Monday, June 14, 2010

pathology IV: CNS

congenital brain anomalies are among the most common birth defects, affecting 10 in 1000 live births. anencephaly is one example, the lack of growth of a brain due to a neural tube defect. its incidence has gone down in recent years because of the knowledge that folic acid supplementation pre-conception can significantly reduce the risk of developing anencephaly. microcephaly refers to abnormally small brain development, and can be due to fetal alcohol syndrome, trisomy 13, or HIV/rubella. polymicrogyri is a condition of excess gyri due to decreased white matter.

hydrocephalus is a condition of increased CSF, generally due to blocked drainage. this might be due to stenosis of the cerebral aqueduct or the foramen Monroe. it is associated with spina bifida and can be identified due to increased cerebral ventricals. normal pressure hydrocephalus is a variant that has intermittently elevated CSF pressure.

spina bifida is another congenital anomaly that results in failure of closure of the dorsal vertebral column, or its overlying skin. it is associated with elevated alpha fetal protein and might be linked to abdominal radiation and congenital rubella. sequelae might include paralysis and incontinence. there are several variants; spina bifida occulta refers to a missing vertebal segments without herniation, while a meningocele is an outpouching of the meninges, and a myelomeningocele is herniation of both the meninges and spinal cord.

cerebral palsy is a movement disorder due to damage to the motor centers of the brain, and most commonly develops in premature infants due to maternal infection during pregnancy. the most common type is spastic CP, which results in an "equinus deformity". patients with this variant are most likely to be able to walk, whereas patients with spastic quadriplegia are least likely to be able to walk. spastic quadriplegia affects all limbs equally and might be accompanied by hemiparetic tremors, which are uncontrollable shaking that affects one side of the body. morphological features of the brain affected by cerebral palsy might include paraventricular leukomalacia (necrosis and softening of paraventricular white matter) and gliosis (neuronal death and astrocyte proliferation).

meningitis is an infection of the meningeal layers which affects 17,000 people per year in the US. viral infections are the most common cause and are generally self limiting. on the other hand, bacterial meningitis is a medical emergency and is most commonly caused by strep pneumonia, e.coli, neisseria meningococci, h. influenza, and listeria monocytogenes. the main signs and symptoms include headache, fever, stiff neck, photophobia, and petechiae, among other things.

CSF analysis can serve to differentiate between the forms of meningitis. in bacterial meningitis, "opening pressure" will be increased, glucose will be decreased, and leukocytosis might be present. viral meningitis might have normal opening pressure, a lower degree of leukocytosis (100 WBC/mm^3 as opposed to 1000),

h. influenza is a meningitis that is caused by the haemophilus influenza bacteria type B, not to be confused with the virus that causes influenza. it is often caused by URI's which then travel through the bloodstream and infect the brain. the top demographic is 6-9 year olds who have recurrent URI's, are in day care, and Native Americans and Inuits have a particularly high incidence. potential complications are hearing loss, seizures, learning / speech / behavioral abnormalities, hydrocephalus.

cryptococcal meningitis is caused by cryptococcus neoformans, which is a yeast that is found in soil worldwide. those who have compromised immune systems are at greatest risk for contracting this disease. microscopy might reveal circular yeast bodies and CSF might grow cryptococcus cultures. fundoscopy might also reveal granulomas and papilledema.

viral meningitis generally follows a systemic viral infection, most commonly by arboviruses. other viruses also play roles, such as HSV-1. VM has a predilection for the temporal lobes, causing hemorrhage.

carcinomatous meningitis is a type of meningitis that is caused by tumors in and around the nervous system, commonly spread from lung, breast cancer, or melanoma. symptoms include headache, mental status changes, difficulty with vision/hearing/swallowing, and loss of sensation. fever is absent unless infection is present as well. CM is diagnosed definitively by tumor cells in the CSF.

brain abscesses are collections of pus, inflammatory / immune cells, and other material that accumulates in the brain due to infection, generally from multiple organisms. streptococcus is the most common cause in adults, and gram negative bacteria a common cause of abscess in infants. abscesses occur when infection causes inflammation, which then is walled off and increases in size, blocking blood flow to the area and causing necrosis of tissue and accumulation of immune cells.

questions
anatomy review...
1. how many pairs of nerves extend from the spinal cord?√√
2. what are the meningeal layers from outside to inside?√√
3. what is the space between the dura and arachnoid mater filled with?√√

congenital forebrain anomalies...
4. how common are birth defects related to the brain compared to other organs?√√
5. anencephaly is due to... √√
6. what is a supplement that might be helpful in reducing the risk for anencephaly and when should it be taken?√√
7. what are the potential etiologies for microcephaly?X†√
8. what is polymicrogyri? X†√

hydrocephalus...
9. what is hydrocephalus? what is commonly due to?XX†√
10. what condition is hydrocephalus associated with?XX√√
11. what is the imaging hallmark of hydrocephalus?X√√
12. describe how hydrocephalus might develop.X√
13. what is normal pressure hydrocephalus?X√√

spina bifida...
14. how common is brain hypoxia?x√
15. what is spina bifida?√√
16. what is a marker that is elevated in spina bifida?√√
17. what are two potential etiologies for spina bifida?XX
18. what are two major sequelae for spina bifida?X†
19. describe the various presentations of spina bifida. which is the most common?X†

cerebral palsy...
20. what is cerebral palsy?√√
21. when does most cerebral palsy develop?√√
22. which demographic is associated with cerebral palsy?√√
23. what is the most common type of CP?X√√
24. what is a characteristic PE finding for spastic hemiplegia?†√√
25. describe the hallmarks of spastic quadriplegia?X√√
26. some children with spastic quadriplegia have...X√√

cerebral palsy morphology, prognosis, sequelae...
27. what are two morphological signs in cerebral palsy?†X
28. what is PVL?X
29. what is gliosis?X√

meningitis introduction...
30. what are the most common causes of meningitis?√√
31. what is the difference in prognosis between viral and bacterial meningitis?√√
32. what are the most common bacterial strains that cause meningitis? X√√
33. how many cases of meningitis occur each year? X√√
34. what is the most common cause of bacterial meningitis for neonates?√√
35. what is the most common cause of bacterial meningitis for infants, adolescents, and young adults?√√
36. what is the most common cause of bacterial meningitis for the elderly?√√
37. what are the main signs and symptoms of meningitis?√

CSF diagnosis...
38. how can the "opening pressure" of CSF differentiate between causes of meningitis?√√
39. describe the white blood cell count in bacterial vs. viral meningitis?√√
40. what are the findings for a cell differential in the CSF of bacterial vs. viral meningitis?†√√
41. describe the CSF to glucose ratio for the different types of meningitis.√
42. describe the protein levels in the various types of meningitis. X√
43. the latex agglutination test is most sensitive for detecting which microorganism?√√

meningococcal meningitis...
44. how common is this form of meningitis?√√
45. what is a characteristic histomorphological feature of MM?X√√†
46. describe the exudate characteristics in MM. X√√√
47. describe the gross morphological changes in MM. X√√
48. what is a dermatological manifestation of MM? X√√
49. what are the potential complications of MM? XX†√

h. influenza meningitis...
50. what is the age of peak incidence for this disease?√√√
51. may follow...√√√
52. what are some risk factors for h. influenza meningitis?√√√
53. which demographic has a higher risk for h. flu meningitis?X√XX
54. what are the potential complications for h. flu meningitis?X√X

aseptic meningitis...
55. what is aseptic meningitis?√√√
56. which two viruses are the most common causes of aseptic meningitis?X√√†
57. what are the symptoms that characterize aseptic meningitis?†√√√

cryptococcal meningitis...
58. what is the etiological agent in CM?√√√√
59. what are the demographics at risk for CM?√√√X
60. which lab tests are used to diagnose CM?†√√√
61. what is another PE finding that can aid in the diagnosis of CM?††X†√

viral meningitis...
62. what are the different types of viral meningitis? √√√√
63. most cases arise as complications of...√√√√
64. what is the most common cause of VM?√XXX√√√√
65. which virus is responsible for 10-20% of cases of VM?√X√X√√√√
66. what is a distinct morphological feature of VM?√XXX√√√√

carcinomatous meningitis...
67. what are the most common non-hematologic causes of CM?√√
68. what are the symptoms of CM?X
69. how is CM diagnosed?†√
70. 1/3 of the patients have...√
71. what percentage of CM patients have fever?√√

brain abscess...
72. what is a brain abscess?√√
73. which demographic has a higher incidence for brain abscesses?√√
74. what is the etiology of most brain abscesses?√√
75. what is the most common bug that causes brain abscesses?√√
76. which is found more commonly in infant brain abscesses, gram negative or positive bacteria?√√
77. describe the pathophysiology of brain abscesses.√√
78. what is the morphology of an aspergillus brain abscess?√

neurocysticercosis...
79. what is neurocysticercosis?√√√
80. what is the morphology of neurocysticercosis?√√√

neurosyphilis...
81. what is neurosyphilis caused by? √√√
82. which variant is associated with the middle cerebral artery or the branches of the basilar artery?√†√√
83. which variant is associated with widespread infection of parenchyma?√††
84. which variant is associated with demyelination and fibrosis of the posterior column of the spinal cord?àX
85. what are gummas?†√√
86. what is the jarisch-herxheimer reaction? what is the prognosis?√√√

progressive multifocal leukoencephalopathy...
87. what is PML caused by? XX√√√
88. what is PML? X√√√√
89. what are some symptoms of PML? X†√X
90. what is the gross morphological feature of PML? X††√XX√

subacute sclerosing panencephalitis...
91. what is SSPE caused by? X√√√
92. describe the course of SSPE. X√√√
93. what is a sign of poor prognosis for SSPE patients?√√√√
94. how is diagnosis of SSPE made? XX√
95. what is a morphological sign of SSPE? XX†X

poliomyelitis...
96. describe the infectious process in poliomyelitis.√√
97. what is post polio syndrome?√√

rabies...
98. what occurs in 1-3 months after rabies exposure? √√
99. what are some symptoms of rabies? √√
100. what is the diagnostic sign for rabies? †√√

AIDS dementia complex...
101. when during the course of AIDS does ADC emerge?√√
102. what are some nonspecific symptoms of ADC?√√
103. what are some late symptoms of ADC?√√
104. describe the pathophysiology of ADC.√√
105. what are the morphological changes of ADC?X√

reye's syndrome...
106. what is reye's syndrome characterized by?†√
107. what is reye's syndrome associated with?√√√

cerebrovascular disease...
108. how prevalent is cerebrovascular disease?√
109. what factors does the survival of ischemic tissue depend on?√
110. describe the histopathology of a stroke within the first 12-24 hours.√
111. describe the histopathology of a stroke at 24 hours.√
112. describe the histopathology of a stroke from 2-14 days.√
113. which heart condition is associated with ischemic stroke?
114. which artery is commonly affected by thrombosis leading to ischemic stroke?
115. what is the leading cause of hemorrhagic stroke?

answers
1. 31 pairs.
2. dura mater, arachnoid mater, pia mater.
3. CSF.

4. most common, 10 in 1000 live births.
5. neural tube defect.
6. folic acid pre-conception.
7. fetal alcohol syndrome, trisomy 18, congenital rubella, congenital HIV.
8. excess gyri due to decreased white matter.

9. excess CSF due to drainage problem.
10. spina bifida.
11. enlarged ventricles.
12. stenosis of the cerebral aqueduct or foramen of monroe.
13. a variant that has enlarged cerebral ventricles but only intermittent elevations of CSF pressure.

14. over 1/3 of the 30,000 children per year in the US that are born with congenital heart disease.
15. failure of closure of dorsal vertebral column or overlying skin.
16. AFP.
17. radiation to the abdomen and congenital rubella.
18. paralysis and incontinence.
19. spina bifida occulta: missing portion of dorsal vertebral body.
meningocele: herniation of the meninges but not the spinal cord.
myelomeningocele: herniation of meninges with spinal cord. most common.

20. a movement disorder related to damage to movement centers of the brain.
21. during pregnancy, due to a maternal infection.
22. premature infants, 8 months or less.
23. spastic.
24. equinus deformity, causing walking on the ball of one foot.
25. all four limbs equally affected, least likely to be able to walk.
26. hemiparetic tremors.

27. paraventricular leukomalacia and gliosis.
28. softening and necrosis of paraventricular white matter due to lack of blood supply.
29. neuronal cell death and phagocytosis, astrocyte proliferation.

30. viral infection, bacterial infection.
31. viral is generally self resolving, bacterial is generally emergency.
32. ecoli, strep pneumonia, h. influenza, neisseria meningitis, listeria monocytogenes. [ESHNL][HESNL][SHENL][SIENL][SENIL]
33. 17,000.
34. ecoli.
35. neisseria meningococci, strep pneumonia.
36. strep pneumonia.
37. fevers, headache, stiff neck, sensitivity to light and sound, petechiae.

38. increased in bacterial, normal in viral, variable in fungal and tubercular.
39. more than 1,000 / mm^3 in bacterial, more than 100/mm^3 in viral.
40. PMN's in bacterial, lymphocytes in other forms of meningitis.
41. decreased to normal in bacterial, normal in viral, decreased in fungal and tubercular.
42. increased in bacterial, fungal, tubercular, normal to increased in viral.
43. h. influenza.

44. most common form in children, second most common form in adults.
45. gram negative diplococci inside neutrophils
46. neutrophilic exudate that causes yellow/tan clouding of meninges.
47. prominent dilated vessels, edema and focal inflammation in the cortex.
48. rapidly spreading petechial rash.
49. mental retardation, permanent brain damage, hydrocephalus, myocarditis, waterhouse-friderichsen syndrome. [brain brain water water heart]

50. 6-9 months.
51. upper respiratory infection
52. recurrent URI's, daycare.
53. native americans and inuits.
54. hearing loss
seizures
learning / behavioral problems
hydrocephalus
[flu hear seize learn water] [hear the fluid water's seizing behavior]

55. signs and symptoms of meningitis are present but no bacteria is cultured in CSF.
56. coxsackie and echovirus.
57. headache, fever, inflammation of the meninges.

58. cryptoccal neoformans, a yeast that is found in soil around the world.
59. those with compromised immune systems.
60. india ink stain microscopy might reveal circular yeast bodies. CSF grows cryptococcus cultures.
61. fundoscopy: papilledema and granuloma.

62. focal, multifocal, diffuse.
63. systemic viral infections.
64. arboviruses transmitted by mosquitos and ticks.
65. HSV.
66. predilection for hemorrhage of the temporal lobe.

67. lung, breast cancer, melanoma.
68. headache
mental status changes
vision / hearing / swallowing difficulty
loss of sensation
69. presence of tumor cells in CSF.
70. headache.
71. only those with supervening infection.

72. mass of immune cells, pus, other material that accumulates in the brain due to infection.
73. immune compromised, first four decades of life.
74. generally more than one microorganism.
75. streptococcus.
76. negative.
77. infection causes inflammation and fibrosis, which leads to mass effect and blockage of blood flow to area, leading to necrosed tissue.
78. relatively slender hyphae with 45 degree angle branches.

79. infection of CNS by larvae of pork tapeworm, taenia solium.
80. multiple calcifications and viable cysts in brain parenchyma.

81. treponema pallidum infection.
82. meningovascular syphilis.
83. paretic syphilis.
84. tabes dorsalis.
85. meningeal granulomas which are well circumscribed masses of granulation tissue resulting from a cell mediated response to treponema.
86. a reaction from the treatment of treponema, resulting in headache, fever/chills, nausea. generally disappears in 24 hours.

87. members of the papovavirus family, usually JC virus.
88. rare demyelinating disease that results in the loss of oligodendrocytes.
89. memory, vision, mental function, coordination loss.
90. irregular areas of granularity in the white matter.

91. a rare complication of measles.
92. develops 1-20 years after measles.
93. deterioration of neurological function.
94. rubeola IgG antibody.
95. white matter hemorrhage and gliosis.

96. virus transmitted via oral/fecal route, replicates in nasopharynx and lymph tissues, leading to viremia, which then turns neurotropic and destroys neurons in the anterior horn and brainstem.
97. recurrent bouts of muscle weakness that occur 20-50 years after the initial polio episode. more of an "autoimmune reaction" than a reactivation of virus.

98. virus travels from peripheral nerves to the brain. time depends on depth and location of bite.
99. headache
irritability
salivation
water intolerance
muscle spasm
100. negri bodies within neurons.

101. late in the process, when CD4 count starts to drop.
102. difficulty concentration
poor coordination, muscle weakness
103.
104. uncertain, but: HIV infects CNS macrophages, which release substances that cause nerve dysfunction and death.
105. cortical atrophy and ventricular enlargement.
106. fever, vomiting
fatty infiltration
swelling of kidneys and brain
107. a previous infection and treatment using aspirin.

108. most prevalent neurologic disorder in terms of mortality and morbidity, third leading cause of death in US.
109. duration of ischemia
rapidity of onset of ischemia
magnitude of ischemia
collateral circulation
110. red neurons: vacuolated, eosinophilic neuronal cytoplasm.
111. neutrophilic infiltrate at the edge of the lesion.
112. neutrophilic / macrophage infiltrate, gliosis.
113. atrial fibrillation.
114. middle cerebral artery.
115. HTN.

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