Neurological Disorders Slide 2 Seizure Disorders

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Neurological Disorders Slide 2 Seizure Disorders

  1. 1. Neurological Disorders
  2. 2. Seizure Disorders <ul><li>Generalized Seizures </li></ul><ul><ul><li>Tonic-clonic (grand mal) </li></ul></ul><ul><ul><li>Absence (petit mal) </li></ul></ul><ul><ul><li>Atonic </li></ul></ul><ul><li>Partial Seizures (starting from a focus) </li></ul><ul><ul><li>Simple </li></ul></ul><ul><ul><ul><li>Localized motor seizure </li></ul></ul></ul><ul><ul><ul><li>Progression of mvmts </li></ul></ul></ul><ul><ul><ul><li>Sensory </li></ul></ul></ul><ul><ul><ul><li>Psychic </li></ul></ul></ul><ul><ul><ul><li>Autonomic </li></ul></ul></ul>
  3. 3. Seizure Disorders <ul><li>Partial Seizures (from a focus) </li></ul><ul><ul><li>Complex </li></ul></ul><ul><li>Partial Seizures evolving to generalized cortical seizure </li></ul><ul><ul><li>Complex </li></ul></ul><ul><li>Causes: </li></ul><ul><ul><li>Scarring </li></ul></ul><ul><ul><li>Drugs/infections causing high fever </li></ul></ul><ul><ul><li>Withdrawl (i.e., NMDA) </li></ul></ul>
  4. 4. Disorders of Development <ul><li>Toxic Chemicals </li></ul><ul><ul><li>Fetal alcohol syndrome </li></ul></ul><ul><ul><ul><li>Lifelong physical and mental impairment caused by maternal alcohol use </li></ul></ul></ul><ul><ul><ul><li>Recent research: nicotinamide, commonly used in patients with autoimmune diseases, may protect against FAS </li></ul></ul></ul>
  5. 5. Disorders of Development <ul><li>Inherited Metabolic Disorders </li></ul><ul><ul><li>Phenylketonuria (PKU) </li></ul></ul><ul><ul><ul><li>hereditary disorder </li></ul></ul></ul><ul><ul><ul><li>caused by absence of enzyme that converts the amino acid phenylalanine to tyrosine </li></ul></ul></ul><ul><ul><ul><li>causes brain damage unless a special diet is implemented soon after birth </li></ul></ul></ul>
  6. 6. Disorders of Development <ul><li>Inherited Metabolic Disorders </li></ul><ul><ul><li>Pyridoxine (vitamin B6) dependency </li></ul></ul><ul><ul><ul><li>Usually presents in the first few days or weeks </li></ul></ul></ul><ul><ul><ul><li>Pyridoxine is used to make gamma aminobutyric acid (GABA) </li></ul></ul></ul><ul><ul><li>Galactosemia </li></ul></ul><ul><ul><ul><li>Excessive galactose in the blood </li></ul></ul></ul><ul><ul><ul><li>Caused by &quot;missing&quot; the enzyme (known as GALT) to convert galactose into glucose </li></ul></ul></ul><ul><ul><ul><li>If untreated, 75% of infants will die (due to medical consequences include an enlarged liver, kidney failure, cataracts, brain damage) </li></ul></ul></ul>
  7. 7. Disorders of Development <ul><li>Inherited Metabolic Disorders </li></ul><ul><ul><li>Tay-Sachs disease </li></ul></ul><ul><ul><li>Lipid storage disorder ( ganglioside G M2 build up in tissues and nerve cells in the brain) </li></ul></ul><ul><ul><li>Caused by insufficient activity of beta-hexosaminidase A </li></ul></ul><ul><li>Down Syndrome </li></ul><ul><ul><li>Approximately one in 800 to 1,000 babies </li></ul></ul><ul><ul><li>Caused by an extra chromosome </li></ul></ul><ul><ul><li>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) </li></ul></ul>
  8. 8. Degenerative Disorders <ul><li>Transmissible Spongiform Encephalopathies </li></ul><ul><ul><li>Aka “prion diseases” (i.e., Creutzfeldt-Jakob disease, “mad cow disease”) </li></ul></ul><ul><ul><li>Rare degenerative brain disorders caused by abnormal prions characterized by tiny holes that give the brain a &quot;spongy&quot; appearance </li></ul></ul><ul><ul><li>Symptoms include personality changes, depression, lack of coordination, involuntary jerking movements called myoclonus, unusual sensations, insomnia, confusion, or memory problems </li></ul></ul>
  9. 9. Degenerative Disorders <ul><li>Parkinson’s Disease </li></ul><ul><ul><li>Degeneration of dopamine-producing neurons in substantia nigra </li></ul></ul><ul><ul><li>Symptoms include slow movement (bradykinesia), inability to move (akinesia), rigid limbs, shuffling gait, and stooped posture </li></ul></ul><ul><ul><li>Lewy bodies: round collections of proteins in the brain </li></ul></ul><ul><ul><li>L-DOPA </li></ul></ul>
  10. 10. Neurological Disorders Q&A <ul><li>What are the early symptoms of Galactosemia? </li></ul><ul><ul><li>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. </li></ul></ul><ul><ul><ul><li>Early symptoms may include: jaundice, vomiting, poor weight gain, feeding difficulties, irritability, lethargy, convulsions </li></ul></ul></ul><ul><li>What ethnic background is associated with PKU? </li></ul><ul><ul><li>Occurs in all ethnic groups but slightly more common in individuals of N European and Native American ancestry </li></ul></ul>
  11. 11. Degenerative Disorders <ul><li>Alzheimer’s Disease </li></ul><ul><ul><li>10% > 65; 50% > 85 </li></ul></ul><ul><ul><li>Irreversible, progressive </li></ul></ul><ul><ul><li>Neurons deteriorate, resulting in the loss of cognitive functions, primarily memory, judgment and reasoning, movement coordination, and pattern recognition </li></ul></ul><ul><ul><li>Predominantly affects the cerebral cortex and hippocampus </li></ul></ul>
  12. 12. Degenerative Disorders <ul><li>Alzheimer’s Disease </li></ul><ul><ul><li>Neuritic plaques: </li></ul></ul><ul><ul><ul><li>the center of these plaques is beta amyloid, surrounded by fragments of deteriorating neurons (esp. neurons that produce acetylcholine) </li></ul></ul></ul><ul><ul><li>Neurofibrillary tangles: </li></ul></ul><ul><ul><ul><li>twisted remnants of a protein tau, essential for maintaining proper cell structure and function </li></ul></ul></ul><ul><ul><li>Gene responsible for production of Apolipoprotein E (ApoE) </li></ul></ul>
  13. 13. Degenerative Disorders <ul><li>“ Guam Disease”--amyotrophic lateral sclerosis </li></ul>
  14. 14. Degenerative Disorders <ul><li>Huntington’s Disease </li></ul><ul><ul><li>One in 10,000 Americans </li></ul></ul><ul><ul><li>Onset usually 30-45; 10-20 yr prognosis post-onset </li></ul></ul><ul><ul><li>Early symptoms may affect cognitive ability or mobility and include depression, mood swings, forgetfulness, clumsiness, involuntary twitching and lack of coordination </li></ul></ul><ul><ul><li>Genetic disorder; 50% likelihood in offspring </li></ul></ul><ul><ul><li>Increased CAG repeat number in a gene coding for a protein with unknown function, called huntingtin </li></ul></ul>
  15. 15. Degenerative Disorders <ul><li>Multiple Sclerosis </li></ul><ul><ul><li>1 case per 1000; leading cause of neurologic disability in early-to-middle adulthood, second only to trauma </li></ul></ul><ul><ul><li>Environmental effect; genetic vulnerability </li></ul></ul><ul><ul><li>Inflammatory demyelinating condition of the central nervous system (CNS) that is generally considered to be autoimmune in nature </li></ul></ul><ul><ul><li>Possibly due to exposure to a molecular sequence that mimics the molecular sequence found in the host tissue </li></ul></ul><ul><ul><li>Sclerotic pla ques </li></ul></ul>
  16. 16. Disorders Caused by Infectious Diseases <ul><li>Encephalitis </li></ul><ul><ul><li>Brain inflammation caused by a virus (i.e., herpes viruses; arboviruses transmitted by mosquitoes, ticks and other insects) </li></ul></ul><ul><ul><li>Symptoms include drowsiness, confusion and disorientation, seizures, sudden fever, severe headache, nausea and vomiting, tremor or convulsions, stiff neck </li></ul></ul><ul><li>Meningitis </li></ul><ul><ul><li>Infection of the meninges, the membranes that surround the brain and spinal cord </li></ul></ul>
  17. 17. Disorders Caused by Infectious Diseases <ul><li>Syphilis </li></ul><ul><ul><li>Treponema pallidum (spirochete) </li></ul></ul><ul><ul><ul><li>Primary Syphilis (~21 days) — incubation; most patients control infection and don’t progress; development of canchre (painless) </li></ul></ul></ul><ul><ul><ul><li>Secondary syphilis (2-12 wks) — spread of rash, sometimes fever, condylomata lata, meningitis & hepatitis  </li></ul></ul></ul>
  18. 18. Disorders Caused by Infectious Diseases <ul><li>Syphilis </li></ul><ul><ul><ul><li>Tertiary Syphilis (1-30 yrs; 30-40% untreated pts) </li></ul></ul></ul><ul><ul><ul><ul><li>Cardiovascular Syphilis </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Gummatous Syphilis </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Neurological syphilis </li></ul></ul></ul></ul>
  19. 19. Disorders Caused by Infectious Diseases <ul><li>Syphilis </li></ul><ul><ul><li>Neurological </li></ul></ul><ul><ul><ul><li>Meningovascular syphilis (5-12 yrs) —stroke & seizure; due to infarction caused by endarteritis </li></ul></ul></ul><ul><ul><ul><li>Parenchymatous syphilis —direct invasion of CNS by spirochetes, & destruction of neurons </li></ul></ul></ul><ul><ul><ul><ul><li>General paresis (15-20 yrs): dementia with confabulation; exhibit slurred speech, and Argyll-Robertson pupils may be seen </li></ul></ul></ul></ul><ul><ul><ul><ul><li>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 </li></ul></ul></ul></ul>
  20. 21. Disorders Caused by Infectious Diseases <ul><li>Herpes </li></ul><ul><ul><li>Herpes Simplex Type 1 ( HSV-1 ) </li></ul></ul><ul><ul><li>Herpes Simplex Type 2 ( HSV-2 ) </li></ul></ul><ul><ul><li>Varicella-zoster virus (aka chickenpox) </li></ul></ul><ul><ul><li>Epstein Barr virus </li></ul></ul><ul><ul><li>Cytomegalovirus </li></ul></ul><ul><ul><li>Human herpesvirus 6 </li></ul></ul><ul><ul><li>Human herpesvirus 7 </li></ul></ul><ul><ul><li>Human herpesvirus 8 </li></ul></ul>
  21. 22. Disorders Caused by Infectious Diseases <ul><li>Herpes </li></ul><ul><ul><li>Herpes Simplex Type 1 ( HSV-1 ) </li></ul></ul><ul><ul><ul><li>Generally orofacial infection </li></ul></ul></ul><ul><ul><li>Herpes Simplex Type 2 ( HSV-2 ) </li></ul></ul><ul><ul><ul><li>Generally genital infection </li></ul></ul></ul><ul><ul><ul><ul><li>Lesions may look like cold sores </li></ul></ul></ul></ul>

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