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Study 2019

Terms in this set (111)

How do you counsel a patient who has tuberous sclerosis?
Their child has a 50% chance of inheriting the disease
What is the MOA of cocaine?
inhibits reuptake of NE and DA
How does 3,4 diaminopyridine act to help those with LEMs?
Blocks potassium at nerve terminals and causes increase in acetylcholine releaseS
Patient presents with generalized dystonia and seizures, their father is in their 40s and have choreoathetosis and cognitive impairment, what are they likely to have?
HD?
-other options: SCA, Wilson
What is the MOA of oxybutnin?
cholinergic
How do you treat a patient with faciobrachial dystonia?
CBZ? Looked like hemifacial spasm
What trinucleitide repeat disorder is inherited in an autosomal recessive fashion?
Friedrich's ataxia, GAA repeats
Balint's syndrome is caused by what?
occipital-parietal lesion
-can be stroke, can be Alzheimer's
Hypokalemic periodic paralysis can be treated with what?
Mexilitine
HypoPP can be associated with what?
thyroid abnormalities, check thyroid studies
Patient in their later years present with painful extremities relieved by cold water, what do they have?
primary erythromyalgia
What EMG features are that of radiation induced neuropathy?
myokymia
What is the main inhibitory neuron of the spinal cord?
glycine
Which thalamic nuclei is involved in circadian rhythm?
Suprachiasmic nuclues
Brainstem evoked potential wave V represents what?
inferior colliculus
What thalamic nuclei is involved in the limbic system?
anterior thalamic
Where does the entorhinal cortex project to?
hippocampal formation
-receives its input from the cingulate gyrus
How do you treat seasonal affective disorder in a patient who does not want medications?
light therapy
What is the best triptan to use in those with menstrual headaches?
frovatriptan?
RBD is due to accumulation in what protein?
alpha synuclein
FTD is due to deficit in what?
-serotonin?
-tau based disorder
In MSA, you can see what on imaging?
cerebellar atrophy
Patient autopsy shows loss of cells in the interomedial cell column, what are they most likely to have?
MSA
What is the triad of those with LBD?
fluctuating cognition, visual hallucinations, spontaneous motor features of parkinsonism
Where are lewy bodies seen in LBD
-cerebral cortices, subcortical regions and brainstem nuclei NOT SNc
-LBD due to cholinergic loss in the nucleus basalis of Meynert
What medications can be used to treat hyperprolactinemia?
cabergoline and bromocriptine
What medications can cause hyperprolactinemia?
metoclopramide, risperdone
Which antipsychotic is most likely to cause a metabolic syndrome?
olanzapine
Kid hits her head, does okay then has signs of uncal herniation. What is she most likely to have?
epidural hematoma
Patient with decrease gag, uvula deviation, shoulder drop.
jugular foramen as this has CN IX, X, XI go through
What should you do for a patient with clinical dementia rating of 0.5?
refer for driving eval
for score of 1, they should not be driving
APP is located on what gene?
chromosome 21
What is the strongest non-genetic RF for developmental of AZ?
age
What alzheimer's medication is approved for treatment of moderate to severe dementia?
mementine
What medication is used for myotonic dystrophy?
mexilitine
What is the MOA of Botox
block presynaptic vessel release of acetylcholine (cleaves SNAP25)
What is the mainstay of treatment of PD?
carbidopa/levodopa
For patient who has PD and significant motor fluctations but does well when he is on, what is a good recommendation for management of care?
Evaluation for DBS
What are risk factors for RCVS?
...
What is the best way to diagnose RCVS?
-ct angio
-angiogram
What is the MOA of guanficine?
alpha 2A adrenoreceptor agonist
What disorder is associated with iron deposition in high concentrations in the SNr, GP
PKAN
Patient has insomnia but has sleep onset <8 minutes and with less than two SOREMPs, what do they have?
primary hypersomnia
Young patient tries to get high, has pyoderma around mouth/nasal area, what do they have?
Most likely toluene toxicity from glu sniffing
What clinical picture of that with those who have OPP poisoning?
HA, miosis, fasciculations, diffuse muscle cramping, weakness, excess secretions (nausea, vomiting, diarrhea, lacrimation)
What medication inhibits microtubule polymerization?
colchicine (not cisplatin, which causes a peripheral neuropathy)
What is the treatment for CMV?
ganciclovir
What are duret hemorrhages?
small areas of hemorrhage in the midbrain and upper pons
Patient presents with severe headaches and shoulder pain with coughing and sneezing as well as dysarthria and cerebellar signs, what are they most likely to have?
Chiari 1 malformation
Patient presents with weakness of the lower extremities and signs of cauda equina, they have a midline tuff, what are they likely to have?
lipomeningocele
Where do purkinje cells project to?
deep cerebellar nuclei (dentate) and to the cerebellar cortex which then project to the contralateral ventral lateral thalamus
What is Sneddon syndrome?
vasculopathic syndrome that involves strokes
can find antiphospholipid antibodies when testing these patients
Patient has dementia, dermatositis and diarrhea, what vitamin are they lacking?
Niacin, vitamin B3
Patients with Friedrich's ataxia have what vitamin deficiency?
vitamin E
Loss of what causes narcolepsy?
orexin
How do you diagnosis circadian rhythm disorders?
sleep history and sleep log +/- actigraphy
First line therapy for OSA?
positive airway pressure
-diagnosis with PSG
NREM parasomnias occur when in sleep cycle?
Stage 3 sleep, slow wave sleep
What do you see on PSG for those with RBD?
sleep without atonia
AD nocturnal frontal lobe epilepsy is associated with what gene?
nicotinin acetylcholine receptor gene
Patients with MMN have what antibodies?
anti-GM1
Anti-Hu is associated with what cancer?
small cell lung cancer
Patient's with cerebellar paraneoplastic disorder most likely have what ab and what cancer is it associated with?
-anti-Yo
-ovarian
Patient has RLS, what do you check and how do you treat?
-check ferritan for iron deficiency
-treat with DA agonist (pramipexole, ropinirole, rotigotien patch)
Know what PLEDs looks like on EEG
...
Patient with central temporal spikes is most likely to clinically present as what?
Benign rolandic epilepsy, young child with symptoms
What medication can cause a severe hypertensive crisis?
selegiline, MAO in GI system usually prevents entrance of large amounts of ingested tyramine
Patient presents with severe microceplahy, what test do you do?
chromosomal analysis
What kind of patients are most likely to have a new mutation pop up?
PD>SCA
Familial fatal insomnia is due to deficits in what?
prion protein
Mothers with neural tube defects are likely to have elevated what in their serum?
alpha fetal protein
Patient unable to understand emotion conveyed by someone else, what is this called?
aprosody
Damage to where can cause akientic mutism?
bilateral medial frontal cortex or anterior cingulate gyrus or anterior corpus callosum
Damage to where can cause lack of ability to recognize familial faces?
fusiform gyrus (bilateral lesions to the occipitotemporal cortex)
Cortical blindness is due to what?
Anton's syndrome = bilateral occipital lobe lesions
If a patient has ulnar neuropathy along with impaired sensation to the medial forearm, where does this localize the problem to?
thoracic outlet, this would be spared in an ulnar nerve mononeuroapthy at the elbow
Patient's with acromegaly can have what type of neuropathy?
bilateral carpal tunnel/median neuropathy
Where does the GP project to?
ventral anterior nucleus of the thalamus
PD is due to what?
Loss of neurons in the SNc so output to the striatum is decreased, there is release of GPi and SNR from inhibition allowing a larger inhibitory signal to the thalamus
Ramsay hunt is due to reactivation of herpes zoster in which ganglion?
geniculate
Gentamicin can cause what?
vestibular toxicity, can cause symptoms of difficulty tracking and + Romberg without visual problems and without cerebellar symptoms
Patient 2 months after transplant has tremor, cognitive problem and __
...
Patient with CHASD2Vasc has point of 2, what should they get for AP or AC?
AC
Young patient with syncopal episodes of BP readings different in UEs than LEs?
Takayasu's disease
Patient comes in with depression on SSRI and develops thunderclap headache, what comes to mind and how do you test?
-RCVS
-CT angio of the head
Young female comes in with branched retinal artery occlusions and history of headaches, what does she have?
Susac's syndrome
What do you see on MRI imaging of Susac's syndrome?
white matter lesions in the central fibers of the corpus callosum and leptomeningeal enhancement
Most common cause of stroke for late epilepsy?
cerebrovascular accident
What gets you the highest score for motor response for GCS?
thumbs up, fist, or peace sign to command
Patient recently was started on warfarin and complains of problems with neuropathy along anterior thigh and problems with hip flexion/psoas muscle, where is the lesions=?
intrapelvic hematoma
Patient has POEMs syndrome, what do you look for?
myeloma (osteosclerotic myeloma with polyneuropathy)
MGUS is with neuropathy is associated with which antibodies?
anti-MAG antibodies
Patient with opsoclonus-myoclonus is related to which abs?
Anti-Ri
Patient comes in with myasthenic crisis, how do you treat?
PLEX>IVIG
How is SMA inherited?
AR
Kennedy's disease is due to what?
X linked recessive
trinucleotide CAG repeat
gynecomastia, androgen receptor deficiency
What do you see on pathology for those who have IBM?
rimmed vacuoles
What should you check before starting CBZ on an asian person?
HLA B 1502?
What labs do you check for those with Kearnes Saere?
lactic acid
What is the msot common seizure induced by photic stimulation?
juvenile myoclonic epilepsy (Unverricht-Lundborg, Lafora body, NCL, MERRF, Sialodosis)
Patient with MS gets drug reaction of chest pain, anxiety, vasodilation, what did they most likely take?
glatiramer acetate
What is associated with alternating bands of destruction and preservation of myelin?
Balo concentric sclerosis
Patient presents with psychomotor delay and eventually has cherry red spot and seizures, what do they likely have?
Tay Sachs (hexosaminidase A deficiency)
Patient has macrocephaly and spasticity, what do they likely have?
either canavan or alexanders
-canavan has aspartoacyclase deficiency
Has precocious puberty been reported in NF1?
YESSS
What is deficient in abetalipoproteinemia?
vitamin A, D, E, K
How do you treat lyme disease?
IV ceftriaxone
What do you see on neuroimaging of patients with schizophrenia?
enlarged ventricle size
What area has been affected in those who are disinhibited, inappropriate?
orbitofrontal cortex
Where do unmyelinated C fibers synapse?
substantia gelatinosa
How is SMA inherited?
autosomal recessive
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