Neurodegen Dz Myopathy MCQ's

1. A 69-year-old well educated African American woman is brought to the physician by her daughter, who has become concerned about her mother's behavior. The mother volunteers at the local library shelving books, but for the past few months she has had trouble remembering where the books go. In addition, she often forgets to turn the stove after cooking her family's long-time favorite dishes. Which of the following brain structures are most likely affected by the disease process in this patient?

Temporal, frontal and parietal cortex, and hippocampus

Brainstem, amygdala, substantia nigra, thalamus, and cerebral cortex

Caudate nucleus, putamen, globus pallidus, and frontal cortex

Periventricular grey matter, medial thalamus, cerebellar vermis and cortex

The symptoms described in the question, such as trouble with memory and forgetting tasks, are characteristic of dementia. The brain structures that are most commonly affected by dementia, specifically Alzheimer's disease, are the temporal, frontal, and parietal cortex, as well as the hippocampus. These areas of the brain are involved in memory, learning, and cognitive function. Damage to these structures can lead to the cognitive decline seen in the patient's symptoms. The other options listed do not involve the specific brain structures associated with memory and cognitive function.

Explanation

The symptoms described in the question, such as trouble with memory and forgetting tasks, are characteristic of dementia. The brain structures that are most commonly affected by dementia, specifically Alzheimer's disease, are the temporal, frontal, and parietal cortex, as well as the hippocampus. These areas of the brain are involved in memory, learning, and cognitive function. Damage to these structures can lead to the cognitive decline seen in the patient's symptoms. The other options listed do not involve the specific brain structures associated with memory and cognitive function.

2. Combination of Parkinsonism and profound dementia is characteristically found in

Parkinson disease

Alzheimer disease

Diffuse Lewy body disease

Spinocerebellar ataxia

The combination of Parkinsonism and profound dementia is characteristically found in diffuse Lewy body disease. This neurodegenerative disorder is characterized by the presence of abnormal protein deposits called Lewy bodies in the brain. These Lewy bodies disrupt the normal functioning of brain cells, leading to symptoms such as tremors, muscle stiffness, cognitive decline, and visual hallucinations. Diffuse Lewy body disease shares some similarities with Parkinson's disease and Alzheimer's disease but is distinguished by the combination of Parkinsonism and profound dementia.

Explanation

The combination of Parkinsonism and profound dementia is characteristically found in diffuse Lewy body disease. This neurodegenerative disorder is characterized by the presence of abnormal protein deposits called Lewy bodies in the brain. These Lewy bodies disrupt the normal functioning of brain cells, leading to symptoms such as tremors, muscle stiffness, cognitive decline, and visual hallucinations. Diffuse Lewy body disease shares some similarities with Parkinson's disease and Alzheimer's disease but is distinguished by the combination of Parkinsonism and profound dementia.

3. Patients in the moderate stage of Alzheimer's disease exhibit atrophy of which of the following structures?

Thalamus

Hypothalamus

Hippocampus

Substantia nigra

Raphe

In the moderate stage of Alzheimer's disease, patients exhibit atrophy of the hippocampus. The hippocampus is a crucial structure in the brain involved in memory formation and consolidation. It plays a vital role in the formation of new memories and spatial navigation. As Alzheimer's disease progresses, the hippocampus is one of the first regions to be affected, leading to memory loss and cognitive decline. Therefore, the atrophy of the hippocampus is a characteristic feature of Alzheimer's disease in its moderate stage.

Explanation

In the moderate stage of Alzheimer's disease, patients exhibit atrophy of the hippocampus. The hippocampus is a crucial structure in the brain involved in memory formation and consolidation. It plays a vital role in the formation of new memories and spatial navigation. As Alzheimer's disease progresses, the hippocampus is one of the first regions to be affected, leading to memory loss and cognitive decline. Therefore, the atrophy of the hippocampus is a characteristic feature of Alzheimer's disease in its moderate stage.

4. Tau proteins are associated with

Normal aging

Parkinson’s dz

Lewy Bodies

Transient Ischemic Attacks

Alzheimer’s dementia

Tau proteins are associated with Alzheimer's dementia. Tau proteins are found in high concentrations in the brain and are involved in stabilizing microtubules, which are essential for maintaining the structure and function of neurons. In Alzheimer's disease, abnormal accumulation and aggregation of tau proteins occur, forming neurofibrillary tangles. These tangles disrupt the normal functioning of neurons and contribute to the development of cognitive decline and memory loss characteristic of Alzheimer's dementia. Tau pathology is a hallmark feature of Alzheimer's disease and is closely linked to the neurodegenerative process in this condition.

Explanation

Tau proteins are associated with Alzheimer's dementia. Tau proteins are found in high concentrations in the brain and are involved in stabilizing microtubules, which are essential for maintaining the structure and function of neurons. In Alzheimer's disease, abnormal accumulation and aggregation of tau proteins occur, forming neurofibrillary tangles. These tangles disrupt the normal functioning of neurons and contribute to the development of cognitive decline and memory loss characteristic of Alzheimer's dementia. Tau pathology is a hallmark feature of Alzheimer's disease and is closely linked to the neurodegenerative process in this condition.

5. Which of the following morphologic changes are characteristic for Alzheimer disease?

Lewy bodies, neurofibrillary tangles, and neuritic plaques

Neurofibrillary tangles, neuritic plaques, and watershed liquefactive necrosis

Cortical atrophy, neurofibrillary tangles, and neuritic plaques

Amyloid angiopathy, loss of neurons in the caudate nucleus and hippocampus, and senile plaques

Cortical atrophy, neurofibrillary tangles, and neuritic plaques are characteristic morphologic changes seen in Alzheimer's disease. Cortical atrophy refers to the shrinking of the cerebral cortex, which is a hallmark feature of the disease. Neurofibrillary tangles are abnormal protein aggregates that form inside nerve cells, disrupting their normal function. Neuritic plaques, also known as senile plaques, are clumps of beta-amyloid protein that accumulate between nerve cells. These three changes are commonly observed in the brains of individuals with Alzheimer's disease and play a significant role in the pathogenesis of the condition.

Explanation

Cortical atrophy, neurofibrillary tangles, and neuritic plaques are characteristic morphologic changes seen in Alzheimer's disease. Cortical atrophy refers to the shrinking of the cerebral cortex, which is a hallmark feature of the disease. Neurofibrillary tangles are abnormal protein aggregates that form inside nerve cells, disrupting their normal function. Neuritic plaques, also known as senile plaques, are clumps of beta-amyloid protein that accumulate between nerve cells. These three changes are commonly observed in the brains of individuals with Alzheimer's disease and play a significant role in the pathogenesis of the condition.

6. You visit an island in the pacific with a small population to do field work. You notice that a significant number of young children and adolescents in the population exhibit a lordotic posture when walking, score poorly on IQ tests, and have scoliosis of the spine. Many of these children have problems walking and the older children are often in wheelchairs. You suspect?

Duchenne muscular dystrophy

Multiple sclerosis

Limb girdle muscular dystrophy

Becker muscular dystrophy

Friedrich’s ataxia

Based on the symptoms described, such as lordotic posture, poor performance on IQ tests, scoliosis of the spine, difficulty walking, and the need for wheelchairs in older children, the most likely explanation is Duchenne muscular dystrophy. This is a genetic disorder that primarily affects boys and leads to progressive muscle weakness and degeneration. The symptoms mentioned align with the characteristic signs of Duchenne muscular dystrophy, making it the most probable explanation.

Explanation

Based on the symptoms described, such as lordotic posture, poor performance on IQ tests, scoliosis of the spine, difficulty walking, and the need for wheelchairs in older children, the most likely explanation is Duchenne muscular dystrophy. This is a genetic disorder that primarily affects boys and leads to progressive muscle weakness and degeneration. The symptoms mentioned align with the characteristic signs of Duchenne muscular dystrophy, making it the most probable explanation.

7. Production of connective tissue and collagen accumulation are characteristically associated with:

Creutzfeldt-Jacob disease

Chronic brain abscess

Multiple sclerosis

Alzheimer disease

Chronic brain abscess is characterized by the production of connective tissue and collagen accumulation. This is due to the chronic inflammation and healing process that occurs in response to the abscess. Creutzfeldt-Jacob disease is a neurodegenerative disorder caused by abnormal proteins, not connective tissue production. Multiple sclerosis is characterized by the destruction of myelin, not connective tissue production. Alzheimer's disease is characterized by the accumulation of beta-amyloid plaques and tau tangles, not connective tissue production.

Explanation

Chronic brain abscess is characterized by the production of connective tissue and collagen accumulation. This is due to the chronic inflammation and healing process that occurs in response to the abscess. Creutzfeldt-Jacob disease is a neurodegenerative disorder caused by abnormal proteins, not connective tissue production. Multiple sclerosis is characterized by the destruction of myelin, not connective tissue production. Alzheimer's disease is characterized by the accumulation of beta-amyloid plaques and tau tangles, not connective tissue production.

8. A 69-year-old African American female is brought to her family physician because of rapidly progressive loss of cognitive function, involuntary jerky movements, and excessive somnolence. Physical examination reveals dementia; no other neurologic signs are noted. Laboratory and instrumental evaluation reveals normal CNS findings, and burst of high-voltage slow-wave activity on EEG. Histopathologic changes of the brain characteristic for the presented disease are shown in the image below. Which of the following is the most likely diagnosis?

Creutzfeld-Jacobs disease

Alzheimer disease

Parkinson disease

Huntington disease

The histopathologic changes of the brain shown in the image are characteristic of Creutzfeld-Jacobs disease. This disease is a rare, degenerative, and fatal brain disorder that affects cognitive function and movement control. The symptoms described in the question, such as rapidly progressive loss of cognitive function, involuntary jerky movements, and excessive somnolence, are consistent with Creutzfeld-Jacobs disease. Additionally, the presence of burst of high-voltage slow-wave activity on EEG is a typical finding in this disease. Therefore, Creutzfeld-Jacobs disease is the most likely diagnosis in this case.

Explanation

The histopathologic changes of the brain shown in the image are characteristic of Creutzfeld-Jacobs disease. This disease is a rare, degenerative, and fatal brain disorder that affects cognitive function and movement control. The symptoms described in the question, such as rapidly progressive loss of cognitive function, involuntary jerky movements, and excessive somnolence, are consistent with Creutzfeld-Jacobs disease. Additionally, the presence of burst of high-voltage slow-wave activity on EEG is a typical finding in this disease. Therefore, Creutzfeld-Jacobs disease is the most likely diagnosis in this case.

9. You are examining a large brain. The patient's clinical diagnosis is psychomotor regression, probably lipid storage disease. Which is the most appropriate stain to confirm the diagnosis?

PAS stain

Congo red

GFAP stain

Oil red O

H & E stain

Oil red O stain is the most appropriate stain to confirm the diagnosis of lipid storage disease in a large brain with a clinical diagnosis of psychomotor regression. Oil red O stain specifically stains lipids, allowing for the visualization of lipid droplets in cells. This stain is commonly used to identify lipid accumulation in various tissues, including the brain, and can help confirm the presence of lipid storage disease in this case.

Explanation

Oil red O stain is the most appropriate stain to confirm the diagnosis of lipid storage disease in a large brain with a clinical diagnosis of psychomotor regression. Oil red O stain specifically stains lipids, allowing for the visualization of lipid droplets in cells. This stain is commonly used to identify lipid accumulation in various tissues, including the brain, and can help confirm the presence of lipid storage disease in this case.

10. A patient who has been treated for Parkinson's disease for about a year presents with purplish, mottled changes to her skin. Which of the following drugs is the most likely cause?

Amantadine

Bromocriptine

Levodopa (alone)

Levodopa + Carbidopa

Pramipexole

This cutaneous response, called livedo reticularis , is characteristically associated with amantadine. Recall that this seldom-used antiparkinson drug probably works by releasing endogenous dopamine and blocking its neuronal reuptake. Livedo reticularis is not associated with levodopa (used with or without carbidopa), nor with the dopamine agonists bromocriptine or pramipexole. You might also recall that amantadine is also used for prophylaxis of some strains of influenza virus infections.

Explanation

This cutaneous response, called livedo reticularis , is characteristically associated with amantadine. Recall that this seldom-used antiparkinson drug probably works by releasing endogenous dopamine and blocking its neuronal reuptake. Livedo reticularis is not associated with levodopa (used with or without carbidopa), nor with the dopamine agonists bromocriptine or pramipexole. You might also recall that amantadine is also used for prophylaxis of some strains of influenza virus infections.