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BRAIN BATTACK > CH38: Degenerative Diseases > Flashcards

CH38: Degenerative Diseases Flashcards

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1
Q

Process of neuronal, myelin or tissue breakdown, degradative productive of which evoke a reaction of phagocytosis and cellular astrogliosis but usually with minimal or no inflammation (p. 1082)

A

Degeneration

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2
Q

Degenerative diseases have 2 outstanding categories (p. 1083)

A
  1. Affect specific parts or functional systems of the nervous system
  2. Begin insidiously after a long period of normal nervous system function and pursue a gradually progressive course
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3
Q

Convulsions disease in Alzheimers disease are rare until late in the illness, when up to how many percent of patients are reportedly have infrequent seizures? (p. 1087)

A

5%

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4
Q

5 deficits that may represent the opening features of Alzheimer disease (p. 1087)

A 
Amnesia
Dysnomia
Visuospatial disorientation
Paranoia and personality changes
Executive dysfunction
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5
Q

In checking for naming, Alzheimer patients will fail (p. 1088)

A

below 8 items in any 1 category

or 25 items total if given to name in all 3

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6
Q

Criteria for Alzheimers: (p. 1088)

A
  1. dementia defined by clinical examination
  2. patient older than 40 years old
  3. deficits in 2 or more areas of cognition
  4. absence of disturbed consciousness
  5. exclusion of other brain diseases
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7
Q

Alzheimers brain is reduced by how many percent (p. 1089)

A

20%

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8
Q

Widespread loss of nerve cells in which layer of entorhinal cortex (p. 1089)

A

layer II

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9
Q

Marked neuronal loss in AD, which structures (p. 1089)

A

hippocampus
medial temporal cortex spec parahippocampal gyri and subiculum
nucleus basalis of Meynert and locus ceruleus

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10
Q

Pathological findings in AD:

A
  1. neurofibrillary tangles
    thick fiber-like strands of silver staining material in the forms of loops, coils or tangled masses
  2. Neuritic plaques
    spherical deposits of amorphous material scattered throughout the cerebral cortex and easily seen by PAS
  3. Granulovacuoular degeneration
    most evident in the pyramidal layer of hippocampus
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11
Q

In AD, Which correlates best with the severity of dementia (p. 1089)

A

Neurofibrillary tangles

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12
Q

If any part of the brain is disproportionately affected by the changes of AD it is the which structure (p. 1089)

A

Hippocampus particularly CA1 and CA2 zones
Entorhinal cortex
Subiculum
Amygdala

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13
Q

Discrete cytoskeletal protein that promotes the assembly of microtubules, stabilizes their structure and participates in synaptic plasticity (p. 1090)

A

Tau composed chemically of b transferrin

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14
Q

Protein toxic to several models of AD (p. 1090)

A

AB42

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15
Q

These are catalytic components of gamma secretase , the enzyme the oriduces the Abeta42 fragment (p. 1091)

A

Presenillin 1 and 2

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16
Q

Product of inadequate functioning of the progranulin gene that is also deposited in the neurons of that may impact the severity of AD; also implicated in the pathogenesis of FTD and motor neuron disease (p. 1091)

A

TDP-43

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17
Q

PET studies indicate that tau deposition in the inferior temporal and parietal cortices, more clearly than amyloid, differentiate normal older individuals from those with MCI or AD and (p. 1091)

A

Tau is more closely aligned with temporal lobe cortical atrophy

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18
Q

Proteins assocaited with early and late AD (p. 1093)

A

EARLY: APP, Presenillin 1, Presenillin 2;
LATE: Apolipoprotein E, Ubiquitin, TREM2

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19
Q

Which ApoE is associated with a tripling of thee risk of developing sporadic Alzheiers disease? (p. 1093)

A

ApoE4

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20
Q

This polymorphism is implicated in the AD causes inadequate phagocytic clearance of amyloid (p. 1093)

A

TREM2

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21
Q

Codes a protein that interacts with PS1 and PS2 and participates in proteasomal degradation (p. 1093)

A

UBQNL1

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22
Q

CT scan finding in AD (p. 1093)

A

disproportionate atrophy of the hippocampi and a corresponding enlargement of the temporal orns of the lateral ventricles

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23
Q

Beyond PET and related imaging studies there are no established biologic markers of Alzheimers disease with a possible exception of the (p. 1094)

A

ratio of tau to Ab42 (tau amyloid ratio)

The ratio is low in AD

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24
Q

Acetylcholinesterase inhibitor (p. 1095)

A

Donepezil

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25
Q

Acetyllcholine receptor antgonist succinulcholine is used prior to general anesthesia. Why need to be careful (p. 1095)

A

The effects may be prolonged in patients taking Donepezil

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26
Q

Which drug is slightly preferable in the treatment of psychosis, aggression or agitation? (p. 1095)

A

Olanzapine

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27
Q

Dementia and parkinsnism are related to NF changes in the cerebral cortex and substantia nigri (p. 1095)

A

Guamanian Parkinson- dementia complex

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28
Q

Dementia from motor neuron disease (p. 1095)

A

Neurofibrillary change is the most prominent feature wheres amyloid plaques are negligible in number or absent

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29
Q

Punch-drunk syndrome

A

chronic traumatic encephalopathy

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30
Q

What are the depoitions in Lobar Atrophies? (p. 1096)

A

Tau, progranulin, amyloid, synuclein

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31
Q

Depositon of agyrophilic intractoplasmic inclusions in FTD (p. 1096)

A

Pick bodies

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32
Q

Diffusely staining ballooned neurons (p. 1096)

A

Pick cells

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33
Q

Two main variants in lobar atrophies (p. 1097)

A 
Behavoiral variant
Language variant (progressive nonfluent, semantic, logopenic)
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34
Q

This disease is defined by diffuse involvement of cortical neurons with Lewy body inclusions and by an absence or inconspicous number of neurofibrillary tangles and amyloid plaque (p. 1097)

A

Lew Body Dementia

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35
Q

Main components of Lew Body (p. 1097)

A

Ubiquitin and alpha- synuclein

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36
Q

Diagnostic criteria for LBD (p. 1097)

A

2/3:
Parkinsonian syndrome
Fluctuations in behvior and cognition
Recurrent hallucinations

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37
Q

Characteristic of Parkinsons in LBD patients (p. 1097)

A

favorably repond to L Dopa INITIALLY or not at all

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38
Q

Most characteristic feature besides the movement disorder in LBD (p. 1098)

A

vacuous, anxious state with intermittent psuchotic or delirious behavior

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39
Q

Parkinsons Disease and LBD VS other dementias via PET (p. 1098)

A

reductions in lenticular nuclei and caudate activity in radiolabled dopamine transporter or dopamine receptor

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40
Q

For blood presssure changes in LBD, this drug is prescribed (p. 1098)

A

Midodrine

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41
Q

Anticholinesterse inhibitor used in reducing delusions, hallucinations and anxiet in LBD (p. 1098)

A

Rivastigmine

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42
Q

A selective serotonin 5-HT2A inverse agonist usd to treat psychosis in Parkinson disease which hs shown some modest benefit for psychosis nd hallucinations in a tria in LBD without worsening motor symptoms (p. 1089)

A

Primavanserin

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43
Q

Dominannt inherited adult- onset dementia with fulminant evolution (p. 1098)

A

Neuroserpinopathy

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44
Q

Findings in neuroserpinopathy (p. 1089)

A

Large eosinophilic, PAS- positive intaraneuronal inclusions that ocntain aggregates f neuroserpin

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45
Q

Triad is dominant inheritance, choreoathetosis, dementia (p. 1089)

A

Huntington disease

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46
Q

The CAG triplet codes for? (p. 1090)

A

glutamine

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47
Q

HDL2 Huntington disease like 2 is associated with?(p. 090)

A

CATCG, expansion of juntophilin 3 gene

HDL2 more associated with Westphal variant

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48
Q

First to appear in Huntingon disese (p. 1099)

A

Alterations of personality; Memory is spared

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49
Q

Opposite of HD and PD in terms of blinking (p. 1100)

A

In PD, blinking is diminshed; in HD blinking is increased

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50
Q

Gross pathology in HD (p. 1100)

A

Gross atrophy bilaterally of the hed of the caudate nucleus and putamen
Check bicaudate-to-cranial ratio
Spares the nucleus accumbens

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51
Q

Comment on size of sequence in HD (p. 1101)

A

The longer sequence on either 2 alleles determines the age of onset
The size of expansion of the normal allele exerting no influence

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52
Q

How many CAG repeats confers risk of developing clinical manifestation at some time re:HD? (p. 1101)

A

36

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53
Q

Pertubations of iron metabolism that may be toxic to neurons is found in (p. 1102)

A

Hallervorden-Spatz disease, now called PANK disease

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54
Q

Adult-onset chorea, myoclonus and rigidity (p. 1102)

A

DRPLA

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55
Q

Drugs that can cause chorea (p. 1102)

A

amphetamines, cocaine, TCA, lithim, isonizid, linezolid

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56
Q

Dopamine antagonis used to partially supre movement disorder of HD (p. 1102)

A

Haloperidol

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57
Q

Drug contraindicated in HDwho are suicidal (p. 1102)

A

Tetrbenazine and Deutetrabenazine

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58
Q

Finding in Famiial Dementia with Spastic Paraparesis patchy but gross swelling of the arterioles which gave teh staining reactions for amyloid (P. 1103)

A

Scholz perivascular plaques

59
Q

Mild dementia wbut wth impairment of retentive memory, dysnomia, dyscalculia, sometimes nonfluent aphasia and deficits in visual integratin; overshadowed by rigidity and spasticity of the limbs and peripheral nerve disorder (p. 1103)

A

Adult Polyglcosan Body Disease

60
Q

CBDG vs PD? (p.1106)

A

in CBGD, rigidity, hyperactive DTR nd Babnski signs are combined with aparaxia

61
Q

Most dependable feature of PD (p. 1106)

A

Early sign of resting alternating tremor that is more prominent in one arm

62
Q

How many percent of PD fail to display the characteristic tremor and approximately how many does not respond to L Dopa?(p. 1106)

A

25%

10% dont respond to LDopa

63
Q

Spastic bulbar palsy vs PD? (p. 1107)

A

Unilateral and bilateral CST
Hyperactive facial reflexes
Spasmodic crying, laughing

64
Q

Most constant and pertinent pathologic finding in both idiopathic and postencepalitic PD?(p. 1108)

A

loss of pigmented cells in the SN and other pigmented nuclei in LC, dorsam motor nucleus of vagus

65
Q

Pigmented nuclei contain eosinophilic cytoplasmic inclusions (p. 1108)

A

Lew bodies

66
Q

Rate limitiing enzme for the synthesis of Dopamine (p. 1108)

A

Tyrosine hydroxylase

67
Q

Earliest changes of the brain in PD (p. 1108)

A

cnges in dorsal glosopharngeal-vagal and anterior olfactory nuclei

68
Q

Neurotoxin that produces irreversible signs of parkinsonism and selective destruction of cells in the SN (p. 1108)

A

MPTP

69
Q

Ubiquiting protein ligase that participates in the removal of unecesary proteins from cells trough the proteasomal sytem (p. 1110)

A

Parkin

70
Q

Dopamine responsive dystonia (p. 1111)

A

Segawa disease

71
Q

COMTinhibitor that extends the plasma half-life and the duration of L-dopa effect by preventing its breakdown (p. 1112)

A

Entecapone

72
Q

Dopamine agonists (p. 1113)

A

Ropinorole

Pramipexole

73
Q

These drugs are not used in PD anymore because of the risk of cardiac valvular damage, particularly in higher dosage (p. 1113)

A

Pergolide

Cabergoline

74
Q

Anticholinergic drugs in PD (p. 1114)

A

Trihexypenidyl
Benztropine
Ethopropazine

75
Q

Mechanism of action is unknown but antagonism of NMDA or release of stored dopamine has been proposed (p. 1114)

A

Amantadine

76
Q

Attempts to slow the disease by vitamin antocoaxidants have met generally negative results except (p. 1114)

A

Coenzyme Q10

77
Q

This drug has been said to provide addtional benefit of suppressing dyskinesias in PD but its hematologic risks have led to its limited use (p. 115)

A

Clozapine

78
Q

DBS targets (p. 1116)

A

posterior and ventral medial parts of the subthalamic nucleus
globus pallidus interna

79
Q

Who will benefit DBS the most? (p. 1116)

A

Patient who maintain mobility but requires a dose of L-dopa that produces unacceptable dyskinesias and who is constantly cycling between on and off periods

80
Q

Mechanism of DBS (p. 1116)

A

high frequency impulses disrupt local neuronal activity; functional equivalent of an ablative lesion

81
Q

Hypotension in PD responds to (p. 1117)

A

Fludricortisone
Droxidopa
Pyridostigmine
Midodrine

82
Q

Post mortem findings in MSA (p. 1117)

A

extensive los of neurons in teh ona compacta of the SN; but no Lew bodies or NF tangles in the remaining cells
Secondary pallidal atrophy

83
Q

Why is there orthostatic hypotension in MSA?(p, 117)

A

los of intermediolateral horn cells and pigmented nuclei in the brain stem

84
Q

Other autonimic features of Shy Drager syndrome (p. 1117)

A 
erectile dysfunction
loss of sweating
dry mouth
miosis
urinary retention or incontinence
85
Q

Features not supportive of MSA (p. 1118)

A 
pill rolling tremor
hallucinations
dementia
family history of ataxia
eye movement are not prominent in MSA
86
Q

MRI findings in MSA (p. 1118)

A

hot cross bun

87
Q

Most common complaint for PSP (p. 1119)

A

unsteadiness of gait

88
Q

Characteristic symptoms of PSP (p. 1119)

A

supranuclear ophthalmoplegi
pseudobulbar palsy
axial dystonia

89
Q

Other eye signs in PSP (p. 1119)

A

ability to converge is los eventually

pupils become small but remain round and reactive to both light nd accomodtive stimuli

90
Q

Face of PSP (p. 1119)

A

worried appearance

91
Q

PSP vs PD (p. 1119)

A

facial expression of PSP is more tonic grimace than lck of movement
lack of tremor
erect rather than stopped posture
promience of occulomotor abnormalities

92
Q

Applause sign (p. 1119)

A

In PSP, fils to stop clapping after neig asked to do so only 3 times

93
Q

MRI finding in PSP (p. 1119)

A

Hummingbird sign

Mouse ears sign

94
Q

Gabanergic agonist of BZD receptor reported to ameliorate the akinesia and rigidity of PSP (p. 11121)

A

Zolpidem

95
Q

Most common presentation for CBGD (p. 11121)

A

asymmetrical clmsine of the limbs

96
Q

Ballooned and chromatolytic neurons with eccentric nucleu in CBGD (p. 1121)

A

neuronal chromasia

97
Q

Corticostriatospinal degeneration (p. 122)

A

Jakob disease

98
Q

2 categories of acanthocytosis with chorea (p. 1122)

A

HARP: hypobetalipoproteiniemia, acanthocytosis, retinitis pigmentosa, pallidal degeneration

Bassen Korenzweig disease: mainly lacks lipid abnormality

99
Q

Disorder with acanthocytosis with gradual development of chorea in middle to late life, characterized by degeneration of the caudata and putamen and a myopathy (p. 1123)

A

McLeod disease

remember: KX protein, Kell antigen

100
Q

Most common mutation in dystonia musculorum deformans (p. 1123)

A

DYT1 which encodes torsin A; deletion of a single glutamate from the torsin A peptide

101
Q

Protein affected in Segawa syndrome (p. 1124)

A

GTP cyclohydrolase

102
Q

Feature of Segawa syndrome (p. 1125)

A

disappearance of marked subsidence of the symptoms after a period of sleep and worsenig as the day progresses

103
Q

Expresion of this trinucelotide is most common in Friedreich Ataxia (p. 1127)

A

GAA tricucleotide repeat within FXN

104
Q

Other notes in Freidreich Ataxia (p. 1127)

A

emotional lability had been prominent
tendon reflexes are abolished
half of patients have cardiomyopathy
gene: frataxin

105
Q

Pathologic finding in Freidreich Ataxia (p. 1128)

A

spinal cord is thin

posterior columns, CST and SC tracts are all depleted of myelinated fibers

106
Q

Vitamen E deficiency vs Freidrich ataxia (p. 1128)

A

absence of dysarthria and of skeletal or cardiac abrnormalities in vitamin-deficiency illness

107
Q

Treatment improves cerebellar symptoms of FA (p. 1128)

A

oral 5- hydroxytryptophan

108
Q

Treatment reducing progression of ventricular hypertrophy in Freideich ataxia (p. 128)

A

idebenone

109
Q

What is the main distinction of Cerebellar Cortical Ataxia and MSA with cerebellar degeneration? (p. 1129)

A

In CC,pontine nuclei is spared.

110
Q

Pathological finding in Holmes type (p. 1129)

A

symmetrical atrophy of the cerebellum mainlythe anterior lobe and vermis

111
Q

Alcoholic cerebellar degeneration vs Holmes type cerebellar cortical ataxia (p. 1129)

A

In alcohol-nutritional disase, there is accompnying polyneuropathy and reduced ankle reflexes

112
Q

Fragile X Tremor- Ataxic Premutation Syndrome (p. 1130)

A

50 to 200 CGG repeat sequence

113
Q

OPCA with neuropathy and sloed eye movements (p. 1131)

A

Wadia type

114
Q

Findings in Machado-Joseph-Azorean Disease (p. 1131)

A

degeneration of the dentate nuclei and spinocerebellar tract and loss of anterior horn cells, neurons of pons, SN and occulomotor nuclei
unstable number of CAG repeating sequences in ataxin-3

115
Q

MRI findings in Machado-Joseph-Azorean diseae (p. 1131)

A

reduced width of the superior and middle cerebellar peduncles, atroph of the frontal and temporal lobe, smallness of the pons and globus pallidus

116
Q

DRPLA vs Huntington (p. 1131)

A

main consideration when chorea is a prominent feature

117
Q

Notes on DRPLA (p. 1131)

A

unstable CAG trinucleotide repeat in ATN1 which codes for protein atrophin 1

118
Q

On paroxysmal ataxias, this drug is proven effective to lessen the attacks in patients with calcium channel gene mutation (p. 112)

A

Oral acetazolamide

119
Q

Triad of ALS (p. 1134)

A

atrophic weaknes of the hands and forearms
fasciculations and slifht spasticity of the arms and legs
generalized hyperreflexia

120
Q

Principal pathologic finding in ALS (p. 1135)

A

loss of nerve cells in teh anterior horns of the spinal cord and motor nuclei of the lower brainstem

121
Q

Comment on SOD1 gene ALS (p. 1136)

A

the nonmotor systems seem to be more affected

122
Q

slower pace than usual ALS, some partients surviving for 15 years or longer
illness presentation is symmetrical
(p. 1136)

A

Progresive Musculular Atrophy

123
Q

Difference between PMA and ALS (p. 1136)

A

tendon reflexes are diminished in PMA

124
Q

Reflex found in progressive bulbar palsy (p. 1136)

A

Bulldogreflex

jaw snaps hut involuntarily

125
Q

20% of ALS suspects have a slowly progressive corticospinal tract disorder that begins with pure spastic paraparesis (p. 1137)

A

Primary Lateral Sclerosis

126
Q

Partial cervical and spinal amyotrophy (p. 138)

A

Hirayama disease

127
Q

40%of forms of ALS with known family history is assocaited with the hexanucelotide expansion of __ (p. 1138)

A

C9orf72 gene

128
Q

Treatment for ALS (p. 1139)

A

Antigultamate agent Riluzole
Edaravone
Masitinib

129
Q

The ability to count to 25 ith a full efort in a single breath correponds to the vital capcity of approximately ___(p. 1140)

A

2.5L

130
Q

This determines teh severity and time of onset of SMA (p. 1141)

A

SMN2

131
Q

Types of SMA (p. 1141)

A

SMA1 Werdnig Hoffmann
SMA2 Dubowitz
SMA3 Kugelberg-Welnder

132
Q

After cystic fibrosis, it is the most frequent cause of death from a recessively inherited disease (p. 1141)

A

Werdng-Hoffman disease

133
Q

Characteristic posture of SMA1: (p. 1141)

A

arms abducted and flexed at the elbow, legsin the frog position with external rotation and abduction at hips and flexion at hips and knees

134
Q

SMA vs fatty acid metaolism disorders (p. 1142)

A

preservaton of tendon reflexes

relative lack of preogression of muscle weaknes

135
Q

This antisense oligonucleotide modifies the spilicing of SMN2 to result in increased production of SMN protein in motor neurons and comepnsate for the mutated SMN1. (p. 1143)

A

Nusinersen

136
Q

Distal muscular atrophy with prominet bulbar signs and less often, ocular palsies (p. 1143)

A

Kennedy Disease

137
Q

Mutation in Kennedy Disease (p. 1143)

A

SLC52A3, a riboflvin transporter

hence some beneficial effect is derived from administration of riboflavin

138
Q

In Leber Herediatary Optic Atrophy, which color is first affected (p. 1146)

A

blue-yellow; red-green is relatively preserved

139
Q

Primary structural change in Leber Hereditary Optic Atrophy (p. 1146)

A

Peripapillary vasculopathy

140
Q

In RP, affected are which laters (p. 1146)

A

BOTH neuroepithelium and pigment epithelium

141
Q

First symptom in RP (p. 1146)

A

nyctalopia

142
Q

Ophthalmoscopic examination in RP (p. 1146)

A

pigmentary deposits that assume the configuration of bone corpuscle, attenuated vessels and pallor of optic discs

143
Q

Patognomonic “dark choroid” pattern (p. 1147)

A

Stargardt Disease

144
Q

Hereditary Hearing Los wth Retinal Disease (p. 1147)

A

RP:
Usher syndrome: with congentital hearing loss
Refsum syndrome: with polyneuropathy
Alstrom sndrome: with hypogonadism
Cockayne syndrome: with dwarfism, mental retardation, premature senility and photosensitive dermatitis

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