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Flashcards in Neuro - Pathology Deck (137)
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1
Q

A 30-year-old female is diagnosed with multiple sclerosis. What do the characteristic periventricular plaques on her MRI represent?

A

Areas of oligodendrocyte loss and reactive gliosis

2
Q

Identify the histologic findings of this biopsy sample from an oligodendroglioma.

A

Fried egg cells (i.e., round nuclei with clear cytoplasm) and a chicken-wire capillary pattern

3
Q

Identify the brain tumor associated with this MRI demonstrating a tumor in the cerebral hemispheres, crossing the corpus callosum.

A

Glioblastoma multiforme

4
Q

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

A

Alzheimer's disease

5
Q

Senile plaques and neurofibrillary tangles characterize what type of dementia?

A

Alzheimer's disease

6
Q

The genes APP, presenilin-1, presenilin-2, and ApoE4 are associated with the familial form of what type of dementia?

A

Alzheimer's disease

7
Q

Alzheimer's disease may cause amyloid angiopathy, which may result in what serious complication?

A

Intracranial hemorrhage

8
Q

What is the difference between the Apo isoforms E2 and E4 in the pathogenesis of Alzheimer's disease?

A

The E4 isoform is a genetic risk factor for familial Alzheimer's disease, whereas the E2 isoform is protective against Alzheimer's disease

9
Q

What is the second most common cause of dementia in the elderly after Alzheimer's Disease?

A

Multi-infarct dementia

10
Q

What degenerative disease of the cerebral cortex affects only the frontal and temporal lobes?

A

Pick's disease

11
Q

An elderly woman is increasingly socially inappropriate and inattentive, and she has difficulty speaking. What type of dementia is most likely to cause this her decrement in executive functioning?

A

Pick's disease

12
Q

What degenerative disease of the cerebral cortex is caused by an -synuclein defect?

A

Lewy body dementia

13
Q

_____ bodies are characterized by intracellular aggregated τ-protein in neurons.

A

Pick

14
Q

What degenerative disease of the cerebral cortex is associated with a rapidly progressive dementia (occurring over weeks to months), myoclonus, and a spongiform cortex?

A

Creutzfeld-Jakob disease

15
Q

What degenerative disease of the cerebral cortex is characterized by parkinsonism, dementia, and hallucinations?

A

Lewy body dementia

16
Q

Name the pathologic proteins responsible for Creutzfeld-Jakob disease.

A

Prions

17
Q

The intracellular neurofibrillary tangles found in patients with Alzheimer's disease are commonly made up of abnormally phosphorylated _____ protein.

A

τ

18
Q

What proportion of patients with Alzheimer;s disease have the familial form?

A

Approximately 10%

19
Q

What potentially treatable forms of dementia must be ruled out before diagnosing a patient with Alzheimer's disease?

A

Wilson's disease, vitamin B12 deficiency, syphilis, and HIV

20
Q

What will cerebrospinal fluid studies of a patient with multiple sclerosis show?

A

Increased immunoglobulin G protein; oligoclonal bands are considered diagnostic of multiple sclerosis

21
Q

A patient presents with sudden loss of vision, internuclear ophthalmoplegia, hemiparesis, and bladder incontinence. What disease does she most likely have?

A

Multiple sclerosis

22
Q

Histologically, what are the periventricular plaques seen in multiple sclerosis?

A

Areas of oligodendrocyte loss and reactive gliosis

23
Q

Name five classic symptoms of multiple sclerosis. Use the mnemonic, SIN.

A

Scanning speech, Intention tremor, Incontinence, Internuclear ophthalmoplegia, Nystagmus

24
Q

What is the treatment for multiple sclerosis?

A

-Interferon or immunosuppressant therapy, as well as symptomatic treatment for incontinence, pain, and spasticity

25
Q

Which imaging modality is considered diagnostic of multiple sclerosis if it has a characteristic appearance?

A

Magnetic resonance imaging

26
Q

Multiple sclerosis causes damage to which of the following: axons, neuron cell bodies, myelin?

A

Myelin only

27
Q

What demographic group is most likely to be affected by multiple sclerosis (race, sex and age)?

A

White women 20-40 years of age

28
Q

Is the course of multiple sclerosis usually rapidly progressive, chronic, insidious, or relapsing and remitting?

A

Relapsing and remitting

29
Q

What syndrome is characterized by symmetric ascending muscle weakness that begins in the distal lower extremities and that typically occurs after or concurrent with an infection?

A

Guillain-Barr syndrome

30
Q

What is the mechanism by which the immune system is induced to destroy myelin in Guillan-Barr syndrome?

A

Molecular mimicry

31
Q

How long does it generally take patients to recover from Guillan-Barr syndrome?

A

Most patients recover in weeks to months

32
Q

Findings in Guillain-Barr syndrome include a(n) _____ (elevated/low/normal) cerebrospinal fluid protein level with a(n) ______ (elevated/low/normal) cell count.

A

Elevated; normal

33
Q

What causes Guillain-Barr syndrome?

A

An autoimmune attack on the myelin sheath due to molecular mimicry with a precipitating infection

34
Q

What is the prognosis for patients with Guillain-Barr syndrome?

A

Almost never fatal, with most patients making a full recovery

35
Q

How is Guillan-Barr treated?

A

Respiratory support is critical during paralysis; plasmapheresis and intravenous immune globulins are given

36
Q

In Guillan-Barr syndrome, what tissue is the target of autoimmune attack?

A

Peripheral myelin

37
Q

Which gastrointestinal bacterial infection is a common inciting event for Guillain-Barr syndrome?

A

Campylobacter jejuni

38
Q

Which rapidly progressive disease is characterized by demyelination of the central nervous system due to destruction of oligodendrocytes in AIDS patients?

A

Progressive multifocal leukoencephalopathy

39
Q

Which virus has been implicated in progressive multifocal leukoencephalopathy?

A

JC virus; almost all individuals have evidence of infection, but the virus is latent except in severely immunocompromised patients

40
Q

In what percentage of AIDS patients is PML seen?

A

2-4%

41
Q

Which demyelinating disease may occur after infections with chickenpox or measles, or with rabies and smallpox vaccinations?

A

Acute disseminated (postinfectious) encephalomyelitis

42
Q

Which demyelinating disease is characterized by multifocal perivenular inflammation and demyelination?

A

Acute disseminated (postinfectious) encephalomyelitis

43
Q

What is the mode of inheritance of metachromatic leukodystrophy?

A

Autosomal recessive

44
Q

In metachromatic leukodystrophy, what is the consequence of sulfatide accumulation in cells?

A

Impaired production of the myelin sheath

45
Q

In metachromatic leukodystrophy, which enzyme is deficient?

A

Arylsulfatase A

46
Q

Which group of hereditary nerve disorders is characterized by defective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath?

A

Charcot-Marie-Tooth disease

47
Q

How do partial seizures differ from generalized seizures?

A

Partial seizures affect one area of the brain and are often preceded by an aura; generalized seizures affect the brain diffusely

48
Q

How do complex and simple partial seizures differ?

A

Simple partial seizures do not impair consciousness, but can have motor, sensory, autonomic, or psychic effects; complex partial seizures impair consciousness

49
Q

Name five possible causes of new-onset seizures in the elderly.

A

Stroke, tumor, trauma, infection, or metabolic abnormalities

50
Q

From which part of the brain do partial seizures most commonly originate?

A

Mesial temporal lobe

51
Q

What is epilepsy?

A

A disorder of recurrent unprovoked seizures

52
Q

How does neuronal firing differ in seizure activity compared to normal function?

A

Firing is synchronized and high-frequency during a seizure

53
Q

What are common causes of seizures in children?

A

Genetic disorders, fever (febrile seizures), trauma, congenital malformations, and metabolic insults

54
Q

A young girl sometimes lapses into a blank stare and then resumes her conversation as if nothing had happened. What kind of seizure may this child be experiencing?

A

Absence seizure

55
Q

What type of seizure is characterized by quick, repetitive jerks?

A

Myoclonic seizure

56
Q

What are common causes of seizures among adults?

A

Tumors, trauma, stroke, and infection

57
Q

A spouse calls 911 and reports that her husband is on the floor thrashing with alternating stiffening movements. What kind of seizure might the patient be experiencing?

A

Tonic-clonic (grand mal) seizure

58
Q

What type of seizure is associated with stiffening (as opposed to jerking)?

A

Tonic seizure

59
Q

A 55-year-old woman presents to the emergency room with a seizure in which she first noted repetitive twitching of her left hand and left side of her mouth. Over 30 seconds, this twitching extended to her left shoulder and was followed by left thigh twitching. What is the most likely location of her seizure?

A

Right frontal lobe

60
Q

Drop seizures are also called _____ seizures.

A

Atonic; atonic seizures can be confused with syncope

61
Q

What are some physiological causes of headaches?

A

Irritation of dura, cranial nerves, and extracranial structures; brain parenchyma itself has no sensory receptors

62
Q

A patient experiences 72 hours of unilateral pulsating pain accompanied by nausea and photophobia; which type of headache does he have?

A

Migraine headache

63
Q

Which disturbances may be associated with the aura preceding a migraine headache?

A

Visual, sensory and speech disturbances

64
Q

Which cranial nerve is irritated in the etiology of migraine headaches?

A

CN V

65
Q

The release of which three substances may be implicated in the etiology of migraine headaches?

A

Substance P, CGRP, vasoactive peptides

66
Q

Which headache is characterized by at least 30 minutes of bilateral steady pain without aura?

A

Tension headache

67
Q

Light and noise can aggravate _____ (migraine/tension/both) headaches.

A

Migraine

68
Q

_____ headaches are repetitive brief headaches characterized by unilateral periorbital pain and autonomic symptoms.

A

Cluster

69
Q

Which type of headache is more common in males than females?

A

Cluster headaches

70
Q

Cluster headache may manifest with _____ (ipsilateral/contralateral) lacrimation, rhinorrhea and Horner's syndrome.

A

ipsilateral

71
Q

A patient presents with the sudden onset of the worst headache of his life. What etiology is suggested by his complaint?

A

Subarachnoid hemorrhage

72
Q

Which concerning pathologies may present as headaches?

A

Meningitis, hydrocephalus, neoplasia, arteritis

73
Q

A 49-year-old man presents with 2 weeks of excruciating, periorbital, left-sided head/face pain. The pain lasts 45 minutes and is succeeded by a dull ache that lasts hours. He admits to tearing and redness of the left eye and his wife says that his eye droops when he is in pain. The pain usually occurs twice a night at about the same time. He is afebrile with a normal exam when in the office. What is the most likely diagnosis?

A

Cluster headaches

74
Q

What is vertigo?

A

The illusion of movement

75
Q

Which is more common: peripheral or central vertigo?

A

Peripheral vertigo

76
Q

Name three common etiologies for peripheral vertigo.

A

Semicircular canal debris, vestibular nerve infection, or Meniere's disease

77
Q

A patient undergoes positional testing for vertigo and is found to have delayed horizontal nystagmus; what type of vertigo does she have?

A

Peripheral vertigo

78
Q

Central vertigo is the result of a lesion in what parts of the brain?

A

Brain stem or cerebellum

79
Q

A patient undergoes positional testing and is found to have immediate nystagmus that switches directions. He has _____ (peripheral/central) vertigo.

A

Central

80
Q

What syndrome is a congenital disorder characterized by facial port-wine stains and ipsilateral leptomeningeal angioma?

A

Sturge-Weber syndrome

81
Q

A toddler is diagnosed with seizures, mental retardation, and glaucoma. Her pediatrician notices a port-wine stain on the face and hemiparesis. What is the most likely diagnosis?

A

Sturge-Weber syndrome

82
Q

A toddler is diagnosed with seizures, mental retardation, and glaucoma. Her pediatrician notices a port-wine stain on the face and hemiparesis. What is the associated brain lesion?

A

Leptomeningeal angioma

83
Q

Tuberous sclerosis is characterized by what three characteristic cutaneous manifestations?

A

Ash leaf spots, shagreen patch, and sebaceous adenomas

84
Q

In addition to its cutaneous manifestations, tuberous sclerosis is characterized by which findings?

A

Hamartomas in the central nervous system, cardiac rhabdomyoma, renal angiomyolipoma, subependymal giant cell astrocytoma, mental retardation, and seizures

85
Q

Name five manifestations of neurofibromatosis type 1 (von Recklinghausen's disease).

A

Caf au lait spots, neurofibromas in the skin, Lisch nodules, optic gliomas, pheochromocytomas

86
Q

What mutation causes neurofibromatosis type 1 (von Recklinghausen's disease)?

A

Mutation of the NF-1 gene on chromosome 17

87
Q

What mutation causes von Hippel-Lindau disease?

A

Mutation of the tumor suppressor gene VHL on chromosome 3

88
Q

What is the inheritance pattern of von Hippel-Lindau disease?

A

Autosomal dominant

89
Q

What neurocutaneous disorder is associated with cavernous hemangiomas in skin, mucosa, and internal organs; renal cell carcinoma; and hemangioblastoma in retina, brain stem, and cerebellum?

A

von Hippel-Lindau disease

90
Q

What is the inheritance pattern of tuberous sclerosis?

A

Autosomal dominant

91
Q

The majority of primary brain tumors in adults are _____ (infratentorial/supratentorial), whereas they are mostly _____ (infratentorial/supratentorial) in children.

A

Supratentorial; infratentorial

92
Q

What percentage of adult brain tumors are metastases? Where are they usually found in the brain?

A

50%; metastases appear as multiple well-circumscribed lesions at the gray-white junction

93
Q

List the three most common primary brain tumors in order of prevalence.

A

Most to least common: glioblastoma multiforme, meningioma, schwannoma

94
Q

Meningiomas arise from what cell type?

A

Arachnoid cells

95
Q

Intracranial schwannomas typically affect what cranial nerve?

A

Cranial nerve VIII

96
Q

What are the histologic findings on biopsy of an oligodendroglioma?

A

Fried egg cells (ie, round nuclei with clear cytoplasm) and a chicken-wire capillary pattern

97
Q

Most pituitary adenomas originate in the anterior pituitary, which is embryologically derived from what structure?

A

Rathke's pouch, which develops from nonneural ectoderm

98
Q

What highly malignant primitive neuroectodermal tumor can compress the fourth ventricle and cause hydrocephalus in children?

A

Medulloblastoma

99
Q

What is the prognosis for a child diagnosed with an ependymoma?

A

Poor

100
Q

What benign childhood tumor is often confused with a pituitary adenoma because it can present with bitemporal hemianopia?

A

Craniopharyngioma

101
Q

What type of staining may help confirm the diagnosis of glioblastoma multiforme?

A

Glial fibrillary acidic protein staining (GFAP)

102
Q

Bilateral schwannomas are typically found in patients with what congenital illness?

A

Neurofibromatosis type 2

103
Q

Pilocytic astrocytomas are associated with what histologic findings?

A

Rosenthal fibers, which are eosinophilic corkscrew fibers

104
Q

Psammoma bodies are commonly found in what type of primary brain tumor?

A

Meningioma

105
Q

What rare and slow-growing primary brain tumor is most often found in the frontal lobes?

A

Oligodendroglioma

106
Q

Pituitary adenomas most commonly secrete what hormone?

A

Prolactin

107
Q

What is the prognosis for a pilocytic astrocytoma?

A

Good; this is a benign, resectable tumor

108
Q

What primary brain tumor of childhood is most commonly found in the fourth ventricle?

A

Ependymoma

109
Q

Perivascular pseudorosettes are characteristic of what primary brain tumor?

A

Ependymoma

110
Q

A craniopharyngioma is embryologically derived from the remnants of what structure?

A

Rathke;s pouch

111
Q

What type of brain tumor is characterized by rosettes or a perivascular pseudorosette pattern of cells?

A

Medulloblastoma

112
Q

True or False? Medulloblastomas are radiosensitive.

A

TRUE

113
Q

Hemangioblastomas are most often found in what region of the brain?

A

The cerebellum

114
Q

The histopathologic appearance of which brain tumor shows foamy cells and high vascularity?

A

Hemangioblastoma

115
Q

Which brain tumor may produce erythropoietin and lead to secondary polycythemia?

A

Hemangioblastoma

116
Q

What are three common presenting symptoms of a brain tumor?

A

Seizures, dementia, and focal neurologic deficits

117
Q

What is the most common childhood supratentorial tumor?

A

Craniopharyngioma

118
Q

What is the prognosis for glioblastoma multiforme?

A

The prognosis is typically grave

119
Q

_____ bodies are laminated calcifications that may be found in a meningioma on histopathology.

A

Psammoma

120
Q

_____ (Meningiomas/Schwannomas/Both) are resectable tumors.

A

Both are resectable

121
Q

Hemangioblastomas are associated with which genetic disease that arises from a mutation on the third chromosome?

A

von Hippel-Lindau disease

122
Q

Which primary brain tumor is typically found in the cerebral hemispheres and may cross the corpus callosum?

A

Glioblastoma multiforme

123
Q

Tooth enamel-like calcification is common in what primary brain tumor?

A

Craniopharyngiomas

124
Q

Describe the histologic appearance of glioblastoma multiforme.

A

Pseudopalisading pleomorphic tumor cells which border central areas of necrosis and hemorrhage

125
Q

Pilocytic astrocytoma will stain positive for what protein marker?

A

GFAP

126
Q

Spindle cells in a whorled pattern with psammoma bodies are characteristic of which primary intracranial tumor?

A

Meningioma

127
Q

A cingulate herniation under the falx cerebri may compress which artery?

A

The anterior cerebral artery

128
Q

How does cerebral herniation cause coma and death?

A

By compressing the brain stem

129
Q

In which part of the brain is the uncus found?

A

The medial temporal lobe

130
Q

Which structure can herniate through the foramen magnum as a result of increased intracranial pressure?

A

The cerebellar tonsils

131
Q

Which cranial nerve is most likely to become stretched due to uncal herniation? What symptoms result?

A

Cranial nerve III; ipsilateral dilated pupil and ptosis

132
Q

With uncal herniation, which artery is most likely to become compressed? What symptom can result?

A

The ipsilateral posterior cerebral artery; contralateral homonymous hemianopia

133
Q

What type of hemorrhages may result from the caudal displacement of the brain stem due to an uncal herniation? Which artery is ruptured?

A

Duret hemorrhages from paramedian artery rupture

134
Q

During uncal herniation, ipsilateral paresis may result from damage to what structure?

A

Contralateral crus cerebri

135
Q

Name four brain lesions that are ring-enhancing on imaging.

A

Metastases, abscesses, toxoplasmosis, AIDS lymphoma

136
Q

Name three uniformly enhancing lesions.

A

Lymphoma, meningioma, metastases (usually ring enhancing)

137
Q

Name a heterogeneously enhancing lesion.

A

Glioblastoma multiforme