Neurological Disorders

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Neurological Disorders
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Seizure Disorders

• Generalized Seizures
• Tonic-clonic (grand mal)
• Absence (petit mal)
• Atonic
• Partial Seizures (starting from a focus)
• Simple
• Localized motor seizure
• Progression of mvmts
• Sensory
• Psychic
• Autonomic

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Seizure Disorders

• Partial Seizures (from a focus)
• Complex
• Partial Seizures evolving to generalized cortical
  seizure
• Complex
• Causes
• Scarring
• Drugs/infections causing high fever
• Withdrawl (i.e., NMDA)

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Disorders of Development

• Toxic Chemicals
• Fetal alcohol syndrome
• Lifelong physical and mental impairment caused by
  maternal alcohol use
• Recent research nicotinamide, commonly used in
  patients with autoimmune diseases, may protect
  against FAS

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Disorders of Development

• Inherited Metabolic Disorders
• Phenylketonuria (PKU)
• hereditary disorder
• caused by absence of enzyme that converts the
  amino acid phenylalanine to tyrosine
• causes brain damage unless a special diet is
  implemented soon after birth

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Disorders of Development

• Inherited Metabolic Disorders
• Pyridoxine (vitamin B6) dependency
• Usually presents in the first few days or weeks
• Pyridoxine is used to make gamma aminobutyric
  acid (GABA)
• Galactosemia
• Excessive galactose in the blood
• Caused by "missing" the enzyme (known as GALT) to
  convert galactose into glucose
• If untreated, 75 of infants will die (due to
  medical consequences include an enlarged liver,
  kidney failure, cataracts, brain damage)

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Disorders of Development

• Inherited Metabolic Disorders
• Tay-Sachs disease
• Lipid storage disorder (ganglioside GM2 build up
  in tissues and nerve cells in the brain)
• Caused by insufficient activity of
  beta-hexosaminidase A
• Down Syndrome
• Approximately one in 800 to 1,000 babies
• Caused by an extra chromosome
• Variety of birth defects (i.e., mental
  retardation, characteristic facial features and,
  often, heart defects, increased infections,
  problems with vision and hearing, and other
  health problems)

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Degenerative Disorders

• Transmissible Spongiform Encephalopathies
• Aka prion diseases (i.e., Creutzfeldt-Jakob
  disease, mad cow disease)
• Rare degenerative brain disorders caused by
  abnormal prions characterized by tiny holes that
  give the brain a "spongy" appearance
• Symptoms include personality changes, depression,
  lack of coordination, involuntary jerking
  movements called myoclonus, unusual sensations,
  insomnia, confusion, or memory problems

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Degenerative Disorders

• Parkinsons Disease
• Degeneration of dopamine-producing neurons in
  substantia nigra
• Symptoms include slow movement (bradykinesia),
  inability to move (akinesia), rigid limbs,
  shuffling gait, and stooped posture
• Lewy bodies round collections of proteins in the
  brain
• L-DOPA

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Neurological Disorders QA

• What are the early symptoms of Galactosemia?
• An infant with classic galactosemia usu appears
  normal at birth. Symptoms usu occur within the
  first few days or weeks of life after the baby
  drinks breast milk or a lactose-containing
  formula.
• Early symptoms may include jaundice, vomiting,
  poor weight gain, feeding difficulties,
  irritability, lethargy, convulsions
• What ethnic background is associated with PKU?
• Occurs in all ethnic groups but slightly more
  common in individuals of N European and Native
  American ancestry

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Degenerative Disorders

• Alzheimers Disease
• 10 gt 65 50 gt 85
• Irreversible, progressive
• Neurons deteriorate, resulting in the loss of
  cognitive functions, primarily memory, judgment
  and reasoning, movement coordination, and pattern
  recognition
• Predominantly affects the cerebral cortex and
  hippocampus

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Degenerative Disorders

• Alzheimers Disease
• Neuritic plaques
• the center of these plaques is beta amyloid,
  surrounded by fragments of deteriorating neurons
  (esp. neurons that produce acetylcholine)
• Neurofibrillary tangles
• twisted remnants of a protein tau, essential for
  maintaining proper cell structure and function
• Gene responsible for production of Apolipoprotein
  E (ApoE)

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Degenerative Disorders

• Guam Disease--amyotrophic lateral sclerosis

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Degenerative Disorders

• Huntingtons Disease
• One in 10,000 Americans
• Onset usually 30-45 10-20 yr prognosis
  post-onset
• Early symptoms may affect cognitive ability or
  mobility and include depression, mood swings,
  forgetfulness, clumsiness, involuntary twitching
  and lack of coordination
• Genetic disorder 50 likelihood in offspring
• Increased CAG repeat number in a gene coding for
  a protein with unknown function, called
  huntingtin

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Degenerative Disorders

• Multiple Sclerosis
• 1 case per 1000 leading cause of neurologic
  disability in early-to-middle adulthood, second
  only to trauma
• Environmental effect genetic vulnerability
• Inflammatory demyelinating condition of the
  central nervous system (CNS) that is generally
  considered to be autoimmune in nature
• Possibly due to exposure to a molecular sequence
  that mimics the molecular sequence found in the
  host tissue
• Sclerotic plaques

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Disorders Caused by Infectious Diseases

• Encephalitis
• Brain inflammation caused by a virus (i.e.,
  herpes viruses arboviruses transmitted by
  mosquitoes, ticks and other insects)
• Symptoms include drowsiness, confusion and
  disorientation, seizures, sudden fever, severe
  headache, nausea and vomiting, tremor or
  convulsions, stiff neck
• Meningitis
• Infection of the meninges, the membranes that
  surround the brain and spinal cord

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Disorders Caused by Infectious Diseases

• Syphilis
• Treponema pallidum (spirochete)
• Primary Syphilis (21 days)incubation most
  patients control infection and dont progress
  development of canchre (painless)
• Secondary syphilis (2-12 wks)spread of rash,
  sometimes fever, condylomata lata, meningitis
  hepatitis 

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Disorders Caused by Infectious Diseases

• Syphilis
• Tertiary Syphilis (1-30 yrs 30-40 untreated
  pts)
• Cardiovascular Syphilis
• Gummatous Syphilis
• Neurological syphilis

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Disorders Caused by Infectious Diseases

• Syphilis
• Neurological
• Meningovascular syphilis (5-12 yrs)stroke
  seizure due to infarction caused by endarteritis
• Parenchymatous syphilisdirect invasion of CNS by
  spirochetes, destruction of neurons
• General paresis (15-20 yrs) dementia with
  confabulation exhibit slurred speech, and
  Argyll-Robertson pupils may be seen
• Tabes dorsalis demyelinization of dorsal roots,
  dorsal root ganglia and posterior columns
  symptoms include lightening pains, wide gait, and
  loss of deep pain, temperature, position and
  vibration sense

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Disorders Caused by Infectious Diseases

• Herpes
• Herpes Simplex Type 1 (HSV-1)
• Herpes Simplex Type 2 (HSV-2)
• Varicella-zoster virus (aka chickenpox)
• Epstein Barr virus
• Cytomegalovirus
• Human herpesvirus 6
• Human herpesvirus 7
• Human herpesvirus 8

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Disorders Caused by Infectious Diseases

• Herpes
• Herpes Simplex Type 1 (HSV-1)
• Generally orofacial infection
• Herpes Simplex Type 2 (HSV-2)
• Generally genital infection
• Lesions may look like cold sores