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USMLE Step I Pathology > Neuro Pathology (Pathoma) > Flashcards

Neuro Pathology (Pathoma) Flashcards

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USMLE® Step 1 flashcards
1
Q

Neural Tube Defects

A

Arise from incomplete closure of the neural tube

Associated w/ low folate levels prior to conception*

Detected during prenatal care by elevated AFP in amniotic fluid and maternal blood

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2
Q

Anencephaly

A

Rostral neural tube closure defect.

Abscence of skull and brain.

Eyes become prominent –> like appearance of the fetus

Polyhydramnios (inability to swallow)

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3
Q

Spina bifida

A

Failure of posterior vertebral arch to close

Can lead to a dimple or patch of hair overlying vetebral defect (spina bifida oculta)

Spina bifida can lead to meningocele or meningnomyelocele

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4
Q

Cerebral Aqueduct Stenosis

A

Congenital stenosis of the channel that drains CSF from 3rd and 4th ventricles

Leads to accumulation of CSF in ventricles (inability to drain)

Presents w/ enlarging head circumference.

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5
Q

Dandy-Walker Malformation

A

Congenital failure of cerebellar vermis to develop

Presents w/ massively dilated 4th vent w/ an absent cerebellum; often accompanied by hydrocephalus

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6
Q

Arnold-Chiari Malformation

A

Herniation of cerebellar tonsils through the foramen magnum.

Obstruction of CSF flow may result in hydrocephalus

May occur in association w/ meningomyelocele and syringomyelia

Type I = Usually Benign
Type 2 = Symptomatic

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7
Q

Syringomyelia

A

Cystic degeneration of the spinal cord.

Arises w/ trauma or in association w/ Arnold-Chiari

Occurs at C8-T1

Sensory loss of pain and temp bilaterally in upper extremety.

Fine touch and position is spared.

Lower motor neurons can be damaged as well. Eventually sympathetic input in lateral horn can be affected –> Horner’s (PAM).

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8
Q

Poliomyelitis

A

Damage to anterior motor horn due to poliovirus infection (fecal-oral).

Lower-motor neuron disease

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9
Q

Wernig-Hoffman

A

Genetic lower motor neuron disease

Autosomal recessive

Floppy baby presentation

Death by a couple years.

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10
Q

Amyotrophic Lateral Sclerosis

A

Degenerative disorder of upper and lower motor neurons (corticospinal tract)

Anterior motor horn involement leads to LMN signs

Lateral corticospinal tract involvement leads to UMN signs.

Atrophy and weakness of hands is an early sign (lack of sensory impairment distinguishes ALS from syringomyelia)

Most cases are sporadic and arises in middle aged adults

*Zinc-copper superoxide dismutase mutation is present in some familial cases –> free radical damage of neurons

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11
Q

Friedrich Ataxia

A

Degenerative disorder of cerebellum and spinal cord tracts

Presents as ataxia (cerebellum) with loss of vibratory sense and proprioception, muscle weakness in lower extremities, and loss of DTR.

AR disorder

Unstable trinucleotide repeat disorder (GAA) in frataxin gene. (fenton reaction –> free radical damage)

Presents in early childhood

Associated w/ hypertrophic cardiomyopathy.

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12
Q

Meningitis

A

Inflammation of leptomeningies (Pia and arachnoid –> PAD)

MC due to an infectious agent

Neonates: GBS, E. coli, and Listeria

Children/Teens: Neisseria Meningitidis –> enters through nasal pharynx –> blood –> meninges

Adults and elderly –> Strep pneumo

Nonvaccinated infant –> Hib

Coxsackievirus –> MC viral cause

Immunocompromised –> Fungi

Present w/ headache, neck stiffness, and fever

Photophobia, vomiting, and altered mental status

Dx w/ LP (L4-L5 without going through pia:

Bacterial: Nphils, low glucose, posiitve gram stain and culture

Viral - lymphocytes and normal CSF glucose

Fungal - lymphocytes w/ low glucose

Complications are seen w/ bacterial meningitis: Death, hydrocephalus, hearing loss, and seizures (Healing issues)

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13
Q

Cerebrovascular Disease

A

Ischemic 85% or hemorrhagic (15%)

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14
Q

Global Cerebral Ischemia

A

Low perfusion (atherosclerosis of large vessel)

Acute decrease in blood flow (shock)

Chronic hypoxia (anemia)

Repeated episodes of hypoglycemia (insulinoma)

Clinical features:
Mild (transient confusion w/ recovery)
Severe (Diffuse necrosis –> death or coma)
Moderate global ischemia (infarcts in watershed areas and damage to highly vulnerable regions –> cortical laminar necrosis. I.e. pyramidal neurons in cerebrum, purkinje layer in cerebellum, and pyramidal neurons elsewhere)

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15
Q

Ischemic Stroke

A

Regional ischemia that results in focal neurologic deficits for >24 hrs.

Symptoms pale infarction at periphery of cortex due to an atherosclerotic plaque rupture at a branch point

  1. Embolic –> hemorrhagic infarciton at periphery of cortex usually from the left side of the heart. MC in MCA from AFIB.
  2. ) Lacunar stroke –> Secondary to hyaline arteriolosclerosis esp. in lenticulostriate vessel. Lake like infarction. Deep structures of brain.

Ischemic stroke leads to liquefactive necrosis.

Red neurons early–> neutrophils, microglial cells, and granulation tissue then ensues –> eventually results in fluid-filled cystic space surrounded by gliosis. 1 day; 1 week; 1 month

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16
Q

Intracerebral Hemorrhage

A

Bleeding into the brain meninges

MC due to rupture of Charcot-Bouchard aneurysms of lenticulostriate vessels resulting in damage to the basal ganglia (strong assn w/ HTN)

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17
Q

Subarachnoid Hemorrhage

A

Bleeding in subarachnoid hemorrhage

Presents as sudden headache w/ nuchal rigididty

LP shows xanthochromia (from heme breakdown; can be bloody or yellow)

Gross: Hemorrhage at base of brain.

Most frequently due to the rupture of berry anuerysm

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18
Q

Berry Aneurysm

A

Thin walled saccular outpouching;* lacks a media layer

Most frequently located in anterior circle of Willis (branch points of ACA)

*Associated w/ Marfans and ADPKD

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19
Q

Epidural Hematoma

A

Bleed above dura (tough to severe –> arterial pressure needed)

  • Secondary to fracture and rupture of Middle Meningeal Artery
  • Lens shaped CT
  • “Talk and Die” syndrome –> lucid interval (hrs. to 1 day) –> rupture/expansion –> fatal herniation
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20
Q

Subdural hematoma

A

Bleed under the dura.

Usually associated w/ trauma and subsequent rupture of bridging veins.

Risk factors = cortical atrophy (alcoholic, elderly, dehyrdration, shaken baby)

Presents w/ progressive neurological signs –> Herniation is lethal complication

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21
Q

Tonsillar Herniation

A

Herniation of cerebellar tonsils through foramen magnum which compresses the brainstem and can cause cardiopulmonary arrest.

22
Q

Subfalcine herniation

A

Herniation under Falx Cerebri –> can compress Anterior Cerebral Artery –> Infarction

23
Q

Uncal herniation

A

Herniation of uncus through tentorum cerebelli compressing CN III, PCA, and duret hemorrhages in brainstem

24
Q

Metachromatic Leukodystrophy

A

Deficiency of arylsulfatase; MC leukodystrophy

Myelin can’t be degraded; accumulates in lysosomes

25
Q

Krabbe Disease

A

Deficiency of galactocerebroside B-galactosidase

Galactocerebroside accumulates in macrophages

26
Q

Adrenoleukodystrophy

A

Impaired addition of coenzyme A to long chain fatty acids

FAs accumulate damaging adrenal gland and white matter.

27
Q

MS

A

AI destruction of CNS myeling and oligodendrocytes

Associated w/ HLA-DR2 (My mom has MS, but I want to be a Dr. Too!)

Associated w/ distance from equator

Women>Men.

Clinical features: Blurred vision in one eye (optic); verigo and scanning speech (brainstem –> sound drunk); and internuclear opthalmoplegia (MLF). Depends on region involved.

Can also involve: Hemiparesis or unilateral loss of sensation (cerebral white matter); lower extremity loss of sensation or weakness (spinal cord); Bowel, bladder, and sexual dysfunction (autonomic nervous system)

Dx: MRI reveals plaques, LP shows increased lymphocytes, increased Ig w/ oligoclonoal IgG bands, and myelin basic protein.

Rx: Acute : high dose steroids
Chronic: B-interferon

28
Q

Subacute Sclerosing Panencephalitis

A

Progressive debilitating encephalitis leading to death

*Due to slowly progressing, persistent infection of the brain by measles.

Infection in infancy; neuro signs later.

Characterized by viral inclusions in both gray and white matter

29
Q

Progressive Multifocal Leukoencephalopaty

A

*JC virus infection of oligodendrocytes.

Immunosuppression leads to reactivation of latent virus

Presents w/ rapidly progressive neuro signs leading to death

30
Q

Central Pontine Myelinolysis

A

Focal demylination of pons

Due to rapid IV correction of hyponatremia (esp. in alcoholic or malnourished

Classically presents as locked-in-syndrome

31
Q

Alzheimer’s Disease

A

Degenerative disease of the cortex.

MCC of dementia

Breakdown of amyloid precursor protein by Beta –> can’t be broken down –> plaques

Clinical features: Slow-onset memory loss. Progressive disorientation. Loss of learned motor skills and language. Behavior and personality changes. Patients become mute and bedridden.

*Focal neurologic deficits not seen early

Most cases are sporadic. (major risk factor is age; Epsilon4 allele of APDE –> increased AB-amyloid and decreased risk of Epsilon 2 allele (4>2!)

Early forms:

  1. ) presenilin1 (and 2)mutation
  2. ) Downsyndrome (amyloid precursor protein is found on chromosome 21)

Pathologic features: Diffuse cerebral atrophy –> narrowing of gyri and widening of sulci –> dilated ventricles = hydrocephalus ex vacuo.

Development of neuritic plaques made of AB-amyloid with entrapped neuritic processes. AB amyloid can deposit around blood vessels –> cerebral amyloid angiopathy

Neurofibrillary tangles –> flame shaped cells –> contain hyperphosphrylated Tau protein

Dx: Presumptive dx is made clinically after excluding other causes. Confirmed on autopsy.

32
Q

Vascular Dementia

A

Multifocal infarction and injury

Due to HTN, atherosclerosis, or vasculitis (i.e. consequence of moderate global cerebral ischemia –> hippocampus)

2nd MCC of dementia

33
Q

Frontotemporal Dementia (Pick’s Disease)

A

Degenerative disease of frontal and temporal cortex; spares parietal and occipital lobes

Round aggregates of tau protein in neurons and cortex

Behavioral and language sx arise early; progresses to dementia.

34
Q

Parkinson’s Disease

A

Degenerative loss of dopaminergic neurons in the substantia nigra pars compacta (basal ganglia)

BG modulates movement. Dopamine from SNpC increases signal to cortex (stimulates excitatory D1 and inhibits inhibitor D2)

Common disorder related to aging. Unkown etiology; rare cases related to drug MPTP

Clinical Features = TRAP
T -remor
R-igidity
A-kinesia/bradykinesia
P-ostural instability and shuffling gate

Round eosinophilic inclusions of alpha-synuclein in BG = Lewy Body

Dementia is a common LATE disease

Early-onset dementia suggests Lewy body dementia

35
Q

Lewy Body dementia

A

Early onset dementia w/ dementia, hallucinaitons, and parkinosnian features with CORTICAL Lewy bodies

36
Q

Huntington’s Disease

A

Degeneration of GABAergic neurons in Caudate nucleus of BG.

Loss of GABA (inhibitory) neuron –> loss of inhibitory hold on cortex–> chorea

AD disorder from trinucleotide repeats of CAG in huntingin gene

Further expansion of trinucleotide repeat occurs during *spermatogenesis –> anticipation

Presentation:
Depression; Chorea; Athetosis; Dementia.

Suicide is a common cause of death

37
Q

Normal Pressure Hydrocephalus

A

Increased CSF results in dilated ventricles

Can cause dementia in adults; usually idiopathic

Presents as triad of : urinary incontinence, gait instability, and dementia (Wet, Wacky and Wobbly)

LP improves sx; treatment is VP shunt.

Stretching of corona raidata

38
Q

Spongiform Encephalopathy

A

Due to the accumulation of misfolded prion protein (PrPc–> PrPsc (Beta-pleated sheet).

Damages to neurons and glial cells –> vaculization of cytoplasm. Spongy degeneration

Can be sporadic, inherited, or transmitted

Most common form is CJD. other forms include vCJD (mad cow in young people w/ exposure; and familial fatal insomnia (inherited form characterized by insomnia and exaggerated startle response)

39
Q

CJD

A

Usually sporadic; rarely arises due to exposure to prion infected tissue

Presesnts w/ RAPIDLY progressive dementia associated w/ ataxia and startle myoclonus.

*Spike-wave complexes seen on EEG

Results in death, usually in

40
Q

Metastatic Tumors

A

About 50% metastatic and 50% primary

Present as multiple-well circumscribed lesions at the Gray-white junciton.

MCC: 1.Lung, 2.Breast 3.Kidney

41
Q

Pilocy(S)tic Astrocytoma

A

Benign tumor of astrocytes

MC CNS tumor in children; usually arises in the cerebellum

Cystic lesion w/ a nodule growing on its wall on imaging.

Histology shows rosenthal fibers (astrocytes w/ thick eosinophilic processes)

GFAP positive

42
Q

Glioblastoma Multiforme

A

Malignant, high-grade tumor of astrocytes

MC malignant primary CNS tumor in adults

Often crosses corpus callosum (butterfly glioma)

Histology: Pseudopallisading necrosis. Endothelial cell proliferation.

GFAP is intermediate filament present.

43
Q

Ependymoma

A

Malignant tumor of ependymal cells; usually in children

MC arises in 4th ventricle; may present w/ hydrocephalus*

Peri-vascular pseduorosettes.

44
Q

Medulloblastoma

A

Malignant tumor derived from granular cells of cerebellum

*Derived from Neuroectoderm origin

Usually arises in children (MC malignant CNS tumor)

Small round blue cells; Homer-Wright rosettes may be present

Poor prognosis; tumor grows rapidly and spreads via CSF (drop metastsis*)

45
Q

Meningioma

A

Benign tumor of arachnoid cells

MC benign tumor in adults

F>M (tumor expresses estrogen receptor)

May present w/ seizures

Imaging reveals a round mass attached to the dura and compresses cortex.

Histology:Whorled cells and psammoma bodies

46
Q

Adult primary brain tumors

A

Usually supratentorial

Glioblastoma Mutliforme is MCC

Olidendroglioma and meningiomas (women) also.

47
Q

Child Brain Tumors

A

Usually infratentorial

Pilocytic Astrocytoma
Ependymoma
Medulloblastoma

48
Q

Schwannoma

A

Adult benign tumor of Schwann cells

Involves cranial or spinal nerves; esp 8th cranial nerve at cerebello-pontine-angle

Presents w/ tinnitus often or hearing deficits.

S100 positive (neurocrest origin)

Bilateral tumors seen in NF2

49
Q

Oligodendroglioma

A

Adult malignant tumor of oligodendrocytes

Calcified tumor in the white matter; usually involving the frontal lobe

May present w/ seizures.

Fried egg appearance on histology (I’d like to have some (oligo) fried eggs).

50
Q

Craniopharyngioma

A

Tumor that arises from epithelial remnants of Rathke’s pouch. Sits on top of Sella Tursica.

Presents w/ supratentorial mass in child or young adult

Optic chiasm compression leads to bilateral hemionopsia (ddx. vs pituitary adenoma**)

Calcified on imaging (from roof of mouth –> teeth are calcified.)

Benign but tends to recur after resection

USMLE Step I Pathology (14 decks)

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