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Neurology > Neuro Pathology > Flashcards

Neuro Pathology Flashcards

1
Q

What is Dementia?

A

Dec in cogntive ability, memory or function w/ intact consciousness

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2
Q

Who is at risk of developing Alzheimers dz?

A

Elderly & Down Synd

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3
Q

Which proteins are altered in the familial form of Alzheimer’s dz?

A
  • Eary onset: APP (21), presenilin-1 (14), Presenlin-2 (1)
  • Late onset: ApoE4 (19)
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4
Q

Which protein is protective against Alzheimers?

A

ApoE2

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5
Q

What are the histo findings w/ Alzheimers?

A
  • Widespread cortical atrophy
  • Dec ACh
  • Senile plaques
  • Neurofibrillary tangles
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6
Q

What are Senile plaques?

A

Extracellular ß-amyloid core

(synthesized by cleaving amyloid precursor protein)

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7
Q

What can Senile plaques cause?

A

May cause amyloid agniopathy→ intracranial hemorrhage

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8
Q

What is Pick’s dz (frontotemporal dementia)?

A
  • Dementia
  • Aphasia
  • Parkinsonian aspects
  • Change in personality
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9
Q

What is spared in Pick’s dz?

A

Parietal lobe & posterior 2/3 of superior temporal gyrus

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10
Q

What are the histo findings in Pick’s dz?

A
  • Pick bodies: spherical tau protein aggregates
  • Frontotemporal atrophy
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11
Q

What is Lewy Body dementia?

A

Parkinsonism w/ demntia & hallucinations

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12
Q

What are the histo findings in Lewy body dementia?

A

Alpha-synuclein defect

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13
Q

What is Creutzfeldt-Jakob dz (CJD)?

A

Rapidly progressive (wk to mo) dementia w/ myoclonus (“startle myoclonus”)

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14
Q

What are the histo findings of CJD?

A
  • Spongiform cortex
  • Prions (ß-pleated sheet resistant to proteases)
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15
Q

What are some causes of dementia?

A
  • Multi-infarct (2nd MCC of dementia in elderly behind alzheimers)
  • Syphilis
  • HIV
  • Vit B1, 3 or 12 def
  • Wilson’s dz
  • NPH (normal press hydrocephalus)
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16
Q

What is Multiple sclerosis?

A

Autoimmune inflam & demyelination of CNS

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17
Q

How do pts w/ MS present?

A
  • Optic neuritis (sudden loss of vision)
  • MLF synd (internuclear opthalmoplgeia)
  • Hemiparesis
  • Hemisensory sx
  • Bladder/bowel incontinence
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18
Q

Who gets MS?

A

Women in 20-30’s

MC in whites

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19
Q

What are the clinical findings in MS?

A
  • Inc protein (IgG) in CSF
  • Oligoclonal bands are dx
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20
Q

What is the gold standard for MS imaging?

A

MRI

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21
Q

What are periventricular plaques?

A

Areas of oligodendrocyte loss & reactive gliosis w/ destruction of axons

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22
Q

What is the tx of MS?

A
  • ß-interferon
  • Immunosuppression
  • Natalizumab
  • Sx tx for neurogenic bladder, spacitity & pain
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23
Q

What is Charcot’s classic triad of MS?

A
  • Scanning speech
  • Intention tremor, incontinence & internuclear opthalmoplegia
  • Nystagmus
  • “SIN”
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24
Q

What is Acute inflam demyelinating polyradiculopathy?

A

Autoimmune cond that destroys Schwann cells→ inflam & demyelination of peripheral nerves & motor fibers

MC variant of Guillian-Barré synd

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25
Q

What does acute infalm demyelinating polyradicuopathy result in?

A

Symmetric ascending muscle weakness/paralysis beginning in LE.

Facial paralysis in 50%

Autonomic function may be severely affected

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26
Q

What is the outcome of pts w/ acute inflam demyelinating polyradiculopathy?

A

Almost all pts survive

Majority recover completely after wks to mo

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27
Q

What are the clinical findings in acute inflam demyelinating polyradiculopathy?

A
  • Inc CSF protein w/ normal cell count
  • Inc protein→ papilledema
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28
Q

What is acute inflam demyelinating polyradiculopathy assoc w/?

A

Infections: Campylobacter jejuni & CMV→ autoimmune attack of peripheral myelin

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29
Q

What is the tx for acute inflam demyelinating polyradiculopathy?

A
  • Resp support critical
  • Plasmapheresis
  • IV immune globulins
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30
Q

What is Progressive multifocal leukoencephalopathy (PML)?

A

Demyelination of CNS d/t destruction of oligodendrocytes

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31
Q

What is PML assoc w/?

A

JC virus

Seen in AIDs pts

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32
Q

What is the outcome in PML pts?

A

Rapidly progressive, usually fatal

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33
Q

What is Acute disseminated (postinfectious) encephalomyelitis?

A

Multifocal perivenular inflam & demyelination after infection (measles or VZV) or vaccinations (rabies, small pox)

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34
Q

What is Metachromatic leukodystrophy?

A

AR lysosomal storage dz, MC d/t arylsulfatase A def

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35
Q

What does build up of sulfatides in Metachromic leukodystrophy lead to?

A

Impaired prodution of myelin sheath

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36
Q

What is Charcot-Marie-Tooth dz also known as?

A

Hereditary motor & sensory neuropathy (HMSN)

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37
Q

What is Charcot-Marie-Tooth dz?

A

Group of hereditary nerve disorders related to the defective prod of proteins involved in the structure & funct of peripheral nerves or the myelin sheath

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38
Q

What is Krabbe’s dz?

A

AR lysosomal storage dz d/t def of galactocerebrosidase

Build up of galactocerebroside→ destroys myelin sheath

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39
Q

What are seizures characterized by?

A

Synchronized, high-freq neuronal firing

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40
Q

What are Partial (focal) seizures?

A
  • Affect 1 area of brain
  • Simple partial
  • Complex partial
  • Epilepsy
  • Status epilepticus
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41
Q

Where do partial seizures orginiate?

A

MC in medial temporal lobe

Proceded by seizure aura

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42
Q

What is a simple partial seizure?

A

Conciousness intact

(motor, sensory, autonomic, psychic)

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43
Q

What is a complex seizure?

A

Impaired consciousness

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44
Q

What is Epilepsy?

A

Disorder of recurrent seizures

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45
Q

What is Status epilepticus?

A

Continuous seizure for >30min or recurrent seizures w/o regaining consciousness b/w seizures for >30 min

Medical emergency

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46
Q

What are the causes of seizures in children?

A
  • Genetic
  • Infection (febrile)
  • Trauma
  • Congenital
  • Metabolic
47
Q

What are the causes of seizures in adults?

A
  • Tumors
  • Trauma
  • Stroke
  • Infection
48
Q

What are the causes of seizures in elderly?

A
  • Stroke
  • Tumor
  • Trauma
  • Metabolic
  • Infection
49
Q

What are the types of Generalized seizures?

A
  • Absence (petit mal)
  • Myoclonic
  • Tonic-clonic (grand mal)
  • Tonic
  • Atonic
50
Q

What is an Absence (petit mal) seizure?

A

3 Hz, no postictal confusion, blank stare

51
Q

What is a Myoclonic seizure?

A

Quick, repetitive jerks

52
Q

What are Tonic-clonic (grand mal) seizures?

A

Alternating stiffening & movement

53
Q

What is a tonic seizure?

A

Stiffening

54
Q

What is an Atonic seizure?

A

“Drop” seizures (falls to floor)

commonly mistaken for fainting

55
Q

What are Cluster headaches?

A

Unilateral brief headaches w/ excrutiating periorbital pain w/ lacrimation & rhinorrhea

56
Q

What can cluster headaches induce?

A

Horner’s synd

57
Q

Who gets cluster headaches?

A

MC in males

58
Q

What is the tx for cluster headaches?

A

Inhaled O2 & sumatriptan

59
Q

What are Tension headaches?

A

Bilateral w/ steady pain

NO photophobia, phonophobia or aura

60
Q

What are Migraines?

A

Pulsating pain w/ nausea, photophobia or phonophobia

may have aura

61
Q

What is the cause of Migraines?

A

Due to irritation of CN V, meninges or BV

62
Q

What is Vertigo?

A

Sensation of spinning while stationary

63
Q

What is Peripheral Vertigo?

A

MC; inner ear etiology

(Semicurcular canal debris, vestibular nerve inf, Meniere’s dz)

64
Q

What is Central vertigo?

A

Brain stem or cerebellar lesion

65
Q

What are the findings in Central vertigo?

A
  • Directional change of nystagmus
  • Skew deviation
  • Diplopia
  • Dysmetria
66
Q

What happens in positional testing of Peripheral vertigo?

A

Delayed horizontal nystagmus

67
Q

What happens in positional testing of Central vertigo?

A

Immediate nystagmus in any direction; may change direction

68
Q

What are the Neurocutaneous disorders?

A
  • Sturge-Weber synd
  • Tuberous sclerosis
  • Neurofibramtosis type I (von Recklinhausen’s dz)
  • von Hippel-Lindau dz
69
Q

What is Sturge-Weber synd?

A

Congenital disorder w/ port-wine stains, typically in the V1 opthalmic distribution, ipsi leptomenigeal angiomas & pheochromocytomas

70
Q

What can Sturge-Weber synd cause?

A
  • Glaucoma
  • Seizures
  • Hemiparesis
  • Mental retardation
71
Q

What are the HAMARTOMAS assoc w/ Tuberous sclerosis?

A
  • Hamartomas in CNS & skin
  • Adenoma sebaceum
  • Mitral regurg
  • Ash-leaf spots
  • cardiac Rhabdomyoma
  • (Tuberous sclerosis)
  • Autosomal dOminant
  • Mental retardation
  • renal Angiomyolipoma
  • Seizures
72
Q

What is Neurofibromatosis type I (von Recklinghausen’s dz)?

A
  • Cafe-au-lait spots
  • Lisch nodules (pigmented iris hamartoma)
  • Neurofibromas in skin
  • Optic gliomas
  • Pheochromocytoma
73
Q

What is the cause of Neurofibromatosis type I?

A

AD, 100% penetrant, variable expression

Mutated NF1 gene on chr 17

74
Q

What is von Hippel-Lindaue dz?

A
  • Cavernous hemangiomas in skin, mucosa & organs
  • Bilateral renal cell CA
  • Heangioblastoma in retina, brain stem, cerebellum
  • Pheochromocytoma
75
Q

What is the cause of von-Hippel Lindau dz?

A

AD; mutated tumor suppressor VHL gene on chr 3

76
Q

Where are Glioblastoma multiformes found?

A

Found in cerebral hemispheres

Can cross corpus callosum (“ Butterfly glioma”)

77
Q

What is found on histology of a Glioblastoma multiforme?

A

“Pseudopalisading” pleomorphic tumor celss–border central areas of necrosis & hemorrhage

Stain astrocytes for GFAP

78
Q

What is the prognosis of Glioblastoma multiforme?

A

Malignant w/ <1 year life expectancy

79
Q

What is the MC primary brain tumor?

A

Glioblastoma multiforme (grade IV astrocytoma)

80
Q

What is the 2nd MC primary brain tumor?

A

Meningioma

81
Q

Where do Meningiomas occur?

A

Convexities of hemispheres (near surface of brain) & parasagittal region

Adjacent to brain attached to dura

82
Q

Where do Meningiomas arise from?

A

Arachnoid cells, are extra-axial (external to brain parenchyma( & may have a dural attachment (“trail”)

83
Q

What are the clinical features of a meningioma?

A

Typically benign & resectable

Often asx; may present w/ seizures or focal signs

84
Q

What is found on histo of a Meningioma?

A

Spindle cells concentrically arranged in a whorled pattern; psammona bodies (laminated calcifications)

85
Q

What is the 3rd MC primary brain tumor?

A

Schwannoma

86
Q

What do Schwannomas arise from?

A

Schwann cells

87
Q

Where are Schwannomas localized?

A

Usually found at cerebellopontine angle

CN VIII→ acoustic schwannoma (aka acoustic neuroma)

88
Q

What is the marker for a Schwannoma?

A

S-100 +

89
Q

What are Bilateral acoustic schwannoma assoc w/?

A

Neurofibromatosis type 2

90
Q

What are the clinical features of a Schwannoma?

A
  • Hearing loss
  • Tinnitus
  • Loss of balance
91
Q

What is the prognosis of an Oligodendroglioma?

A

Relatively rare, slow growing

92
Q

What are the histo findings of Oligodendroglioma?

A
  • Chicken-wire capillary pattern
  • Oligodendrocytes= “fried egg” cells–round nuclei w/ clear cytoplasm
  • Cells often calcified
93
Q

Where are Oligodrogliomas located?

A

Frontal lobes

94
Q

What is the MC pituitary adenoma?

A

Prolactinoma

95
Q

What are the clinical features of a Pituitary adenoma?

A

Bitemporal hemianopia & hyper or hypopituitarism

96
Q

Where are Pilocytic (low-grade) astrocytomas located?

A
  • Well Circumscribed
  • In children found in posterior fossa
  • May be Supratentorial
97
Q

What is the prognosis of Pilocytic astrocytoma?

A

Benign; good prognosis

98
Q

What is found on histo in Pilocytic astrocytoma?

A

GFAP +

Rosenthal fibers–eosinophilic, corkscrew fibers

Cystic + solid (gross)

99
Q

What is a Medulloblastoma?

A

Highly malignant cerebellar tumor

Form of primitive neuroectodermal tumor

100
Q

What is found on histo in Medulloblastoma?

A
  • Homer-Wright rosettes
  • Solid
  • Small blue cells
  • Radiosensitive
101
Q

What are the clinical features of Medulloblastoma?

A
  • Can compress 4th ventricle causing hydrocephalus
  • Can send “drop mets” to SC
102
Q

Where are Ependymomas found?

A

4th ventricle

103
Q

What can Ependymomas cause?

A

Hydrocephalus

104
Q

What is the prognosis of an Ependymoma?

A

Poor

105
Q

What will be found on histo in an Ependymoma?

A
  • Perivascular pseudorosettes
  • Rod-shaped blepharoplasts (basal ciliary bodies) found near nucleus
106
Q

Where are Hemangioblastomas found?

A

Cerebellar

107
Q

What are Hemangioblastomas assoc w/?

A

vvon Hippel-Lindaue synd when found w/ retinal angiomas

108
Q

What can Hemangioblastomas produce?

A

EPO→ 2° polycythemia

109
Q

What is found on histo in Hemangioblastoma?

A

Foamy cells & high vascularity are characteristic

110
Q

What is a Craniopharyngioma?

A

Benign childhood tumor, confused w/ a pituitary adenoma (may cause bitemporal heminopia)

111
Q

What is the MC childhood supratentorial tumor?

A

Craniopharyngioma

112
Q

What are Craniopharyngiomas derived from?

A

Remnants of Rathke’s pouch

113
Q

What is found on histo in Craniopharyngioma?

A

Calcification is common (enamel-like)

Neurology (9 decks)

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