NEURO Flashcards
1
Q
1A. ↑ICP #1
Mech (5)?
A
MECH:
- Mass effect (ie contusion, hematoma, tumor, edema)
- Generalized brain swelling (ie ischemic state)
- ↑venous pressure (ie ven obstruction, venous sinus thrombosis, HF)
- ↑CSF production (ie SAH, Meningitis, choroid plexus tumor)
- Obstruction to CSF flow / abs (ie Hydrocephalus, meningeal dz)
2
Q
1B. ↑ICP #2
Mech of Pres (2), Pres (11)?
A
- High CSF pressure
- Neural tis compression
PRES:
- Htn
- Bradycardia
- Lethargy
- Missile Vomiting
- Vertigo
- Headache
- Neck + Shoulder rigidity/pain
- Facial weakness
- Papilledema, visual field loss + diplopia
- Hearing distortion + tinnitus
- Anosmia
3
Q
1C. ↓ICP #1
Mech (2, incl 4 sub-mech in 1st)?
A
MECH:
- CSF leak
- Trauma
- Spontaneous dural tear
- Post-CNS surgery / Lumbar Puncture
- Post-Thoracotomy (leak into pleural space) - CSF fistula
3
Q
1D. ↓ICP #2
Pres (5, incl 2 sub-divs of 1st)?
A
PRES: Symptoms range from mild to severe.
- Headache
- Frontal or Occipital (“throbbing”)
- Postural (w/in 30s of assuming erect posture) - Neck stiffness
- Photophobia
- Dizziness
- Nausea
4
Q
1E. Communicating Hydrocephalus
Mech, Causes (3)?
A
MECH =↓CSF abs by arachnoid villi (->↑ICP).
CAUSES:
- CONGENITAL IMPAIRMENT of arachnoid villi (ie low #, improper functioning)
- Arachnoid villi
5
Q
1F. Normal Pressure Hydrocephalus
Pres (3: “Wet, Wacky, Wobbly”)?
A
PRES:
- Urinary incontinence
- Dementia REVERSIBLE (due to cerebral cortex atrophy)
- Ataxic gait
6
Q
1G. Hydrocephalus Ex Vacuo
Pres?
A
PRES: ICP NORMAL
7
Q
2A. Subfalcine Herniation
Comp?
A
COMP:
- Compression of ACA -> Infarction
8
Q
2B. Uncal Herniation
Comp (3)?
A
COMP:
- Compression of IPSI CN III -> CN III Palsy
- Compression of IPSI PCA -> CONTRA Hemianopsia (w macular sparing)
- Compression of CONTRA Crus Cerebri -> IPSI Paralysis
- > ‘False Localization’ sign (dysfunction distant from expected anatomical location of pathology)
9
Q
2C. Cerebellar Tonsil Herniation
Assoc, Comp?
A
ASSOC:
- Chiari II Malformation
COMP:
- Compression of Brainstem -> Coma + Death
10
Q
3A. Cerebellar Pathologies
General Pres?
A
Disturbances of balance, gait + coordinated motor actions.
** NO paralysis. NO inability to start/stop movement. **
11
Q
3B. LATERAL Cerebellar Lesion
General Pres?
A
Propensity to FALL towards INJURED / IPSILATERAL side.
12
Q
3C. MEDIAL Cerebellar Lesion
General Pres (5)?
A
- Bilateral motor deficits
- Truncal Ataxia
- Wide Gait
- Head tilting
- Nystagmus
13
Q
3D. Cavernous Sinus Sx
CN affected, Etiologies (3), Pres (3)?
A
CN 6 (Abducens nerve) commonly affected.
ET:
- Mass effect
- Fistula
- Thrombosis
PRES:
- Opthalmoplegia
- ↓Corneal sensation
- ↓Maxillary sensation
14
Q
3E. Kluver-Bucy Sx
Lesion Location, Pres (5 “Hypers” + 1)?
A
Bilateral Amygdala lesion.
PRES:
- Hypermetamorphosis: approaching of visual stimuli as if
they were new
- Hyperorality
- Hyperphagia
- Hypersexuality (with loss of sexual preference)
- Hyperdocility
- Anterograde Amnesia
15
Q
3F. Horner Sx (sympathetic chain lesion)
Def’n, Assoc (2, incl 3 conditions that produce 1st),
Pres (“PAM”)?
A
Interruption of Sym nerve supply to eye.
ASSOC:
- Lesion of SC ABOVE T1
- Brown-Sequard Sx
- Pancoast tumor
- Syringomyelia (LATE) - Cluster Headaches (may induce)
PRES: * “PAM” *
- Ptosis
- Anhidrosis
- Miosis
16
Q
4A. “Peripheral neuropathy with sensorimotor dysfunction”
Causes?
A
CAUSES:
- Vit B12 def
17
Q
4B. UMN Facial Lesion
Lesion Locations (2 options), Pres?
A
- Motor Cortex (face area) OR
- Connection b/w cortex + Facial nucleus.
PRES = CONTRALATERAL paralysis of LOWER face.
18
Q
5A. Syringomyelia
Lesion Location, Assoc, Pres?
A
Damage to Ant White Commissure of Spinothalamic tract.
MC = C8 - T1
ASSOC:
- Arnold-Chiari II malformation
PRES: “Person with hand on burning hot element not feeling heat and not removing hand.”
- BILATERAL “cape-like” loss of temp + pain sensations
in upper extremities -> Flaccid Paralysis
19
Q
5B. MS
Lesion Locations (3),
Mech (3 steps), HLA,
Pres (Epi + TRIAD + 5),
DX (2, incl 2 findings for each)?
A
- Cerebral WM (cervical region) -> Hemiparesis
- Brainstem -> Vertigo + Scanning Speech mimics drunkeness
- ANS -> Incontinence (bladder + bowel)
MECH: Autoimmune infl
Auto-ab against myelin sheath ->Demyelination ->
Random + asymmetric lesions.
HLA-DR2.
PRES: “Young white woman” Relapsing / remitting course
- Charcot’s Triad 1: “Intention tremor, Scanning speech, Nystagmus”
- Vertigo
- Hemiparesis
- Bilateral Internuclear Opthalmoplegia
- Blurred vision in 1 eye
- Incontinence (bladder + bowel)
DX:
- FLAIR MRI: *Gold standard*
-> GREY periventricular plaques (areas of oligo loss)
\+ axon destruction
-> Multiple WHITE matter lesions (areas of demyel)
- Lumbar Puncture:
->↑IgG with IgG bands
->↑Lymphocytes
20
Q
5C. Tabes Dorsalis
Lesion Locations (2 Tracts), Mech, Causes, Pres (General + 3), Signs (4)?
A
- Dorsal Column tract nerves
- Lateral Corticospinal tract nerves
MECH: Demyelination -> Subacute combined degeneration.
CAUSES:
- 3ry Syphilis / Neurosyphilis
PRES: BILATERAL signs BELOW lesion
- Impaired sensation + proprioception
- Sensory Ataxia
- “Shooting / lightning” pain
SIGNS:
- Romberg +
- DTRs absent
- Argyll Robertson pupils (reactive to accommodation but not to light)
- Charcot’s joints
21
Q
5D. Subacute Combined Degeneration
Lesion Locations (3: Column + 2 Tracts), Mech, Causes (2)?
A
- Dorsal Columns
- Lat Corticospinal Tracts
- Spinocerebellar Tracts
MECH: Demyelination -> Subacute combined degeneration.
CAUSES:
- Vit B12 or Vit E deficiency
- AIDS
22
Q
5E. Poliomyelitis
Causative Bug, Lesion Location, Path, Pres (5), DX (2)?
A
Polio virus (fecal-oral transmission).
Ant Motor Horn. (Virus repl in oropharynx + S.I before reaching CNS)
PATH: LMN destruction.
PRES:
- LMN signs
- Fever + malaise
- Headache
- Sore throat
- Nausea + ab pain
DX:
- Virus recovered from throat or stool
- CSF: Lymphocytes present
23
Q
5F. Werdnig-Hoffman Dz (aka Infantile Spinal Muscular Atrophy)
Lesion Location, Inher, Path, Pres (2), Prog?
A
Ant Motor Horn.
AR.
PATH: LMN destruction.
PRES:
- “Floppy baby” at birth
- Tongue fasciculations
PROG: Death ~ 7 months, however some live for a few years.
24
5G. ALS / Lou Gehrig's Dz
Genetics, Path, Pres (Epi + 2), Prog, RX?
Mutation / defect in Superoxide Dismutase.
(-> Free Radical injury to neurons)
PATH: Free Radical injury to neurons
PRES: Arises in middle-age
- Early sign = atrophy + weakness of HANDS
- Both LMN + UMN signs
PROG: Progressive and fatal
RX:
- Riluzole:↓presynaptic Glutamate release. Modestly lengthens survival.
25
5H. Brown-Sequard Sx
Def'n, Pres (6: 2 LEVEL of lesion + 3 BELOW lesion + 1)?
'Hemisection' injury of SC = injury of 1/2 SC.
PRES:
- AT LEVEL of lesion:
- IPSI loss of all sensation
- LMN signs
- BELOW lesion:
- CONTRALAT pain + temp loss (ST tract)
- IPSI proprioception, tactile + vibration loss (Dorsal Column)
- IPSI UMN signs (CS tract)
- Horner Sx if lesion above T1
26
6A. Epidural Hematoma
*Arterial = High P*
Anatomy, Etiology, Mech,
Pres (CLUE + 2), Appearance on CT (2)?
Rupture of Middle Meningeal art due to fracture of Temporal bone.
ET = Trauma
MECH: High P = rapid expansion = Transtentorial Herniation
(Dura mater SEVERED from skull).
PRES = "Lucid interval" followed by "progressive↓in consciousness."
*** Death within 6 hrs ***
CT:
- "Lens / "biconvex disk"-shaped hematoma- b/w suture pts / not crossing suture lines.
- CAN cross falx + tentorium
27
6B. Subdural Hematoma
*Venous = Low P*
Anatomy, Etiology, Mech,
Pres (4 Epi + 2 CLUES),
Appearance on CT (3)?
Rupture of Bridging Veins.
ET = Deceleration Injuries: Brain crashes into skull.
MECH: Low P = Slow bleeding.
*Gyri preserved since P distributed equally*
PRES: MC in elderly, patients on Anticoagulants, Alcoholics, Shaken Babies (all have smaller and more fragile bridging veins).
- "Gradual onset / Progressive s + s"
- "Fluctuating L.O.C."
CT:
- "Crescent-shaped" hematoma- crosses suture lines.
- "Midline shift"
- CANNOT cross falx + tentorium
28
6C. SAH
Anatomy, Etiology (2), Mech,
Pres (CLUE), Micro Finding,
Comp (2)?
Aneurysm in Circle of Willis. Spreads throughout SA space.
ET: *** NO Trauma ***
- Arterial / Venous Malformation
- Berry Aneurysm rupture
MECH =↑ICP
PRES = "Worst headache of my life" (sudden headache with nuchal rigidity)
*Rapid time course*
FINDING = Xanthochromea (RBCs in lumbar puncture with yellow on top).
COMP: 2-3 days afterward, risk of:
- Vasospasm (due to bl breakdown)
- Rebleed
29
6D. Intraparenchymal / Intracerebral Hemorrhage (ICH)
MC Lesion Locations (2), Causes (4), Mech,
Pres (Epi + 1)?
MC in Basal Ganglia + Internal Capsule.
CAUSES:
* - Systemic Htn
- Amyloid angiopathy
- Vasculitis
- Neoplasm
MECH = Microaneurysm rupture
PRES: MC in Hypertensive patients
- Neuro deficits
30
6E. Berry Aneurysm
RF (4), Seen In (3), Comp?
RF:
- Advanced age
- Black
- Smokers
- Htn
SEEN IN:
- ADPKD
- Ehlers-Danlos Sx
- Marfan's Sx
COMP: Rupture -> SAH or Hem Stroke
31
6F. Cerebrovascular Disease due to ISCHEMIA
Most Vulnerable Lesion Locations (4)?
* - Hippocampus
- Neocortex
- Cerebellum
- Watershed areas
32
6G. Ischemic Stroke
RX (incl 2 Indications)?
RX:
- tPA
* If NO risk of hem
* Within 3 - 4.5 hrs of onset
33
6H. Lacunar Ischemic Stroke
```
Lesion Locations (2), Mech (3 steps, incl vessels involved),
Imaging, Comp?
```
- Thalamus -> Pure SENSORY Stroke
- Internal Capsule -> Pure MOTOR Stroke
MECH: Lenticulostriate vessels:
Htn -> Hyaline Arteriosclerosis -> Infarction (small cystic areas).
IMAGING: Small cystic areas of infarction
COMP:
- Hemibalism
34
6I. Thrombotic Ischemic Stroke
Mech, Imaging?
MECH: Clot formation, usually over atherosclerotic plaque,
at site of infarction.
IMAGING: PALE infarct (ie non-hem) at periphery of cortex
35
6J. Hypoxic Ishemic Stroke
Lesion Location, Mech, Assoc / Cause?
MC affects Watershed areas.
MECH = Hypoperfusion / Hypoxemia
ASSOC:
- Common during CV surgeries
36
6K. Damage to Watershed Zones
Def'n of Watershed Zones, Damage Seen In (2), Pres (2)?
Watershed Zones = areas lying B/W ANT + Middle Cerebral art and B/W POST + Middle Cerebral art.
SEEN IN:
- Severe Hypotension
- Hypoxic Ischemic Stroke
PRES:
- Upper Arm / Upper Leg weakness
- Defects in higher-order visual processing
37
6L. T.I.A
Def'n, Pres?
FOCAL ischemia lasting
PRES: Brief and reversible episode of focal neuro dysfunction.
38
6M. Global Cerebral Ischemia
Causes (4), Pres (Mild vs Moderate vs Severe)?
CAUSES:
- ↓perfusion (ie Atherosclerosis)
- Acute ischemia /↓in bl flow (ie Cardiogenic Shock)
- Chronic hypoxia (ie Anemia)
- Repeated hypoglycemic episodes (ie Insulinoma)
PRES: Based on magnitude + duration of insult.
- MILD -> Transient confusion with prompt recovery
- MODERATE (ie in Watershed Areas)
- SEVERE -> Vegetative state
39
6N. Hemorrhagic Stroke (intracerebral bleeding)
MC Lesion Location, Causes (5)?
MC in Basal Ganglia.
CAUSES:
- Htn
- Anticoagulation
- Cancer (abnormal vessels / vessel fragility)
- Aneurysm rupture
- Ischemic Stroke followed by reperfusion (vessel fragility)
40
7A. Guillain-Barre Sx (acute autoimmune infl demyelinating polyneuropathy)
Pres (2)?
PRES:
- Rapidly progressive leg weakness that ascends
- Following Upper Resp or GI infection (ie c jejuni)
41
7B. Acute Infl Demyelinating Polyradiculopathy
*MC variant of Guillain-Barre Sx*
Mech (2 steps), Assoc, Etiologies (3),
Pres (2), CSF Findings,
RX (3)?
MECH:
Autoimmune destruction of Schwann cells + peripheral myelin ->
Demyelination and Infl of peripheral nerves + motor fibers.
ASSOC:
- C jejuni + CMV infections
ET: (etiologies for autoimmune attack of peripheral myelin)
- Molecular mimicry
- Inoculations
- Stress
PRES:
- "Symmetric ASCENDING muscle weakness / paralysis beginning in lower extremities"
- Papilledema (due to infl /↑in plasma proteins)
FINDINGS:
- CSF: 'Albuminocytologic Dissociation' =↑CSF protein with normal cell count
RX:
- Resp support
- Plasmapheresis
- IV Ig
42
7C. Charcot-Marie Tooth Disease
(HMSN: Hereditary Motor + Sensory Neuropathy)
Lesion Locations, Def'n, Inher, Pres (2)?
- Myelin sheath
- Peripheral nerves
Group of hereditary progressive nerve disorders.
Related to defective production of PROTEINS involved in
myelin sheath and/or peripheral nerves.
AD (typically).
PRES:
- Scoliosis
- Foot deformities: flat or high ARCHES
43
7D. Progressive Multifocal Leukoencephalopathy (PML)
```
Lesion Location (Cells), Def'n + Causative Bug, RF,
Pres (Epi + General), Prognosis?
```
Oligodendrocytes.
DESTRUCTION of Oligo by JC virus -> Demyelination of CNS.
RF:
- Natalizumab
PRES: 2-4% of AIDS patients (immunosup -> reactivation of latent viral infection)
- RAPIDLY prog neuro signs
PROG: Usually fatal
43
7E. Acute Disseminated (Post-infectious/-immunization) Encephalomyelitis
Etiologies (2 Categories), Def'n?
Demyelination + PERIVENULAR Infl (multifocal).
ET:
- Measles or VZV infection
- Rabies or Smallpox vaccination
44
7F. Adrenoleukodystrophy
```
Lesion Locations (3), Inher, Mech (3 steps),
Pres (Epi + General)?
```
- Nervous System
- Adrenal gland
- Testes
X-Linked.
MECH:
Impaired addition of Coenzyme A to Long-Chain F.A ->
Buildup of Long-Chain F.A ->
Damage to NS, Adrenal gland, Testes.
PRES: MC in males. Progressive disease that can lead to adrenal crisis and coma / death.
45
7G. Krabbe Disease
```
Lesion Location (Cells), Inher, Mech (3 steps),
Pres (3), Micro Finding?
```
Macrophages.
AR.
MECH: *Destruction of CNS Myelin*
Def of Galactocerebrosidase ->
Buildup of Galactocerebroside + Psychosine in Macrophages ->
DESTRUCTION of myelin sheath (CNS).
PRES:
- Devel delay
- Optic atrophy
- Peripheral neuropathy
FINDING:
- Globoid cells
46
7H. Metachromatic Leukodystrophy
Lesion Location (Cells), Inher, Mech (5 steps), Pres (2)?
Oligodendrocytes (lysosomes of).
AR.
```
MECH: *Destruction of Oligodendrocytes*
Deficiency of arylsulfatase A ->
Buildup of Sulfatides ->
Impaired PRODUCTION of myelin sheath ->
Myelin can't be degraded -> Myelin accum in Oligo lysosomes ->
Central + Peripheral demyelination.
```
PRES:
- Ataxia
- Dementia
47
7I. Central Pontine Myelinosis
Lesion Location, Causes, Pres (Epi + 1)?
Pons (FOCAL demyelination).
CAUSES:
- RAPID IV correction of Hyponatremia in severely malnourished patients
PRES: Severely malnourished patient
- "Locked In" Sx: acute bilateral paralysis
48
8A. Headache
Locations Affected (3), Conditions Seen In (5)?
* Pain due to irritation of these structures*
- Dura mater
- Cranial nerves
- Extracranial structures
SEEN IN:
- Arteritis
- Neoplasia
- Hydrocephalus
- Meningitis
- SAH
49
8B. Cluster Headache
Duration, Pres (2)?
15 min - 3 hr. *Repetitive*
PRES: *Repetitive brief headaches*
- Excrutiating PERIORBITAL pain with lacrimation + rhinorrhea
- (May induce Horner's Sx)
50
8C. Tension Headache
Duration?
4-6 hrs. *Constant / Steady*
50
8D. Migraine
```
Locations Affected (3), Etiology, Mech (3 substances released),
Duration, Pres (3)?
```
- Meninges
- CN 5
- Bl vessels
ET: Neurovascular disorder
MECH: Substances released:
- CGRP (Calcitonin Gene-Related Peptide)
- Substance P
- Vasoactive peptides
4-72 hrs.
PRES: *Pulsating pain*
- May have aura
- Nausea
- Photophobia / Phonophobia
52
8E. Peripheral Vertigo
Lesion Location, Etiologies (3),
'Positional Testing' Findings (Class of Nystagmus + 2 Chars)?
Inner Ear.
ET: *Inner Ear*
- Meniere's Dz
- Semicircular Canal debris
- Vestibular NERVE infection
PT FINDINGS: Unidirectional Nystagmus
- DELAY b/w onset of nystagmus + vertigo sensation
- Nystagmus may not last
52
8F. Central Vertigo
Lesion Locations (2 options), Etiologies (2),
'Positional Testing' Findings (2 Class of Nystagmus),
Pres (2 other)?
- Brainstem
- Cerebellum
ET: *Brainstem or Cerebellum*
- Post Fossa tumor
- Stroke affecting Vestibular NUCLEI
* THINK: Nuclei are CENTRAL*
PT FINDINGS:
- Bidirectional Nystagmus
- Vertical Nystagmus
PRES:
- Diplopia
- Dysmetria
54
9A. Dementia
Locations Earliest / Most Severely Affected (2), Causes (5)?
- Hippocampus
- Temporal lobe
CAUSES:
- Alzheimer's (MCC of Dementia in elderly)
- Vit B12 def
- HIV
- Syphilis
- Wilson's Dz
55
9B. Alzheimer Disease (AD)
RF, Pres (General + 3), Labs (Neurotransmitter Level),
Micro Findings (2), Imaging,
Prognosis, Comp?
RF:
- Down Sx
PRES: *Insidious onset*
- Progressive memory impairment + disorientation
- Aphasia + Apraxia
- Mood alterations + Personality changes
LABS: Ach LOW
FINDINGS:
- Neuritic / Senile Plaques: EXTRACEL b-amyloid core entangled with neuritic processes
- Neurofibrillary Tangles: INTRACEL hyperphosphorylated tau protein
- > insoluble
* ** Tangles correlate with degree of dementia ***
IMAGING: Widespread Cortical atrophy
PROG: Progression to bed-ridden state + muteness -> Death.
COMP:
- Hydrocephalus Ex Vacuo
56
9C. FAMILIAL Alzheimer's Disease
Inher, Genetics (Early Onset vs Late Onset)?
AD.
- Early Onset: APP (21)
- Late Onset: ApoE4 (19) [ApoE2 is protective!]
* APP = Amyloid Precursor Protein. APP -> b-amyloid.
* Apo = Apolipoprotein gene
57
9D. Pick's Disease (Frontotemporal Dementia)
Pres (4), Micro Finding, Imaging, Comp?
PRES:
- Personality + Behavioral changes (EARLY)
- Progressive Dementia (LATER)
- Aphasia
- Parkinsonian aspects
FINDING:
- Pick bodies: INTRACEL round aggregates of tau protein
IMAGING: Frontal + Temporal atrophy
COMP:
- Hydrocephalus Ex Vacuo
58
9E. Lewy Body Dementia
Pres (2), Micro Finding?
PRES:
- Dementia + Hallucinations
- Parkinsonian aspects
FINDING:
- Lewy Bodies: inclusions of a-synuclein (due to a-synuclein defect)
*a-synuclein = abundant protein in brain. Found mainly in presynaptic terminals.
59
9G. Creutzfeldt-Jakob Disease (CJD)
Inher, Pres (Epi + 1), Micro Finding?
85% sporadic, 15% hereditary.
PRES: Young Adults
- RAPIDLY PROG dementia + myoclonus ->
Death within 6-12 months
FINDING:
- Spongy Degeneration: INTRACEL vacuoles
60
9F. Prion Diseases
3 Subtypes, Def'n / Mech, Pres?
- Sporadic (C-J dz)
- Inherited (G-S-S sx)
- Acquired (Kuru)
Conversion of a normal, usually a-helical prion protein (PrPc) ->
b-pleated protein (PrpSc).
* PrpSc resists protease degradation
* PrpSc is transmissible
PRES: *Due to accum of PrpSc -> Spongiform Encephalopathy*
Dementia + Ataxia -> Death
61
9H. Variant CJD (Mad Cow Disease)
Etiology?
ET = Exposure to bovine spongiform encephalopathy
63
10B. Huntington Disease
Lesion Location, Inher, Molec Genetics, Mech,
Pres (Onset + 6: "AACDDP"), Labs (2 Neurotransmitter Levels)?
Striatal Nuclei: GABAergic neurons in Caudate Nucleus (Basal Ganglia).
AD.
CAG repeat expansion in huntingtin gene (chr 4) ->
Anticipation + Genomic Imprinting.
('Anticipation' due to repeat expansion during Spermatogenesis)
MECH:
Degen of Striatal Nuclei (main inhibitors of mov't) -> Hyperkinetic.
PRES: Onset = 20-40.
- Aggression
- Athetosis
- Chorea
- Dementia
- Depression
- Personality changes
LABS:
- Ach↓
- GABA ↓
63
10A. Parkinson Disease
Lesion Location, Mech (2, incl NT levels),
Pres ("TRAP" + 4), Labs (2), Micro Findings (2)?
Substantia Nigra (Striatum of Basal Ganglia).
MECH:
- Loss of dopaminergic neurons from Substantia Nigra
- > Dopamine depletion.*↓Dopamine *
- Excess Cholinergic activity. * ↑Ach *
PRES:
- Tremor: Resting "pill rolling" tremor (disappears with movement)
- Rigidity: "Cogwheel rigidity" in extremities
- Akinesia / Bradykinesia
- Postural instability (stooped)
- Shuffling gait
- Expressionless / masked facies
- Dementia (late manifestation)
- Depression
LABS:
- Ach ↑
- Dopamine ↓
FINDINGS:
- 'Lewy Bodies' in affected neurons
- Neuron depigmentation
64
11B. Tuberous Sclerosis
Def'n, Inher, Pres ("HAMARTOMAS")?
Benign tumors in Brain, Skin, Eyes, Heart + Lungs.
AD.
PRES: * "HAMARTOMAS" *
- Hamartomas (in CNS + Skin)
- Adenoma Sebaceum
- Mitral Regurg
- 'Ash-Leaf spots'
- Rhabdomyoma (Cardiac)
- (Tuberous Sclerosis)
- (O)
- MR
- Angiomyolipoma (Renal)
- Seizures
65
11A. Sturge-Weber Sx
```
Lesion Locations (2), Genetics (General), Def'n,
Pres (3 lesion types), Comp (4)?
```
- Leptomeninges
- Skin of Face
Congenital disorder. Occurs sporadically.
ANGIOMAS that involve the leptomeninges + skin of face.
PRES:
- Leptomeningeal angiomas: IPSIlat
- Pheochromocytomas
- 'Port-Wine Stains': V1 distr of trigeminal nerve
COMP:
- MR
- Seizures
- Hemiparesis
- Glaucoma
66
11C. Neurofibromatosis Type 1 (Von Recklinghausen's Dz)
Def'n + Lesion Locations (4), Inher, Genetics,
Pres (5: 2 Skin, 2 Eyes, 1 general)?
Neurofibromas (noncancerous) in:
- Brain
- Eye
- PNS
- Skin (on or just underneath)
AD.
Mutated NF1 gene on chr 17. 100% penetrant, variable expressivity.
PRES:
- 'Cafe-au-Lait' spots
- Neurofibromas in skin
- 'Lisch nodules' (pigmented hamartomas in iris)
- Optic gliomas
- Pheochromocytomas
67
11D. Neurofibromatosis Type 2
Pres?
PRES:
| - Bilateral Schwannoma
69
11E. Neurofibromatosis with severe Diastolic Htn
Pheochromocytoma
70
11F. Von Hippel-Lindau Disease
Def'n, Inher, Genetics, Pres (4 tumor types)?
Benign cysts + tumors in multiple organ systems with
*** POTENTIAL FOR MALIGNANT CHANGE ***
(eg Hemangioblastoma).
AD.
Mutated VHL (tumor suppressor) gene on chr 3.
PRES:
- Cavernous Hemangiomas in organs, skin + mucosa
- Hemangioblastomas in Brainstem, Cerebellum + Retina
- Bilateral Renal Cell Carcinoma
- Pheochromocytomas
71
12A. Glioblastoma Multiforme (Grade 4 Astrocytoma)
Cellular Origin + Lesion Location,
Pres, Micro Findings (2), Appearance on Imaging (2),
Prognosis?
Astrocytes (GFAP +).
Found in Cerebral Hemispheres.
*Can cross Corpus Callosum => "Butterfly glioma"*
PRES:
- Feeling of head fullness + headaches when standing
FINDINGS: (Brain Biopsy)
- "Pseudopalisading" pleomorphic tumor cells
- Tumor cells border central areas of hem + necrosis
IMAGING: (MRI)
- Lobulated mass
- HETEROGENEOUSLY-enhancing (enhances with contrast) lesion
PROG: Grave.
72
12B. Meningiomas
Cellular Origin, Lesion Locations (2),
Micro Finding, Appearance on Imaging (2),
Prognosis?
Arachnoid cells (external to brain).
- Convexities of hemispheres
- Parasagittal region
FINDINGS:
- Psammoma bodies
IMAGING:
- Round mass attached to Dura
- UNIFORMLY-enhancing lesion
PROG: Resectable!
73
12C. Schwannoma
Cellular Origin, Lesion Locations (2), Assoc, Prog?
Schwann cells.
- Cerebello-Pontine angle
- CN 8 (if localized here = 'Acoustic Schwannoma')
ASSOC:
- NF Type II: Bilateral Schwannoma
PROG: Resectable!
74
12D. Oligodendroglioma
Cellular Origin, Lesion Location,
Pres / Chars (3), Micro Findings (2)?
Oligodendrocytes (often calcified in oligodendroglioma).
MC in Frontal lobes.
PRES / CHARS:
* Relatively rare
* Slow-growing
* Malignant
FINDINGS:
- Oligodendrocytes = "fried egg" cells
- "Chicken wire" capillary pattern
75
12E. Pituitary Adenoma (Adult) / Craniopharyngioma (Child)
Embryological Origin, Pres (2), Micro Finding, Prognosis?
Rathke's Pouch.
PRES:
- Hypo- or Hyperpituitarism
- Bitemporal Hemianopia (due to pressure on optic chiasm)
FINDINGS:
- Calcification common (b/c Rathke's Pouch is very tooth-like)
PROG: (Craniopharyngioma)
- Tends to recur after resection
76
12F. Pilocytic (Low-Grade) Astrocytoma
Cellular Origin + Lesion Location,
Micro Finding, Appearance on Imaging,
Prognosis?
Astrocytes (GFAP +).
Cerebellum. *May be SUPRAtentorial*
FINDINGS:
- 'Rosenthal fibers'
IMAGING:
- "Cystic lesion" with "mural nodule"
PROG: Benign = good prognosis!
76
12G. Medulloblastoma (a PNET: Primitive Neuroectodermal Tumor)
Lesion Location, Chars (4), Micro Findings (2), Comp, Prog?
Anatomy (2), Microscopic findings (2), Radiosensitivity, Prognosis?
Cerebellum.
CHARS:
- Radiosensitive
- Rapidly-growing
- Highly malignant
- Spreads via CSF
FINDINGS:
- Rosettes: H-W or Perivascular Pseudo-
- "Small blue cells"
COMP:
- Can compress 4th Ventricle -> Hydrocephalus
PROG: Poor
77
12H. Ependymoma
Lesion Location, Micro Findings (2), Comp, Prognosis?
4th Ventricle.
FINDINGS:
- Rosettes: Perivascular Pseudo-
- ROD-shaped blepharoblasts (basal ciliary bodies) near nucleus
COMP:
- Hydrocephalus
PROG: Poor
78
12I. Hemangioblastoma
Lesion Location, Assoc, Pres, Micro Findings (2)?
Cerebellum.
ASSOC:
- Von Hippel-Lindau Sx (when found with Retinal Angioma)
PRES:
- Polycythemia (tumor can produce EPO)
FINDINGS:
- 'Foamy Cells'
- High vascularity
79
12J. 1ry CNS Lymphoma
CB Assoc, Pres (Topography), DDX?
EBV.
PRES: Focal OR Multiple
DDX: *Differentiate from Toxoplasmosis*
80
13A. CN 3 Palsy
Pres (CLUE + 1), Seen In (3)?
PRES:
- "Down and out" gaze
- Dilated pupil
SEEN IN:
- P Comm Stroke
- Epidural Hematoma
- Uncal Herniation
81
13B. Facial Nerve (CN 7) Palsy
Lesion Location, Mech, Assoc (5), Pres, Course, RX?
CN 7 LMN lesion = lesion to LOWER division of Facial nucleus.
MECH:
Complete destruction of Facial nucleus itself or its efferent fibres.
ASSOC:
- Herpes Simplex or Zoster (less common)
- Lyme Disease
- Sarcoidosis
- Diabetes
- Tumors
PRES: IPSILATERAL facial paralysis
- "UNILATERAL facial drooping" -> "drooping smile"
-> "inability to close eye on
affected side"
COURSE: Gradual recovery in most cases.
RX = Corticosteroids
82
14A. Niemann-Pick Disease
Mech, Pres (Epi + CLUE + 3)?
```
MECH:
Sphingomyelinase def (genetic) -> Sphingomyelin accumulation.
```
PRES: Infant
- "Cherry-red spots on macula"
- Failure to thrive
- Neurodegeneration
- Hepatosplenomegaly
83
14B. Wernicke Encephalopathy
Lesion Location, Etiology, Pres (Epi + 3), Comp?
*** Reversible ***
Atrophy of Mamillary Bodies.
ET = Thiamine deficiency (ie Alcoholics)
PRES: Alcoholics
- Confusion
- Gait Ataxia
- Ocular palsies
COMP (if thiamine def not corrected in time) = Korsakoff Sx
85
14C. Korsakoff Sx
Lesion Locations (2), Precursor, Pres (CLUE + 1)?
*** NOT reversible ***
- Mamillary Bodies
- Dorsomedial Nuclei (Hypothalamus).
PRECURSOR = Wernicke Encephalopathy / uncorrected Thiamine def
PRES:
- "Confabulation"
- Antero- + Retrograde Amnesia
86
14D. Steven-Johnson Sx
Drugs Causing (3), Pres (3 steps)?
(Epilepsy Drugs)
- Lamotrigine
- Ethosuximide
- Carbamazepine
PRES:
Prodrome of fever + malaise ->
Rapid onset of erythematous/purpuric macules (ocular, oral,genital) ->
Necrosis + sloughing of macules.
88
15A. Toxoplasmosis
Pres, Imaging?
PRES: Brain abscess in HIV
IMAGING: "Ring-enhancing" brain lesions on CT / MRI
89
15B. Congenital Toxoplasmosis
Pres (Triad)?
PRES:
1. Chorioretinitis
2. Hydrocephalus
3. Intracranial calcifications
