Movement Disorder 3- MJ Flashcards
Which disease?
•Impaired biliary copper excretion leads to accumulation of copper in several organs (liver, brain, cornea)
Wilson disease
The following are sxs of which disease?
•Symptoms include liver and CNS
- Liver – N/V/fatigue/jaundice/muscle cramps
- CNS - tremors or uncontrolled movements, muscle stiffness, problems with speech, swallowing, or physical coordination
Wilson Disease
What are the 2 treatment options for Wilson Disease?
- Chelating agents (D-penicillamine, Trientine)
- Oral zinc
The following describes which tx option for Wilson Disease?
•remove extra copper from the body by releasing it from organs into the bloodstream
Chelating agents (D-penicillamine, Trientine)
MOA of which med used to tx Wilson Disease?
•Contains a free sulfhydryl group that functions as a copper chelating moiety**
D-penicillamine
ADEs of which med used to tx Wilson Disease?
•Early onset:
- fever
- cutaneous eruptions
- lymphadenopathy
- neutropenia
- thrombocytopenia,
- proteinuria
•Late onset-months to years: proteinuria
D-penicillamine
Txs for Wilson Disease:
What should you give patients who are taking D-penicillamine in order to prevent pyridoxal phosphate deficiency**
Pyridoxine
How does trientine (a copper chelator) differ from D-penicillamine?
- by a lack of sulfhydryl group
- Chelates copper by forming a stable complex with its four constituent nitrogens
The following describes which drug used to treat Wilson Disease?
- Chelates copper by forming a stable complex with its four constituent nitrogens and is as effective as D-penicillamine
- MOA: Functions principally by removing copper from less strongly bound sites on proteins and membranes, and it increases renal copper excretion
Trientine
What is the pharmacokinetics of Trientine, a med used to tx Wilson Disease?
Poorly absorbed; only 1 percent of the ingested amount is excreted in the urine.
Treatments for Wilson Disease
T/F: Trientene has more adverse effects than D-penicillamine
FALSE
Trientine has fewer ADEs than D-penicillamine
Wilson Disease treatments: Trientine
- Hypersensitivity reactions and pancytopenia are _____
- Neurologic worsening is seen but appears to be ______ common than with D-penicillamine
- rare
- less
Treatments of Wilson disease:
Trientine is not specific to copper, it also chelates _____. Because of this, what should you not coadminister with this medication?
Iron
So don’t give iron! The trientine iron complex is nephrotoxic
Wilson Disease Treatment: Trientine
- Copper deficiency induced by trientine can lead to _____ overload in the liver.
- This excess______ may potentiate liver injury
iron
Monitoring:
Adequacy of trientine treatment (for Wilsons Disease) should be determined by measuring _____ _____ ______, which should be in the range of 200 to 500 mcg (3 to 8 micromoles) daily
24-hour copper excretion
MOA of which med used to tx Wilson Disease?
- interferes with the absorption of copper
- Induces metallothionein (an endogenous chelator of metals) in enterocytes which has a greater affinity for copper than for zinc, causing it to bind luminal copper and thereby preventing its entry into the circulation
- Excreted in stool
Oral Zinc
Treatments for Wilson Disease:
- The 2 forms of oral zinc salts are zinc acetate, zinc sulfate and zinc gluconate.
- Which one has better absorption? Which one is more tolerable?
- Best absorption= zinc acetate
- More tolerable (than zinc sulfate) w/ respect to GI side effects= Zinc gluconate
What are the 2 adverse effects of oral zinc?
- GI upset
2. Elevation in serum amylase (also seen in saliva) and lipase w/o clinical evidence of pancreatitis
What disease?
- disorder of the motor neurons of the ventral horn of the spinal cord (lower motor neurons) and the cortical neurons that provide their afferent input (upper motor neurons)
- gradual deterioration and death of motor neurons
- rapidly progressive weakness, muscle atrophy, fassciculations, spasticity, dysarthria, dysphagia, resp. compromize
- Sensory, autonomic and oculomotor function generally spared
ALS
Suspected etiology of which disease?
•Evidence that glutamate reuptake may be abnormal in the disease, leading to accumulation of glutamate and excitotoxic injury
ALS
Which med used to treat ALS?
- Presynaptic and postsynaptic effects
- Inhibits glutamate release**
•Inactivation of voltage-dependent Na+ channels**
•Ability to interfere with intracellular events that follow transmitter binding at excitatory amino acid receptors**
Riluzole
Which ALS med’s in vitro action is blocked by pertussis toxin?
Riluzole
Which med? (used to tx ALS)
- absorbed orally and is highly protein bound
- High fat meals decrease absorption
- extensive metabolism in the liver by both CYP–mediated hydroxylation and glucuronidation
Riluzole
What are the 5 ADEs of riluzole? (drug used to tx ALS)
“HANDE”
- _HTN*_
- _Abd pain*_
- _Neuromuscular and arthralgia, tremor*_
- _Decreased lung function*_
- _Elevations of serum transaminases (periodic monitoring recommended)*_
“HANDE”
Which ALS drug has the following:
- Slows decline of physical function- MOA unknown
- Free radical scavenger
- Prevents oxidative damage to cell membranes
Edaravone
Which drug (Riluzole or Edaravone) is indicated for patients with ALS who:
- Have a disease duration of <2yrs
- Living independently
- Have an FVC ≥80 percent
Edaravone
What are the 4 ADEs of Edaravone (used to tx ALS) and why should you be cautious of giving this to a pt w/ asthma?
- Injection site contusion
- Gait disturbance
- HA
- Allergic rxns (b/c of sodium bisulfite)
sulfites can cause serious asthmatic reactions in as <5% of asthmatic pts
What are the 3 meds you can give for symptomatic therapy of ALS, specifically spasticity?
- _Baclofen *_
- _Tizanidine *_
- _Clonazepam *_
What does Baclofen do to tx ALS?
•Baclofen acts to restore the lost inhibition by stimulating postsynaptic GABA receptors
(note- Baclofen works postsynaptically and TIzanidine acts presynaptically)
How does Tizanidine help tx ALS?
•Tizanidine acts presynaptically to stimulate GABA release from spinal cord inhibitory interneuron.
(note- Baclofen works postsynaptically and TIzanidine acts presynaptically)
MOA of TIzanidine or Baclofen? (used to tx ALS)
- GABAB receptor agonist= ______
- agonist of α2 adrenergic receptors in the CNS=_______
•GABAB receptor agonist= Baclofen (B for Baclofen)
•Agonist of α2 adrenergic receptors in the CNS= Tizanidine
- Increases presynaptic inhibition of motor neurons
What is the 1 ADE of Baclofen? (used to tx ALS)
Sedation
T/F: an Alternative for delivery of Baclofen for tx of ALS is:
directly into the space around the spinal cord using a surgically implanted pump and an intrathecal catheter
True
What are the 3 ADEs of Tizanidine? (used to tx ALS)
“DAD”
•Drowsiness
•Asthenia (abnormal physical weakness or lack of energy)
•Dizziness
Which drug class can be used to tx ALS and are effective antispasticity agents but can contribute to respiratory depression in pts w/ advanced ALS?
Benzodiazepines (Diazepam or Clonazepam)
What is the MOA of Benzodiazepines (Diazepam/clonazepam) in the tx of ALS
•Enhances the inhibitory effect of GABAA receptors
What are the 4 ADEs (“CADS”) of Benzodiazepines in the tx of ALS?
What other condition exhibits these effects?
- Amnesia
- Confusion
- Drowsiness
- Slurred speech
Other condition: Being Drunk
Which disease?
- Neuromuscular disease characterized by weakness and marked fatigability of skeletal muscle
- Exacerbations and partial remissions occur frequently
- Defect in the synaptic transmission- autoimmune response primarily to the ACh receptor at the post-junctional end plate.
Myasthenia Gravis
What are the 2 drugs used to tx Myasthenia Gravis and what is their mechanism?
Anticholinesterase drugs: (“NPs treat Myasthenia Gravis”)
- Pyridostigmine
- Neostigmine
What is the mechanism of Neostigmine and Pyridostigmine, anticholinesterase drugs used to tx myasthenia Gravis
Inhibit the action of the metabolizing enzyme acetylcholinesterase
Tx of Myasthenia Gravis:
What is the drug of choice to reverse too much acetylcholine?
Atropine (anticholinergic)
What are the 6 ADEs of anticholinesterase medications? What 2 are most important because they could be life threatening?
ADEs: “SLUDGE”
- Salivation
- Lacrimation
- Urination
- Diarrhea
- GI upset
- Emesis
Most important:
- Cardio
- Respiratory
- Pupils will be constricted–> how to judge it
