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Pathology > Neuro: Pathology > Flashcards

Neuro: Pathology Flashcards

1
Q
  • Failure of Rostral Neuropore closure
  • Characherized by the absence of the Scalp, Skull, and Large portions of the Cortex
  • Anterior neuropore closure @ day 25
  • Posterior neuropore closure @ day 27
  • Increased levels of AFP and ACh
A

Anencephaly

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2
Q
  • Failure of Caudal neopore closure
  • Severity depends on the degree of closure as well as the location of the Fusion defect
  • Elevated AFP in Maternal serum or Amniotic fluid
A

Spina Bifida

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3
Q
  • Small Tuft of hair overlying the defect without any visible Herniate contents
  • Asymptomatic
  • Missing Lumbar or Sacral processes
  • Failure of the Vertebral arches to close w/out Herniation of Intraspinal contents
A

Spina Bifida Occulta

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4
Q
  • Protrusion of the Dura mater and Arachnoid, forming a Lumbosacral cyst
  • Failure of Vertebral arches to close w/ Herniation of Meninges but NOT the Spinal cord
A

Spina Bifida Meningocele

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5
Q
  • Herniated Lumbosacral sac
  • Depending on the location: Paralysis and Loss of Deep Tendon reflexes and Sensation in the lower extremities as well as Incontinence
  • A/w Type II Arnold-Chiari syndrome
A

Spina Bifida Meningomyelocele

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6
Q
  • Mental Retardation
  • Herniation of Meninges and Brain
A

Spina Bifida Meningoencephalocele

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7
Q
  • Herniation of Meninges, Brain, and CSF-containing Ventricles
A

Spina Bifida Meningohydroencephalocele

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8
Q
  • Most severe form of Spina Bifida w/ Myelomeningocele
  • Flattened plate like mass of nervous tissue w/ no overlying membrane
  • More prone to life threatening Infections and Meningitis
  • Split Brain
A

Spina Bifida w/ Myeloshisis

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9
Q
  • Neural tube Birth defect
  • Posterior Neural tube fails to close by the 27th day
  • Vertebrae over defect fail to form and remain Unfused and Open
  • Spinal cord remains exposed
  • Motor and Sensory defects
  • Chronic Infections
  • Bladder disfunction
  • A/w Anencephaly
A

Rachischisis

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10
Q
  • Enlarging Head circumference
  • Dilation of the ventricles (Cranial suture lines not fused)
  • Congenital Stenosis between 3rd and 4th Ventricle
  • Accumulation of CSF
  • Common in Newborns
A

Cerebral Aqueduct Stenosis

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11
Q
  • Agenesis / Congenital failure of the Cerebellar Vermis Hypoplasia and Splenium of Corpus Callosum
  • **Failure ** to Open of the Foramina of Luschka and Foramina of Magendie
  • Massively dilatated 4th Ventricle (Posterior fossa) w/out Cerebellum
  • Accompanied by Hydrocephalus
A

Dandy-Walker Malformation

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12
Q
  • Herniation of the Cerebellar Tonsils ONLY
  • May not show neurologic symptoms until Adolescens or Adult life
  • Cerebellar Ataxia
  • Obstructive Hydrocephalus
  • Brain stem compression
  • Syringomyelia
  • Large Foramen magnum
A

Arnold-Chiari Type I

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13
Q
  • Congenital downward displacement of Cerebellar vermis and Tonsils through the Foramen magnum
  • A/w obstruction of CSF and Hydrocephalus and Meningomyelocele
  • Difficulty swallowing (compression of the Nucleus Ambiduus)
  • Loss of Pain / Temperature sensation along the Back of the Neck and Shoulders
  • Cranial Nerve and Brain stem dysfunction results in Respiratory failure and Death
A

Arnold-Chiari Type II

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14
Q
  • Cystic degeneration of the Spinal cord
  • Arises w/ Trauma and A/w Type I Arnold-Chiari malformation, Usually C8 – T1
  • Sensory (Pain, Temp. Loss) w/ sparing of Fine touch w/ “Cape-like” distribution – Anterior white commissure of the Spinothalamic Tract w/ sparing of the Dorsal column
  • Muscle atrophy and Weakness – dmg to lower motor neurons of Ant. Horn
  • A/w Horner Syndrome w/ Ptosis, Miosis, and Anhydrosis
A

Syringomyelia

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15
Q
  • Sympathetic trunk nerves get damaged, same side
  • Miosis (constricted pupil)
  • Ptosis (droopy eyelid)
  • Anhidrosis (decreased sweating)
  • A/ diseases may be Congential or Acquired
A

Horner Syndrome

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16
Q
  • Dmg to Anterior Motor Horn due to Poliovirus Infection (Piconaviridae Enterovirus)
  • Fecal-oral transmission
  • Sore throat, malaise
  • Lower motor neuron – Flaccid paralysis w/ Muscle atrophy
  • Fasciculations - small uncontrolled muscle twitch
  • Weaness w/ decreased Muscle tone
  • Impaired reflexes
  • Negative Babinski sign (downgoing toes)
A

Poliomyelitis

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17
Q
  • Inherited degeneration of the Anterior Motor Horn
  • Autosomal recessive
  • “Floppy baby”
  • Death occurs w/in a few years after birth
A

Werding-Hoffman Disease

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18
Q
  • Degenerative disorder of the Upper and Lower motor neurons of the Corticospinal tract - Anterior Horns
  • Zinc-copper Superoxide Dismutase mutation (SOD1) w/ Familial cases (chrom 21) – free radical injury in neurons
  • Anterior motor horn degn. – Lower motor neuron signs, Flaccid paralysis w/ muscle atrophy, Fascicultations, Weakness, Impared reflexes, Negative Babinski
  • Lateral corticospinal tract degn. – Upper motor neuron signs, Spastic paralysis w/ Hyperreflexia, Increased tone, Positive Babinski
A

Amyotrophic Lateral Sclerosis (ALS)

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19
Q
  • Degn. disorder of Cerebellum (Ataxia) and Spinal cord (Loss of Vibratory sense and Proprioception, Muscle weakness in Lower extremities, and loss of Deep tendon reflexes)
  • Autosomal Recessive – unstable trinucleotide repeat (GAA) in Frataxin gene
  • Presents in early childhood
  • Pts. are wheelchair bound w/in a few years
  • A/w Hypertrophic cardiomyopathy (HCM)
  • Frataxin – essential for Mitochondrial iron regulation – Free radical damage via Fenton reaction
A

Friedreich Ataxia

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20
Q
  • Group B Streptococci (Streptococcus agalactia)
  • Listeria monocytogenes
  • E. coli (Escherichia coli)
A

Neonatal Meningitis

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21
Q
  • Pneumococci (Streptococcus pneumonia)
  • Meningococci (Neisseria meningitidis)
  • Influenzae Type B (Haemophilus influenza Type B) (non-vaccinated kids)
A

Meningitis (6 mos. – 6 yrs.)

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22
Q
  • Meningococci (Neisseria meningitidis)
  • Polio (Picornavirdae enterovirus)
  • Pneumococci (Streptococcus pneumonia)
A

Meningitis (6 yrs. 60 yrs)

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23
Q
  • Cryptococcosis (Cryptococcus neoformans)
A

Fungal Meningitis

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24
Q
  • Coxsackie A & B (Picornaviridae enterovirus)
  • Echovirus (Picornaviridae enterovirus B)
  • Mumps (Paramyxoviridae rubulavirus)
  • Polio (Picornavirdae enterovirus)
A

Aseptic Meningitis

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25
Q

Bacterial Profile of Meningitis?

A
  • Protein: ↑ ↑
  • Glucose: ↓ ↓
  • Cell Infiltration: Neutrophils and PMNs
  • Pressure: ↑
  • Gram stain and Culture often Identify causative agent
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26
Q

Viral Profile of Meningitis?

A
  • Protein: ↑ or Normal
  • Glucose: Normal
  • Cell Infiltration: Lymphocytes w/ normal CSF
  • Pressure: Normal
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27
Q

Fungal Profile of Meningitis?

A
  • Protein: ↑
  • Glucose: ↓
  • Cell Infiltration:Lymphocytes
  • Pressure: ↑
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28
Q

Two Basic Types of Cerebrovascular Disease?

A
  1. Ischemia
    • Focal
      • Thrombotic stroke
      • Embolic stroke
      • Lacunar stoke
    • Global
  2. Hemorrhage
    • Intracerebral
    • Subarachnoid
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29
Q
  • Low perfusion (atherosclerosis)
  • Acute decrease in blood flow (Cardiogenic shock, Overall decrease) - Chronic hypoxia (anemia)
  • Repeated episodes of Hypoglycemia (Insulinoma)
  • Mild – Transient confusion w/ prompt recovery
  • Moderate – Infarcts in watershed areas
    • Pyramidal neurons of Cerebral cortex – Laminar necrosis
    • Pyramidal neurons of Hippocampus (Temporal lobe) – long term memory
  • Severe – Diffuse necrosis w/ ‘vegetative state’
A

Global Cerebral Ischemia

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30
Q
  • Rupture of an Atherosclerotic plaque
  • Pale infarct at the periphery of the Cortex
  • Atherosclerosis usually develops at Branch points (Bifurcation of Internal Carotid and Middle cerebral artery, in Circle of Willis)
A

Thrombotic Stroke

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31
Q
  • Due to Thromboemboli
  • Commonly arising from the Left side of the Heart (Atrial fibrillation)
  • Usually involves the Middle Cerebral artery
  • After lysis of Emboli - Hemorrhagic infarct at the Periphery of the Cortex
A

Embolic Stroke

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32
Q
  • Occurs secondary to Hyaline arteriolosclerosis
  • Complication of Benign HTN, Diabetes mellitus
  • Most commonly involves Lenticulostriate vessels, resulting in small cystic areas of infarction
  • Involvement of the Internal capsule leads to a pure Motor stroke
  • Involvement of the Thalamus leads to a pure Sensory Stroke
A

Lacunar Stroke

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33
Q

Progression of Ischemic Stroke?

A
  • 12 hrs after Infarction: Eosinophilic change in the cytoplasm of neurons (“Red neurons”) is an early microscopic finding
  • 24 hrs after infarction:
    • Neutrophils (days 1 - 3)
    • Microglial cells (days 4 – 7)
    • Gliosis (weeks 2 – 3)
  • Formation of Fluid-filled cystic space surrounded by Gliosis – Caseating necrosis and Liquefactive necrosis
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34
Q
  • Bleeding into the Brain parenchyma
  • Rupture of Charcot-Bouchard microaneurysms of Lenticulostriate vessels – Deep bleeding in Paranchyma of Brain
  • A/w HTN, reduced HTN decreases incidence by half
  • Severe Headache, Nausea, Vomiting, and eventual Coma
A

Intracerebral Hemorrhage

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35
Q
  • Bleeding into the Subarachnoid space
  • Sudden Headache “Worse Headache of my Life” w/ Nuchal rigidity
  • Lumbar puncture shows Xanthochromia (yellow hue due to Bilirubin breakdwon)
  • A/w Berry aneurysm – Saccular (aneurysm) outpouchings that lack media layer, esp. Anterior Circle of Willis @ branch points of the Anterior communicating artery
  • A/w Marfan syndrome (FBN1 on Chr. 15 misfolding of Fibrillin-1 – connective tissue weakness) and ADPKD (MVP, Hepatic cysts)
  • Other causes AV malformations and an Anticoagulated state
A

Subarachnoid Hemorrhage

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36
Q
  • Blood between Dura mater and Skull – bleeding seperates
  • Fracture of the Temporal bone w/ rupture of the Middle meningeal artery
  • A/w a blow to the head, head trauma
  • “Lucid interval” may precede Neurologic signs
  • Lens-shaped lesion on CT scan
  • Herniation – a lethal complication, pushes brain to other side
A

Epidural Hematoma

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37
Q
  • Blood under the Dura mater, covering the surface of the brain
  • Tearing of Bridging veins that lie between the Dura and Arachnoid
  • Trauma
  • Crescent-shaped on CT
  • Progressive neurologic signs – leading toward Herniation (lethal)
  • Increased rate in Elderly due to age-related cerebral atrophy, stretching the veins
A

Subdural Hematoma

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38
Q
  • Displacement of the cerebellar tonsils into the Foramen magnum
  • Compression of the Brain stem leads to Cardiopulmonary arrest
A

Tonsillar Herniation

39
Q
  • Displacement of the Cingulate gyrus under the Falx cerebri
  • Compression of the Anterior cerebral artery leads to infarction
A

Subfalcine Herniation

40
Q
  • Displacement of the Temporal lobe uncus under the Tentorium cerebelli
  • Compression of CN III (oculomotor) leads to the eye moving “down and out” and a Dilated pupil
  • Compression of Posterior cerebral artery leads to Infarction of Occipital lobe (Contralateral homonymous hemianopsia)
  • Rupture of the Paramedian artery leads to Duret (brainstem) hemorrhage
A

Uncal Herniation

41
Q
  • Autosomal recessive lysosomal storage disease
  • Arylsulfatase A Deficiency (Autosomal recessive)
  • Most common Leukodystrophy
  • Sulfatides cannot be degraded and Accumulate in the Lysosome of Oligodendrocytes (lysosomal storage disease) -> impaired production of myelin sheath
  • Central and Peripheral Demyelination w/ Ataxia and Dementia
  • Accumulation of Sulfatides
A

Metachromatic Leukodystrophies

42
Q
  • Autosomal recessive Lysosomal Storage dieseae
  • Deficiency of Galactocerebrosidase
  • Galactocerebroside and Psychosine destroy myelin sheath, accumulates in Macrophages
  • Peripheral neuropathy
  • Developmental delay
  • Optic atrophy
  • Globoid cells
A

Krabbe disease

43
Q
  • Mostly Males
  • Impaired Metabolism and addition of Coenzyme A to Long-chain Fatty-acids (X-linked defect)
  • Accumulation of Fatty-acids damages Adrenal glands (Adrenal gland crisis) and White matter of the Brain
  • **Long-term progression **-> Death
A

Adrenoleukodystrophy

44
Q
  • Autoimmune inflammation / destruction of CNS myelin and Oligodendrocytes (demyelination)
  • Chronic CNS disease of Young Women (20 – 30 yrs old) (F > M) - Brain and Spinal cord
  • A/w HLA-DR2
  • Regions away from Equator
  • Blurred vision in ONE eye (optic nerve) (Marcus Gunn pupils)
  • Vertigo and Scanning speech mimicking Alcohol intoxication (Brain stem)
  • Internuclear ophthalmoplegia (medial longitudinal fasciculus)
  • Hemiparesis or Unilateral loss of sensation (cerebral white matter)
  • Lower extremeity loss of sensation (spinal cord)
  • Bowel, Bladder, and Sexual dysfunction (ANS)
  • Dx: MRI and Lumbar puncture (increased Lymphocytes, Immunoglobulins w/ Oligoclonal IgG bands, Myelin basic protein)
  • Gray appearing plaques in White matter
  • Tx: Acute: IV Solumedrol, Chronic: High-dose steroids, and Long-term β-INF
A

Multiple Sclerosis

Charcot classic triad of MS is a SIN

  • Scanning speech
  • Intention tremor (also Incontinence and Internuclear ophthalmoplegia)
  • Nystagmus (involuntary eye movement)
45
Q
  • Progressive, Debilitating encephalitis – Leads to Death
  • Slowly progressive
  • Persistent infection of the Brain by Measles virus (Paramyxoviridae Morbillivirus – Rubeola, Togaviridae Rubivirus – German Measles - Rubella)
  • Infection occurs in Infancy – Neurologic signs arise years later (during childhood)
  • Viral inclusions w/in Neurons (gray matter) and Oligodendrocytes (white matter)
A

Subacute Sclerosisng Panencephalitis

46
Q
  • JC virus infection of Oligodendrocytes (white matter) at multiple foci (Papovaviridae Polyomavirus)
  • Immunosuppression (AIDS or Leukemia) leads to reactivation of the Latent virus
  • Demyelination of CNS due to destruction of Oligodendrocytes
  • Presents w/ Rapidly progressive neurologic signs (visual loss, Weakness, Dementia) which leads to Death
A

Progressive Multifocal Leukoencephalopathy

47
Q
  • Focal demyelination of the Pons (Anterior Brain stem)
  • Due to rapid intravenous correction of Hyponatremia (alcoholic)
  • Severly malnourished pts. (Liver disease)
  • Presents as Acute bilateral paralysis “Locked-In” Syndrome
  • Loss of voluntary movement from head to toe, but can still move eyes
A

Central Pontine Myelinolysis

48
Q
  • Degenerative disease of Cortex, ↓ ACh
  • Widespread cortical atrophy
  • Narrowing Gyri and Widening Sulci
  • Slow-onset memory loss and Progressive disorientation
  • Loss of learned Motor skills and Language
  • Changes in Behaviour and Personality
  • Patients become Mute and Bedridden
  • Infection is common cause of Death
  • Risk increases w/ Age
  • E4 allele of Apolipoprotein E (ApoE4) a/w increased Risk on Chrom 19, ApoE2 on Chr. 19 is protective
  • E2 allele w/ decreased Risk
  • Familial cases a/w Presenilin 1 (Chrom 14) and Presenilin 2 (Chrom 1) mutations
  • A/w Down syndrome by age 40 due to p-APP on Chrom 21
  • Cerebral atrophy, Neuritic ‘Senile’ plaques, AB amyloid (APP) chromosome 21
  • Neurofibrillary tangles – aggregates of fibers Hyperphosphorylated Tau Protein
  • Loss of Cholinergic neurons in the Nucleus Basalis of Meynert
  • Senile plaques: extracellular β-amyloid core; may cause amyloid angiography → Intracranial hemorrhage
  • αβ-amyloid syn. by cleaving the precursor APP
A

Alzheimer Disease (AD)

49
Q
  • Consequene of Moderate Global Ischemia
  • Multifocal infarction and Injury due to Hypertension, Atherosclerosis, or Vasculitis
  • 2nd most common cause of dementia
A

Vascular Dementia

50
Q
  • Frontotemporal atrophy
  • Dementia, Aphasia, Parkinsonian aspects, Change in personality
  • Degenerative disease of the Frontal and Temporal Cortex
  • Spares the Parietal and Occipital lobes and Posterior 2/3 of Superior temporal gyrus
  • Round aggregates of Tau protein (Pick bodies) in neurons of the Cortex
  • Behavioral and Language symptoms aries early; progress to dementia
A

Pick Disease

(frontotemporal dementia)

51
Q
  • Degenerative loss of Dopaminergic neurons (Dopamine-secreting) pigmented neurons in the Substantia nigra of the Basal ganglia (Midbrain)
  • Nigrostriatal pathway of Basal ganglia uses Dopamine to initiate movement
  • α-synuclein, parkin, DJ-1, and PINK1 genes and proteins
  • Aging, rare cases a/w MPTP exposure
  • “TRAPS” – Tremor, Rigidity, Akinesia/bradykinesia (exp.less face), Postural instability, Shuffling gait
  • Pigmented neurons in the Substantia nigra and Round, Eosinophilic inclusions of alpha-synucleain (Lewy bodies – Hallucinations)
  • ‘Pill-rolling’ tremor
A

Parkinson Disease

52
Q
  • Degeneration of GABAergic neurons in the Caudate nucleus of the Basal ganglia – Inhibitory neural transmitter
  • Loss of Spiny striated neurons that normally dampen motor activity
  • Autosomal Dominant disorder (Chromsome 4p16)
  • Trinucleotide repeat (CAG) in huntingtin gene (40 - 55, 70+), Normal (6 - 34 repeats)
  • Repeats during Spermatogenesis leads to Anticipation
  • Chorea (snake-like) movements progress to Dementia and Depression
  • ~40 years old
  • Suicide is common cause of death
A

Huntington Disease

53
Q
  • Increased CSF – Dilated Ventricles
  • Dementia in Adults (Idopathic)
  • Triad – Urinary incontinence, Gait instability, Dementia (“Wet, Wobbly, Wacky”)
  • Lumbar puncture improves symptoms
  • Tx: Ventriculoperitoneal shunting
A

Normal Pressure Hydrocephalus

54
Q
  • Degenerative Prion protein disease
  • PrPC -> PrPSC (Beta pleated sheet)
  • Sporadic conversion
  • Inherited Familial forms
  • Transmitted
  • Pathologic protein not degradable
  • Intracellular vacuoles dmgs. Neurons and Glial cells
A

Spongiform Encephalopathy

55
Q
  • Rapidly progressive Dementia (weeks to months) w/ Myoclonus (“startle myoclonus”)
  • Most common spongiform encephalopathy
  • Sporadis, rarely arises due to exposure
  • Rapidly progressive Dementia a/w Ataxia (cerebellar involvement) and Startle myoclonus (involuntary contractile w/ sensation)
  • Hallucinations followed by Parkinsonian features
  • Sharp waves seen on EEG
  • Death w/in 1 year
A

Creutzfeldt-Jakob disease (CJD)

56
Q
  • An inherited form of prion disease characterized by:
  • Severe Insomnia
  • Exaggerated Startle Response
A

Familial Fatal Insomnia

57
Q
  • Malignant, high-grade Tumor of Astocytes
  • Most common CNS tumor in Adults
  • Cerebral Hemisphere, Crosses the Corpus callosum
  • “Butterfly” lesion
  • Regions of necrosis surrounded by tumor cells ‘pseudopalisading’ and Endothelial cell proliferation
  • Pleomorphic Tumor cells (astrocytes) are GFAP positive
  • Poor prognosis (~1 year survival)
A

Glioblastoma Multiforme (GBM)

58
Q
  • Bening 1• tumor of Arachnoid cells, Brain Tumor
  • Most common benign CNS tumor in adults
  • More commonly seen in Women (presents w/ Estrogen receptors), and rare in children
  • Seizures; tumor compresses but DOES NOT invade the Cortex
  • Convexities of Hemispheres, near Brain surface
  • May have Dural attachment “Dural Tail”
  • Round mass attached to the Dura on imaging -> presses on the Cortex -> causes seizures
  • ‘Whorled pattern’ w/ Psammoma bodies on histology w/ Pink cytoplasm
A

Meningioma

59
Q
  • Bening tumor of Schwann cells (PNS)
  • Involves Cranial or Spinal nerves; w/in the Cranium
  • Most commonly seen in CN VIII at the Cerebellopontine angle
  • Loss of hearing and Tinnitus – ‘ringing in the ears’ -> Acoustic schwannoma (aka acoustic neuroma)
  • Tumor cells are S-100 positive
  • Multiple pigminted skin lesions and Optic nerve glioma a/w Neurofibromatosis Type 1
  • Bilat. Tumor cells seen in Neurofibromatosis Type 2 (NF-2)
  • Tx: Stereotactic radiosurgery
A

Schwannoma

60
Q
  • Malignant tumor of Oligodendrocytes
  • Imaging reveals a Calcified tumor in the White matter
  • Usually involving the Frontal lobe
  • May present w/ Seizures
  • ‘Chicken-wire’ capillary pattern
  • ‘Fried-egg’ apperance of cells on biopsy - round nuclei w/ clear cytoplasm
A

Oligodendroglioma

61
Q
  • **Benign tumor of Astrocytes, **good prognosis
  • Most common CNS tumor in Children
  • Usually arises in the Cerebellum (Posterior Fossa)
  • Cystic lesion w/ Mural nodule on imaging, **Well circumscribed **(Cystic + solid (gross))
  • GFAP positive
  • Rosenthal fibers (thick corkscrew Eosinophilic processes of Astrocytes) on Biopsy
A

Pilocytic Astrocytoma

62
Q
  • Malignant tumor derived from the Granular cells of the Cerebellum midline (Neuroectoderm)
  • Usually arises in Children
  • Small, Round Blue cells; Homer-Wright rosettes may be present: small cells wrap around neurotic proccess
  • Tumor grows Rapidly and spreads via CSF
  • Metastasis to the Cauda equina is termed ‘Drop metastasis’ -> Spinal cord
  • Can compress 4th ventricle -> Hydrocephalus
  • Poor prognosis
A

Medulloblastoma

63
Q
  • Malignant tumor of Ependymal cells
  • Usually seen in Children
  • Most commonly arises in the 4th Ventricle
  • May present w/ Hydrocephalus -> beneath the Tentorium – blocking CSF flow in CNS
  • Perivascular Pseudorosettes are characteristic finding on biopsy
  • Calcification is common (Tooth enamel-like)
A

Ependymoma

64
Q
  • Benign Childhood tumor arises from Epithelial remnants of Rathke’s pouch (Surface ectoderm Anterior pituitary)
  • Supratentorial tumor mass in Children and Young Adult
  • Compress the Optic chiasm -> Bitemporal hemianopsia (a/w tumors of pituitary)
  • ‘Tooth-like’ Calcifications
  • Tends to recur after Resection
A

Craniopharyngioma

65
Q
  • Initially dementia and Visual hallucinations followed by Parkinsonian features
  • Progressive Cognitive decline similar to Alzheimer disease
  • α-synuclein defect
A

Lewy Body Dementia

66
Q
  • Most common varieant of Guillain-Barre syndrome
  • Autoimmune against Schwann cells -> Inflammation and Demyelination of PNS and motor fibers
  • Ascending muscle weakness / paralysis beginning in the lower extremities
  • Facial paralyis (50%)
  • Increased CSF protein w/ normal cell count (albuminocytologic dissociation)
  • Increased protein -> Papilledema
  • A/w Campylobacter jejuni and CMV -> autoimmune attack of peripheral myelin due to molecular mimicry inoculations, and stress
  • No definitive link to pathogens
A

Acute Inflammatory Demyelinating Polyradiculopathy

67
Q
  • Multifocal perivenular Inflammation and Demyelination after infection (commonly Measles or VZV) or certain vaccines (e.g. Rabies, Smallpox)
A

Acute Disseminated (Post-infectious) Encephalomyelitis

68
Q
  • a.k.a Hereditary Motor and Sensory neuropathy (HMSN)
  • Group of progressive hereditary nerve disorders related to the defective production of Proteins involved in the Structure and Function of Peripheral nerves of the Myelin Sheath
  • Typically Autosomal dominant pattern
  • A/w Scoliosis and Foot deformities (High and Flat arches)
A

Charcot-Marie-Tooth Disease

69
Q
  • Affect 1 area of the Brain
  • Most commonly originate in Medial Temporal Lobe
  • Often preceded by Seizure aura
    • Simple, Partial - (consciousness intact) - motor sensory, autonomic, psychic
    • Complex, Partial - (impaired consciousness)
A

Partial (focal) Seizures

70
Q
  • Absence (petit mal) - 3 Hz, no postictal confusion, blank stare
  • Myoclonic - quick, repetitive jerks
  • Tonic-clonic - (grand mal) - alternating stiffening and movement
  • Tonic - stiffening
  • Atonic - “drop” seizures (falls to floor); commonly mistaken for fainting
A

Generalized Seizures

71
Q
  • Most often composed of Stromal cells in small blood vessels w/in Cerebellum, Brain Stem, and Spinal cord
  • A/w von Hippel-Lindau Syndrome when found w/ Retinal agiomas
  • Due to ectopic production of Erythropoietin can lead to -> A/w 2• Polycythemia (paraneoplastic syndrome = increased RBC count)
  • Closely arranged
  • Thin walled capillariey w/ minimal Paranchyma composed of Endothelial cells, Pericytes, and Stromal cells
A

Hemangioblastoma

72
Q
  • Most commonly Prolactinoma
  • Bitemporal Hemianopia (decreased vision or blindness), Vertigo, Nausea, Vomiting
  • Amenorrhea, Galactorrhea (Increased milk production - women)
  • Sexual dysfunction, Hypogonadism, Gynocomastia (men)
  • Shows normal visual field above, patient’s perspective below) due to Pressure on Optic Chiasm
  • Hyper- or Hypo-Pituitarism are sequelae
A

Pituitary Adenoma

73
Q
  • Vitamin B1 (Thiamine) deficiency
  • Uncommon in individuals w/ varied diet
  • A/w Chronic alcoholism
  • Capillary proliferation, Hemorrhage, Necrosis, and Hemosiderin deposition are often found in the Mammillary bodies and the Periaqueductal gray matter
  • Results in Paralysis of Extraocular muscles
A

Wernicke-Korsakoff disease

74
Q
  • Diverticulum of malformed brain tissue extending through a defect in the Cranium
  • Most often occurs in the Posterior Fossa
  • Comparable extensions of brain occur through the Cribiform plate in the Anterior Fossa (“sometimes misleadingly referred to as a “Nasal Glioma”)
A

Encephalocele

75
Q
  • Abnormal generation and migration of Neurons results in malformations of the Forebrain that may be focal or involve entire structures
  • Small Head Circumferance
  • A/w Chromosome abnormalities, Fetal Alcohol syndrome, HIV-1 infection in utero
  • Reduction in the number of neurons that reach the Neocortex leads to a Simplification of the Gyral Folding
A

Microcephaly

76
Q
  • Bilateral optic neuritis and Spinal cord demyelination
  • Anti-bodies (Ab) against Aquaporin-4, the major protein in Astrocytes and areas of demyelination
  • Loss of Aquaporin-4
  • Ab injure through Complement-dependent mechanisms
  • White cells are common in CSF (including Neutrophils)
  • Vascular deposition of Immunoglobulin and Complement
  • Tx: reduce Ab burden, Plasmapheresis, Depletion of B cells w/ anti-CD20 Ab
A

Neuromyelitis Optica

77
Q
  • gene 178 of PrPc​
  • gene 129 of PrPc
  • homozygotes Met/Met at gene 129 of PrPc
A

Prion Proteins

  • gene 178 of PrPc = Fatal Familial Insomnia (Asp) –> results in encoding Metionine at 129
  • gene 129 of PrPc = Famiial Forms (Met or Val)
  • Homozygous Met/Met = Creuzfeldt-Jakob Varient
78
Q
  • Degeneration of Frontal and/or Temporal Lobes
  • Alterations in personality, behavior and language (aphasias) precede memory loss
  • Global dementia and Early onset Dementia
  • A/w Cellular Inclusions of FTLD-tau and w/ TDP43 (FTLD-TDP) found in the nucleus (phosphorylated and ubiquitinated)
A

Frontaltemporal Lobe Dementia

79
Q
  • Accumulation of Storage Material w/in Neurons –> followed by death of the Neurons
  • Seizures, Generalized loss of Neurologic function
A

Neuronal Storage Disease

80
Q
  • Gm2 gangliosidoses a lysosomal storage diseases caused by an inability to Catabolize Gm2 gangliosides
  • Accumulates in the Heart, Liver, Spleen, Nervous System
  • Central and Autonomic nervous systems and Retina dominate the clinical picture
  • Cherry-red spot thus appears in the Macula
  • Cytoplasmic inclusions w/ Onion-skin layers
  • Mutations in the α-subunit locus on chromosome 15 causes a severe deficiency of Hexosaminidase A
  • Ashkenazic Jews (1:30)
A

Tay Sachs Disease

81
Q
  • Rare disorder in which Lipid pigments accumulate in Neurons
  • Neuronal dysfunction leads to a combination of Blindness, Cognitive, and Motor Deterioration and Seizures.
A

Ceroid Lipofuscinosis

82
Q
  • Impared oxygen-carrying capacity of Hemoglobin
  • CO interactions w/ Cytochrome C oxidase inhibiting electron transport in mitochondria
  • Selective injury to layers III and V of Cerebral cortex, Sommer center of the Hippocampus, and Purkinje cells
  • Bilateral necrosis of the Globus Pallidus
  • Demyelination of White matter tracts as Late event
A

Carbon Monoxide Poisoning (CO)

83
Q
  • Preferentially affects the Retina; degeneration of Retinal Ganglion cells causes Blindness
  • Bilateral necrosis of the Putamen and Focal White-matter necrosis also occur when the exposure is severe
  • Disruption of Oxidative phosphorylation and Nonenzymatic protein modification
  • Ingestion of illicit liquor (moonshine)
A

Methanol Poisoning

84
Q
  • Degeneration of both Ascending and Descending Spinal tracts
  • Defect in Myelin formation
  • Bilateral symmetrical Numbness, Tingling, and slight Ataxia in the Lower Extremities
  • Complete Paraplegia may occur
  • Starts in the Mid-Thoracic level
  • Swelling in Myelin layers produces vacuoles, with eventual Axon degeneration
A

Vitamin B12 Defeciency

85
Q
  • Wernicke Encephalopathy - acute appearance of a combination of psychotic symptoms and Opthalmoplegia
  • Acute symptoms are reversible when treated w/ Thiamine –> if untreated results in Korsakoff syndrome
  • Disturbances in Short-term memory (MedialDorsal Lesions in Thalamus) and Confabulation
  • Common in Chronic Alcoholism
  • Focal hemorhage and necrosis in the Mamillary bodies and the walls of the 3rd and 4th Ventricles
  • Development of Cystic space w/ Hemosiderin-laden Macrophages
A

Thiamine Deficiency

86
Q

Aβ Protein

A

Alzheimer Disease

87
Q

Tau Protein

A
  • Alzheimer Disease
  • Frontotemporal lobar degeneration
  • Parkinson Disease w/ LRRX2 mutations
  • Progressive supranuclear palsy
  • Corticobasal degeneration
88
Q

TDP-43 Protein

A
  • Frontotemporal lobar degeneration
  • Amyotrophic lateral sclerosis (ALS)
89
Q

FUS protein

A
  • Frontotemporal lobar degeneration
  • Amyotrophic lateral sclerosis
90
Q

α - synuclein Protein

A
  • Parkinson Disease
  • Multiple system atrophy
91
Q

Polyglutamine Aggregates

A
  • Huntington Disease
  • Spinocerebellar Ataxia (some forms)
  • Spinal Bulbar Muscular Atrophy

Pathology (36 decks)

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