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Neuro- Pathology Flashcards

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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
Acute disseminated (post infectious) encephalomyelytis
Multifocal periventricular inflammation and demyelination after infection or vaccine Rapid change in mental status and multifocal neuro symptoms
26
Charcot Marie Tooth dz
Hereditary MOTOR and SENSORY neuropathy Related to the defective production of proteins involved in the peripheral nerve or myelin sheath AD inheritance
27
CMT dz- S&S
Foot deformities (pes cavus), lower extremity weakness, and sensory deficits
28
Krabbe dz
AR lysosomal storage dz- results from deficiency of galactocerebrosidase Buildup of galactocerebroside and psychosis destroys myelin sheath
29
Krabbe dz
Signs and symptoms: peripheral neuropathy, developmental delay, optic atrophy, and globoid cells
30
Metachromic leukodystrophy
AR lysosomal storage dz- results from deficiency of Arylsulfatase A Buildup of sulfates --> impaired production and destruction of myelin sheath
31
Metachromic leukodystrophy- S&S
Central and peripheral demyelination with ataxia and dementia
32
Progressive multifocal leukoencephalopathy
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
33
PML- S&S
Problems with speech, memory, coordination; quick decline MRI shows non-enhancing areas of demyelination
34
Adrenoleukodystrophy
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
35
Partial (focal) seizures
Affects a localized/ single region of the brain (generally medial temporal lobe) Preceded by seizure aura
36
Focal seizure: Simple partial
consciousness intact Motor, sensory, autonomic, psychic
37
Focal seizure: Complex partial
IMPAIRED consciousness
38
Epilepsy
disorder of recurrent seizures excludes febrile seizures
39
Status epilepticus
continuous or recurring seizure that may result in brain injury (>5 min)
40
Generalized seizure
Diffuse 5 types: Absence, myoclonic, tonic-clonic, tonic, atonic
41
Absence (petit mal)
3Hz, no postictal confusion, blank stare
42
Myoclonic
quick, repetitive jerks
43
Tonic-clonic (grand mal)
alternating stiffening and movement
44
Tonic
stiffening
45
Atonic
"drop" seizures (falls to the floor) may be mistaken for fainting
46
Seizure causes (by age)
Children: genetic, infection (febrile), trauma, congenital, metabolite accumulation Adults: trauma, stroke, tumor, infection Elderly: stroke, tumor, trauma, metabolite, infection
47
Headache
Pain due to irritation of structures- such as the dura, CHs, or extra cranial structures (more common in females)
48
Cluster headache- S&S
Unilateral, 12min-3hr, repetitive, brief headaches Excruciating periorbital pain with lacrimation and rhinorrhea May present with Horner syndrome
49
Cluster headache- tx
Acute: sumatriptan, 100% O2 Chronic: verapamil
50
Cluster vs. trigeminal neuralgia
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
Tension headache- S&S
Bilateral, >30 min duration (typically 4-6 hrs), steady pain, no photo or phonophobia. NO AURA
52
Tension headache- tx
Analgesics, NSAIDs, acetaminophen Chronic: TCADs (amitriptyline)
53
Migraine headache- S&S
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
Migraine headache- tx
Acute: NSAIDs, sumatriptans, dihydroergotamine Prophylaxis: lifestyle changes (diet, sleep, exercise), beta blockers, CCBS, amitriptyline, topiramate, valproate
55
Migraine- POUND
``` Pulsatile One-day duration Unilateral Nausea Disabling ```
56
Vertigo
sensation of spinning (while actually stationary)
57
Peripheral vertigo
Inner ear etiology (more common) Caused by debris, infection, Meniere dz (accumulation of endolymph fluid; tx: diuretics) Positional testing delayed horizontal nystagmus
58
Meniere dz- triad
Sensorineural hearing loss Vertigo Tinnitus
59
Central vertigo
Brainstem or cerebellar prob Cause: stroke or posterior fossa tumor
60
Findings
Directional change of nystagmus, skew deviation, diplopia, dysmetria (lack of coordinated hand movement- overshoot/ undershoot) Focal neurologic findings
61
Neurocutaneous disorders: STURGE-Weber syndrome
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
Tuberous Sclerosis- HAMARTOMA
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
Neurofibromatosis Type I (NF I)- CiCLOP
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
von-Hippel Lindau dz
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
Adult primary brain tumors (6)- Adults go to the GM SHOP
1. Glioblastoma multiforme (grade IV astrocytoma) 2. Meningioma 3. Hemangioblastoma 4. Schwannoma 5. Oligodendroglioma 6. Pituitary adenoma
66
Childhood primary brain tumors (5)- this kid is an EC PiMP
1. Pilocytic (low grade) astrocytoma 2. Medulloblastoma 3. Ependymoma 4. Craniopharyngioma 5. Pinealoma EC-PiMP
67
Glioblastoma multiforme (grade IV astrocytoma)- bad news bears :(
Highly malignant and poor prognosis :( | Located in cerebral hemispheres- can cross corpus callosum
68
Glioblastoma multiforme- histology
Pseudopalisading, pleomorphic tumor cells- central areas of necrosis and hemorrhage Stains for GFAP (astrocytes)
69
Meningioma
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
Meningioma- histology
Arises from arachnoid cells and may have dural attachment (tail) Spindle cells concentrically in a whorled pattern; psammoma bodies (laminated calcifications)
71
PSaMMoma bodies
Characteristic calcifications found in: Papillary thyroid cancer Serous papillary cystadenocarcinoma of the ovary Meningioma Malignant mesothelioma
72
Hemangioblastoma
Often cerebellar and associated with VHL (w/ retinal angiomas) Can produce EPO --> leading to secondary polycythemia
73
Hemangioblastoma- histology
Closely arranged, thin wall capillaries, with minimally intervening parenchyma
74
Schwannoma
Classically at the cerebellopontine angle (or another PERIPHERAL nerve) Often localized to CN VIII- vestibular schwanomma
75
Schwannoma- histology
Schwann cell origin- will stain S-100 +
76
Vestibular schwannoma- S&S
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
Oligodendroglioma
Rare, slow growing Most often in frontal lobes
78
Oligodendroglioma- histology
Chicken-wire capillary pattern Fried-egg appearance of cells (dense round nuclei with surrounding clear cytoplasm) Often calcified
79
Things that have a "fried egg appearance"
Owl-eye inclusions: CMV Fried egg shape: Multiple myeloma (plasma cells) Oligodendroglioma (oligodendrocytes) Dysgerminoma/ Seminoma (germ cells- oocyte/ spermatocyte)
80
Pituitary adenoma
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
Pituitary adenoma- histology
Hyperplasia of a single type of endocrine cell found in pituitary (e.g. lactotrophy, gonadotroph, somatotroph, and cortiocotroph)
82
Childhood brain tumors- pilocytic astrocytoma
Low-grade, usually well-circumscribed; good prognosis Most often found in the posterior fossa (cerebellum) GFAP +
83
Pilocytic astrocytoma histology
GFAP+; rosenthal fibers; eosinophilic, corcksvrew fibers Cystic and solid (gross)
84
Medulloblastoma
Highly malignant CEREBELLAR tumor (neuroectodermal) Can cause noncommunicating hydrocephalus due to compression of the 4th ventricle (and send drop metastases to spinal cord)
85
Medulloblastoma- histology
Horner-Wright rosettes, small blue cells
86
Ependymoma :(
Most commonly found around the 4th ventricle Can cause hydrocephalus Poor prognosis
87
Ependymoma- histology
PERI-VASCULAR rosettes (pseudorosettes) | Rod shaped blepharoplasts (basal ciliary bodies) found near nucleus
88
Craniopharyngioma
Childhood tumor that may be confused with pituitary adenoma (as both cause bitemporal hemianopia) Derived from RATHKE pouch remnants
89
Craniopharyngioma- histology
Calcification is common, cholesterol crystals found in motor-oil like fluid within tumor
90
Pinealoma
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
Pinealoma
Histologically similar to GERM CELL TUMORS (e.g seminoma/ dysgerminoma)
92
Cingulate/ subfalcine herniation (under falx cerebri)
Can compress anterior cerebral artery (supplies legs- sensory and motor cortex)
93
Downward transtentorial (central) herniation
Can displace brainstem caudally, rupture basilar artery branches --> Duret hemorrhage (usually fatal)
94
Uncal (medial temporal lobe) herniation
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
Cerebellar tonsillar herniation (into foramen magnum)
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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