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MS 2 - Unit 3 > Pathoma 9.1-9.2, 17, 18.5 > Flashcards

Pathoma 9.1-9.2, 17, 18.5 Flashcards

1
Q

What is the diagnosis? Antibodies against presynaptic Ca channels of the NMJ; Paraneoplastic syndrome, most often of a small cell carcinoma of the lung; Imparied ACh release; Proximal muscle weakness that improves with use

A

Lambert-Eaton Syndrome Eyes are usually spared; Acetylcholinesterase inhibitors do not improve symptoms; Resolves with resection of cancer

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2
Q

What is anencephaly?

A

Anencephaly is a neural tube defect that results in absence of the skull and brain - disruption of the cranial end of the neural tube; “Frog-like” appearance of fetus; Maternal polyhydramnios because fetus can’t swallow amniotic fluid

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3
Q

Protrusion of the meninges is what form of spina bifida?

A

Meningocele

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4
Q

Protrusion of the meninges AND spinal cord is what form of spina bifida?

A

Meningomyelocele

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5
Q

What is the diagnosis? Hydrocephalus in a newborn; Enlarging head circumference due to dilation of the ventricles; Accumulation of CSF in the ventricular space

A

Cerebral Aqueduct Stenosis Congenital stenosis of the channel that drains CSF from the 3rd to the 4th ventricle

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6
Q

What is the diagnosis? Massively dilated 4th ventricle with an absent cerebellum; Often accompanied by hydrocephalus

A

Dandy-Walker Malformation Congenital failure of the cerebellar vermis to develop

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7
Q

What is the diagnosis? Congenital downward displacement of cerebellar vermis and tonsils through the foramen magnum; Obstruction of CSF flow commonly results in hydrocephalus; Often seen with meningomyelocele

A

Arnold-Chiari Malformation (Type II)

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8
Q

Where is the most common place for Syringomyelia to occur?

A

C8 to T1 Syringomyelia is cystic degeneration of the spinal cord Sensory loss of pain and temperature, spares fine touch and position sense (cape-like distribution)

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9
Q

Poliomyelitis due to poliovirus results in flaccid paralysis and muscle atrophy, fasciculations, impaired reflexes and a negative Babinski sign (lower motor neuron signs). Where is the damage in the spinal cord?

A

Degeneration of anterior motor horn

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10
Q

What is the diagnosis? Inherited degeneration of the anterior motor horn; Autosomal recessive; Presents as “floppy baby;” Death occurs within a few years of birth

A

Werdnig-Hoffman Disease

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11
Q

Lack of sensory impairment distinguishes ALS from what other diagnosis?

A

Syringomyelia

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12
Q

What is the diagnosis? Degenerative disorder of the cerebellum and spinal cord; Subsequent ataxia; Loss of vibratory sense and proprioception, muscle weakness in the lower extremities, loss of deep tendon reflexes; Presents in early childhood; Complication is hypertrophic cardiomyopathy

A

Friedreich Ataxia Autosomal recessive; expansion of unstable trinucleotide repeat (GAA) in the frataxin gene

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13
Q

Frataxin is essential for what?

A

Mitochondrial iron regulation; loss results in iron build-up with free radical damage; this is the gene impaired in Friedreich Ataxia

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14
Q

Thrombotic stroke is due to:

A

rupture of an atherosclerotic plaque Atherosclerosis commonly develops at branchpoints, for example the bifurcation of IC and MCA in the circle of willis; results in pale infarct at the periphery of the cortex

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15
Q

Embolic stroke is due to:

A

Thromboemboli Most common source of emboli is the left side of the heart (ie a-fib); usually involves MCA; results in hemorrhagic infarct at the periphery of the cortex

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16
Q

Lacunar stroke is due to:

A

Hyaline arteriosclerosis Most commonly involves the lenticulostriate vessels, resulting in small, cystic areas of infarction; involvement of the internal capsule = pure motor stroke; involvement of the thalamus = pure sensory stroke

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17
Q

What is the anatomical (as opposed to clinical) result of an ischemic stroke?

A

Liquefactive necrosis Eosinophilic change –> Necrosis –> fluid-filled cystic space surrounded by gliosis

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18
Q

Intracerebral hemorrhage is bleeding into the brain parenchyma. It’s classically due to:

A

Rupture of Charcot-Bouchard microaneurysms of the lenticulostriate vessels; complication of HTN;

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19
Q

The most common site for intracerebral hemorrhage is ehre?

A

Basal ganglia Presents as severe HA, nausea, vomiting and eventual coma

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20
Q

What is the diagnosis? Sudden onset worst HA of a person’s life; Nuchal rigidity; Lumbar puncture shows xanthochromia (yellow hue from bilirubin);

A

Subarachnoid hemorrhage Can be due to rupture of a berry aneurysm (most common), AV malformations, anticoagulated state; Most frequently in the anterior circle of willis at branch points of ACA; Can be seen in Marfan syndrome and AD polycystic kidney disease

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21
Q

Epidural hematoma is blood where?

A

BEWTEEN dura and skull; trauma; fracture of temporal bone –> rupture of middle meningeal artery; Herniation is a lethal complication

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22
Q

A lens-shaped lesion on head CT suggests?

A

Epidural hematoma

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23
Q

Subdural hematoma is blood where?

A

UNDER the dura, blood covering surface of brain; presents with progressive neurologic signs; trauma; tearing of bridging veins between dura and arachnoid; Herniation is a lethal complication

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24
Q

A crescent-shaped lesion on head CT suggests?

A

Subdural hematoma

25
Q

Rupture of the paramedian artery leads to what?

A

Duret (brainstem) hemorrhage A complication of uncal herniation

26
Q

What is the diagnosis? Most common leukodystrophy; Deficiency in arylsulfatase; Autosomal recessive

A

Metachromatic Leukodystrophy, a lysosomal storage disease

27
Q

What is the diagnosis? Galactocerebroside accumulates in macrophages

A

Krabbe disease Autosomal recessive deficiency of galactocerebrosidase

28
Q

What is the diagnosis? Impaired addition of Co-A to long chain fatty acids; X-linked

A

Adrenoleukodystrophy Accumulation of fatty acids damages adrenal glands and white matter of the brain.

29
Q

Multiple sclerosis is associated with what HLA?

A

HLA-DR2

30
Q

Lumbar puncture in the diagnosis of MA would reveal?

A

Increased lymphocytes; Myelin basic protein; Increased immunoglobulins with oligoclonal IgG bands on high resolution electrophoresis

31
Q

Subacute Sclerosing Panencephalitis is a progressive encephalitis leading to death, and is a complication of:

A

Measles virus

32
Q

JC virus infection of oligodendrocytes is called:

A

PML - Progressive Multifocal Leukoencephalopathy can happen as the result of immunosuppression; rapidly progressive neurologic signs and death

33
Q

What is the diagnosis? Focal demyelination of the pons; Can result in “locked-in” syndrome (bilateral paralysis); Can be due to rapid correction of hyponatremia;

A

Central Pontine Myelinolysis

34
Q

What Apo allele is associated with DECREASED risk for Alzheimer’s Disease?

A

E2 E4 - increased risk

35
Q

What is the diagnosis? Degenerative disease of the frontal and temporal cortex; Spares the parietal and occipital lobes

A

Pick Disease

36
Q

What is the diagnosis? Degeneration of GABAergic neurons in the caudate nucleus of the basal ganglia

A

Huntington Disease

37
Q

What is a common cause of death in HD?

A

Suicide

38
Q

What is the diagnosis? Increased CSF in dilated ventricles; Can cause dementia in adults - wet, wobbly, wacky

A

Normal Pressure Hydrocephalus

39
Q

Familial Fatal Insomnia is what kind of disease?

A

Spongiform Encephalopathy Inherited form of prion disease

40
Q

T/F: In children, primary tumors are usually infratentorial.

A

True In adults, primary tumors are usually supratentorial

41
Q

What is the diagnosis? Malignant, high-grade astrocyte tumor; “Butterfly” lesion because tumor arises in the cerebrum and crosses corpus callosum; tumor cells are GFAP positive

A

Glioblastoma Multiforme

42
Q

What is the diagnosis? Benign tumor of arachnoid cells; More common in women; May present as seizures; Histology shows a whorled pattern; Psammoma bodies may be present

A

Meningioma

43
Q

What is the diagnosis? Benign tumor of Schwann cells; Involves cranial or spinal nerves most commonly CN VIII at the cerebellopontine angle (loss of hearing or tinnitus); Tumor cells are S-100 positive; Bilateral tumors are seen in Neurofibromatosis Type II

A

Schwannoma

44
Q

What is the diagnosis? Malignant tumor of oligodendrocytes; Calcified tumor of white matter usually in frontal lobe; “Fried egg” appearance of cells on biopsy

A

Oligodendroglioma

45
Q

What is the diagnosis? Benign tumor of astrocytes; Usually arises in the cerebellum; Imaging reveals a cystic lesion with a mural nodule; Biopsy shows Rosenthal fibers; Cells are GFAP positive

A

Pilocytic astrocytoma

46
Q

What is the diagnosis? Malignant tumor derived from granular cells of the cerebellum; Usually seen in children; Small round blue cells on histology; Homer-Wright rosettes may be present; Poor prognosis

A

Medulloblastoma Metastasis to the cauda equina = “drop metastasis”

47
Q

What is the Diagnosis? Malignant tumor of ependymal cells; Usually seen in children; Commonly arises in the 4th ventricle –> can present with hydrocephalus; Perivascular Pseudorosettes on biopsy

A

Ependymoma

48
Q

What is the diagnosis? Tumor that arises from epithelial remnants of Rathke’s pouch; Presents as a supratentorial mass in a child or young adult; May compress optic chiasm –> bitemporal hemianopsia; Calcifications seen on imaging (derived from “tooth-like” tissue); Benign but recurs after resection

A

Craniopharyngioma

49
Q

What cells are seen in the inflammatory infiltrate of rhinitis?

A

Eosinophils

50
Q

What is a complication of repeated bouts of rhinitis?

A

Nasal polyps

51
Q

What is the diagnosis? Triad of asthma, aspirin-induced bronchospasms, nasal polyps

A

Aspirin-intolerant asthma

52
Q

What is the diagnosis? Benign tumor of nasal mucosa composed of large blood vessels and fibrous tissue; Classically seen in adolescent males; Presents with profuse epistaxis

A

Angiofibroma

53
Q

What is the diagnosis? Malignant tumor of nasopharyngeal epithelium; Association with EBV; Classicallt seen in African children and Chinese adults; Pleomorphic keratin + epithelial cells in a background of lymphcytes

A

Nasopharyngeal carcinoma

54
Q

How does nasopharyngeal carcinoma often present?

A

Involvement of cervical lymph nodes

55
Q

What is the most common cause of acute epiglottitis , especially in nonimmunized children?

A

H. influenzae type B

56
Q

What is the diagnosis? Inflammation of the upper airway; Most common cause is parainfluenza virus; Barking cough and inspiratory stridor

A

Croup aka Laryngotracheobronchitis

57
Q

What is the diagnosis? Nodule that arises on the true vocal cord; Often due to excessive use of vocal cords, usually bilateral

A

Singer’s nodule aka Vocal Cord Nodule

58
Q

What is the diagnosis? Benign papillary tumor of the vocal cord; Due to HPV 6 and 11; Presents with hoarseness

A

Laryngeal Papilloma

59
Q

What is the diagnosis? Squamous cell carcinoma, usually arises from the epithelial lining of the vocal cord; Risk factors are alcohol and tobacco

A

Laryngeal Carcinoma

MS 2 - Unit 3 (45 decks)

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