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Pediatrics 4

  1. Pediatric Neurology

 

    1. Increased intracranial pressure (ICP)
      (      illustration 1  illustration 2  illustration 3  illustration 4  illustration 5  illustration 6  illustration 7  illustration 8  illustration 9)
      1. Etiology
        1. congenital or acquired (from trauma, lesion, infection)
        2. also see Pediatric Oncology (See section XI of this lesson)
      2. Pathophysiology: swelling caused by irritation or bleeding into brain tissue
      3. Findings
        1. infant: bulging fontanels, widened cranial sutures, high-pitched crying, irritable (illustration)
        2. child: headache, nausea, vomiting, lethargy, diplopia, seizures
      4. Management
        1. principle of maintaining cerebral perfusion pressure (CPP), which equals mean arterial blood pressure minus ICP
        2. medications: osmotic diuretics, antihypertensives, anti-seizure
        3. raise head of bed
        4. mechanical ventilation, oxygen
        5. in severe cases, ICP monitor
        6. minimize external stimuli
    2. Hydrocephalus
      1. Etiology: congenital, acquired, or idiopathic
      2. Pathophysiology: depends on type
        1. communicating: impaired absorption of CSF within the subarachoid space (illustration)
        2. noncommunicating: obstruction of the flow of CSF through the ventricular system
      3. Findings
        1. changes from baseline neurological status, and often same as increased ICP
        2. infant (signs of increased intracranial pressure)
          1. bulging fontanels, increased head circumference
          2. "setting sun" eyes, pupils slow to constrict to light
          3. eats poorly
          4. high-pitched cry
          5. variable pulse, changes in respirations
        3. older child (signs of increased intracranial pressure)
          1. headaches
          2. dizziness
          3. vomiting
          4. diplopia
          5. ataxia
      4. Management
        1. surgical placement of ventriculoperitoneal (VP) shunt in neonates, infants, and older children
        2. monitor for increased intracranial pressure
        3. measure head circumference
      5. Complications: shunt infection and malfunction
    3. Seizures
      1. Definition: brief malfunctions of the brain's electrical system that result from cortical neuronal discharge
      2. Etiology: cerebral, biochemical, post-traumatic, febrile, idiopathic, most common neurologic dysfunction in children
      3. Pathophysiology
        1. trigger electrical discharges affecting nearby normal cells
        2. spreads throughout brain reaching midbrain reticular regions
        3. possible generalized seizure with neurological symptoms
      4. Findings
        1. determined by site of origin
        2. may include altered levels of consciousness, involuntary movements and changes in perception
        3. seizure may be a finite event, with limited manifestations
        4. may have an aura
        5. status epilepticus is continuous seizure lasting more than 30 minutes or a series of seizures from which the child does not regain a premorbid level of consciousness
        6. febrile seizures: occur in association with a fever, usually greater than 38.8 degrees Celsius (101.8 degrees Fahrenheit)
        7. may be nonrecurrent (acute) such as febrile episode or recurrent (chronic) such as epilepsy
    4. Epilepsy
      1. Definition: chronic seizure disorder with recurrent & unprovoked seizures.
      2. Etiology: unknown. international classification: partial and generalized
        1. partial: limited to a particular local area of the brain
        2. generalized: involves both hemispheres of the brain
      1. Pathophysiology: see seizures
      2. Management see seizure precautions, seizure care, postictal care 
        1. supportive
          1. airway: suction, oxygen
          2. safety: loosen clothing, place on floor, clear area, remove restrictive clothing
        2. labs: toxicology screen, ABG's, electrolytes, anticonvulsant drug level, possible spinal tap findings (illustration)
        3. pharmacology - antiepileptic drugs
        4. goal: to control seizures and/or reduce frequency
    5. Neural tube defects (NTDs)
      1. Anencephaly
        1. most serious
        2. both cerebral hemispheres are absent
        3. condition is incompatible with life
      2. Spina bifida (SB)
        1. etiology: incomplete closure of vertebrae and neural tube; unknown cause
        2. pathophysiology
          1. spina bifida occulta: defect not visible externally
          2. spina bifida cystica: visible defect with an external sack-like protrusion
            • meningocele: encases meninges and spinal fluid but no neural elements; no neurological deficit
            • meningomyelocele: contains meninges, spinal fluid, and nerves; varying neurological deficit
    6. Cerebral palsy (CP)
      1. Etiology
        1. disorders characterized by early onset and impaired movement and posture; non-progressive
        2. cause: abnormality in extrapyramidal or pyramidal motor system (cortex, basal ganglia, cerebellum)
        3. can occur prenatally, perinatally, or postnatally
        4. may be accompanied by perceptual problems, language deficits and intellectual involvement
      2. Pathophysiology
        1. classifications: athetoid, spastic, ataxic, mixed
        2. associated defects: mental retardation and cognitive impairment, hearing or visual losses, attention deficit disorder (ADD)
        3. high metabolic rate, leading to increased calorie expenditure
      3. Findings
        1. primary disturbances: abnormal muscle tone and coordination
        2. spastic movement in one or more extremity
        3. athetoidmovements
        4. primitive reflexes persist
        5. disturbances in gait, abnormal posture
        6. impairments in speech and swallowing
      4. Diagnostics: physical exam, EEG, CT
      5. Management
        1. physiological: nutrition, musculoskeletal, respiratory, integumentary
        2. cognitive
    7. Down syndrome (trisomy 21)
      1. Etiology: extra group G chromosome, chromosome 21. Associated with late maternal age
      2. Pathophysiology: depends on which region of chromosome 21 was altered
        1. distinctive facial features
        2. heart defects
        3. mental retardation (varies from severe retardation to low average intelligence)
        4. dermatologic changes
        5. incomplete embryogenesis
      3. Manifestations:
        1. facial features
          1. brachycephaly
          2. flat occiput
          3. protruding tongue
          4. high-arch palate
          5. almond shaped eyes
          6. low slung ears
        2. body features
          1. short, broad hands with simian crease (illustration)
          2. short, broad neck
          3. dry skin
          4. large space between great toe and second toe
      4. Management
        1. supportive: specific to body system affected
        2. promotion of developmental progress
        3. cognitive
    8. Werdnig-Hoffmann syndrome ("floppy infant" syndrome)
      1. Etiology: inherited autosomal-recessive disorder
      2. Pathophysiology:
        1. skeletal muscles atrophy
        2. arises form pathology of anterior horn cells of spinal cord brain stem and motor nuclei of brain stem
      3. Clinical manifestation: variable onset
        1. earlier onset, increased severity.
        2. weakness, legs externally rotated, abducted and flexed at hips
        3. limited range of arm movement
        4. weak cry
      4. Diagnostics: electromyography, physical exam
      5. Management: supportive, with emphasis on respiratory and musculoskeletal systems
      6. Prognosis: early death
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