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Adams 10th > Chapter 39 - Degenerative > Flashcards

Chapter 39 - Degenerative Flashcards

1
Q 
This is the major symptom of Alzheimer disease
A. Gradual development of forgetfulness 
B. Echolalia
C. Dyscalculia
D. Apraxia
A

A. Gradual development of forgetfulness (p. 1064)

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2
Q 
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
A

D. A and B only (p. 1067)

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3
Q 
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
A

B. Beta secretase (p. 1068)

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4
Q 
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
A

D. 21 (p. 1070)

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5
Q 
Earliest clinical symptom in Alzheimer disease*
A. Failure of episodic memory
B. Executive dysfunction
C. Dysnomia
D. Apathy
A

A. Failure of episodic memory (p. 1065)

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6
Q

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

A

C. Neuronal loss in layer II of entorhinal cortex (p. 1067)

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7
Q 
Genes associated with early onset of Alzheimer disease except
A. APP
B. PS1
C. PS2
D. Apo E
A

D. Apo E (p. 1070)

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8
Q 
Genes associated with autosomal dominant inheritance in Alzheimer disease except
A. APP
B. PS1
C. PS2
D. Apo E
A

D. Apo E (p. 1070)

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9
Q 
This isoform of Apo E is associated with tripling the risk of developing sporadic Alzheimer disease
A. E1
B. E2
C. E3
D. E4
A

D. E4 (p. 1070)

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10
Q

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

A. Diminished activity in the parietal association regions and the medial temporal lobes (p. 1071)

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11
Q 
Heredofamilial frontotemporal atrophy in which parkinsonism is prominent is linked to a mutation in which chromosome?
A. 15
B. 16
C. 17
D. 21
A

C. 17 (p. 1074)

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12
Q 
Main components of the Lewy Body*
A. Ubiquitin and synuclein
B. Beta amyloid
C. Tau
D. Neurofibrillary tangles
A

A. Ubiquitin and synuclein (p. 1075)

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13
Q 
This drug has shown benefit in reducing delusions, hallucinations, and anxiety in Lewy Body dementia
A. L-dopa
B. Olanzapine
C. Risperidone
D. Rivastigmine
A

D. Rivastigmine (p. 1075)

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14
Q 
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
A

D. Neuroserpinopathy (p. 1076)

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15
Q

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

A. Excessive long repeats of CAG within the short arm of chromosome 4 (p. 1076)

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16
Q 
Which of the following drugs should be avoided in Huntington’s disease patients?*
A. Haloperidol
B. Levodopa
C. Reserpine
D. Clozapine
A

B. Levodopa (p. 1079)

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17
Q 
Neuroacanthocytosis is associated with a mutation in which chromosome?
A. 9q
B. 9p
C. 19q
D. 19p
A

A. 9q (p. 1080)

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18
Q 
Aberrant protein in neuroacanthocytosis
A. Huntingtin
B. Chorein
C. Ubiquitin
D. Alpha synuclein
A

B. Chorein (p. 1080)

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19
Q

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

A

D. Adult polyglucosan body disease (p. 1081)

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20
Q 
Usual blink rate in a normal person
A. 5-10/min
B. 12-20/min
C. 20-25/min
D. 25-30/min
A

B. 12-20/min (p. 1082)

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21
Q 
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
A

B. Froment sign (p. 1083)

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22
Q 
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
A

D. Myerson sign (p. 1084)

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23
Q 
Rate-limiting enzyme for dopamine synthesis
A. Phenylalanine hydroxylase
B. Tyrosine hydroxylase
C. DOPA decarboxylase
D. Beta hydroxylase
A

B. Tyrosine hydroxylase (p. 1086)

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24
Q 
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
A

B. Parkin (p. 1087)

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25
Q 
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
A

D. A and B only (p. 1090)

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26
Q 
Extends the plasma half life and duration of L-dopa effect by preventing its breakdown
A. Carbidopa
B. Benserazide
C. Entacapone
D. Pramipexole
A

C. Entacapone (p. 1091)

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27
Q 
Use of this dopamine agonist leads to high risk of cardiac valvular damage
A. Bromocriptine
B. Lisuride
C. Ropinirole
D. Pramipexole
E. Pergolide
A

E. Pergolide (p. 1091)

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28
Q 
Parkinson drug that cannot be given to a patient with angle closure glaucoma
A. L-dopa
B. Ropinorole
C. Pramipexole
D. Benztropine
A

D. Benztropine (p. 1092)

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29
Q 
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
A

E. Amantadine (p. 1092)

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30
Q 
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

A. Domperidone (p. 1093)

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31
Q 
A decline in efficacy of L-dopa at the end of dose interval may be treated with the following except
A. More frequent dosing
B. Addition of dopamine agonist
C. COMT inhibitor
D. None of the above
A

D. None of the above (p. 1093)

32
Q 
This antipsychotic medication has been said to provide an additional benefit of suppressing dyskinesias in advanced Parkinson disease
A. Olanzapine
B. Clozapine
C. Risperidone
D. Quetiapine
A

B. Clozapine (p. 1093)

33
Q 
Features of MSA that distinguish it from Parkinson disease except
A. Symmetry of signs
B. Good response to L-dopa
C. Absence or minimal tremor
D. Early presence of autonomic features
A

B. Good response to L-dopa (p. 1096)

34
Q 
Hot cross bun sign on T2 MRI
A. Alzheimer disease
B. Parkinson disease
C. MSA
D. PSP
A

C. MSA (p. 1096)

35
Q 
Mutation in this gene is associated with MSA
A. SCNA
B. PARK 2
C. LRRK 2
D. COQ 2
A

D. COQ 2 (p. 1096)

36
Q 
Reflexive upturning of eyes upon forced closure of the eyelids
A. Myerson sign
B. Uhthoff phenomenon
C. Bell phenomenon
D. Pulfrich phenomenon
A

C. Bell phenomenon (pp. 1096-1097)

37
Q

Features of PSP that distinguishes it from PD except
A. Tonic grimacing facial expression
B. Lack of tremor
C. Stooped posture
D. Prominence of oculomotor abnormalities
E. None of the above

A

C. Stooped posture (p. 1097)

38
Q 
Mouse ears configuration on MRI due to atrophy of the dorsal midbrain
A. Alzheimer disease
B. Parkinson disease 
C. MSA
D. PSP
A

D. PSP (p. 1097)

39
Q 
Pathologic characteristics of corticobasal degeneration
A. Neuronal achromasia
B. Corticobasal bodies
C. Both A and B
D. None of the above
A

C. Both A and B (p. 1099)

40
Q 
Pattern of inheritance of Friedreich ataxia
A. Autosomal dominant
B. Autosomal recessive
C. X-linked dominant
D. X-linked recessive
A

B. Autosomal recessive (p. 1102)

41
Q 
Aberrant protein in Friedreich ataxia, which is a mitochondrial matrix protein whose function is to prevent intramitochondrial overloading
A. Ataxin-1
B. Ataxin-2
C. Ataxin-3
D. Frataxin
A

D. Frataxin (p. 1102)

42
Q

EMG findings in ALS except
A. Widespread fibrillations and fasciculations
B. Presence of focal motor conduction block
C. Sensory nerve action potentials are normal
D. Motor amplitudes become progressively lower as disease progresses

A

B. Presence of focal motor conduction block (p. 1112)

43
Q 
Features distinguishing Inclusion Body Myositis from ALS except
A. Normal corticospinal function
B. Preservation of DTRs in weak muscles
C. Finger flexor weakness
D. None of the above
A

D. None of the above (p. 1114)

44
Q 
Familial cases of ALS are inherited predominantly by what pattern?
A. Autosomal dominant
B. Autosomal recessive
C. X-linked dominant
D. X-linked recessive
A

A. Autosomal dominant (p. 1115)

45
Q 
The following genes are associated with adult onset ALS except
A. SOD1
B. FUS
C. TARDP5P
D. ALS2
A

D. ALS2 (p. 1114)

46
Q 
The following genes are associated with ALS with frontotemporal dementia except
A. SOD1
B. FUS
C. TARDP5P
D. None of the above
A

A. SOD1 (p. 1114)

47
Q 
Antiglutamate agent that was shown to slow the progression of ALS and improve survival in patients with disease of bulbar onset
A. Riluzole
B. Guanidine HCl
C. Cyclophosphamide
D. TRH
A

A. Riluzole (p. 1115)

48
Q 
Mutation of this protein is associated with familial cases only*
A. Presenilin 1
B. Presenilin 2
C. Apolipoprotein E
D. Ubiquilin 1
A

D. Ubiquilin 1 (p. 1070)

49
Q 
Ratio of these AB proteins is critical to the neuronal toxicity of amyloid in Alzheimer's disease*
A. AB 45 to AB 42
B. AB 42 to AB 40
C. AB 40 to AB 45
D. AB 45 to AB 40
A

B. AB 42 to AB 40 (p. 1068)

50
Q 
Apathy, disinhibition, perseveration, poor judgment, and eating disorders are characteristic of this disease*
A. Alzheimer disease
B. Behavioral variant FTLD
C. Primary progressive aphasia
D. Lewy body dementia
A

B. Behavioral variant FTLD (p. 1074)

51
Q 
Which of the following drugs for PD is an ergot derivative?*
A. Ropinirole
B. Pramipexole 
C. Pergolide
D. Benztropine
A

C. Pergolide (p. 1091)

52
Q 
Which type of SMA has the worst prognosis?*
A. I (Werdnig-Hofmann)
B. II (Dubowitz)
C. III (Wohlfart-Kugelberg-Walender)
D. IV
A

A. I (Werdnig-Hofmann) (p. 1116)

53
Q 
Vertigo is the prominent feature
A. Episodic ataxia type 1
B. Episodic ataxia type 2
C. Both
D. None of the above
A

B. Episodic ataxia type 2 (p. 1108)

54
Q 
Between the attacks, the patient is normal or has only minimal ataxia and nystagmus
A. Episodic ataxia type 1
B. Episodic ataxia type 2
C. Both
D. None of the above
A

B. Episodic ataxia type 2 (p. 1108)

55
Q 
Ataxic episodes are prevented strikingly by the administration of oral acetazolamide
A. Episodic ataxia type 1
B. Episodic ataxia type 2
C. Both
D. None of the above
A

B. Episodic ataxia type 2 (p. 1108)

56
Q 
This disorder has been found to be a mutation of the calcium channel gene on chromosome 19
A. Episodic ataxia type 1
B. Episodic ataxia type 2
C. Both
D. None of the above
A

B. Episodic ataxia type 2 (p. 1108)

57
Q 
Episodes of ataxia are precipitated by exercise
A. Episodic ataxia type 1
B. Episodic ataxia type 2
C. Both
D. None of the above
A

A. Episodic ataxia type 1 (p. 1108)

58
Q 
Myokymia is present between attacks
A. Episodic ataxia type 1
B. Episodic ataxia type 2
C. Both
D. None of the above
A

A. Episodic ataxia type 1 (p. 1108)

59
Q 
The disorder is caused by an abnormality of the K channel gene on chromosome 12
A. Episodic ataxia type 1
B. Episodic ataxia type 2
C. Both
D. None of the above
A

A. Episodic ataxia type 1 (p. 1108)

60
Q 
Most common early symptom in corticobasal degeneration
A. Asymmetrical clumsiness of the limbs
B. Rigidity
C. Tremor
D. Dysarthria
A

A. Asymmetrical clumsiness of the limbs (p. 1098)

61
Q 
This disorder is due to DYT1 mutation, which codes for the protein torsin A
A. Dystonia musculorum deformans
B. Segawa syndrome
C. X-linked dystonia parkinsonism
D. Wilson disease
A

A. Dystonia musculorum deformans (p. 1099)

62
Q 
This disorder is linked to mutation in a gene on chromosome 14q for the protein GTP cyclohydrolase 1 that is implicated in the synthesis of tetrahydrobiopterin, a cofactor for tyrosine hydroxylase
A. Dystonia musculorum deformans
B. Segawa syndrome
C. X-linked dystonia parkinsonism
D. Wilson disease
A

B. Segawa syndrome (p. 1100)

63
Q 
This disorder is due an unstable number of CAG repeats in a gene coding for ataxin-3
A. DRPLA
B. SCA1
C. SCA2
D. SCA3 (Machado-Joseph)
A

D. SCA3 (Machado-Joseph) (p. 1107)

64
Q 
The gene defect in this disease is an unstable CAG trinucleotide repeat in the gene that codes for the protein atrophin
A. DRPLA
B. SCA1
C. SCA2
D. SCA3 (Machado-Joseph)
A

A. DRPLA (p. 1107)

65
Q 
Most frequent form of SMA
A. SMA I (Werdnig-Hoffman disease)
B. SMA II (Dubowitz disease)
C. SMA III (Wohlfart-Kugelberg-Welander)
D. SMA IV
E. Kennedy syndrome
F. Fazio-Londe disease
A

A. SMA I (Werdnig-Hoffman disease) (p. 1117)

66
Q 
Severe infantile type of SMA
A. SMA I (Werdnig-Hoffman disease)
B. SMA II (Dubowitz disease)
C. SMA III (Wohlfart-Kugelberg-Welander)
D. SMA IV
E. Kennedy syndrome
F. Fazio-Londe disease
A

A. SMA I (Werdnig-Hoffman disease) (p. 1117)

67
Q

Opthalmoscopic exam triad in retinitis pigmentosa EXCEPT
A. Pigmentary deposits that assume the configuration of bone corpuscles
B. Attenuated vessels
C. Pale optic discs
D. Papilledema

A

D. Papilledema (p. 1122)

68
Q 
Dark choroid pattern on fluorescein angiography
A. Leber optic atrophy
B. Retinitis pigmentosa
C. Stargardt disease
D. All of the above
A

C. Stargardt disease (p. 1123)

69
Q 
The ratio of these products in Alzheimer's disease is critical to the neuronal toxicity of amyloid*
A. AB 42 to AB 40
B. AB 40 to AB 45
C. AB 45 to AB 42
D. AB 42 to AB 41
A

A. AB 42 to AB 40 (p. 1068)

70
Q

True of Alzheimer’s disease*
A. Marked reduction in choline acetyltransferase in the hippocampus and neocortex
B. Loss of cholinergic synthetic capacity in the raphe nucleus of the medulla
C. Increased concentration of glutamate in cortical and subcortical areas
D. Increased concentration of substance P, somatostatin, and CCK

A

A. Marked reduction in choline acetyltransferase in the hippocampus and neocortex (p. 1069)

71
Q

Results of the ADAGIO trial*
A. MAO-B inhibitors slowed disease progression as seen on later followup
B. There are mixed results regarding slowing of disease progression with rasagiline
C. Rasagiline is superior in controlling tremors in Parkinson’s Disease patients
D. None of the above

A

B. There are mixed results regarding slowing of disease progression with rasagiline (p. 1092)

72
Q 
Gene implicated in familial hemiplegic migraine*
A. CACNA1A
B. SCN1A
C. SCN2B
D. KCNA1
E. CACNB4
A

A. CACNA1A (p. 1103, Table 39-6)

73
Q 
Genes involved in ALS EXCEPT*
A. SOD1
B. APP
C. FUS
D. TARDP5P
E. VAPB
A

B. APP (Table 39-6 p. 1114)

74
Q

NOT true of neurodegenerative disease*
A. Begins after a long time of normal nervous system function
B. Does not have periods of relative stability
C. Family members often do not have signs of the disease
D. None of the above

A

B. Does not have periods of relative stability (p. 1061)

75
Q 
Drug of choice for delusions, hallucinations, and anxiety in Lewy-body dementia*
A. Tacrine
B. Donepezil
C. Rivastigmine
D. Galantamine
E. Memantine
A

C. Rivastigmine (p. 1075)

Adams 10th (27 decks)

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