XXIII - The Nervous System

Question 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.” SEE SLIDE 23.1.

Answer 1

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

Question 2

These are round, faintly basophilic, PAS-positive, concentrically lamellated aggregates of polyglucosans that range between 5 and 50 micrometers, and are located wherever there are astrocytic end processes, especially in the subpial and perivascular zones, seen more frequently with advancing age. SEE SLIDE 23.2.

Answer 2

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

Question 3

Cells which produce myelin in the CNS.

Answer 3

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

Question 4

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

Answer 4

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

Question 5

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

Answer 5

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

Question 6

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

Answer 6

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

Question 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.

Answer 7

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

Question 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.

Answer 8

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

Question 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.

Answer 9

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

Question 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.

Answer 10

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

Question 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.

Answer 11

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

Question 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.

Answer 12

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

Question 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.

Answer 13

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

Question 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”).

Answer 14

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

Question 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.

Answer 15

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

Question 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.

Answer 16

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

Question 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.

Answer 17

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

Question 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. Usually seen after hypotensive episodes.

Answer 18

Border zone (“watershed”) infarcts. Most at risk: border zone between anterior and middle cerebral artery. (TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1264

Question 19

Pattern of necrosis seen in nonhemorrhagic infarcts of the brain.

Answer 19

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

Question 20

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

Answer 20

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

Question 21

Patients with this type of intracranial hemorrhage complains of having “the worst headache I’ve ever had”/ thunderclap headache.

Answer 21

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

Question 22

Most common location for berry/saccular aneurysms.

Answer 22

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

Question 23

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.

Answer 23

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

Question 24

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

Answer 24

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

Question 25

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

Answer 25

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

Question 26

Most common location for berry aneurysms.

Answer 26

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

Question 27

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.

Answer 27

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

Question 28

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.

Answer 28

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

Question 29

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

Answer 29

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

Question 30

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.

Answer 30

Epidural Hematoma. SEE SLIDE 23.3. (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 870

Question 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.

Answer 31

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

Question 32

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.

Answer 32

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

Question 33

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

Answer 33

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

Question 34

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.

Answer 34

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

Question 35

Blood vessel involved in epidural hematoma.

Answer 35

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

Question 36

Blood vessels involved in subdural hemorrhage.

Answer 36

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

Question 37

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.

Answer 37

Myelomeningocele. SEE SLIDE 23.4. (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

Question 38

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

Answer 38

Meningocoele. SEE SLIDE 23.4. (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

Question 39

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

Answer 39

Anencephaly. SEE SLIDE 23.5. (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

Question 40

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.

Answer 40

Encephalocele. SEE SLIDE 23.5. (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 872

Question 41

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

Answer 41

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

Question 42

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.

Answer 42

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

Question 43

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.

Answer 43

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

Question 44

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.

Answer 44

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

Question 45

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. SEE SLIDE 23.6.

Answer 45

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

Question 46

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

Answer 46

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

Question 47

CSF findings in bacterial meningitis.

Answer 47

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

Question 48

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

Answer 48

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

Question 49

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.

Answer 49

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

Question 50

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” (deformed bones). There is loss of both axons and myelin in the dorsal roots, with pallor and atrophy in the dorsal columns of the spinal cord.

Answer 50

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

Question 51

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.

Answer 51

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

Question 52

CSF findings in tuberculous meningitis.

Answer 52

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

Question 53

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.

Answer 53

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

Question 54

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

Answer 54

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

Question 55

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.

Answer 55

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

Question 56

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. SEE SLIDE 23.7.

Answer 56

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

Question 57

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. SEE SLIDE 23.8.

Answer 57

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

Question 58

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

Answer 58

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

Question 59

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.

Answer 59

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

Question 60

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 (SEE SLIDE 23.9), secretory and microcystic.

Answer 60

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

Question 61

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

Answer 61

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

Question 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. SEE SLIDE 23.10

Answer 62

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

Question 63

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

Answer 63

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

Question 64

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

Answer 64

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

Question 65

Profound memory disturbances as a result of thiamine deficiency.

Answer 65

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

Question 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”.

Answer 66

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

Question 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.

Answer 67

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

Question 68

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

Answer 68

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

Question 69

It is the most common cause of dementia in the elderly, characterized by presence of plaques and neurofibrillary tangles, composed of tau proteins. SEE SLIDE 23.11.

Answer 69

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

Question 70

Mutation in Friedreich ataxia.

Answer 70

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

Question 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”. SEE SLIDE 23.12.

Answer 71

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

Question 72

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

Answer 72

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

Question 73

Mutation in Huntington disease.

Answer 73

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

Question 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.

Answer 74

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

Question 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.

Answer 75

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

Question 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.

Answer 76

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

Question 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.

Answer 77

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

Question 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.

Answer 78

Cutaneous neurofibroma. SEE SLIDE 23.13. (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 899

Question 79

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

Answer 79

Plexiform neurofibroma. SEE SLIDE 23.13. (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 899

Question 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).

Answer 80

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

Question 81

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

Answer 81

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

Question 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.” SEE SLIDE 23.14.

Answer 82

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

Question 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.

Answer 83

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

Question 84

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

Answer 84

Inflammation of peripheral nerve, manifested as perivenular and endoneurial infiltration by lymphocytes, macrophages, and a few plasma cells. SEE SLIDE 23.15. (TOPNOTCH)

Question 85

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

Answer 85

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

Question 86

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

Answer 86

Axonal neuropathy (TOPNOTCH)

Question 87

Morphology: Perifascicular Atrophy

Answer 87

Dermatomyositis. SEE SLIDE 23.16. (TOPNOTCH)

Question 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.

Answer 88

Inclusion body myositis (TOPNOTCH)

Question 89

Morphology: Panfascicular atrophy

Answer 89

Spinal Muscular Atrophy (Infantile Motor Neuron Disease. SEE SLIDE 23.17. (TOPNOTCH)

Question 90

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

Answer 90

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. SEE SLIDE 23.18 (TOPNOTCH)

Question 91

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

Answer 91

Duchenne Muscle Dystrophy (TOPNOTCH)

Question 92

What is the histologic pathology that is usually seen in Duchene muscle dystrophy and is rare in Becker Muscle Dystrophy?

Answer 92

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

Question 93

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

Answer 93

Myotonic Dystrophy. SEE SLIDE 23.19 (TOPNOTCH)

Question 94

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

Answer 94

Myasthenia Gravis (TOPNOTCH)

Question 95

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

Answer 95

Inclusion body myositis (TOPNOTCH)

Question 96

Grotton lesions (scaling erythematous eruption or dusky red patches over the knuckles, elbows, and knees) are seen in what disease entity? SEE SLIDE 23.20.

Answer 96

Dermatomyositis (TOPNOTCH)

Question 97

Morphology: the most consistent pathologic findings in skeletal muscle are 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” SEE SLIDE 23.21.

Answer 97

Mitochondrial Myopathies (Oxidative phosphorylation Disease) (TOPNOTCH)

Question 98

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.

Answer 98

Axonal degeneration (TOPNOTCH)

Question 99

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

Answer 99

Segmental demyelination (TOPNOTCH)

Question 100

Morphology: Endoneurial arterioles show thickening, hyalinization, and intense PAS positivity in their walls and extensive reduplicaton of the basement membrane. SEE SLIDE 23.22

Answer 100

Peripheral Neuropathy in DM Type 2 (TOPNOTCH)

Question 101

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.

Answer 101

Myotonic Dystrophy (TOPNOTCH)

Question 102

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

Answer 102

Lipid Myopathies (TOPNOTCH)

Question 103

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

Answer 103

Small cell carcinoma of the lungs (TOPNOTCH)

Question 104

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

Answer 104

Gliosis(TOPNOTCH)

Question 105

Lafora bodies

Answer 105

Myoclonic Epilepsy(TOPNOTCH)

Question 106

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

Answer 106

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

Question 107

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

Answer 107

48 hours(TOPNOTCH)

Question 108

The most widely accepted explanation for diffuse axonal injury

Answer 108

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

Question 109

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

Answer 109

Diffuse axonal injury(TOPNOTCH)

Question 110

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

Answer 110

1 week(TOPNOTCH)

Question 111

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

Answer 111

2 weeks(TOPNOTCH)

Question 112

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

Answer 112

1-3 months(TOPNOTCH)

Question 113

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

Answer 113

12-24 hours(TOPNOTCH)

Question 114

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

Answer 114

24 hours to 2 weeks(TOPNOTCH)

Question 115

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

Answer 115

2 weeks(TOPNOTCH)

Question 116

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

Answer 116

Repair(TOPNOTCH)

Question 117

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

Answer 117

Subacute changes(TOPNOTCH)

Question 118

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

Answer 118

Early change(TOPNOTCH)

Question 119

Morphology: Mononuclear cell perivacular cuffs and neuronophagia of the anterior horn motor neurons of the spinal cord. SEE SLIDE 23.23.

Answer 119

Poliomyelitis(TOPNOTCH)

Question 120

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

Answer 120

Subacute encephalitis(TOPNOTCH)

Question 121

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?

Answer 121

Paraventricular subependymal regions(TOPNOTCH)

Question 122

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

Answer 122

Cerebellar capillary hemangioblastoma(TOPNOTCH)

Question 123

Antoni A and Antoni B patterns of growth are seen in what tumors of the CNS? SEE SLIDE 23.24.

Answer 123

Schwannoma. In Antoni A areas, cells align to produce nuclear palisading, with alternating bands of nuclear and anuclear areas called VEROCAY BODIES. (TOPNOTCH) Robbins Basic Pathology, 9th ed, p 807.

Question 124

Morphology: Histologic appearance similar to anaplastic astrocytoma with the additional feature of necrosis and vascular or endothelial cell proliferation. SEE SLIDE 23.25

Answer 124

Glioblastoma multiforme(TOPNOTCH)

Question 125

Pseudopalisading. SEE SLIDE 23.25

Answer 125

Glioblastoma multiforme(TOPNOTCH)

Question 126

Glomeruloid body

Answer 126

Glioblastoma multiforme(TOPNOTCH)

Question 127

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

Answer 127

Medial dorsal nucleus of the thalamus(TOPNOTCH)

Question 128

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

Answer 128

Friedreich Ataxia(TOPNOTCH)

Question 129

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

Answer 129

Ataxia Telangiectasia(TOPNOTCH)

Question 130

Gross morphology: pallor of the substantia nigra and locus ceruleus

Answer 130

Parkinson Disease(TOPNOTCH)

Question 131

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

Answer 131

Pick Disease(TOPNOTCH)

Question 132

What are the 3 major microscopic abnormalities of Alzheimer disease?

Answer 132

Neuritic/Senile PlaquesNeurofibrillary tanglesAmyloid angiopathy. SEE SLIDE 23.11. (TOPNOTCH)

Question 133

What is the principal clinical manifestation of Alzheimer disease?

Answer 133

Dementia(TOPNOTCH)

Question 134

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

Answer 134

Alzheimer Disease(TOPNOTCH)

Question 135

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)

Answer 135

Alzheimer Disease(TOPNOTCH)

Question 136

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. SEE SLIDE 23.11. What is the most predominant component of this plaque core?

Answer 136

AB peptide(TOPNOTCH)

Question 137

Neurofibrillary tangles are bundles of filaments in the cytoplasm of the neurons that displace or encircle the nucleus. SEE SLIDE 23.11. What is the major component of this structure?

Answer 137

Tau proteins(TOPNOTCH)

Question 138

Hirano bodies. SEE SLIDE 23.11

Answer 138

Alzheimer’s disease (TOPNOTCH)

Question 139

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

Answer 139

Cerebellar capillary hemangioblastoma(TOPNOTCH)

Question 140

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

Answer 140

Renal Cell Carcinoma(TOPNOTCH)

Question 141

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.

Answer 141

Cortical hamartomas or “tubers’ of Tuberous Sclerosis (TOPNOTCH)

Question 142

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

Answer 142

Neurofibromatosis Type 1(TOPNOTCH)

Question 143

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

Answer 143

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

Question 144

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

Answer 144

Primary CNS Lymphoma(TOPNOTCH)

Question 145

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.

Answer 145

Well differentiated fibrillary astrocytomas(TOPNOTCH)

Question 146

Morphology: Perivascular pseudorosettes SEE SLIDE 23.26

Answer 146

Ependymoma(TOPNOTCH)

Question 147

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.

Answer 147

Amyotorphic Lateral Sclerosis(TOPNOTCH)

Question 148

Morphology: composed of bipolar cells with long, thin “hairlike” processes that are GFAP- Positive; Rosenthal fibers, eosinophilic granular bodies, and microcysts are often present. SEE SLIDE 23.27.

Answer 148

Pilocytic Astrocytoma (TOPNOTCH)

Question 149

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

Answer 149

Desmoplastic variant of Medulloblastoma(TOPNOTCH)

Question 150

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.

Answer 150

Huntington Disease(TOPNOTCH)

Question 151

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

Answer 151

Glioma and Meningioma (TOPNOTCH)

Question 152

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

Answer 152

Idiopathic orbital inflammation (TOPNOTCH)

Question 153

What is the most common malignancy of the eyelid?

Answer 153

Basal cell carcinoma (TOPNOTCH)

Question 154

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

Answer 154

Keratoconus (TOPNOTCH)

Question 155

Morphology: deposition of calcium in Bowmans layer

Answer 155

Calcific Band keratopathy (TOPNOTCH)

Question 156

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

Answer 156

Actinic band keratopathy (TOPNOTCH)

Question 157

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

Answer 157

Fuchs dystrophy (TOPNOTCH)

Question 158

Also known as the End-Stage Eye

Answer 158

Phthisis Bulbi (TOPNOTCH)

Question 159

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

Answer 159

Glaucomatous optic nerve damage (TOPNOTCH)

Question 160

Flexner Wintersteiner Rosettes

Answer 160

Retinoblastoma (TOPNOTCH)

Question 161

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

Answer 161

Age related macular degeneration (TOPNOTCH)

Question 162

“Waxy pallor” of the optic disk. SEE SLIDE 23.28

Answer 162

Retinitis Pigmentosa (TOPNOTCH)

Question 163

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

Answer 163

Retinitis Pigmentosa (TOPNOTCH)

Question 164

Elschnig’ spots

Answer 164

Malignant hypertension (TOPNOTCH)

Question 165

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

Answer 165

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

Question 166

Cytoid bodies

Answer 166

Hypertension (TOPNOTCH)

Question 167

Macular star

Answer 167

Malignant hypertension (TOPNOTCH)

Question 168

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

Answer 168

Sympathetic ophthalmia (TOPNOTCH)

Question 169

What is the most common intraocular malignancy in adults?

Answer 169

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

Question 170

What is the most common primary intraocular malignancy in adults?

Answer 170

Uveal melanoma (TOPNOTCH)

Question 171

These are the resident monocyte-lineage population of CNS that proliferate and accumulate in response to injury.

Answer 171

Microglia (TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1254

Question 172

Subfalcine(cingulate) herniation leads to compression of what blood vessel?

Answer 172

Anterior cerebral artery(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1255

Question 173

Transtentoria(uncinate) herniation compress what structures?

Answer 173

CN III and Posterior Cerebral artery(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1255

Question 174

Patient X who had a moderate traumatic injury presented with lethargy. 12 hours later it he progressed rapidly to having dilated pupils and impairment of ocular movements. This is most likely due to:

Answer 174

Transtentorial (uncinate) herniation/CN III compression (TOPNOTCH)

Question 175

A malformation characterized by reduction in the number of gyri, agyria in extreme cases

Answer 175

Lissencephaly(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1257

Question 176

Infarcts in the supratentorial periventricular white matter in premature infants presenting with chalky yellow plaques consisting of discrete regions of white matter necrosis and calcification

Answer 176

Perventricular leukomalacia(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1259

Question 177

Most common site of contusions in the brain

Answer 177

Frontal and temporal lobes(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1260

Question 178

Vascular injury presenting with slowly evolving neurologic symptoms, often with a delay from the time of injury(most often within 48 hours of injury) Grossly, it appears as a collection of freshly clotted blood along the brain surface, without extension into the depths of sulci.

Answer 178

Subdural hematoma(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1262

Question 179

After a vehicular crash, patient presented with quadriplegia and respiratory distress. Spinal cord injury was suspected. This can be due to damage to what level of vertebra?

Answer 179

Above C4(TOPNOTCH)

Question 180

CNS cells most sensitive to ischemia

Answer 180

Neurons(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1264

Question 181

Clinicopathologic syndrome arising in the setting of malignant hypertension, characterized by diffuse cerebral dysfunction, including headaches, confusion, voming, and convulsion, and sometimes leading to coma

Answer 181

Acute hypertensive encephalopathy(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1268

Question 182

Risk factor most commonly associated with deep brain parenchymal hemorrhages.

Answer 182

Hypertension(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1268

Question 183

Most common cause of bacterial meningitis in adolescents and young adults.

Answer 183

Neisseria meningitidis(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1272

Question 184

Typical CSF findings in brain abscess

Answer 184

Increased WBC, increased protein, normal glucose(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1273

Question 185

Most serious complications of chronic TB meningitis

Answer 185

Arachnoid fibrosis(producing hydrocephalus) and Obliterative endarteritis(causing arterial occlusion and infarction)(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1274

Question 186

Morphology: perivascular accumulation or lymphocytes and/or neutrophils, multiple foci of necrosis of gray and white matter, single-cell neuronal necrosis with phagocytosis of debris, microglial nodules

Answer 186

Viral Encephalitis(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1275

Question 187

It produces brain abscess, most ofen in the cerebral cortex and deep gray nuclei, central necrosis, petechial hemorrhages surrounded by acute and chronic inflammation, macrophage infiltration, and vascular proliferation; CT and MRI studies may show multiple ring enhancing lesions. SEE SLIDE 23.29.

Answer 187

Toxoplasmosis of the CNS(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1280

Question 188

A rapidly progressive encephalitis, cerebral malaria, is the complication with the highest mortality caused by what etiologic agent?

Answer 188

Plasmodium falciparum(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 1280

Question 189

HSV-1 infection has predilection to what part of the brain?

Answer 189

Temporal lobe(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1281

Question 190

This group of degenerative disorders is caused by “spreading” of misfolded proteins, allowing a pathogenic protein to acquire many of the characteristics of an infectious agent. Accumulation of PrPSC in neural tissue seems to the cause of pathologic changes in these diseases.

Answer 190

Prion diseases(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1281

Question 191

The most common prion disease that manifest clinically as a rapidly progressive dementia.

Answer 191

Creutzfeldt-Jakob Disease (TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1283

Question 192

The fundamental abnormality in this disease is the accumulation of A-beta and tau in specific brain regions.

Answer 192

Alzheimers Disease(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1288

Question 193

Syndrome caused by deletions in mitochondrial DNA, characterized by ophthalmoplegia, pigmentary degeneratation of the retina, and complete heart block

Answer 193

Kearns-Sayre syndrome(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1245

Question 194

Mitochondrial disoder caused by mutations in either mtDNA or the nuclear genome causing subacute necrotizing encephalopathy

Answer 194

Leigh syndrome(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1245

Question 195

A 12 year old student presents with lethargy, fever, and headaches. These progress to irritability, anxiety, and confusion, with difficulty in swallowing. He refuses any drink being offered. A few days later, he slips into a coma, and dies. On history, he was bitten by a stray dog 4 months ago. At autopsy, where would the characteristic infected neurons with cytoplasmic inclusions most likely be found? (A) frontal and parietal cerebral cortex (B) hippocampus and cerebellum (C) globus pallidus and medulla (D) pineal gland and pituitary

Answer 195

hippocampus and cerebellum (TOPNOTCH) Robbins Pathologic Basis of Disease 8th ed

Question 196

A 19 year old male presents with fever and changes in mood and behavior. His neck is supple, and shows no lateralizing signs, or cranial nerve defects. Memory is impaired. A lumbar tap is performed with a slight lymphocytosis and protein elevation. Bacterial cultures were negative. On PCR, herpes simplex virus I was detected. THe histologic changes of necrotizing inflammation and Cowdry Type A bodies would most likely be found in (A) temporal lobes (B) occipital lobes (C) cerebellar vermis (D) parietal lobes

Answer 196

temporal lobes (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 876

Question 197

A 78 year old woman in a nursing home is found dead in her bed. She has had a 5 year history of progressive disorientation, memory loss, and alteration of mood and behavior. In her final year, she was mute and could not perform activities of daily living without assistance. Her relatives requested an autopsy, and her brain showed cortical atrophy with widening of the sulci. Histology showed multiple plaques and neurofibrillary tangles. The plaques are collections of neuritic processes with a central core of extracellular (A) amyloid (B) tau protein (C) glycogen (D) sphingomyelin

Answer 197

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

Question 198

A 45 year old actor begins to experience hand tremors, and eventually develops a shuffling gait, a stooped posture, and diminished facial expressions. His mental faculties are intact. What is the expected histologic change in his brain? (A) loss of pigmented catecholaminergic neurons in his midbrain (B) loss of neurons in the striatum (C) foci of hemorrhage and necrosis in the mamillary bodies (D) multiple Lewy bodies in his cerebral cortex

Answer 198

loss of pigmented catecholaminergic neurons in his midbrain (pallor of substantia nigra in Parkinsonism) (B - Huntington disease; C - Wernicke encephalopathy; D - Lewy body dementia/disease) (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 893-894

Question 199

A 30 year old saleslady has untreated hypertension. While at work, her colleagues heard her complain of an excruciating headache then lose consciousness a few seconds later. She was pronounced dead at the ER. An autopsy is performed, which showed massive subarachnoid hemorrhage, and a ruptured aneurysm at the right anterior cerebral artery. Her kidneys were markedly enlarged, with multiple 3-4 cm diameter cysts. Which of the following is accurate? (A) the aneurysm is neoplastic (B) the aneurysm was likely present at birth (C) her kidney disease is heritable, in an autosomal dominant fashion (D) her kidney disease more commonly manifests in early childhood

Answer 199

her kidney disease is heritable, in an autosomal dominant fashion (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 569-570, 866-867

Question 200

A 26 year old backpacker presents with seizures. On imaging, there are four 1-2 cm cysts in his cerebral cortex. He undergoes surgery, where two were removed. Histopathology showed an ovoid structure with a tegument, underlying smooth muscle, three oval suckers, all surrounded by an intense inflammmatory infiltrate of plasma cells, lymphocytes, and eosinophils. The surrounding brain tissue is gliotic. The patient acquired this infection by ingesting (A) raw pork (B) raw beef (C) ova from cat litter (D) raw freshwater crabs

Answer 200

raw pork (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 880

Question 201

A 33 year old woman has been having intermittent headache for 3 years. She develops a seizure. Imaging studies show a 4 cm well circumscribed intracranial mass at the convexity of the right cerebral cortex, compressing the adjacent brain. The tumor is surgically excised, and histopathology showed cells in syncytial whorls, some in fascicles. There are occasional concentric calcifications interspersed. There is no significant mitosis, necrosis, pleomorphism, or brain invasion. Her tumor is (A) a metastasis from a thyroid primary (B) a primary lymphoma (C) a glioma (D) a meningioma

Answer 201

a meningioma (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 886

Question 202

A 40 year old lawyer presents with seizures and loss of consciousness. On imaging, there is a contrast-enhancing, intracranial mass straddling the corpus callosum, with a “butterfly” appearance. An open biopsy is performed, which showed a densely cellular tumor of with astrocytic cell processes, foci of necrosis with surrounding pseudo-palisading of tumor cell nuclei, vascular proliferation, and numerous mitoses. His tumor (A) is a glioma (B) is poorly circumscribed (C) confers a dire prognosis (D) all of the above

Answer 202

all of the above (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 882

Question 203

Principal cells that are responsible for repair and scar formation in the brain (Gliosis)

Answer 203

Astrocytes. When activated, they enlarge and develop multiple stout, ramifying processes (Gemistocytic astrocyte). In long-standing gliosis, they begin to shrink, with cellular processes becoming more tighly interwoven (Fibrillary astrocyte with Rosenthal fibers) (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 812

Question 204

Thick, elongated, brightly eosinophilic protein aggregates seen in astrocytic processes in CHRONIC GLIOSIS and in some LOW-GRADE GLIOMAS. SEE SLIDE 23.27.

Answer 204

Rosenthal fibers (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 812

Question 205

Irreversible ischemic injury category (Early, Subacute, or Repair): Red neurons

Answer 205

Acute. 12-24 hours. (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 815

Question 206

Irreversible ischemic injury category (Early, Subacute, or Repair): Necrosis, influx of macrophages, reactive gliosis

Answer 206

Subacute. 24 hours to 2 weeks. (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 815

Question 207

Irreversible ischemic injury category (Early, Subacute, or Repair): Removal of necrotic tissue, gliosis (pseudolaminar necrosis)

Answer 207

Repair. More than 2 weeks. (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 815

Question 208

Protein inclusion seen in Parkinson disease

Answer 208

Alpha-Synuclein. An inclusion containing alpha-synuclein is called a LEWY BODY. (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 836

Question 209

Protein inclusion seen in Alzheimer disease

Answer 209

Beta amyloid and Tau protein(TOPNOTCH)Robbins Basic Pathology, 9th ed., p 836

Question 210

Alzheimer-related lesions. PLAQUE, TANGLE or TAU PROTEIN: Focal spherical collections of neuritic processes, often around a central amyloid (Beta-amyloid) core. Found in the hippocampus and amygdala usually.

Answer 210

Plaque. SEE SLIDE 23.11. (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 838

Question 211

Alzheimer-related lesions. PLAQUE or TANGLE: Bundles of paired helical filaments visible as basophilic fibrillary structures. A major component is an abnormally hyperphosphorylated TAU protein. Not specific for Alzheimer’s.

Answer 211

Tangle. SEE SLIDE 23.11. (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 838

Question 212

In Parkinson’s Disease, where are Lewy bodies appear first?

Answer 212

Interesingly, it appears first in the medulla, and then the pons, before involvement of the substantia nigra. (M>P>S in alphabetical order) (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 839

Question 213

Often located midline in the cerebellum in children, and lateral in adults. On histology, lesion is extremely cellular with sheets of anaplastic (small blue) cells.

Answer 213

Medulloblastoma (TOPNOTCH)Robbins Basic Pathology, 9th ed., p 845