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CHAPTER 45
CHRONIC DISORDERS OF
NEUROLOGIC FUNCTION
SEIZURE DISORDER
• Transient neurologic event of paroxysmal
abnormal or excessive cortical electrical
discharges that are manifested by
disturbances of skeletal motor function,
sensation, autonomic visceral function,
behavior, or consciousness
• Due to an alteration in membrane potential
that makes certain neurons abnormally
hyperactive and hypersensitive to changes in
their environment
BRAIN AND CEREBELLAR
DISORDERS
BRAIN AND CEREBELLAR
DISORDERS (CONT.)
• Generalized seizures involve the entire brain
from the onset of the seizure
• Partial seizures are those in which abnormal
electrical activity is restricted to one brain
hemisphere
• Status epilepticus is a continuing series of
seizures without a period of recovery
between seizure episodes and can be life-
threatening
BRAIN AND CEREBELLAR
DISORDERS (CONT.)
Diagnosis and Treatment of Seizures
• Electroencephalograms assess the electrical
patterns of brain regions
• Laboratory studies identify
metabolic/nutritional deficits, infections, and
exposure to toxins
• Antiseizure medications prevent or reduce
seizure episodes; seizure precautions prevent
injury
DEMENTIA
• A syndrome associated with many
pathologic processes and characterized by
progressive deterioration and continuing
decline of memory and other cognitive
changes
• Important to first rule out manageable causes
of dementia; often cause unknown
DEMENTIA (CONT.)
• The dementia of Alzheimer disease is
characterized by degeneration of neurons in
temporal and frontal lobes, brain atrophy,
amyloid plaques, and neurofibrillary tangles
• Cause remains unknown, although genetic
and environmental triggers are suggested
• Synthesis of brain acetylcholine is deficient
and treatment is aimed at increasing
acetylcholine levels by reducing
acetylcholine reuptake
DEMENTIA (CONT.)
PARKINSON DISEASE
• May be idiopathic or a consequence of the
use of certain drugs
• Dopamine deficiency in the basal ganglia is
associated with symptoms of motor
impairment
• Difficulty initiating and controlling movements
results in akinesia, tremor, and rigidity
• Tremor occurs at rest and hand tremors
exhibit pill-rolling movements
• Attempts to passively move the extremities
are met with cogwheel rigidity
PARKINSON DISEASE (CONT.)
PARKINSON DISEASE (CONT.)
• General lack of movement, loss of facial
expression, drooling, propulsive gait, and
absent arm swing
• Treatment is aimed at restoring brain
dopamine levels or activity by administration
of dopamine precursors, dopamine agonists,
monoamine oxidase inhibitors, and
anticholinergics
• Antidepressant therapy may be needed and
surgical procedures may be helpful for motor
symptoms
PARKINSON DISEASE (CONT.)
CEREBRAL PALSY
• Diverse group of crippling syndromes that
appear during childhood and involve
permanent, nonprogressive damage to
motor control areas of the brain
• Classified on the basis of neurologic signs and
symptoms, with the major types involving
spasticity, ataxia, dyskinesia, or a mix of one
or more of the three
CEREBRAL PALSY (CONT.)
• Etiology may include prenatal infections, or
diseases of the mother; mechanical trauma
to the head before, during, or after birth;
exposure to nerve-damaging poisons or
reduced oxygen supply to the brain
• Treatment varies according to the nature and
extent of brain damage
• Muscle relaxants, anticonvulsant drugs,
orthopedic surgery, casts, braces, and
traction are among the therapies used
HYDROCEPHALUS
• Characterized by abnormal accumulation of
fluid in the cerebroventricular system
• Normal pressure hydrocephalus is due to an
increased volume of CSF
• Obstructive hydrocephalus is due to an
obstruction to the flow of CSF
• Communicating hydrocephalus occurs due
to abnormal absorption of CSF
HYDROCEPHALUS (CONT.)
HYDROCEPHALUS (CONT.)
• Medical treatment is limited
• Obstructions may be corrected surgically
• The most effective treatment for
management of hydrocephalus is surgical
correction employing a shunt
HYDROCEPHALUS (CONT.)
CEREBELLAR DISORDERS
• Cerebellum is responsible for coordinated
control of muscle action, excitation and
inhibition of postural reflexes, and
maintenance of balance
• Etiology of cerebellar disorders includes
abscess, hemorrhage, tumors, trauma, viral
infection, or chronic alcoholism
• Clinical manifestations include ataxia,
hypotonia, intention tremors, and
disturbances in gait and balance
MULTIPLE SCLEROSIS
• MS is a demyelinating disease of the CNS that
primarily affects young adults
• Risk of contracting MS is greater for persons
living above the 37th parallel
• Cause unknown, but immunologic
abnormalities and environmental factors are
suspected
• Demyelination can occur throughout the CNS
but often affects the optic and oculomotor
nerves and spinal nerve tracts
SPINAL CORD AND
PERIPHERAL NERVE
DISORDERS
MULTIPLE SCLEROSIS
• Symptoms are slowly progressive; the disease
is marked by exacerbations and remissions
• Symptoms may include double vision,
weakness, poor coordination, and sensory
deficits; bowel and bladder control may be
lost; memory impairment is common
• Management is symptomatic; short-term
steroid therapy may be helpful during acute
exacerbations, and immune-modifying drugs
may slow progression of symptoms
MULTIPLE SCLEROSIS (CONT.)
SPINA BIFIDA
• Developmental anomaly characterized by
defective closure of the bony encasement of
the spinal cord (neural tube) through which
the spinal cord and meninges may or may
not protrude
• If anomaly not visible, condition is called
spina bifida occulta
• If there is an external protrusion of the saclike
structure, the condition is called spina bifida
cystica, and further classified according to
the extent of neural involvement
• Treatment is based on the severity of the
defect and neurologic dysfunction
AMYOTROPHIC LATERAL
SCLEROSIS
• ALS is a progressive disease affecting both
the upper and lower motor neurons
• Cause remains unknown
• Weakness and wasting of the upper
extremities usually occur, followed by
impaired speech, swallowing, and respiration
• Typically occurs between the ages of 40-60
years and affects men more than women
AMYOTROPHIC LATERAL
SCLEROSIS (CONT.)
• Clinical manifestations include weakness,
atrophy, cramps, stiffness, and irregular
twitching of muscle fibers
• Diagnosis is based on clinical signs and
symptoms, EMG, nerve conduction studies,
MRI, and serum laboratory testing
• The glutamate inhibitor, riluzole, may be
helpful in managing ALS
SPINAL CORD INJURY
• SCI is usually traumatic, a result of motor
vehicle accidents, falls, penetrating wounds,
or sports injuries
• The cord may be compressed, transected, or
contused
• Further injury may result from hemorrhage,
swelling, and ischemia
SPINAL CORD INJURY (CONT.)
SPINAL CORD INJURY (CONT.)
• Spinal shock occurs immediately following SCI
and is characterized by temporary loss of
reflexes below the level of injury
• Muscles are flaccid; skeletal and autonomic reflexes
are lost
• Neurogenic shock may occur after SCI due to
peripheral vasodilation
• Hypotension and circulatory collapse can occur;
high spinal cord injuries can affect respiratory
muscles, leading to ventilatory failure
SPINAL CORD INJURY (CONT.)
SPINAL CORD INJURY (CONT.)
• Autonomic dysreflexia is an acute reflexive
response to sympathetic activation below the
level of injury
• Visceral stimulation (full bladder or bowel) and
activation of pain receptors below the injury are
common stimuli
• Manifestations include hypertension, bradycardia,
flushing above the level of injury, and clammy skin
below the level of injury
• Prompt removal of the offending stimulus;
aggressive blood pressure management may be
needed
SPINAL CORD INJURY (CONT.)
• Treatment includes appropriate stabilization
of spinal vertebrae
• May be accomplished surgically with internal
fixation or with external fixation and bracing
• High-dose methylprednisolone may be used
to decrease secondary injury
• Intensive rehabilitation is required to maximize
function
GUILLAIN-BARRÉ SYNDROME
• Characterized by muscle weakness that
begins in the lower extremities and spreads to
the proximal spinal neurons
• Cause unknown; postinfectious immunologic
mechanism is suspected
• Progressive ascending weakness or paralysis,
may affect respiratory muscles
• Treatment is supportive; spontaneous
recovery usually occurs
BELL PALSY
• Bell palsy, or neuropathy of the facial nerve;
paralysis of the muscles on one side of the
face
• Symptoms include unilateral facial weakness,
facial droop and diminished eye blink,
hyperacusis, and decreased lacrimation
• Often self-limiting condition with unknown
cause
• Treatment is supportive, and spontaneous
recovery usually occurs
BELL PALSY (CONT.)

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Pathophysiology Chapter 45

  • 1. CHAPTER 45 CHRONIC DISORDERS OF NEUROLOGIC FUNCTION
  • 2. SEIZURE DISORDER • Transient neurologic event of paroxysmal abnormal or excessive cortical electrical discharges that are manifested by disturbances of skeletal motor function, sensation, autonomic visceral function, behavior, or consciousness • Due to an alteration in membrane potential that makes certain neurons abnormally hyperactive and hypersensitive to changes in their environment
  • 4. BRAIN AND CEREBELLAR DISORDERS (CONT.) • Generalized seizures involve the entire brain from the onset of the seizure • Partial seizures are those in which abnormal electrical activity is restricted to one brain hemisphere • Status epilepticus is a continuing series of seizures without a period of recovery between seizure episodes and can be life- threatening
  • 5. BRAIN AND CEREBELLAR DISORDERS (CONT.) Diagnosis and Treatment of Seizures • Electroencephalograms assess the electrical patterns of brain regions • Laboratory studies identify metabolic/nutritional deficits, infections, and exposure to toxins • Antiseizure medications prevent or reduce seizure episodes; seizure precautions prevent injury
  • 6. DEMENTIA • A syndrome associated with many pathologic processes and characterized by progressive deterioration and continuing decline of memory and other cognitive changes • Important to first rule out manageable causes of dementia; often cause unknown
  • 7. DEMENTIA (CONT.) • The dementia of Alzheimer disease is characterized by degeneration of neurons in temporal and frontal lobes, brain atrophy, amyloid plaques, and neurofibrillary tangles • Cause remains unknown, although genetic and environmental triggers are suggested • Synthesis of brain acetylcholine is deficient and treatment is aimed at increasing acetylcholine levels by reducing acetylcholine reuptake
  • 9. PARKINSON DISEASE • May be idiopathic or a consequence of the use of certain drugs • Dopamine deficiency in the basal ganglia is associated with symptoms of motor impairment • Difficulty initiating and controlling movements results in akinesia, tremor, and rigidity • Tremor occurs at rest and hand tremors exhibit pill-rolling movements • Attempts to passively move the extremities are met with cogwheel rigidity
  • 11. PARKINSON DISEASE (CONT.) • General lack of movement, loss of facial expression, drooling, propulsive gait, and absent arm swing • Treatment is aimed at restoring brain dopamine levels or activity by administration of dopamine precursors, dopamine agonists, monoamine oxidase inhibitors, and anticholinergics • Antidepressant therapy may be needed and surgical procedures may be helpful for motor symptoms
  • 13. CEREBRAL PALSY • Diverse group of crippling syndromes that appear during childhood and involve permanent, nonprogressive damage to motor control areas of the brain • Classified on the basis of neurologic signs and symptoms, with the major types involving spasticity, ataxia, dyskinesia, or a mix of one or more of the three
  • 14. CEREBRAL PALSY (CONT.) • Etiology may include prenatal infections, or diseases of the mother; mechanical trauma to the head before, during, or after birth; exposure to nerve-damaging poisons or reduced oxygen supply to the brain • Treatment varies according to the nature and extent of brain damage • Muscle relaxants, anticonvulsant drugs, orthopedic surgery, casts, braces, and traction are among the therapies used
  • 15. HYDROCEPHALUS • Characterized by abnormal accumulation of fluid in the cerebroventricular system • Normal pressure hydrocephalus is due to an increased volume of CSF • Obstructive hydrocephalus is due to an obstruction to the flow of CSF • Communicating hydrocephalus occurs due to abnormal absorption of CSF
  • 17. HYDROCEPHALUS (CONT.) • Medical treatment is limited • Obstructions may be corrected surgically • The most effective treatment for management of hydrocephalus is surgical correction employing a shunt
  • 19. CEREBELLAR DISORDERS • Cerebellum is responsible for coordinated control of muscle action, excitation and inhibition of postural reflexes, and maintenance of balance • Etiology of cerebellar disorders includes abscess, hemorrhage, tumors, trauma, viral infection, or chronic alcoholism • Clinical manifestations include ataxia, hypotonia, intention tremors, and disturbances in gait and balance
  • 20. MULTIPLE SCLEROSIS • MS is a demyelinating disease of the CNS that primarily affects young adults • Risk of contracting MS is greater for persons living above the 37th parallel • Cause unknown, but immunologic abnormalities and environmental factors are suspected • Demyelination can occur throughout the CNS but often affects the optic and oculomotor nerves and spinal nerve tracts
  • 21. SPINAL CORD AND PERIPHERAL NERVE DISORDERS
  • 22. MULTIPLE SCLEROSIS • Symptoms are slowly progressive; the disease is marked by exacerbations and remissions • Symptoms may include double vision, weakness, poor coordination, and sensory deficits; bowel and bladder control may be lost; memory impairment is common • Management is symptomatic; short-term steroid therapy may be helpful during acute exacerbations, and immune-modifying drugs may slow progression of symptoms
  • 24. SPINA BIFIDA • Developmental anomaly characterized by defective closure of the bony encasement of the spinal cord (neural tube) through which the spinal cord and meninges may or may not protrude • If anomaly not visible, condition is called spina bifida occulta • If there is an external protrusion of the saclike structure, the condition is called spina bifida cystica, and further classified according to the extent of neural involvement • Treatment is based on the severity of the defect and neurologic dysfunction
  • 25. AMYOTROPHIC LATERAL SCLEROSIS • ALS is a progressive disease affecting both the upper and lower motor neurons • Cause remains unknown • Weakness and wasting of the upper extremities usually occur, followed by impaired speech, swallowing, and respiration • Typically occurs between the ages of 40-60 years and affects men more than women
  • 26. AMYOTROPHIC LATERAL SCLEROSIS (CONT.) • Clinical manifestations include weakness, atrophy, cramps, stiffness, and irregular twitching of muscle fibers • Diagnosis is based on clinical signs and symptoms, EMG, nerve conduction studies, MRI, and serum laboratory testing • The glutamate inhibitor, riluzole, may be helpful in managing ALS
  • 27. SPINAL CORD INJURY • SCI is usually traumatic, a result of motor vehicle accidents, falls, penetrating wounds, or sports injuries • The cord may be compressed, transected, or contused • Further injury may result from hemorrhage, swelling, and ischemia
  • 29. SPINAL CORD INJURY (CONT.) • Spinal shock occurs immediately following SCI and is characterized by temporary loss of reflexes below the level of injury • Muscles are flaccid; skeletal and autonomic reflexes are lost • Neurogenic shock may occur after SCI due to peripheral vasodilation • Hypotension and circulatory collapse can occur; high spinal cord injuries can affect respiratory muscles, leading to ventilatory failure
  • 31. SPINAL CORD INJURY (CONT.) • Autonomic dysreflexia is an acute reflexive response to sympathetic activation below the level of injury • Visceral stimulation (full bladder or bowel) and activation of pain receptors below the injury are common stimuli • Manifestations include hypertension, bradycardia, flushing above the level of injury, and clammy skin below the level of injury • Prompt removal of the offending stimulus; aggressive blood pressure management may be needed
  • 32. SPINAL CORD INJURY (CONT.) • Treatment includes appropriate stabilization of spinal vertebrae • May be accomplished surgically with internal fixation or with external fixation and bracing • High-dose methylprednisolone may be used to decrease secondary injury • Intensive rehabilitation is required to maximize function
  • 33. GUILLAIN-BARRÉ SYNDROME • Characterized by muscle weakness that begins in the lower extremities and spreads to the proximal spinal neurons • Cause unknown; postinfectious immunologic mechanism is suspected • Progressive ascending weakness or paralysis, may affect respiratory muscles • Treatment is supportive; spontaneous recovery usually occurs
  • 34. BELL PALSY • Bell palsy, or neuropathy of the facial nerve; paralysis of the muscles on one side of the face • Symptoms include unilateral facial weakness, facial droop and diminished eye blink, hyperacusis, and decreased lacrimation • Often self-limiting condition with unknown cause • Treatment is supportive, and spontaneous recovery usually occurs