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62 Cards in this Set
- Front
- Back
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Patient presents with drooping eyelids, fatigue, trouble swallowing, blurred vision, respiratory difficulties, partial paralysis and recurrent episodes of muscle weakness after exercise - diagnosis
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MYASTHENIA GRAVIS
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Myasthenia gravis affects _ more
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WOMEN
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Myasthenia gravis chiefly affects what muscles
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Supplied by cranial nerves
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In myasthenia gravis there is defect in _
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Signaling at neuromuscular junction
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Describe Tensilon testing
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- Injection of Endrophonium is administered
- Test is positive for Myasthenia Gravis if muscles respond in 30 to 45 seconds after injection - Improvement in strength may persist for 5 minutes |
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Binding site of nicotinic N1 receptor
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Alpha 1 subunit
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Subunit of N1 receptor affected in typical MG
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Alpha 1 subunit
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Acquired slow channel syndrome that is similar to MG but not as severe affects _
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DELTA SUBUNIT
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In neonatal myasthenia gravis which subunit of N1 is affected
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Gamma
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80-90% of patients with myasthenia gravis have autoantibodies to _
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N1 Ach receptor
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Possible triggers of myasthenia gravis
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- Drugs (penicillamine)
- Thymoma - Virus - genetics |
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Auto immune disorder against voltage gated Ca channels is called _
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Lambert Eaton Syndrome
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Ach receptor consists of _ subunits
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5
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N2 is different from N1 Ach receptor in that it consists of _
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Only alpha and beta subunits
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Which tests should be done in patient being worked up for Myasthenia Gravis
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1) Tensilon test
2)Serum antibody immunoprecipitation test against fetal or adult N1 receptors 3) Repetitive nerve stimulation 4) Scan of thymus |
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Which drugs are used for treatment of myasthenia gravis
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Anticholinesterase inhibitors
- Endrophonium - used for diagnostic tests - Physostigmine - one of the first used - Neostigmine - Pyridostigmine - most prescribed |
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Inactivates Ach
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Acetyl cholinesterase
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Other than anticholinesterase inhibitors what treatments are used for Myasthenia Gravis
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Steroid medications
Immunosuppressive medications Thymectomy |
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Patient presents with pill rolling tremor, bradykinesia, rigidity and postural instability - diagnosis
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PARKINSONS
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Major input to substantia nigra
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Caudate and putamen
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Parkinsons disease is caused by _
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Loss of pigmented DA producing cells from substantia nigra
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When do you start showing symptomps in Parkinsons
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When 80-90% of DA producing neurons in substantia nigra are gone
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Characteristic neuropathology of Parkinsons is presence of _
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LEWY BODIES
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Which radio tracer is used for PET scanning when look for Parkinsons
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6-fluoro-dopa
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How can you increase synthesis of DA
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By using L dopa
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Why can L dopa be administered to increase dopamine production but dopamine itself cannot when treating Parkinsons
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DOPAMINE does NOT cross BBB
L DOPA CROSSES BBB |
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Carbidopa is usually administered with L DOPA - why
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To block conversion of L dopa to dopamine outside of brain since dopamine has peripheral effects
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Which enzyme breaks down dopamine
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MAO
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Name drugs that block degradation of dopamine
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MAO INHIBITORS
DEPRENYL SELEGILINE TOLCAPONE ENTACAPONE |
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Name dopamine receptor agonist
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Bromocriptine
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Marker for DA turnover
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HVA - homovallinilic acid
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Describe breakdown of DA
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DA - DOPAL - DOPAC- HVA
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Side effect of L dopa is _
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Hallucinations
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Name drugs that induce Parkinson like symptoms
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RESERPINE (for hypertension) - prevents repackaging of DA
CHLORPROMAZINE (antipsychotic) - dopamine receptor antagonist |
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Anticholinergics have some use in treating Parkinsons - T/F
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TRUE
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MAO inhibitor and dopamine reuptake blockers prevent _ damage and show at least some protection against _
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MPTP
PARKINSONS |
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Patient presents with hallucinations, delusions, bizzare behavior, social withdrawal and emotional blunting - diagnosis
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SCHIZOPHRENIA (affects 1% of people in US, 9-13% suicide rate)
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In SCHIZOPHRENIA there is altered orientation of _ which involved in cells _
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Hippocampal pyramidal cells
Migration and adhesion |
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Enlarged ventricles are noted in _
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Schizophrenics
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What is biochemically wrong in schizophrenia
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ELEVATED DOPAMINE LEVELS
Elevated dopamine metabolites in CSF - HVA Elevated numbers of DA receptors (D2) in brain |
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Mechanism of action of typical antipsychotics
Side effects? |
Block DA TRANSMISSION - Parkinson like side effects
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Activation of 5HTa and 5HTc receptors causes _
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Hallucinations (this receptor is activated by LSD)
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Mechanism of action of atypical antipsychotics
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Block serotonin
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Progressive impairment of cognitive function (dementia)occurs in patients with _
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ALZHEIMERS
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Hallmarks of Alzheimers
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Plaques and tangles - protein amyloid beta peptide and tau protein
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Amyloid proteins stain _
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Blue with iodine
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Plaque in Alzheimers is composed of _ derived from _
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Amyloid beta peptide
Proteolysis of Amyloid precursor protein (APP) |
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Why are amyloid beta peptides neurotoxic
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Increase intracellular Ca
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Gene for Alzheimers is found on _
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Chromosome 21
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Primary pathological event in Alzheimers
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Extracellular deposition of amyloid beta peptide
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_ are reduced in Alzheimers
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Ach and CHAT
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Mutation of those genes can lead to Alzheimers
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APP - amyloid precursor protein
Presenilin Apolipoprotein E |
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How is Huntingtons inherited
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Autosomal dominant
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In Huntingtons neurons in _ die
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Corpus striatum
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Gene responsible for Huntingtons has been isolated on chromosome _ - it encodes protein called _
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4
Huntingtin |
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What goes wrong on genetic level in patients with Huntingtons
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INCREASE IN CAG REPEATS (from normal 10-20 to 120)
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In patients with Huntingtons there is excessive _
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Glutamate- blockage of GABA receptor and release of excessive glutamate - EXCITOTOXICITY
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Why does excessive glutamate in patients with Huntingtons kills cells
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NMDA receptors let Ca in
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Which drugs are used to control choreic movements in patients with Huntingtons
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Benzodiazepines - GABA enhancers
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In patients with stroke there is extra release of _
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GLUTAMATE --> EXCITOTOXICITY
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Ischemia triggers _
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GLutamate release from oxygen-starved neurons
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Alcohol for a long time increases _ --> this _ receptors --> withdrawal seizures
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GABA
DOWNREGULATES |
