neuro Flashcards
(139 cards)
1
Q
causes of Intracranial htn aka pseudotumor cerebri
A
vit a derivatives, tetracycline abx, hormonal contraceptives, lithium, minocycline, tamoxifen, coricosteroid withdrawal.
2
Q
parkinson’s dz
A
loss of dopamime-producig neurons in the substantia nigra
3
Q
Creutzfeld jakob dz
A
myocloic jerks, rapid progression towards dementia, akinetic mutism with specific EEG findings.
mean duration of illness: 4-5 mo
prions, small infections particouls that continu protein replace normal proteins over time, causing neurotoxicity.
dx: diffusion weigted MRI- see hyperintense lesions of corpus striatum, caudate head, putamen, cerebral cortex
dx requires positive real time quaking induced conversion RT-AQuIC test Or progressive dementia with MRI findings, EEG findings
EEG- periodic sharp wave complexes
4
Q
subacute combined degeneration of spinal cord
A
degeneration of posterior and lateral columns of spinal cord due to b12 dficieny.
sx limb weakness, ataxia, megoblastic anemia, GI sx, neuro sx like dementia, depression, muscle weakness
5
Q
anterior trigeminothalamic tract
A
pain and temperature info from the face, head, and neck.
6
Q
prosopagnosia
A
facial recognition
fusiform gyrus
-ventral stream on ventral surgace of temporal lobe on lateral side of fusiform gyrus
7
Q
gerstmann’s syndrome
A
right to left confusion, finger agnosia, dysgraphia and dyslexia, dyscalculia, contralateral hermianoia, lower quadrantanopia
inferior parietal lobule
associated iwth brain lesions in dominant (usually left) hemisphere, including angular and supramarginal gyri near temporal and parietal lobe junction
8
Q
frontal lobes
A
motor function, problem solving, spontaneity, memory, language, initiation, judgement, impulse control, social and sexual behavior
9
Q
L frontal lobe
A
language related movement
10
Q
R frontal lobe
A
non verbal abilities
11
Q
increases risk for MCI and dementia
A
HTN, HLD, DM
12
Q
adrenoleukodystrophy
A
x linked dz of peroxisomal fatty acid beta oxidation
very long chain fatty acids accumulate in tissues, such as myein in CNS and adnrenal cortex.
adrenal failure
sx vision and hearing impairment, aphasia, hyperactivity, paralysisi, seizures, muscle weakness, adrenal failure, coma
13
Q
level increases after seizures
A
prolactin.
within 20 minutes of generalized tonic clonic.
increased by 5-30x
can exclude pseudoseizures
14
Q
Meige syndrome
A
often misdiagnoses as tmj dysfunction
sx incresed forceful blinking (blepharospasm) tongue/jaw contractions (oromandibular dystonia). occaussional tongue protrusions, cervical dystonia,
botox helps, no treatment
15
Q
parkinson’s dz
A
shaking, stiffness, difficulty walking, balance, coordination
16
Q
whipple disease
A
caused by gram + bacterium, tropheryma whippelii.
malabsorption syndrome with small intestine involvement, joint pain, CNS and CV sx
17
Q
sleep
A
stages 1-3 NREM
REM
adults sleep s 75% NREM
stage 1: light sleep, 5%
alpha and theta waves
2: little deeper, 50% sleep time
sleep spindles and k complexes on EEG
3: deeper sleep of slow delta wave on eeg, 10-20%
REM: 20-25%, eeg resembles wake time.
each REM and NREM cycle is ab 90-120 minutes with REM periods increased in 2n half of night.
Newborns get circadian rhythym at 3 mo
-REM at sleep onset
-increased REM time with dec NREM time
-get NREM at 3 mo
aging adults: stage 3 of NREM decreased, slee time in stage 1 NREM increases.
18
Q
10x increase risk of Alzhemer’s DZ
A
hx of TBI and associated apolipoprotein E epsilon 4 allele
19
Q
dementia pugilistica aka punch syndrome
A
subtye of chronic traumatic encephalopathy, nuerodegenerative dz with features of dementia
associated with buildupof beta amyloid deposits.
20
Q
kluver-bucy syndrome
A
lesions to bilateral anterior temporal lobes/amygdala cause hypersexualitiy, hyperphagia, hyperorality, visual agnosia, docility, hypermetamorphosis
associated w/ FTT
21
Q
Ethosuximide MOA
A
lowers T type calcium currents
absence seizures
SE nausea, HAs, dizziness, hyperactivity
22
Q
MS diagnosis
A
can be MRI + clinical findings or clinical findings alone
2 or more attacks involving different parts of CNS, separated by at least 1 month, and lasting minimum of 24 hours.
23
Q
hypothalamus function
A
sleep, hydration, temperature.
works with pons to control sleep wake cycle
anterior: cooling and parasympathetic
(a/c=anterior cooling)
posterior: heating, sympathetic
24
Q
thalamus
A
body’s sensory relay station
25
blood brain barrier cells
endothelial
astrocyte end feet
pericytes
26
black curtain coming down over eye,
amaurosis fugax
internal carotic artery occlusion, TIA
27
naps for narcolepsy
10-20 min 2x/day
28
HARP Syndrome
Hypobetalipoproteinemia- disorder of lipoprotein metabolism characterized by decreased plasma concentrations of low-density lipoprotein (LDL)-cholesterol and apolipoprotein B (apoB).
Acanthocytosis- misshaped RBCs
Retinitis pigmentosa
Pallidal degeneration- Neurodegeneration involving the globus pallidus,a part of the basal ganglia that is involved in the regulation of voluntary movemen
29
frontotemporal dementia
aka pick dz
behavioral changes,
increase in disinhibition
atrophy of BL frontal lobes
30
forebrain, prosencephalon, gives rise to telencephalon and diencephalon.
what does diencephalon give rise to?
thalamus
hypothalamus
epithalamus
retina
pineal gland
3rd ventricle
31
alzheimer's dz
extracellular amyloid plaques: consist of amyloid beta which is generated from amyloid precursor protein
neurofibrillary tangles- formed from hyperphospholylated tau protein
Hirano bodies: intracellular eosinophilic rod shaped aggregates formed from actin or actin associated proteins found in neurons.
degeneration of cholinergic neurons in the nucleus basalis of Meynert
amyloid deposits, neuritic plaques, neurofibrillary tangles from tau protein
APP on chromosome 21 is cleaved by proteases, producing insoluble beta-amyloid.
32
Binswanger's dz
dementia + 2 of the following: htn, vascular dz, cerebral vascualar dz, subcortical dysfuntion (neurogenic bladder, muscle rigidity, gait abnormalities), BL CT/MRI abnormalties like leukoaraisis or attenuation of white matter on CT or MRI
sx: reflex asymmetries, rigidity, limb ataxia, syncope, pseudobulbar palsy
33
temporal lobe epilepsy
mc focal epilepsy in adults.
mc cause is mesial temporal sclerosis with hippocampal sclerosis and temporal lobe atrophy.
mc etiology- prolonged febrile seizures and anoxia at birth, temporal lobe infections, masses
complex partial seizures: focal with impaired awareness are common
34
periodic sharp waves on eeg
Creutzfeldt jakob dz
35
mamillary body lesions
psychosis, memory deficits, confabulation
36
amygdala lesions
hypersexuality, hyperorality, hyperphagia
37
thalamus lesions
medial- language
right- visual memory
mood,
sleep wake cycle-anterior and medial thalamus
lateral geniculate nucleus receives projections from retina
lateral= light (vision)
medial geniculate nucleus is involved in auditory processing
medial=music (auditory)
38
jacksonian march
simple focal seizure that begins in finger, toe and corner of the mouth then spreads to ipsilateral area of body.
from distal part of limb, towards ipsi face, through primary motor cortex in succession
39
internuclear opthalmoplegia
Multiple sclerosis
injury to Medial Longitudinal fasciculus (MLF)
adduction + CL abduction with nystagmus , diplopia
40
synringomyelia
atrophy in hands, weakness, senory loss. pain and temp in a shawl-like distribution across shoulders
diminished reflexes.
tx- surgical
41
prosopagnosia
cannot recall visual stimulation that requrires memory
fusiform gyrus lesions associated with face recognition
42
basilar migraine
transient headache, quadriplegia, stupor, blindness, coma
43
Alzheimers dz treatment
REVERSIBLE cholinesterase inhibitiors-donepezil, rivastigmine, galantamine
-they increase vagal tone, cardiac patients should avioid.
-donepezil and rivastigmine are associated with vivid dreams.
-SEs of reversible cholinesterase inhibitors are GI upset
nmda receptor antagonist- memantine
--decreases excitotoxicity
44
subclavian syndrome
L ARM claudication
stenosis of subclavian artery causing reversal of blood flow in vertebral artery
"stealing blood flow:
45
MRI in schizophrenia
abnormalities of dorsolateral prefrontal cortex.
reduced vol in prefrontal, thalamic, hippocampal, superior temperoal gyrus.
increased lateral and 3rd ventricle volume and basal ganglia
decreased blood flow in frontal lobes
46
chronic lead poisoning
CNS deficits, peripheral neuropathy, nephropathy, htn, anemia, short term memroy loss, depression, nausea, abdominal pain.
47
myotonic dystrophy aka DM2 aka proximal myotonic myopathy
mild grip or percussion myotonia and weakness in the neck flexors, elbow extensors, finger flexors, hip flexors.
tetranucleotide repeat
CCTG
zinc finger protein 9 gene
DM1 is more severe-auto dominant, cataracts, conduction abnormalities, respiratory muscle involvement.
DM1 is autodominant trinucleotide repeat of CTG in Dystrophia myotonica protein kinase gene (DMPK)
48
DM1 aka steinert's dz
Myotonic dystrophy type 1
Muscle dz causing multi organ damage
cranial muscle wasting/weakness.
distal limb weakness
percussion and grip myotonia, muscle weakness in the face, neck, forearm, intrinsic hand and foot dorsiflexors. muscle pain, respiratory muscle involvement, dysphagia, dysarthria.
cataracts, cardiac conduction abnormalities, infertility, insulin resistance.
autosomal dominant
CTG trinucleotide repeat region
49
cluster headache abortive therapy
sumatriptan
with supplemental O2
for prophylaxis and treatment- verapamil
eeg monitoring for potential SEs
unilateral orbital pain, temple pain; associated with autonomic sx like rhinorrhea, conjunctival injection, lacrimation , miosis, ptosis
other treatment:
galcanezumab, lithium, topiramate
50
subacute lead exposure
asymmetric primary motor neuropathy
51
primary progressive aphasia (PPA)
subtype of frontal temporal dementia.
clinical diagnosis. atrophy of frontal lobes.
52
signs of meningitis + periodic lateralized epileptiform discharges (PLEDs)
HSV
tx acyclovir
53
pattern of atonic seizures
slow spikes and waves
this is typical in Lennox-Gastaut syndrome as well.
54
Gastaut Geschwind syndrome
hyperreligiosity, circumstantiality, hypergraphia, hyposexuality, intense emotional responses.
focal seizures with impaired awareness (complex partial seizures)
temporal lobe epilepsy, left side comon
55
spongiform encephalopathy
prion protein carred on short arm of chromosome 20
56
MC primary lesion leading to brain mets
NSCLC
-melanoma and SCLC are less commo but have greater risk to metastasize to brain
order of brain mets:
1. NSCLC
2. Breast
3. Renal, GI, SCLC, melanoma
57
familial hemiplegic migraines
auto dominant
get migraines with auras that have unilateral hemiparesis and occassional cerebellar sx
pt freq get treated for stroke
genetic variants: FHMI1
associated w episodic ataxia 2 spinocerebellar ataxia type 6 and childhood absence epilepsy
58
produces ACTH
anterior pituitary
also AP makes TSH, FSH, LH, GH, prolactin
"FLATPEG"
e for endorphins
59
sleepwalking
NREM sleep,
ages 8-12
shouldn't wake child
60
neurotransmitters affected in alzhemier's
ACH & NE
61
cortical blindness
aka Anton's Syndrome
caused by BL occipital damage due to ischemia, trauma or other ie hypertensive encephalopathy with preeclampsia.
62
wernicke's area
temporoparietal junction, usually L side
wernickes is wordy- can still speak
speech comprehesion, temporal lobe on L side of brain
63
broca's
L side of frontal lob, speech production
64
mortality rate at 3 mo of pts with delirium
22-33%
65
NARP syndrome
neuropathy, ataxia, retinitis pigmentosa (NARP)
proximal weakness, sensory motor neuropathy, ataxia, retinitis,
due to mitochondrail DNS d/o
66
Wilson DZ
auto recessive
mutation on ATP7B gene chrom 13
copper transport deficit, causing deposits in brain and liver
sx cirrhosis, tremor, ataxia, psycosis, depression
kayser-fleischer rings
dx by serum ceruloplasmin.
tx: copper chelators like penacillamine.
zinc increases fecal copper extretion
67
benign rolandic seizures
MC childhood epilepsy
usually outgrow by adolescent
occur at nighttime, inc risk of fatal apiration
68
3 types of alpha-synucleinopathy
Parkinson's Dz
Demential with lewy bodies
multliple system atrophy
69
tauopathies
aggregation of tau protein in brain
alzheimers DZ
Picks Dz (frontotemproal dementia)
corticobasal degeneration
70
cholinesterase inhibitors
Donepezil (reversible)
Rivastigmine
Galantamine
Tacrine
to treat Alzheimers Dementia
71
avoid in lewy body dementia
FGA ie haldol
inc eps, pts are very sensitiv to strong D2 antagonism, can become paralyzed
72
involved in sleep/wakefullness
ventrolateral preoptic nuclei
73
dx dementia w lewy bdies on imaging
reduced dopamine transporter uptake in basal ganglia on SPECT imaging
74
overall diag of DLB
cognitive impairment + 2 clinical features:
fluctuating cognition
REM sleep behavior DO
Visual hallucinations
parkinsonism
75
paraneoplastic cerebellar degeneration
paraneoplastic sydrome associated w tumors of lung (small cell), ovary, breast, hodkin's lymphoma
mc are ovanian and small cell
rapid progression
key finding: dysarthria, ataxia (midline and all limbs), nystagmus, nausea, vomitting, diploplia
76
FDA approved for tourettes
pimozide
haloperidol
aripiprazole
77
cells that produce myeline
oligodendrcytes
78
williams syndrome
rare neurodevelopmental dzdue to deletion of 26 genes from chromosome 7
elfin face
low nasal bridge
unusual cheerful social demeaner
developmental delay
strong language skills
cardiovascular problems like aortic stenoisi
hyperacusis
strabismus
79
fabry dz
x linked lysosomal do
2nd most prevalent lysosomal dz after Gauchers
acroparesthesias, (painful numbness)
GI sx/pain, corneal opacity, hypohidrosis, angiokeratomas, tinnitus, hearing loss
Fabry: deficiency in alpha galatosidase A (AGA)
Gaucher’s: glucocerebrosidase deficiency
80
watershed infarcts
at the border zones at the junction of 2 different drainage areas
10% of all infarcts
2 types- external or internal
81
increases after stroke due to damage of neurons
Ca2+
due to depolarization of neurons=heightened electrical activity=increased glutatmate
82
SE of pheynytoin
diplopia, ataxia, hirsutism, neuropathy, gingival hyperplasia
83
located in mesencephalon
production of dopamine, ACH
84
visual processing
thalamus
thalamus does all sensory processing except smell
85
can visualize a seizure focus more precisely
PET
***detects region where glucose uptake is LOW (hypometabolism), associated with site of seizure origin
86
interprets visual stimuli and facillitates object recognition
temporal lobe
87
pathophysiology of NMS
reduced serum iron stores decrease dopamine receptor function
88
cryptococcal menigitis
AIDS, cd4 < 100
india ink test
tx amphotericin B, flucytosine
89
herpes simplex encephalitis
sudden onset of sx
HA, ABDOMINAL sx, seizure, leukocytosis
can cause gustatory and olfactory hallucinations also
PERIODIC LATERALIZED epieptiform discharges (PLEDs)
tx IV acyclovir
90
prevalence of ApoE-e4 in general AD population
50%
MC genetic risk factor
APP on chrom 21, presenilin-1 on chrom 14, presenilin-2 on chrom 1 make up 1%
91
tx of serotonin syndrome
remove agents
BP (esmolol, nitroprusside)
benzos for myoclonic jerking and agitation
IV hydration to precent renal failure
92
neurotransmitter produced in tuberomammillary nucleus
histamine, itching
in hypothalamus, promotes arousal
93
chromosome associated w narcolepsy
6
94
csf of chronic meningits
lymphocytic pleocytosis
dec glucose
elevated protein
4 weeks or more
95
kleine-levin syndrome
male adolescents
cyclical episodes
excessive daytime sleepiness
cognitive impariement
hyperphagia
apathy
hypersexuality
unknown cause
96
AD pathologic findings
amyloid plaques, neurofibrillary tangles
amyloid plaques- amyloid beta from APP
neurofibrillary tangles-hyperphosphorylated tau protein
97
REM sleep behavioral disorder (RBD)
kicking, screaming, punching, hitting during dream.
associated w Parkinson's dz, muscle system atrophy patients, Lewy body dementia
98
apraxia
inability to execute learned, purposeful movements like hair brushing
difficulty drawing or navigating ones neighborhood
NON DOMINANT (mc right) or BL parieto-occipital regions
99
mimics TIAs
somatosensory seizures, migraines
100
MC pathological finding in epilepsy
hippocampal sclerosis
65-80% of epilepsy involve mesial temporal sclerosis with gliosis and pyramidal cell loss in hippocampus
101
drawing the clock wrong, all numbers on the right side
L sided hemiparesis and L sided neglect
affected area is R parietal lobe
102
hippus
autonomic phenomenon
pupils dialte and contract with rhythmic pattern
can be normal or indicate subclinical or continuing seizure recurrence
103
longest sleep stage
stage 2- 50%
sleep spindles and K-complexes on EEG
104
mechanism of narcolepsy
dysfunctional hypocretin peptide pathway
105
thing to tell patients treated with antipsychotics that have Lewy Body Dementia
black box warning on all antipsychotics
1.5-1.7 increased risk of all cause mortality
106
dressing apraxia
inability to execute learned purposeful movements like hair brushing
localized to non dominant biliateral parieto occipital regions
R parietal lobe
107
lobe responsible for auditory and visual processing, retaining memories, language comprehension, naming
temporal lobes
superior temporal gyrus-wernicke's area respnsible for speech comprehension
108
treatment for TCA toxicity
IV sodium bicarbonate
109
mesencephalon
midbrain
tectum
cerebral aqueduct
tegmentum
cerebral peduncles
110
diencephalon
thalamus, hypothalamus
111
hindbrain
pons, cerebellum
112
myasthenia gravis
antibodies block , destroy or inactivate nueromuscular acetylcholine receptors.
113
entacapone
COMT inhibitor, allows levodopa to reach brain, prevents COMT from metabolzing it.
114
part of brain responsible for smell
LIMBIC
temporal
frontal
115
conduction aphasia
left hemispheric dominant lesions
area is arcuate fasciculus that connects Wernicke's and Broca's area.
inability to repeat normal speech and possibility of paraphasic errors and hesitancy.
associated iwth right hemiparesis, reight hemisensory loss, right hemianopsia, limb apraxia
116
paroxysmal hemicrania
primary HA disorder. attacks, facial pain 2-30 min, many times per day, autonomic sx like lacrimationm conjuntival injection,
horner syndrome
tx : indo methacin
117
sydenham's chorea
results from childhood infection with group A beta hemolytic strep 20-30% of pts with rhematic fever.
thought to be related to chorea gravidarum. nonrhythmic movements of arms, legs, trunk. due to elevated estrogen.
118
korsakoff syndrome
chronic complication of thiamine deficiency
anterograde AND retrograde amnesia
119
wernicke
ophthalmoplegia
peripheral neuropathy
ataxia
nystagmus
delirium
punctate lesions of gray nuclei
persistent amnestic syndrome cna remain after thiamine repletion
120
MC primary lesion leading to brain mets
Non small cell lc
121
medulloblastomas
primary neuroectodermal tumors
mc primary tumor in children
highly malignant, but radiosensitive
122
serotonin syndrome
clonus within exposure to serotonergic drug within 5 weeks.
cocain inhibits uptake of serotonin
confusion
hyperthermia
myoclonus
tachycardia
hypertension
hyperreflexia
123
limbic encepahitis
anti nmda receptor encephalitis
young women
associated with ovarian teratomas, paraneoplaistic syndrome
pychosis, anterograde amnesia, SI, dyskenesias, sentral resp dysfucntion, autonoimc instability
T2 flair in mesial temporal lobes
124
phenytoin SE
dipolopia, ataxia, hirsutism, neuropathy, gingival hyperplasia
125
endartecrectomy
70% or more stenosis
126
paroxysmal hemicrania
headache disorder
2-3 min many times per day
autonomic sx: lacrimation, conjunctival injection, Horner Syndrome
Neck Movements may trigger episodes.
tx: indomethacin
127
elevated in ETOH withdrawl, causes autonomic sx
glutamate
128
macroglia cell types
astrocytes: support endothelial cells that form BBB
oligodendrocutes: insulte axons by making myelin in CNS
shwann cells: produce myelin in PNS
ependymal cells: line ventricles and are part of CSP production
radial glia- primary progenitors capable of becoming neurons, astrocytes, oligodendrocytes.
129
types of cells in braine
neurons
glial cells: astrocytes, oligodendrocytes, microglia
130
part of brain responsible for sensory integration and somatosensory formation
parietal lobe!
temporal: hearing, naming, viusal recognition
frontal : executive fuction, social conduct, judgement, insight, motor function
occipital: visual functions
131
dementia with lewy bodies
dementia plus 2 features:
cognitive fluctuations
visual hallucinations
Rapid eye movement REM
sleep behavior disorder- RBD
parkinsonism
most specific test: PET and SPECT
AVOID haldol, they are sensive to antipsychotics , can be paralyzed. seroquel or clozaril are less likely to cause severe sx
LEW BODY INCLUSIONS: round, eosiniophilic intraneuronal cyctoplasmic inclusions
132
modafinil
dopanime reuptake inhibitor
SE:htn, in HR, diarrhea, HA, insomnia
FDA approved for narcolepsy, shift work DO, OSA excessive tiredness.
133
fragile x associated ataxia syndrome
similar to parkinsons
see generalized cortical and cerebellar atrophy with inc T2 signal intensity on MRI.
134
tabes dorsalis
demyelination in posterior dorsal columns of SC secondary to untreaed syphilis
sx: weakness, paresthesias, dimished reflexes, locomotor ataxia
135
genetic Alzheimer's chormonsome
apolipoproptein E
Chrom 19
136
Parkinsons
loss of dopaminergic neurons in substantia nigra
137
genetic risk for early onset alzheimers
presenilin 1 & 2
amyloid precursor protein gene
apolipoprotein E4
138
pathology of huntingtons
accumulation of intracellular toxic proteins
overproduction of huntingtin, synaptic vesicle associated protein appears to trigger NMDA receptor mediated excitotoxiciity
ultimately resulting in degeneration of neostriatum (caudate nucleus and putamen)
139
domains on MMSE
orientation
recall
attention
calculation
language manipulation and constructional praxis
