Uworld- Neuropathology

Front 1

b/l carpal tunnel syndrome:

Back 1

Patient on long standing hemodialysis
due to deposition of b2 micro globulin

Front 2

Polymyositis: type of inflammation?

Back 2

Endomyseal inflammation

Front 3

Dermatomyositis: type of inflammation?

Back 3

Perifascicular inflammation

Front 4

GBS: Clinical findings? Microscopic features?

Back 4

Clinical findings:
1. Ascending type of motor paralysis
2. Proximal before distal
3. Areflexia
4. Glove stocking paraethesias
5. CN VII palsy

Microscopy:
1. Segmental Demyelination
2. Endoneural inflammation

Front 5

"Floppy child syndrome": associated dz?

Back 5

Werdnig Hoffman's dz:
Anterior horn cell damage.

Front 6

Eaton Lambert syndrome? association?

Back 6

A/bodies against presynaptic ca channels-->
Interferes with ACh release--->
MUSCLE WEAKNESS

Associated with Small cell ca of lungs(Paraneoplastic condition).

Front 7

Intracerebral hemorrhage:
aneurysm associated?
risk factor?

Back 7

Charcot Bouchard aneurysm
HTN

Front 8

Intrathoracic expansion and spread of lung Ca:
associated syndromes/effects?

Back 8

1. Phrenic nerve inflitration: C3-C5
2. Recurrent laryngeal nerve infiltration
3. Horner' syndrome
4. SVC syndrome
5. Esophagus

1. Hiccups, dyspnea(diaphragm)
2. Hoarseness
3. I/L miosis , ptosis, anydrosis
4. Edema- UL, facial; headache
5. Dysphagia

("C3,4,5 keeps the diaphragm alive")

Front 9

Irreversible liquefactive necrosis: time period within which it sets in?
Replaced with?

Back 9

Within 10 days.
Replaced with astroglial scar tissue.

Front 10

First symptoms of normal pressure hydrocephalus? symptoms- reversible/irreversible?

Back 10

Urinary incontinence
Ataxia
(Dementia appears later)

helps to distinguish from alzheimer's dz


symptoms are reversible

Front 11

Normal pressure hydrocephalus: pathophysiology?

Back 11

Poor absorption thru arachnoid villi--> eventual/slow accumulation of CSF

Front 12

Pick's disease: features?

Back 12

Progressive dementia
Intracytoplasmic Pick bodies
Cortical atrophy-FRONTOTEMPORAL
Knife edge gyri
(PICK mnemonic)

Pick bodies: stain with silver stain

Front 13

Pick disease: atrophy- lobes involved/description?
Clinical presentation?

Back 13

"Walnut Brain"
Neurodegeneration with Atrophy of Frontal and Temporal Lobes;

Presents with:
1. DEMENTIA
2 .APHASIA

Front 14

Pick's dz: dx?

Back 14

Microscopy:
Pick bodies
Pick cells: swollen neurons

Front 15

Creutzfeldt-Jacob dz: pathophysiology? course of dz?

Back 15

Normally PrP has alpha helical structure.
In CJ dz: protein converts to beta pleated sheet isoform
B-pleated forms resistant to protease mediated degradation
therefore accumulate--> INTRAcellularly
Produce SPONGIFORM encephalopathy

Course: rapidly progressing dementia

Front 16

Tetanus: dx?

Back 16

Clinical dx:

History:
H/O penetrating wound
H/O past vaccination

Physical signs:
1. Trismus
2. Sardonic smile
3. Muscle spasm
4. Ophisthotonus

Front 17

% of fibers that participate in decussation?

Back 17

90%

Front 18

Anterior corticospinal tract?

Back 18

Fibers that do not participate in decussation form anterior corticospinal tract.

Front 19

Cluster headaches: symptoms?

Back 19

1. Pain-
i. Rapid onset,
ii. Severe,
iii. Non pulsatile,
iv. Periorbital,
v. U/L and
vi. Temporal
vii. Occurs on same time every day(night time)
viii. Lasts 1/2-2hrs

2. Nasal congestion
3. Ptosis
4. Lacrimation

Front 20

Tic douloureux: pain features?(compare with cluster headaches)

Back 20

Pain lasts for seconds
sudden "electric shock" like pain

Front 21

Pseudotumor cerebri: complications?

Back 21

Optic nerve atrophy--> blindness

Front 22

Triad symptoms of Meniere's dz?

Back 22

1. Tinnitus
2. Vertigo
3. Hearing loss

Front 23

Differentiate symptoms of acoustic neuroma, labyrinthitis and Meniere's?

Back 23

Acoustic neuroma: Progressive and constant
Meniere's: episodic symptoms with exacerbations and remission
Labyrinthitis: Acute symptoms(not episodic)

Front 24

Schwannoma: Microscopic features?

Back 24

1. Highly cellular areas: Antoni A
2. Myxoid low cellular areas: Antoni B
3. Palisading appearance of Antoni A
4. S100 +ve (Universal)

Front 25

Cells that function to produce Reactive gliosis(fibrosis)?

Back 25

Astrocytes

Front 26

Synaptophysin +ve cells?

Back 26

Neuronal cells

Front 27

GFAP +ve cells?

Back 27

Glial cells:
Astrocytes
Oligodendrocytes
Ependymal cells

Front 28

First step towards management of stroke?

Back 28

CT- non contrast (hyperdensity- hematoma)
(Clinically- hemorrhagic stroke sudden onset
embolic stroke evolve gradually)

Front 29

Berry aneurysm: risk factors? rupture effect?

Back 29

1. ADPKD
2. Marfan
3. ED

Rupture effect: SAH(headache- no neurologic deficit)

Front 30

Charcot Bouchard aneurysm: risk factors? rupture effect?

Back 30

HTN

Effect:
Intracerebral hemorrhage-
basal ganglia,
internal capsule,
thalamus,
pons(focal deficits)

Front 31

Lobar parenchymal hemorrhage: commonest cause?

Back 31

Amyloid angiopathy

(HTN causes hemorrhage in basal ganglia and thalamus)

Front 32

Charcot Marie Tooth dz is caused by mutation by genes responsible for synthesis of _____?

Back 32

Myelin

Front 33

Sturge Weber's syndrome: Skull radiograph finding ?

Back 33

"Tram track" calcifications

Front 34

Von Hippel-Lindau dz:
Inheritance pattern?
genetic defect: chromosome affected?
characteristic findings?

Back 34

Inheritance: AD

Chromosome 3

findings:
1. capillar hemangioblastoma in retina/cerebellum
2. cysts/neoplasms in kidney, liver, pancreas.
3. Risk for b/l RCC

Front 35

Osler-Weber-Rendu syndrome? inheritance? complications?

Back 35

Hereditary hemorrhagic telangiectasia
AD
ruptMost sensitive test for diagnosing SAH?ure of telangiectasias--> Epistaxis, GI bleed, hematuria

Front 36

Imaging study of choice/first step to Ix SAH? 2nd step?

Back 36

1. CT without contrast
2. CSF exam- xanthochromia

Front 37

Most sensitive test for diagnosing SAH?

Back 37

CSF examination showing xanthochromia

Front 38

CJD: risk factors?
clinical findings?
microscopic features?

Back 38

1. Contaminated electrodes
2. Corneal transplant
3. Growth hormone preparation

Clinicals-
Progressive dementia
Myoclonic jerks

Microscopic features:
Vacuoles in cytoplasm of-
1. neutrophils and
2. neurons
(spongiform appearance)

Front 39

1st area of the brain to be damaged during global hypoxia?

Back 39

Hippocampus

Front 40

Cells most susceptible to ischemia?

Back 40

1. Pyramidal cells of hippocampus
2. Purkinje cells of cerebellum

Front 41

Pseudobulbar palsy seen in?

Back 41

Central pontine myelinolysis
Head, neck muscle weakness
"pseudo bulbar" because nuclei of core nerves intact

Front 42

Central pontine myelinolysis: commonly affected area of pons?
Clinical findings?

Back 42

Basis pontis: Corticobulbar and corticospinal tracts.


Acute Paralysis ("Locked-In Syndrome")
Spastic Quadriparesis
Mental Changes
Dysarthria
Dysphagia
Diplopia
Loss of Consciousness

Front 43

Tremulousness in alcoholics due to?

Back 43

Ethanol binds to GABA-A receptors--> potentiates GABA effect--> sedation

Long term ethanol--> down regulates GABA receptors
--> up regulation of NMDA(excitatory)
--> stimulate synthesis of NE, dopamine, serotonin

Front 44

1st symptom to appear on withdrawal?

Back 44

Tremors

Front 45

Withdrawal symptoms? pattern of appearance etc?

Back 45

1) 5-10hrs after pt's last drink
2) Maximal intensity in 2-3 days
3) Subsides in 4-5 days after alcohol consumption

Front 46

Withdrawal symptoms?

Back 46

1. Tremors
2. Autonomic dysfunction- HR, resp, temperature
3. GI distress
4. Agitation/insomnia/anxiety
5. Tonic clonic seizures- within 48 hrs (10% of its)
6. Delirium tremens- 48-72 hrs later. fatal condition- rise in bp, temp, perspiration, hallucination, confusion

Front 47

Complications of SAH:

Back 47

1. Arterial vasospasm(surrounding the ruptured aneurysm)--> Ischemia
2. Rebleed
3. Hydrocephalus

Front 48

Dx of arterial vasospasm in SAH?

Back 48

Transcranial color doppler

Front 49

Tabes dorsalis:
Pathology?
Lapse of no of years after initial infection?

Back 49

Demyelinatination of dorsal columns and dorsal nerve roots.
20-30 years after initial infection.

Front 50

First microscopic feature to appear after ischemia? when?

Back 50

12-48 hrs later : red apoptotic neuron

time line following ischemia to neural tissues?
12-48 hrs: Red neurons
24-72 hrs: Necrosis and neutrohilic infiltration
3-5 days: Macrophage infiltration
1-2wks: Reactive gliosis/ vascular proliferation
>2wks: Glial scar
>1month: cystic area surrounded by gliosis

Front 51

Red neuron: microscopic feature?

Back 51

1. Eosinophilic cytoplasm
2. Pyknotic nuclei
3. Loss of Nissl substance(RER)

Front 52

MCC of recurrent lobar hemorrhage? prognosis?

Back 52

1. Cerebral amyloid angiopathy
good prognosis,
low mortality
benign clinical course

Front 53

Li fraumeni syndrome: genetic defect?

Back 53

Mutation of p53 on chromosome 17

Front 54

Retinoblastoma: genetic defect?

Back 54

RB1 a/oncogene defect on chromosome 13

Front 55

Subacute sclerosing pan encephalitis:
associated virus?
pathology?
Microscopy?
clinical features?
Clinical course/prognosis?

Back 55

Measles- enveloped RNA- paramyxovirus
***Missing M protein***
Missing M protein prevents the clearance of the virus from the csf and allows its persistence--->
inflammation--->gliosis and demyelination

Microscopy:
Intranuclear viral inclusions- neurons and glial cells

Slow course but fatal
Clinical features?
Non specific neurologic deficits
Progressive dementia: children

Front 56

Oligoclonal bands:

Back 56

1. MS
2. GBS
3. Subacute sclerosing panencephalitis

Front 57

Patient groups typically affected with PML?

Back 57

1. Lymphoma(afflicted)
2. Leukemics
3. HIV patients

Front 58

Dzs associated with dementia: relevant to step 1:

Back 58

1. Alzheimer's
2. Parkinson's
3. Wilson's
4. AIP
5. B12 deficiency
6. CJD
7. Neurosyphilis
8. Subacute sclerosing panencephalitis

Front 59

Huntington's: neurotransmitter defect?

Back 59

GABA decrease

Front 60

Most common cause of death in ALS?

Back 60

Respiratory infections

Front 61

ALS:
Macroscopic findings?
Microscopic findings?
Clinical?
Genetic?
Rx?

Back 61

Macro: Thin anterior roots

Micro:
Ant horn cell neuron loss
Lateral CS tracts
Loss of nuclei in CN V, IX, X, XII

Clinical:
UMN/LMN signs

Rx?
Riluzole: glutamate a/agonist
MAO: reduces glutamate release

Front 62

Pineal gland tumor:
Type?
Associated symptoms?

Back 62

Germinoma
Associated symptoms:
1. Precocious puberty- B-hcg production
2. Obstructive hydrocephalus- Aqueductal compression
3. Parinaud syndrome- compression of tectal area of brain

(POP)