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61

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

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

62

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

63

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

64

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

65

Profound memory disturbances as a result of thiamine deficiency.

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

66

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

67

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

68

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

69

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

70

Mutation in Friedreich ataxia.

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

71

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

72

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

73

Mutation in Huntington disease.

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

74

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

75

This is the most common form of neurodegeneration affecting the motor system. It is characterized by muscle atrophy and hypereflexia 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

76

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

77

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

78

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

79

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

80

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 (cafe au lait spots).

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

81

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

82

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

83

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

84

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)

85

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

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

86

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

Axonal neuropathy (TOPNOTCH)

87

Morphology: Perifascicular Atrophy

Dermatomyositis (TOPNOTCH)

88

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)

89

Morphology: Panfascicular atrophy

Spinal Muscular Atrophy (Infantile Motor Neuron Disease (TOPNOTCH)

90

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

1. Variation in fiber diameter 2. Increased numbers of internalized nuclei 3. Degeneration, necrosis, and phagocytosis of muscle fibers 4. Regeneration of muscle fibers 5. Proliferation of endomysial connective tissue (TOPNOTCH)