of hip
dislocation, consideration should be given to performing surgical soft
tissue procedures that reduce muscle spasm around the hip girdle,
including an adductor tenotomy or psoas transfer and release.
- A
rhizotomy procedure in which the roots of the spinal nerves are divided
has produced considerable improvement in selected patients with severe
spastic diplegia. A tight heel cord in a child with spastic hemiplegia may
be treated surgically by tenotomy of the Achilles tendon.
- Quadriplegia is managed with motorized wheelchairs, special feeding devices, modified typewriters, and customized seating arrangements.
- Communication
skills may be enhanced by the use of Bliss symbols, talking typewriters,
and specially adapted computers including artificial intelligence
computers to augment motor and language function.
- Behavior problems interfere with the development of a child with CP; assistance of a psychologist or psychiatrist.
- Learning and attention deficit disorders and mental retardation managed by a psychologist and educator.
- Strabismus, nystagmus, and optic atrophy –consult with ophthalmologist - in the initial assessment.
- Lower urinary tract dysfunction should receive prompt assessment and treatment.
- Several drugs have been used to treat Spasticity, including dantrolene sodium, the benzodiazepines, and baclofen.
Intrathecal
baclofen - used - in selected children with severe spasticity.
Botulinum
toxin - management of spasticity in specific muscle groups, - positive response in - patients studied.
Patients
with incapacitating athetosis occasionally respond to levodopa, and children with dystonia may benefit from
carbamazepine or trihexyphenidyl.
Palpation
Applying the palm of the hand to the chest
Thrills
increased precordial pulsation (apical in left
ventricular hypertrophy and basal and right sided in right ventricular
hypertrophy)
diastolic shock (in the pulmonary area in pulmonary
hypertension)
The apex beat, normally in the fourth or fifth
intercostal space within the mid-clavicular line
pulse wrist
(radial) or inguinal region (femoral).
Sinus arrhythmia (increase
in rate on inspiration with decrease
on expiration)
bounding pulse
weak pulse
collapsing (
femoral pulses may be absent, or delayed
Percussion
right cardiac border does not extend beyond the right
sternal edge
the upper border is at the level of the second
intercostal space
determine cardiac size
Diminished or absent cardiac dullness is found in
emphysema and pneumothorax.
Auscultaition
The ranges for heart rate in infancy and
childhood are:
Newborn 70/120
Infant 80/160
Preschool child
75/120
School child 70/110
Auscultate areas -
Mitral
Tricuspid
Pulmonary
Aortic
3rd & 4th left intercostal
spaces,below left clavicle.
Auscultatory assessment
cardiac rhythm
heart sounds
murmurs.
Third heart sound
ejection click
intensity of heart sounds
Description of murmurs should include
1) site,
2) intensity (graded
0—6) with point of maximum intensity,
3) timing (systolic:
pan, early or late; or diastolic: early diastolic, mid-diastolic or
presystolic,
4) propagation (mitral
systolic murmurs radiate to the left axilla, aortic systolic to the neck,
aortic regurgitant down the left sternal edge) and
5) variation with position. Coarctation of the aorta may produce a murmur audible
over the back.
6) Variation
with respiration
venous hum
pericardial friction
rub
to his ear.
other systems, e.g. by hepatic enlargement in cardiac
failure.
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