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54 Cards in this Set
- Front
- Back
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... - Rhythmic oscillation of agonist and antagonist muscles
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tremor
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... - Seen in all individuals to some degree when the arms are outstretched
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physiologic tremor
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... tremor:
-Postural or kinetic tremor -Commonly involves the head -fine motor skills -nervousness -gets worse when nerves are upset |
essential tremor
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essential tremor – tremor that gets worse at endpoint
-can be difficult to treat Treatment -May improve with small amounts of ... -... blockers -... (barbiturate) -Topiramate, pregabalin, gabapentin -Weighted utensils Treat if: 1) functional problems 2) embarrassing to them |
alcohol
Beta Primidone |
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... tremor:
-aka kinetic tremor -no tremor seen at rest -most problematic with voluntary movements |
cerebellar
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Rest Tremor:
-... tremor (‘pill rolling”) -Most obvious when the limb is fully supported and at rest -When a person’s doing stuff, they won’t tremor. They will get rhythmic movement tremor when they are at rest. |
Parkinsonian
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...:
-(abnormal) Sustained muscular spasm that can be focal (individual body parts) or generalized |
dystonia
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Focal Dystonias:
... (very common) -Involuntary spasms of the orbicularis oculi, resulting in eye closure -Usually very easy to treat with Botox |
Blepharospasm
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Focal Dystonias:
... (cervical dystonia) -Involuntary twisting, turning, and tilting of the neck, often associated with pain and tremor -The treatment of choice is Botox |
Torticollis
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Focal dystonias:
... Dystonia -Facial grimacing, at times isolated to jaw opening or closing -... Syndrome consists of eye and jaw movements |
Oromandibular
Meige’s |
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focal dystonias:
... - Intermittent spasms of the vocal cords -tight vocal cords -tx. botox (done by ENT specialist) |
Spasmodic Dysphonia
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... - Sudden, fast, irregular movements, usually repeated in the same muscle group (patient has some control)
-Motor: simple vs complicated -Vocal: simple vs complicated Treatment: -Usually don’t require treatment unless they are troublesome (usually treated often in kids due to social issues) -Education and reassurance -Medications *Clonidine (alpha-receptor blocker) *Neuroleptics |
Tics
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... syndrome: multiple motor tics with at least one vocal tic
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Tourette’s
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...:
-Quick, irregular, often semi-purposeful movements -Huntington’s disease |
Chorea
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...:
Violent type of flinging movements occurring on one side of the body -Nothing purposeful about it |
Hemiballismus
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...:
Sudden, fast movements, usually repeated in the same body part Can be treated with various anticonvulsants -Valproate (seizure medicine) -Lamotrigine (seizure medicine) -Levetriacetam (seizure medicine) -Clonazepam (benzodiazapine – also used for seizures) Hiccups are an example |
Myoclonus
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... – (chewing type movement)
-Combined chorea and dystonic movements, usually in the face or lower jaw, but may be generalized -Commonly associated with exposure to neuroleptic medications -The best way to manage this condition is to prevent it from occurring in the first place |
tardive dyskinesia
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what are the 4 cardinal features of Parkinson’s disease?
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Tremor (at rest)
Bradykinesia Rigidity Postural instability |
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Pathophysiology of Parkinson’s Disease:
Marked striatal ... depletion -Probably a ... component and most certainly an ... component (people in more rural environments are more likely to get parkinson’s) <...% DA loss is asymptomatic ~...% DA loss for symptom manifestations -Most commonly starts out on one side of the body. As it progresses, it spreads to the other side of the body Severity of DA loss best correlates with ... in PD |
DA (dopamine)
genetic environmental 50 70 bradykinesia |
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Drug Classes for Treatment of PD:
Dopaminergic agents -...– most obviously used -Dopamine ... (requip, mirapex) COMT inhibitors MAO-B inhibitors Anticholinergics Amantadine |
Levodopa
agonists |
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PD:
Levodopa (carbidopa/levodopa) -Most effective drug for parkinsonian symptoms (best for patients with ...) -First developed in the late 1960s; rapidly became the drug of choice for PD -Rapid peripheral decarboxylation to dopamine without a decarboxylase inhibitor (DCI: carbidopa) -Side effects: nausea, postural hypotension, dyskinesias, motor fluctuations -... of administration is important (wears off after 4-6 hours) |
true parkinson’s
timing |
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PD:
Anticholinergics -... depletion®cholinergic over activity -Initially used in the 1950s -Effective mainly for ... and ... -Common agents (Start low, go slow): *Trihexyphenidyl: 2-15 mg/day *Benztropine: 1-8 mg/day Side effects: -Dry mouth, sedation, delirium, confusion, hallucinations, constipation, urinary retention |
Dopaminergic
tremor and rigidity |
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PD:
Dopamine Agonists -Directly stimulate dopamine receptors -No metabolic conversion; bypasses ... neurons -No absorption delay from competition with dietary amino acids -... half-life than levodopa -Monotherapy or adjunct therapy -May delay or reduce motor fluctuations & ... associated with levodopa |
nigrostriatal
Longer dyskinesias |
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PD:
Main dopamine agonists: ... and ... |
pramipexole (Mirapex) and Ropinirole (Requip)
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PD:
Dopamine Agonist Side Effects (pretty much same as levadopa) -Nausea, vomiting -Dizziness, postural hypotension -Headache -Dizziness -... & somnolence -Dyskinesias -Confusion, hallucinations, paranoia, ... behaviors |
Drowsiness
compulsive |
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PD:
...: -tolcapone (monitor for liver toxicity), entacapone -Potentiate LD: prevent peripheral degradation by inhibiting catechol O-methyl transferase (prevents degredation of levadopa. Extends the life) -Reduces LD dose necessary for a given clinical effect -Helpful for both early and fluctuating Parkinson’s disease -May be particularly useful for patients with “brittle” PD, who fluctuate between off and on states frequently throughout the day |
COMT Inhibitors
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PD:
...: -Antiviral agent; PD benefit found accidentally -Tremor, bradykinesia, rigidity & ... Exact mechanism unknown; possibly: -enhancing release of stored dopamine -inhibiting presynaptic reuptake of catecholamines -dopamine receptor agonism -NMDA receptor blockade Side effects —autonomic, psychiatric 200-300 mg/day |
Amantadine
dyskinesias |
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PD:
...: -selegiline, rasagiline -Clinically active by inhibiting dopamine metabolism in brain -May be ... -Side effects: insomnia, hallucinations, nausea (rarely), OH -Potential interactions with tricyclics and SSRI antidepressants |
MAO-B inhibitors
neuroprotective |
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PD:
... intervention may be indicated when medical management is no longer possible |
Surgical
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...:
-can be induced by a wide variety of disease processes -are the result of other causes; don’t respond to Parkinson’s medications |
secondary parkinsonism
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Secondary parkinsonism:
Drug-induced parkinsonism -Any drug that blocks striatal ... receptors can cause symptoms indistinguishable from idiopathic Parkinson’s disease *Older neuroleptic medications *... (most common) *Even the “atypical” neuroleptic medications can -Stopping the offending ... can result in resolution of parkinsonism |
D2 dopamine
Metaclopramide medication |
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Secondary parkinsonism:
Neuroleptic medications that block the extrastriatal ... receptors, such as clozapine, do not cause parkinsonism |
D4 dopamine
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Secondary Parkinsonism:
Toxins -MPTP (1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine) -... (failed attempts of suicide, gas leaks) *Causes necrosis of the basal ganglia *Usually occurs weeks after recovery from coma -... (any welders, miners exposed) -Methanol, cyanide |
Carbon monoxide
Manganese |
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Secondary Parkinsonism:
Cerebrovascular disease -Small-vessel infarcts in the ... -Commonly associated with cognitive dysfunction (dementia) Trauma -... encephalopathy (Boxers, contact sports) *Parkinsonism *Dementia *Ataxia |
basal ganglia
Pugilistic |
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Secondary Parkinsonism:
Aftermath of ... -Oliver Sachs, MD’s book “Awakenings” -50% of survivors of the ... epidemic that occurred from 1917-1925 developed parkinsonism, often decades after the acute infection |
encephalitis
encephalitis |
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These are cardinal features of what disorder?
1) gait ataxia (shows up first) -magnetic gait, feet sticking to the floor 2) urinary incontinence 3) cognitive decline -short term memory loss |
Normal pressure hydrocephalus
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Normal Pressure Hydrocephalus:
Pathophysiology -A form of ... hydrocephalus -Up to 30% of affected individuals have a history of spontaneous or traumatic subarachnoid ... or ... -Even though CSF pressure is ..., the increase in fluid in the lateral ventricles exerts pressure on the walls of the ventricles and surrounding structures |
communicating
hemorrhage meningitis normal |
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Normal Pressure Hydrocephalus:
Diagnosis -... or ... of the brain -Nuclear cysternogram -High-volume lumbar puncture (... test) -Measure pressure (should be normal) -Look for improvement in symptoms -May need to repeat |
MRI or CT
Fisher |
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What is the treatment for normal pressure hydrocephalus?
-Very difficult to predict with a high degree of certainty who will significantly improve with this ~50% |
shunt placement
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If you put someone on Sinemet, and their symptoms worsen, you know that they do not have ...
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Parkinson’s disease
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Multiple system atrophy:
Clinical Features -multiple areas of the brain are not working -... degeneration: akinetic-rigid parkinsonism, usually ... tremor. -... degeneration: cerebellar signs, especially ataxia and dysarthria, along with parkinsonism. May also have upper motor neuron findings -Shy-Drager Syndrome: ... dysfunction |
Striatonigral
without Olivopontocerebellar autonomic |
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Multiple system atrophy:
Clinical features summarized: hypokinesia poorly responsive to ..., with prominent autonomic and or cerebellar dysfunction |
levodopa
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Multiple system atrophy:
Treatment -Primarily ... -Must be very cautious when offering a trial of levodopa (Sinimet can often make things worse) |
symptomatic
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...:
Clinical considerations -Early onset of gait ataxia with a tendency to fall backwards as well as episodes of freezing -Gaze palsy (initially downgaze) -Cognitive dysfunction -Tremor is usually absent -There is usually little to no response to levodopa -Treatment is symptomatic |
Progressive Supranuclear Palsy
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...: (rare)
Pathophysiology -An autosomal recessive condition associated with impairment of copper excretion -Copper accumulates in various organ systems, including the CNS, liver, eye, heart, and kidney. -will cause cirrhosis of the liver |
Wilson’s disease
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Wilson’s Disease Clinical features:
Age at onset ranges from 5-50 years, peaking between 8 and 16 years Neurological symptoms are present in 40% of affected individuals at the onset -... disorders (tremor, dystonia, rigidity, bradykinesia, etc…) -..., particularly in adults -... symptoms ... dysfunction (cirrhosis, chronic active hepatitis) Deposition of ... in iris seen on slit lamp examination (Kayser-Fleischer rings) |
Movement
Dysarthria Psychiatric Liver copper |
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Wilson’s disease:
Evaluation -... brain: shows generalized atrophy, including the brainstem, as well as abnormalities in the basal ganglia -(blood and urine tests) Measure serum ceruloplasmin (will be ...) and 24 hour urinary copper (will be ...) -Slit lamp examination to look for ... rings (present in 99.9%) -Consider ... biopsy |
MRI
decreased increased Kayser-Fleischer liver |
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Wilson’s disease Treatment:
...: -Blocks the absorption of copper in the gut -50mg three times a day -Too slow acting to use as initial treatment, but is commonly used for maintenance therapy -Can also be used in pregnancy ...: -Acts by means of reductive chelation of copper -Initially, as much as 10-15 mg of copper may be excreted per 24 hours -Serious hypersensitivity reactions can occur with this agent -Can cause serious bone marrow suppression |
Zinc
Penicillamine |
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Wilson’s disease Treatment (cont):
...: -A newer chelating agent that has fewer side effects than penicillamine -Does not mobilize as much copper initially compared with penicillamine Low-... diet: limit lobster, chocolate, and liver ... transplantation Consultation with Wilson’s Disease specialist |
Trientine
copper Liver |
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Huntington’s disease:
Clinical features -Various movement problems, with ... being the most common motor manifestation -Bradykinesia coexists with chorea -Cognitive decline -Psychiatric abnormalities -An abnormality of saccadic ... movements may be the first neurological signs -Typical onset is in the ... or ... decade, but 10% may have symptoms before the age of 20 (juvenile Huntington’s) |
chorea
eye fourth or fifth |
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Huntington’s Disease:
Pathophysiology -Autosomal ... -Defect is on chromosome ... and consists of an expansion of the usual number of trinucleotide sequence CAG (>... CAG repeats is diagnostic for HD) -Subsequent generations may show symptoms earlier due to ... -This results in the production of a protein called “...” -There is progressive degeneration of the ... |
dominant
4 40 amplification huntingtin caudate |
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HD Diagnosis:
Can be diagnosed with ... testing -Must consider medical, legal, and psychosocial ramifications of ... testing before proceeding Usually can be diagnosed based on history, examination, and family history |
genetic
genetic |
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HD:
Treatment is symptomatic -Chorea is managed with neuroleptics (... most commonly) or ... such as lorazepam or clonazepam -... care to assist in managing the myriad of psychological problems associated with this disorder (people can become suicidal) -Physical, occupational, speech therapies when needed |
haloperidol
anxiolytics Psychiatric |
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HD:
Prognosis -The duration of illness from onset to death is about ... years for adult-onset disease and about ...-... years for juvenile onset |
15
8-10 |
