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Neuro Pathology Flashcards

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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
What does acute infalm demyelinating polyradicuopathy result in?
Symmetric ascending muscle weakness/paralysis beginning in LE. Facial paralysis in 50% Autonomic function may be severely affected
26
What is the outcome of pts w/ acute inflam demyelinating polyradiculopathy?
Almost all pts survive Majority recover completely after wks to mo
27
What are the clinical findings in acute inflam demyelinating polyradiculopathy?
* Inc CSF protein w/ normal cell count * Inc protein→ papilledema
28
What is acute inflam demyelinating polyradiculopathy assoc w/?
Infections: Campylobacter jejuni & CMV→ autoimmune attack of peripheral myelin
29
What is the tx for acute inflam demyelinating polyradiculopathy?
* Resp support critical * Plasmapheresis * IV immune globulins
30
What is Progressive multifocal leukoencephalopathy (PML)?
Demyelination of CNS d/t destruction of oligodendrocytes
31
What is PML assoc w/?
JC virus Seen in AIDs pts
32
What is the outcome in PML pts?
Rapidly progressive, usually fatal
33
What is Acute disseminated (postinfectious) encephalomyelitis?
Multifocal perivenular inflam & demyelination after infection (measles or VZV) or vaccinations (rabies, small pox)
34
What is Metachromatic leukodystrophy?
AR lysosomal storage dz, MC d/t arylsulfatase A def
35
What does build up of sulfatides in Metachromic leukodystrophy lead to?
Impaired prodution of myelin sheath
36
What is Charcot-Marie-Tooth dz also known as?
Hereditary motor & sensory neuropathy (HMSN)
37
What is Charcot-Marie-Tooth dz?
Group of hereditary nerve disorders related to the defective prod of proteins involved in the structure & funct of peripheral nerves or the myelin sheath
38
What is Krabbe's dz?
AR lysosomal storage dz d/t def of galactocerebrosidase Build up of galactocerebroside→ destroys myelin sheath
39
What are seizures characterized by?
Synchronized, high-freq neuronal firing
40
What are Partial (focal) seizures?
* Affect 1 area of brain * Simple partial * Complex partial * Epilepsy * Status epilepticus
41
Where do partial seizures orginiate?
MC in medial temporal lobe Proceded by seizure aura
42
What is a simple partial seizure?
Conciousness intact | (motor, sensory, autonomic, psychic)
43
What is a complex seizure?
Impaired consciousness
44
What is Epilepsy?
Disorder of recurrent seizures
45
What is Status epilepticus?
Continuous seizure for \>30min or recurrent seizures w/o regaining consciousness b/w seizures for \>30 min Medical emergency
46
What are the causes of seizures in children?
* Genetic * Infection (febrile) * Trauma * Congenital * Metabolic
47
What are the causes of seizures in adults?
* Tumors * Trauma * Stroke * Infection
48
What are the causes of seizures in elderly?
* Stroke * Tumor * Trauma * Metabolic * Infection
49
What are the types of Generalized seizures?
* Absence (petit mal) * Myoclonic * Tonic-clonic (grand mal) * Tonic * Atonic
50
What is an Absence (petit mal) seizure?
3 Hz, no postictal confusion, blank stare
51
What is a Myoclonic seizure?
Quick, repetitive jerks
52
What are Tonic-clonic (grand mal) seizures?
Alternating stiffening & movement
53
What is a tonic seizure?
Stiffening
54
What is an Atonic seizure?
"Drop" seizures (falls to floor) commonly mistaken for fainting
55
What are Cluster headaches?
Unilateral brief headaches w/ excrutiating periorbital pain w/ lacrimation & rhinorrhea
56
What can cluster headaches induce?
Horner's synd
57
Who gets cluster headaches?
MC in males
58
What is the tx for cluster headaches?
Inhaled O2 & sumatriptan
59
What are Tension headaches?
Bilateral w/ steady pain NO photophobia, phonophobia or aura
60
What are Migraines?
Pulsating pain w/ nausea, photophobia or phonophobia may have aura
61
What is the cause of Migraines?
Due to irritation of CN V, meninges or BV
62
What is Vertigo?
Sensation of spinning while stationary
63
What is Peripheral Vertigo?
MC; inner ear etiology (Semicurcular canal debris, vestibular nerve inf, Meniere's dz)
64
What is Central vertigo?
Brain stem or cerebellar lesion
65
What are the findings in Central vertigo?
* Directional change of nystagmus * Skew deviation * Diplopia * Dysmetria
66
What happens in positional testing of Peripheral vertigo?
Delayed horizontal nystagmus
67
What happens in positional testing of Central vertigo?
Immediate nystagmus in any direction; may change direction
68
What are the Neurocutaneous disorders?
* Sturge-Weber synd * Tuberous sclerosis * Neurofibramtosis type I (von Recklinhausen's dz) * von Hippel-Lindau dz
69
What is Sturge-Weber synd?
Congenital disorder w/ port-wine stains, typically in the V1 opthalmic distribution, ipsi leptomenigeal angiomas & pheochromocytomas
70
What can Sturge-Weber synd cause?
* Glaucoma * Seizures * Hemiparesis * Mental retardation
71
What are the **HAMARTOMAS** assoc w/ Tuberous sclerosis?
* **H**amartomas in CNS & skin * **A**denoma sebaceum * **M**itral regurg * **A**sh-leaf spots * cardiac **R**habdomyoma * (**T**uberous sclerosis) * Autosomal d**O**minant * **M**ental retardation * renal **A**ngiomyolipoma * **S**eizures
72
What is Neurofibromatosis type I (von Recklinghausen's dz)?
* Cafe-au-lait spots * Lisch nodules (pigmented iris hamartoma) * Neurofibromas in skin * Optic gliomas * Pheochromocytoma
73
What is the cause of Neurofibromatosis type I?
AD, 100% penetrant, variable expression Mutated NF1 gene on chr 17
74
What is von Hippel-Lindaue dz?
* Cavernous hemangiomas in skin, mucosa & organs * Bilateral renal cell CA * Heangioblastoma in retina, brain stem, cerebellum * Pheochromocytoma
75
What is the cause of von-Hippel Lindau dz?
AD; mutated tumor suppressor VHL gene on chr 3
76
Where are Glioblastoma multiformes found?
Found in cerebral hemispheres Can cross corpus callosum (" Butterfly glioma")
77
What is found on histology of a Glioblastoma multiforme?
"Pseudopalisading" pleomorphic tumor celss--border central areas of necrosis & hemorrhage Stain astrocytes for GFAP
78
What is the prognosis of Glioblastoma multiforme?
Malignant w/ \<1 year life expectancy
79
What is the MC primary brain tumor?
Glioblastoma multiforme (grade IV astrocytoma)
80
What is the 2nd MC primary brain tumor?
Meningioma
81
Where do Meningiomas occur?
Convexities of hemispheres (near surface of brain) & parasagittal region Adjacent to brain attached to dura
82
Where do Meningiomas arise from?
Arachnoid cells, are extra-axial (external to brain parenchyma( & may have a dural attachment ("trail")
83
What are the clinical features of a meningioma?
Typically benign & resectable Often asx; may present w/ seizures or focal signs
84
What is found on histo of a Meningioma?
Spindle cells concentrically arranged in a whorled pattern; psammona bodies (laminated calcifications)
85
What is the 3rd MC primary brain tumor?
Schwannoma
86
What do Schwannomas arise from?
Schwann cells
87
Where are Schwannomas localized?
Usually found at cerebellopontine angle CN VIII→ acoustic schwannoma (aka acoustic neuroma)
88
What is the marker for a Schwannoma?
S-100 +
89
What are Bilateral acoustic schwannoma assoc w/?
Neurofibromatosis type 2
90
What are the clinical features of a Schwannoma?
* Hearing loss * Tinnitus * Loss of balance
91
What is the prognosis of an Oligodendroglioma?
Relatively rare, slow growing
92
What are the histo findings of Oligodendroglioma?
* Chicken-wire capillary pattern * Oligodendrocytes= "fried egg" cells--round nuclei w/ clear cytoplasm * Cells often calcified
93
Where are Oligodrogliomas located?
Frontal lobes
94
What is the MC pituitary adenoma?
Prolactinoma
95
What are the clinical features of a Pituitary adenoma?
Bitemporal hemianopia & hyper or hypopituitarism
96
Where are Pilocytic (low-grade) astrocytomas located?
* Well Circumscribed * In children found in posterior fossa * May be Supratentorial
97
What is the prognosis of Pilocytic astrocytoma?
Benign; good prognosis
98
What is found on histo in Pilocytic astrocytoma?
GFAP + Rosenthal fibers--eosinophilic, corkscrew fibers Cystic + solid (gross)
99
What is a Medulloblastoma?
Highly malignant cerebellar tumor Form of primitive neuroectodermal tumor
100
What is found on histo in Medulloblastoma?
* Homer-Wright rosettes * Solid * Small blue cells * Radiosensitive
101
What are the clinical features of Medulloblastoma?
* Can compress 4th ventricle causing hydrocephalus * Can send "drop mets" to SC
102
Where are Ependymomas found?
4th ventricle
103
What can Ependymomas cause?
Hydrocephalus
104
What is the prognosis of an Ependymoma?
Poor
105
What will be found on histo in an Ependymoma?
* Perivascular pseudorosettes * Rod-shaped blepharoplasts (basal ciliary bodies) found near nucleus
106
Where are Hemangioblastomas found?
Cerebellar
107
What are Hemangioblastomas assoc w/?
vvon Hippel-Lindaue synd when found w/ retinal angiomas
108
What can Hemangioblastomas produce?
EPO→ 2° polycythemia
109
What is found on histo in Hemangioblastoma?
Foamy cells & high vascularity are characteristic
110
What is a Craniopharyngioma?
Benign childhood tumor, confused w/ a pituitary adenoma (may cause bitemporal heminopia)
111
What is the MC childhood supratentorial tumor?
Craniopharyngioma
112
What are Craniopharyngiomas derived from?
Remnants of Rathke's pouch
113
What is found on histo in Craniopharyngioma?
Calcification is common (enamel-like)

Neurology (9 decks)

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