Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Neuro > Unit II week 1 > Flashcards

Unit II week 1 Flashcards

1
Q

Myopathy vs. Denervation

A

Myopathy = primary disease of muscle –> PROXIMAL weakness and atrophy

  • elevated CK
  • EMG changes

Dennervation = DISTAL weakness and atrophy

  • NORMAL CK
  • Different EMG changes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Response to Denervation

A

Atrophy of muscle

Over time, end-organ “loses” ability to receive a nerve fiber input and be functionally restored

Approx 2 years for muscle

Variable for different types of sensory endings

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

PNS vs. CNS myelin

A

Myelin = 70% lipids, 30% protein

  • Lipids in CNS and PNS are the same
  • Protein compositions differ in CNS and PNS → allergic reaction against PNS myelin does not cause central demyelination and vice versa
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Segmental demyelination (1 characteristic feature)

A

Demyelinating type of peripheral nerve disorder

breakdown and loss of myelin over a few segments - axon remains intact and there is no change in neuronal body

  • Onion bulb neuropathy: onion bulb formation (hypertrophy of nerves) with repeated attack and repair of myelin
  • Have working axon, but conduction velocity decreased

EX) Inflammatory demyelinative neuropathies

EX) Charcot-Marie-Tooth disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Astrocyte reaction to injury

A

expansion of cytoplasmic volume and synthesis of intracytoplasmic intermediate glial filaments (GFAP+)

CANNOT fill large holes of tissue damage (do not make COLLAGEN)

Chronic gliosis results in cytoplasmic expansion and extension of cell processes, but NO EXTRACELLULAR COLLAGEN → astrocytes CANNOT fill in large holes of tissue damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Microglia

A

major phagocytic cell of nervous system, proliferate/respond to injury

Sentinels within brain, monitor immunologic signals, awaiting need for response to tissue injury

Replenished by blood monocytes

High level of activity –> when damage occurs, microglia to lots of cleaning up, but astrocytes cannot make collagen to fill space –> large empty hole remains

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Ischemic neuron –> ?

what appearance?

A

total necrosis –> loss of neuron and removal

Appearance: acutely damaged, “RED DEAD” neuron + loss of nucleus + loss of basophilic Nissl substance → EOSINOPHILIA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What happens when you transect an axon?

what does it look like pathologically?

A

Wallerian degeneration

necrosis of axon distal to transection

→ Neuroaxonal swelling - Swollen axonal process by silver stains at site of transection/injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is GFAP staining used for?

A

used to highlight full cytoplasmic volume of astrocyte on immunostaining

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Intracytoplasmic intermediate glial filaments (GFAP+)

A

key protein of astrocyes

-released by astrocytes in response to tissue injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Concussion (Mild Traumatic Brain Injury)

A

alteration in mental status caused by biomechanical forces that may or may not cause loss of consciousness

Differs from severe TBI only in degree, and regions of brain affected (confined to junction of white/gray matter immediately beneath cortex)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Hallmarks of mild TBI (2)(aka concussion) and common symptoms

A

Hallmarks = confusion and amnesia

Common symptoms: headache, dizziness, poor attention, inability to concentrate, memory problems, fatigue, irritability depressed mood, intolerance of bright light or loud noise, and sleep disturbance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Grading scale of concussion

A

Grade 1 = Confusion without amnesia or LOC
Grade 2 = Confusion and amnesia
Grade 3 = LOC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Second Impact Syndrome

mechanism?

A

usually fatal, second concussion while still suffering effects of an earlier concussion

Loss of CNS vasculature autoregulation → cerebral vessels lose tone, fill with blood, ICP rises → reduced cerebral perfusion, ischemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Concussion management (4)

A

Observation for 24 hours

CT scan to check for intracranial bleeding if LOC occurred

Treat sleep disturbances

Acetaminophen for headaches and bodily pain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Highest incidence of head injuries?

A

Economically disadvantaged populations within major cities

Males 2x higher risk

Peak age = 25-35yrs

Smaller peaks from 0-4 yrs (shaken baby) and over 65 yrs (falls)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Most common causes of head injury (5)

A
  • Traffic and transport injuries
  • Assaults
  • Homicides
  • Suicides
  • Falls
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Forces responsible for TBI (4)

A

1) Contact phenomena
2) Acceleration/Deceleration
3) Penetrating
4) Secondary injury (hypoxia, hypotension)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Contact phenomena

A

results from object striking the head

→ Local effects (lacerations of scalp, fractures of skull, epidural hematomas, cerebral contusions)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Acceleration/Deceleration

A

results from rapid head movement that can create shear, tensile, and compressive strains

Translational or rotational

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Translational (Acceleration/Deceleration)

A

(falls, restrained occupants in MVA)

Head movement in a single plane the instant after impact

Results in stretching, tearing of veins between brain and dura (subdural hematoma) and bruising of brain as it impacts skull (contusion)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Rotational

Acceleration/Deceleration

A

(high speed MVA, ejection, auto-ped, motorcycle)

Results from head moving in more than one plane

Results in microscopic tearing of nerve cells in brain

No recognizable injury detected without microscope

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Penetrating head injury

A

(GSW, knives, tree branches)

Results from combination of contact phenomena and distant translational injury

Results in direct cranial and cerebral injury as well as translational injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Contact phenomena injuries

A

Skull fracture
Extradural hematoma
Epidural hematoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Skull fractures

5 types of fractures

A

Results from contact phenomenon

1) Linear
2) Depressed
3) Basilar
4) Diastatic
5) Growing fractures of infancy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Linear skull fracture

A

crack, not displaced

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Depressed skull fracture

A

depression of bone in toward the brain

comminuted bone fragments may or may not be driven into brain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Basilar skull fracture

A

crack at base of skull, common with high-velocity blunt injuries

May extend through cribriform plate or petrous bone

→ highly associated with CSF leak, meningitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Signs of skull base fracture (6)

A
  • CSF rhinorrhea or otorrhea
  • Bilateral periorbital haematomas (racoon eyes)
  • Subconjunctival hemorrhage
  • Bleeding from external auditory meatus
  • Battle’s sign (bruising behind ear)
  • Facial nerve palsy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Diastatic skull fracture

A

traumatic separations of skull at suture lines

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Growing fractures of infancy

A

skull is soft, so it gets punched in, and dura tears, and then skull pops back out

Can then have herniation of arachnoid into fracture site

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Extradural or Epidural Hematoma

A

intracranial, extradural ARTERIAL bleeding often due to skull fractures (affecting middle meningeal artery)

** “LUCID INTERVAL” →progressive obtundation/coma as hematoma expands

Low mortality rate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Treatment of Extradural or Epidural Hematoma

A

TX = surgical removal of mass lesion

Prognosis depends on time from injury to evacuation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Translational Injuries (Acceleration injury) (2)

A

1) Subdural hematoma

2) Cerebral contusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Subdural hematoma

A

hemorrhage into subdural space due to rupture of BRIDGING VEINS that connect cortical surface of brain with sagittal sinus

Due to translational accelerations from high velocity mechanisms

Associated with:

  • Cerebral contusions
  • Elevated intracranial pressure
  • Distant secondary cerebral injury
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Subdural hematoma treatment

A

prompt surgical removal of blood clot, control ICP, restore adequate cerebral blood flow

**High mortality rate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Cerebral Contusion

A

superficial hemorrhagic contusion of brain (often where brain hits skull at anterior cranial fossa, and greater wing of sphenoid bone)

Due to high velocity translational and impact injuries

Hemorrhage into areas of damaged brain → mass effect and herniation with secondary brain injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Treatment of cerebral contusion

A

medical management of brain swelling, occasional surgical evacuation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

_______ is a Rotational Injury

Acceleration-Deceleration Injury

A

Diffuse Axonal Injury (DAI)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Diffuse Axonal Injury (DAI)

A
  • shearing of axons → Axon spheroids, aka “retraction balls” (appear when coma exceeds 6 hours)
  • Patient unconscious from moment of injury
  • No evidence of injury on CT, MRI may show punctate hemorrhages over white large white matter tracts
  • Corpus callosum and brainstem most commonly affected
  • Axonal degeneration may continue for years after injury
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Primary Injury vs. Secondary injury

A

Primary Injury: occurs at moment of impact - irreversible injury

Second injury: due to inadequate resuscitation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Secondary injury is often due to… (3)

A

1) Hypoxia
2) Altered cerebral blood flow (dysautoregulation)
3) Release of free radical mediators → break down BBB → interstitial edema → brain swelling, elevated ICM, further hypoxia, dysautoregulation, and herniation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Pathophysiology of traumatic brain injury

A

effect of a mass lesion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Intracranial compensation

A

Brain is non-compressible

Any increase in intracranial volume decreases CSF or CBV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Where is CSF displaced?

A

primarily into spinal subarachnoid space

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Where is blood displaced?

A

venoconstriction of CNS capacitance vessels displaces blood into jugular venous system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Exhaustion compensation

A
  • Once limited homeostatic mechanisms are exhausted, additional small increases in volume of a mass lesion produce marked elevations in ICP
  • Raised ICP may decrease CBF, resulting in vicious cycle
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Herniation

A

ICP rises not equally distributed throughout skull and pressure gradients develop

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Example of lateral herniation

A

cingulate herniation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Example of downwards herniation

A

Transtentorial herniation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

Excitotoxicity

A

mechanical forces cause massive neuronal depolarization + massive NT release

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Mechanism of excitotoxicity leading to vasogenic edema

A

Neurons damaged and killed by overactivation of receptors for excitatory neurotransmitter, glutamate
→ lots of Ca2+ into cell→ activate enzymes and free radicals→ damage to cell structures and BBB
→ Vasogenic Edema

Also cause immediate K+ spike extracellularly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Vasogenic edema

A

increased permeability due to BBB damage causes additional brain swelling

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Mechanism in excitotoxicity leading to cytotoxic edema

A

Astrocytes attempt to clear glutamate and K+ extracellularly

-Glutamate transporter relies on high Na+ out, and low K+ in → pump cannot function with high K+ out → pump reverses and makes excitotoxicity worse (K+ and glutamate into cell)

→ *CYTOTOXIC EDEMA: astrocyte swelling in response to ionic shifts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Cytotoxic edema

A

astrocyte swelling in response to ionic shifts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

What happens to cerebral blood flow when astrocytes swell due to cytotoxic edema?

A

Swelling of astrocytes abutting capillaries → increased capillary resistance, and decreased cerebral blood flow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

What are the main consequences of excitotoxicity?

A

1) Vasogenic edema
2) Cytotoxic edema
3) Decreased cerebral blood flow (due to astrocyte swelling)
4) Lose zone of autoregulation (too much or too little blood flow)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Herniation Syndromes

A

forcible displacement of brain tissue across falx, tentorium, or foramen magnum

Secondary injury caused by mass effect (trauma, ischemia, neoplasm, infection, and hydrocephalus)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

Classic signs of increased ICP

A

progressive lethargy and poor responsiveness (obtundation)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

Subfalcine Herniation

A

cingulate gyrus pushed away from expanding mass and herniates beneath falx cerebri
→ Anterior cerebral artery kinked

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

Central herniation

A

downward pressure centrally → bilateral uncal herniation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

Uncal herniation

A

uncus herniates across tentorial edge and downward into posterior fossa → compresses midbrain and ipsilateral cerebral peduncle

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Which deficits are seen with uncal herniation?

A

→ third nerve palsy (pupil dilated on ipsilateral side) and contralateral hemiparesis/hemiplegia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

Kernohan’s Notch

A

when uncal herniation compresses the opposite cerebral peduncle against the tentorial edge → hemiparesis IPSILATERAL to mass lesion and herniated uncus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

Duret Hemorrhage

A

characteristic hemorrhage in brain stem associated with uncal herniation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

Tonsillar Herniation

A

cerebellar tonsils herniate downward into foramen magnum = “Coning”

Compresses medulla → Cushing’s reflex (bradycardia, hypertension in setting of high ICP)

-Typically due to mass lesion in posterior fossa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

Lumbar puncture in setting of intracranial mass lesion can precipitate ________

A

herniation syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

3 main responses assessed with the Glasgow Coma Scale

A

1) Eye opening
2) BEST motor response
3) Verbal response

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

Review glasgow coma scale

A

DO IT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

Syndrome of delirium

A
  • rapidly developing disorder of attention characterized by an inability to maintain a coherent line of thought
  • ACUTE confusional state, with prominent attentional problems and a toxic-metabolic encephalopathy
  • Fluctuating level of consciousness, incoherent speech
  • Toxic and metabolic causes usually found
  • Typically reversible
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

What’s more common - delirium hypoaroused or hyperaroused?

A

HYPOAROUSED with lethargy and somnolence more common than type with hyperarousal + agitation and restlessness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

Pathophysiology of Delerium

A

diffuse brain dysfunction due to disruption of normal brain homeostasis

Widespread neuronal dysfunction affecting arousal systems in brainstem and diencephalon, and cortical regions

If insult is corrected within a few days, normal brain function can be restored - prolonged insult may damage neurons irreversibly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

Common etiologies of delirium (7)

A

1) Drugs and toxins (intoxication and withdrawal) = # 1 cause
2) Metabolic disorders
3) Cardiac, pulmonary, renal, hepatic, endocrine, and nutritional diseases also possible causes

4) Infections/Inflammatory causes
- Meningitis, encephalitis, CNS vasculitis, systemic infection

5) Traumatic brain injury
6) Stroke, hemorrhage, edema
7) Seizure disorders

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

Evaluation of a patient with delirium

A

1) History and physical (complete mental status exam not necessary)
2) Lab tests (metabolic panel, CBC, urinalysis, urine tox screen, EKG, CXR, CT/MRI of brain)
3) Lumbar puncture possible
4) Electroencephalogram

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

Treatment of delirium

A

1) Prompt attention to etiology

2) Provide adequate sleep
- Avoid daytime sedation and naps

3) Environmental manipulations (clock, calender, pictures of family, TV)
4) Can give drugs for agitation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

Syndrome of dementia

A

-acquired and persistent impairment in intellectual function with deficits in at least three domains - memory, language, visuospatial skills, complex cognition, and emotion/personality - that is of sufficient severity to interfere with usual social and occupational function

CHRONIC

  • Normal level of consciousness and normal attention
  • Aphasia
  • Toxic and metabolic causes not usually found
  • Typically irreversible - but does NOT NEED to be progressive or irreversible
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

Reversible causes of dementia

A

10-20% of dementia cases

Drugs and toxins, mass lesions (tumor, subdural hematoma), hydrocephalus, systemic illness, inflammatory disease, infectious disease, depression, mild TBI

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

Irreversible causes of dementia

A

80-90% of dementia cases

Alzheimer’s, frontotemporal lobal degeneration, vascular dementia, Lewy Body Dementia, Parkinson’s, Huntington’s, Creutzfeldt-Jakob Disease, HIV-associated dementia, severe TBI

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

Evaluation of dementia

A

History and physical (mental status, general, neurologic)
CMP, CBC, TSH, B12, RPR
MRI or CT scan of brain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

Cortical Dementia

2 examples of diseases

A

cerebral cortex bears major burden of neuropathology

1) Alzheimer’s
2) Frontotemporal Dementia (FD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

Alzheimer’s Disease onset, survival, patient population

A

Cortical Dementia

most common dementia in elderly

5-10% prevalence over age 65, more common in women

Survival after onset = 6-12 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

Three typical stages of Alzheimer’s

A

Stage I (1-3 years): amnesia most notable, mild anomia, apathy

Stage II (2-10 years): dementia obvious, fluent aphasia, visuospatial dysfunction, anosognosia, neuropsychiatric features

Stage III (8-12 years): severe mental and physical incapacity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

Neuropathology of Alzheimer’s

A

Cerebral atrophy, loss of cortical neurons/synapses

Neuritic (amyloid) plaques and neurofibrillary tangles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
84
Q

Etiology of Alzheimer’s (2 possible theories)

A

1) Amyloid precursor protein (chromosome 21) overproduces amyloid
- APOE gene epsilon allele

2) Relative deficiency in acetylcholine → cholinergic hypothesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
85
Q

Frontotemporal Dementia (FD)

A

degenerative disease of frontal/temporal lobes → changes in behavior and comportment, but not significant changes in memory

-Presents with disinhibition, apathy, and executive dysfunction while memory still normal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
86
Q

Subcortical Dementia

2 examples of diseases

A

subcortical gray matter structures (basal ganglia, thalamus, brainstem nuclei) bear burden of neuropathology

1) Parkinson’s Disease
2) Huntington’s Disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
87
Q

Parkinson’s Disease (PD)

sx (4) and neuropathology (2)

A

Sx: tremor, bradykinesia, rigidity, postural instability

Neuropathology:

1) Lewy bodies found in substantia nigra
2) Dopamine deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
88
Q

Huntington’s Disease (HD)

typically shows atrophy in what brain region?

2 symptoms?

genetics?

A

Typically shows caudate atrophy

Sx = dementia and chorea (jerky involuntary movements)

AD
Increased polyglutamine repeats (CAG) in Huntington gene on chr4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
89
Q

White Matter Dementia

2 diseases?

A

cerebral white matter bears burden of neuropathology

1) Binswanger’s Disease
2) Normal Pressure Hydrocephalus (NPH)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
90
Q

Binswanger’s Disease

A

vascular dementia caused by long standing HTN and lacunar infarction of cerebral white matter

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
91
Q

Normal pressure hydrocephalus (NPH)

A

Increased CSF → dilated ventricles → stretching of corona radiata

  • LP improves symptoms
  • “Wet, wobbly, and wacky”
  • causes symptoms of dementia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
92
Q

Mixed Dementia

2 diseases?

A

affects many different brain regions

1) Multi-infarct dementia
2) Creutzfeldt-Jakob Disease (CJD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
93
Q

Multi infarct dementia

A

vascular dementia with repeated strokes that erode cognitive function progressively

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
94
Q

Creutzfeldt-Jakob Disease (CJD)

A

rapidly progressive, fatal, potentially transmissible

RAPIDLY progressive dementia, myoclonus

Human prion disease, often sporadic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
95
Q

Treatment of Dementia

A

-Irreversible dementia → avoid drugs that worsen mental status, use low dose atypical antipsychotic drugs for neuropsych symptoms, antidepressants, informed counseling

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
96
Q

Neurodegenerative Disorders

A

spontaneous death of neuronal populations with location of neurons determining clinical presentation

We categorize these illnesses by clinical presentation, but there is considerable interaction and overlap between their mechanisms

-Characterized by intra/extracellular abnormal protein accumulation
Appear to be disorders of protein conformation and metabolism
Genetic and environmental effects

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
97
Q

Prion Disease

A

conformational change in protein constitutes the transmissible agent → propagation of abnormal protein conformation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
98
Q

Prion

A

small infectious pathogen, contains proteins, but no nucleic acid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
99
Q

Clinical features of prion diseases

A
  • Ataxia, abnormal movements, neuropsychiatric features
  • Sporadic, heritable, or transmissible
  • Uniformly fatal
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
100
Q

Alzheimer’s Disease

A

early memory and visuospatial problems

  • BOTH amyloid plaques and neurofibrillary tangles
  • Acetylcholine deficit
  • Typically see changes in cortex and hippocampus (relative sparing of deeper structures)
  • Results in diffuse atrophy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
101
Q

Frontotemporal Dementia (FTD) (4)

A

-early behavioral, executive and/or language problems

  • May manifest with: neurofibrillary tangles, ubiquitin inclusions, tau reactive intra-neuronal inclusions, or CDP-43 deposition
  • ->Pick Bodies = Tau-reactive intraneuronal inclusions

No specific findings on autopsy

Higher incidence of psychosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
102
Q

Lewy Body dementia

A

early parkinsonian features, psychosis, fluctuating consciousness

  • Lewy bodies with synuclein protein
  • Dopamine and ACh deficit
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
103
Q

Progressive supranuclear palsy (PSP)

A

bradykinesia, rigidity, falls, abnormal vertical eye movements

Lose volitional eye movements, retain reflex eye movements

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
104
Q

Delirium (10 features)

A

1) Develops over short period of time (hours to days)
2) Change from baseline that FLUCTUATES during course of day (fluctuating arousal)
3) ATTENTION deficits
4) Alterations in memory, language, construction, perception, and mood
5) Spontaneous speech may be incoherent, rambling, shifting
6) Sleep disturbances
7) Possible psychosis
8) Neurological motor signs (tremor, altered tone, asterixis, myoclonus, hyperreflexia)
9) Autonomic disturbances (tachycardia, diaphoresis, pupillary dilation)
10) Bottom up impairment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
105
Q

11 features of dementia

A

1) Cognitive deficits interfere with independence in everyday activity
2) Top down impairment
3) Normal level of arousal and attention
4) Insidious onset GRADUALLY, over years
5) Changes in personality language, and complex cognition prior to memory problem
6) Perceptual disturbances
7) Impaired VISUOSPATIAL skills
8) Impaired recognition in given sensory modality (agnosia)
9) Decreased motivation (apathy)
10) Disturbances of emotion (Depression)
11) Disturbances in sleep

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
106
Q

Long term outcomes of in-hospital delirium (4)

A

1) Longer hospital stays
2) Increased risk of death
3) Increased risk of disability and functional dependence
4) Increased risk of future cognitive impairment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
107
Q

Infarction can be ____ or ______

Infarction differs from hemorrhage how?

A

Thrombotic or Embolic

Hemorrhage → blood dissects along planes of least resistance

Infarction/occlusion → deficits in territories of vascular distribution

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
108
Q

Ischemic Stroke vs. TIA

A

Ischemic Stroke (ischemic injury to brain, persistent clinical deficit at 24 hrs)

TIA (ischemic neurological deficits, completely resolved by 24 hours)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
109
Q

Large Vessel Ischemic stroke

results in what deficits?

A

partial or complete occlusion of a major artery causes deficits in multiple systems

MCA → Hemiparesis, hemisensory deficit, hemianopsia

Aphasia (Dominant) and Neglect (non-dominant)

110
Q

Small Vessel Ischemic stroke

results in what deficits?

A

occlusions (atheromatous, lipohyalinosis) of small penetrating vessels deep in the brain) cause isolated motor (or sensory) deficit on one side of body (hemiparesis/hemisensory loss)

Can also be caused by hypoperfusion due to systemic hypotension, cardiopulmonary bypass, isolated major vessel stenosis, vasospasm, hypercoagulation, or inflammatory processes

Small vessels are too small for a catheter

111
Q

Lacunar infarct

A

1 cm or less in size

due to small vessel infarcts

Can be caused by chronic HTN or arteriosclerotic vascular disease

Many lacunar infarcts can eventually result in multi-infarct dementia

112
Q

Types of hemorrhage (5)

A

1) Intracerebral Hemorrhage
2) Intraparenchymal hemorrhages
3) Subarachnoid hemorrhage
4) Subdural hemorrhage
5) Epidural hemorrhage

113
Q

Types of intracerebral hemorrhage?

A

Deep (ganglionic, putamenal, striatocapsular)

Lobar: Frontal > Parietal > Occipital > Temporal

114
Q

Etiology of Deep Intracerebral Hemorrhage

causes?
outcome?
most common in what races?

A

HTN and age
Poor outcome
Non-white and asians at higher risk

115
Q

Etiology of Lobar Intracerebral Hemorrhage

causes?
outcome?
most common in what races?

A

Age, dementia, coagulation
HTN

Surprisingly well tolerated

White/Asian higher risk

116
Q

Intraparenchymal hemorrhages

A

formed blood clots that dissect into brain

most commonly due to HTN and age

117
Q

Causes of Intraparenchymal Hemorrhage (9)

A

1) HTN and age = #1 cause
2) Coagulation disease, or therapeutic anticoagulation
3) AVMs (arteriovenous malformations)
4) Cavernous (venous) Angioma
5) Aneurysms
6) Vasculitis
7) Hemorrhage into tumors
8) Infection
9) Amyloid angiopathy

118
Q

AVMs (arteriovenous malformations)

A

No arteriovenous SHUNT = no intervening bed of capillaries between arteries and veins → blood builds up in veins –> hemorrhage

Associated with aneurysms

Responsible for 2% of all intracranial hemorrhages

Thought to be congenital

119
Q

Cavernous Angioma

A

AD mutation (cerebral cavernous malformation 1, CCM1) common in denver

No arterial component, only venous leaks

120
Q

Aneurysm common locations in brain

A

Most commonly on anterior cerebral artery (40%)→ middle cerebral artery (34%) → posterior communicating artery (20%) → basilar artery (4%)

121
Q

Amyloid angiopathy

A

recurrent lobar hemorrhage typical in elderly→ progressive dementia and disability

122
Q

Typical location of intraparenchymal hemorrhages (4)

A

basal ganglia, thalamus, pons, and cerebellar deep gray matter

Atypical = deep white “lobar” hemorrhages

123
Q

Presentation of intraparenchymal hemorrhages

A

mild headache, deficit, nausea → progress over minutes to hours adding decreased level of consciousness

Hemiparesis progressing to hemiplegia

124
Q

Subarachnoid hemorrhage

A

right next to brain under arachnoid layer, typically involves aneurysms

125
Q

Causes of subarachnoid hemorrhage (3)

A

trauma, aneurysmal rupture, arteriovenous malformations

126
Q

Why do we get aneurysms?

A

Branch points lack media

127
Q

Presentation of subarachnoid hemorrhage

A

cataclysmic, “firecracker” explosion in head, severe headache

Cranial nerve palsy (III especially)

Hemiparesis

Sudden onset of neurological deficits + headache, nausea, vomiting, depressed level of consciousness

High mortality rate

128
Q

Subdural hemorrhage

causes?
appearance?

A

between dura and arachnoid layer, typically torn/ruptured bridging vein, looks like a banana (CONCAVE) hematoma

Causes: “trivial traumas”, small brain in big skull

129
Q

Epidural hemorrhage

causes?
appearance?

A

between skull and dura, typically ARTERIAL (middle meningeal)

collect in CONVEX (lemon/lens) shaped hematoma

Causes: “trivial traumas”, small brain in big skull

“LUCID INTERVAL”

Associated with skull fractures

130
Q

Nontraumatic brain hemorrhage

often is what type of hemorrhage?

what are common causes (elderly vs. young)?

A

More often involvement of brain parenchyma (see intraparenchymal hemorrhage section above)

Think first - CHRONIC HTN

Elderly → Cerebral amyloid angiopathy

Young patient → recreational drug use, immunocompromised, cancer pt receiving chemo, patients with vaso-invasive infections, patient with iatrogenic or intrinsic coagulation deficits

131
Q

Non-Atherosclerotic causes of stroke in young patients (5)

A

1) Vasculopathies:
- Fibromuscular Dysplasia (FMO)
- Moya-Moya
- Spontaneous arterial dissection

2) Hematological disorders
3) Inflammatory mechanisms
4) Venous infarction
5) Vasospasm

132
Q

Fibromuscular Dysplasia (FMO)

A

Vasculopathic cause of non-atherosclerotic stroke in young patients

-arterial media hypertrophies causing areas of segmental occlusion/stenosis

Typically involves renal artery, carotid, and vertebral

Associated with arterial dissection and intracranial saccular aneurysms

Possible association with HSV-1

Women in 30s and 40s

133
Q

Moya-Moya

A

Vasculopathic cause of non-atherosclerotic stroke in young patients

non-atherosclerotic focal occlusion of middle cerebral artery

Children, and women in 30s and 40s

Intimal hyperplasia

Possible association with EBV

Associated with secular aneurysms and dissection

134
Q

Spontaneous Arterial Dissection

A

tear in endothelial lining of artery → blood dissects between endothelium and adventitia → occlusion or stenosis of artery, with emboli distally

Possible association with HSV-1

135
Q

Hematological disorders (6)

A

1) Protein C deficiency, Protein S deficiency, Antithrombin deficiency, or Factor V Leiden, or Prothrombin gene → venous thrombosis
2) Malignancies
3) Sickle Cell Anemia
4) Hyperviscosity states
5) Oral contraceptive
6) Antiphospholipid antibodies (triad = spontaneous miscarriage, thrombocytopenia, recurrent large vessel thrombosis-arterial or venous)

136
Q

Prevention of hemorrhage (2)

A

1) Control BP and other vascular risk factors
- Smoking cessation
- HTN is number one risk factor for nontraumatic brain hemorrhage

2) Manage initial ischemic event with antiplatelet or anticoagulants to prevent hemorrhagic transformation

137
Q

Emergency Management of Intracranial Hemorrhage

A

1) Rapid diagnosis
2) Place intracranial pressure monitoring device or drainage device
3) Emergency surgery (+/-)
4) Support patients before definitive procedure can be performed
5) Diuresis
6) Reduction of blood CO2

138
Q

Prevention of stroke (5)

A

1) Modify risk factors
2) Aspirin
3) Thienopyridines (Clopidogrel, Prasugrel, Ticlopidine)
4) Anticoagulants
5) Endarterectomy in symptomatic patients with high grade (70-90%) stenosis)

139
Q

Emergency management of stroke

A

1) Thrombolytic agents (TPA)
2) Rehydration (lowers blood viscosity, prevent stroke progression)
3) Lumbar puncture:
4) Aspirin (+/- Clopidogrel)
5) IV Heparin

140
Q

What happens if you give TPA too late?

A

Use TPA too late → can get reperfusion injury (especially in gray matter)

141
Q

Why do you do a lumbar puncture in setting of suspected stroke/hemorrhage or AMS (altered mental status)?

A

Used to exclude neurosyphilis, vasculitis, or other inflammatory conditions that can mimic stroke

Determine if there is a subarachnoid hemorrhage

142
Q

Modifiable risk factors for stroke

A

1) Hypertension
2) Lipid disorders (high LDL, low HDL)
3) Insulin resistance
4) Homocysteine elevation
- Treat with folate, B6, and B12
5) Smoking
6) Obesity
7) Physical inactivity
8) Diabetes
9) Alcohol abuse

143
Q

Structural risk factors for stroke

A

mostly cardiac

Afib, CHF, valvular disorders (arterial stenosis), atrial septal defect (PFO), infectious endocarditis, intravascular/cardiac tumors (Atrial myxoma)

144
Q

Main difference between ischemic and hemorrhagic stroke?

A

= TEMPO

Large ischemic stroke - mass effect and elevated ICP takes days to evolve

Large hemorrhagic stroke- mass effect and elevated ICP evolves in minutes to hours (headache, nausea, vomiting, lethargy, progressive LOC + focal neurodeficit)

145
Q

_______ fusion is associated with pilocytic astrocytoma which is a grade ____ glioma

A

BRAF

grade I

146
Q

IDH mutations are present in which 5 gliomas (2 lineages)

A

1) Oligodendroglioma –> Anaplastic oligodendroglioma

2) Diffuse astrocytoma –> anaplastic astrocytoma –> secondary glioblastoma

147
Q

what mutation results in a primary glioblastoma

A

EGFR amplification

148
Q

IDH + ______ are the mutations associated with oligodendrogliomas (and anaplastic oligodendrogliomas)

A

1p19q codeletion

149
Q

Astrocytoma, oligodendroglioma, ependymoma, ganglioma =________, derived from the ___________

A

GLIOMAS

NEUROECTODERM

150
Q

Pilocytic astrocytoma

A

Most common glioma seen in childhood

Well circumscribed, non-infiltrative, minimal tendency to undergo malignant upgrading

151
Q

Typical locations of pilocytic astrocytoma

A

Cerebellum, optic nerves/chiasm, and hypothalamic region

*Children often have tumors in POSTERIOR FOSSA

152
Q

Treatment of pilocytic astrocytoma

A

surgical excision alone (some locations require other therapy as well)

153
Q

Appearance (gross + microscopic) of pilocytic astrocytoma

A

Grossly = cystic

Microscopic = hair-like “piloid” astrocytes and Rosenthal fibers (eosinophilic intracytoplasmic glial filaments)
-May have vascular calcifications

154
Q

Genetics of pilocytic astrocytomas

A

BRAF-KIAA fusion → Constitutive activation of BRAF gene = FAVORABLE feature in slow growing tumors

155
Q

Ganglioglioma

location?
grade?

A

Location: usually in temporal lobe

Well demarcated = grade I

156
Q

Ganglioglioma appearance (gross + microscopic)

A

Grossly = cystic, more likely to be calcified

Microscopic = increased number of jumbled, cytologically abnormal neurons mixed into a low grade glial background
-Microcysts with mucin

157
Q

Ganglioglioma genetics

A

may have BRAF mutation

158
Q

Choroid plexus papilloma typical location in children vs. adults

can result in what complication?
grade?

A

Children → Lateral ventricles
Adults → 4th ventricle

Produce HYDROCEPHALUS by BLOCKING CSF flow (not by CSF overproduction)

grade I

159
Q

Choroid plexus papilloma appearance

A

intraventricular, closely mimic choroid plexus but more “papillary formations”, more abundant/crowded cells covering surface

Low mitotic rate, mild nuclear atypia

160
Q

Grade I brain tumors (3)

A

tumors of low proliferative potential, possibility of cure with surgical resection alone

1) Pilocytic Astrocytoma
2) Ganglioglioma
3) Choroid plexus papilloma

161
Q

Grade II brain tumors (3)

A

infiltrative, low proliferative level, but often recur.
Tend to progress to higher grade
TX: watchful waiting or external beam cranial irradiation

1) Diffuse Astrocytoma
2) Oligodendroglioma
3) Ependyoma

162
Q

Diffuse Astrocytoma

what age?
progression/infiltration?

A

Common glioma

Occur in young adults, Age = 30s-50s

Potential to progress in grade

Infiltrative, no borders, cannot be surgically resected, only debulked

163
Q

Location of diffuse astrocytomas

A

white matter of cerebral hemisphere

164
Q

Appearance of diffuse astrocytomas (5)

A

1) Mild hypercellularity
2) Mild nuclear pleomorphism
3) Irregular distribution of tumor astrocytes
4) Variations in nuclear features and cytoplasmic content

  • *5) Absence of necrosis, mitotic activity, and microvascular proliferation
  • Main difference between anaplastic astrocytoma
165
Q

Genetic anomolies in Diffuse astrocytoma (3)

how does this differ from the genetic anomolies found in oligodendrogliomas?

A

Diffuse Astrocytoma:
IDH1/2 mutation + no LOH 1p, 19q + p53/ATRX mutation

Oligodendroglioma:
IDH1/2 mutation + LOH 1p, 19q + no p53/ATRX mutation

166
Q

Oligodendroglioma

age?
infiltration?
prognosis?

A
  • Common glioma
  • Mostly adults
  • Infiltrative, no clear borders, cannot be surgically resected
  • Better overall prognosis that diffuse astrocytoma
167
Q

Location of oligodendrogliomas

A

cerebral

White matter of cerebral hemisphere but quickly spread to overlying cortex → seizures

168
Q

Appearance of oligodendrogliomas (5)

A

1) Calcified significantly
2) Vascular (“Chicken wire”) vascular pattern)
3) Round monotonous nuclei, equal spacing, minimal cytoplasm
4) “Fried egg” appearance
5) Minimal/absent mitotic activity

169
Q

Genetics of oligodendrogliomas

A

IDH1/2 mutation + LOH 1p, 19q + no p53/ATRX mutation

Can be diagnostic in differentiating oligodendroglioma or anaplastic oligodendroglioma from an astrocytoma

170
Q

Ependymoma

location in adults vs. children
prognosis?

A

mostly, but not always in ventricles

Children → 4th ventricle → HYDROCEPHALUS
**Less favorable diagnosis

Adults → Spinal cord
-Typically well demarcated, full excision may be possible

171
Q

Appearance of ependyomas

A

Gross:

1) Calcified
2) Masses protrude up from 4th ventricle as exophytic masses

Microscopic:

1) ependymal differentiation
2) mild-moderate cell density
3) few mitotic figures
4) Minimal necrosis or microvascular proliferation

172
Q

Grade III brain tumors (3)

A

histological evidence of malignancy (nuclear atypia, high mitotic activity)
TX: adjuvant radiation and/or chemo

1) Anaplastic Astrocytoma
2) Anaplastic Oligodendroglioma
3) Anaplastic ependyoma

173
Q

Anaplastic Astrocytoma

A

Common glioma, progression from diffuse astrocytoma

174
Q

What can be used to label mitotic rate?

what would this stain be important for differentiating?

A

MIB-1+ nuclear labeling → important, used to look at cells undergoing mitosis

Increased mitotic rate = difference between diffuse/anaplastic astrocytoma

175
Q

Appearance of anaplastic astrocytoma (5)

A

1) increased mitotic rate
2) DOES NOT show necrosis or microvascular proliferation
3) Crowded tumor cells
4) Nuclear hyperchromatism
5) variation in cell shape and size

**DO NOT show enhancement on preop imaging studies

176
Q

Anaplastic Oligodendroglioma appearance (2)

A

1) Round, uniform nucleus, scant cytoplasm, “fried egg” appearance
2) microvascular proliferation present

DO show enhancement on preop imaging studies

177
Q

Anaplastic ependymoma

difference with ependyoma?

location/age?

recurrence?

A

Higher mitotic activity than ependyoma

Typically in childhood and in 4th ventricle

Histological criteria not very indicative of prognosis

Can recur in distant sites due to CSF dissemination

178
Q

Grade IV brain tumors (2)

A

cytologically malignant, mitotically active, necrosis prone neoplasms

Rapid disease, often fatal

1) Glioblastoma
2) Medulloblastoma

179
Q

Glioblastoma

A

Most common and most malignant glioma

Most occur in 50s and 60s

Not surgically curable, debulking only

180
Q

Primary vs. secondary GBM

A

Typically arise de novo (“primary GBM”) –> via EGFR pathway

But if diagnosis with lower grade astrocytoma first → “secondary GBM” (via IDH+ pathway)*

181
Q

Genetics of primary GBM

A

EGFR typical mutation (diagnostic) in high grade GBM

182
Q

Appearance of GBM

A

Hemorrhagic, necrotic, multifocal

Deceptively well demarcated, but microscopically infiltrative

183
Q

WHO criteria for GMB (4)

A

need 3/4

1) Nuclear abnormalities (nuclear atypia, hyperchromatism, variation in nuclear size and shape)
2) Mitotic activity
3) Microvascular proliferation
4) Necrosis

184
Q

Medulloblastoma

location?
age?
spread?

A
  • Malignant embryonal tumor of cerebellum (primitive neuroectodermal tumor)
  • Usually in children (3-8 years old) - most common malignant brain tumor in children
  • Spreads throughout CSF pathways
  • -> Outcome is better if it has not spread in CSF
185
Q

Medulloblastoma presenting symptoms

A

Increased ICP → headache, vomiting, papilledema

Disturbances of gait, nystagmus, and dysmetria (cerebellar involvement)

186
Q

Appearance of medulloblastoma?

A

Patternless sheets of small embryonal cells with scant cytoplasm (“small blue cells”)

minimal differentiation

187
Q

Meningiomas (4)

A

Typically grade I, but can be life-threatening if in a location difficult to resect from

Peak incidence is WOMEN IN THEIR 50’S

Arise from arachnoid “cap” cell within arachnoid villus

May penetrate dura, occlude venous sinuses, invade bone, and cause hyperostosis

188
Q

Hemangiopericytoma

A

meningeal-based tumor

Less favorable prognosis than meningioma, high recurrence rate

Accumulates more mutations as grade increases

189
Q

Schwannomas (3)

A

(aka neurofibromas)

  • Benign, can rarely degenerate into malignant form (neurofibrosarcoma)
  • Slow growing
  • Found on cranial nerve 8, other cranial nerves, and spinal nerve roots
190
Q

Most brain tumors are ______ and of _______ etiology

A

SPORADIC

UNKNOWN

191
Q

Metastases into brain come from where?

appearance? location?

A

Typically mets from breast, lung, kidney, melanoma, and GI tract cancer

Usually discrete, non-infiltrating tumors with variable surrounding edema

Typically allows complete surgical resection

Typically occur in cortex

192
Q

Grade I treatment vs. Grade II-IV treatment?

A

Grade I: surgical cure

Grade II-IV:

  • Only surgical debulking, rather than surgical cure is achievable
  • Radiation + chemo

–> + second resection when tumor recurs (especially in grade III-IV)

193
Q

IDH1 and IDH2

A

isoforms of enzyme Isocitrate dehydrogenase

Frequently mutated in gliomas

IDH1 → mutated in 70-80% of grade II and III astrocytomas, oligodendrogliomas, oligoastrocytomas, and secondary (but not primary) glioblastomas

NOT present in pilocytic astrocytomas

Mutation → make tumor cells less viable, increase susceptibility to oxidative damage

BETTER PROGNOSIS with IDH mutation

194
Q

Glial tumors in pediatric vs. adults

A

Look similar, but have different genetic markers

Peds = pilocytic astrocytoma, no IDH mutation - BRAD-KIAA fusion

Adults = IDH mutation +/- LOH 1qp19 +/- p53/ATRX mutation

195
Q

LOH 1pq19

A

unbalanced translocation, fusion → fusion protein

All adult oligodendrogliomas defined by presence of LOH 1p19q AND IDH mutation (either IDH1 or IDH2)

Prognostic, diagnostic, and predictive

196
Q

Tumors that tend to spread via CSF (2)

A

Medulloblastoma

Anaplastic Ependyoma

197
Q

Demyelinationg diseases

A

group of diseases, primarily acquired, and associated with myelin destruction with RELATIVE sparing of axons

198
Q

Five subtypes of MS

A
  1. Relapsing-remitting (RRMS)
  2. Primary progressive (PPMS)
  3. Secondary progressive (SPMS)
  4. Clinically Isolated syndrome (CIS)
  5. Radiologically Isolated Syndrome (RIS)
199
Q

Relapsing Remitting MS

A

sporadic episodes of new or worsened symptoms and signs (over 2-10 days), with variable improvement over 1-6 months

85% of patients present with this type

200
Q

Primary Progressive MS

A

never have relapses, just slow progression

15% present with this type

201
Q

Secondary progressive MS

A

relapsing-remitting MS, which converts to progressive disease

202
Q

__% of RRMS will convert to SPMS

A

50+%

203
Q

Clinically isolated syndrome

A

“first attack”, don’t fit criteria for full dx yet

204
Q

Radiologically isolated syndrome

A

individuals scanned for “non”-MS symptoms, yet have apparent MS on MRI scans

205
Q

Age of presentation MS

A

75% between 15-45 years

206
Q

MS: males or females

A

2/3 are women

207
Q

Race most commonly affected by MS

A

Caucasians

208
Q

Neuropathology of MS: Early and late

A

Lesions evolve over time

  1. Early → perivascular lymphocytic infiltrate with T/B cells, macrophages, complement, IgG, and complement
    a. Demyelination with RELATIVE axonal sparing
  2. Later → more of a glial scar, modest inflammation
  3. Condition CHANGES with age - relapsing, to stable, etc.
209
Q

In MS: ___ matter lesions seen early pathologically/on MRI

A

Grey

210
Q

What is seen in the meninges, especially in progressive MS

A

Lymph node-like structure with B cells

211
Q

MS is both ____ and _____

A

inflammatory and degenerative

Inflammation wanes with time

212
Q

Criteria for MS diagnosis

A

i. Multiple lesions of CNS disseminated in time and space
- 2 or more symptoms lasting at least 24hrs, 30+ days apart (RRMS) or minimum of 12 months of progression of sx (PPMS)
- 2 separate locations in brain/spinal cord

ii. No other cause identified
iii. Objective abnormalities on neurological exam

213
Q

Clinical symptoms of early MS (6)

A
  1. UNIFOCAL, paresthesias (numbness, tingling)
  2. Monocular loss of vision (optic or retrobulbar neuritis)
  3. Gait problems, weakness
  4. Diplopia (double vision)
  5. Urinary urgency/frequency
  6. Constipation
214
Q

Clinical symptoms of late MS (7)

A

Symptoms above + …

  1. Multifocal symptoms
  2. Fatigue, depression
  3. Sexual dysfunction
  4. Cognitive dysfunction
  5. Pain
  6. Dysphagia
  7. Problems due to immobility (e.g. infections and DVT)
215
Q

Neurologic exam in MS

A

i. Usually ASSYMMETRIC “upper motor neuron” / pyramidal tract signs (weakness, spasticity, Babinski sign)
ii. Decreased visual acuity, optic atrophy, and afferent pupillary defect
iii. Eye movement abnormalities (nystagmus, internuclear ophthalmoplegia)
iv. Sensory loss
v. Cerebellar signs (ataxia, tremor, dysarthria, balance, coordination)
vi. Mood - depression, labile affect
vii. Cognitive dysfunction

216
Q

MRI in MS

A

lesions in periventricular regions, corpus callosum, juxtacortical, spinal cord, and brainstem/cerebellum

  1. T1 “holes” = axonal damage,bad long term prognostic sign
    - Chronic sign
  2. T1 with contrast-enhancing lesions=BBB breakdown (early stage lesion)
    - Leakage of dye that lights up in brain due to BBB damage
  3. T2 hyperintense/bright lesions = acute or chronic lesions, many bright dots on T2 and FLAIR images
  4. Atrophy (especially gray matter) = bad long-term prognostic sign
217
Q 
CSF analysis MS:
protein-?
WBC-?
Glucose-?
IgG-?
Myelin basic protein-?
A
  1. Protein mild elevations
  2. WBC mild elevations (lymphocytes)
  3. Glucose = ALWAYS NORMAL
  4. Abnormal IgG production + oligoclonal bands
  5. Myelin basic protein elevations (nonspecific)
218
Q

Evoked potentials MS

A

prolonged conduction times consistent with demyelination

219
Q

Goal of therapy in MS

A

lifelong brain health

220
Q

Behavioral changes in MS treatment

A

good sleep, exercise, no smoking, vitamin D, low salt diet

221
Q

Aggressive treatment of comorbidities in MS treatment

A

HTN, diabetes, spine disease, hip/knee disease, primary sleep disturbances

222
Q

Treatment of acute attacks in MS treatment

A

high dose corticosteroids, plasma exchange (in severe cases, unresponsive to steroids)

223
Q

Symptomatic therapy in MS treatment

A

physical, occupational, speech, rehab psychological therapies, etc.

224
Q

Immunotherapy in MS treatment

A

goal = reduce relapses, reduce progression to disability as related to relapses, and reduce MRI changes = No Evidence of Disease Activity (NEDA)

225
Q

Acute alcohol intoxication –> what CNS effect?

A

Cerebral edema

226
Q

Chronic alcohol use –> what CNS effects (2)

A

1) Cerebellar degeneration, neuronal loss
- Purkinje cells in cerebellum particularly susceptible

2) Cerebral atrophy, especially damage to white matter early in disease
* Vitamin deficiency in alcoholics may also play a role in addition to direct toxicity

227
Q

Fetal Alcohol Syndrome symptoms

A

hyperreactivity, poor motor skills, learning difficulties

Severely affected kids have mental retardation

228
Q

Hepatic Encephalopathy is due to…

A

elevation of blood ammonia, above 200 ug/dL

229
Q

Hepatic encephalopathy is precipitated by events that cause ammonia levels to rise. These causes include… (2)

A

GI hemorrhage, severe cirrhosis

230
Q

Ammonia is derived from ______________________ and produced in _____________________

with liver failure…
with portal hypertension…

A
  • derived from catabolism of proteins
  • produced in colon from protein by urease-containing bacteria

With liver failure, ammonia NOT converted to urea

With portal hypertension, ammonia bypasses the liver completely

231
Q

Pathogenesis of hepatic encephalopathy

A

Ammonia readily crosses BBB → taken up by Alzheimer type II astrocytes → astrocytes rich in glutamine synthetase

→ convert Glutamate + NH4+ → glutamine → glutamine then taken up by neurons and converted back to glutamate
=EXCITOTOXICITY

Results in edema and damage to the cortex and basal ganglia

232
Q

Treatment of hepatic encephalopathy (3)

A

restrict protein
suppress bacteria with abx
use lactulose to acidify colon contents

233
Q

Symptoms of hepatic encephalopathy (2)

A

1) confusion

2) asterixis

234
Q

Non-Wilsonian Hepatocerebral Degeneration

A

Form of Chronic Hepatic Encephalopathy

-due to repeated bouts of HE –> hronic liver failure and irreversible neurological damage

235
Q

Wilson’s Disease

A

inherited disorder of copper metabolism → liver cirrhosis

Produces identical neurological changes to HE (damage to basal ganglia)

Damage due to repeated bouts of HE and NOT due to direct brain toxicity by copper

236
Q

Symptoms of Wilson’s Disease (2)

A

Jaundice (around 12 yrs old) and/or movement disorder (due to basal ganglia damage)

237
Q

Wernicke-Korsakoff Syndrome

A

oculomotor abnormalities and mental symptoms

Vitamin B1/Thiamine deficiency

Most common in alcoholics or patients with hyperemesis

Example of toxic-metabolic problem that shows selective vulnerability

238
Q

Wernicke’s Encephalopathy (3 symptoms)

A

1) Ocular disturbances (nystagmus, ocular motility problems)
2) Changes in mental state (confusion)
3) Unsteady gait (ataxia)

due to thiamine (B1) deficiency

239
Q

Korsakoff’s Psychosis

A

1) severe memory impairment without any intellectual disabilities
- often accompanies wernicke’s encephalopathy

Due to thiamine (B1) deficiency

240
Q

Wernicke-Korsakoff Synfrome effect on brain

A

Mammillary bodies involved in almost all cases

Also involves hypothalamus, medial thalamus, periaqueductal grey, floor of 4th ventricle

Causes hemorrhage and necrosis especially in mamillary bodies

Histology varies with stage and severity

Edema, necrosis, demyelination, neuron loss, gliosis

241
Q

Cobalamin Deficiency

3 main consequences

A

vitamin B12 deficiency

1) Megaloblastic anemia
2) Neurological complications → cognitive deficits, subacute combined degeneration
3) Spinal cord disease → involvement of ascending (sensory) and descending (motor) tracts in spinal cord (myelin tracts)

242
Q

Vitamin B12 absorption

A

B12: main dietary source is animal products (meat, dairy)

Intrinsic Factor: glycoprotein produced by parietal cells in stomach

IF binds B12 in stomach
–> Without IF, less than 1% of dietary B12 absorbed

B12-IF complex transported to terminal ileum
→ binds receptors, absorbed

243
Q

How to get B12 deficiency (3)

A

1) Strict vegan diet
2) Problem with malabsorption
3) Autoimmune disorder - ab to gastric parietal cells / intrinsic factor = Pernicious Anemia

**Vitamin stored in many tissues, depleted in 3-4 years

244
Q

Symptoms of Cobalamin Deficiency (3)

A

symptoms develop over weeks

initial:

1) slight ataxia
2) numbness in LE
3) tingling in lower extremities

Can progress rapidly to spastic weakness or complete paraplegia

245
Q

Central Pontine Myelinolysis caused by…

A

Sustained hyponatremia due to excessive secretion of ADH (vasopressin), which prevents water diuresis when serum Na+ levels low

→ occurs when chronically hyponatremic patients have their serum Na+ rapidly corrected

246
Q

Symptoms of central pontine myelinolysis

A

pontine dysfunction with pseudobulbar palsy

247
Q

Acute Bacterial Meningitis

A

infection of subarachnoid space

Medical emergency - start empiric abx urgently (within 60 min of arrival to ER)

248
Q

Clinical presentation of acute bacterial meningitis (9)

A

Classic triad:

1) fever
2) headache
3) neck stiffness

4) +/- altered mental status
5) Seizures
6) nausea/vomiting
7) myalgia
8) cranial nerve palsies (III, VI, VII, VIII)
9) Focal deficits (hemiparesis, ataxia, gaze preference)

249
Q

Bacteria can enter the subarachnoid space via what 3 routes

A

1) bloodstream (most common)
2) adjacent intracranial infection (sinusitis, otitis, etc.)
3) congenital / traumatic / surgical defects in skull/spinal column

250
Q

Causes of acute bacterial meningitis (5)

Less than 2 months old

A

1) Strep agalactiae (group B strep)
2) Gram-Negative Rods (E. Coli)
3) Listeria monocytogenes (in really young, really old, or alcoholics - must add Ampicillin to abx therapy)
4) Strep pneumoniae
5) H. Influenzae

251
Q

Causes of acute bacterial meningitis (4)

2-23 months old

A

1) Strep pneumoniae (50%)
2) Neisseria meningitides (10-15%)
3) Strep agalactiae (group B strep) (10-15%)
4) H. Influenzae (5-10%)

252
Q

Causes of acute bacterial meningitis (3)

23-34 years old

A

1) Neisseria meningitides (40%)
2) Strep pneumoniae (40%)
3) H. Influenzae (5-10%)

253
Q

Causes of acute bacterial meningitis (3)

> 35 years old

A

1) Strep pneumoniae (50-70%)
2) Neisseria meningitides (10-25%)
3) H. Influenzae (1-10%)

254
Q

Lumbar puncture in acute bacterial meningitis

A

1) Low glucose
2) High protein
3) Pleocytosis
4) > 80% Neutrophils

255
Q

When you gram stain your LP and it says…

Gram negative diplococci = ?

Gram positive diplococci = ?

Gram positive cocci in chains = ? or ?

Gram negative rod = ?

Gram positive rod = ?

A

Gram negative diplococci = Neisseria

Gram positive diplococci = Strep (pneumococcus)

Gram positive cocci in chains = Strep (pneumococcus or Group B) or enterococcus

Gram negative rod = enteric GNs

Gram positive rod = Listeria

256
Q

Diagnosis of acute bacterial meningitis

A

1) LUMBAR PUNCTURE is key
2) Culture and gram stain LP
3) Blood culture
4) Neuroimaging (CT/MRI)

257
Q

If you get neuroimaging prior to doing an LP in the setting of suspected acute bacterial meningitis what should you do first?

A

Neuroimaging prior to LP:

Get blood cultures and begin EMPIRIC ABX + CORTICOSTEROIDS started first

258
Q

Corticosteroids in treatment of acute bacterial meningitis

A

can be given prior to first dose of abx

continue if pneumococcus identified

259
Q

Treatment of acute bacterial meningitis:

Neonates, infants (1-3 months)

A

Ampicillin + Cefotaxime

260
Q

Treatment of acute bacterial meningitis:

3 months - 50 years

A

Ceftriaxone, Cefotaxime, Vancomycin

261
Q

Treatment of acute bacterial meningitis:

Greater than 50 years

A

Ceftriaxone + Vancomycin + Ampicillin

262
Q

Treatment of acute bacterial meningitis:

Any nosocomial acquired infection, recent head trauma/neurosurgery, immunocompromised, alcoholics

A

Vancomycin + Meropenem +/- Ampicillin

263
Q

LP of viral vs. bacterial meningitis

WBC
Cell type
Glucose
Protein
Cultures
Useful stains/tests
A 
Bacterial:
WBC = 100-10,000
Cell type = PMN (80-95%)
Glucose = low
Protein = elevated
Cultures = + (bacteria)
Useful stains = gram stain
Viral:
WBC = 10-2,000
Cell type = mononuclear (lymphocyte)
Glucose = normal
Protein = normal/slightly elevate
Cultures = + (viral) - (bacterial)
Useful stains = PCR
264
Q

LP in encephalitis

WBC
Cell type
Glucose
Protein
Cultures
Useful stains/tests
A 
WBC = 10-2,000
Cell type = Mononuclear (lympocyte)
Glucose = normal
Protein = elevated
Cultures = + viral, +/- Bacterial
Useful stains = PCR, MRI
265
Q

3 of the most common causes of viral meningitis

A

Enteroviruses
HSV-2
Arboviruses (west nile virus, WNV, Zika virus)

266
Q

Clinical Features of viral meningitis (3)

A

Headache, fever, meningeal irritation (stiff neck)

Milder than with bacterial meningitis

267
Q

Diagnosis of viral meningitis

A

CSF → normal glucose + lymphocytic pleocytosis + high protein

PCR amplification of viral genomic material from CSF = MOST important diagnostic test for viral meningitis

268
Q

Viral encephalitis

A

infection of brain tissue rather than just subarachnoid space

269
Q

Clinical features of viral encephalitis

A

1) Altered consciousness
2) fever
3) headache
4) Seizures
5) Focal neurological signs
- -> Personality changes, AMS, aphasia, hemiparesis, ataxia, cranial nerve palsies, visual field loss, tremors, myoclonus, Parkinsonism

270
Q

Causes of viral encephalitis

A

1) 60% of cases have unknown cause
2) Autoimmune encephalitis (ab to NMDA receptor) possible cause
3) Most important causes in US = Arboviruses (WNV, Zika), and HSV-1

271
Q

Diagnosis of viral encephalitis

A

1) CSF pleocytosis
2) EEG abnormalities
3) CT and MRI showing focal encephalitis
4) PCR amplification of viral nucleic acid from CSF
5) WNV IgM in CSF (diagnostic of WNV encephalitis)

Neuro (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions