Central Nervous System Flashcards

Pathoma CNS Chp 17

1
Q

Anencephaly

Pathology:

Symptoms/Signs:

Labs:

A

Path:
A NTD of the cranial end causing absence of a skull & brain.

Signs:
1) Frog-fetus app
2) Maternal polyhydramnios

Labs:
Low Folate levels
High AFP

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2
Q

Path:
A NTD of the cranial end causing absence of a skull & brain.

Signs:
1) Frog-fetus app
2) Maternal polyhydramnios

Labs:
Low Folate levels
High AFP

A

Anencephaly

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3
Q

Spina bifida occulta

Pathology:

Symptoms/Signs:

Labs:

A

Path:
A NTD of the caudal end causing a dimple or patch of hair over the spinal defect

Signs:
1) Dimple or hairy patch on spine

Labs:
Low Folate
High AFP

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4
Q

Path:
A NTD of the caudal end causing a dimple or patch of hair over the spinal defect

Signs:
1) Dimple or hairy patch on spine

Labs:
Low Folate
High AFP

A

Spina bifida occulta

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5
Q

Spina bifida

Pathology:

Symptoms/Signs:

Labs:

A

Patho:
A NTD of the caudal end causing a cystic protrusion along the spine

Signs:
1) Meningocele (meninges)
2) Meningomyelocele (meninges & spinal cord)

Labs:
Low Folate
High AFP

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6
Q

Patho:
A NTD of the caudal end causing a cystic protrusion along the spine

Signs:
1) Meningocele (meninges)
2) Meningomyelocele (meninges & spinal cord)

Labs:
Low Folate
High AFP

A

Spina bifida

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7
Q

Cerebral Aqueduct Stenosis

Pathology:

Symptoms/Signs:

Labs:

A

Patho:
Congenital stenosis of the aqueduct that drains the 3rd into the 4th ventricle resulting in CSF accumulation in the ventricular spaces

Signs:
1) Enlarged head circumference with dilated ventricles

Labs:
Normal CSF production from choroid plexus

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8
Q

Patho:
Congenital stenosis of the aqueduct that drains the 3rd into the 4th ventricle resulting in CSF accumulation in the ventricular spaces

Signs:
1) Enlarged head circumference with dilated ventricles

Labs:
Normal CSF production from choroid plexus

A

Cerebral Aqueduct Stenosis

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9
Q

Arnold-Chiari (II) Malformation

Pathology:

Symptoms/Signs:

Associated conditions:

A

Path:
Extension of the cerebellar tonsils into the foramen magnum obstructs CSF

Signs:
1) Hydrocephalus

Ass:
Meningomyelocele
Syringomyelia

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10
Q

Path:
Extension of the cerebellar tonsils into the foramen magnum obstructs CSF

Signs:
1) Hydrocephalus

Ass:
Meningomyelocele
Syringomyelia

A

Arnold-Chiari (II) Malformation

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11
Q

Dandy-Walker Malformation

Pathology:

Symptoms/Signs:

A

Path:
Failure of the cerebellar vermis to form (no where for CSF to go!)

Signs:
1) Massive dilation of the 4th ventricle
2) Absent cerebellum
3) Hydrocephalus

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12
Q

Path:
Failure of the cerebellar vermis to form (no where for CSF to go!)

Signs:
1) Massive dilation of the 4th ventricle
2) Absent cerebellum
3) Hydrocephalus

A

Dandy-Walker Malformation

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13
Q

Syringomyelia

Pathology:

Symptoms/Signs:

Associated conditions:

A

Path:
Cystic degeneration of the spinal cord (C8-T1) due to trauma or Arnold-Chiari (II) malformation

Signs:
1) Sensory loss or pain & temperature in cape-like distribution (anterior white commissure)

2) Spared fine touch (dorsal column)

Complication:
Syrinx expansion (compression of the upper spinal tracts) results in:

1) Damaged neurons of the anterior horn
-Muscle atrophy/weakness
- Decreased muscle tone/reflexes

2) Damaged lateral horn of hypothalamospinal tract aka Horner syndrome (ptosis, miosis, & anhidrosis)

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14
Q

Path:
Cystic degeneration of the spinal cord (C8-T1) due to trauma or Arnold-Chiari (II) malformation

Signs:
1) Sensory loss or pain & temperature in cape-like distribution (anterior white commissure)

2) Spared fine touch (dorsal column)

Complication:
Syrinx expansion (compression of the upper spinal tracts) results in:

1) Damaged neurons of the anterior horn
-Muscle atrophy/weakness
- Decreased muscle tone/reflexes

2) Damaged lateral horn of hypothalamospinal tract aka Horner syndrome (ptosis, miosis, & anhidrosis)

A

Syringomyelia

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15
Q

Poliomyelitis

Pathology:

Symptoms/Signs:

A

Path:
Damaged anterior motor horn due to poliovirus inf

Signs:
1) Lower motor neuron signs
- Flaccid paralysis,
- Muscle atrophy/fasciculations/weakness
- Impaired reflexes & -ve Babinski sign (downfacing toes)

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16
Q

Path:
Damaged anterior motor horn due to poliovirus inf

Signs:
1) Lower motor neuron signs
- Flaccid paralysis,
- Muscle atrophy/fasciculations/weakness
- Impaired reflexes & -ve Babinski sign (downfacing toes)

A

Poliomyelitis

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17
Q

Werdnig-Hoffman Disease

Pathology:

Symptoms/Signs:

A

Path:
Degenerated anterior motor horn (auto rec)

Signs:
1) Floppy baby
2) Death within a few years

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18
Q

Path:
Degenerated anterior motor horn (auto rec)

Signs:
1) Floppy baby
2) Death within a few years

A

Werdnig-Hoffman Disease

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19
Q

Amyotrophic Lateral Sclerosis (ALS)

Pathology:

Symptoms/Signs:

Causes:

A

Path:
Degeneration of the upper & lower motor neurons of the corticospinal tract that lacks sensory impairment that appears sporadically in middle aged adults

Signs:
1) Early sign (atrophy & weakness of the hands

2) Anterior horn degeneration (LMN)
- Flaccid paralysis,
- Muscle atrophy/fasciculations/weakness
- Impaired reflexes & -ve Babinski sign (downfacing toes)

3) Lateral corticospinal tract degeneration (UMN)
- Spastic paralysis & Hyperreflexia
- Increased muscle tone
- +ve Babinski

Causes:
1) Zinc-copper superoxide dismutase (SODI) in familial cases which leads to free-radical injuries in neurons

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20
Q

Path:
Degeneration of the upper & lower motor neurons of the corticospinal tract that lacks sensory impairment that appears sporadically in middle aged adults

Signs:
1) Early sign (atrophy & weakness of the hands

2) Anterior horn degeneration (LMN)
- Flaccid paralysis,
- Muscle atrophy/fasciculations/weakness
- Impaired reflexes & -ve Babinski sign (downfacing toes)

3) Lateral corticospinal tract degeneration (UMN)
- Spastic paralysis & Hyperreflexia
- Increased muscle tone
- +ve Babinski

Causes:
1) Zinc-copper superoxide dismutase (SODI) in familial cases which leads to free-radical injuries in neurons

A

Amyotrophic Lateral Sclerosis (ALS)

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21
Q

Amyotrophic Lateral Sclerosis (ALS)

Pathology:

Symptoms/Signs:

Associated conditions:

A

Path:
Degeneration of the cerebellum & spinal cord tracts due to an unstable GAA repeat in the Frataxin gene resulting in accumulation of iron in mitochondria & free radicals

Signs:
1) Cerebellum degeneration (Ataxia)

2) Spinal cord degenerations
- Loss of vibratory sense & proprioception
- Muscle weakness & loss of deep tendon reflex

Ass:
Hypertrophic cardiomyopathy

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22
Q

Path:
Degeneration of the cerebellum & spinal cord tracts due to an unstable GAA repeat in the Frataxin gene resulting in accumulation of iron in mitochondria & free radicals

Signs:
1) Cerebellum degeneration (Ataxia)

2) Spinal cord degenerations
- Loss of vibratory sense & proprioception
- Muscle weakness & loss of deep tendon reflex

Ass:
Hypertrophic cardiomyopathy

A

Amyotrophic Lateral Sclerosis (ALS)

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23
Q

Bacterial meningitis

Pathology:

Causes:

Diagnostic test: & Labs:

Symptoms/Signs:

Complications:

A

Path:
Inflammation of the leptomeninges

Causes:
1) Unvaccinated children
- Group B strep
- Listeria monocytogenes
- E. coli

2) Kids/Teens
- N. meningitis

3) Adults/Elderly
- S. pneumoniae

Tests & Labs:
1) LP (L4-L5) with gram stain & culture
2) Kernig sign
3) Neutrophils & low CSF Glucose

Signs:
1) Headache, Nuchal rigidity, & fever
2) Photophobia
3) Altered mental status
4) Vomiting

Complications:
1) Death (herniation & cerebral edema)
2) Hydrocephalus, hearing loss, & seizures

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24
Q

Path:
Inflammation of the leptomeninges

Causes:
1) Unvaccinated children
- Group B strep
- Listeria monocytogenes
- E. coli

2) Kids/Teens
- N. meningitis

3) Adults/Elderly
- S. pneumoniae

Tests & Labs:
1) LP (L4-L5) with gram stain & culture
2) Kernig sign
3) Neutrophils & low CSF Glucose

Signs:
1) Headache, Nuchal rigidity, & fever
2) Photophobia
3) Altered mental status
4) Vomiting

Complications:
1) Death (herniation & cerebral edema)
2) Hydrocephalus, hearing loss, & seizures

A

Bacterial meningitis

Pathology:

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25
Viral meningitis Pathology: Causes: Diagnostic test: & Labs: Symptoms/Signs:
Path: Inflammation of the leptomeninges Causes: 1) Unvaccinated neonates - H. influenza 2) Children via fecal-oral route - Coxsackie virus Tests & Lab: LP (L4-L5) Lymphocytes with normal CSF Glucose Signs: 1) Headache, Nuchal rigidity, & fever 2) Photophobia 3) Altered mental status 4) Vomiting
26
Path: Inflammation of the leptomeninges Causes: 1) Unvaccinated neonates - H. influenza 2) Children via fecal-oral route - Coxsackie virus Tests & Lab: LP (L4-L5) Lymphocytes with normal CSF Glucose Signs: 1) Headache, Nuchal rigidity, & fever 2) Photophobia 3) Altered mental status 4) Vomiting
Viral meningitis
27
Fungal Meningitis Pathology/Causes: Diagnostic test: & Labs: Symptoms/Signs:
Path: Inflammation of meninges in immunocompromised patients (HIV/AIDS) Test/Labs: LP & Staining Lymphocytes & low CSF Glucose Signs: 1) Headache, Nuchal rigidity, & fever 2) Photophobia 3) Altered mental status 4) Vomiting
28
Path: Inflammation of meninges in immunocompromised patients (HIV/AIDS) Test/Labs: LP & Staining Lymphocytes & low CSF Glucose Signs: 1) Headache, Nuchal rigidity, & fever 2) Photophobia 3) Altered mental status 4) Vomiting
Fungal Meningitis
29
Global Cerebral Ischemia Pathology: Causes: Symptoms/Signs:
Path: Ischemia of the brain Causes: 1) Atherosclerosis (low perfusion) 2) Anemia (Chronic hypoxia) 3) Insulinoma (repeated episodes of hypoglycemia) Signs: 1) Mild global ischemia (transient confusion with a prompt recovery) 2) Severe global ischemia (infarcts in watershed areas that damage vulnerable areas)
30
Path: Ischemia of the brain Causes: 1) Atherosclerosis (low perfusion) 2) Anemia (Chronic hypoxia) 3) Insulinoma (repeated episodes of hypoglycemia) Signs: 1) Mild global ischemia (transient confusion with a prompt recovery) 2) Severe global ischemia (infarcts in watershed areas that damage vulnerable areas)
Global Cerebral Ischemia
31
Severe Global cerebral ischemia of the cerebral cortex pyramidal neurons causes what?
Laminar necrosis
32
Severe Global cerebral ischemia of the Hippocampus pyramidal neurons causes what?
Long term memory loss
33
Severe Global cerebral ischemia of the cerebellum Purkinje neurons causes what?
Impaired sensory perception & motor function
34
Thrombotic stroke Pathology: Histological finding:
Path: Rupture of an atherosclerotic plaque (branch point of ICA & MCA in the circle of Willis) Histo: Pale infarct at the cortex's periphery
35
Embolic stroke Pathology: Histological finding:
Path: Thromboemboli from Afib (left heart) that obstructs the MCA Histo: Hemorrhagic infarct
36
Path: Rupture of an atherosclerotic plaque (branch point of ICA & MCA in the circle of Willis) Histo: Pale infarct
Thrombotic stroke
37
Path: Thromboemboli from Afib (left heart) that obstructs the MCA Histo: Hemorrhagic infarct
Embolic stroke
38
Lacunal stroke Pathology: Histological finding:
Path: Secondary to hyaline arteriosclerosis due to HTN or diabetes that obstructs the lenticulostriate vessels Histo: Small cystic areas of infarction 1) Internal capsule causes pure motor stroke 2) Thalamus causes a pure sensory stroke
39
Path: Secondary to hyaline arteriosclerosis due to HTN or diabetes that obstructs the lenticulostriate vessels Histo: Small cystic areas of infarction 1) Internal capsule causes pure motor stroke 2) Thalamus causes a pure sensory stroke
Lacunal stroke Pathology: Histological finding:
40
Ischemic stroke Pathology: Histological finding:
Path: Liquefactive necrosis Histo: 12hrs = eosinophilic change 24hrs = coagulative necrosis 1-3 days = neutrophils 4-7 days = microglial cells 2-3 wks = tissue granulation
41
Path: Liquefactive necrosis Histo: 12hrs = eosinophilic change 24hrs = coagulative necrosis 1-3 days = neutrophils 4-7 days = microglial cells 2-3 wks = tissue granulation
Ischemic stroke
42
Intracerebellar hemorrhage Pathology: Cause: Signs:
Path: Bleeding into parenchyma that primarily impacts the basal ganglia Cause: Rupture of A Charcot-Bouchard microaneurysm in the lenticulostriate vessels (a complication of HTN) Signs: 1) Severe headache 2) N/V 3) Eventual coma
43
Path: Bleeding into parenchyma that primarily impacts the basal ganglia Cause: Rupture of A Charcot-Bouchard microaneurysm in the lenticulostriate vessels (a complication of HTN) Signs: 1) Severe headache 2) N/V 3) Eventual coma
Intracerebellar hemorrhage
44
Subarachnoid hemorrhage Pathology: Cause: Signs: Tests/Histology:
Path: Bleeding into the subarachnoid space Cause: Rupture of a berry aneurysm in the ACA Signs: 1) Worst headache of my life 2) Nuchal rigidity Tests/Histo: LP showing xanthochromia (yellow CSF)
45
Path: Bleeding into the subarachnoid space Cause: Rupture of a berry aneurysm in the ACA Signs: 1) Worst headache of my life 2) Nuchal rigidity Tests/Histo: LP showing xanthochromia (yellow CSF)
Subarachnoid hemorrhage
46
Epidural hematoma Pathology: Causes: Tests: Complication:
Path: Bleeding separates the dura from skull Causes: Fractured temporal bone with ruptured middle meningeal artery (fast bleed!!!) Tests: CT shows a lens shaped lesion (lemon) Complication: Herniation (Lethal)
47
Path: Bleeding separates the dura from skull Causes: Fractured temporal bone with ruptured middle meningeal artery (fast bleed!!!) Tests: CT shows a lens shaped lesion (lemon) Complication: Herniation (Lethal)
Epidural hematoma
48
Subdural hematoma Pathology: Causes: Symptoms/signs: Diagnostic tests: Complication:
Path: Bleeding beneath the dura Causes: Trauma or age-related cerebral atrophy can both tear the bridging veins (slow bleed) Signs: 1) Progressive neurological deficits Tests: Crescent lesion on CT (banana) Complication: Herniation (lethal)
49
Path: Bleeding beneath the dura Causes: Trauma or age-related cerebral atrophy can both tear the bridging veins (slow bleed) Signs: 1) Progressive neurological deficits Tests: Crescent lesion on CT (banana) Complication: Herniation (lethal)
Subdural hematoma
50
Tonsillar herniation Pathology: Causes: Symptoms/Signs:
Path: Cerebellar tonsils herniate into the foramen magnum & compress the brainstem Causes: Increased ICP or mass effect Signs: 1) Cardiopulmonary arrest
51
Path: Cerebellar tonsils herniate into the foramen magnum & compress the brainstem Causes: Increased ICP or mass effect Signs: 1) Cardiopulmonary arrest
Tonsillar herniation
52
Subfalcine herniation Pathology: Causes: Symptoms/Signs:
Path: Cingulate gyrus herniates under the falx cerebri & compresses the anterior cerebral artery Causes: Increased ICP or mass effect Signs: 1) Infarction
53
Path: Cingulate gyrus herniates under the falx cerebri & compresses the anterior cerebral artery Causes: Increased ICP or mass effect Signs: 1) Infarction
Subfalcine herniation
54
Uncal herniation Pathology: Causes: Symptoms/Signs:
Path: Temporal lobe herniates under the tentorium cerebelli & compresses the Oculomotor nerve (CNIII), Posterior cerebral artery, & ruptures the Paramedian artery Causes: Increased ICP or mass effect Signs: 1) Down & out turned eye with miosis 2) Contralateral homonymous hemianopsia (occipital lobe infarct) 3) Duret (brainstem) hemorrhage
55
Path: Temporal lobe herniates under the tentorium cerebelli & compresses the Oculomotor nerve (CNIII), Posterior cerebral artery, & ruptures the Paramedian artery Causes: Increased ICP or mass effect Signs: 1) Down & out turned eye with miosis 2) Contralateral homonymous hemianopsia (occipital lobe infarct) 3) Duret (brainstem) hemorrhage
Uncal herniation
56
Metachromatic leukodystrophy Pathology:
Path: Arylsulfatase deficiency causing buildup of myelin in oligodendrocytes (impaired myelin degradation)
57
Path: Arylsulfatase deficiency causing buildup of myelin in oligodendrocytes (impaired myelin degradation)
Metachromatic leukodystrophy
58
Adrenoleukodystrophy Pathology:
Path: Impaired addition of coenzyme A onto long chain FA's causing buildup in the adrenals & white brain matter (X-linked)
59
Path: Impaired addition of coenzyme A onto long chain FA's causing buildup in the adrenals & white brain matter (X-linked)
Adrenoleukodystrophy
60
Krabbe disease Pathology:
Path: Glucocerebroside B-Galactosidase deficiency causing Glucocerebroside build up in macrophages
61
Path: Glucocerebroside B-Galactosidase deficiency causing Glucocerebroside build up in macrophages
Krabbe disease
62
Multiple sclerosis: Pathology: Causes/Etiology: Symptoms/Signs: Diagnostic tests/Histological findings: Treatments: -Acute vs Long term
Path: Autoimmune destruction of CNS myelin & oligodendrocytes (Demyelination) Causes/Etiology: HLA-DR2 genotypes & women (20-30yrs) Signs: 1) Blurry vision in one eye (optic nerve) 2) Vertigo & drunk-like speech (brainstem) 3) Intranuclear ophthalmoplegia (medial longitudinal fasciculitis "eyes can't look to same side at the same time") 4) Hemiparesis/Unilateral sensation loss (white matter) 5) Sensation loss/weakness in the lower extremities (spinal cord) 6) Bowel, Bladder, & Sexual dysfunction (ANS) Tests/Histo: 1) LP (high lymphocytes, Oligoclonal IgG bands, & Myelin basic proteins) 2) MRI (plaques in white matter) 3) Gross exam (gray plaques in white matter) Treatment: Acute attacks = High dose steroid Longterm = Interferon B (slows prog)
63
Path: Autoimmune destruction of CNS myelin & oligodendrocytes (Demyelination) Causes/Etiology: HLA-DR2 genotypes & women (20-30yrs) Signs: 1) Blurry vision in one eye (optic nerve) 2) Vertigo & drunk-like speech (brainstem) 3) Intranuclear ophthalmoplegia (medial longitudinal fasciculitis "eyes can't look to same side at the same time") 4) Hemiparesis/Unilateral sensation loss (white matter) 5) Sensation loss/weakness in the lower extremities (spinal cord) 6) Bowel, Bladder, & Sexual dysfunction (ANS) Tests/Histo: 1) LP (high lymphocytes, Oligoclonal IgG bands, & Myelin basic proteins) 2) MRI (plaques in white matter) 3) Gross exam (gray plaques in white matter) Treatment: Acute attacks = High dose steroid Longterm = Interferon B (slows prog)
Multiple sclerosis:
64
Subacute sclerosing panencephalitis Pathology: Cause: Symptoms/Signs:
Path: Progressive & deadly encephalitis of the brain Causes: Infantile Measels infection that persists Signs: 1) Neurological deficits appear in childhood
65
Path: Progressive & deadly encephalitis of the brain Causes: Infantile Measels infection that persists Signs: 1) Neurological deficits appear in childhood
Subacute sclerosing panencephalitis
66
Progressive Multifocal Leukoencephalopathy Pathology: Cause: Symptoms/Signs:
Path: Reactivation JC virus infection in the oligodendrocytes of immunocompromised (AIDS/Leukemia) people Cause: Reactivation of latent JC virus Signs: 1) Rapidly progressive neuro (vision loss, weakness, & dementia) 2) Death
67
Path: Reactivation JC virus infection in the oligodendrocytes of immunocompromised (AIDS/Leukemia) people Cause: Reactivation of latent JC virus Signs: 1) Rapidly progressive neuro (vision loss, weakness, & dementia) 2) Death
Progressive Multifocal Leukoencephalopathy
68
Central Pontine Myelinolysis Pathology: Cause: Symptoms/Signs:
Path: Focal demyelination of the pons (anterior brainstem) in severely malnourished patients (alcoholics or liver disease) Cause: Rapid IV hyponatremia correction Signs: 1) Acute bilateral paralysis (Locked in synd)
69
Path: Focal demyelination of the pons (anterior brainstem) in severely malnourished patients (alcoholics or liver disease) Cause: Rapid IV hyponatremia correction Signs: 1) Acute bilateral paralysis (Locked in synd)
Central Pontine Myelinolysis
70
Alzheimer's Pathology: Causes: Symptoms/Signs: Histological findings: Complication:
Path: Degeneration of the cortex Causes: 1) E4 Apolipoprotein E 2) Familial presenilin 1 & 2 mutations 3) Down syndrome (40yrs) Signs: 1) Slow onset memory loss that progresses from acute to Longterm memory 2) Motor & language skill loss 3) Behavior/Personality changes Histo: 1) AB-Plaques & Neurofibrillary tau tangles 2) Cerebral atrophy with narrowed gyri & widened sulci 3) Loss of cholinergic neurons in the nucleus of basalis of Meynert Complication: Infection as the main COD & Hemorrhaging (amyloid deposits in BVs)
71
Path: Degeneration of the cortex Causes: 1) E4 Apolipoprotein E 2) Familial presenilin 1 & 2 mutations 3) Down syndrome (40yrs) Signs: 1) Slow onset memory loss that progresses from acute to Longterm memory 2) Motor & language skill loss 3) Behavior/Personality changes Histo: 1) AB-Plaques & Neurofibrillary tau tangles 2) Cerebral atrophy with narrowed gyri & widened sulci 3) Loss of cholinergic neurons in the nucleus of basalis of Meynert Complication: Infection as the main COD & Hemorrhaging (amyloid deposits in BVs)
Alzheimer's
72
Vascular dementia Pathology:
Path: Multifocal infarction due to HTN, Atherosclerosis, or vasculitis
73
Path: Multifocal infarction due to HTN, Atherosclerosis, or vasculitis
Vascular dementia
74
Parkinsons disease Pathology: Symptoms/Signs: Histology: Complication:
Path: Degeneration of the frontal & temporal cortex (sparing the parietal & occipital lobes) Signs: "TRAP" 1) Tremor (pinwheel at rest) 2) Rigidity (cogwheel) 3) Akinesia/Bradykinesia 4) Postural instability/Shuffling gait Histo: 1) Lewy bodies (round & eosinophilic inclusions of alpha synclein) 2) Loss of pigmented neurons in substantia nigra Complication: Lewy body dementia
75
Path: Degeneration of the frontal & temporal cortex (sparing the parietal & occipital lobes) Signs: "TRAP" 1) Tremor (pinwheel at rest) 2) Rigidity (cogwheel) 3) Akinesia/Bradykinesia 4) Postural instability/Shuffling gait Histo: 1) Lewy bodies (round & eosinophilic inclusions of alpha synclein) 2) Loss of pigmented neurons in substantia nigra Complication: Lewy body dementia
Parkinsons disease
76
Lewy body dementia Pathology: Symptoms/Signs: Histology:
Path: A complication of Parkinson disease Signs: 1) Dementia 2) Hallucinations 3) Parkinsonian features Histo: Cortical lewy bodies
77
Path: A complication of Parkinson disease Signs: 1) Dementia 2) Hallucinations 3) Parkinsonian features Histo: Cortical lewy bodies
Lewy body dementia
78
Huntington disease Pathologies: Causes: Symptoms/Signs: Complication:
Path: Degeneration of GABAergic neurons in the caudate nucleus of the basal ganglia Causes: Avg age ~40yrs Expanded CAG huntingtin gene (repeats during spermatogenesis leads to anticipation) Signs: 1) Chorea with dementia & depression Complication: Suicide is the main COD
79
Path: Degeneration of GABAergic neurons in the caudate nucleus of the basal ganglia Causes: Avg age ~40yrs Expanded CAG huntingtin gene (repeats during spermatogenesis leads to anticipation) Signs: 1) Chorea with dementia & depression Complication: Suicide is the main COD
Huntington disease
80
Normal pressure hydrocephalus Pathology: Symptoms/Signs: Treatments:
Path: Increased CSF dilates the ventricles causing dementia Signs: "Wet, Wobbly, & Wacky" 1) Urine incontinence 2) Gait instability 3) Dementia Treatment: Acute (LP) Long-term (Ventriculoperitoneal shunting
81
Path: Increased CSF dilates the ventricles causing dementia Signs: "Wet, Wobbly, & Wacky" 1) Urine incontinence 2) Gait instability 3) Dementia Treatment: Acute (LP) Long-term (Ventriculoperitoneal shunting
Normal pressure hydrocephalus
82
Bovine spongiform encephalopathy Pathology:
Path: CJD variant "mad cow"
83
Creutzfeldt-Jakob disease (Spongiform encephalopathy) Pathology: Symptoms/Signs: Causes: Histology: Complications:
Path: Conversion of normal prions to B-pleated form (PrPsc) Signs: 1) Rapidly progressive dementia associated with ataxia & startle myoclonus Causes: Exposure to prion-infected human tissue (hGH or corneal transplant) Histo: 1) Spongy degeneration (intracellular vacuoles) 2) Spike-wave complexes on EEG Complication: Death within a year
84
Path: Conversion of normal prions to B-pleated form (PrPsc) Signs: 1) Rapidly progressive dementia associated with ataxia & startle myoclonus Causes: Exposure to prion-infected human tissue (hGH or corneal transplant) Histo: 1) Spongy degeneration (intracellular vacuoles) 2) Spike-wave complexes on EEG Complication: Death within a year
Creutzfeldt-Jakob disease (Spongiform encephalopathy)
85
Familial Fatal Insomnia Pathology:
Path: Severe insomnia & an exaggerated startle response
86
Glioblastoma Multiforme Pathology: Histological findings: Outcome:
Path: Malignant Astrocyte tumor common in adults Histo: 1) Butterfly lesion in the cerebral hemisphere (crosses corpus callosum) 2) Pseudopalisading (necrotic regions surrounded by the tumor) 3) Endothelial proliferation 4) GFAP +ve Outcome: Poor prognosis
87
Path: Malignant Astrocyte tumor common in adults Histo: 1) Butterfly lesion in the cerebral hemisphere (crosses corpus callosum) 2) Pseudopalisading (necrotic regions surrounded by the tumor) 3) Endothelial proliferation 4) GFAP +ve Outcome: Poor prognosis
Glioblastoma Multiforme
88
Meningioma Pathology: Symptoms/Signs: Histology:
Path: Benign arachnoid cell tumor that compresses (NOT invades) the cortex (usually in women) Signs: 1) Seizures Histology: 1) Round mass attached to the dura with a whorled pattern & psammoma bodies
89
Path: Benign arachnoid cell tumor that compresses (NOT invades) the cortex (usually in women) Signs: 1) Seizures Histology: 1) Round mass attached to the dura with a whorled pattern & psammoma bodies
Meningioma
90
Schwannoma Pathology: Symptoms/Signs: Histology: Complication:
Path: Benign schawnn cell tumor that invades cranial & spinal nerves (especially CN VIII) at the cerebellar pontine angle Signs: 1) Hearing loss & Tinnitus Histo: S-100+ve tumor cells Complication: Bilateral tumor indicates type 2 Neurofibromatosis
91
Path: Benign Schawnn cell tumor that invades cranial & spinal nerves (especially CN VIII) at the cerebellar pontine angle Signs: 1) Hearing loss & Tinnitus Histo: S-100+ve tumor cells Complication: Bilateral tumor indicates type 2 Neurofibromatosis
Schwannoma
92
Oligodendroglioma Pathology: Symptoms/Signs: Histology:
Path: Malignant tumor of oligodendrocytes of the frontal lobe Signs: 1) Seizure Histo: Calcified tumor in the white matter "fried-egg app"
93
Path: Malignant tumor of oligodendrocytes of the frontal lobe Signs: 1) Seizure Histo: Calcified tumor in the white matter "fried-egg app"
Oligodendroglioma
94
Pilocytic Astrocytoma Pathology: Histology: | "C-GEAR"
Path: Benign astrocyte tumor (most common in children) Histo: 1) Cystic lesion with a mural nodule (cerebellum) 2) Rosenthal fibers 3) Eosinophilic granular bodies 4) GFAP +ve tumor cells
95
Path: Benign astrocyte tumor (most common in children) Histo: 1) Cystic lesion with a mural nodule (cerebellum) 2) Rosenthal fibers 3) Eosinophilic granular bodies 4) GFAP +ve tumor cells
Pilocytic Astrocytoma
96
Medulloblastoma Pathology: Histology: Complication:
Path: Malignant granular cell (neuroectoderm) tumor in children with a poor prognosis Histo: 1) Small round blue cells 2) Homer wright rosettes Complication: Metastasis to CSF to the cauda equina "drop metastasis"
97
Path: Malignant granular cell (neuroectoderm) tumor in children with a poor prognosis Histo: 1) Small round blue cells 2) Homer wright rosettes Complication: Metastasis to CSF to the cauda equina "drop metastasis"
Medulloblastoma
98
Ependymoma Pathology: Symptoms/Signs: Histology:
Path: Malignant ependymal cell tumor in the 4th ventricles (children) Signs: 1) Hydrocephalus Histo: Perivascular pseudorosettes
99
Path: Malignant ependymal cell tumor in the 4th ventricles (children) Signs: 1) Hydrocephalus Histo: Perivascular pseudorosettes
Ependymoma
100
Craniopharyngioma Pathology: Symptoms/Signs: Histology: Complications:
Patho: Benign epithelial remnant of Rathke's tumor that presents as a supratentorial mass in children/young adults Signs: 1) Bilateral hemianopsia (compressed optic nerve) Histo: Calcifications with tooth-like tissue Complication: It recurs post resection
101
Patho: Benign epithelial remnant of Rathke's tumor that presents as a supratentorial mass in children/young adults Signs: 1) Bilateral hemianopsia (compressed optic nerve) Histo: Calcifications with tooth-like tissue Complication: It recurs post resection
Craniopharyngioma