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review / wrong answers > Pathoma - CNS Review > Flashcards

Pathoma - CNS Review Flashcards

1
Q

Horner Syndrome

A

Loss of sympathetic innervation on the affected side of the face secondary to stroke

Due to damage to the lateral horn of the hypothalamospinal tract (above T1)

Presents with ptosis, miosis, and anhydrosis 
No mydriasis (dilation) response to cocaine drops
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2
Q

Function of cerebrocerebellum (aka neocerebellum)

A

Found in the lateral cerebellum

Responsible for fine movements of the hands and face

Lesions here would cause dysdiadochokinesis (impairment of rapidly alternating hand movements) and intention tremor

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3
Q

Function of spinocerebellum

A

Found in the anterior vermis (central) of the cerebellum

Responsible for trunk balance, vocal control, and saccades of the eye

Area most damaged by chronic alcohol use

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4
Q

Function of vestibulocerebellum (aka archicerebellum)

A

Mostly responsible for eye movements

Damage to this area lead to problems of pursuit an nystagmus (but not saccades)

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5
Q

Fanconi Syndrome

A

Caused by taking expired Tetracyclines

Type 2 renal tubular acidosis

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6
Q

Histology of Parkinsons

A

Loss of pigment in substantia nigra

Lewy bodies – round, eosinophilic inclusions composed of alpha-synuclein

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7
Q

Parkinson’s dementia vs. Lewy Body dementia

A

Both have Parkinsonian features

Parkison’s:

  • Dementia occurs LATE in disease
  • Affects the substantia nigra

Lewy Body:

  • Dementia occurs EARLY in disease
  • Lewy bodies found in CORTEX
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8
Q

Dandy-Walker malformation

A

Congenital failure of the cerebellar vermis to develop

Presents as massive dilation of the 4th ventricle with absent cerebellum

Often accompanied by hydrocephalus

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9
Q

Arnold Chiari malformation

A

Herniation of cerebellar tonsils through the foramen magnum

Type II – obstruction of the CSF resulting in hydrocephalus

Often associated with meningiomyelocele

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10
Q

Syringomyelia

A

Cystic degeneration of the spinal cord

Caused by trauma or Arnold Chiari Type I

Occurs at C8-T1 (most inferior nerves of the arms)

Spinal involvement

  • Anterior white commissure of the spinothalamic tract (bilat loss of temp and pain in upper extremities with sparing of fine touch and proprioception)
  • May extend to anterior horn (LMN signs)
  • May disrupt lateral horn or hypothalamospinal tract (Horner syndrome)
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11
Q

Friedrich Ataxia

A

Degenerative disorder of the cerebellum and spinal cord

Cerebellum - ataxia
Spinal cord - loss of vibrations, proprioception, muscle weakness and loss of DTRs

Due to expansion of trinucleotide repeat (GAA) on the frataxin gene (impairment of mitochondrial function)

Associated with cardiomyopathy

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12
Q

Poliomyelitis

A

Cause by poliovirus (fecal-oral)

Damage to anterior horn (LMN signs)
Will be asymmetric weakness

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13
Q

Werdnig-Hoffman disease

A

Same as poliomyelitis (damage to anterior horn - LMN) but due to inherited degeneration of anterior horn

Will be symmetric weakness (vs. Poliomyelitis which will be asymmetric)

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14
Q

ALS (Amyotrophic lateral sclerosis)

A

Disorder of upper (lateral corticospinal tract) and lower motor neurons (anterior horn damage)

Can differentiate from syringomyelia because there is no loss of pain and temp

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15
Q

Progression of liquefactive necrosis secondary to ischemia in the brain

A
  • Red neurons (eosinophilic cytoplasm, pyknotic nuceli, loss of Nissl substance) appear first (12 hours after infarction)
  • Necrosis occurs at 24 hours
  • Neutrophils and microglia arrive between 1 day and 1 week
  • Healing around 1 week (reactive gliosis and vascular proliferation around the necrotic area)
  • Gliotic cyst around 1 month
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16
Q

Charcot Bouchard microaneurysm

A

Affects small vessels (such as lenticulostriate which provide to the basal ganglia)

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17
Q

Berry aneurysm

A

Most commonly occurs at circle of willis (junction of anterior communicating artery)

Rupture –> subarachnoid hemorrhage (WHOL) or hemorrhagic stroke

18
Q

Causes of global cerebral ischemia

A

1) Poor perfusion (e.g. atherosclerosis)
2) Decreased blood flow (e.g. shock)
3) Hypoxia (e.g. anemia)
4) Repeated episodes of hypoglycemia

19
Q

Metachromatic leukodystrophy

A

Leukodystrophy = problem with myelin

Lysosomal storage disease due to deficiency of arylsulfate. Myelin accumulates in the lysosomes

Most common leukodystrophy

Findings: central and peripheral demyelination with ataxia and dementia

20
Q

Krabbe disease

A

Leukodystrophy = problem with myelin

Deficiency in galactocerebrosidase

Findings: peripheral neuropathy, developmental delay, optic atrophy, globoid cells

21
Q

Adrenoleukodystrophy

A

Leukodystrophy = problem with myelin

X-linked disorder of fatty acid metabolism

Causes accumulation of fatty acids in the adrenal glands and white matter

22
Q

Charcot-Marie-Tooth disease

A

Defect in production of peripheral nerves and myelin sheath

Associate with foot deformities, lower extremity weakness, and sensory deficits

23
Q

Subacute Sclerosing Panencephalitis (SSPE)

A

Caused by measles virus

Viral inclusions within both neurons (gray matter) and oligodendrocytes (white matter) . . . hence “pan”

24
Q

Progressive multifocal leukencephalopathy

A

Caused when latent JC virus is stimulated by immunosuppression

Rapidly progressive neuro signs leading to death

25
Q

Central pontine myelinolysis

A

Focal demyelination of the pons due to rapid IV correction of hyponatremia (e.g. V2 receptor antagonists for the treatment of SIADH)

Presents as “locked in” syndrome - can’t move anything except the eyes

26
Q

Alzheimer’s

A

Degenerative disease of the cortex

Due to A-beta amyloid production from breakdown of APP

Increased risk with E4 allele of apolipoprotein E
Decreased risk with E2

Findings:

  • Cerebral atrophy (narrowing or gyri, widening of sulci, dialtion of ventricles)
  • Neuritic plaques containing AB amyloid and entangled neuritic processes
  • Neurofibrillary tangles conatining hyperphosphorylated tau proteins (tau - microtubule associated protein)
27
Q

Pick Disease

A

Degenerative disease of frontal and temporal cortex

Round aggregates of tau protein (Pick bodies)

Behavioral (frontal) and language (temporal) symptoms

28
Q

Parkinson’s

A

Loss of dopaminergic neurons in the substantia nigra

Clinical features - TRAPS
T - Tremor
R - Rigidity
A - Akinesia/bradykinesia
P - Postural instability
S - Shuffling gait

Histology:

  • Loss of pigment in substantia nigra
  • Lewy bodies (eosinophilic inclusions composed of alpha-synuclein)
29
Q

Huntington Disease

A

Degeneration of GABA neurons in the caudate nucleus (part of striatum)

Expanded trinucleotide repeats (CAG)
-Further expansion of repeats leads to anticipation

Presents with chorea
Can progress to dementia and depression

30
Q

Spongiform encephalopathy

A

Degenerative disease due to prion protein (conversion of PrP to beta-pleated form)

Cretzfledt-Jakob is most common form
-usually sporadic

Variant cJD - mad cow

31
Q

Most common primary tumors of CNS mets

A

Lung, breast, kidney

32
Q

5 cells of the CNS and their tumors in adults and kids

A

Neurons (only non-glial cell)

  • Kids - Medulloblastoma
  • Adults - None

Astrocytes (BBB)

  • Kids - Pilocytic astrocytoma
  • Adults - Glioblastoma multiforme

Oligodendrocytes (myelin)

  • Kids - None
  • Adults - Oligodendroglioma

Ependymal (line ventricles)

  • Kids - Ependymoma
  • Adults - None

Meningothelial (line the brain)

  • Kids - None
  • Adults - meningioma
33
Q

Glioblastoma Multiforme

benign/malignant, cell type, adult/kids, special characteristics, histology, markers

A

Malignant tumor of astrocytes
Most common primary malignant CNS tumor in ADULTS

“Butterfly lesion” - arises in the cerebral hemisphere and crosses the corpus callosym

Histology:
-Regions of necrosis surrounded by tumor cells (pseudopalisading)

GFAP (intermediate filament of glial cells) positive

34
Q

Meningioma

benign/malignant, cell type, adult/kids, special characteristics, histology, markers

A

Benign tumor of the arachnoid cells
Most common primary benign CNS tumor in adults
-More common in women (tumor expresses estrogen receptor)

Tumor compresses, but does not invade the cortex
Presents as seizures

Histology

  • Whorled pattern
  • Psammoma bodies may be present
35
Q

Schwannoma

benign/malignant, cell type, adult/kids, special characteristics, histology, markers

A

Benign tumor of Shwann cells (myelinate CNS)

Involves cranial or spinal nerves

  • Within cranium most frequently involves CN VIII at the cerebellopontine angle
  • Presents as loss of hearing and tinnitus

Tumor cells are S100 positive

Bilateral vestibular schwannomas found in neurofibromatosis type 2

36
Q

Oligodendroglioma

benign/malignant, cell type, adult/kids, special characteristics, histology, markers

A

Malignant tumor of oligodendrocytes

Occurs in adults

Imaging reveals a calcficied tumor in the white matter (remember that oligodendrocytes myelinate)

  • Usually involves the frontal lobe
  • May present as seizures

Histology
-Fried egg appearance

37
Q

Pilocytic astrocytoma

benign/malignant, cell type, adult/kids, special characteristics, histology, markers

A

Benign tumor of astrocytes

Occurs in children

Usually arises in the cerebellum (infratentorial)

Imaging reveals cystic lesions with a mural nodule

Histology:
Rosenthal fibers (thick eosinophilic processes of astrocytes)

GFAP positive (intermediate filament of glial cells)

38
Q

Medulloblastoma

A

Malignant tumor derived from granular cells of the cerebellum (neuroectoderm)

Occurs in children

Histology:
Small, round blue cells
Homer-Wright rosettes (small blue cells wrapped around pink areas of neuritic processes)

Poor prognosis
Spreads to the CSF
Metastasis to cauda equina - ‘drop metastasis’

39
Q

Ependymoma

A

Malignant tumor of ependymal cells (line the ventricles)

Occurs in children

Most commonly in the 4th ventricle (infratentorial)
-May present with hydrocephalus

Histology:
Perivascular pseudorosettes

40
Q

Craniopharyngioma

A

Tumor that arises from epithelial remnants of Rathke’s pouch (connection between embryonic development of anterior pituitary from the pharynx)

Occurs in children but is SUPRAtentorial (think of location of pituitary(

May compress the optic chiasm and lead to bitemporal hemianopsia

Imaging often reveals calcifications (derived from “tooth-like” tissue of the pharynx)

Benign but may recur after resection

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