Mix6

Question 1

Opsoclonus-myoclonus is a paraneoplastic syndrome assoc with what type of cancer?

Answer 1

Neuroblastoma (overexpressed N-myc). Kids < 4 yo. Occurs anywhere along sympathetic chain but mostly in the adrenal medulla.

Question 2

Spinal muscular atrophy presents in infancy as delayed motor development with flaccid paralysis (hypotonia, decrased DTR). The mutation responsible for spinal muscular atrophy is in SMN1 that codes for what protein? How does this mutation result in the clinical manifestations of the Dz?

Answer 2

SMN1 codes for a protein essential in the assembly of snRNP (and if this mutated, then splicesome can’t form). Defective snRNP formation results in impaired splicesome formation and degeneration of the anterior horn cells in the spinal cord.

Question 3

What class of medication is most assoc with development of drug induced parkinsonism?

Answer 3

Presents as cogwheel rigidity, resting tremor, masked face. Antipsychotics (1st gen > 2nd gen) like haloperidol, fluphenazine, and trifluoperazine. Caused by D2 receptor blockade in nigrostriatal pathway. Can be treated with drug reduction or switching to centrally acting muscarinic agent (benztropine or trihexyphenidyl).

Question 4

How would a patient with cerebellar ataxia respond in the Romberg test?

Answer 4

Romberg test is for proprioception to distinguish sensory from cerebellar ataxia. If patient sways when their eyes are closed this indicates sensory ataxia (damage to the posterior column and peripheral nerves) generally due to B12 deficiency or tabes dorsalis.

If there is no change in unsteadiness with eyes closed, this is likely due to cerebellar ataxia from impaired motor coordination (negative Romberg)

Question 5

What are the results of a lesion in the nondominant parietal lobe?

Answer 5

Lesions in the nondominant hemisphere contralateral neglect, anosognosia (lack of awareness of illness), and constructional apraxia (inability to draw objects).

Question 6

___ is an immature defense mechanism that involves misattributing one’s own unconscious, undesired thoughts or feelings to another person who does not actually have them.

Answer 6

projection (immature defense mechanism)

** By projecting these unacceptable feelings onto another person, the Pt can be relieved of these thoughts without having to deal with the internal conflict of self-expression.

Question 7

What medications can be used to treat pheochromocytoma?

Answer 7

a-adrenergic AR antagonists like phenoxybenzamine and phentolamine.

Question 8

Is Babinski sign upgoing or downgoing plantar reflex?

Answer 8

upgoing. Indicates UMN lesion.

** UMN lesions cause spastic paralysis, hyperreflexia, and upgoing plantar reflex due to the loss of descending inhibition of the LMN in the anterior horn.

Question 9

What’s the MOA of ramelteon in insomnia Rx?

Answer 9

Ramelteon = melatonin R agonist (safe for geriatric patients).

Binds to melatonin R in suprachiasmatic nucleus.

Question 10

How do opiates like morphine exert their analgesic effects?

Answer 10

All opiates bind to GPCR coupled to Gi proteins. In the spine, opiates bind to Mu R on primary afferent neurons&raquo_space; closure of VG Ca2+ channels. Also bind to Mu R on postsynaptic neurons&raquo_space; K+ efflux&raquo_space; hyperpolarization.

Question 11

What’s the first line Rx for trigeminal neuralgia? What’s it’s MOA?

Answer 11

Carbamazepine. Neuroleptic med that reduces ability of Na+ channels to recover from inactivation&raquo_space; inhibition of high frequency neuronal firing.

Question 12

What are the 2 major side effects of carbamazepine use that you need to look out for?

Answer 12

BM suppression and SIADH (can progress to hyponatremia)

Question 13

Ketamine, felbamate, and memantine are examples of meds that block which receptor?

Answer 13

NMDA

Question 14

Fluoxetine and sertraline are both examples of what drug class?

Answer 14

SSRI

Question 15

What’s the MOA of ethosuximide for absence seizures?

Answer 15

Blocks T type Ca2+ channels and decreases Ca2+ current in thalamic neurons.

Major side effect = headache, worsening anxiety/agitation and insomnia.

Question 16

Donepezil is used as a treatment for Alzheimer’s by what action?

Answer 16

AChE inhibitor

Question 17

Why do patients with early Alzheimer’s disease also have a risk of intracranial hemorrhage?

Answer 17

Because b-amyloid can deposit in the media/adventitia of cerebral vessels (amyloid angiopathy)&raquo_space; vessel weakening with intracranial hemorrhage.

Question 18

What’s the MOA of using bupropion in treating depression?

Answer 18

It’s an atypical antidepressant. Works like amphetamines in that it inhibits reuptake of NE and dopamine.

Look out for amphetamine effects (tachycardia, insomnia) and seizures in anorexic/bulimic patients.

Question 19

What drug would you use to prevent serotonin syndrome in Pt taking an SNRI?

Answer 19

Cyproheptadine (5HT R antagonist)

Question 20

A patient presents with progressively worsening dizziness, limb and truncal ataxia, dysarthria, and visual disturbances (diplopia, oscillopsia) and a L lung mass. It was found that he had pareneoplastic cerebellar degeneration. What serum antibodies could confirm this Dx?

Answer 20

Paraneoplastic cerebellar degeneration is due to an immune response against tumor cells that cross reacts with Purkinje neuron Ag&raquo_space; acute onset of rapid degeneration of the cerebellum.

Anti-Yo, anti-P/Q, and anti-Hu Ab can be found in serum.

Question 21

If a Pt presented suddenly with vertigo, nystagmus, ipsilateral lung ataxia, and truncal lateropulsion (falling toward lesion) then what is the most likely cause?

Answer 21

Cerebellar infarction

Question 22

A cerebellar hemangioblastoma in association with congenital cysts of the kidneys, liver, and pancreas is highly suggestive of ___, an autosomal dominant condition.

Answer 22

VHL (Von Hippel Lindau)

Question 23

___ is an inherited peripheral nervous system tumor syndrome in which Pt develops neurofibromas, optic nerve gliomas, Lisch nodules (pigmented nodules of iris), and café au lait spots.

Answer 23

NF1 aka Von Recklinghausen’s Dz.

Question 24

___ is a rare sporadic congenital neurocutaneous disorder characterized by the presence of cutaneous facial angiomas and leptomeningeal angiomas. Skin involvement typically affects small capillary BV following the distribution of the trigeminal nerve branches V1 and V2. Also assoc with mental retardation, seizures, hemiplegia, and skull radiopacities (tram track calcifications)

Answer 24

Sturge Weber Syndrome

Mutation in neural crest derived cells with somatic mosaicism affecting mutation in one copy of GNAQ.

Question 25

Huntington’s Dz is assoc with an autosomal dominant CAG trinucleotide repeat seq in the gene that codes for huntingtin protein. How does this end up causing neuronal damage?

Answer 25

Gain of function mutation. Expansion of the proteins polyglutamine region (CAG repeats) causes gain of function in which the abnormal huntingtin causes increased histone deacetylation&raquo_space; transcriptional repression and silencing the genes needed for neuronal survival.

• Remember that insertion of acetyl group can weaken DNA-histone bond to allow for ^ transcription.

Can possibly give HDAC inhibitors to help upregulate survival genes.

Question 26

What are the chronic effects of ethanol use that can cause tolerance?

Answer 26

Acute effects = GABA-a potentiation&raquo_space; sedation

Chronic effects = downregulation of GABA receptors and weak inhibition of excitatory NMDA R&raquo_space; upregulation of these receptors.

Withdrawal effects are due to CNS Excitation and sympathetic hyperactivity.

Question 27

What substance causes the greatest number of overdose deaths in the US?

Answer 27

Opioids (including prescription pain meds and heroin)

Question 28

What drugs should you use for the acute treatment of agitation and psychosis assoc with delirium?

Answer 28

First gen antipsychotics (haloperidol) - blocks dopamine D2 R&raquo_space; ^ cAMP

Question 29

What drug class does clozapine belong to, and what is it’s primary indication for?

Answer 29

Clozapine = 2nd gen antipsychotic used for Pt with treatment refractory schizophrenia.

Question 30

Naltrexone, disulfiram, and acamprosate are all indicated in the management of a Pt with chronic alcohol abuse. What are their different MOA and indications for use?

Answer 30

Naltrexone (oral and injection) acts as a Mu-opioid R antagonist and can be given while the Pt is still drinking. It blocks reward pathway for EtOH.

Disulfiram inhibits aldehyde dehydrogenase&raquo_space; hangover effects.

Acamprosate modulates glutamine neurotransmission at the NMDA R once abstinence is achieved.

Question 31

What’s the MOA of using varenicline for nicotine addiction?

Answer 31

It’s a partial neuronal nicotinic R agonist and prevents nicotine stimulation of the mesolimbic dopamine system.

Question 32

At what age to children develop an understanding of the concept of gender?

Answer 32

3-5

Question 33

At what age to children develop a sense of permancy of their gender?

Answer 33

5-6

Question 34

Would symmetrical enlargement of the ventriculi be characteristic of communicating or noncommunicating hydrocephalus?

Answer 34

Communicating.

Communicating hydrocephalus normally occurs secondary to dysfunction or obliteration of the subarachnoid villi. This is usually a result of a meningeal infection (including tuberculous meningitis) or SAH/intraventricular hemorrhage.

Question 35

Involuntary movements like lip smacking and choreoathetoid movements as a result of prolonged antipsychotic use is known as:

Answer 35

tardive dyskinesia

Occurs as a result of long term treatment with dopamine antagonists. Happens mostly with older Pt and 1st gen antipsychotics (i.e. haloperidol)