Neuro diseases Flashcards

1
Q

What is the most common NMJ disorder? It’s a ____ disorder characterized by _____

A

Myesthenia gravis
Autoimmune
General muscle weakness

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2
Q

Main subtype of GBS in NA

A

Acute inflammatory demyelinated polyneuropathy

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3
Q

Describe the pattern of paralysis in GBS

A

Acute ascending flaccid paralysis

lower –> upper limbs

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4
Q

Mechanism of autoimmunity in GBS?

A

Molecular mimicry (–> T cells –> B cells –> Abs –> macrophages)

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5
Q

Demyelination in GBS is ___

A

Segmental

Schwann cells remyelinating at first

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6
Q

Damage to ___ nerve can cause respiratory failure/death in GBS

A

Phrenic nerve

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7
Q

3 diagnostic tests in GBS

A

Lumbar puncture
NCV/EMG
PFTs

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8
Q

What will NCV/EMG show in GBS

A

reduced/blocked NCV

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9
Q

What will CSF show in GBS

A

Albuminocytologic dissociation

High protein/albumin without high WBCs

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10
Q

Treatment for GBS

A

supportive + pain management
IVIg & plasmapheresis can help speed recovery but don’t impact prognosis
STEROIDS DON’T HELP IGs are already floating around

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11
Q

Nadir of GBS usually reached within ___

A

1 month

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12
Q

Carpal tunnel syndrome involves compression of the __ nerve

A

Median

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13
Q

in carpal tunnel syndrome what are the main symptoms/sensations and where

A

pain, numbness, tingling (starts as dull ache)
of thumb, index, middle finger, ring finger on lateral side
Also muscle weakness/clumsiness

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14
Q

What makes up the roof of the carpal tunnel?

A

Transverse carpal ligament = flexor retinaculum

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15
Q

How many nerve are within the carpal tunnel?

A

Only one (median)

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16
Q

What atrophy can be seen in carpal tunnel

A

Thenar muscle (recurrent branch of median nerve)

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17
Q

Why is the sensation at the central base of palm unaffected in peripheral neuropathy

A

Palmar branch of median nerve is unaffected because branches off upstream of carpal tunnel

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18
Q

5 treatments for carpal tunnel

A
Behaviour modification/ergonomics
Physical therapy
Splinting
Corticosteroids
Surgical division of transverse carpal ligament
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19
Q

Name 4 patterns of MS progression

A

1) Relapsing-remitting (90% @ diagnosis)
2) Secondary progressive (50% become this)
3) Primary progressive
4) Progressive-relapsing

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20
Q

Most often earliest manifestation of MS = ____; can cause what defect that can be seen on physical exam?

A
Optic neuritis (inflammatory demyelination of optic nerve)
Relative afferent pupillary defect
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21
Q

What is Lhermitte sign?

A

Shooting electric sensation travelling down spine when you flex neck
Seen in demyelinating diseases like MS

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22
Q

Cerebellar involvement in MS leads to what pattern of symptoms?

A

Charcot neurological traid: Disarthria/scanning speech, Nystagmus, Intention tremor

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23
Q

1st choice test for MS

What do you see?

A

MRI of brain + spine with/without Gado

Shows white matter PLAQUES

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24
Q

Gado enhances active lesions up to ____ after attack

A

6 weeks

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25
Other than MRI, what diagnostics can be done for MS? (2)
1) Visual evoked potentials (EEG) - slowed optic nerve conduction 2) CSF (lymphocytic pleocytosis, oligoclonal bands (intrathecal IgG synth), myelin basic protein
26
Summarize the McDonald Criteria for MS diagnosis
1) Dissemination in time: 2+ attackes 30+ days apart (or MRI showing enhancing + non-enhancin lesions, or CSF oligoclonal bands) 2) Dissemination in space: lesions in 2+ diff regions of CNS confirmed by objective diagnostic findings
27
Treatment of acute MS exacerbation
High-dose IV glucocorticoids 2nd line: phalsmapheresis Other options: ACTH gel (if corticosteroids don't work), IVIg, cyclophosphamide
28
What is a disease-modifying MS drug that suppresses T cell activity and can be used to prevent exacerbations in all types of MS? Rom?
Interferon beta | SubQ
29
What is acute transverse myelitis? Most common cause?
Acute inflammation of grey/white matter in 1+ adjacent spinal cord segment (usually thoracic) MS = most common cause (others include vasculitis, SLE, anti-PL syndrome, other autoimmune disorders/infections)
30
Testing for acute transverse myelitis
MRI (spine) for cord swelling, r/o CSF analysis Brain MRI for plaques (MS) Testing for various causes
31
Acute transverse myelitis is diff that GBS how?
GBS doesn't locate to a specific spinal segment! ATM has SEGMENTAL deficits
32
Prognosis of Acute Transverse Myelitis
1/3 recover, 1/3 have lasting weakness/urinary urgency, 1/3 bedbound + incontinent
33
Mydriasis vs miosis
``` Mydriasis = fixed or excessive pupil dilation Miosis = excessive contriction ```
34
What are the 3 "types" of pain
1. Nociceptive (direct or indirect (inflamm)) 2. Neuropathic pain (peripheral or central nerve damage) 3. Centralized pain (no stimulus)
35
Sensory nerve fibre types from largest/fastest to smallest/slowest! (5)
``` A alpha (proprioception) A beta (touch/pressure) A delta (pain/temp) B (visceral afferents, autonomic preganglionics) C (pain, temp, autonomic postganglionics) ```
36
Which nerve fibre type is unmyelinated?
C
37
Spinothalamic tract carries ___ signals and is part of the ___ pathway
Pain & temp | Anterolateral
38
Spinal lesions: A lesion of GREY matter will affect ____ A lesion of WHITE matter affects ____ (what levels/sides)
GREY --> at that spinal level only, ipsilaterally (dematome/myotome) WHITE --> sensory and/or motor BELOW, side depends on location of crossing
39
CN V = ? VII = ? | Crosses over where?
Trigeminal, facial | Pons
40
unique location where a large number of motor and sensory fibers travel to and from the cortex
Internal capsule
41
Headaches: 4 EMERGENT + 4 URGENT red flags
Emergent: thunderclap, fever/meningismus, papilledema w/ focal signs or lowered LOC, acute glaucoma Urgent: temporal arteritis, papilledema w/out above, relevant systemic illness, new headache w/ cognitive change in elderly patient
42
What is a coup-countrecoup injury
Concussion injury where injury occurs on the 2 opposite sides
43
Concussion is a subset of ___
mTBI
44
Severity of TBI is classified using what? What would mild be?
GCS | Mild = 13-15
45
Most common neurodegenerative etiology of dementia? Most common non-neurodegenerative etiology?
``` Alzheimers (>50% of dementia) Cerebrovascular disease (20%) ```
46
Define aphasia and paraphasia
Aphasia = impairment of communication/understanding of speech and/or writing Paraphasia can occur in aphasia, and is producing unintended words/sounds (e.g. saying "hat" instead of "bat" = phonemic paraphasia, saying "week" instead of "month" = semantic paraphasia)
47
What is the DSM-5 criteria for dementia?
Significant decline in cognition (learning/memory, language, executive fn, attention, perceptual-motor and/or social) - INTERFERES w/ daily life - NOT exclusively during delirium or explained by another disorder
48
The mini-mental state examination (MMSE) includes what domains? (6) Cutoff score for dementia?
1) Orientation 2) Registration (immediate memory) 3) Attention/calculation 4) Recall (STM) 5) Language (speaking/understanding) 6) Copying a picture (visuospatial) <25/30 = dementia
49
What test is often added to the MMSE? What other assessment tool has this? What is one component it has that the MMSE doesn't which is often ADDED to the MMSE? Cutoff for dementia?
Clock-drawing test (spatial + abstract thinking) Montreal cognitive assessment (MOCA) - abstraction (finding similarities b/w things) Cutoff = 25/30
50
Important lab tests in all pts presenting with dementia-like symptoms
B12 deficiency, hypothyroidism
51
Lab markers for B12 and folate deficiency
High homocysteine in both | High methylmalonic acid only in B12
52
Imagine in patients presenting with potential dementia
Noncontrast CT or MRI
53
Dementia AKA
Major neurocognitive disorder
54
In vascular dementia what might you see on brain imaging?
Lacunar infarcts (subcortical stroke)
55
What binds the NDMA receptor?
Glutamate
56
3 modalities of treatment for dementia
1) Memory training 2) Cholinesterase inhibitors 3) Mementine
57
___substances should be avoided in dementia as they may lead to further deterioration in cognitive funcitoning
Anticholinergic
58
Examples of cholinesterase inhibitors that can be prescribed for Alzheimers/dementia When are they contraindicated?
Dona Riva dances at the nursing home Gala: Donepezil, Rivastigmine, Galantamine
59
How does memantine work for dementia? ADEs? Indications?
NDMA-receptor antagonist, reduces glutamate-induced Ca-mediated excitotoxicity Mod-advanced Alzheimers, vascular dementia ADEs impact CNS (headache, dizziness, confusion, seizures, etc)
60
What is Wernike encephalopathy caused by? Classic triad
Thiamine deficiency | Confusion + ataxia + opthalmoplegia
61
3 distinct features of Lewy Body Dementia
Visual hallucinations Parkinsonism Attention impairment
62
In pseudodementia, cognitive deficits are associated with ____ and usually improve after ____
Major depression | Antidepressant therapy
63
What subset of dementia is associated with early changes in personality/apathy
Frontodemoral dementia (behavioural variant, most common)
64
What type of dementia often present with asymmetric/focal deficits (e.g. hemiparesis),
Vascular dementia (lacunar infarcts common)
65
Prognosis of Alzheimer disease & causes of death
3-10 year survival post-diagnosis | Infections (difficulty swallowing--> pneumonia), dehydration, malnutrition, falls
66
Pathophys of Alzheimers
1) Amyloid beta plaques (extracellular in grey matter) 2) Neurofibrillary tangles (intracellular hyperphosphorylated tau protein, neurotoxic) 3) Cholinergic neuron degeneration --> ACh deficiency
67
Define adaptive functioning
Ability to handle common life demands, independence compared to others of age/background
68
Global developmental delay
Significant delay in 2+ major developmental domains in children <5yo
69
Diagnostic criteria for intellectual disability
Impaired intellectual + adaptive functioning in at least one of the following domains: 1) Conceptual (incld language, math, memory, judgement) 2) Social 3) Practical (ADLs)
70
Neurodevelopmental disorders (12) are divided into what 6 categories in DSM-5?
1. ASD 2. ADHD 3. Specific learning disorder 4. Intellectual disabilities (ID & GDD) 5. Communication disorders 6. Motor disorders
71
4 key features of ASD
1. Deficits in social-emotional reciprocity 2. Deficits in nonverbal communication 3. Difficulty developing, maintaining, understanding social relationships 4. Restrictive/repetitive behaviours and/or hypo-hyper-reactivity to specific sensory stimuli
72
ADHD involves what 2 categories of behaviours
Inattention | Hyperactivity & impulsivity
73
Dyslexia is a form of ___ disorder
Specific learning disorder
74
ID is classified as what on an IQ test?
At least 2 SD below mean (<70 if mean 100, SD = 15)
75
Stuttering is what disorder?
Childhood-onset fluency disorder (type of communication disorder)
76
What class of drug is used for Tic disorders when non-medical interventions are exhausted?
Antidopaminergic drugs (tetrabenazine, fluphenazine, risperidone)
77
Common ADHD drugs (2)
Methylphenidate (Ritalin) | Amphetamines (e.g. Adderall)
78
What is the mutation in fragile X syndrome?
Trinucleotide repeat (CGG) in UTR of FMR1 on X chromosome (encodes FMRP)
79
Why does trinucleotide repeat expansion prevent transcription of FMRP?
The cytosines are methylated --> promoter not accessible
80
What is "anticipation" in genetics?
Conditions becomes more severe w/ successive generations | Common in trinucleotide repeat expansion disorders, which can expand more and more with each generation
81
Characteristic physical features of Fragile X syndrome
Long narrow face w/ prominent jaw/forehead Large ears, face, testes Pes planus, hypermobility, mitral valve prolapse (CT weakness)
82
Cognitive/motor impacts of fragile X?
ID Delayed speech & motor development Hyperactivity, autistic behaviour
83
Disorders associated w/ fragile X? (3)
ASD ADHD Epilepsy
84
Can females have symptoms of Fragile X syndrome?
Yes but generally more mild, depends on pattern of X chromosome inactivation
85
What are premutation carriers in Fragile X?
have 55-200 repeats --> mild symptoms, full mutation is >200 (up to 44 is normal)
86
Diagnosis of Fragile X is by...
DNA test
87
Premutation carriers of fragile X can present with what 2 related disorders?
1) Fragile X-associated Tremor Ataxia Syndrome (FXTAS) - adult-onset tremor, ataxia, cognitive/memory issues 2) FMR1-related POI
88
By what age should a child stand/walk alone
12 months
89
By what age should a child run
18 months
90
By what age should a child sit w/out support
6 mo
91
By what age should a child say mama/dada indiscriminately?
9 mo
92
Disease with hyperorality memory loss, emotional changes, insatiable appetite, extreme sexual behaviour, indifference
Kluver-Bucy syndrome | lesions affecting bilateral temporal lobes, especially the hippocampus and amygdala
93
The locus coeruleus is part of the ___ in the ____ and produces____
RAS Pons Norepinephrine
94
The Raphe nucleus in the midbrain produces
Serotonin
95
The ventral tegmentum in the midbrain has ___ neurons
Dopaminergic
96
Nucleus accumbens is part of the ___ and is part of the _____. How so?
Basal ganglia Reward circuit Dopamine from VTA --> desire + motivation Serotonin from raphe nuclei --> inhibition
97
Name the blood vessels affected in epidural, subdural, and subarachnoid hematomas
Epidural: meningeal vessels Subdural: bridging veins Subarachnoid: cerebral arteries, usually in circle of willis (usually s/2 aneurysm)
98
Acute hemorrhages appear ___ on noncontrast CT
Hyperdense (bright)
99
Which cranial hemorrhage type appears lens-shaped? Crescent-shaped?
``` Epidural = lense (sutures) Subdural = crescent ```
100
Pia mater extends as ___ from conus medularis --> coccyx | Clinical relevance?
Filum terminale | Where you do LPs (not enough space in the subarachnoid space where the SC runs)
101
Passage of CSF through ventricles
Lateral ventricles --> interventricular foramina (of Monro) --> 3rd ventricle --> cerebral aqueduct --> 4th ventricle --> central canal
102
Choroid plexus is where? What cell type produces CSF?
Lateral, 3rd, and 4th ventricles | Ependymal cells
103
CSF drains from subarachnoid space into ____ via ____
``` Superior sagittal sinus Arachnoid villi (granulations) ```
104
CSF enters subarachnoid space via? (3)
``` Lateral apertures (foramen of Luschka) x 2 Median aperture (foramen of Magendie) ```
105
Broca's area (location & function)
``` L frontal (usually) Speech production ```
106
Wenicke's area (location & function)
``` L temporal (usually) Speech comprehension ```
107
2 main watershed areas in the brain
ACA-MCA | MCA-PCA
108
New definition of TIA
Temporary, focal ischemia causing neurological deficits NO infarction NO permanent LoF
109
Most common sites of atherosclerosis in the brain?
Branching points, esp internal carotid & MCA
110
What is a paradoxical embolism
Clot moves from vein --> artery without getting lodged in pulmonary circulation because of an ASD or other defect
111
What is a lacunar stroke?
A noncortical infarct (of the small penetrating arteries supplying internal capsule, BG, pons, thalamus), and thus lacks cortical signs (no aphasia, hemianopsia, agnosia, apraxis)
112
2 tissue zones in a stroke
``` Ischemic core (tissue dies) Ischemic penumbra (preserved by collateral circ, may survive if quick action taken) ```
113
_____is defined as extracellular accumulation of fluid resulting from disruption of the blood-brain barrier (BBB) and extravasations of serum proteins, while ____ is characterized by cell swelling caused by intracellular accumulation of fluid.
Vasogenic edema | Cytotoxic edema
114
How can stroke lead to herniation?
Inflammation disrupts BBB --> vasogenic edema --> mass effects
115
What is the FAST acronym for stroke
Facial drooping Arm weakness Speech difficulties (aphasia, dysarthria) Time!!
116
How is FLAIR MRI diff than T2-weighted?
Similar in that grey matter is brighter than white, but different because it removes signal from CSF
117
T1 vs T2 MRI
T1 --> FAT is bright, white matter brighter than grey | T2 ---> Fat & WATER is bright (so CSF shows up), white matter darker than grey
118
For both ischemic & hemorrhagic strokes, ___ is the most important nonmodifiable RF & ____ is the most important modifiable RF!
Age | Arterial hypertension
119
2o prevention options after stroke
``` Antiplatelet therapy (aspirin, clopidogrel) Manage RFs (hypertension, AFib, DM, lipids, thrombophilia) ```
120
Initial drug used for status epilepticus
Benzodiazepines: lorazepam, diazepam, midazolam
121
Name 5 common seizure drugs
``` Lamotrigine Valproate Carbamazapine Phenobarbital (children) Topiramate ```
122
Define status epilepticus
>5 min continuous or >2 seizures w/out regaining full consciousness interictally
123
Min temp for febrile seizure
38C
124
What are 3 factors that make febrile seizure "complex" (only need at least 1)
>15 min Focal >1 in 24 hours
125
Normal CSF pressure is less than
200 mm H2O
126
2 bacteria and 3 viruses that most cause meningitis?
``` Neisseria meningitidis (meningococcal), Streptococcus pneumoniae Herpes simplex, HIV, enteroviruses ```
127
What pathogen might cause meningitis at birth
Maternal Group B Strep
128
Classic triad of meningitis?
Headaches Fever Nuchal rigidity (only 50% present with full triad!)
129
Indications for imaging before LP (suspected meningitis)
``` LP FAILS: Focal neuro deficits Altered mental status Immunocompromised, Intracranial hypertension Lesions (in brain) Seizures (doesn't apply to children) ```
130
Non-blanching rash in suspected meningitis raises suspicion of
Meningococcal meningitis (Neisseria meningitidis)
131
Prevention of meningitis?
Vaccines: meningococcal, Hib
132
Which pathogens causing meningitis require postexposure prophylaxis for contacts?
N meningitidis | H influenzae
133
Empiric AB therapy for meningitis
Vancomycin Ceftriazone +ampicillin if Listeria risk (old, newborn, pregnant, immunocomp) +Dexamethasone before/concurrently to reduce inflamm
134
Acyclovir should be used for what types of encephalitis
HSV | VZV
135
Atrophy vs dystrophy
``` Atrophy = reduction in SIZE of muscle fibres Dystrophy = reduction in NUMBER Of muscle fibres ```
136
Typically, proximal weakness = ___, distal = ____
``` Proximal = muscle Distal = nerve ```
137
General pathophys of myasthenia gravis
Autoantibodies against postsynaptic nicotinic ACh receptor (thymus involved)
138
Diagnosis of myasthenia gravis
AChR Ab EMG showing decreased response after repetitive nerve stimulation (do a chest CT to r/o thymoma) Other tests like Simpson test (looking upward 1 min), ice test (improvement)
139
First line treatment for myasthenia gravis
Cholinesterase inhibitors: Pyridostigmine
140
If pt suddenly deteriorates after responding well to treatment, they might have what going on? Features?
``` Cholinergic crisis (affects both NAChRs & MAChRs) SLUDGE: salivation, lacrimation, urination, defecation, GI disturbances, emesis Also fasiculations, tachycardia ```
141
What can trigger myasthenic crisis? Treatment?
Infection, surgery/anesthetics, pregnancy, meds | Treatment: IVIg, plasmapheresis, intubation
142
Basic pathophys of BMD/DMD
Mutation of dystrophin gene on X chromosome Frameshift/nonsense --> DMD Missense/inframe deletion --> BMD Messed up dystrophin --> muscle cell necrosis, replaced w/ CT & fat (pseudohypertophy, weakness)
143
What sign is characteristic of DMD?
Gower maneuver (walking up thighs using hands)
144
Heart complication of DMD?
Dilated cardiomyopathy | Arrhythmias
145
Gait characteristic of DMD?
``` Duchenne limp (waddling) Bilateral Trendelenburg compensated w/ curved upper body ```
146
Toe-walking in DMD caused by?
Contracture/shortening of heel chords | Can be seen earlier as a compensation for hyperlordosis & weakness of hip flexors
147
Diagnosis of DMD
High CK, aldolase | Genetic analysis --> dystrophin gene mutation
148
Treatment for DMD
``` Not great :( Glucocorticoids ACEi/BB to slow dilated cardiomyopathy Supportive therapy, surgeries for contracture/scoliosis Exon-skipping therapies ```
149
Describe a positive Rinne test
Air > bone
150
decibel level where 50% correct signals
Auditory threshold
151
What is otosclerosis?
Overgrowth of stapes footplate --> fixation of bone
152
What is cholesteatoma?
Chronic otitis media | Keratinizing squamous epithelium grows from tympanic membrane into middle ear mucosa --> inflamm, can destroy middle ear
153
What is the Meniere disease? What is the Meniere triad?
Endolymph hydrops (impaired resorption) 1) Peripheral vertigo 2) Fluctuating unilateral SNHL 3) Unilateral tinnitus
154
In what type of hearing loss is it easier to hear in noisy environments?
Conductive
155
What is the most common cause of SNHL?
Presbycusis (age-related progressive damage to hair cells in organ of Corti esp at narrow/stiff basal end)
156
What are the TORCH infections
``` Toxoplasmosis Others (syphilis, varicells, parvovirus B19, listeriosis) Rubella Cytomegalovirus Herpes Simplex Virus ```
157
What is glue ear
Chronic OM w/ effusion | ETD (esp after acute OM) --> neg middle ear pressure --> transudate builds up and can't drain
158
5yo is old enough to undergo what general type of hearing testing
Pure tone audiometry
159
Inheritance of neurofibromatosis
Autosomal dominant inheritance (or spontaneous mutation)
160
What is a neurofibroma?
Benign nerve sheath tumor originated from neural crest cells | Affects myelinated nerves
161
Common types of tumors in Neurofibromatosis Type 2 | Another common symptoms
Vestibular schwannomas (acoustic neuromas) Cerebral/spinal tumors: meningiomas, ependymomas) Neurofibromas not actually as common? Early-onset cataracts
162
What is an acoustic neuroma? AKA?
AKA Vestibular Shwannoma Benign tumor arising from Schwann cells in CNVIII within internal acoustic meatus (usually unilateral, bilateral suggestions neurofibromatosis Type 2)
163
What nerve other than CN VIII moves through the internal acoustic meatus?
CN VII (facial)
164
Is horizontal or vertical nystagmus more alarming?
Vertical (more indicative of potential central cause)
165
Central vs peripheral nausea: which has worse nausea? Which worse imbalance? Which more motion-sensitive?
Central --> worse imbalance Peripheral --> worse nausea Peripheral --> more motion-sensitive
166
3 requirements of brain death
1) Coma w/ identified cause 2) Absence of brainstem reflexes 3) Apnea (**before apnea testing make sure temp high enough, BP high, no chemical disturbances/drugs that could confound)
167
Fundamental pathophys of parkinson's
Degeneration of dopaminergic neurons in the substantia nigra | Lewy body formation
168
Decreases in ACh synthesis by nucleus basalis of Meynert are associated with __ and ___ diseases. Increases associated with ___ disease
Decreases: Alzheimer, Huntington Increases: Parkinson
169
The earliest prodrome sign of parkinson's is often what? Why?
Constipation (early Lewy Body formation at intestinal autonomic neurons)
170
Parkinsonism triad
Bradykinesia + rigidity + resting tremot
171
Describe a parkinsonian gait | What are fenistation, propulsion, and freezing?
Shuffling, small steps Fenistation = increasingly quick steps Freezing = can't start/continue movement Propulsion = forward-leaning
172
In parkinson's do motor symptoms usually begin uniliaterally or bilaterally?
Unilaterally
173
Name 6 drug categories for parkinson's
1) L-DOPA (dopamine precursor that can cross BBB) 2) Carbidopa (reduces peripheral conversion of L-DOPA --> dopamine) 3) COMT inhibitors (reduce central/peripheral dop metabolization) 4) MAO-B inhibs (reduce central dop metab) 5) Dopamine agonists 6) Anticholinergic drugs 7) NMDA antagonists
174
___ is the most affective drug for motor symptoms of parkinsons, but leads to unavoidable ____. Strategy to reduce this?
L-DOPA Unavoidable motor fluctuations (hypokinesia, dyskinesia) Take really frequently throughout the day
175
Don't prescribe L-DOPA if you have ____
Glaucoma (increases intraocular pressure)
176
Example of NMDA antagonist used in parkinson's
Amantadine
177
Carbidopa inhibits what enzyme?
Dopamine decarboxylase
178
Inheritance of huntington disease
Autosomal dominant inheritence | CAG repeats in huntingtin gene, inherited w/ ANTICIPATION
179
How are GABA, ACh, and dopamine affected in Huntington?
Less GABA Less ACh More dopamine
180
Neuronal degeneration/gliosis occurs where in huntington?
Striatum (but then progresses to thalamus, cortex)
181
Motor symptoms in Huntington's might change how as the disease progresses?
Start hyperkinetic/choreatic movements (indirect pathway affected) Both pathways affected --> overall DECREASE of excitatory thalamic transmission to cortex --> HyPOkinetic/akinetic symptoms
182
ALS generally starts with what symptoms (though presentation is variable)
Asymmetric weakness in hands/feet
183
What is sensory gait
Loss of proprioceptive input (uncontrolled diabetes, B12 deficiency) --> stomping gait (esp in dark)
184
What is ataxic gait
Wide-based, clumsy/staggering (like drunk) Cerebellar disease Titubation when standing (swaying around) - occurs with eyes open unlike Romberg
185
Characteristics of hemiplegic gait (after stroke)
Flexor hypertonia of upper limb, extensor hypertonia of lower limb Foot drop Circumduction of food Loss of normal arm swing
186
"Shock-like movements" =
myoclonus
187
Jerking movements that randomly move around diff parts of body =
Chorea
188
How to distinguish postural tremor (i.e. essential tremor) from true resting trmoe
How rapidly it manifests after new posture assumed (immediate for postural, delayed for rested = "resetting" or "reemergence")
189
Abnormal characteristic postures/movements, produced by slow/sustained muscle contraction distorting limbs, trunk, neck, face, mouth
Dystonia
190
Define corticobulbar tract
Motor pathway from motor cortex to motor nuclei of CNs in brainstem
191
Affect of UMN lesion on reflexes
Acute atonia/hyporeflexia (spinal shock) | FOLLOWED by the hyperreflexia/spasticity
192
Upgoing plantar response occurs in a ____motor neuron lesion
Upper
193
How do you differentiate UMN and LMN lesions of facial nerve?
UMN lesion --> paralysis only in lower half of face (upper half has ipsi + contralateral UMN innervation, redundancy) LMN would affect full side of face
194
___ supplies brain regions for lower limbs, ___ supplies hand/face
ACA lower limbs | MCA hand/face
195
The oculomotor nerve (CN III) controls all eye movement muscles except ___ and ____ What else does it control?
Except lateral rectus (CN VI) & superior oblique (CN IV) | Also levitator palpebrae (eyelid) + pupillary constrictors (parasymp division)
196
Why are anterior and posterior spinal cord affected differently by spinal artery infarcts?
Only 1 anterior spinal artery --> bilateral impacts of infarction 2 posterior spinal arteries --> only affected on 1 side
197
EMG/NCV signs for demyelination and axonal loss?
Demyelination --> slowed CV | Axonal loss --> reduced amplitude of CMAP/SNAP
198
What process has to occur before repair when axonal contents damaged?
Wallerian degeneration
199
B12 deficiency can cause what severe neuropathy?
Subacute combined degeneration (loss of dorsal columns/corticospinal tracts --> hyperreflexia, loss of proprioception/vib sensation)
200
painful, asymmetrical, asynchronous sensory and motor peripheral neuropathy involving isolated damage to at least 2 separate nerve areas
Mononeuritis multiplex (multiple mononeuropathies)
201
What is the most common cause of peripheral neuropathy? General presentation?
Diabetes Usually DSPN (distal (length-dependent) symmetric sensorimotor polyneuropathy) Mixed demyelinating & axonal