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Flashcards in Nervous System Deck (87):
1

Failure of posterior vertebral arches to close

Spina bifida

2

Posterior vertebral arch defect most often limited to one or two vertebrae

Spina bifida occulta

No clinically apparent abnormalities

3

Posterior vertebral arches defect complicated by herniation of meninges through the defect

Spina bifida cystica

4

Hydrocephalus that occurs without obstruction or increased CSF production in disorders characterized by decreased cerebral mass, such as ischemic brain atrophy or advanced Alzheimer disease.

hydrocephalus ex vacuo

5

The increased volume of CSF is entirely within the ventricles

Internal hydrocephalus

6

The increased volume of CSF is confined to the subarachnoid space

External hydrocephalus

7

This is a downward displacement of the cerebellar tonsils and medulla through the foramen magnum.

Arnold-Chiari malformation

8

Neural tube defect that is almost always a characteristic of Arnold-Chiari malformation

Thoracolumbar meningomyelocele

9

Characteristics include facial abnormalities and developmental defects such as microcephaly, atrial septal defect, mental and growth retardation, and other anomalies.

Fetal alcohol syndrome

10

This autosomal dominant disorder includes nodular proliferations of multinucleated atypical astrocytes forming tubers (small white nodules scattered in the cerebral cortex and periventricular areas), adenoma sebaceum of the skin, and angiomyolipoma of thekidney.

Tuberous sclerosis syndrome

11

The most frequent sites of thrombotic occlusion in stroke

carotid bifurcation and the middle cerebral artery

12

The most frequent site of embolic occlusion in stroke

middle cerebral artery

13

Infarction caused by an obstruction of small vessels in the brain

lacunar strokes

14

Pure motor lacunar stroke most often results from lesions affecting the

internal capsule

15

Pure sensory lacunar stroke most often results from lesions affecting the

thalamus

16

Most common cause of intracerebral hemorrhage

Hypertension

17

Minute dilations at small artery bifurcations associated with hypertension and may be sites of hemorrhagic rupture in the brain

Charcot-Bouchard aneurysms

18

Most common site of intracerebral hemorrhage

Basal ganglia and thalamus

19

Hematoma due to arterial hemorrhage in the head

Epidural hematoma


Due to trauma

20

Epidural hematoma is caused by bleeding in

Middle meningeal artery

21

Clinical characteristics include a short period of consciousness (lucid interval), followed by rapidly developing signs of cerebral compression.

Epidural hematoma

22

The cause is venous bleeding, most often from bridging veins joining the cerebrum to venous sinuses within the dura.

Subdural hematoma


Gradual signs of cerebral compression
Osmotic imbibing of water resulting to slowly enlarging tumorlike mass

23

Most common portal of entry of infection in CNS

Hematogenous


No lymphatics enter the CNS

24

In neonates and infants, pyogenic meningitis is most frequently caused by what 3 organisms?

group Bstreptococci, Escherichia coli and Listeria

25

In older infants, children, and young adults, the pyogenic meningitis is most frequently caused by what 2 organisms?

Streptococcus pneumoniae (pneumococcus) and Neisseria meningitidis

26

Hemorrhagic destruction of the adrenal cortex, acute hypocorticism with circulatory collapse, and disseminated intravascular coagulation.

Waterhouse-Friderichsen syndrome

27

Most common cause of cerebral abscess

Middle ear

28

The most common agent of severe viral encephalitis.

herpes simplex virus

29

Viral infection characterized by degeneration and necrosis of anterior horn cells of the spinal cord.

Poliomyelitis

30

Location of negri bodies in rabies

Hippocampus and purkinje cells of the cerebellum

31

Giant cells with eosinophilic inclusions involving both the nucleus and cytoplasm are characteristics of what viral infection?

CMV

32

Vehicles for HIV entry into the nervous system and may serve as the viral reservoir.

Monocyte-macrophage

33

Most common CNS manifestation of HIV

AIDS dementia complex


Direct effect of HIV

34

Cause of spongiform encephalopathy

Prion disease

35

In the past, this disease was transmitted by ritual ingestion of human brain by cannibals of New Guinea

Kuru

36

Prion disease that has been putatively transmitted by corneal transplantation.

Creutzfeldt-Jakob disease (subacute spongiform encephalopathy)

37

The cause is persistent infection with an altered measles virus; patients are infected in infancy but an asymptomatic interval of several years is followed by neurologic manifestations in late childhood or early teenage years.

Subacute sclerosing panencephalitis


Lack of M component of measles virus

38

Progressive multifocal leukoencephalopathy is caused by

JC polyoma type of papovavirus


preferentially infects oligodendrocytes, thus causing demyelination

39

The most common of the demyelinating diseases.

Multiple sclerosis


Confined to CNS

40

Nystagmus, intention tremor, and scanning speech are triad of? Seen in what cases?

Charcot triad; multiple sclerosis

41

This acute inflammatory demyelinating disease primarily involves peripheral nervous system and is often preceded by viral infection, immunization, or allergic reactions.

Guillain-Barré syndrome

42

Clinical manifestations include ascending muscle weakness and paralysis beginning in the lower part of the lower extremities and ascending upward; respiratory failure and death can occur but most patients recover.

Guillain-Barré syndrome

43

Causes albumino-cytologic dissociation of CSF, or a greatly increased protein concentration with only modest increase in cell count, which is an important diagnostic finding.

Guillain-Barré syndrome

44

The most important cause of dementia.

Alzheimer disease

45

Intracytoplasmic proximal dendritic eosinophilic inclusions consisting of actin? A morphologic abnormality of?

Hirano bodies; Alzheimer's disease

46

The second most frequent cause of dementia after Alzheimer disease

Multi-infarction dementia

47

Mamillary bodies are characteristic of what brain condition?

Alcohol encephalopathy

48

Associated with hypertension and is characterized by the presence of multiple lacunar infarcts and progressive demyelination limited to the subcortical area, with characteristic sparing of the cortex.

Binswanger disease (subcortical leukoencephalopathy)

49

This disorder clinically resembles Alzheimer disease characterized by marked cortical atrophy, especially of the temporal and frontal lobes; swollen neurons; and round intracytoplasmic inclusions consisting of neurofilaments.

Pick disease

50

Mode of inheritance of huntington disease

Autosomal dominant

51

Most commonly involved site of huntington disease

Striatum (caudate nucleus and putamen) and frontal complex

52

Huntington disease is characterized by increased number of what trinucleotide repeats within the huntingtin gene?

CAG

53

Histologic manifestations include depigmentation of the substantia nigra and locus ceruleus; damaged cells contain highly characteristic eosinophilic intracytoplasmic inclusions (Lewy bodies)

Parkinson disease (paralysis agitans)

54

Degeneration of upper and lower motor neurons is characteristic.

Amyotrophic lateral sclerosis (ALS, Lou Gehrig disease)

55

Other term for Amyotrophic lateral sclerosis (ALS)

Lou Gehrig disease

56

Parkinsonism with autonomic dysfunction and orthostatic hypotension.

Shy-Drager syndrome

57

The most common form of motor neuron disease.

ALS


Morphologic characteristics include degeneration and atrophy of the lateral corticospinal tracts, as well as of the anterior motor neurons of the cord.

58

Clinical manifestations include symmetric atrophy and fasciculation (lower motor neuron signs), as well as hyperreflexia, spasticity, and pathologic reflexes (upper motor neuron signs).

ALS

59

Motor neuron diaease in which brain stem and cranial nerve involvement predominate; characteristic findings include difficulty in swallowing and speaking and termination in respiratory failure.

Progressive bulbar palsy

60

An autosomal recessive lower motor neuron disease that manifests clinically in infancy.

Werdnig-Hoffmann syndrome (infantile progressive spinal muscular atrophy)

61

In adults, the majority of intracranial tumors are

supratentorial

62

In children, the majority of intracranial tumors are

infratentorial

63

The most common primary brain tumors

Astrocytomas

64

3 Astrocytomas that do not infiltrate the brain include

pilocytic astrocytomas, pleomorphic xanthroastrocytomas, and subependymal giant cell astrocytomas

65

Diffuse astrocytomas that is classified as WHO grade II.

Low-grade fibrillary astrocytomas

66

Diffuse astrocytomas that is classified as WHO grade III.

Anaplastic astrocytomas

67

Diffuse astrocytomas that is classified as WHO grade IV.

Glioblastoma multiforme (GBM)

68

This is the most common primary intracranial malignancy

Glioblastoma multiforme (GBM)

69

This brain neoplasm is associated with marked anaplasia and pleomorphism; pronounced vascular changes with endothelial hyperplasia occur. Areas of necrosis and hemorrhage are surrounded by a “pseudopalisade” arrangement of tumor cells.

Glioblastoma multiforme (GBM)

Very poor prognosis

70

This neoplasm presents as a slow-growing tumor in the middle-age group and typically arises and characterized by closely packed cells with large round nuclei surrounded by a clear halo of cytoplasm (“fried egg” appearance)

Oligodendroglioma

71

Location of ependymoma

Fourth ventricle

72

Histologic characteristics of this CNS neoplasm include tubules or rosettes with cells encircling vessels orpointing toward a central lumen; tumor cells characteristically demonstrate blepharoplasts, rod-shaped structures near the nucleus representing basal bodies of cilia.

Ependymoma

73

This is the second most common primary intracranial neoplasm

Meningioma

74

2 WHO grade II variants of meningioma

clear cell and chordoid variants

75

2 WHO grade III variants of meningioma

papillary and rhabdoid variants

76

Histologic characteristics of this brain neoplasm include a whorled pattern of concentrically arranged spindle cells and laminated calcified psammoma bodies.

Meningioma

77

A highly malignant tumor of the cerebellum characterized by sheets of closely packed cells with scant cytoplasm arranged in a rosette or perivascular pseudorosette pattern.

Medulloblastoma

78

Oncogene amplification in neuroblastoma

N-myc

79

Hemangioblastoma occur in

Cerebellum

80

Brain neoplasm that produces erythropoietin leading to secondary polycythemia

Hemangioblastoma

81

Third most common primary intracranial neoplasm.

Acoustic neuroma (schwanomma)

82

Other term for schwanomma

Neurilemmoma

83

Histological pattern of schwanomma characterized by interlacing bundles of elongated cells with palisading nuclei

Antoni A


Antoni B: looser, less cellular pattern than Antoni A

84

This disorder is characterized by hereditary (usually autosomal recessive) night blindness with progressive loss of central vision

Retinitis pigmentosa

85

This disorder is a major cause of impaired vision in the elderly.

Macular degeneration of the aged (senile macular degeneration)

86

This is the most common form of glaucoma.

Open-angle glaucoma

87

The tumor is the prototype of the “two-hit” hypothesis of Knudson.

Retinoblastoma

First deletion (“hit”) is inherited in germ line cells in familial cases or occurs as a somatic mutation in sporadic cases.
Second deletion (“hit”) results from somatic mutation in both familial and sporadic cases.
Both deletions are required for tumor development.