Neurodegenerative Flashcards
Mild cognitive impairment is defined as…
by cognitive impairment that does not interfere with activities of daily living, is not severe enough to classify the patient as demented, and whereby the patient retains general cognitive function.
Impairments may be in one or more cognitive domains such as attention, memory, language, executive, or visuospatial function.
What is the mode of inheritance of inherited forms of Alzheimer Disese?
Autosomal Dominant
How many % are inherited forms of Alzheimer Disease?
5% of all cases of AD
Where is Presenilin-2 gene located? What is its significance?
Chromosome 1
early onset AD
Where is Presenilin-1 gene located? What is its significance?
Chromosome 14 accounts for
70% to 80% of early-onset aggressive familial cases of AD
Where is Apolipoprotein-E3 gene located? What is its significance?
Chromosome 19
triples the risk of AD as well as leading to an late age of onset
Where is Amyloid Precursor Protein (APP) gene located?
Chromosome 21
Trace Papez Circuit
HeMan ATe Cat
Entorhinal cortex –> Hippocampus –> Fornix –> Mammillary Body –> Anterior Thalamus —> Entorhinal Cortex —> Hippocampus
What type of memory is the earliest symptom and mostly impaired in Early Alzheimer Disease?
Long Term Memory —> Explicit memory: —> episodic memory (specific events and contexts) autobigoraphical memory
What is the earliest histopathologic finding in alzheimer disease?
Amyloid deposition in cortical and leptomeningeal blood vessel wall
What is the FDG-PET finding in Alzheimer Disease?
FDG- PET scanning may reveal decreased glucose metabolism in parietotemporal regions in AD
What is the FDG-PET finding in Frontotemporal Dementia?
FDG- PET scanning may reveal decreased glucose metabolism in frontal and anterior temporal regions
What is the FDG-PET finding in Huntington Disease?
FDG- PET scanning may reveal decreased glucose metabolism in the head of caudate nucleus
What is the FDG-PET finding in Parkinson Disease?
FDG-PET scanning in Parkinson’s disease usually does not show a decrease in metabolism.
What is the most specific histopathologic finding of Alzheimer Disease?
Neuritic plaques and neurofibrillary tangles
What type of neurons found in locus coeruleus?
noradrenergic neurons
What type of neurons found in media and dorsal raphe nuclei?
serotonergic neurons
What type of neurons found in nucleus accumbens and ventral tegmental area?
dopaminergic neruons
What type of neurons found in substantia nigra pars compacta?
dopaminergic neurons
What type of neurons found in substantia nigra pars reticulata?
GABAergic neurons
Which chromosome is involved in FrontoTemporal Dementia?
Chromosome 17 (most common)
Chromosome 3 and 9
Which are involved in akinetic mutism?
anterior cingulate–frontal–subcortical circuit
- globus pallidus interna
- bilateral ACA infarcts to the frontal lobes and other lesions to the medial frontal lobes
What is the mode of inheritance of Huntington’s disease and the mutation involved?
Autosomal dominant trinucleotide repeat disorder resulting from the expansion of CAG repeats on chromosome 4p in a region that codes for the Huntington’s protein
What is the triad of Wernicke’s Encephalopathy?
triad of confusion, ataxia, and ophthalmoplegia
What is the manifestation of Korsakoff Disease?
Korsakoff’s disease is the chronic phase of thiamine deficiency and presents with anterograde and retrograde amnesia, and is classically associated with confabulation as a result of poor memory.
What is the histopathologic appearance of Creutzfeldt-Jakob Disease?
Neuronal loss
glial cell proliferation
absent inflammatory response
vacuolization of the neuropil, which produces the characteristic spongiform appearance
What is the gene involved in Creutzfeldt-Jakob Disease and gene involved?
prion protein gene (PRNP) coding for PrP is located on chromosome 20p
Buzzwords
Cortical Ribbon Sign
Creutzfeldt-Jakob Disease
Buzzwords
Hippocampal-Lumpy Bumpy Sign
Alzheimer Disease
Buzzwords
Thinning of the head of the caudate
Huntington Disease
Buzzwords
Calcification in the basal ganglia
Fahr’s Disease
Buzzwords
Eye of the Tiger Sign
pantothenate kinase-associated neurodegeneration (PKAN)
Hallervorden-Spatz Disease
Buzzwords
Negri Bodies
Rabies
Buzzwords
Pick’s Bodies
Fronto-Temporal Dementia
Buzzwords
Bunina Bodies
Amyotrophic Lateral Sclerosis
What is the mechanism of action of Riluzole?
inhibitor of glutamate release, slows disease progression, prolonging survival by approximately 3 months.
Once started: blood counts and liver enzymes should be checked routinely 1 month
Histopathologic feature that correlate best with the severity of dementia:
A. neurofibrillary tangles B. amyloid plaques C. neuronal loss D. A and B E. A and C
E. A and C
Earliest histopathologic feature of Alzheimer Disease:
A. neurofibrillary tangles B. amyloid plaques C. neuronal loss D. A and B E. A and C
B. amyloid plaques
Mechanism of Action of Memantine
A. NMDA Agonist
B. NMDA Antagonist
C. Acetylcholinesterase Inhibitor
D. Acetylcholinesterase agonist
B. NMDA Antagonist
Mechanism of Action of Rivastigmine
A. NMDA Agonist
B. NMDA Antagonist
C. Acetylcholinesterase Inhibitor
D. Acetylcholinesterase and butylcholinesterase Inhibitor
D. Acetylcholinesterase and butylcholinesterase Inhibitor
Which of the following is not a Tauopathy?
A. Alzheimer Disease
B. Lewy Body Dementia
C. Progressive Supranuclear Palsy
D. Corticobasal Degeneration
B. Lewy Body Dementia
Where can you localize lesions affecting semantic memory (autobiographical memory)? What are examples?
Bilateral Hippocamus
- hypoxic-anoxic syndrome
- Alzheimer Disease
- Herpes Simplex Encephalitis
What is the anatomic region involved in Immediate Recall?
A. Perisylvian Cortex of Dominant Hemisphere
B. Association Cortex
C. Occipital Lobes
D. Anterior Inferior temporal and Frontal Lobes
A. Perisylvian Cortex of Dominant Hemisphere
What is the anatomic region involved in Working Memory?
A. Perisylvian Cortex of Dominant Hemisphere
B. Association Cortex
C. Occipital Lobes
D. Prefrontal cortex, medial temporal lobes, dorsomedial thalamus
D. Prefrontal cortex, medial temporal lobes, dorsomedial thalamus
What is the anatomic region involved in Semantic Memory?
A. Perisylvian Cortex of Dominant Hemisphere
B. Anterior, inferior temporal and Frontal Lobes
C. Occipital Lobes
D. Prefrontal cortex, medial temporal lobes, dorsomedial thalamus
B. Anterior, inferior temporal and Frontal Lobes
What is the anatomic region involved in Episodic Memory?
A. Perisylvian Cortex of Dominant Hemisphere
B. Association Cortex
C. Occipital Lobes
D. Anterior Inferior temporal and Frontal Lobes
B. Association Cortex
What is the anatomic region involved in Procedural Memory?
A. Perisylvian Cortex of Dominant Hemisphere
B. Association Cortex
C. Premotor and Motor Cortex, Basal Ganglia, Cerebellum
D. Anterior Inferior temporal and Frontal Lobes
C. Premotor and Motor Cortex, Basal Ganglia, Cerebellum
What is the anatomic region involved in Visual Memory?
A. Perisylvian Cortex of Dominant Hemisphere
B. Association Cortex
C. Occipital Lobes
D. Anterior Inferior temporal and Frontal Lobes
C. Occipital Lobes
Which type of memory is involved in agitation or confusion?
A. Working Memory
B. Semantic Memory
C. Episodic Memory
D. Immediate Recall
D. Immediate Recall
Which type of memory is primarily involved in Wernicke-Korsakoff Syndrome?
A. Working Memory
B. Semantic Memory
C. Procedural Memory
D. Immediate Recall
A. Working Memory
Which type of memory is primarily involved in Fronto-temporal Dementia?
A. Working Memory
B. Semantic Memory
C. Episodic Memory
D. Immediate Recall
B. Semantic Memory
Which type of memory has a function on repetition?
A. Working Memory
B. Semantic Memory
C. Episodic Memory
D. Immediate Recall
D. Immediate Recall
Which type of memory has a function on short-term recall of objects, plans, names and sequencing?
A. Working Memory
B. Semantic Memory
C. Episodic Memory
D. Immediate Recall
A. Working Memory
Which type of memory has a function on recall for facts and relationships?
A. Working Memory
B. Semantic Memory
C. Episodic Memory
D. Immediate Recall
B. Semantic Memory
Which type of memory has a function on recall for temporally organized events?
A. Working Memory
B. Semantic Memory
C. Episodic Memory
D. Immediate Recall
C. Episodic Memory
Which type of memory has a function on operational recall (“how to”)?
A. Working Memory
B. Procedural Memory
C. Episodic Memory
D. Immediate Recall
B. Procedural Memory
Where will you localize pseudobulbar affect?
A. Bilateral Corticobulbar pathways
B. Bilateral Frontal Operculum
C. Bilateral Anterior Thalamic Nuclei
D. Bilateral Dorsomedial Thalamic Nuclei
A. Bilateral Corticobulbar pathways
Pick Bodies
Intranuclear or Intracytoplasmic?
Intracytoplasmic
Buzzwords
intracytoplasmic inclusions that contain accumulations of abnormal tau proteins
Pick Bodies
