IM Neurology Flashcards
1
Q
Stroke
definition
A
sudden onset of a neurological deficit fromt eh death of brain tissue. stroke is the third most common cause of death in the united states
2
Q
Stroke
risk factors
A
same as MI:
htn
diabetes
hyperlipidemia
tobacco smoking
3
Q
Stroke is caused by
A
caused by a sudden blockage in the flow of blood to the brain in 85% of cases and by bleeding in 15% of cases. a cerebral vessel is blocked either by a trhmbosis occurring in the vessel or by an embolus to the vessl
4
Q
Stroke
emboli
A
heart: afib, valvular heartd disease, dvt paradoxically getting into the brain trhough a patent foramen ovale
carotid stenosis
5
Q
Stroke Middle Cerebral Artery
presentation
A
(more than 90%)
weakness of sensory loss on the opposite of the lesion
homonymous hemianopsia
aphasia if the stroke occurs on the same side as the speech center. this is left side in 90% of pts
6
Q
homonymous hemianopsia
A
loss of visual field on the opposite side of the stroke. a left sided mca stroke results in loss of the right visual fields. the eye cant see the right side so the eyes deviate to the left.
so the eye looks towards the side of the lesion
7
Q
Stroke Anterior cerebral artery
presentation
A
personality/cognitive defects such as confustion
urinary incontinence
leg more than arm weakness
8
Q
Stroke Posterior cerebral artery
presentation
A
ipsilateral sensory loss of the face, ninth and 10th cranial nerves
contralatleral sensory loss of the libs
limb ataxia
9
Q
Stroke
best initial test
A
CT scan of the head without contrast
10
Q
Stroke
most accurate test
A
MRI
11
Q
Stroke diagnostic testing
A
do a CT first to exlcued hemorrhage as a cause of the stroke prior to initiating treatment
CT scan needs 4-5 days to reach 95% sensitivity
MRI needs 24-48 hours to reach 95% sensitivity
12
Q
best initial therapy for nonhemorrhagic stroke
A
less than hourse since onset of stroke: thrombolytics (some places go up to 4.5 hours)
more than 3 hours since onset of stroke: aspirin
hemorrhagic stroke: nothing
13
Q
how do you treat a pt with a nonhermorrhagic storke who is already on apsirin
A
add dipyerimadole
or
switch to clopidogrel
14
Q
when can you drain a hemorrhagic stoke
A
posterior fossa
15
Q
treatment for prevention of a stroke
A
either aspirin or clopidogre, do not combine them you will only make pt bleed. use dipyridamole with aspirin as an equivalent of clopidogrel
16
Q
thrombolytics
A
can sometimes be used up to 4.5 hours (must get conformed consent)
standard of care is under 3 hours since onset and are fda approved
17
Q
Statins with stroke
A
every pt with a storke hsould be started on a statin medication regarldess of ldl. although target based therapy for lipid maagement is unclear at this time, we want to bring the ldl to 70 or at least under 100
18
Q
stroke=
A
statin (put pt on one)
19
Q
acute blood appears what color on a CT
A
white
20
Q
Evaluation of causes of stroke and their treatment
echocardiogram
A
surgical replacement or repair of certain damaged valves
Thrombi: heparin followed by warfarin to an INR of 2 to 3. rivaroxaban and dabigatran are aletnative medications
pfo
21
Q
Evaluation of causes of stroke and their treatment
EKG
A
a fib or flutter is treated with warfarin to an INR of 2 to 3 as long as the arrhythmia persists
22
Q
Evaluation of causes of stroke and their treatment
holter monitor
A
if the intial ekg is normal a holter monitor should be perormed to detect atrial arrhytthmias with greater sensitivity
23
Q
Evaluation of causes of stroke and their treatment
carotid duplex us
A
carotid stenosis is a frequent cause of emboli to the brain. if a pt has symptomatic cerebrovascular disease and more than 70% stenosis is detected, surgical correction of the narrowing should be performed. endartectomy is superior to carotid angioplasty.
24
Q
Evaluation of causes of stroke and their treatment
endarterectomy
A
has not value for milder stenosis (under 50%), it is unclear is endarterectomy will benefit moderate stenosis (50-70%), if the stenosis is 100% however no inteverntion is needed there is no point in opening a passaged that is 100% occluded
25
stroke pts should be place don telemetry to
dtect afib/flutter
26
carotid angioplasty and stenting is what with storke
not proven and is always the wrong answer
27
control of risk factors for stroke
diabetes to a hemoglobin A1C below 7%
hypertension
reduce LDL to at least below 100 if carotid stenosis is the cause of the stroke
tobacco smoking should be stopped
28
carotid stenosis is considered and equivalent of
coronary artery disease, so control the LDL to less than 100mg/dl
29
Headache, What is the most likely dx
migraine
visual disturbance (flasehs, sparks, stars, luminous hallucinations), photophobia, aura, relationshiop to menses, association with food (chocolate, red wine, cheese), precipitated by emotions, associated with nausea and vomiting
30
Headache, What is the most likely dx
cluster headache
frequent, short duration, high intesnity, headaches, with men affected 10 times more than women
31
Headache, What is the most likely dx
giant cell (temporal) arteritis
visual distubance, systemic symptoms such as muscle pain, fatigue, and weakness. jaw claudication
32
Headache, What is the most likely dx
pseudotumor cerebri
associated with obestiy, venous sinus thrombosis, oral contraceptives, and vitamin A toxicity. Mimics a brain tumor with nausea, vomiting, and visual distrubance
33
Physical Exam
tension headache
no physical finding
34
Physical Exam
migraine
no physical findings usually, but reare cases have aphasia, numbness, dysarthria, or weakness
35
Physical Exam
cluster headach
red rearing eye with rhinorrhea, horner syndrome occasionally
36
Physical Exam
giant cell (temporal) arteritis
visual loss, tenderness of the temporal area
37
Physical Exam
pseudotumor cerebri
papilledema with diplopia from sixth crnial nerve (abducens) palsy
38
evaluate for glaucoma with
a headach and a red eye
39
Diagnostic tests
tension, migraine, and cluster headaches
no specific diagnostic tests, do a head CT or MRI to exclude intracranial mass lesions if the diagnosis is unclear or the syndrome has recently started. there is no reason to perform imaging if there is a clear hx of headach of a particular type
40
Diagnostic tests
pseudotumor cerebri
the diagnosis cnnot be made wihtout a CT or MRI to exclude an intracranial mass lesion and a lumbar puncture showing increased pressure, CSF fluid is normal just higher pressure
41
Diagnostic tests
giant cell arteritis
markedly elevated ESR and the most accureate test is a biopsy
42
Treatment
tension headache
nsaids and other analgesics
43
Treatment
migraine
triptans or ergotamine as abortive therapy
44
Treatment
cluster headach
triptans, ergotamine, or 100% oxygen as abortive therapy
45
Treatment
giant cell temporal arteritis
prednisone
46
Treatment
pseudotumor cerebri
weight loss
acetazolamide to decrease production of cerebrospinal fluid
steroids help
repeated lumbar puncture rapidly lowers inracranial pressure
place a ventriculoperitoneal shunt or fenestrate (cut into) the optic nerve if medical therapy does not control it
47
it is critical to start steroids without waitng for biopsy in
giant cell arteritis
48
abortive therapy for migraine and cluster headache
both of these can be rapidly interrupted by either ergotamine or one of the triptans. the main idfference is that 100% oxygen, prednisone, and lithium are effective at interrupting cluster headaches but not migraines.
49
Provide cluster prophylaxis with
verapamil
50
prophylactic therapy for migraine
pts experiencing 3 or more migraine headaches per month should be starte don treatment to prevent them
propranolol is the best
51
other migraine preventative meds
ccb
tca
ssris, topiramate
botulinum toxin injection
52
Tension headach summary
b/l bandlike pressure
lasts 4-6 hours
normal pe
nsaids/tylenol
53
migraine summary
w/ or w/o aura
related to food emotions menses
rare: pahasia, numness, dysarthria
avoid triggers
nsaids
5ht1 agonists (triptans)
if 3 attacks/month
propranolol
sodium valproate
54
cluster headach summary
episodic pain
unilateral periorbital intense pain
lacrimation
eye reddening
nasal stuffiness
lid ptosis
sumatriptan
octreotide
oxygen
verapamil
prednisone
sodium valproate
55
trigeminal neuralgia unimproved by meds needs
gamma knife surgery
56
peripheral neuropathy treatment
there is no clear routinely effective treatment with postherpetic neuralgia
57
Trigeminal Neuralgia
definition
idiopathic disorder of the fifth cranial nerve resulting in severe, overwhelming pain in the face.
58
Trigeminal Neuralgia
presentation
attacks can be precipitated by shewing touching the face or pronouncing certain words in which the tongue strikes the back of the front teeth.
pts describe the pain as feeling as if a knife is being stuck into the face
59
trigem neuralgia
testing
no diagnostic test
60
trigem neuralgi
treatment
oxcarbazepine or carbamazepine
baclofen and lamotrigin have also been effective
gamma knife surgery or surgical decompression if meds dont work
61
Postherpetic Neuralgia
definition
hepres zoster reactivation, or shingles, is associated with a pain syndrome after resolution of the vesicular lesions in about 15% of cases
62
Postherpetic Neuralgia
treatment
antiherpetics like acyclovir famcyclovir, of valganciclovir reduce the incidence
steroids dont work!
63
Postherpetic Neuralgia
pain management
tca
gabapentin
pregabalin
carbamazepine
or phenytoin until an effective one is found
topical capsaicin is helpful
most antiepileptic meds have some beneficial effect in neuro pain but non work in more than 50-70% of pts
64
Prevention of herpes zoster (shingles)
zoster vaccine is indicated in all persons above the age of 60 to prevent shingles. this vaccin is similar to the varicella vaccine routinely administered to children to prevent chicken pox, except the dose is higher
65
gerneralized tonic-clonic seizures are caused by
```
hypo or hypernatremia
hypoxia
hypoglycemia
any cns infection (encephalitis, meningitis, abscess)
any cns anatomic abnormality (trauma, stroke, tumor)
hypocalcemia
uremia (elevated creatinine)
hepatic failure
alcohol, barbiturate, and benzo w/drawal
cocaine toxicity
hypmagnesemia (rare)
```
66
Seizure diagnostic tests
an electoencephaloram would not be the right answer unless all of these tests wer done and were normal including a ct or mri ofth ehead
there is no point in doing an eeg to dientify the cause of a seizure if there is a clear metabolic, toxic, or anatomic defect causing the seizure
67
seizures of unclear etiology are called
epilepsy
68
confusion is to coma and seizure as angina
is to mi
69
treatment of status epilepticus
best initial therapy
benzos for a persistent seizure (lorazepam or diazepam iv)
if the seizure persists then give phenytoin or fosphenytoin
70
phenytoin and fosphenytoin
have the same efficacy but fosphenytoin has fewer side effects
like lidocaine phenytoin is a class 1b antiarrhythmic, and when given iv it is associated with hypotension and AV block
fosphenytoin does not do this and so it can b given rapidly
71
if benzos and phenytoin and fosphenytoin do not stop the seizure
administer phenobarbital
72
ultimate therapy for unresolving siezure is to use
a neuromuscular blocking agent like succinylcholine vecuronium or pancuronium to allow you to intubate the pt and then give genreal ansethesia such as midazolam or propofol. the pt must be placed on a ventilator before propafol
73
partial seizure
like the name implies this is a seizure that is focal to one part of the body. for instance, a ptmay have a seizure that is limited just to an arm or leg. partial seizures can either be simple (intact consciousness) or complex (loss or alteration of consciousness)
74
tonic-clonic seizure
this is a generalized seizure with varying phases of muscular rigidity (tonic) followed by jerking of th emuscles of the body for several minutes (clonic)
75
absence (petit-mal) seizures
consciousness is impaired only briefly. the pt often remains upright and gives a nomral appearance or seems to be staring into space, occur more often in children
76
treatment of status epilepticus treatment
1. benzos
2. fosphenytoin
3. phenobarbital
4. general anesthesia
77
indications for treatment of seizuresbe
status eplepticus or with focal neurological signs
abnormal eeg or lesion on CT
family hx of seizures
78
do you start seizure meds after first seizure
no
79
treatment of epilepsy
best treatment is not clear
levetiracetam, phenytoin, valproic acid, and carbamazepine are all eqully effective
alternate treatment with gabapentin, topiramate, lamotrigine, oxcarbazepine or levetericatam
80
what antiseizure med has the least amount of side effects
levetericatem
81
ethosuximid is the best therapy for
absence seizures
82
if seizures are not treated by changing meds or adding a second med
surgery
83
alcohol withdrawal seizure treatment
benzos
84
discontinuing seizure med
pt must be seizure free for 2 years
sleep deprivation eeg is best way to tell if there is possibility of recurrence (can elicit abnomral activity but not very specific)
85
Subarachnoid Hemorrhage
Definition/etiology
caused by rupture of an aneurysm tht is usually located in the anterior portion of the circle of willis. aneurysms ware present in 2% of routine autopsis. the vast majority never rupture
86
Subarachnoid Hemorrhage
is most frequent with
polycystic kidney disease
tobacco smoking
htn
hyperlipidemia
high alcohol consumption
87
Subarachnoid Hemorrhage
what is the most likely diagnosis
look for the sudden onset of an extremely severe headachwith menigneal irritation (stiff neck, photophobia) and fever. fever is secondary to blood irritating the meninges. loss of consciousness occurs in 50% from the sudden increase in intracranial pressure. focal neurological complications aoccur in as many as 30%
88
how sah differs from meningitis
very sudden onset
loss of consciousness
89
Subarachnoid Hemorrhage
best initial test
ct w/out contrast (95%) sensitive
90
Subarachnoid Hemorrhage
most accurate test
lumbar puncture showing blood
91
Subarachnoid Hemorrhage
LP
may show xanthochromia, which is a yellow discoloration of CSF fromt he breakdown of red blood cells in the CSF. LP is necesary only for the 5% that have a false negative CT scan. CSF may have an increaed WBC which may mimic meningitis but the ratio of RBC to WBC will be normal
92
normal WBC to RBC
1 WBC to 500-1000 RBC
93
contrast on head CT
improves detection of mass lesions like cancer or abscess
dont use contrast when looking for blood
94
Ekg with intracranial bleeding
may show large or inverted T waves suggestive of myocardial ischemia (cerebral T waves). this is thought o be from excessive sympathetic activity
95
Angiography with Intracranial bleeding
determines the site of the aneurysm in order to guide repair of the lesion
96
Subarachnoid Hemorrhage
treatment
no way to reverse the hemorrhage
97
Subarachnoid Hemorrhage
nimodipine
(calcium chanel blocker) prevents subsequent ischemic stroke
98
Subarachnoid Hemorrhage
embolization
(coiling) uses a catheter to gloc up the site of lbeeding to prevent a repeated hemorrhage. an interventional neuroradiologist places platinum wire into the site of hemorrhage. embolization is superior to urgical clipping in terms of survival and complications
99
Subarachnoid Hemorrhage
ventriculoperitoneal shunt
SAH is associated with hydrocephalus. place a shunt only if hydrocephalus develops
100
Subarachnoid Hemorrhage
seizure prophylaxis
phenytoin is generally given to prevent seizures. if the question asks "which of the following is indicated?" antiepileptic therapy is the answer, although controversial
101
bacterial meningitis treatment
ceftriaxone and vancomycin
102
Amterio spinal artery infarction
loss of all function except for the posterior column (poistion and vibratory sensation intact)
flaccid paralysis below the level of the infarction
loss of deep tendon relfexes (DTRs) at the level of the infarction
evolves into spastic paraplegia several weeks later
loss of pain and temp
extensor plantar response
103
Subacute combined degeneration of the cord
from b12 deficiency or neurosyphilis
position and vibratory sensation are lost
104
Spinal trauma
acute onset of limb weakness and/or sensory disturbance below the level of th einjury with the severity in proportion to the degree of injury.
sphincter function impaired
loss of DTRs at the level of the injury followed by hyperreflexia below the level of the trauma.
105
spinal trauma initial treatment
glucocorticoids
106
Brown-Sequard Syndrome
after unilateral hemisection of the spinal cord you lose pain and temperature on the contralateral side from teh injury and lose motor function, position and vibratory sense on the ipsilateral side of the injury
107
Syringomyelia
definition/etiology
fluid filled dilated cenral canal in the spinal cord. this wiedening buble or cavitation first damages neural fibers passing near the center of the spine.
cause by tumor or sever trauma tot he spine or is congenital
108
Syringomyelia
what is the most likely diagnosis
look for the loss of pain and temp bilaterally across teh upper back and both arms.
look for the phrase capelike distribution of deficits
also cause lack of reflexes and muscle atrophy in the same b/l distribution
109
Syringomyelia
diagnostic tests
mri is the most accurate test
110
Syringomyelia
treatment
surgical removal of tumor if present and drainage of fluid from the cavity
111
Brain Abscess
definition
collection of infected material within the parenchyma of the brain tissue acting as a space-occupying lesion
112
Brain Abscess
etiology
can spread from contiguous infection in the sinuses, mastoid air cells, or otitis media.
anything that leads to bacteremia can allow infected material to lodge in the brain
pneumonia and endocarditis cause bacteremia which causes brain abscess
113
Brain Abscess
presentation
look for headache, nausea, vomiting, fever, seizures, and focal neurological findings
no way to distinguish from cancer w/o biopsy
cancer can give a fever
114
Brain Abscess
best initial test
head ct or mri will show a ring or contrast enhancing lesion that will ikely have surrounding edema and mass effect
115
Brain Abscess
most accurate test
brain biopsy
116
in the brain, cancer and infection are indistinguishable based on
an imaging study alone
117
Brain Abscess
CSF
unlikely to be helpful and lp is ci bc of the possibility of herniation
118
Brain Abscess
Microbiology
Biopsy is essential to distinguiash abscess from cancer as well as to determine the organism and its sensitivity
can be staph stre gram neg and anaerobs
frequently mixed bugs so a precise dx is important given that duration of treatment is long (6-8 weeks iv, 2-3 months orally)
119
Brain Abscess
Treatment
empiric therapy with penicllin plus metro plus ceftraixone (or cefepime) is acceptable while waitin gfor the results of culture.
vanco can be sued instead of penicillin particularly if there has been recent neurosurgery and the risk of staph is greater
120
Tuberous Sclerosis
Neurological abnormalities
seizures
progressive psychomotor retardation
slowly progressive mental deterioration
121
Tuberous Sclerosis
skin
adenoma sebaceum (reddend facial nodules)
shagreen patches (leathery plaques on the trunk)
ash leaf (hypopigmented patches)
122
Tuberous Sclerosis
other lesions
retinal lesions
cardiac rhabdomyomas
123
Tuberous Sclerosis
treatment
no specific treatment, control seizures
124
Neurofibromatosis
von recklinhausen disease
neurofibromas
eight cranial nerve tumors
cutaneous hyperpigmented lesions (cafe au lait spot)
meningioma and gliomasTuberous Sclerosis
125
Neurofibromatosis
treatment
there is no specific treatment
eight crnial nerve lesions may need surgical decompresion to help preserve hearing
126
Sturge-Weber Syndrome
port wine stain of the face
seizures
homonymous hemianopsia
hemiparesis
mental subnormality
skull xray shows calcification of angiomas
tbere is no treatment beyond controlling seizures
127
Neurofibromas
soft
flesh colored
attached to peripheral nerves
128
Essential Tremor
occurs at both rest and with intention (reaching for things) the remor is gretes in the hands, but can affect the head as well, exam is otherwise normal. the tremor may affect some manual skills such as hadnwriting or the use of a computer
129
Essential Tremor
what makes it worse
caffeine
130
Essential Tremor
best therapy
propranolol
gets better with alcohol
131
Parkinsonism
definition
the loss of cells int he substantia nigra resulting in a decrease in dopamine which leads to a significant movement disorder presenting with tremor gait distrubance and rigidity
132
Parkinsonism
etiology
head traua
anitpsychoitic (thoazine)
encephalitis
reserpine
metoclopromide
idiopathic
133
Parkinsonism
most common cause
idiopathic
134
Parkinsonism
diagnosis
no test, clearly on clinical presentation
135
Parkinsonism
presentation
50-60
tremor, rigidity, bradykinesia, shuffling gate and a lot of falls
cogwheel rigidity is the slowing of movement on passive lfexion or extension
limited facial expression (hypomimia)
small writing (micrographia)
postural instability is orthostatic hypotension
syncope
136
most frquent Parkinsonism questions
treatment
137
Parkinsonism Treatment
antihcholinergics
mild disease
benztropine and trihexyphenidyl
relieve tremor and rigidity
unclear why it works
ae are dry mouth, worse bph and consitpation
138
Parkinsonism Treatment
amantadine
mild disease
increase release of dopamine from substantia nigra
give to pts older than 60 who dont tolerate anticholinergis
139
Parkinsonism Treatment
dopamine agonists
severe disease
pramipexole and ropinirole
best initial treatment for severe disease
140
Parkinsonism Treatment
levodop/carbidopa
severe disease
most effective med
associated with on/off phenomena which results in episodes of insufficient dopamine (off) characterized by bradycardia
use if pt has this on/off thing
141
Parkinsonism Treatment
COMT inhibitors
severe disease
tolcapone and entacapone
extend the duration of levodopa/carbidopa by blocking hte metabolism of dopamine
use only with carbidopa/levodopa
142
Parkinsonism Treatment
MAO inhibitors
severe disease
rasagiline selegiline
on its own or adjunct to lveodopa/carbidopa
block metabolism of dopamine
143
Parkinsonism Treatment
deep brain stimulation
severe disease
electrical stimulation is highly effective for tremors and rigidity in some pts
144
avoid what with mao inhibitors
tyramine containing foods (chees e)
they precipitate hypertension
145
what slows the prpgression pf parkinsonism
mao inhibitors
146
lewy body dementia
parkinsonism with dementia
147
shy-drgaer syndrome
parkinsonism predominantly with orthostasis
148
pt with sever parkinsonism presenting with psychosis and confusion
cant stop parkinson drugs bc pt will get bradykinesia, use antipsychotics with fewest effects (clozapine)
149
spasticity
painful contracted muscles form damage to the central nervous system is called spasticity
associated with ms
baclofen, dantrolen and the cnetral acting alpha agonsit tizanadine may all owrk
150
Restless leg syndrome
pts rpeort an uncomfortable sensation in the legs that is creepy and crawly at night
worsened by caffeine
releived by moving the legs
can kick while sleeping
151
Restless leg syndrome
treatment
treat with dopamine agonists like pramipexole
152
Huntington Disease
definition/etiology
hereditary disease characterized by CAG trinucleotide repeat sequences on chromosome 4
153
Huntington Disease
what is the most likely diagnosis
choreaform movement disorder (dyskinesia)
dementia
behavior chagnes (irritability, moodiness, antisocial behavior)
onset between the ages of 30 and 50 with a familyx
154
Huntington Disease
movement disorder
starts with fidgetiness or restlessness progressing to dystonic posturing rigidity and akinesia
155
Huntington Disease
diagnostic test
specific genetic test, 99% sensitive, cag tri repeat
sx triad of movement memory and mood
156
Huntington Disease
treatment
no treatment can reverse it
dyskinsease is treted with tetrabenazine
pyschosis is treated with haloperidol quetiapine or a trial of different antipsychotics
157
Huntington Disease
ct or mri of head show
caudate nucleus involvement
158
Tourette Disorder
idopathic disorder of:
vocal tics, grunts, and coprolalia
motor tics (sniffing, blinking, frowning
obsessive-compulsive behavior
159
Tourette Disorder
dx
no specific diagnostic tests
160
Tourette Disorder
treatment
fluphenazine, clonazepam, pimozide, or other neuroleptic mediaction
methylphenidate and ADHD treatment are intrinsic to Tourette Disorder management
161
Multiple Sclerosis
definition
idiopathic disorder exclusively of CNS white matter
mre common in white women who live in colder climates
162
Multiple Sclerosis
What is the most likely diagnosis
multiple neurological deficits
most common presentation is focal sensory sx with gait and balance problems
blurry bision or visual distubrance form optic neuritis is no longer as common as the first presentation
163
Multiple Sclerosis
most common abnormalities
optic neuritis then motor and sensory
164
Multiple Sclerosis
least common abnormalities
cognitive defects and dementia
sexual function remains rleatively intact
165
Multiple Sclerosis
other findings
fatigue
spasticity and hyperreflexia
cerebellar deficits
166
internuclear ophthalmoplegia (INO)
is the inability to adduct one eye with nystagmus in the other eye
characteristic of ms
167
Multiple Sclerosis
diagnostic tests
mri is best initial and most accurate
lp show csf with amild elevatin in protein and fewer than 50-100 WBCs . oligoclonal bands are found in about 85% of pts but ar enot specific to ms
168
oligoclonal bands are the answer
in the 3-5% of pts with equivocal or nondiagnostic MRI
169
Multiple Sclerosis
treatment, best initial for acute disease
high dose steroids
170
Multiple Sclerosis
drugs that prevent relapse and progression
```
glatiramer (copolymer 1)
beta-interferon
fingolimod (oral)
natalizumab
mitoxantrone
azathiprine
cyclophospamid
```
171
in ms steroids do what
shorten the duration of exacerbation
172
natalizumab
inhibitor of alpha 4 intergrin
development of progresive mutlifocal leukoencephalopathy (PML) (multiopel white atter hypodense lesions)
173
Multiple Sclerosis
best choice for prevetnion of relapse
glatiramer and beta interferon
174
Motor Neuron Disease and Amyotrophic Lateral Sclerosis
Definition/etiology
the cause of als is unknow it is a loss exclusively of upper and lower motor neurons
175
Motor Neuron Disease and Amyotrophic Lateral Sclerosis
what is the most likely diagnosis
weakness of unclear etiology starting int he 20s to 40s with a unique combination of upper and lower motor neuron loss
176
Motor Neuron Disease and Amyotrophic Lateral Sclerosis
most serious presentation
difficulty in chewing and swallowing and a decrease in gag reflex, leads to pooling of saliva in the pharynx and frequent episodes of aspiration
a weak cough and loss of swallowing are also bad
177
Motor Neuron Disease and Amyotrophic Lateral Sclerosis
sensory and sphincters
are spared
178
Motor Neuron Disease and Amyotrophic Lateral Sclerosis
umn presentation
weakness
spasticity
hyperreflexia
extensor plantar responses
179
Motor Neuron Disease and Amyotrophic Lateral Sclerosis
lmn presentation
weakness
wasting
fasciculation
180
in als the most common cause of death is
respiratory failure
181
Motor Neuron Disease and Amyotrophic Lateral Sclerosis
diagnostic tests
elecromyography reveals loss of neural innervation in multiple muscle groups
cpk levels are elevated
182
Motor Neuron Disease and Amyotrophic Lateral Sclerosis
treatment
riluzole reduces glutamate bluidup in neurons and may prevent progression of disease
baclofen treats spasticity
cpap and bipap help with resp diff secondary to muscle weakness
tracheostomy and maintenance on a ventilator is necessary with advanced disease
183
Charcot-Marie tooth disease
definition
genetic disorder with loss of both mtor and sensory innervation
184
Charco-Marie tooth disease
presentation
distal weakness and sensory loss
wasting in the legs
decreased deep tendon relfexes
tremor
pes cavus (foot deformity with a high arch)
legs look like inverted champagne bottles
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Charco-Marie tooth disease
most accurate test
electromyography
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Charco-Marie tooth disease
treatment
none
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Peripheral Neuropathy
most common cause
diabeetus
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Peripheral Neuropathy
other causes
uremia
alcoholism
paraproteinemias like moncolonal gammopathy of unkown significance
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Peripheral Neuropathy
best initial therapy
pregabalin or gabapentin
tca and most seizure meds are effective in some people
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ulnar Neuropathy
precipitating event
manifestations
biker, pressure on palms of hands, trauma to the medial side of elbow
wasting of hypthenar eminence, pain in fourth and fifth fingers
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radial Neuropathy
precipitating event
manifestations
pressur eof inner, upper arm. falling asleep with arm over back of chair, using crutches and pressure in the axilla
wrist drop
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lateral cutanesou nerv of thigh Neuropathy
precipitating event
manifestations
obesity, pregnancy, sitting with crossed legs
pain/numbness of outer aspect of one thigh
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tarsal tunner (tibial nerve) Neuropathy
precipitating event
manifestations
worsens with wlaking
pain/numbness in ankle and sole of foot
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peroneal Neuropathy
precipitating event
manifestations
hgih boots, pressur eon back of knee
weak foot with decreased dorsiflexion and eversion
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MEdian Neuropathy
precipitating event
manifestations
typists, carpenters, working with hands
thenar wasting, pain/numbness in first 3 fingers
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Facial (7th cranial) nerver palsy or bell palsy
etiology
most are idiopathic can be from lyme sarcoidosis herpes or tumors
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Facial (7th cranial) nerver palsy or bell palsy
presentation
paralysis of the entire side of the face is classic
stroke will paralyze only the lower half of the face bc the upper havlf of the race receives innervation from both cerebral hemispheres
difficulty closing the eye
if pt can wrinkle forhead worry about stroke
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Facial (7th cranial) nerver palsy or bell palsy
2 additional features
hyperacusis: sounds are extra loud bc the 7th cranial nerve nromally supplies the stapedius muscle which acts as a shock absorber on the ossicels of the middle ear
tast disturbances: the sevent cranial nerve supplies the senastion of taste the anterior 2/3rds of the tongue
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Facial (7th cranial) nerver palsy or bell palsy
what is the most likely dx
face feels stiff
pulled to one side
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Facial (7th cranial) nerver palsy or bell palsy
diagnostic tests
no test is usually done
most accurate test is electromyography and nerve conduction studies
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Facial (7th cranial) nerver palsy or bell palsy
treatment
most pts have full recovers w/o treatment
best initianl therapy is prednisone
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Facial (7th cranial) nerver palsy or bell palsy
most common complication
corneal ulceration bc it is hard to close the eye
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Acute Inflammatory Polyneuropathy (guillan-barre syndrome)
definition
autoimmune damage ofmultiople peripheral nerves
no cns involvment
a circulating antibody attacks the myelin sheaths of the peripheral nerves removing theri insulation
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Acute Inflammatory Polyneuropathy (guillan-barre syndrome)
associated with
campylobacter jejuni infection
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Acute Inflammatory Polyneuropathy (guillan-barre syndrome)
what is the most likely dx
look for weakness in the legs that ascends from the feet and moves toward the chest, associated with a loss of DTRs. a few pt have a mild sensory disturbance. the main problem is that hwen GBS hits the diaphragm, it is assocaited with respiratroy muscle weakness. Autonomic dysfunction with hypotension, hypertension, or tachycardia can occur
206
ascending weakness + loss of reflexes =
GBS
207
Acute Inflammatory Polyneuropathy (guillan-barre syndrome)
diagnostic tests
the most specific diagnostic test is nerve conduction studies/electromyography
these show a decrease in the propagation of electrical impulses along the nerves, but it takes 1-2 weeks to become abnormal
csf shows increased protein with a normal cell count
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Acute Inflammatory Polyneuropathy (guillan-barre syndrome)
Tests of mrespiratory muscle involvement
when the diaphragm is involved there is a decreae in forced vital capacity and peak inspiratyor pressure. inspiration is the active part of breathing and the pt loses the strength to nhale. pfts tell who might die from BGS
209
Acute Inflammatory Polyneuropathy (guillan-barre syndrome)
treatment
intravenous immunoglobulin or pasmapheresis are equal in efficacy, dont combine them
210
what does not help GBS
prednisone
211
most urgent thing to do in GBS
pfts, peak inspiratoyr pressure or decrease in fvc are signs of resp doom
212
Myasthenia Gravis
definition
disorder of muscular weakness from the production of antibodies against acetylcholine receptors at the neuromuscular junction
213
Myasthenia Gravis
presentation
double vision
difficulty chewing
dysphonia or weakness of limbs worse at the end of the day
214
Myasthenia Gravis
pe
psosis
weakness with sustained activity
normal pupillary response
215
severe Myasthenia Gravis affects
respiratory muscles
216
Myasthenia Gravis
best initial test
acetylcholine receptor antibodies (80-90% sensitive).
this is a better first answer than edrophonium testing
for pts w/o those antibodies get anti-musk antibodies (muscle specific kinase)
217
Myasthenia Gravis
edrophonium
short acting inhibitor of acetylcholinesterase. the bump up in acetylcholin levels is associated with aclear improvement in motor function that lasts for a few minutes
218
Myasthenia Gravis
most accurate test
electromyogrpahy shows decreases strength with repetitive stimulation
219
Myasthenia Gravis
what imaging should be done
chest cray ct or mri are done to look for thymom or thymic hyperplasia
ct with contrast is best
220
Myasthenia Gravis
best initial treatment
neostigmine or pyridostigmine these are longer acting versions of edrophonium
221
Myasthenia Gravis
most appropriate next step after neo and pyridostigmine
thymectomy if pt isunder age 60
if over age 60 use prednisone
azathiprine tacrolimus cyclophosphamide or mycophenolate are used in order to get the pt off of steroids before serious adverse effects occur
want to suppress t cell function in order to control antibodies made against acetylcholine receptors
222
deat from gbs
is rare but is from dysautonomia and respiratory failure
223
Myasthenia Gravis
management of acute crisis
presents with severe overwhelming disease with profound weakness or respiratory involvmeent it is treated with ivig or plasmapheresis
224
Dementia
alzheimer's disease is by far the most commonc ause of dementia. since there is no specific test for alzheimers disease your challenge is to know how far to go in testing to diagnose it and what the other dementia syndromes are
225
Dementia
Diagnostic testing
mri of brain
vdrl or rpr to exlude syphilis
b12 with possible methylmalonic acid level
thyroid function test
226
Dementia
treatment
donepezil, rivastigmine, and galantamine are equal in efficacly all increase acetylcholine levels
memantine
227
Dementia
lewy body dementia
assocaited wiht parkinsons disease
treat both parkinsons disease and alzheimer's disease with levodopa/carbidopa
228
frontotemporal dementia
emotional and socail appropriateness are lost first
memory deteriorates later
no special therpy beyond ach medications
229
Creutzfeldt-jakob disease
rapidly progressive dementia
myoclonic jerks
normal head mri or ct
csf with 14-3-3 protein
biopsy is most accurate
no specific therapy
