Chapter 39 - Degenerative Flashcards
This is the major symptom of Alzheimer disease A. Gradual development of forgetfulness B. Echolalia C. Dyscalculia D. Apraxia
A. Gradual development of forgetfulness (p. 1064)
Feature/s of Alzheimer disease that correlate best with the severity of dementia* A. Neurofibrillary tangles B. Quantitative neuronal loss C. Amyloid plaques D. A and B only
D. A and B only (p. 1067)
Enzyme that cleaves Amyloid Precursor Protein that yields the neurotoxic amyloid proteins seen in Alzheimer disease A. Alpha secretase B. Beta secretase C. Gamma secretase D. Delta secretase
B. Beta secretase (p. 1068)
Chromosome where gene coding for Amyloid Precursor Protein is located (clue: this provides an explanation for Alzheimer changes in Down syndrome) A. 18 B. 19 C. 20 D. 21
D. 21 (p. 1070)
Earliest clinical symptom in Alzheimer disease* A. Failure of episodic memory B. Executive dysfunction C. Dysnomia D. Apathy
A. Failure of episodic memory (p. 1065)
Pathology seen in early Alzheimer disease*
A. Diffuse cortical atrophy
B. Hippocampal atrophy
C. Neuronal loss in layer II of entorhinal cortex
D. Parietal lobe atrophy
C. Neuronal loss in layer II of entorhinal cortex (p. 1067)
Genes associated with early onset of Alzheimer disease except A. APP B. PS1 C. PS2 D. Apo E
D. Apo E (p. 1070)
Genes associated with autosomal dominant inheritance in Alzheimer disease except A. APP B. PS1 C. PS2 D. Apo E
D. Apo E (p. 1070)
This isoform of Apo E is associated with tripling the risk of developing sporadic Alzheimer disease A. E1 B. E2 C. E3 D. E4
D. E4 (p. 1070)
SPECT findings in early Alzheimer disease*
A. Diminished activity in the parietal association regions and the medial temporal lobes
B. Increased activity in the parietal association regions and the medial temporal lobes
C. Diminished activity in the frontal and the medial temporal lobes
D. Increased activity in the frontal and the medial temporal lobes
A. Diminished activity in the parietal association regions and the medial temporal lobes (p. 1071)
Heredofamilial frontotemporal atrophy in which parkinsonism is prominent is linked to a mutation in which chromosome? A. 15 B. 16 C. 17 D. 21
C. 17 (p. 1074)
Main components of the Lewy Body* A. Ubiquitin and synuclein B. Beta amyloid C. Tau D. Neurofibrillary tangles
A. Ubiquitin and synuclein (p. 1075)
This drug has shown benefit in reducing delusions, hallucinations, and anxiety in Lewy Body dementia A. L-dopa B. Olanzapine C. Risperidone D. Rivastigmine
D. Rivastigmine (p. 1075)
This is a dominantly inherited, adult-onset dementia with a fulminant evolution suggestive of encephalopathy and seizures, characterized by intraneuronal inclusions of protease inhibitors A. Frontotemporal lobar degeneration B. Lewy Body Dementia C. Argyrophilic Grain Disease D. Neuroserpinopathy
D. Neuroserpinopathy (p. 1076)
Mutation in Huntington’s disease
A. Excessive long repeats of CAG within the short arm of chromosome 4
B. Excessive long repeats of CAG within the long arm of chromosome 4
C. Excessive long repeats of CAG within the short arm of chromosome 9
D. Excessive long repeats of CAG within the long arm of chromosome 9
A. Excessive long repeats of CAG within the short arm of chromosome 4 (p. 1076)
Which of the following drugs should be avoided in Huntington’s disease patients?* A. Haloperidol B. Levodopa C. Reserpine D. Clozapine
B. Levodopa (p. 1079)
Neuroacanthocytosis is associated with a mutation in which chromosome? A. 9q B. 9p C. 19q D. 19p
A. 9q (p. 1080)
Aberrant protein in neuroacanthocytosis A. Huntingtin B. Chorein C. Ubiquitin D. Alpha synuclein
B. Chorein (p. 1080)
A progressive disease in adults characterized by spasticity, chorea, dementia, and sensory polyneuropathy with PAS-positive structures in neural and astrocytic processes
A. Jakob disease
B. Guamanian Parkinson-dementia-ALS complex
C. Mast syndrome
D. Adult polyglucosan body disease
D. Adult polyglucosan body disease (p. 1081)
Usual blink rate in a normal person A. 5-10/min B. 12-20/min C. 20-25/min D. 25-30/min
B. 12-20/min (p. 1082)
Rigidity and cogwheeling in Parkinson Disease are elicited or enhanced by having the patient engage the opposite limb in a motor task requiring some degree of concentration A. Negro sign B. Froment sign C. Hoffman sign D. Myerson sign
B. Froment sign (p. 1083)
Inability to inhibit blinking in response to a tap over the bridge of the nose or glabella A. Negro sign B. Froment sign C. Hoffman sign D. Myerson sign
D. Myerson sign (p. 1084)
Rate-limiting enzyme for dopamine synthesis A. Phenylalanine hydroxylase B. Tyrosine hydroxylase C. DOPA decarboxylase D. Beta hydroxylase
B. Tyrosine hydroxylase (p. 1086)
Genetic defect associated with this protein accounts for 50% of early onset inherited PD and 20% of sporadic early onset cases A. Alpha synuclein B. Parkin C. Ubiquitin esterase D. DJ-1
B. Parkin (p. 1087)
Increases the bioavailability of L-dopa by inhibiting its peripheral conversion to dopamine A. Carbidopa B. Benserazide C. Entacapone D. A and B only E. A, B, C
D. A and B only (p. 1090)
Extends the plasma half life and duration of L-dopa effect by preventing its breakdown A. Carbidopa B. Benserazide C. Entacapone D. Pramipexole
C. Entacapone (p. 1091)
Use of this dopamine agonist leads to high risk of cardiac valvular damage A. Bromocriptine B. Lisuride C. Ropinirole D. Pramipexole E. Pergolide
E. Pergolide (p. 1091)
Parkinson drug that cannot be given to a patient with angle closure glaucoma A. L-dopa B. Ropinorole C. Pramipexole D. Benztropine
D. Benztropine (p. 1092)
This drug is said to act by antagonism of NMDA or release of stored dopamine and has side effects of leg swelling and worsening of congestive heart failure A. Bromocriptine B. Pramipexole C. Ropinorole D. Trihexyphenidyl E. Amantadine
E. Amantadine (p. 1092)
Dopaminergic chemoreceptor antagonist which can be given to patients with PD who experience nausea with L-dopa A. Domperidone B. Metoclopramide C. Ondansetron D. Palonosetron
A. Domperidone (p. 1093)