PEDIATRIC NEUROLOGICAL CONDITIONS Unit 4: Part 2 Module

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PEDIATRIC NEUROLOGICAL CONDITIONS

• Unit 4 Part 2 Module 3

Prepared by C. Sargo RN (EC) Reviewed by Maj.
Quinn RN (EC)
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REFERENCES

• C 290- Nelsons Essentials of Pediatrics
• C 306- Toronto Sick Kids manual
• 277- Toronto Notes
• 2001 Anti-infective Guidelines
• Class handout

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OUTLINE

• Embryonic Development of the CNS
• Primary Pathways and Symptoms Of Neurological
  Disorders in Children
• Neural Tube Defects
• Acute Pediatric Coma
• Seizure Disorders

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OUTLINE

• Headaches
• Head Injuries
• Meningitis
• Hypotonia in the Neonate
• Hydrocephalus
• Childhood Neurological Malignancies

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EMBRYONIC DEVELOPMENT OF THE CNS

• CNS arises from the neural plate of embryonic
  ectoderm
• Neural plate gives rise to neural tube
• Neural tube forms the brain , spinal cord and
  neural crest cells which form the peripheral NS,
  meninges, melanocytes and adrenal medulla
• NT begins to form on day 22 of gestation
• The lumen of the NT forms the ventricles and
  central canal of the spinal cord

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PRIMARY PATHWAYS OF CNS DISORDERS

• Congenital anomalies e.g NTD- spina bifida
• Congenital inborn errors of metabolism e.g.
• Infection - e.g meningitis
• Neoplasm - brain tumors
• Vascular anomalies - e.g. AV malformation

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PRIMARY PATHWAYS OF CNS DISORDERS

• Nutritional deficiency
• Autoimmunity
• Cellular degeneration
• Intoxication - acute poisoning
• Trauma - e.g acute head injury
• Anoxia

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PRIMARY SIGNS SYMPTOMSOF CNS DISORDERS

• Cognitive deficits- memory,mood , concentration,
  personality changes
• Seizures
• Headache
• Dizziness

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PRIMARY SIGNS SYMPTOMSOF CNS DISORDERS

• Vision or hearing loss
• Impairment of swallowing or respiration
• Weakness/numbness/paresthesia
• Difficulty walking/talking
• Incontinence

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NEURAL TUBE DEFECTS

• Spina bifida
• - Defective closure of the caudal end of NT at
  the end of 4th week of gestation
• - Results in anomalies of the lumbar and sacral
  vertebrae or spinal cord
• - Range of severity of CNS affects
• - Preventable with pre-conceptual Folic acid
  supplements 0.4 mg /day

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NEURAL TUBE DEFECTS

• Spina bifida "oculta" (meaning "hidden" in latin)
• - There may be no signs or symptoms
• - The spinal arch has not closed, but the spinal
  cord underneath has retained its normal position
  and is not damaged
• - Skin of back intact, small dimple or tuft of
  hair may be present over affected vertebrae
• - A child could grow up and never know that he or
  she has the defect

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MYELOCELE
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ANENCEPHALY

• Defective closure of the rostral neural tube
  results in anencephaly or encephalocele
• Neonates with anencephaly have a rudimentary
  brainstem , or midrain , no cortex or cranium
• Rapidly fatal condition if born alive

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ANENCEPHALY
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ANENCEPHALY
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NEURAL TUBE DEFECTS

• Diastemamyelia
• - A bone or fibrous band divides spinal cord in
  two longitudinal sections
• - Associated lipoma may be present, which tethers
  cord to vertebra
• - SS include weakness, numbness in feet, urinary
  incontinence, decreased or absent reflexes in
  feet
• - Rx - surgery to free cord

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ENCEPHALOCELE

• Skull defect with exposure of meninges alone or
  meninges and brain
• Sometimes defect can cause protrusion of frontal
  lobe through the nose

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ENCEPHALOCELE
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MACROCEPHALY

• Results from
• Macrocrania- increased skull thickness
• Hydrocephalus- enlarged ventricles
• Megalencephaly -enlargement of brain

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MICROCEPHALY

• Causes include
• - Premature closure of skull sutures
  ((craniosynostosis)
• - Microencephaly - small brain due to insult (
  infectious, toxic, metabolic, vascular)
  sustained in the perinatal or early infancy
  period e.g rubella,CMV, Fetal alcohol syndrome
• - Genetic disorder - microencephaly vera
• - Many syndromes and metabolic disorders are
  associated See table 18-1, PG 769, Nelsons

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PEDIATRIC COMA

• Most common pattern in children is diffuse
  impairment of cerebral hemispheres
• Less commonly results from brainstem dysfunction

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PEDIATRIC COMA

• Useful mnemonics for Differential Dx of causes
• T - trauma
• I - insulin/hypoglycemia/inborn errors of
  metabolism/intususception
• P - Psychiatric
• S - seizures, stroke, shock, shunt malfunction

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PEDIATRIC COMA

• A- alcohol abuse
• E- electrolytes, encephalopathy, endocrinopathy
• I - infection
• O - overdose/ingestion
• U - uremia

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PEDIATRIC COMA-INITIAL APPROACH

• Primary Survey
• - ABCs - C-spine precautions
• - Pediatric Glasgow Coma Scale
• - Vital signs including rectal temperature
• - Check for signs of obvious trauma
• - Check for SS of raided ICP
• - Hypoglycemia- give glucose 0.5 g/kg( D50W, 1-2
  ml/kg IV empirically - chemstrip sugar low
• - Narcan empirically 0.1 mg/kg if pupils
  small/pinpoint

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PEDIATRIC COMA-INITIAL APPROACH

• Secondary survey
• - History - known underlying cause, acute fever,
  trauma, ingestion, PMH, Medications, allergies,
  last meal
• - General CPX including CNS exam
• - Look for evidence of infection, intoxication,
  traumatic and metabolic causes
• - Fontanelle, neck stiffness, neck bruits, fundi(
  retinal hemorrhages), oculomotor movements
• - Breathing patterns
• - Motor responses ( focalizing/lateralizing
  signs)

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PEDIATRIC COMA-INITIAL APPROACH

• Investigations - depends on potential etiology
  and clinical condition
• Blood work may include
• - CBC, cultures, glucose, electrolytes, BUN,
  creatinine, Calcium, magnesium, LFTs, ammonium,
  blood clotting screen, ABG

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PEDIATRIC COMA-INITIAL APPROACH

• Diagnostic Imaging
• - CT of head essential if focal causes suspected
  e.g trauma not if diffuse cause e.g infection
• - CRX, C-spine XR, Flat plate of Abdomen, limb XR
  
• - urinalysis, CS, latex agglutination
• - LP- CSF analysis
• - ECG
• - EEG

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PEDIATRIC COMA-INITIAL APPROACH

• Further management directed at underlying cause
• Serial Glasgow coma scale assessments
• Maintain homeostasis with
• - Oxygen/CO2
• - IV fluids
• - Acid base , electrolytes
• - Nutrition

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PEDIATRIC GLASGOW COMA SCALE

• Eye Opening
• Spontaneous 4
• To speech 3
• To pain 2
• No Response 1

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PEDIATRIC GLASGOW COMA SCALE

• Best Verbal Response
• Oriented (Infant coos or babbles) 5
• Confused (Infant irritable cries) 4
• Inappropriate words (Infant Cries to pain) 3
• Incomprehensible sounds (Infant Moans to pain) 2
• No Response 1

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PEDIATRIC GLASGOW COMA SCALE

• Best Motor Response
• Obeys (Infant moves spontaneous/purposefully) 6
• Localizes (infant withdraws to touch) 5
• Withdraws to pain 4
• Abnormal Flexion to pain (Decorticate) 3
• Extensor Response to pain (Decerebrate) 2
• No Response 1

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PEDIATRIC GLASGOW COMA SCALE

• Scoring- Total 3-15
• Minor head injury 13-15
• Moderate head injury 9-12
• Severe head injury (coma) lt 8
• Confers Significant Mortality Risk lt 8

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SEIZURES

• Classification of Seizures
• Partial
• Generalized

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SEIZURES

• Simple Partial Seizure
• - Affects only part of brain (focal, motor or
  sensory)
• - Formerly called focal seizures
• May progress to generalized seizures
• Complex Partial Seizure
• Partial seizure with affective or behavioral
  changes
• Absence seizures( spells)

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SEIZURES

• Febrile Seizure
• - Associated with temperature gt38C
• - Occurs in children lt6 years old (prevalence is
  2 to 4 among children lt5 years old)
• - No signs or history of underlying seizure
  disorder
• - Often familial
• - Uncomplicated and benign if seizure is of
  short duration (lt15 minutes), only 1 in 24
  hours,and normal CNS exam after seizure
• - Involves tonic-clonic movements, bilaterally

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SEIZURES

• HISTORY
• Previous episodes (i.e., known seizures)
• Nature of Current Seizure episode
• - Onset (sudden or gradual), time ,
  setting, duration of seizure
• - Whether consciousness has been regained since
  onset of seizure activity
• - post ictal state - drowsiness, stupor,
  headache, transient paralysis

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SEIZURES

• HISTORY ( continued)
• Sequence of seizures
• Type of seizure (generalized or partial)
• Association with fever, cyanosis, incontinence
• Association with head injury
• Ingestion of poisonous substance or other
  poisoning (e.g., lead encephalopathy)

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SEIZURES

• HISTORY ( continued)
• Medication use -compliance with anticonvulsant
  therapy if child known to have seizures
• Other chronic disease
• Allergies
• Symptoms of inter-current illness (e.g., fever,
  malaise, cough)
• Growth/Developmental history
• Family Hx. Of seizure disorders

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SEIZURE DISORDERS

• Differential Diagnosis
• - Epilepsy
• - Drugs (non-compliance with prescription,
  withdrawal syndrome, overdose, multiple drug
  abuse)
• - Hypoxia
• - Brain tumor
• - Infection (e.g., meningitis)
• - Metabolic disturbances (e.g., hypoglycemia,
  uremia, liver failure, electrolyte disturbance)
• - Head injury

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SEIZURE DISORDERS

• Diagnostic tests
• - Pulse oximetry
• - Glucose, electrolytes, calcium, magnesium
• - Anticonvulsant drug levels in on Rx
• - Diagnostic Imaging for undiagnosed cases
• - EEG
• - CT or - MRI
• - ? LP- CSF analysis

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SEIZURE DISORDERS

• Complications
• - Hypoxia during seizures
• - Status epilepticus
• - Arrhythmia
• - Injury during seizure (e.g., from a fall)
• - Brain damage
• - Death

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MANAGEMENT ACUTE SEIZURE

• ABCs are the first priority
• Ensure airway is clear and patent
• Suction secretions as necessary
• Insert oropharyngeal airway
• Assist ventilation as needed by means of Ambu-bag
  with oxygen

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MANAGEMENT ACUTE SEIZURE

• Oxygen as necessary to maintain oxygen saturation
  gt97
• Start IV therapy with normal saline, adjusting
  rate according to state of hydration
• Nurse child in side-lying position
• Keep child warm
• Give nothing by mouth until child has fully
  recovered

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MANAGEMENT ACUTE SEIZURE

• Pharmacologic Interventions
• Lorazepam (Ativan), 0.050.10 mg/kg IV (maximum
  4 mg per dose), repeat q10min for 2 more doses
  (administer slowly over 5 minutes, maximum rate 2
  mg/min)
• OR
• Diazepam (Valium), 0.3 mg/kg IV (maximum 5 mg
  per dose for child lt5 years old, 10 mg per dose
  for child gt5 years old)
• Repeat q5-10 min for 2 more doses (administer
  slowly over 5 minutes, maximum rate 2 mg/min)

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MANAGEMENT ACUTE SEIZURE

• If cannot get IV access - give per rectum
• - diazepam (Valium) 0.5 mg/kg per dose PR
  (maximum dose 10 mg)
• - repeat q510min for total of 2 doses (maximum
  rate 2 mg/min)

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MANAGEMENT ACUTE SEIZURE

• Monitoring and Follow-Up
• - Admit
• - Identify focal neurological deficits, clinical
  conditions e.g infection
• - Observe for return to normal level of
  consciousness
• - Monitor vital signs, ABCs, pulse oximetry (if
  available)
• - Monitor closely for continued seizure activity

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MANAGEMENT CHRONIC SEIZURE

• Provide reassurance
• Client Education
• - Explain prognosis - most children outgrow
• - Emphasize importance of adhering to medication
  regimen
• - Counsel about first aid during seizures
• - Advise supervision during swimming, use of
  helmets
• - Advise that the child be treated as a normal
  child would be
• - Advise about possible teratogenic effects of
  medications (e.g., phenytoin) for sexually active
  females

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MANAGEMENT CHRONIC SEIZURE

• Commonly Used Anticonvulsants
• carbamazepine (Tegretol)
• lamotrigine (Lamictal)
• phenobarbital (Phenobarb) - kids lt 2
• phenytoin (Dilantin)
• primidone (Mysoline)
• valproic acid (Depakene)
• vigabatrin (Sabril)

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MANAGEMENT CHRONIC SEIZURE

• Monitoring and Follow-Up
• - Follow up every 6 months if seizures are well
  controlled, more frequently if child is having
  breakthrough seizures
• - Assess adherence to medication regimen
• - Monitor serum drug levels every 6 months if
  stable, more frequently if necessary
• - Refer urgently if child is having breakthrough
  seizures
• - Consider neurological follow-up if symptoms are
  not controlled on current medications

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HEADACHES

• Occurs in 20 of school-age children. Onset may
  occur at any age
• The most common causes of headache in children
• - benign vascular headaches (leading to migraine)
• - muscle contraction (leading to tension
  headaches)

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HEADACHES

• Vascular Organic Causes
• - Arteriovenous malformation
• - Berry aneurysm
• - Cererbral infarction
• - Intracranial hemorrhage

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HEADACHES

• Other causes
• Infection
• Trauma
• Toxic Effects
• Psychogenic
• Organic -Traction

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HEADACHES

• Food allergy or sensitivity
• Refractive error
• Ocular muscle imbalance
• Temporomandibular joint (TMJ) dysfunction
• Hypertension

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HEADACHES

• History - CHLORIDE PEPPS
• Associated symptoms - ROS
• PMH
• Family HX
• Social Hx - stress, environmental factors
• CPX including Neurological exam

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HEADACHES

• Physical exam- Physical findings are usually
  minimal with headaches
• - Blood pressure usually normal
• - Temperature may be elevated with infectious
  process (e.g., meningitis)
• - Height and weight

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HEADACHES

• HEENT
• - Pained facial expression
• - Nuchal rigidity ( neck stiffness)
• - Funduscopic examination (disks, blood
  vessels) results usually normal
• - Spasm or tenderness of neck muscle, tenderness
  of TMJ

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HEADACHES

• HEENT Exam
• - Deficits of cranial nerves
• - Purulent rhinorrhea
• - Halitosis, dental abscesses
• - Cephalic bruits use bell of stethoscope over
  the fronto-temporal areas and orbits

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HEADACHES

• Neurologic Examination
• - Level of consciousness,
• - Mental status general demeanor, confusion,
  depression, stress
• - Cutaneous lesions (café au lait spots)
• - Focal abnormalities (e.g., tics, limb paresis)
• - Sensory deficits
• - Abnormal deep tendon reflexes

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CLINICAL CHARACTERISTICS OF SPECIFIC HEADACHES

• Organic -Traction
• - Headaches increase rapidly in frequency and
  severity
• - Headache is worst upon awakening in the
  morning, diminishes during the day
• - Headache wakens child from sleep
• - Aggravated by coughing or valsalva maneuver
• - May be relieved by vomiting
• - Associated symptoms focal neurological
  findings altered gait changes in behavior,
  personality, cognition or learning ability

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CLINICAL CHARACTERISTICS OF SPECIFIC HEADACHES

• Migraine
• - Headache - pulsatile (throbbing)
• - Headaches are periodic, separated by
  symptom-free intervals
• - Associated with at least three of the following
  symptoms abdominal pain and nausea or vomiting,
  aura (motor, sensory, visual), family history of
  migraine
• - Unilateral or bilateral
• - Headache relieved by sleep

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CLINICAL CHARACTERISTICS OF SPECIFIC HEADACHES

• Tension Headache
• - Band-like tightness or pressure in the
  bifrontal, occipital or posterior cervical
  regions
• - Seen at any age
• - Lasting for days or weeks but not disrupting
  regular activities
• - Not associated with a prodrome
• - Associated symptoms tight neck muscles, sore
  scalp, nausea, vomiting and aura are uncommon

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CLINICAL CHARACTERISTICS OF SPECIFIC HEADACHES

• Refractive Error
• - Persistent frontal headache, which is worse
  while reading or doing schoolwork
• TMJ Dysfunction
• - Temporal headache
• - Associated symptoms local jaw discomfort,
  malocclusion (crossbite), decreased range of
  motion of mouth, click with jaw movement, bruxism
  (grinding of teeth)

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CLINICAL CHARACTERISTICS OF SPECIFIC HEADACHES

• Chronic Sinusitis
• - Frontal headache
• - Tenderness to percussion over the frontal,
  maxillary or nasal sinuses
• - Associated symptoms prolonged rhinorrhea and
  congestion, chronic cough and postnasal drip,
  anorexia, low-grade fever, malaise
• It is unusual for children lt10 years old to have
  recurrent headaches secondary to chronic sinusitis

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HEADACHES

• Complications
• - Recurrent or chronic headaches can be
  debilitating and may cause absences from school
  and social withdrawal
• - Intracranial lesions, masses or infections are
  life-threatening
• Diagnostic Tests
• - Most headaches can be diagnosed from the
  history and physical examination.
• - For recurrent or chronic headache, diagnostic
  information may include daily headache record
• - CT scan if suspect organic cause

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MANAGEMENT OF HEADACHES

• Goals of treatment depend on the cause
• Acute
• - Rule out serious organic pathology
• - Relieve pain
• Recurrent or Chronic
• - Relieve pain
• - Prevent recurrence
• - Avoid disruption of normal life tasks, such as
  attending school

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MANAGEMENT OF HEADACHES

• Consult a physician immediately in the following
  circumstances
• - Concern about an underlying organic cause for
  headaches
• - Uncertainty about the diagnosis
• - Headaches are chronic and unresponsive to
  simple analgesia

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MANAGEMENT OF HEADACHES

• Supportive reassurance and education are
  appropriate for non-organic headaches
• - Advise parents or caregiver that headaches in
  children are common and real
• - Reassure family that headache is unlikely to
  indicate brain tumor
• - Explain underlying pathophysiology of vascular
  and muscle contraction headaches (which are
  benign and have a favorable prognosis)
• - Counsel about avoiding factors that trigger
  headaches

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MANAGEMENT OF HEADACHES

• - Identify stressors and advise on how to deal
  with them
• - Counsel about use of medications (dose,
  frequency, side effects)
• - Relaxation and Imagery Therapy
• - Abdominal breathing exercises
• - Visual imagery exercises

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MANAGEMENT OF HEADACHES

• For tension headaches and mild -moderate
  migraines,
• - Acetaminophen (Tylenol), 10-15 mg/kg per dose
  (usually analgesic of choice)
• - Children gt6 years old may be given 325 mg, and
  children gt12 years old may be given 325-650 mg
  PO q4h prn.
• OR
• - Nonsteroidal anti-inflammatory drugs (NSAIDs)
• ibuprofen (Motrin), 5-10 mg/kg per dose PO q8h
  prn, to daily maximum of 40 mg/kg

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MANAGEMENT OF HEADACHES

• Avoid narcotics
• Migraine prophylaxis
• Conduct follow-up visits
• - Review headache diary if unable to identify
  cause on first visit, as well as to monitor
  management
• - Reinforce balanced health habits of sleep,
  exercise and diet

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HEAD INJURIES

• HISTORY-Ascertain the following
• - Mechanism of injury
• - Time of injury
• - Loss of consciousness (a brief seizure at the
  time of injury) may not be clinically significant
• - Loss of memory , amnesia
• - Irritability

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HEAD INJURIES

• History (continued)
• - Visual disturbance
• - Disorientation
• - Abnormal gait
• - Lethargy, pallor or agitation may indicate
  severe injury
• - Vomiting
• - Symptoms of increased intracranial pressure
  (vomiting, headache, irritability)

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HEAD INJURIES

• Physical Examination
• - Vital Signs
• - Tachypnea
• - Bradycardia (with hypertension - Cushing
  response)
• - Hypertension
• - Hypotension

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HEAD INJURIES

• Signs of Skull Fracture
• - Hematotympanum
• - Periorbital or post-auricular ecchymosis
• - Cerebrospinal fluid otorrhea or rhinorrhea
• - Depressed fracture or penetrating injury
• - Palpate scalp for hematomas and contusions,
  underlying depressions, which may signify
  depressed skull fracture

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HEAD INJURIES

• Neurologic Examination
• - Pediatric Glasgow coma scale
• - Papilledema
• - Pupillary light reflexes (PERRLA)
• - Cranial nerve examination
• - Movement of extremities
• - Abnormal posture (decorticate or decerebrate)
• - Muscle flaccidity, spasticity
• - Plantar responses

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HEAD INJURIES

• Classification of HI Canadian Pediatric Society
• Mild
• Moderate
• Severe

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HEAD INJURIES

• MANAGEMENT MILD INJURY
• Children with mild intracranial injury may be
  discharged home
• An instruction sheet should be given to the
  parents or caregiver concerning observation and
  precautions
• See Home Care Instructions for minor head
  injuries

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HEAD INJURIES

• MODERATE TO SEVERE INJURY
• - ABCs first priority
• - C-spine control
• - Suture scalp lacerations, as major blood loss
  can occur
• - Start IV therapy with normal saline to keep
  vein open (unless the child is in shock from
  other injuries)
• - Restrict fluids to 60 of normal intake (except
  in cases of shock)
• - Oxygen

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HEAD INJURIES

• MODERATE TO SEVERE INJURY
• - Elevate head of bed by 30 to 45
• - Place head and neck in midline position
• - Minimize stimuli (e.g., suctioning and
  movement)
• - To control increased intracranial pressure
  above measures
• plus establish controlled hyperventilation
• - CT scan of head
• - C-spine x-ray

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HEAD INJURIES

• MODERATE TO SEVERE INJURY
• - Diuretics if intracranial pressure is increased
  (and there is documented deterioration) despite
  measures outlined abovemannitol, 0.5-1 g/kg IV
• - Monitor ABCs, vital signs, pulse oximetry,
  level of consciousness (with serial pediatric
  Glasgow coma scores), intake and output

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MENINGITIS

• Definition
• - Inflammation of the meningeal membranes of the
  brain or spinal cord
• - Most cases (70) occur in children lt5 years old
• - May be secondary to other localized or systemic
  infections (e.g., otitis media).
• Causes
• - Meningitis may be caused by bacteria, viruses,
  fungi and (rarely) parasites.

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MENINGITIS

• Bacterial
• - In children lt1 month old group B
  Streptococcus, Escherichia coli
• - In children 4-12 weeks old E. coli, Hemophilus
  influenzae type B, Streptococcus pneumoniae,
  group B Streptococcus, Neisseria meningitidis
  (meningococcal)
• - In children 3 months to 18 years old
  Streptococcus pneumoniae (most common cause), N.
  meningitidis, H. influenza type B (rare)
• - Mycobacterium tuberculosis

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MENINGITIS

• Viral
• - Approximately 70 strains of enteroviruses
• Fungal
• - Candida
• Aseptic
• - Lyme disease

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MENINGITIS

• Transmission
• - Meningitis caused by H. influenzae airborne
  droplets and secretions
• - Meningococcal meningitis (caused by N.
  meningitidis) direct contact with droplets or
  secretions

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MENINGITIS

• Incubation
• - Meningitis caused by H. influenzae 2-4 days
• - Meningococcal meningitis (caused by N.
  meningitidis) 2-10 days
• Contagion
• - Meningitis caused by H. influenzae moderate
  high risk of transmission in daycare centers and
  other crowded environments
• - Meningococcal meningitis (caused by N.
  meningitidis) low spreads most rapidly in
  crowded conditions

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MENINGITIS

• Communicability
• - Meningitis caused by H. influenzae as long as
  organisms are present non-communicable within
  24-48 hours after treatment is started
• - Meningococcal meningitis (caused by N.
  meningitidis) until organism is no longer
  present in secretions from nose and mouth

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MENINGITIS

• HISTORY - In children lt12 months old
• - Usually preceded by URTI
• - High fever
• - Irritability
• - Child sleeps "all the time
• - Child is "not acting right
• - Child cries when moved or picked up

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MENINGITIS

• History- Infant lt 12 months
• - Child won't stop crying
• - "Soft spot bulging"?
• - Vomiting
• - Poor feeding
• - Seizures may develop
• - Rash (purple spots

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MENINGITIS

• History- Older children
• - Preceding URI
• - Fever
• - Photophobia
• - Headache that becomes increasingly severe
• - Headache made worse with movement, especially
  bending forward
• - Vomiting( without nausea)

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MENINGITIS

• History- Older children
• - Neck pain
• - Back pain
• - Changes in level of consciousness, progressing
  from irritability through confusion, drowsiness
  and stupor to coma
• - Seizures may develop
• - Rash (purple spots)

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MENINGITIS

• PHYSICAL EXAM
• - Temperature- elevated
• - Tachycardia or bradycardia with increased
  intracranial pressure
• - Blood pressure normal (low if septic shock has
  occurred)
• - Child in moderate-to-acute distress
• - Flushed

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MENINGITIS

• PHYSICAL EXAM ( continued)
• - Level of consciousness variable
• - Possible enlargement of the cervical nodes
• - Focal neurologic signs
• - photophobia
• - nuchal rigidity (in children gt12 months old)
• - positive Brudzinski's sign ( in children gt12
  months)
• - positive Kernig's sign (in children gt12
  months )

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MENINGITIS

• DIFFERENTIAL DIAGNOSIS
• - Bacteremia
• - Sepsis
• - Septic shock
• - Brain abscess

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MENINGITIS

• COMPLICATIONS
• - Seizures
• - Coma
• - Blindness
• - Deafness
• - Death
• - Palsies of cranial nerves III, VI, VII, VIII

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MENINGITIS

• Diagnostic Tests
• - Blood culture x3, drawn 15 minutes apart
• - Urine for routine and microscopy, culture and
  sensitivity
• - Throat swab for culture and sensitivity
• - CBC, lytes, creatinine,glucose, INR,platelets,
  ABGs
• - LP

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MENINGITIS

• Management
• - Admit- Bed rest
• - Nothing by mouth
• - Foley catheter (optional if the child is
  conscious)
• - Start IV therapy with normal saline, and adjust
  rate according to state of hydration

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MENINGITIS

• Pharmacotherapy
• Antipyretic
• - Acetaminophen (Tylenol) 1015 mg/kg q4h prn
• Antibiotics- Infants lt6 Weeks Old
• - Ampicillin (Ampicin), 75 mg/kg per dose, IV q6h
  (maximum 2.5 g/dose)
• AND
• Gentamicin (Garamycin), 2.5 mg/kg per dose q8h

101
MENINGITIS

• Antibiotics -Infants 6 Weeks to 3 Months Old
• Ampicillin (Ampicin), 75 mg/kg per dose, IV q6h
  (maximum 2.5 g/dose)
• AND
• Ceftriaxone (Rocephin), 80 mg/kg per dose, IV
  q12h (for the first 48 hours) (maximum 2 g/dose,
  4 g/day)

102
MENINGITIS

• Antibiotics -Children 3 Months to 18 Years Old
• ceftriaxone (Rocephin), 80 mg/kg per dose, IV
  q12h (for the first 48 hours) (maximum 2 g/dose,
  4 g/day)

103
MENINGITIS

• Monitor
• ABCs, vital signs
• Level of consciousness, intake
• Urine output
• Watch for focal neurological symptoms
• Monitor serum sodium

104
MENINGITIS

• Prevention and Control - Meningitis Caused by
  Hemophilus influenzae
• - A vaccine is now routinely given to infants as
  part of the usual childhood immunizations.
• - The type of vaccine and the immunization
  schedule vary by province
• - The vaccine is usually given at 2, 4, 6 and 18
  months of age, along with the DPTP vaccine.

105
MENINGITIS

• Meningococcal Meningitis -prevention
• - Vaccines for certain subtypes are available and
  are sometimes used in epidemics
• Chemoprophylaxis for household contacts
• Rifampin (Rifadin)
• Infants lt1 month old 5 mg/kg bid for 2 days
• Children 10 mg/kg bid for 2 days
• Adults 600 mg bid for 2 days

106
CASE STUDY

• Elaine, a 7 month old girl is brought in by
  mother for 6 month well child visit
• Child born at term , birth weight 7.5 pounds
• Pregnancy healthy, mom thought fetus was kicking
  less than in utero than she had with her previous
  baby
• Labor /delivery were uneventful

107
CASE STUDY

• Health to date good, feeding well
• Showed visual attention at 2-3 weeks, smiled
  socially at one month
• Pushed self up on arms while prone at 2 months,
• Rolled over at 4 months, no longer does this
• No longer reaches for mobile in crib or toys like
  rattle
• No attempts to sit up- cannot balance

108
CASE STUDY

• Physical Examination results
• - Infant lies quietly on table , watches examiner
  intently
• - Growth parameters including head circumference
  are normal
• - Vital signs are normal
• - See-saw breathing, frog leg posture noted
• - Cranial nerves are normal except eyes did not
  follow past the midline, and head turning
  strength was decreased

109
CASE STUDY

• Physical Examination results
• - When she is pulled to a sitting position by the
  hands , her head lags far behind and her arms are
  fully extended at the elbows
• - She could not raise her arms off table
• - When a rattle was placed in her hand , she
  manipulates the toys which she regards from the
  corner of her eye
• - Deep tendon reflexes were absent
• - Pain sensation intact

110
CASE STUDY

• Resolution
• Elaine was judged to have hypotonia with a
  neuromuscular cause in part because of her alert
  appearance and absent DTRs
• Neuropathic abnormalities on EMG and muscle
  biopsy confirmed a diagnosis of spinal muscular
  atrophy
• Family received genetic counseling and become
  involved in a support group
• Elaine died of respiratory failure in a chronic
  care hospital at age 17 months

111
HYPOTONIA IN ONFANTS

• Definition
• Lower-than-normal muscular resistance to passive
  motion across a joint
• Muscle strength is a key component of this
  resistance

112
ETIOLOGY-HYPOTONIA IN INFANTS

• Central causes
• - Neonatal asphyxia and /or intracranial
  hemorrhage
• - Chromosomal disorders - trisomy 21
• - CNS malformations- NTDs
• - Metabolic/endocrine causes - hypothyroidism,
  celiac disease, inborn errors of metabolism)
• - Benign congenital hypotonia ( a Dx of exclusion
  only)
• - Direct trauma - spinal cord transection

113
ETIOLOGY-HYPOTONIA IN INFANTS

• Anterior horn Cell
• - Spinal muscle atrophy( genetic)
• Peripheral nerve
• - Guillain-Barre syndrome, hereditary
  neuropathies, metabolic, toxins, trauma

114
ETIOLOGY-HYPOTONIA IN INFANTS

• Neuromuscular junction
• - Myasthenia gravis, infant botulism
• Muscle
• - Muscular dystrophies( Duchenne), congenital
  myopathies, myotonic dystrophy

115
NEUROLOGIC SIGNS of CENTRAL LESION

• Alertness - decreased, Cry -decreased
• Muscle power- normal--gt decreased
• Muscle bulk - normal---gt decreased
• Fasiculations- absent
• DTRs - increased
• Primitive newborn reflexes persist or reappear
• Plantar response- extensor
• Sensation - normal

116
NEUROLOGIC SIGNS of ANTERIOR HORN CELL LESION

• Alertness normal, Cry- normal/weak
• Muscle power- decreased
• Muscle bulk - proximal atrophy
• Fasiculations- present
• DTRs - decreased to absent
• Primitive newborn reflexes - absent
• Plantar response- flexor or nonreactive
• Sensation - normal

117
NEUROLOGIC SIGNS of PERIPHERAL LESION

• Alertness/Cry normal
• Muscle power- decreased
• Muscle bulk - distal atrophy
• Fasiculations- variable
• DTRs - decreased
• Primitive newborn reflexes - absent
• Plantar response- flexor or non-reactive
• Sensation - decreased

118
NEUROLOGIC SIGNS - LESION NEUROMUSCULAR JUNCTION

• Alertness normal, Cry weak
• Muscle power- fluctuating weakness
• Muscle bulk - normal
• Fasiculations- absent
• DTRs - normal to decreased
• Primitive newborn reflexes - absent
• Plantar response- flexor
• Sensation - normal

119
NEUROLOGIC SIGNS - MUSCLE LESION

• Alertness normal Cry normal/weak
• Muscle power- decreased
• Muscle bulk - decreased
• Fasiculations- absent
• DTRs - decreased
• Primitive newborn reflexes - absent
• Plantar response- flexor to nonreactive
• Sensation - normal

120
ASSESSMENT OF HYPOTONIA

• HISTORY
• Onset (acute or gradual), progression
• Past history of any acute illness (e.g.,
  meningitis)
• Family history of myopathy
• Social history infantparent interaction,
  siblings history (many babies are floppy
  because of lack of stimulation)

121
ASSESSMENT OF HYPOTONIA

• Associated Symptoms
• Respiratory
• Feeding difficulties
• Seizures
• Fasciculations
• Ptosis
• Delays in reaching developmental milestones

122
ASSESSMENT OF HYPOTONIA

• Inappropriate weight gain
• Maternal health problems (e.g., hypertension,
  diabetes mellitus)
• Physiologic insults during pregnancy or delivery
• Maternal use of neurotoxic drugs
• Neonatal problems- Apgars, sepsis, respiratory
• Family History

123
ASSESSMENT OF HYPOTONIA

• PHYSICAL EXAM
• General survey- dysmorphic features
• Vital signs
• General physical examination to rule out any
  underlying cause
• Complete - detailed CNS exam
• Assessment of developmental milestones for age
• Assessment of primitive reflexes

124
MANAGEMENT OF HYPOTONIC INFANT

• Diagnostic approach
• - CBC, electrolytes, BUN, creatinine, calcium.
  CPK, bilirubin, LFTs, urinalysis
• - Consider Head U/S, CT scan, MRI, LP if central
  cause suspected
• - Chromosomes studies if dysmorphic features
• -

125
MANAGEMENT OF HYPOTONIC INFANT

• Diagnostic approach
• - DNA analysis for dystrophies
• - EMG, nerve conduction studies
• - Tensilon test for myasthenia gravis
• - Muscle/nerve biopsies

126
HYDROCEPHALUS

• Increased CSF volume
• Communicating hydrocephalus
• - Results from unsatisfactory absorption of CSF
  by the arachnoid gratulations or overproduction
  of CSF by the choroid plexus
• Non-communicating hydrocephalus
• - Results from an obstruction to CSF flow ,
  causing enlargement of only those ventricles
  proximal to the obstruction

127
HYDROCEPHALUS

• Congenital- Aqueductal anomalies
• - Primary aqueductal stenosis, or secondary to
  intrauterine infections i.e. varicelal, mumps,
  TORCH
• - Dandy-Walker malformation
• - Chiaria malformation
• - Myelomeningocele

128
HYDROCEPHALUS

• Acquired
• - Post meningitis
• - Post hemorrhage- (SAH, IVH)
• - Masses - vascular malformations, neoplastic

129
HYDROCEPHALUS

• Clinical presentation -age related
• - Increased head circumference
• - Irritability, lethargy, poor feeding, vomiting
  -infant
• - Headache, lethargy, vomiting- older child
• - Bulging anterior fontanelle
• - Widened cranial sutures
• - Cracked pot sound on cranial percussion
• - Scalp vein dilatation

130
HYDROCEPHALUS

• Clinical presentation -
• - Sunset sign - eyes deviate downward
• - Episodic bradycardia, apnea
• - Loss of color and peripheral vision(older
  child)
• Cranial nerve palsies - e.g abnormal pupil
  size/reactivity, EOMs, nystagmus
• Spasticity limbs
• - Hyperreflexia, clonus

131
HYDROCEPHALUS

• Diagnostic Investigations
• Ultrasound of skull- through anterior fontanelle
• - Shows ventricular enlargement
• CT of head
• - Shows ventricular enlargement, peri-ventricualr
  lucency, narrow/absent sulci, /- 4 th
  ventricular enlargement

132
HYDROCEPHALUS

• RX
• Serial Spinal taps
• Surgery- remove obstruction if possible
• Shunts
• Acetazolamide- decreases blood flow to choroidal
  arteries , therefore decreasing CSF production

133
HYDROCEPHALUS

• Complications
• - Shunt blockages
• - Infection of shunt
• - Over shunting
• - Seizures
• - Blindness
• - Cranial nerve dysfunction
• - ICP
• - Cognitive impairment

134
CHILDHOOD MALIGNANCIES

• Cancer is the most common cause of disease
  related deaths in children 1-19 years
• Incidence has increased slowly, but mortality
  rates have declined significantly
• Common cancers in childhood include
• - Leukemias
• - Lymphomas
• - Brain tumors

135
CHILDHOOD MALIGNANCIES Clinical
Clues/Manifestations

• Hematologic
• - Pallor, anemia, petechiae, thrombocytopenia,
  neutropenia, fever, paryngitis
• - Signifies bone marrow infiltration
• - Example- Leukemia, neuroblastoma

136
CHILDHOOD MALIGNANCIES Clinical
Clues/Manifestations

• Systemic
• - Fever, weight loss, night sweats, painless
  lymphadenopathy ( Hodgkins, Non-Hodgkins
  lymphoma)
• - Bone pain, limp, arthralgias ( Osteosarcoma,
  Ewings sarcoma)
• - Cutaneous lesions ( neuroblastoma, leukemias)

137
CHILDHOOD MALIGNANCIES Clinical
Clues/Manifestations

• - Soft tissue mass ( Osteosarcoma, Ewings
  sarcoma)
• - Vomiting/ Diarrhea, abdominal mass (Lymphoma)
• - Thoracic mass- Lymphoma, neuroblastoma
• - Visual disturbances, headache, ataxia, cranial
  nerve palsies, papilledema ( Brain tumour)

138
CHILDHOOD MALIGNANCIES Clinical
Clues/Manifestations

• Ophthalmologic signs
• - Leukokoria ( retinoblastoma)
• - Peri-orbital ecchymosis ( neuroblastoma)
• - Ptosis, miosis (neuroblastoma)
• - Exopthalmous, proptosis ( orbital tumour)
  lymphoma,

139
BRAIN TUMOURS

• Primarily infratentorial involving cerebellum,
  midbrain, brainstem
• Glial( cerebellar astrocytomas most common)
• Presenting SS
• - Poor feeding, Vomiting , FTT( failure to
  thrive)
• - Arrest or regression of developmental
  milestones
• - Morning headache, increased head circumference
• ( hydrocephalus)
• - Diploplia, nystagmus,papilledema
• - Focal neuro deficits, seizures , ataxia

140
BRAIN TUMOURS

• Diagnosis
• Comprehensive history and complete CPX
• Careful CNS exam
• Rule out other causes - infection/trauma/metabolic
  
• CT head and/or MRI
• Referral to Pediatric neurosurgery

141
LYMPHOMA

• Third most common childhood cancer
• Hodgkins
• - Occurs in older child gt 15 years, similar to
  adult
• - Presents as a painless firm lymhadenopathy
• Non- Hodgkins
• - Occurs in younger child 7-11 years
• - Rapid growing , commonly metastesizes
• - Common disease sites abdomen, mediastinal mass,
  head/neck mass
• - SS vary according to site affected

142
NEUROBLASTOMA

• Most common cancer occurring in the first year of
  life
• Neural crest tumour arising from sympathetic
  tissues
• Adrenal medulla- 45
• Retroperitoneal- 25
• Posterior mediastinum - 20
• Pelvis - 4
• Neck - 4

143
NEUROBLASTOMA

• Presents a s a neck mass or chest mass, or
  abdominal mass( adrenal gland)
• Hypertension, headache,palptation,sweating (
  increased catecholamines), diarrhea, hypokalemia,
  FTT, are other possible SS
• Direct extension to spinal cord - compression
• Metastases common at presentation

144
NEUROBLASTOMA

• Referral to Pediatric oncology
• Diagnostic tests can include
• - LFTs, renal function, ferritin, urine( VMA,
  HVA)
• - CT scan chest, abdomen
• - Bone scan
• - Bone marrow examination
• - Tissue biopsy
• Rx surgery/radiation/chemotherapy /- bone
  marrow transplant