Question 2: The main component of neurofibrillary tangles is:
Correct. The main component of NFTs is hyperphosphorylated Tau protein.
Incorrect. The main component of NFTs is hyperphosphorylated Tau protein.
Incorrect. The main component of NFTs is hyperphosphorylated Tau protein.
Incorrect. The main component of NFTs is hyperphosphorylated Tau protein.
Question 3: Neurofibrillary tangles occur also in:
Incorrect.
Incorrect.
Incorrect.
Correct!
Question 4: Dementia can be caused by all of the following except:
Incorrect. CJD, MS with diffuse involvement of the hemispheres, and diffuse axonal injury can cause dementia. A large MCA infarct will mainly cause hemiparesis.
Incorrect. CJD, MS with diffuse involvement of the hemispheres, and diffuse axonal injury can cause dementia. A large MCA infarct will mainly cause hemiparesis.
Correct. CJD, MS with diffuse involvement of the hemispheres, and diffuse axonal injury can cause dementia. A large MCA infarct will mainly cause hemiparesis.
Incorrect. CJD, MS with diffuse involvement of the hemispheres, and diffuse axonal injury can cause dementia. A large MCA infarct will mainly cause hemiparesis.
Question 5: Most cases of Alzheimer's disease are:
Incorrect. Only a small proportion of Alzheimer disease cases are autosomal dominant. The vast majority are due to interaction of genetic and other intrinsic and environmental factors (multifactorial). The most important genetic risk factor is the ApoE genotype.
Incorrect. Only a small proportion of Alzheimer disease cases are autosomal dominant. The vast majority are due to interaction of genetic and other intrinsic and environmental factors (multifactorial). The most important genetic risk factor is the ApoE genotype.
Correct. Only a small proportion of Alzheimer disease cases are autosomal dominant. The vast majority are due to interaction of genetic and other intrinsic and environmental factors (multifactorial). The most important genetic risk factor is the ApoE genotype.
Incorrect. Only a small proportion of Alzheimer disease cases are autosomal dominant. The vast majority are due to interaction of genetic and other intrinsic and environmental factors (multifactorial). The most important genetic risk factor is the ApoE genotype.
Question 6: The amount of beta amyloid made by cells of a patient with Down syndrome is:
Incorrect.
Correct!
Incorrect.
Incorrect.
Question 7: The earliest changes in Alzheimer's disease are usually found in the:
Incorrect. The earliest changes of Alzheimer's disease appear in the entorhinoral cortex and from there they spread initially to the hippocampus and then to the neocortex.
Correct. The earliest changes of Alzheimer's disease appear in the entorhinoral cortex and from there they spread initially to the hippocampus and then to the neocortex.
Incorrect. The earliest changes of Alzheimer's disease appear in the entorhinoral cortex and from there they spread initially to the hippocampus and then to the neocortex.
Incorrect. The earliest changes of Alzheimer's disease appear in the entorhinoral cortex and from there they spread initially to the hippocampus and then to the neocortex.
Question 8: A patient with large ventricles, dementia, incontinence, and abnormal gait should:
Incorrect.
Correct!
Question 9: Which type of Alzheimer plaque is more important clinically:
Incorrect. Only neuritic plaques are clinically important. Diffuse plaques may be seen in large numbers in old, non-demented persons.
Correct. Only neuritic plaques are clinically important. Diffuse plaques may be seen in large numbers in old, non-demented persons.
Incorrect. Only neuritic plaques are clinically important. Diffuse plaques may be seen in large numbers in old, non-demented persons.
Question 10: The pathology shown in this Bielschowsky silver stain below occurs in:
Incorrect. The image shows neurofibrillary tangles with a Bielschowsky stain. NFTs occur in both, post-traumatic dementia and several forms of Parkinson's disease, including the Guam Parkinson-Dementia complex and post-encephalitic Parkinsonism.
Incorrect. The image shows neurofibrillary tangles with a Bielschowsky stain. NFTs occur in both, post-traumatic dementia and several forms of Parkinson's disease, including the Guam Parkinson-Dementia complex and post-encephalitic Parkinsonism.
Correct. The image shows neurofibrillary tangles with a Bielschowsky stain. NFTs occur in both, post-traumatic dementia and several forms of Parkinson's disease, including the Guam Parkinson-Dementia complex and post-encephalitic Parkinsonism.
Incorrect. The image shows neurofibrillary tangles with a Bielschowsky stain. NFTs occur in both, post-traumatic dementia and several forms of Parkinson's disease, including the Guam Parkinson-Dementia complex and post-encephalitic Parkinsonism.
Question 11: The pathology shown below occurs in:
Incorrect. The picture illustrates vacuolization (spongiosis), which is a key feature of CJD (spongiform encephalopathy). While some degree of spongiosis appears in other degenerative conditions, the pronounced changes in this picture are most consistent with CJD.
Incorrect. The picture illustrates vacuolization (spongiosis), which is a key feature of CJD (spongiform encephalopathy). While some degree of spongiosis appears in other degenerative conditions, the pronounced changes in this picture are most consistent with CJD.
Correct. The picture illustrates vacuolization (spongiosis), which is a key feature of CJD (spongiform encephalopathy). While some degree of spongiosis appears in other degenerative conditions, the pronounced changes in this picture are most consistent with CJD.
Incorrect. The picture illustrates vacuolization (spongiosis), which is a key feature of CJD (spongiform encephalopathy). While some degree of spongiosis appears in other degenerative conditions, the pronounced changes in this picture are most consistent with CJD.
Question 12: The best match for the illustrated changes is:
Incorrect. The image shows atrophy of the caudate nuclei and enlargement of the lateral ventricles, consistent with Huntington's disease. There is a high rate of suicide among patients with Huntington's disease.
Correct. The image shows atrophy of the caudate nuclei and enlargement of the lateral ventricles, consistent with Huntington's disease. There is a high rate of suicide among patients with Huntington's disease.
Incorrect. The image shows atrophy of the caudate nuclei and enlargement of the lateral ventricles, consistent with Huntington's disease. There is a high rate of suicide among patients with Huntington's disease.
Incorrect. The image shows atrophy of the caudate nuclei and enlargement of the lateral ventricles, consistent with Huntington's disease. There is a high rate of suicide among patients with Huntington's disease.
Question 13: The best match for the illustrated changes is:
Correct. The image is a beta amyloid immunostain and shows beta amyloid deposits in a plaque and a blood vessel. Persons with Alzheimer's disease have pronounced memory loss and spatial disorientation.
Incorrect. The image is a beta amyloid immunostain and shows beta amyloid deposits in a plaque and a blood vessel. Persons with Alzheimer's disease have pronounced memory loss and spatial disorientation.
Incorrect. The image is a beta amyloid immunostain and shows beta amyloid deposits in a plaque and a blood vessel. Persons with Alzheimer's disease have pronounced memory loss and spatial disorientation.
Incorrect. The image is a beta amyloid immunostain and shows beta amyloid deposits in a plaque and a blood vessel. Persons with Alzheimer's disease have pronounced memory loss and spatial disorientation.
Question 14: The best match for the illustrated changes is:
Incorrect. The picture shows absence of pigment in the substantia nigra, which is characteristic of Parkinson's disease.
Incorrect. The picture shows absence of pigment in the substantia nigra, which is characteristic of Parkinson's disease.
Incorrect. The picture shows absence of pigment in the substantia nigra, which is characteristic of Parkinson's disease.
Correct. The picture shows absence of pigment in the substantia nigra, which is characteristic of Parkinson's disease.
Question 15: Axonal swellings in diffuse axonal injury contain:
Correct. Axonal swellings in diffuse axonal injury contain beta amyloid precursor protein. Beta amyloid is present in Alzheimer's plaques.
Incorrect. Axonal swellings in diffuse axonal injury contain beta amyloid precursor protein. Beta amyloid is present in Alzheimer's plaques.
Incorrect. Axonal swellings in diffuse axonal injury contain beta amyloid precursor protein. Beta amyloid is present in Alzheimer's plaques.
Question 16: The CSF in Alzheimer's disease shows:
Correct!
Incorrect.
Question 17: Elevation of protein 14-3-3 in CSF occurs in:
Incorrect. Both, CJD and Alzheimer's disease may show elevation of 14-3-3 in CSF. CJD can be distinguished from Alzheimer's disease in most cases, based on the rapidity of its course and its clinical manifestations.
Incorrect. Both, CJD and Alzheimer's disease may show elevation of 14-3-3 in CSF. CJD can be distinguished from Alzheimer's disease in most cases, based on the rapidity of its course and its clinical manifestations.
Correct. Both, CJD and Alzheimer's disease may show elevation of 14-3-3 in CSF. CJD can be distinguished from Alzheimer's disease in most cases, based on the rapidity of its course and its clinical manifestations.
Incorrect. Both, CJD and Alzheimer's disease may show elevation of 14-3-3 in CSF. CJD can be distinguished from Alzheimer's disease in most cases, based on the rapidity of its course and its clinical manifestations.
Question 18: Which of the following APO E genotypes confers the lowest risk for dementia:
Correct. The lowest risk for dementia is associated with ApoE 2/2 and the highest with ApoE 4/4.
Incorrect. The lowest risk for dementia is associated with ApoE 2/2 and the highest with ApoE 4/4.
Incorrect. The lowest risk for dementia is associated with ApoE 2/2 and the highest with ApoE 4/4.
Incorrect. The lowest risk for dementia is associated with ApoE 2/2 and the highest with ApoE 4/4.
Incorrect. The lowest risk for dementia is associated with ApoE 2/2 and the highest with ApoE 4/4.
Question 19: The pathology shown is associated with:
Incorrect. The picture shows atrophy of the frontal and temporal lobes (lobar atrophy) and is consistent with Pick's disease. Pick's disease and other frontotemporal dementias do not have the pronounced memory loss that Alzheimer's patients have. They have behavior and personality changes and language dysfunction, the latter because of involvement of the motor speech area.
Incorrect. The picture shows atrophy of the frontal and temporal lobes (lobar atrophy) and is consistent with Pick's disease. Pick's disease and other frontotemporal dementias do not have the pronounced memory loss that Alzheimer's patients have. They have behavior and personality changes and language dysfunction, the latter because of involvement of the motor speech area.
Incorrect. The picture shows atrophy of the frontal and temporal lobes (lobar atrophy) and is consistent with Pick's disease. Pick's disease and other frontotemporal dementias do not have the pronounced memory loss that Alzheimer's patients have. They have behavior and personality changes and language dysfunction, the latter because of involvement of the motor speech area.
Correct. The picture shows atrophy of the frontal and temporal lobes (lobar atrophy) and is consistent with Pick's disease. Pick's disease and other frontotemporal dementias do not have the pronounced memory loss that Alzheimer's patients have. They have behavior and personality changes and language dysfunction, the latter because of involvement of the motor speech area.
Question 20: Frontotemporal dementias may be accompanied by degeneration of all of the following structures except:
Incorrect. Cerebellar degeneration is not a feature of frontotemporal dementias.
Correct. Cerebellar degeneration is not a feature of frontotemporal dementias.
Incorrect. Cerebellar degeneration is not a feature of frontotemporal dementias.
Incorrect. Cerebellar degeneration is not a feature of frontotemporal dementias.
Question 21: Many patients with Alzheimer's disease also have cerebral amyloid angiopathy
Correct!
Incorrect.
Question 22: Ubiquitin is important for degradation of:
Incorrect.
Incorrect.
Incorrect.
Correct!
Question 23: Which of the following is least likely to be affected in Parkinson's disease?
Incorrect. The caudate nucleus is unlikely to be affected in Parkinson's disease. The nucleus basalis and hypothalamus are routinely affected. The cerebral cortex is affected in diffuse Lewy body disease.
Incorrect. The caudate nucleus is unlikely to be affected in Parkinson's disease. The nucleus basalis and hypothalamus are routinely affected. The cerebral cortex is affected in diffuse Lewy body disease.
Incorrect. The caudate nucleus is unlikely to be affected in Parkinson's disease. The nucleus basalis and hypothalamus are routinely affected. The cerebral cortex is affected in diffuse Lewy body disease.
Correct. The caudate nucleus is unlikely to be affected in Parkinson's disease. The nucleus basalis and hypothalamus are routinely affected. The cerebral cortex is affected in diffuse Lewy body disease.
Question 24: The pathology shown in this ubiquitin immunostain of the cerebral cortex is associated with:
Correct. The picture illustrates cortical Lewy bodies with the ubiquitin stain. Patients with diffuse Lewy body disease have dementia and hallucinations.
Incorrect. The picture illustrates cortical Lewy bodies with the ubiquitin stain. Patients with diffuse Lewy body disease have dementia and hallucinations.
Incorrect. The picture illustrates cortical Lewy bodies with the ubiquitin stain. Patients with diffuse Lewy body disease have dementia and hallucinations.
Incorrect. The picture illustrates cortical Lewy bodies with the ubiquitin stain. Patients with diffuse Lewy body disease have dementia and hallucinations.
Question 25: MPTP damages:
Incorrect.
Correct!
Incorrect.
Incorrect.
Question 26: Parkinsonian manifestations may develop following poisoning with:
Correct!
Incorrect.
Incorrect.
Incorrect.
Question 27: Parkinsonian manifestations may develop with all of the following except:
Incorrect. Parkinsonism is not a feature of MS.
Incorrect. Parkinsonism is not a feature of MS.
Correct. Parkinsonism is not a feature of MS.
Incorrect. Parkinsonism is not a feature of MS.
Question 28: Which of the following is not a trinucleotide repeat disorder:
Correct!
Incorrect.
Incorrect.
Incorrect.
Question 29: The pathology shown below is least likely to be caused by:
Correct. The image group atrophy, consistent with denervation. Myofiber atrophy is least likely to develop with the GBS because axons are relatively preserved. On other hand, diabetic neuropathy is an axonal neuropathy and SMA and ALS are lower motor neuron diseases.
Incorrect. The image group atrophy, consistent with denervation. Myofiber atrophy is least likely to develop with the GBS because axons are relatively preserved. On other hand, diabetic neuropathy is an axonal neuropathy and SMA and ALS are lower motor neuron diseases.
Incorrect. The image group atrophy, consistent with denervation. Myofiber atrophy is least likely to develop with the GBS because axons are relatively preserved. On other hand, diabetic neuropathy is an axonal neuropathy and SMA and ALS are lower motor neuron diseases.
Incorrect. The image group atrophy, consistent with denervation. Myofiber atrophy is least likely to develop with the GBS because axons are relatively preserved. On other hand, diabetic neuropathy is an axonal neuropathy and SMA and ALS are lower motor neuron diseases.
Question 30: The pathology shown below causes:
Correct. The picture shows degeneration of the lateral and anterior corticospinal tracts which may be due to ALS or spinal cord transection above that level. This pathology affects the axons of the upper motor neurons and causes spasticity, brisk reflexes, and up-going plantar responses. Lower motor neurons and their axons are not involved. Therefore, there is no muscle atrophy.
Incorrect. The picture shows degeneration of the lateral and anterior corticospinal tracts which may be due to ALS or spinal cord transection above that level. This pathology affects the axons of the upper motor neurons and causes spasticity, brisk reflexes, and up-going plantar responses. Lower motor neurons and their axons are not involved. Therefore, there is no muscle atrophy.
Incorrect. The picture shows degeneration of the lateral and anterior corticospinal tracts which may be due to ALS or spinal cord transection above that level. This pathology affects the axons of the upper motor neurons and causes spasticity, brisk reflexes, and up-going plantar responses. Lower motor neurons and their axons are not involved. Therefore, there is no muscle atrophy.
Incorrect. The picture shows degeneration of the lateral and anterior corticospinal tracts which may be due to ALS or spinal cord transection above that level. This pathology affects the axons of the upper motor neurons and causes spasticity, brisk reflexes, and up-going plantar responses. Lower motor neurons and their axons are not involved. Therefore, there is no muscle atrophy.
Question 31: The muscle pathology (ATPase stain) shown in this severely hypotonic infant is caused by:
Incorrect. This is an ATPase histochemical stain showing denervation (fiber type grouping). In a hypotonic infant, this is most likely due to spinal muscular atrophy, which is caused by SMN1 deletion. At a later age, denervation atrophy may develop as a result of PMP 22 duplications (Charcot-Marie-Tooth disease). PMP 22 deletion causes neuropathy with pressure palsies.
Correct. This is an ATPase histochemical stain showing denervation (fiber type grouping). In a hypotonic infant, this is most likely due to spinal muscular atrophy, which is caused by SMN1 deletion. At a later age, denervation atrophy may develop as a result of PMP 22 duplications (Charcot-Marie-Tooth disease). PMP 22 deletion causes neuropathy with pressure palsies.
Incorrect. This is an ATPase histochemical stain showing denervation (fiber type grouping). In a hypotonic infant, this is most likely due to spinal muscular atrophy, which is caused by SMN1 deletion. At a later age, denervation atrophy may develop as a result of PMP 22 duplications (Charcot-Marie-Tooth disease). PMP 22 deletion causes neuropathy with pressure palsies.
Incorrect. This is an ATPase histochemical stain showing denervation (fiber type grouping). In a hypotonic infant, this is most likely due to spinal muscular atrophy, which is caused by SMN1 deletion. At a later age, denervation atrophy may develop as a result of PMP 22 duplications (Charcot-Marie-Tooth disease). PMP 22 deletion causes neuropathy with pressure palsies.
Question 32: The pathology shown is most likely to occur in:
Incorrect. The picture shows midline cerebellar degeneration (nutritional cerebellar degeneration).This is most likely to occur in a malnourished demented patient. Pes cavus and arrhythmias indicate Friedreich's ataxia, a primarily spinal ataxia. Intestinal resection may cause subacute combined degeneration of the spinal cord.
Incorrect. The picture shows midline cerebellar degeneration (nutritional cerebellar degeneration).This is most likely to occur in a malnourished demented patient. Pes cavus and arrhythmias indicate Friedreich's ataxia, a primarily spinal ataxia. Intestinal resection may cause subacute combined degeneration of the spinal cord.
Incorrect. The picture shows midline cerebellar degeneration (nutritional cerebellar degeneration).This is most likely to occur in a malnourished demented patient. Pes cavus and arrhythmias indicate Friedreich's ataxia, a primarily spinal ataxia. Intestinal resection may cause subacute combined degeneration of the spinal cord.
Correct. The picture shows midline cerebellar degeneration (nutritional cerebellar degeneration).This is most likely to occur in a malnourished demented patient. Pes cavus and arrhythmias indicate Friedreich's ataxia, a primarily spinal ataxia. Intestinal resection may cause subacute combined degeneration of the spinal cord.
Question 33: Frataxin is located in the:
Correct. Frataxin is a mitochondrial protein. The gene encoding frataxin is a nuclear gene.
Incorrect. Frataxin is a mitochondrial protein. The gene encoding frataxin is a nuclear gene.
Incorrect. Frataxin is a mitochondrial protein. The gene encoding frataxin is a nuclear gene.
Incorrect. Frataxin is a mitochondrial protein. The gene encoding frataxin is a nuclear gene.
Question 34: Which of the following is not a polyglutamine disorder:
Incorrect.
Correct!
Incorrect.
Incorrect.
Question 35: Which of the following is a polyglutamine disorder:
Incorrect. Only autosomal dominant spinocerebellar ataxias are polyglutamine disorders (they encode CAG trinucleotide repeats). Friedreich's ataxia is associated with a GAA trinucleotide repeat. The fragile X syndrome is associated with the CGG repeat. Most OPCA cases are sporadic and are not associated with trinucleotide repeats.
Correct. Only autosomal dominant spinocerebellar ataxias are polyglutamine disorders (they encode CAG trinucleotide repeats). Friedreich's ataxia is associated with a GAA trinucleotide repeat. The fragile X syndrome is associated with the CGG repeat. Most OPCA cases are sporadic and are not associated with trinucleotide repeats.
Incorrect. Only autosomal dominant spinocerebellar ataxias are polyglutamine disorders (they encode CAG trinucleotide repeats). Friedreich's ataxia is associated with a GAA trinucleotide repeat. The fragile X syndrome is associated with the CGG repeat. Most OPCA cases are sporadic and are not associated with trinucleotide repeats.
Incorrect. Only autosomal dominant spinocerebellar ataxias are polyglutamine disorders (they encode CAG trinucleotide repeats). Friedreich's ataxia is associated with a GAA trinucleotide repeat. The fragile X syndrome is associated with the CGG repeat. Most OPCA cases are sporadic and are not associated with trinucleotide repeats.
