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Flashcards in XXIII - The Nervous System Deck (195):
1

This pattern of neuronal cell injury leads to shrunken individual cell bodies and nuclei. They are prominently stained by eosin, leading to the term "red neurons."

Acute hypoxic/ischemic injury(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 860

2

These are round, faintly basophilic, PAS-positive, concentrically lamellated aggregates of polyglucosans that range between 5 and 50 μm, and are located wherever there are astrocytic end processes, especially in the subpial and perivascular zones, seen more frequently with advancing age.

Corpora amylacea (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861

3

Cells which produce myelin in the CNS.

Oligodendrocytes (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861

4

Cells which line the ventricles, and are located in the region of the obliterated central canal of the spinal cord.

Ependymal cells (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861

5

These are responsible for the secretion of CSF. It has a specialized epithelial covering with a fibrovascular stroma that may contain meningothelial cells.

Choroid plexus (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861

6

These are bone marrow-derived cells that function as the phagocytes of the CNS.

Microglia (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861

7

IT is the accumulation of excess fluid within the brain parenchyma. The brain is softer than normal and often appears to "overfill" the cranial vault. In generalized edema the gyri are flattened, the intervening sulci are narrowed, and the ventricular cavities are compressed.

Cerebral Edema (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861

8

This occurs when the integrity of the normal blood-brain barrier is disrupted. With increased vascular permeability, fluid shifts from the vascular compartment into the intercellular spaces of the brain.

Vasogenic edema (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861

9

This is due to an increase in intracellular fluid secondary to neuronal, glial, or endothelial cell membrane injury, as might be encountered in an individual with a generalized hypoxic/ischemic insult or with exposure to some toxins.

Cytotoxic edema(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 861

10

Refers to the accumulation of CSF leading to dilation of the ventricular system which resulted from an obstacle or disruption of flow seen most commonly at the foramen of Monroe or aqueduct of Sylvius.

Noncommunicating hydrocephalus (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 862

11

Refers to the accumulation of CSF leading to dilation of the ventricular system due to reduced resorption of CSF. All of the ventricular system is enlarged.

Communicating hydrocephalus (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 862

12

This refers to dilation of the ventricular system with a compensatory increase in CSF volume secondary to a loss of brain parenchyma, as may occur after infarcts or with a degenerative disease.

Hydrocephalus ex vacuo (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 862

13

This ccurs when unilateral or asymmetric expansion of a cerebral hemisphere displaces the cingulate gyrus under the edge of falx. This may be associated with compression of branches of the anterior cerebral artery.

Subfalcine (cingulate) herniation(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 862

14

This occurs when the medial aspect of the temporal lobe is compressed against the free margin of the tentorium. As the temporal lobe is displaced, the third cranial nerve is compromised, resulting in pupillary dilation and impairment of ocular movements on the side of the lesion ("blown pupil").

Transtentorial (uncinate) herniation (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 862

15

This refers to displacement of the cerebellar tonsils through the foramen magnum. This pattern of herniation is life-threatening, because it causes brain stem compression and compromises vital respiratory and cardiac centers in the medulla.

Tonsillar herniation (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 862

16

These linear or flame-shaped lesions usually occur in the midline and paramedian regions, which are hemorrhagic lesions that accompany transtentorial herniation. Presence of these lesion implies poor prognosis.

Duret hemorrhages(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 862

17

In the setting of this condition, the brain is swollen, with wide gyri and narrowed sulci. The cut surface shows poor demarcation between gray and white matter. Results from generalized reduction of cerebral perfusion, usually below systolic pressures of less than 50mmHg.

Global Cerebral Ischemia (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 863

18

These are wedge-shaped areas of infarction that occur in those regions of the brain and spinal cord that lie at the most distal fields of arterial perfusion.

Border zone ("watershed") infarcts(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 863

19

Pattern of necrosis seen in nonhemorrhagic infarcts of the brain.

Liquefaction necrosis(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 863

20

This is a disease in which amyloidogenic peptides are deposit in the walls of medium- and small-caliber meningeal and cortical vessels, which results in the weakening of the vessel wall and increases the risk of hemorrhage.

Cerebral amyloid angiopathy (CAA) (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 863

21

Hemorrhages associated with cerebral amyloid angiopathy (CAA) are called due to involvement of the cerebral hemispheres?

Lobar hemorrhages(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 863

22

The most frequent cause of subarachnoid hemorrhage. It is a thin-walled outpouching of an artery. At the neck of the aneurysm, the muscular wall and intimal elastic lamina stop short and are absent from the aneurysm sac itself; the sac is made up of thickened hyalinized intima. The adventitia covering the sac is continuous with that of the parent artery

Saccular (berry) aneurysm(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 866

23

Patients with this type of intracranial hemorrhage complains of having "the worst headache I've ever had"/ thunderclap headache.

Subarachnoid Hemorrhage (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 866

24

Most common location for berry/saccular aneurysms.

Anterior communicating artery in the Circle of WIllis (40%)(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 867

25

This is a disease in which amyloidogenic peptides are deposit in the walls of medium- and small-caliber meningeal and cortical vessels, which results in the weakening of the vessel wall and increases the risk of hemorrhage.

Cerebral amyloid angiopathy (CAA) (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 863

26

Hemorrhages associated with cerebral amyloid angiopathy (CAA) are called due to involvement of the cerebral hemispheres?

Lobar hemorrhages(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 863

27

The most frequent cause of clinically significant subarachnoid hemorrhage.

Rupture of a saccular (berry) aneurysm(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 866

28

A type of vascular malformation which are characterized by of ectatic venous channels.

Venous angiomas (varices) (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868

29

Most common location for berry aneurysms.

Anterior communicating artery in the Circle of WIllis (40%)(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 867

30

The involved vessels in this condition resemble a tangled network of wormlike vascular channels. Microscopically, they are enlarged blood vessels separated by gliotic tissue, often with evidence of prior hemorrhage.

Arteriovenous malformations (AVM)(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868

31

This is an inflammatory disorder that involves multiple small to medium-sized parenchymal and subarachnoid vessels and is characterized by chronic inflammation, multinucleated giant cells and destruction of the vessel wall. Affected individuals manifest a diffuse encephalopathic clinical picture, often with cognitive dysfunction; improvement occurs with steroid and immunosuppressive treatment.

Primary angiitis of the CNS (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868

32

A type of vascular malformation which are characterized by microscopic foci of dilated, thin-walled vascular channels separated by relatively normal brain parenchyma and occurring most frequently in the pons.

Capillary telangiectasias(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868

33

Injury which may occur from collision of the brain with the skull opposite the site of impact.

Contrecoup injury(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868

34

Minute aneurysms in vessels that are less than 300 μm in diameter, associated with chronic hypertension.

Charcot-Bouchard microaneurysms (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868

35

This leads to accumulation of arterial blood between the dura and the skull. The expanding hematoma has a smooth inner contour that compresses the brain surface. Clinically, patients may experience a lucid interval between the moment of trauma and development of neurologic symptoms.

Epidural Hematoma (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 870

36

This is an inflammatory disorder that involves multiple small to medium-sized parenchymal and subarachnoid vessels and is characterized by chronic inflammation, multinucleated giant cells and destruction of the vessel wall. Affected individuals manifest a diffuse encephalopathic clinical picture, often with cognitive dysfunction; improvement occurs with steroid and immunosuppressive treatment.

Primary angiitis of the CNS (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868

37

This describes reversible altered consciousness from head injury in the absence of contusion. The characteristic transient neurologic dysfunction includes loss of consciousness, temporary respiratory arrest, and loss of reflexes.

Concussion(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 869

38

Injury which may occur from collision of the brain with the skull opposite the site of impact.

Contrecoup injury(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868

39

These are wedge shaped lesions of the brain parenchyma which occur secondary to trauma. The broad base of the wedge spans the surface and centered on the point of impact.

Contusions(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 868

40

Clinically, patients may experience a lucid interval between the moment of trauma and development of neurologic symptoms.

Epidural Hematoma (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 870

41

Blood vessel involved in epidural hematoma.

Middle meningeal artery(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 870

42

This describes reversible altered consciousness from head injury in the absence of contusion. The characteristic transient neurologic dysfunction includes loss of consciousness, temporary respiratory arrest, and loss of reflexes.

Concussion(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 869

43

This is a diverticulum of malformed CNS tissue extending through a defect in the cranium. It most often involves the occipital region or the posterior fossa.

Encephalocele(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

44

Blood vessels involved in subdural hemorrhage.

Bridging veins(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 870

45

This is an extension of CNS tissue through a defect in the vertebral column, most commonly in the lumbosacral region. both The meninges and spinal cord parenchyma are included in the cystlike structure visible just above the buttocks.

Myelomeningocele(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

46

Herniation of the meninges through a defect in the vertebral column.

Meningocoele(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

47

This is a malformation of the anterior end of the neural tube, with absence of the brain and top of skull.

Anencephaly(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

48

This is a diverticulum of malformed CNS tissue extending through a defect in the cranium. It most often involves the occipital region or the posterior fossa.

Encephalocele(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

49

Cerebral malformation characterized by an enlarged posterior fossa. The cerebellar vermis is absent, and replaced by a large, midline cyst that is lined by ependyma and is contiguous with leptomeninges on its outer surface.

Dandy-Walker malformation (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

50

Characterized by an increased number of irregularly formed gyri that result in an irregular bumpy or cobblestone-like surface.

Polymicrogyria(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

51

Characterized by a disruption of the normal midline patterning. In severe forms the brain is not divided into hemispheres or lobes and may be associated with facial midline defects like cyclopia.

Holoprosencephaly (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

52

Consists of a small posterior fossa and a misshapen midline cerebellum with downward extension of vermis through the foramen magnum; hydrocephalus and a lumbar myelomeningocele are typically also present.

Arnold-Chiari malformation Type II(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

53

Consists of low-lying cerebellar tonsils which extend through the foramen magnum at the base of the skull. This can lead to obstruction of CSF flow and compression of the medulla, resulting in symptoms of headache or cranial nerve deficits.

Arnold-Chiari malformation type I(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

54

Cerebral malformation characterized by an enlarged posterior fossa. The cerebellar vermis is absent, and replaced by a large, midline cyst that is lined by ependyma and is contiguous with leptomeninges on its outer surface.

Dandy-Walker malformation (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

55

Spinal cord abnormality characterized by a discontinuous or confluent expansion of the ependyma-lined central canal of the cord.

Hydromyelia(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 873

56

CSF findings in bacterial meningitis.

Increased pressureabundant neurophilsprotein - elevatedglucose - reduced(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874

57

This is an inflammatory process of the leptomeninges and CSF within the subarachnoid space.

Meningitis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874

58

In acute cases of this condition, an exudate is evident within the leptomeninges over the surface of the brain. On microscopic examination, neutrophils fill the entire subarachnoid space in severely affected areas or may be found predominantly around the leptomeningeal blood vessels.

Acute meningitis(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874

59

Caused by invasion of the brain by Treponema pallidum and manifests as insidious but progressive loss of mental and physical functions with mood alterations (including delusions of grandeur), terminating in severe dementia. There is parenchymal damage particularly in the frontal lobe, characterized by loss of neurons with proliferation of microglia (rod cells) and gliosis.

Paretic neurosyphilis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 875

60

A form of neurosyphilis, resulting from damage to the sensory nerves in the dorsal roots producing impaired joint position sense and resultant ataxia, loss of pain sensation, leading to skin and "Charcot joints". There is loss of both axons and myelin in the dorsal roots, with pallor and atrophy in the dorsal columns of the spinal cord.

Tabes dorsalis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 875

61

CSF findings in bacterial meningitis.

Increased pressureabundant neurophilsprotein - elevatedglucose - reduced(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874

62

Presents with generalized symptoms of headache, malaise, mental confusion, and vomiting. The subarachnoid space contains a gelatinous or fibrinous exudate, most often at the base of the brain, obliterating the cisterns and encasing cranial nerves. There may be discrete white granules scattered over the leptomeninges. Obliterative endarteritis may be seen.

Tuberculous meningitis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874

63

CSF findings in tuberculous meningitis.

moderate pleiocytosis - predominantly monocytesprotein - elevatedglucose - medorately reduced/normal(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874

64

Caused by invasion of the brain by Treponema pallidum and manifests as insidious but progressive loss of mental and physical functions with mood alterations (including delusions of grandeur), terminating in severe dementia.

Paretic neurosyphilis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 874

65

This virus tends to localize in the paraventricular subependymal regions of the brain, which results in a severe hemorrhagic necrotizing ventriculoencephalitis and choroid plexitis.

Cytomegalovirus (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 877

66

These are discrete lesions with central liquefactive necrosis and a surrounding fibrous capsule. On microscopic examination, there is exuberant neovascularization around the necrosis that is responsible for the marked edema and formation of granulation tissue. Outside the fibrous capsule is a zone of reactive gliosis.

Brain Abscesses (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 876

67

The pathognomonic finding of this disease is a spongiform transformation of the cerebral cortex and deep gray matter structures (caudate, putamen); consists of a multifocal process that results in the uneven formation of small, empty, microscopic vacuoles of varying sizes within the neuropil and sometimes in the perikaryon of neurons.

Creutzfeldt-Jakob Disease (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 880

68

Viral encephalitis which involves inferior and medial regions of the temporal lobes and the orbital gyri of the frontal lobes. Perivascular inflammatory infiltrates are usually present, and Cowdry type A intranuclear viral inclusion bodies can be found in both neurons and glia.

Herpes Encephalitis(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 876

69

Reactivation of varicella in adults manifests as a painful, vesicular skin eruption in the distribution of one or a few dermatomes.

Shingles / Herpes Zoster(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 876

70

This virus tends to localize in the paraventricular subependymal regions of the brain, which results in a severe hemorrhagic necrotizing ventriculoencephalitis and choroid plexitis.

Cytomegalovirus (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 877

71

This lesion caused by JC virus, consist of patches of irregular, ill-defined destruction of the white matter that enlarge. Each lesion is an area of demyelination, in the center of which are scattered lipid-laden macrophages and a reduced number of axons.

Progressive Multifocal Leukoencephalopathy (PML) (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 877

72

These are predominantly benign tumors of adults, usually attached to the dura, and arising from the meningothelial cell of the arachnoid. Histologic patterns include syncitial, fibroblastic, psammomatous, secretory and microcystic.

Meningiomas(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 885

73

Five most common primary sites of carcinoma which may metastasize to the brain.

LungBreastSkin (melanoma)KidneyGIT(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 885

74

These are infiltrative tumors that form gelatinous, gray masses, and may show cysts, focal hemorrhage, and calcification. On microscopic examination, the tumor is composed of sheets of regular cells with spherical nuclei containing finely granular chromatin (similar to normal oligodendrocytes) surrounded by a clear halo of cytoplasm.

Oligodendrogliomas(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 883

75

A nonimmune process characterized by loss of myelin involving the center of the pons, most often after rapid correction of hyponatremia.

Central pontine myelinolysis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 889

76

These are inherited dysmyelinating diseases in which the clinical symptoms derive from either abnormal myelin synthesis or turnover.

Leukodystrophies (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 889

77

These are predominantly benign tumors of adults, usually attached to the dura, and arising from the meningothelial cell of the arachnoid. Histologic patterns include syncitial, fibroblastic, psammomatous, secretory and microcystic.

Meningiomas(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 885

78

Profound memory disturbances as a result of thiamine deficiency.

Korsakoff syndrome(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 890

79

An autoimmune demyelinating disorder characterized by distinct episodes of neurologic deficits, separated in time, attributable to white matter lesions that are separated in space. Affected areas show multiple, well-circumscribed, slightly depressed, glassy, gray-tan, irregularly shaped lesions, termed "plaques".

Multiple Sclerosis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 887

80

A nonimmune process characterized by loss of myelin involving the center of the pons, most often after rapid correction of hyponatremia.

Central pontine myelinolysis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 889

81

These are inherited dysmyelinating diseases in which the clinical symptoms derive from either abnormal myelin synthesis or turnover.

Leukodystrophies (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 889

82

This condition occurs in the setting of thiamine deficiency and chronic alcoholism. Characterized by foci of hemorrhage and necrosis, particularly in the mammillary bodies but also adjacent to the ventricle, especially the third and fourth ventricles.

Wernicke encephalopathy(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 890

83

Profound memory disturbances as a result of thiamine deficiency.

Korsakoff syndrome(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 890

84

Characterized by "flapping" tremor, depressed levels of consciousness, which may lead to coma. Result of decreased hepatic function.

Hepatic encephalopathy (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 891

85

It is the most common cause of dementia in the elderly, characterized by presence of plaques and neurofibrillary tangles, composed of tau proteins.

Alzheimer's disease(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 893

86

Mutation in Friedreich ataxia.

GAA trinucleotide repeat (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 895

87

This is a clinical syndrome characterized by masked facies, stooped posture, slowness of voluntary movement, festinating gait rigidity, and a "pill-rolling" tremor. There are single or multiple, intracytoplasmic, eosinophilic, round to elongated inclusions that often have a dense core surrounded by a pale halo or "Lewy bodies".

Parkinson disease (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 894

88

It is an inherited autosomal dominant disease characterized clinically by progressive movement disorders and dementia, with degeneration of the striatum (caudate and putamen).

Huntington Disease (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 894

89

Mutation in Huntington disease.

CAG trinucleotide repeats(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 895

90

It is an autosomal recessive progressive illness, generally beginning in the first decade of life with gait ataxia, followed by hand clumsiness and dysarthria.

Friedreich ataxia (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 895

91

Mutation in Friedreich ataxia.

GAA trinucleotide repeat (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 895

92

This is the most common form of neurodegeneration affecting the motor system. It is characterized by muscle atrophy and hyper-reflexia due to loss of both upper and lower motor neurons.

Amyotrophic Lateral Sclerosis (Motor Neuron Disease; Lou Gehrig's Disease) (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 895

93

This autosomal dominant disorder is characterized by neurofibromas (plexiform and solitary), gliomas of the optic nerve, pigmented nodules of the iris (Lisch nodules), and cutaneous hyperpigmented macules (café au lait spots).

Type 1 Neurofibromatosis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 901

94

This is one of the most common life-threatening diseases of the peripheral nervous system. It may develop spontaneously or after a systemic infection (usually viral) or other stress. Usually presents with rapidly progressive, ascending motor weakness that may lead to death from failure of respiratory muscles.

Guillain-Barré Syndrome (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 899

95

These are benign tumors arising from Schwann cells, which are well-circumscribed encapsulated, firm, gray masses but may also have areas of cystic and xanthomatous change.

Schwannomas (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 899

96

Present in the dermis and subcutaneous fat, these well-delineated but unencapsulated masses are composed of spindle cells. The stroma of these tumors is highly collagenized and contains little myxoid material.

Cutaneous neurofibroma.(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 899

97

These tumors may arise anywhere along a nerve, may be multiple, and is not easily separated from the nerve.

Plexiform neurofibroma(TOPNOTCH)Robbins Basic Pathology, 8th ed., p 899

98

This autosomal dominant disorder is characterized by neurofibromas (plexiform and solitary), gliomas of the optic nerve, pigmented nodules of the iris (Lisch nodules), and cutaneous hyperpigmented macules (café au lait spots).

Type 1 Neurofibromatosis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 901

99

This is an autosomal dominant disorder in which patients develop a range of tumors, most commonly bilateral vestibular (acoustic) schwannomas and multiple meningiomas.

Type 2 Neurofibromatosis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 901

100

An autosomal dominant syndrome characterized by the development of hamartomas and benign neoplasms involving the brain and other tissues. The lesions are firm areas of the cortex that, in contrast to the softer adjacent cortex, have been likened to potatoes, hence the appellation "tubers."

Tuberous Sclerosis (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 901

101

An autosomal dominant inherited disease in characterized by development of hemangioblastomas within the cerebellar hemispheres, retina, and less commonly the brain stem and spinal cord. A cerebellar capillary hemangioblastoma, is the principal neurologic manifestation of the disease.

von Hippel-Lindau Disease (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 901

102

What is the dominant histopathologic finding in Immune mediated neuropathies like Guillain- Barre Syndrome?

Inflammation of peripheral nerve, manifested as perivenular and endoneurial infiltration by lymphocytes, macrophages, and a few plasma cells(TOPNOTCH)

103

What is the most common type of peripheral neuropathy seen in adult-onset DM?

Symmetric neuropathy that involves distal sensory and motor nerves (TOPNOTCH)

104

In patients with peripheral neuropathy, what is the predominant histologic findings?

Axonal neuropathy (TOPNOTCH)

105

Morphology: Perifascicular Atrophy

Dermatomyositis (TOPNOTCH)

106

Morphology: presence of rimmed vacuoles within myocytes and are highlighted by basophilic granules at their periphery. In addition, the vacuolated fibers may also contain amyloid deposits that stain with Congo Red.

Inclusion body myositis (TOPNOTCH)

107

Morphology: Panfascicular atrophy

Spinal Muscular Atrophy (Infantile Motor Neuron Disease (TOPNOTCH)

108

What are the 5 histopathologic abnormalities common to Duchenne Muscular Dystrophy and Becker Muscular Dystrophy?

1. Variation in fiber diameter2. Increased numbers of internalized nuclei3. Degeneration, necrosis, and phagocytosis of muscle fibers4. Regeneration of muscle fibers5. Proliferation of endomysial connective tissue (TOPNOTCH)

109

Morphology: In later stages of this disease, all muscles eventually become almost totally replaced by fat and connective tissue

DMD (TOPNOTCH)

110

What is the histologic pathology that is usually seen in DMD and is rare in BMD?

Enlarged, rounded, hyaline fibers that have lost their normal cross striation, believed to be hypercontracted fibers (TOPNOTCH)

111

Morphology: Ring fiber ( subsarcolemmal band of cytoplasm that appears distinct from the center of the fiber) and Sarcoplasmic mass

Myotonic Dystrophy (TOPNOTCH)

112

Morphology: Disuse changes with Type 2 fiber atrophy, post synaptic membrane is simplified, with loss of AChRs from the region of the synapse. Immune complexes as well as the MAC of the complement cascade can be found along the post synaptic membrane as well

Myesthenia Gravis (TOPNOTCH)

113

Electron Microscopy: tubular and filamentous inclusions are seen in the cytoplasm and the nucleus, and they are composed of B-amyloid or hyperphosphorylated tau.

Inclusion body myositis (TOPNOTCH)

114

Grotton lesions (scaling erythematous eruption or dusky red patches over the knuckles, elbows, and knees) are seen in what disease entity?

Dermatomyositis (TOPNOTCH)

115

Morphology: the most consistent pathologic finding in skeletal muscle is aggregates of abnormal mitochondria that stain with modified Gomori Trichrome and fiber appears irregular on cross section "ragged red fibers" and on electron microscope, they contain "parking lot inclusions"

Mitochondrial Myopathies (Oxidative phosphorylation Disease) (TOPNOTCH)

116

This is general reaction of the motor unit which is a result of primary destruction of the axon, with secondary disintegration of its myelin sheath.

Axonal degereration (TOPNOTCH)

117

This general reaction of the motor unit occurs when there is dysfunction of the Schwann cell or damage to the myeling sheath; there is no primary abnormality of the axon

Segmental demyelination (TOPNOTCH)

118

Morphology: endoneurial arterioles show thickening, hyalinization, and intense PAS positivity in their walls and extensive reduplicaton of the basement membrane.

Peripheral Neuropathy in DM Type 2 (TOPNOTCH)

119

Of all the dystrophies, this type is the only one that shows pathologic changes in the intrafusal fibers of muscle spindles, with fiber splitting, necrosis and regeneration

Myotonic Dystrophy (TOPNOTCH)

120

Morphology: the principal morphologic characteristic is accumulation of lipid within myocytes. The myofibrils are separated by vacuoles that stain with oil red O or Sudan black

Lipid Myopathies (TOPNOTCH)

121

Lambert Eaton Myasthenic Syndrome is a paraneoplastic process which commonly develops in what type of carcinoma?

Small cell carcinoma of the lungs (TOPNOTCH)

122

The most important histopathological indicator of CNS injury, regardless of etiology

Gliosis(TOPNOTCH)

123

Lafora bodies

Myoclonic Epilepsy(TOPNOTCH)

124

Gross morphology: gyri are flattened, intervening sulci are narrowed, and the ventricular cavities are compressed

Generalized cerebral edema(TOPNOTCH)

125

A syndrome of sudden, deep intracerebral hemorrhage that follows even minor head trauma by an interval of 1-2 weeks

Spat-apoplexie (Delayed post traumatic haemorrhage) (TOPNOTCH)

126

Subdural hematomas most often become manifest approximately how many hours after the injury?

48 hours(TOPNOTCH)

127

The most widely accepted explanation for diffuse axonal injury

mechanical forces damage the integrity of the axon at the node of Ranvier, with subsequent alterations in axoplasmic flow(TOPNOTCH)

128

Morphology: wide asymmetric distribution of axonal swellings that appear within hours of the injury and are best demonstrated with silver impregnation techniques or with immunoperoxidase stains for AB protein

Diffuse axonal injury(TOPNOTCH)

129

How long does it take for the clot to lyse in subdural hematomas?

1 week(TOPNOTCH)

130

How long does it take for fibroblasts to grow from the dural surfaces into the hematoma after subdural hematoma/

2 weeks(TOPNOTCH)

131

How long does it take for the development hyalinazed connectice tissue in SDH

1-3 months(TOPNOTCH)

132

Gross morphology: brain is swollen, gyri are widened, and the sulci are narrowed

Global cerebral ischemia(TOPNOTCH)

133

In the setting of global ischemia, early histological changes are seen after how many hours after the insult?

12-24 hours(TOPNOTCH)

134

In the setting of global ischemia, sub acute changes are seen after how many hours after the insult?

24 hours to 2 weeks(TOPNOTCH)

135

In the setting of global cerebral ischemia, repair is seen after approximately how many weeks?

2 weeks(TOPNOTCH)

136

In the setting of global ischemia, this is characterized by evenutal removal of all necrotic tissue, loss of normally organized CNS structure, and gliosis

Repair(TOPNOTCH)

137

In the setting of global ischemia This is characterized by necrosis of tissue, influx of macrophages, vascular proliferation and reactive gliosis

Subacute changes(TOPNOTCH)

138

In the setting of global ischemiaThis is characterized by acute neuronal cell damage

Early change(TOPNOTCH)

139

Morphology: pathognomonic finding is a spongiform transformation of the cerebral cortex

CJD(TOPNOTCH)

140

Morphology: mononuclear cell perivacular cuffs and neuronophagia of the anterior horn motor neurons of the spinal cord

(TOPNOTCH)

141

In the immunosuprressed individual, the most common pattern of involvement in viral meningitis is?

Subacute encephalitis(TOPNOTCH)

142

Although any type of cell within the CNS can be infected with CMV, the virus tends to localize at what particular area in the brain?

Paraventricular subependymal regions(TOPNOTCH)

143

What is the principal neurologic manifestation of Von Hippel Lindau Disease?

Cerebellar capillary hemangioblastoma(TOPNOTCH)

144

Antoni A and Antoni B patterns of growth are seen in what tumors of the CNS?

Schwannoma(TOPNOTCH)

145

Morphology: histologic appearance similar to anaplastic astrocytoma with the additional feature of necrosis and vascular or endothelial cell proliferation

Glioblastoma multiforme(TOPNOTCH)

146

Pseudopalisading

Glioblastoma multiforme(TOPNOTCH)

147

Glomeruloid body

Glioblastoma multiforme(TOPNOTCH)

148

Morphology: bipolar cells with long, thin hairlike processes that are GFAP positive; Rosenthal fibers, eosinophilic granular bodies, and microcysts are often present

Pilocytic astrocytoma(TOPNOTCH)

149

Lesion of what area of the brain appear to be the best correlate of the memory disturbance and confabulation seen in Korsakoff syndrome?

Medial dorsal nucleus of the thalamus(TOPNOTCH)

150

Morphology: foci of hemorrhage and necrosis, particularly in the mammillary bodies but also adjacent to the ventricle, especially the third and fourth ventricles

Wernicke encephalopathy(TOPNOTCH)

151

The spinal cord shows loss of axons and gliosis in the posterior columns, the distal portions of the corticospinal tracts, and the spinocerebellar tracts.

Friedreich Ataxia(TOPNOTCH)

152

The abnormalities are predominantly in the cerebellum,with loss of Purkinje and granule cells; there is also degeneration of the dorsal columns, spinocerebellar tracts and anterior horn cells and a peripheral neuropathy

Ataxia Telangiectasia(TOPNOTCH)

153

Gross morphology: palor of the substantia nigra

Parkinson Disease(TOPNOTCH)

154

Gross morphology: the brain shows pronounced, frequently asymmetric, atrophy of the frontal and temporal lobes with conspicuous sparing of the posterior two thirds of the superior temporal gyrus

Pick Disease(TOPNOTCH)

155

What are the 3 major microscopic abnormalities of Alzheimer disease?

Neuritic/Senile PlaquesNeurofibrillary tanglesAmyloid angiopathy(TOPNOTCH)

156

What is the principal clinical manifestation of Alzheimer disease?

Dementia(TOPNOTCH)

157

What is the most common cause of dementia in the elderly?

Alzheimer Disease(TOPNOTCH)

158

Gross morphology: variable degree of cortical atrophy with widening of the cerebral sulci that is most pronounced in the frontal, temporal, and parietal lobes with compensatory ventricular enlargement (hydrocephalus ex vacuo)

Alzheimer Disease(TOPNOTCH)

159

Neurotic plaques are focal, spherical collection of dilated, tortuous, silver staining neuritic processes often around a central amyloid core, which may be surrounded by clear halo, and can be stained with Congo red. What is the most predominant component of this plaque core?

AB peptide(TOPNOTCH)

160

Neurofibrillary tangles are bundles of filaments in the cytoplasm of the neurons that displace or encircle the nucleus. What is the major component of this structure?

Tau proteins(TOPNOTCH)

161

Hirano bodies

Alzheimer's disease (TOPNOTCH)

162

What is the principal neurologic manifestation of Von Hippel Lindau Disease?

Cerebellar capillary hemangioblastoma(TOPNOTCH)

163

Patients with Von Hippel Lindau Disease has the propensity to develop what type of carcinom?

Renal Cell Carcinoma(TOPNOTCH)

164

These are firm areas of the cortex that, in contrast to the softer adjacent cortex, have been likened to potatoes and are composed of haphazardly arranged neurons that lack the normal laminar organization of neocortex.

Cortical hamartomas or "tubers' of Tuberous Sclerosis (TOPNOTCH)

165

Malignant Peripheral Nerve Sheath Tumor (MPNST, Malignant Schwannoma) is strongly associated with what type of Familial Tumor Syndrome?

Neurofibromatosis Type 1(TOPNOTCH)

166

What are the 5 most common carcinoma that metastasize to the brain?

Lung, breast, Melanoma, Kidney, and GIT. (TOPNOTCH)

167

What is the most common CNS neoplasm in immunosuppressed patients, including those with AIDS and immunosuppression after transplantaion?

Primary CNS Lymphoma(TOPNOTCH)

168

Morphology: composed of sheets of regular cells with spherical nuclei containing finely granular chromatin surrounded by a clear halo of cytoplasm and calcification if present in as many as 90% of these tumors.

Oligodendroglioma(TOPNOTCH)

169

Morphology: characterized by amild to moderate increase in the number of glial cell nuclei, somewhat variable nuclear pleomorphism, and an interveing feltwork of fine, GFAP positive astrocytic cell processes that give the background a fibrillary appearance.

Well differentiated fibrillary astrocytomas(TOPNOTCH)

170

Morphology: perivascular pseudorosettes

Ependymoma(TOPNOTCH)

171

Morphology: the anterior roots of the spinal cord are thin; the precentral gyrus may be atrophic and demonstrates a reduction the number of anterior horn neurons throughout the length of the spinal cord with associated reactive gliosis. Remaining neurons often contain Bunina bdoies and PAS positive cytoplasmic inclusions.

Amyotorphic Lateral Sclerosis(TOPNOTCH)

172

Morphology: composed of bipolar cells with long, thin "hairlike" processes that are GFAP- Positive; Rosenthal fibers, eosinophilic granular bodies, and microcysts are often present.

Pilocytic Astrocytoma (TOPNOTCH)

173

Morphology: similar to anaplastic astrocytoma with the additional features of necrosis and vascular or endothelial cell proliferation, each of which often has a stereotypic appearance.

Glioblastoma Multiforme(TOPNOTCH)

174

Morphology: characterized by areas of stromal response with collagen and reticulin deposition and nodules of cells forming "pale islands" that have more neuropil and lack the reticulin deposition

Desmoplastic variant of Medulloblastoma(TOPNOTCH)

175

Gross morphology: the brain is small and shows striking atrophy of the caudate nucleus and the putamen. The globus pallidus may be atrophied secondarily, and the lateral and third ventricles are dilated.

Huntington Disease(TOPNOTCH)

176

What are the two most common primary tumors of the optic nerve?

Glioma and Meningioma (TOPNOTCH)

177

Morphology: characterized histologically by chronic inflammation and replacement of orbital fat by fibrosis

Idiopathic orbital inflammation (TOPNOTCH)

178

What is the most common malignancy of the eyelid?

Basal cell carcinoma (TOPNOTCH)

179

Morphology: histological hallmark is the thinning of the cornea with breaks in the Bowman's layer

Keratoconus (TOPNOTCH)

180

Morphology: deposition of calcium in Bowmans layer

Calcific Band keratopathy (TOPNOTCH)

181

This type of keratopathy develops in patients who are exposed chronically to high levels of ultraviolet light

Actinic band keratopathy (TOPNOTCH)

182

Morphology: numerous drop like excrescences - guttata- protrude downward from Descemet's membrane

Fuchs dystrophy (TOPNOTCH)

183

Also known as the End-Stage Eye

Phthisis Bulbi (TOPNOTCH)

184

Morphology: diffuse loss of ganglion cells and thinning of the retinal nerve fiber layer, in advanced cases, the optic nerve is both cupped and atrophic

Glaucomatous optic nerve damage (TOPNOTCH)

185

Flexner Wintersteine Rosettes

Retinoblastoma (TOPNOTCH)

186

Morphology: a neovascular membrane is positioned between the retinal pigment epithelium and Bruchs membrane

Age related macular degeneration (TOPNOTCH)

187

"waxy pallor" of the optic disk

Retinitis Pigmentosa (TOPNOTCH)

188

In this condition, both rods and cones are lost to apoptosis

Retinitis Pigmentosa (TOPNOTCH)

189

Elschnig' spots

Malignant hypertension (TOPNOTCH)

190

What is known to be a reliable histological marker of diabetes mellitus in the eye?

Thickening of the BM of the epithelium of the pars plicata of the ciliary body (TOPNOTCH)

191

Cytoid bodies

Hypertension (TOPNOTCH)

192

Macular star

Malignant hypertension (TOPNOTCH)

193

Morphology: characterized by diffuse granulomatous inflammation of the urea. Plasma cells are typically absent, but eosiophils may be identified in the infiltrate

Sympathetic ophthalmia (TOPNOTCH)

194

What is the most common intraocular malignancy in adults?

Metastasis to the uvea, typically to the choroid (TOPNOTCH)

195

What is the most common primary intraocular malignancy in adults?

Uveal melanoma (TOPNOTCH)