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Neurology > Neuro- Pathology > Flashcards

Neuro- Pathology Flashcards

1
Q

Dementia- defn

A

Decrease in cognitive ability, memory, or function- WITH INTACT CONSCIOUSNESS

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2
Q

Alzheimer dz- important associations

A

Most common cause in elderly

Down syndrome pts have higher risk

Associated with: ApoE2, ApoE4 (sporadic form)
APP, and presenilin 1 and 2 (familial forms with earlier onset)

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3
Q

Alzheimer dz- histology

A

Diffuse cortical atrophy (narrowing of gyri/ widening of sulci)

Senile plaques in gray matter- dark brown/ amyloid plaques

Neurofibrillary tangles- pink intracellular deposits of tau protein (insoluble)

In this case, pink is NOT amyloid! The amyloid is darker brown in color

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4
Q

Frontotemporal dementia (Pick dz)- key points

A

Behavior changes or aphasia

May have associated movement disorders (parkinsonism, ALS-like UMN and LMN degeneration)

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5
Q

Frototemporal dementia- histology

A

Frontal and temporal degeneration

Pick bodies (inclusions of hyperphosphorylated tau) or ubiquitinated TDP-43

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6
Q

Lewi body dementia- key points

A

Dementia and visual hallucinations FOLLOWED by parkinsonian features

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7
Q

Lewy body dementia- histology

A

Intracellular Lewy bodies (alpha synuclein deposits) in cortex

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8
Q

Vascular dementia- key points

A

Results from multiple arterial infarcts and/or chronic ischemia

Causes step-wise decline in cognitive ability and late-onset memory impairment

Common in elderly (2nd to Alzheimer)

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9
Q

Vascular dementia- histology

A

multiple cerebellar infarcts

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10
Q

CJD- key points

A

Rapidly progressive (wks to mo) dementia with myoclonus (startle)

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11
Q

CJD- histology

A

CSF + for protein 14-3-3

Spongiform cortex

Prions (beta pleated sheet is resistant to proteases)

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12
Q

Other causes of dementia

A

Syphilis, HIV, hypothyroidism, vitamin B1, B3, or B12 def, Wilson disease, normal pressure hydrocephalus

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13
Q

Osmotic demyelination syndrome (central pontine myelinolysis)- S&S

A 
Acute paralysis
Dysarthria
Dysphagia
Diplopia
Loss of consciousness
"Locked-in syndrome"- basilar artery stroke (quadriplegia with retained consciousness)
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14
Q

CPS- cause

A

Iatrogenic; rapid correction of HYPOnatremia

vs. rapid correction of HYPERnatremia causes cerebral edema/herniation

“From low to high, your pons will die… from high to low, your brain will blow”

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15
Q

Multiple sclerosis- cause

A

Autoimmune inflammation and demyelination of CNS (brain and spinal cord)

Damages oligodendrocytes of the CNS

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16
Q

MS- S&S

A

Diffuse; most often affects women in their 20s and 30s; Caucasians living further from the equator

Lower extremity weakness, difficulty controlling hand movements, hemiparesis, INO, Marcus-Gunn pupil (afferent pupillary defect), bladder and bowel dysfunction (due to effects on ANS)

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17
Q

Charcot triad of MS (SIN)

A

Scanning speech
Intention tremor, (+ Incontinence, and INO)
Nystagmus

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18
Q

Lhermitte’s sign

A

Shock through spine when bending the neck (+ for patients with MS)

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19
Q

MS- histology

A

Increased IgG level and myelin basic protein in CSF

Oligoclonal bands (immunoglobulins in CSF) are diagnostic

MRI- gold standard; shows periventricular plaques (oligodendrocyte loss and reactive gliosis- with destruction of axons)

White matter lesions separated in space and time

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20
Q

MS- Tx

A

Slow progression with dz modifying therapies (beta-interferon, glatiramer, natalizumab)

Acute flares are tx with IV steroids

Neurogenic bladder tx with catheterization, muscarinic antagonists (oxybutinin)

Spasticity (baclofen, GABAb receptor agonists)

Pain (opioids)

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21
Q

Acute inflammatory demyelinating polyradiculopathy (Guillain Barre)

A

Autoimmune condition- destroys Schwann cells (inflammation and demyelination of peripheral nerves and motor fibers)

Characterized by symmetric, ascending muscle weakness (beginning at the lower extremities)

Can see facial paralysis (in 50%)

May see autonomic dysregulation (cardiac irregularities, HTN, hypotension)

Majority survive- takes them weeks to months

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22
Q

Guillain Barre- findings

A

Increased CSF protein, with normal cell count (albuminocytologic dissociation)

May cause papilledema

Nerve conduction studies will show slowed conduction velocity, prolongation of distal latency (sensory-evoked potentials), decreased F-waves (delayed motor nerve response)

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23
Q

Guillian Barre associations

A

Campylobacter jejuni (molecular mimicry)

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24
Q

Guillian Barre- tx

A

Respiratory support– critical until recovery

Can also give plasmapheresis, IV immunoglobulin

Steroids DO NOT HELP

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25
Q

Acute disseminated (post infectious) encephalomyelytis

A

Multifocal periventricular inflammation and demyelination after infection or vaccine

Rapid change in mental status and multifocal neuro symptoms

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26
Q

Charcot Marie Tooth dz

A

Hereditary MOTOR and SENSORY neuropathy

Related to the defective production of proteins involved in the peripheral nerve or myelin sheath

AD inheritance

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27
Q

CMT dz- S&S

A

Foot deformities (pes cavus), lower extremity weakness, and sensory deficits

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28
Q

Krabbe dz

A

AR lysosomal storage dz- results from deficiency of galactocerebrosidase

Buildup of galactocerebroside and psychosis destroys myelin sheath

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29
Q

Krabbe dz

A

Signs and symptoms: peripheral neuropathy, developmental delay, optic atrophy, and globoid cells

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30
Q

Metachromic leukodystrophy

A

AR lysosomal storage dz- results from deficiency of Arylsulfatase A

Buildup of sulfates –> impaired production and destruction of myelin sheath

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31
Q

Metachromic leukodystrophy- S&S

A

Central and peripheral demyelination with ataxia and dementia

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32
Q

Progressive multifocal leukoencephalopathy

A

Demyelination of the CNS due to destruction of oligodendrocytes

Seen in 2-4% of AIDS patients (reactivation of latent JC virus)

Rapidly progressive and usually fatal

Increased risk associated with natalizumab and rituximab

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33
Q

PML- S&S

A

Problems with speech, memory, coordination; quick decline

MRI shows non-enhancing areas of demyelination

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34
Q

Adrenoleukodystrophy

A

X-linked genetic disorder- typically affects males

Disrupts metabolism of very long chain fatty acids –> builds up in NS, adrenal gland, testes

Over time, can lead to long-term coma/death and adrenal gland crisis

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35
Q

Partial (focal) seizures

A

Affects a localized/ single region of the brain (generally medial temporal lobe)

Preceded by seizure aura

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36
Q

Focal seizure: Simple partial

A

consciousness intact

Motor, sensory, autonomic, psychic

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37
Q

Focal seizure: Complex partial

A

IMPAIRED consciousness

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38
Q

Epilepsy

A

disorder of recurrent seizures

excludes febrile seizures

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39
Q

Status epilepticus

A

continuous or recurring seizure that may result in brain injury (>5 min)

40
Q

Generalized seizure

A

Diffuse

5 types: Absence, myoclonic, tonic-clonic, tonic, atonic

41
Q

Absence (petit mal)

A

3Hz, no postictal confusion, blank stare

42
Q

Myoclonic

A

quick, repetitive jerks

43
Q

Tonic-clonic (grand mal)

A

alternating stiffening and movement

44
Q

Tonic

A

stiffening

45
Q

Atonic

A

“drop” seizures (falls to the floor)

may be mistaken for fainting

46
Q

Seizure causes (by age)

A

Children: genetic, infection (febrile), trauma, congenital, metabolite accumulation

Adults: trauma, stroke, tumor, infection

Elderly: stroke, tumor, trauma, metabolite, infection

47
Q

Headache

A

Pain due to irritation of structures- such as the dura, CHs, or extra cranial structures (more common in females)

48
Q

Cluster headache- S&S

A

Unilateral, 12min-3hr, repetitive, brief headaches

Excruciating periorbital pain with lacrimation and rhinorrhea

May present with Horner syndrome

49
Q

Cluster headache- tx

A

Acute: sumatriptan, 100% O2

Chronic: verapamil

50
Q

Cluster vs. trigeminal neuralgia

A

Trigeminal neuralgia- also present with severe, repetitive, unilateral pain- but pain is distributed along CN V distribution (and typically only lasts for < 1min)

Cluster- repetitive, several, unilateral pain, but often last for 15min- 3 hrs

51
Q

Tension headache- S&S

A

Bilateral, >30 min duration (typically 4-6 hrs), steady pain, no photo or phonophobia. NO AURA

52
Q

Tension headache- tx

A

Analgesics, NSAIDs, acetaminophen

Chronic: TCADs (amitriptyline)

53
Q

Migraine headache- S&S

A

Unilateral, 4-72 hr duration, pulsating pain with nausea, photophobia or phonophobia

May have an AURA

Cause: irrigation of CN V, meninges, or blood vessels –> releases substance P, calcitonin gene-related peptide, vasoactive peptide

54
Q

Migraine headache- tx

A

Acute: NSAIDs, sumatriptans, dihydroergotamine

Prophylaxis: lifestyle changes (diet, sleep, exercise), beta blockers, CCBS, amitriptyline, topiramate, valproate

55
Q

Migraine- POUND

A 
Pulsatile
One-day duration
Unilateral
Nausea
Disabling
56
Q

Vertigo

A

sensation of spinning (while actually stationary)

57
Q

Peripheral vertigo

A

Inner ear etiology (more common)

Caused by debris, infection, Meniere dz (accumulation of endolymph fluid; tx: diuretics)

Positional testing delayed horizontal nystagmus

58
Q

Meniere dz- triad

A

Sensorineural hearing loss
Vertigo
Tinnitus

59
Q

Central vertigo

A

Brainstem or cerebellar prob

Cause: stroke or posterior fossa tumor

60
Q

Findings

A

Directional change of nystagmus, skew deviation, diplopia, dysmetria (lack of coordinated hand movement- overshoot/ undershoot)

Focal neurologic findings

61
Q

Neurocutaneous disorders: STURGE-Weber syndrome

A

Somatic, congenital, GNAQ gene mutation

STURGE-Weber

Sporadic, port-wine Stain
Tram track calcifications (opposing gyro)
Unilateral
Retardation (intellectual disability)
Glaucoma, GNAQ gene
Epilepsy
62
Q

Tuberous Sclerosis- HAMARTOMA

A

Congenital
Caused by mutation in the hamartin gene (encoded by TSC1- chr 9) –> involved in mTOR signaling and vesicular transport
OR mutation in tuberin gene (encoded by TSC2- chr 16)

Hamartomas in brain (benign growths)
Ash-leaf spots (hypo pigmented macules)
Mitral regugitation
Angiofibroma
Rhabdomyoma (cardiac)
Tuberous sclerosis
autosomal dOminant
Mental retardation
Angiomyolipoma (renal)- benign tumors composed of blood vessels, smooth muscle, and fat
Seizures, Shagreen patches
63
Q

Neurofibromatosis Type I (NF I)- CiCLOP

A

AD; caused by mutation in NF1 gene (tumor suppressor (negatively regulates RAS- neurofibromin on chr 17- von Recklinghausen dz)

Neurofibromas- derived from neural crest cells

S&S: cafe-au-lait spots, neurofibromas, Lisch nodules (pigmented iris hamartomas), optic glioma, pheochromocytoma

CiCLOP

Cafe au lait spots
(i)
Cutaneous neurofibroma
Lisch nodules (in iris)
Optic gliomas
Pheochromocytoma
64
Q

von-Hippel Lindau dz

A

Mutation in chr 3 (VHL gene- associated with renal cell carcinoma)

S&S: hemangioblastoms (high vascularity & hyperchromatic nuclei) in retina, brain stem, cerebellum, and spine; angiomatosis (cavernous hemangiomas in skin, mucosa, organs); bilateral renal cell carcinoma; pheochromocytoms

65
Q

Adult primary brain tumors (6)- Adults go to the GM SHOP

A
  1. Glioblastoma multiforme (grade IV astrocytoma)
  2. Meningioma
  3. Hemangioblastoma
  4. Schwannoma
  5. Oligodendroglioma
  6. Pituitary adenoma
66
Q

Childhood primary brain tumors (5)- this kid is an EC PiMP

A
  1. Pilocytic (low grade) astrocytoma
  2. Medulloblastoma
  3. Ependymoma
  4. Craniopharyngioma
  5. Pinealoma

EC-PiMP

67
Q

Glioblastoma multiforme (grade IV astrocytoma)- bad news bears :(

A

Highly malignant and poor prognosis :(

Located in cerebral hemispheres- can cross corpus callosum

68
Q

Glioblastoma multiforme- histology

A

Pseudopalisading, pleomorphic tumor cells- central areas of necrosis and hemorrhage
Stains for GFAP (astrocytes)

69
Q

Meningioma

A

Typically benign

Occurs in parasagittal (midline) region and surface of brain (makes sense– meningioma are at the meninges- brain surface)

May present with seizures or focal neuro signs

Tx: surgery or resection

70
Q

Meningioma- histology

A

Arises from arachnoid cells and may have dural attachment (tail)

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

71
Q

PSaMMoma bodies

A

Characteristic calcifications found in:

Papillary thyroid cancer
Serous papillary cystadenocarcinoma of the ovary
Meningioma
Malignant mesothelioma

72
Q

Hemangioblastoma

A

Often cerebellar and associated with VHL (w/ retinal angiomas)

Can produce EPO –> leading to secondary polycythemia

73
Q

Hemangioblastoma- histology

A

Closely arranged, thin wall capillaries, with minimally intervening parenchyma

74
Q

Schwannoma

A

Classically at the cerebellopontine angle (or another PERIPHERAL nerve)

Often localized to CN VIII- vestibular schwanomma

75
Q

Schwannoma- histology

A

Schwann cell origin- will stain S-100 +

76
Q

Vestibular schwannoma- S&S

A

Patient presents with sensorineural hearing loss, tinnitus, vertigo, hydrocephalus, increased ICP; associated with NF 2

NOT to be confused with Meniere dz (peripheral vertigo- S&S: tinnitus, sensorineural hearing loss, and vertigo)

77
Q

Oligodendroglioma

A

Rare, slow growing

Most often in frontal lobes

78
Q

Oligodendroglioma- histology

A

Chicken-wire capillary pattern
Fried-egg appearance of cells (dense round nuclei with surrounding clear cytoplasm)
Often calcified

79
Q

Things that have a “fried egg appearance”

A

Owl-eye inclusions: CMV

Fried egg shape:
Multiple myeloma (plasma cells)
Oligodendroglioma (oligodendrocytes)
Dysgerminoma/ Seminoma (germ cells- oocyte/ spermatocyte)

80
Q

Pituitary adenoma

A

Most commonly a prolactinoma (lactotroph adenoma) or non-functioning adenoma

S&S: bitemporal hemianopia (due to pressure on optic chiasm); results in hypo/hyper pituitarism

81
Q

Pituitary adenoma- histology

A

Hyperplasia of a single type of endocrine cell found in pituitary (e.g. lactotrophy, gonadotroph, somatotroph, and cortiocotroph)

82
Q

Childhood brain tumors- pilocytic astrocytoma

A

Low-grade, usually well-circumscribed; good prognosis
Most often found in the posterior fossa (cerebellum)

GFAP +

83
Q

Pilocytic astrocytoma histology

A

GFAP+; rosenthal fibers; eosinophilic, corcksvrew fibers

Cystic and solid (gross)

84
Q

Medulloblastoma

A

Highly malignant CEREBELLAR tumor (neuroectodermal)

Can cause noncommunicating hydrocephalus due to compression of the 4th ventricle (and send drop metastases to spinal cord)

85
Q

Medulloblastoma- histology

A

Horner-Wright rosettes, small blue cells

86
Q

Ependymoma :(

A

Most commonly found around the 4th ventricle
Can cause hydrocephalus
Poor prognosis

87
Q

Ependymoma- histology

A

PERI-VASCULAR rosettes (pseudorosettes)

Rod shaped blepharoplasts (basal ciliary bodies) found near nucleus

88
Q

Craniopharyngioma

A

Childhood tumor that may be confused with pituitary adenoma (as both cause bitemporal hemianopia)

Derived from RATHKE pouch remnants

89
Q

Craniopharyngioma- histology

A

Calcification is common, cholesterol crystals found in motor-oil like fluid within tumor

90
Q

Pinealoma

A

Pineal gland tumor; can cause Parinaud syndrome (vertical gaze palsy- inability to move eyes up)

Can cause hydrocephalus (due to compression of cerebral aqueduct

Precocious puberty in males (due to beta-hCG production)

91
Q

Pinealoma

A

Histologically similar to GERM CELL TUMORS (e.g seminoma/ dysgerminoma)

92
Q

Cingulate/ subfalcine herniation (under falx cerebri)

A

Can compress anterior cerebral artery (supplies legs- sensory and motor cortex)

93
Q

Downward transtentorial (central) herniation

A

Can displace brainstem caudally, rupture basilar artery branches –> Duret hemorrhage (usually fatal)

94
Q

Uncal (medial temporal lobe) herniation

A

Can compress ipsilateral CN III (dilated, down and out gaze)

Compress ipsilateral PCA (contralateral homonymous hemianopia- with macular sparing)

Compress contralateral crus cerebri- cerebral peduncle- carries motor info (causes ipsilateral (to herniation, but contralateral to crus cerebri) paresis- since herniation of the LEFT temporal lobe would cause injury to the RIGHT crus cerebri, but paresis of the LEFT side- therefore considered an IPSILATERAL paresis, relative to the original source of the lesion/ herniation)

95
Q

Cerebellar tonsillar herniation (into foramen magnum)

A

Coma and death if herniation compresses brain stem

Otherwise- similar to chiari I???- cape-like distribution of pain/sensory loss

Neurology (5 decks)

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