neurological conditions and diseases (at birth)
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Neurological Neurological conditions and conditions and
diseasesdiseases
Post Basic PaediatricsPost Basic Paediatrics
18 April 201218 April 2012
Neurological conditions Neurological conditions and diseasesand diseases
Part IPart I At birth (Congenital, acquired)At birth (Congenital, acquired)
MacrocephalyMacrocephaly MicrocephalyMicrocephaly Spine defectSpine defect Other developmental defectOther developmental defect Birth trauma/HIEBirth trauma/HIE
Part IIPart II During development (Congenital, acquired)During development (Congenital, acquired)
MeningitisMeningitis SeizureSeizure HeadacheHeadache Stroke/VascularStroke/Vascular Neoplasm/TumourNeoplasm/Tumour TraumaTrauma ComaComa
Neurological Neurological conditions and conditions and
diseasesdiseases
Part IPart IAt birth (Congenital, acquired)At birth (Congenital, acquired)
At birth (Congenital, At birth (Congenital, acquired)acquired)
MacrocephalyMacrocephaly MicrocephalyMicrocephaly Spine defectSpine defect Other developmental defectOther developmental defect Birth trauma/HIEBirth trauma/HIE
MacrocephalyMacrocephaly Macrocephaly during the neonatal period results Macrocephaly during the neonatal period results
from enlargement of any component or “space” from enlargement of any component or “space” of the head of the head
The components or spaces of the head most The components or spaces of the head most likely to enlarge are the scalp,likely to enlarge are the scalp, skull, subdural skull, subdural space, subarachnoid space, brain parenchyma, space, subarachnoid space, brain parenchyma, intraparenchymal vessels, and ventricles. intraparenchymal vessels, and ventricles.
Macrocephaly: CausesMacrocephaly: Causes
SCALPSCALP caput succedaneum, caput succedaneum, subgaleal hemorrhage, and subgaleal hemorrhage, and cephalohematoma cephalohematoma
Caput SuccedaneumCaput Succedaneum Caput succedaneum is due to edema between the Caput succedaneum is due to edema between the
skin and the epicranial aponeurosis. skin and the epicranial aponeurosis. It presents as a mass, usually located in the vertex, It presents as a mass, usually located in the vertex,
that crosses the sutures and extends over several that crosses the sutures and extends over several bones. bones.
The mass is soft, superficial, and pitting. The mass is soft, superficial, and pitting. The edema results from compression of the scalp by The edema results from compression of the scalp by
the uterus or suction on the scalp if a vacuum the uterus or suction on the scalp if a vacuum
extractor was used during deliveryextractor was used during delivery
Subgaleal HemorrhageSubgaleal Hemorrhage Subgaleal hemorrhage is due to blood between the Subgaleal hemorrhage is due to blood between the
epicranial aponeurosis and the external periosteum. epicranial aponeurosis and the external periosteum. Subgaleal hemorrhage presents as an evenly spread Subgaleal hemorrhage presents as an evenly spread
mass throughout a large portion of the scalp.mass throughout a large portion of the scalp. The mass is firm, fluctuant, crosses suture lines, and The mass is firm, fluctuant, crosses suture lines, and
increases in size after birth (sometimes at an alarming increases in size after birth (sometimes at an alarming speed)speed)
CephalohematomaCephalohematoma Cephalohematoma presents as a localized mass Cephalohematoma presents as a localized mass
that does not cross suture lines. that does not cross suture lines. It is usually unilateral and over the parietal bone. It is usually unilateral and over the parietal bone. The blood collects between the external The blood collects between the external
periosteum and the bone. periosteum and the bone. The mass is firm, tense, and confined to an The mass is firm, tense, and confined to an
individual bone. individual bone. The edge of the mass may feel like a ridge. The edge of the mass may feel like a ridge. Underlying linear fracture is detected in 10% to Underlying linear fracture is detected in 10% to
25% of cases. 25% of cases. Cephalohematoma is produced by forces that Cephalohematoma is produced by forces that
tend to separate the periosteum from the bone. tend to separate the periosteum from the bone.
Macrocephaly: CausesMacrocephaly: Causes
SKULLSKULL -Osteopetrosis -Osteopetrosis Osteopetrosis is a disorder characterized by Osteopetrosis is a disorder characterized by
overgrowth of brittle bones. overgrowth of brittle bones. This results in thick, dense, and fragile bones. This results in thick, dense, and fragile bones. The bony tissue overgrowth results in The bony tissue overgrowth results in
encroachments of the: encroachments of the: (1) bone marrow leading to anemia, (1) bone marrow leading to anemia, (2) cranial nerves foramina leading to deafness, (2) cranial nerves foramina leading to deafness,
blindness, or other signs of cranial nerve blindness, or other signs of cranial nerve dysfunction, dysfunction,
(3) Pacchioni bodies producing communicating (3) Pacchioni bodies producing communicating hydrocephalus and macrocephaly. hydrocephalus and macrocephaly.
Macrocephaly: CausesMacrocephaly: Causes SUBDURAL SPACESUBDURAL SPACE -Subdural -Subdural
hematomas hematomas Progressive increases in head Progressive increases in head
circumference may be noted during the circumference may be noted during the third week of life. third week of life.
Subdural hematomas present with Subdural hematomas present with irritability or hyperalertness, or with irritability or hyperalertness, or with signs of focal cerebral disturbances signs of focal cerebral disturbances such as seizures, hemiparesis, or gaze such as seizures, hemiparesis, or gaze preference. preference.
The causes of subdural hematomas are The causes of subdural hematomas are trauma and coagulation disorders. trauma and coagulation disorders.
Subdural hematoma is diagnosed by CT Subdural hematoma is diagnosed by CT of the brain. of the brain.
Macrocephaly: CausesMacrocephaly: Causes SUBARACHNOID SPACESUBARACHNOID SPACE
Patients with benign enlargement of the Patients with benign enlargement of the subarachnoid space are usually not born subarachnoid space are usually not born macrocephalic; macrocephalic;
However, some patients with this condition may have However, some patients with this condition may have excessive head growth during the neonatal period. excessive head growth during the neonatal period.
The presence of bilateral enlarged frontal The presence of bilateral enlarged frontal subarachnoid spaces (>5.7 mm), widening of the subarachnoid spaces (>5.7 mm), widening of the Sylvian fissure (>7.6 mm) and other sulci, and Sylvian fissure (>7.6 mm) and other sulci, and normal or minimally enlarged ventricles establishes normal or minimally enlarged ventricles establishes the diagnosis. the diagnosis.
The anterior fontanelle is large and soft to palpation. The anterior fontanelle is large and soft to palpation. Family members, most often the father, may also Family members, most often the father, may also
have a large head have a large head
Macrocephaly: CausesMacrocephaly: Causes
BRAIN PARENCHYMABRAIN PARENCHYMA - -megalencephalymegalencephaly Parenchymal space enlargement occurs Parenchymal space enlargement occurs
in in neurocutaneous disorders, neurocutaneous disorders, Soto syndrome, Soto syndrome, metabolic megalencephalies, and metabolic megalencephalies, and some degenerative disorders. some degenerative disorders. Brain TumorsBrain Tumors
Macrocephaly: CausesMacrocephaly: Causes
Vein of Galen AneurysmVein of Galen Aneurysm Neonates with aneurysm of the vein of Neonates with aneurysm of the vein of
Galen may be macrocephalic at birth. Galen may be macrocephalic at birth. The most common neonatal presentations The most common neonatal presentations
of vein of Galen aneurysm in the neonatal of vein of Galen aneurysm in the neonatal period are cardiac failure, cerebral period are cardiac failure, cerebral infarction, or cerebral bleed. infarction, or cerebral bleed.
Macrocephaly can be caused by the large Macrocephaly can be caused by the large size of the vein of Galen aneurysm, but size of the vein of Galen aneurysm, but most often it is caused by an obstruction of most often it is caused by an obstruction of the aqueduct of Sylvius. the aqueduct of Sylvius.
A cranial bruit is often present in neonates A cranial bruit is often present in neonates with vein of Galen aneurysm. with vein of Galen aneurysm.
Macrocephaly: CausesMacrocephaly: Causes Hydrocephalus Hydrocephalus
Increased amount of CSF within the ventricles Increased amount of CSF within the ventricles of the brainof the brain
May be caused by obstruction of CSF flow or May be caused by obstruction of CSF flow or by overproduction or inadequate reabsorption by overproduction or inadequate reabsorption of CSFof CSF
May result from congenital malformation or be May result from congenital malformation or be secondary to injury, infection, or tumorsecondary to injury, infection, or tumor
HydrocephalusHydrocephalus
Types:Types: Communicating hydrocephalus: Communicating hydrocephalus:
-- Results from unsatisfactory absorption of Results from unsatisfactory absorption of CSF by the arachnoid gratulations or CSF by the arachnoid gratulations or overproduction of CSF by the choroid overproduction of CSF by the choroid plexusplexus
Non-communicating Non-communicating hydrocephalus:hydrocephalus:
-- Results from an obstruction to CSF flow , Results from an obstruction to CSF flow , causing enlargement of only those causing enlargement of only those ventricles proximal to the obstructionventricles proximal to the obstruction
Hydrocephalus: CausesHydrocephalus: Causes
CongenitalCongenital Aqueductal anomaliesAqueductal anomalies- - Primary aqueductal stenosis, or Primary aqueductal stenosis, or
secondary to intrauterine infections i.e. secondary to intrauterine infections i.e. varicella, mumps, TORCHvaricella, mumps, TORCH
-- Dandy-Walker malformationDandy-Walker malformation
-- Chiari malformationChiari malformation
-- MyelomeningoceleMyelomeningocele
Hydrocephalus: CausesHydrocephalus: Causes
AcquiredAcquired-- Post meningitisPost meningitis
-- Post hemorrhage- (SAH, IVH)Post hemorrhage- (SAH, IVH)
-- Masses - vascular malformations, Masses - vascular malformations, neoplasticneoplastic
Clinical PresentationClinical Presentation Increased head circumferenceIncreased head circumference-- Irritability, lethargy, poor feeding, vomiting -Irritability, lethargy, poor feeding, vomiting -
infantinfant-- Headache, lethargy, vomiting- older childHeadache, lethargy, vomiting- older child-- Bulging anterior fontanelleBulging anterior fontanelle-- Widened cranial suturesWidened cranial sutures-- Cracked pot sound on cranial percussionCracked pot sound on cranial percussion- Scalp vein dilatationScalp vein dilatation
Clinical PresentationClinical Presentation Sunset sign - eyes deviate downwardSunset sign - eyes deviate downward
-- Episodic bradycardia, apneaEpisodic bradycardia, apnea
-- Loss of color and peripheral vision(older child)Loss of color and peripheral vision(older child)- Cranial nerve palsies - e.g abnormal pupil Cranial nerve palsies - e.g abnormal pupil
size/reactivity, EOM’s, nystagmussize/reactivity, EOM’s, nystagmus- Spasticity limbsSpasticity limbs
-- Hyperreflexia, clonusHyperreflexia, clonus
Hydrocephalus: Hydrocephalus: AssessmentAssessment
Assessment findings depend on Assessment findings depend on age of onset and age of onset and amount of CSF in the brainamount of CSF in the brain
Infant to 2 years:Infant to 2 years: Enlarging head size, bulging, non-pulsating fontanels, Enlarging head size, bulging, non-pulsating fontanels,
downward rotation of eyes (sunset), poor feeding, downward rotation of eyes (sunset), poor feeding, vomiting, lethargy, irritability, high-pitched cry and vomiting, lethargy, irritability, high-pitched cry and abnormal muscle toneabnormal muscle tone
Older Children:Older Children: Changes in head size less commonChanges in head size less common Signs of increased ICP (vomiting, ataxia, headache) Signs of increased ICP (vomiting, ataxia, headache)
commoncommon Alteration in consciousness and papilloedema late Alteration in consciousness and papilloedema late
signssigns
Hydrocephalus: Hydrocephalus: AssessmentAssessment
Diagnostic Investigations:Diagnostic Investigations: Ultrasound of skull- through anterior fontanelleUltrasound of skull- through anterior fontanelle
Shows ventricular enlargementShows ventricular enlargement CT of headCT of head
- Shows ventricular enlargement, peri-ventricualr Shows ventricular enlargement, peri-ventricualr lucency, narrow/absent sulci, +/- 4 th ventricular lucency, narrow/absent sulci, +/- 4 th ventricular enlargementenlargement
Treatments:Treatments: Serial Spinal tapsSerial Spinal taps Surgery- remove obstruction if possibleSurgery- remove obstruction if possible ShuntsShunts Acetazolamide- decreases blood flow to choroidal Acetazolamide- decreases blood flow to choroidal
arteries , therefore decreasing CSF productionarteries , therefore decreasing CSF production
Hydrocephalus: Hydrocephalus: AssessmentAssessment
Complications:Complications:-- Shunt blockagesShunt blockages-- Infection of shuntInfection of shunt-- Over shuntingOver shunting-- SeizuresSeizures-- BlindnessBlindness-- Cranial nerve dysfunctionCranial nerve dysfunction-- ICPICP-- Cognitive impairmentCognitive impairment
Shunts Shunts Insertion of a flexible tube into the lateral Insertion of a flexible tube into the lateral
ventricle of the brainventricle of the brain Catheter is the threaded under the skin and the Catheter is the threaded under the skin and the
distal end positioned in the peritoneum (common) distal end positioned in the peritoneum (common) or the right atriumor the right atrium
Shunt drains excess CSF from the lateral Shunt drains excess CSF from the lateral ventricles; fluid is the absorbed by the ventricles; fluid is the absorbed by the peritoneum or absorbed in the general circulation peritoneum or absorbed in the general circulation via the right atriumvia the right atrium
Shunts : Nursing Shunts : Nursing InterventionsInterventions
Pre-operativePre-operative Monitor head circumferenceMonitor head circumference Monitor for signs of ICPMonitor for signs of ICP Small frequent feedingsSmall frequent feedings
Post-operativePost-operative Position on opposite side of surgery or backPosition on opposite side of surgery or back Avoid sedationAvoid sedation Monitor for signs of ICPMonitor for signs of ICP Educate parents concerning signs and Educate parents concerning signs and
symptoms of shunt infection or shunt symptoms of shunt infection or shunt malfunctionmalfunction
Macrocephaly: CausesMacrocephaly: Causes
Posthemorrhagic HydrocephalusPosthemorrhagic Hydrocephalus Posthemorrhagic hydrocephalus is the most Posthemorrhagic hydrocephalus is the most
common type of hydrocephalus in the neonatal common type of hydrocephalus in the neonatal period. period.
Posthemorrhagic hydrocephalus may be Posthemorrhagic hydrocephalus may be communicating or noncommunicating. communicating or noncommunicating.
It is usually the consequence of intraventricular It is usually the consequence of intraventricular hemorrhage. hemorrhage.
Intraventricular hemorrhage usually occurs as a Intraventricular hemorrhage usually occurs as a consequence of germinal matrix hemorrhage. consequence of germinal matrix hemorrhage.
Germinal matrix hemorrhages are unusual after 34 Germinal matrix hemorrhages are unusual after 34 weeks gestational age. weeks gestational age.
Macrocephaly: CausesMacrocephaly: Causes Posthemorrhagic HydrocephalusPosthemorrhagic Hydrocephalus
Germinal matrix hemorrhages are classified Germinal matrix hemorrhages are classified based on brain ultrasound in four grades. based on brain ultrasound in four grades.
Grade I intraventricular hemorrhage refers to Grade I intraventricular hemorrhage refers to the presence of subependymal bleed; the presence of subependymal bleed;
Grade II intraventricular hemorrhage refers to Grade II intraventricular hemorrhage refers to extension of the subependymal bleed into the extension of the subependymal bleed into the ventricles but without ventricular dilatation; ventricles but without ventricular dilatation;
Grade III intraventricular hemorrhage refers Grade III intraventricular hemorrhage refers to subependymal bleed with extension of the to subependymal bleed with extension of the bleed into the ventricles and hydrocephalus; bleed into the ventricles and hydrocephalus; and and
Grade IV intraventricular hemorrhage refers Grade IV intraventricular hemorrhage refers to subependymal bleed with extension of the to subependymal bleed with extension of the bleed into the parenchyma as a result of bleed into the parenchyma as a result of venous infarcts venous infarcts
At birth (Congenital, At birth (Congenital, acquired)acquired)
MacrocephalyMacrocephaly MicrocephalyMicrocephaly Spine defectSpine defect Other developmental defectOther developmental defect Birth trauma/HIEBirth trauma/HIE
MicrocephalyMicrocephaly Causes include:Causes include:
-- Premature closure of skull sutures Premature closure of skull sutures (craniosynostosis)(craniosynostosis)
- Microencephaly Microencephaly - small brain due to insult ( infectious, toxic, small brain due to insult ( infectious, toxic,
metabolic, vascular) sustained in the perinatal metabolic, vascular) sustained in the perinatal or early infancy period e.g rubella,CMV, Fetal or early infancy period e.g rubella,CMV, Fetal alcohol syndrome, Genetic disorder - alcohol syndrome, Genetic disorder - microencephaly vera, many syndromes and microencephaly vera, many syndromes and metabolic disordersmetabolic disorders
AnencephalyAnencephaly Defective closure of the rostral neural tube Defective closure of the rostral neural tube
results in anencephaly or encephaloceleresults in anencephaly or encephalocele Neonates with anencephaly have a rudimentary Neonates with anencephaly have a rudimentary
brainstem, or midrain , no cortex or craniumbrainstem, or midrain , no cortex or cranium Rapidly fatal condition if born aliveRapidly fatal condition if born alive
At birth (Congenital, At birth (Congenital, acquired)acquired)
MacrocephalyMacrocephaly MicrocephalyMicrocephaly Spine defectSpine defect Other developmental defectOther developmental defect Birth trauma/HIEBirth trauma/HIE
Neural Tube DefectsNeural Tube Defects
Spina bifidaSpina bifida DiastematomyeliaDiastematomyelia
Spina Bifida Spina Bifida (myelodysplasia) (myelodysplasia)
Neural tube defects that develop during the first Neural tube defects that develop during the first trimester of fetal developmenttrimester of fetal development
Defect can occur at any place along the spinal Defect can occur at any place along the spinal canalcanal
Unknown etiology; thought to be associated with Unknown etiology; thought to be associated with folic acid deficiency in mother’s diet prenatallyfolic acid deficiency in mother’s diet prenatally
Degree of disability dependent on location of the Degree of disability dependent on location of the defect & if spinal nerves involveddefect & if spinal nerves involved
Spina Bifida Spina Bifida (myelodysplasia) (myelodysplasia)
Defective closure of the caudal end of NT at the Defective closure of the caudal end of NT at the end of 4th week of gestationend of 4th week of gestation
Results in anomalies of the lumbar and sacral Results in anomalies of the lumbar and sacral vertebrae or spinal cordvertebrae or spinal cord
Range of severity of CNS defectRange of severity of CNS defect Preventable with pre-conceptual Folic acid Preventable with pre-conceptual Folic acid
supplements 0.4 mg /daysupplements 0.4 mg /day
Spina Bifida Spina Bifida
OccultaOcculta MeningoceleMeningocele MyelomeningoceleMyelomeningocele
Spina bifida “Occulta"Spina bifida “Occulta" Spina bifida "occulta" (meaning "hidden" in latin)Spina bifida "occulta" (meaning "hidden" in latin) Posterior vertebral arches fail to fusePosterior vertebral arches fail to fuse No herniation of meninges or spinal cordNo herniation of meninges or spinal cord May have a tuft of hair or dimpling over the May have a tuft of hair or dimpling over the
lumbarsacral arealumbarsacral area No loss of functionNo loss of function
MeningoceleMeningocele Posterior vertebral arches fail to fusePosterior vertebral arches fail to fuse Sac-like protrusion containing meninges and Sac-like protrusion containing meninges and
cerebral spinal fluidcerebral spinal fluid No spinal nerve involvementNo spinal nerve involvement
MyelomeningoceleMyelomeningocele Sac-like herniation containing meninges, CSF, and Sac-like herniation containing meninges, CSF, and
spinal nerves imbedded in the wall of the sacspinal nerves imbedded in the wall of the sac There may be no signs or symptoms There may be no signs or symptoms The spinal arch has not closed, but the spinal cord The spinal arch has not closed, but the spinal cord
underneath has retained its normal position and is underneath has retained its normal position and is not damagednot damaged
Skin of back intact, small dimple or tuft of hair may Skin of back intact, small dimple or tuft of hair may be present over affected vertebraebe present over affected vertebrae
A child could grow up and never know that he or she A child could grow up and never know that he or she has the defecthas the defect
Nursing Care – Spina Nursing Care – Spina Bifida Bifida
Neurological statusNeurological status Assess degree of sensation at or below lesionAssess degree of sensation at or below lesion Leg movementLeg movement Neurogenic bladderNeurogenic bladder Measure head circumferenceMeasure head circumference High risk of hydrocephalusHigh risk of hydrocephalus High risk for infectionHigh risk for infection High risk for impaired skin integrityHigh risk for impaired skin integrity Altered urinary eliminationAltered urinary elimination Bowel incontinence/constipationBowel incontinence/constipation Impaired physical mobilityImpaired physical mobility
Nursing Care – Spina Nursing Care – Spina Bifida Bifida
SacSac Monitor for leakage of spinal fluidMonitor for leakage of spinal fluid Monitor skin integrity of sacMonitor skin integrity of sac Assess for infection- Sac or systemicAssess for infection- Sac or systemic Position infant on side or abdomenPosition infant on side or abdomen Apply wet, sterile, saline dressingApply wet, sterile, saline dressing Do not allow sac to dry outDo not allow sac to dry out
Nursing Care – Post-Nursing Care – Post-operativeoperative
Defect/sac is surgically closed within 48 hoursDefect/sac is surgically closed within 48 hours Observe for latex allergiesObserve for latex allergies Neurogenic bladder: straight catheterizationNeurogenic bladder: straight catheterization Neurogenic bowel: bowel management programNeurogenic bowel: bowel management program Monitor for signs/ symptoms of hydrocephalusMonitor for signs/ symptoms of hydrocephalus
DiastematomyeliaDiastematomyelia
-- A bone or fibrous band divides spinal cord in two A bone or fibrous band divides spinal cord in two longitudinal sectionslongitudinal sections
-- Associated lipoma may be present, which tethers Associated lipoma may be present, which tethers cord to vertebracord to vertebra
-- Signs and symptoms include weakness, numbness in Signs and symptoms include weakness, numbness in feet, urinary incontinence, decreased or absent feet, urinary incontinence, decreased or absent reflexes in feetreflexes in feet
-- Treatment - surgery to free cordTreatment - surgery to free cord
At birth (Congenital, At birth (Congenital, acquired)acquired)
MacrocephalyMacrocephaly MicrocephalyMicrocephaly Spine defectSpine defect Other developmental defectOther developmental defect Birth trauma/HIEBirth trauma/HIE
EncephaloceleEncephalocele
Skull defect with exposure of Skull defect with exposure of meninges alone or meninges and meninges alone or meninges and brainbrain
Sometimes defect can cause Sometimes defect can cause protrusion of frontal lobe through protrusion of frontal lobe through the nosethe nose
At birth (Congenital, At birth (Congenital, acquired)acquired)
MacrocephalyMacrocephaly MicrocephalyMicrocephaly Spine defectSpine defect Other developmental defectOther developmental defect Birth trauma/HIEBirth trauma/HIE
Hypoxic-ischemic Hypoxic-ischemic Encephalopathy Encephalopathy
Hypoxic ischemic encephalopathy (HIE) refers to the CNS dysfunction associated with perinatal asphyxia.
HIE is of foremost concern in an asphyxiated neonate because of its potential to cause serious long-term neuromotor sequelae among survivors.
A simple and practical classification of HIE by severity of manifestations provided by Levene
Hypoxic-ischemic Hypoxic-ischemic Encephalopathy Encephalopathy
Hypoxic-ischemic encephalopathy often involves the Hypoxic-ischemic encephalopathy often involves the brain and the brainstem. brain and the brainstem.
Very severe hypoxic-ischemic encephalopathy may Very severe hypoxic-ischemic encephalopathy may involve the brain, brainstem, spinal cord, and muscle. involve the brain, brainstem, spinal cord, and muscle.
Magnetic resonance imaging of the brain in neonates Magnetic resonance imaging of the brain in neonates with hypotonia due to hypoxic-ischemic with hypotonia due to hypoxic-ischemic encephalopathy shows loss of gray-white matter encephalopathy shows loss of gray-white matter interface, cortical necrosis, or neuronal loss of the interface, cortical necrosis, or neuronal loss of the basal ganglia and thalamus. basal ganglia and thalamus.
Mental RetardationMental Retardation
Significant below average Significant below average intellectual functioning which is intellectual functioning which is associated with impaired learning associated with impaired learning difficultiesdifficulties
CausesCauses Pre-natalPre-natal PerinatalPerinatal Post-natalPost-natal
Mental RetardationMental Retardation: : CausesCauses
• Pre-natal -Pre-natal -Genetic DisordersGenetic Disorders• Chromosomal aberrations- e.g. Chromosomal aberrations- e.g. Down syndrome Down syndrome
(trisomy 21 )(trisomy 21 )• Disorders with autosomal-dominant Disorders with autosomal-dominant
inheritance- e.g. inheritance- e.g. Tuberous sclerosisTuberous sclerosis• Disorders with autosomal-recessive Disorders with autosomal-recessive
inheritance- metabolic disorder; e.g. inheritance- metabolic disorder; e.g. PhenylketonuriaPhenylketonuria
• X-linked mental retardation- X-linked mental retardation- Fragile X syndromeFragile X syndrome• Maternal infections- e.g. Maternal infections- e.g. Rubella infection during Rubella infection during
the first month of pregnancy the first month of pregnancy • Toxic substances- Toxic substances- fetal alcohol syndromefetal alcohol syndrome• Toxemia of pregnancy and placental Toxemia of pregnancy and placental
insufficiencyinsufficiency
Mental RetardationMental Retardation: : CausesCauses
• Perinatal (Perinatal (This period refers to 1 week before This period refers to 1 week before birth to 4 weeks after birth )birth to 4 weeks after birth )• Infections -e.g. Infections -e.g. herpes simplex type 2 herpes simplex type 2 • Delivery problems – e.g. birth Delivery problems – e.g. birth asphyxia asphyxia • Other perinatal problemsOther perinatal problems
• Retinopathy of prematurity Retinopathy of prematurity • Hyperbilirubinemia Hyperbilirubinemia
Mental RetardationMental Retardation: : CausesCauses
• PostnatalPostnatal• InfectionsInfections
• Bacterial and viral infections of the brain during childhood Bacterial and viral infections of the brain during childhood may cause meningitis and encephalitis and result in may cause meningitis and encephalitis and result in permanent damage permanent damage
• Toxic substancesToxic substances –e.g. Lead poisoning –e.g. Lead poisoning • Other postnatal causesOther postnatal causes
• Childhood malignancies, brain tumors Childhood malignancies, brain tumors • TraumaTrauma
• Psychosocial problemsPsychosocial problems –e.g. Severe maternal –e.g. Severe maternal mental illness mental illness
• Unknown causesUnknown causes • no cause can be identified in approximately 30% of cases of no cause can be identified in approximately 30% of cases of
severe mental retardation and in 50% of cases of mild severe mental retardation and in 50% of cases of mild mental retardation mental retardation
MR – Classifications MR – Classifications MildMild
Slow learner, can work, marry, have children, Slow learner, can work, marry, have children, may need assistance with crisismay need assistance with crisis
ModerateModerate Needs life supervisionNeeds life supervision
SevereSevere Needs a caretaker for basic needsNeeds a caretaker for basic needs
ProfoundProfound
Interventions Interventions
Goal is to promote Goal is to promote Optimal developmentOptimal development Family supportFamily support Community referralsCommunity referrals
Cerebral Palsy Cerebral Palsy A non-progressive motor disorder of the CNS A non-progressive motor disorder of the CNS
resulting in alteration in movement and postureresulting in alteration in movement and posture Cause is trauma, hemorrhage, anoxia or infection Cause is trauma, hemorrhage, anoxia or infection
before, during or after birthbefore, during or after birth 1/3 of children have some degree of mental 1/3 of children have some degree of mental
retardationretardation Classified as:Classified as:
SpasticSpastic Spasticity (hypertonicity of muscle groups)Spasticity (hypertonicity of muscle groups) AthetoidAthetoid Worm-like movements of extremitiesWorm-like movements of extremities AtaxicAtaxic Disturbed coordinationDisturbed coordination MixedMixed
Cerebral Palsy – Cerebral Palsy – Assessment Assessment
May have hypertonicity or hypotonia of varying May have hypertonicity or hypotonia of varying degrees on different extremitiesdegrees on different extremities
May have scissoring of the legsMay have scissoring of the legs Absence of expected reflexes or presence of Absence of expected reflexes or presence of
reflexes that extend beyond expected agereflexes that extend beyond expected age Failure to meet developmental milestonesFailure to meet developmental milestones Difficulty swallowingDifficulty swallowing Altered speechAltered speech
Nursing Care Nursing Care
Impaired physical mobilityImpaired physical mobility Self-care deficitSelf-care deficit Altered nutrition: less than body Altered nutrition: less than body
requirementsrequirements High risk for injury related to High risk for injury related to
neuromuscular, perceptual or neuromuscular, perceptual or cognitive impairmentscognitive impairments
Treatment Treatment
Self-care is a goal for all childrenSelf-care is a goal for all children Team approachTeam approach NutritionNutrition Increased caloric intakeIncreased caloric intake Special feeding devicesSpecial feeding devices Community referralsCommunity referrals Emotional supportEmotional support
At birth (Congenital, At birth (Congenital, acquired)acquired)
MacrocephalyMacrocephaly MicrocephalyMicrocephaly Spine defectSpine defect Other developmental defectOther developmental defect Birth trauma/HIEBirth trauma/HIE
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