Neuro - Pathology Flashcards
(135 cards)
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
What is the most common cause of dementia in the elderly?
A
Alzheimer’s disease
3
Q
Senile plaques and neurofibrillary tangles characterize what type of dementia?
A
Alzheimer’s disease
4
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
5
Q
Alzheimer’s disease may cause amyloid angiopathy, which may result in what serious complication?
A
Intracranial hemorrhage
6
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
7
Q
What is the second most common cause of dementia in the elderly after Alzheimer’s Disease?
A
Multi-infarct dementia
8
Q
What degenerative disease of the cerebral cortex affects only the frontal and temporal lobes?
A
Pick’s disease
9
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
10
Q
What degenerative disease of the cerebral cortex is caused by an -synuclein defect?
A
Lewy body dementia
11
Q
_____ bodies are characterized by intracellular aggregated τ-protein in neurons.
A
Pick
12
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
13
Q
What degenerative disease of the cerebral cortex is characterized by parkinsonism, dementia, and hallucinations?
A
Lewy body dementia
14
Q
Name the pathologic proteins responsible for Creutzfeld-Jakob disease.
A
Prions
15
Q
The intracellular neurofibrillary tangles found in patients with Alzheimer;s disease are commonly made up of abnormally phosphorylated _____ protein.
A
τ (tau)
16
Q
What proportion of patients with Alzheimer;s disease have the familial form?
A
Approximately 10%
17
Q
What potentially treatable forms of dementia must be ruled out before diagnosing a patient with Alzheimer;s disease?
A
Wilson;s disease, vitamin B12deficiency, syphilis, and HIV
18
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
19
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
20
Q
Histologically, what are the periventricular plaques seen in multiple sclerosis?
A
Areas of oligodendrocyte loss and reactive gliosis
21
Q
Name five classic symptoms of multiple sclerosis. Use the mnemonic, SIN.
A
Scanning speech, Intention tremor, Incontinence, Internuclear ophthalmoplegia, Nystagmus
22
Q
What is the treatment for multiple sclerosis?
A
β-Interferon or immunosuppressant therapy, as well as symptomatic treatment for incontinence, pain, and spasticity
23
Q
Which imaging modality is considered diagnostic of multiple sclerosis if it has a characteristic appearance?
A
MRI
24
Q
Multiple sclerosis causes damage to which of the following: axons, neuron cell bodies, myelin?
A
Myelin only
25
What demographic group is most likely to be affected by multiple sclerosis (race, sex and age)?
White women 20-40 years of age
26
Is the course of multiple sclerosis usually rapidly progressive, chronic, insidious, or relapsing and remitting?
Relapsing and remitting
27
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?
Guillain-Barr syndrome
28
What is the mechanism by which the immune system is induced to destroy myelin in Guillan-Barr syndrome?
Molecular mimicry
29
How long does it generally take patients to recover from Guillan-Barr syndrome?
Most patients recover in weeks to months
30
Findings in Guillain-Barr syndrome include a(n) _____ (elevated/low/normal) cerebrospinal fluid protein level with a(n) ______ (elevated/low/normal) cell count.
Elevated; normal
31
What causes Guillain-Barr syndrome?
An autoimmune attack on the myelin sheath due to molecular mimicry with a precipitating infection
32
What is the prognosis for patients with Guillain-Barr syndrome?
Almost never fatal, with most patients making a full recovery
33
How is Guillan-Barr treated?
Respiratory support is critical during paralysis; plasmapheresis and intravenous immune globulins are given
34
In Guillan-Barr syndrome, what tissue is the target of autoimmune attack?
Peripheral myelin
35
Which gastrointestinal bacterial infection is a common inciting event for Guillain-Barr syndrome?
Campylobacter jejuni
36
Which rapidly progressive disease is characterized by demyelination of the central nervous system due to destruction of oligodendrocytes in AIDS patients?
Progressive multifocal leukoencephalopathy
37
Which virus has been implicated in progressive multifocal leukoencephalopathy?
JC virus; almost all individuals have evidence of infection, but the virus is latent except in severely immunocompromised patients
38
In what percentage of AIDS patients is PML seen?
2-4%
39
Which demyelinating disease may occur after infections with chickenpox or measles, or with rabies and smallpox vaccinations?
Acute disseminated (postinfectious) encephalomyelitis
40
Which demyelinating disease is characterized by multifocal perivenular inflammation and demyelination?
Acute disseminated (postinfectious) encephalomyelitis
41
What is the mode of inheritance of metachromatic leukodystrophy?
Autosomal recessive
42
In metachromatic leukodystrophy, what is the consequence of sulfatide accumulation in cells?
Impaired production of the myelin sheath
43
In metachromatic leukodystrophy, which enzyme is deficient?
Arylsulfatase A
44
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?
Charcot-Marie-Tooth disease
45
How do partial seizures differ from generalized seizures?
Partial seizures affect one area of the brain and are often preceded by an aura; generalized seizures affect the brain diffusely
46
How do complex and simple partial seizures differ?
Simple partial seizures do not impair consciousness, but can have motor, sensory, autonomic, or psychic effects; complex partial seizures impair consciousness
47
Name five possible causes of new-onset seizures in the elderly.
Stroke, tumor, trauma, infection, or metabolic abnormalities
48
From which part of the brain do partial seizures most commonly originate?
Mesial temporal lobe
49
What is epilepsy?
A disorder of recurrent unprovoked seizures
50
How does neuronal firing differ in seizure activity compared to normal function?
Firing is synchronized and high-frequency during a seizure
51
What are common causes of seizures in children?
Genetic disorders, fever (febrile seizures), trauma, congenital malformations, and metabolic insults
52
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?
Absence seizure
53
What type of seizure is characterized by quick, repetitive jerks?
Myoclonic seizure
54
What are common causes of seizures among adults?
Tumors, trauma, stroke, and infection
55
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?
Tonic-clonic (grand mal) seizure
56
What type of seizure is associated with stiffening (as opposed to jerking)?
Tonic seizure
57
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?
Right frontal lobe
58
Drop seizures are also called _____ seizures.
Atonic; atonic seizures can be confused with syncope
59
What are some physiological causes of headaches?
Irritation of dura, cranial nerves, and extracranial structures; brain parenchyma itself has no sensory receptors
60
A patient experiences 72 hours of unilateral pulsating pain accompanied by nausea and photophobia; which type of headache does he have?
Migraine headache
61
Which disturbances may be associated with the aura preceding a migraine headache?
Visual, sensory and speech disturbances
62
Which cranial nerve is irritated in the etiology of migraine headaches?
CN V
63
The release of which three substances may be implicated in the etiology of migraine headaches?
Substance P, CGRP, vasoactive peptides
64
Which headache is characterized by at least 30 minutes of bilateral steady pain without aura?
Tension headache
65
Light and noise can aggravate _____ (migraine/tension/both) headaches.
Migraine
66
_____ headaches are repetitive brief headaches characterized by unilateral periorbital pain and autonomic symptoms.
Cluster
67
Which type of headache is more common in males than females?
Cluster headaches
68
Cluster headache may manifest with _____ (ipsilateral/contralateral) lacrimation, rhinorrhea and Horner;s syndrome.
ipsilateral
69
A patient presents with the sudden onset of the worst headache of his life. What etiology is suggested by his complaint?
Subarachnoid hemorrhage
70
Which concerning pathologies may present as headaches?
Meningitis, hydrocephalus, neoplasia, arteritis
71
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?
Cluster headaches
72
What is vertigo?
The illusion of movement
73
Which is more common: peripheral or central vertigo?
Peripheral vertigo
74
Name three common etiologies for peripheral vertigo.
Semicircular canal debris, vestibular nerve infection, or Meniere;s disease
75
A patient undergoes positional testing for vertigo and is found to have delayed horizontal nystagmus; what type of vertigo does she have?
Peripheral vertigo
76
Central vertigo is the result of a lesion in what parts of the brain?
Brain stem or cerebellum
77
A patient undergoes positional testing and is found to have immediate nystagmus that switches directions. He has _____ (peripheral/central) vertigo.
Central
78
What syndrome is a congenital disorder characterized by facial port-wine stains and ipsilateral leptomeningeal angioma?
Sturge-Weber syndrome
79
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?
Sturge-Weber syndrome
80
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?
Leptomeningeal angioma
81
Tuberous sclerosis is characterized by what three characteristic cutaneous manifestations?
Ash leaf spots, shagreen patch, and sebaceous adenomas
82
In addition to its cutaneous manifestations, tuberous sclerosis is characterized by which findings?
Hamartomas in the central nervous system, cardiac rhabdomyoma, renal angiomyolipoma, subependymal giant cell astrocytoma, mental retardation, and seizures
83
Name five manifestations of neurofibromatosis type 1 (von Recklinghausen;s disease).
Caf au lait spots, neurofibromas in the skin, Lisch nodules, optic gliomas, pheochromocytomas
84
What mutation causes neurofibromatosis type 1 (von Recklinghausen;s disease)?
Mutation of the NF-1 gene on chromosome 17
85
What mutation causes von Hippel-Lindau disease?
Mutation of the tumor suppressor gene VHL on chromosome 3
86
What is the inheritance pattern of von Hippel-Lindau disease?
Autosomal dominant
87
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?
von Hippel-Lindau disease
88
What is the inheritance pattern of tuberous sclerosis?
Autosomal dominant
89
The majority of primary brain tumors in adults are _____ (infratentorial/supratentorial), whereas they are mostly _____ (infratentorial/supratentorial) in children.
Supratentorial; infratentorial
90
What percentage of adult brain tumors are metastases? Where are they usually found in the brain?
50%; metastases appear as multiple well-circumscribed lesions at the gray-white junction
91
List the three most common primary brain tumors in order of prevalence.
Most to least common: glioblastoma multiforme, meningioma, schwannoma
92
Meningiomas arise from what cell type?
Arachnoid cells
93
Intracranial schwannomas typically affect what cranial nerve?
Cranial nerve VIII
94
What are the histologic findings on biopsy of an oligodendroglioma?
Fried egg cells (ie, round nuclei with clear cytoplasm) and a chicken-wire capillary pattern
95
Most pituitary adenomas originate in the anterior pituitary, which is embryologically derived from what structure?
Rathke;s pouch, which develops from nonneural ectoderm
96
What highly malignant primitive neuroectodermal tumor can compress the fourth ventricle and cause hydrocephalus in children?
Medulloblastoma
97
What is the prognosis for a child diagnosed with an ependymoma?
Poor
98
What benign childhood tumor is often confused with a pituitary adenoma because it can present with bitemporal hemianopia?
Craniopharyngioma
99
What type of staining may help confirm the diagnosis of glioblastoma multiforme?
Glial fibrillary acidic protein staining (GFAP)
100
Bilateral schwannomas are typically found in patients with what congenital illness?
Neurofibromatosis type 2
101
Pilocytic astrocytomas are associated with what histologic findings?
Rosenthal fibers, which are eosinophilic corkscrew fibers
102
Psammoma bodies are commonly found in what type of primary brain tumor?
Meningioma
103
What rare and slow-growing primary brain tumor is most often found in the frontal lobes?
Oligodendroglioma
104
Pituitary adenomas most commonly secrete what hormone?
Prolactin
105
What is the prognosis for a pilocytic astrocytoma?
Good; this is a benign, resectable tumor
106
What primary brain tumor of childhood is most commonly found in the fourth ventricle?
Ependymoma
107
Perivascular pseudorosettes are characteristic of what primary brain tumor?
Ependymoma
108
A craniopharyngioma is embryologically derived from the remnants of what structure?
Rathke;s pouch
109
What type of brain tumor is characterized by rosettes or a perivascular pseudorosette pattern of cells?
Medulloblastoma
110
True or False? Medulloblastomas are radiosensitive.
True
111
Hemangioblastomas are most often found in what region of the brain?
The cerebellum
112
The histopathologic appearance of which brain tumor shows foamy cells and high vascularity?
Hemangioblastoma
113
Which brain tumor may produce erythropoietin and lead to secondary polycythemia?
Hemangioblastoma
114
What are three common presenting symptoms of a brain tumor?
Seizures, dementia, and focal neurologic deficits
115
What is the most common childhood supratentorial tumor?
Craniopharyngioma
116
What is the prognosis for glioblastoma multiforme?
The prognosis is typically grave
117
_____ bodies are laminated calcifications that may be found in a meningioma on histopathology.
Psammoma
118
_____ (Meningiomas/Schwannomas/Both) are resectable tumors.
Both are resectable
119
Hemangioblastomas are associated with which genetic disease that arises from a mutation on the third chromosome?
von Hippel-Lindau disease
120
Which primary brain tumor is typically found in the cerebral hemispheres and may cross the corpus callosum?
Glioblastoma multiforme
121
Tooth enamel-like calcification is common in what primary brain tumor?
Craniopharyngiomas
122
Describe the histologic appearance of glioblastoma multiforme.
Pseudopalisading pleomorphic tumor cells which border central areas of necrosis and hemorrhage
123
Pilocytic astrocytoma will stain positive for what protein marker?
GFAP
124
Spindle cells in a whorled pattern with psammoma bodies are characteristic of which primary intracranial tumor?
Meningioma
125
A cingulate herniation under the falx cerebri may compress which artery?
The anterior cerebral artery
126
How does cerebral herniation cause coma and death?
By compressing the brain stem
127
In which part of the brain is the uncus found?
The medial temporal lobe
128
Which structure can herniate through the foramen magnum as a result of increased intracranial pressure?
The cerebellar tonsils
129
Which cranial nerve is most likely to become stretched due to uncal herniation? What symptoms result?
Cranial nerve III; ipsilateral dilated pupil and ptosis
130
With uncal herniation, which artery is most likely to become compressed? What symptom can result?
The ipsilateral posterior cerebral artery; contralateral homonymous hemianopia
131
What type of hemorrhages may result from the caudal displacement of the brain stem due to an uncal herniation? Which artery is ruptured?
Duret hemorrhages from paramedian artery rupture
132
During uncal herniation, ipsilateral paresis may result from damage to what structure?
Contralateral crus cerebri
133
Name four brain lesions that are ring-enhancing on imaging.
Metastases, abscesses, toxoplasmosis, AIDS lymphoma
134
Name three uniformly enhancing lesions.
Lymphoma, meningioma, metastases (usually ring enhancing)
135
Name a heterogeneously enhancing lesion.
Glioblastoma multiforme
