Flashcards in Movement Disorders Deck (16):
-how do you acquire this?
-age of onset
Acquire: autosomal dominant
Characterized by: chronic progressive chorea, psychological changes, dementia
-brain cells waste away
-Decrease in GABA and GABA receptors in basal ganglia. There is an imbalance of dopamine and Ach that cause disease manifestations.
(GABA and Ach reduced & dopamine normal)
*GABA and Ach are inhibitory NT
*Dopamine is excitatory NT
-Sx in adult/child
-early psychological manifestations
-other early/late physical signs
-chorea affects the limbs and trunk
Juvenile Onset: RARE
-Depression* (lack of initiative, spontaneity, inability to concentrate)
-Impulsive behavior (almost like manic bipolar)
Early: fidgeting, restlessness
Late: chorea, dystonic posturing, progressive rigidity, akinesia, dementia
-MRI - caudate atrophy (caudate: one of the structures that make up the basal ganglia)
-PET: abnormal metabolic changes in the caudate
-Genetic Testing: sensitive and specific
Tx: can only treat sx
-downregulate dopaminergic neurotransmission
-suppress chorea (Tetrabenazine; breaks down dopamine & Neuroleptics/antipsychotics; deplete cerebral dopamine)
Depression: Prozac, Zoloft, Aventyl(Nortriptyline; TCA)
Postural rigidity and instability: Klonopin(clonazepam), Depakote (Valproic Acid)
Antipsychotics: Resperdal(Resperidone) & Olanzapine (Zyprexa)
-how do you acquire?
-describe the tremor
-where can tremor occur?
-highest incidence at what age?
-what makes the tremor worse?
Describe: affects both sides of the body symmetrically, more prominant with ACTION than rest, frequency of tremor is constant. Amplitude may vary.
Tremor may occur in hands, head, hands and head, or voice if laryngeal muscles affected.
Highest incidence in 60+, but may begin at any age.
-stress, sleep deprivation, stimulants
*ETOH may relieve essential tremor
Essential Tremor Tx
Atenolol- beta blocker of choice for those w/ asthma or bronchospasm
Mysoline (PRimidone)- anticonvulsant
Gabapentin (Neurontin) - anticonvulsant
-age at onset
T- resting/posture tremor
P- postural instability
generally dx between 45-65, but can occur at earlier stages.
-loss of dopamine in the substantia nigra
-defective alpha-synuclein and lewy bodies
-Increase in cholinergic interneuron activity d/t degeneration of dopamine pathways (contributing to tremor)
What is the leading cause of neurologic dz in pts over 65?
-Tremor; mainly hands and feet, pill rolling, usually unilateral, appears at rest and disappears with movement and sleep
-Rigidity: cog-wheel, ratchet-like movement, resistance to movement
-Akinesia (Bradykinesia); slowness in initiating and performing movements, difficult turning, frozen in place esp. when moving through doorway or preparing to turn.
-Postural Instability: lean forward to maintain center of gravity, shuffling steps w/o arm swing, prone to falls
-loss of blinking
-dementia (late stage)
-gold standard is neuropathologic exam
-2 of 3 cardinal manifestations must be present
-other confirmation is autopsy
-...heather says some docs give carbadopa/levodopa to see if pts respond.
-Tx of sx
-Tx of complications:
*max out on these drugs before moving on to deep brain stimulation
Tx of Complications:
-Hallucinations: decrease levadopa/dopamine agonists or Zyprexa
-Orthostatic Hypotension: TED hose, slow rising
-Sexual Disturbance: Viagra/Dopamine agonist
-Constipation; stop offending medication, bulking agents, no regalan (dopamine receptor antagonist making their sx worse by blocking dopamine)
-Freezing/DIfficulting initiating: tape doorway, mark floor at turning point
-akinesia/rigidity: exercise and PT
-Dysarthria/hypophonia: Speech Therapy
Is DBS more effective than L-dopa?
No, though DBS is like electrical dopamine, PO Levodopa at highest dosage has better effects than DBS at highest dosage.
Restless Leg Syndrome
-what is this?
-Pt description of sensation
aka: wittmaack-Ekboms Syndrome
What: uncontrollable urges to move limbs in order to stop uncomfortable, painful, or odd sensation in the body, most commonly the legs
-unknown; something in substantia nigra
-reading, plane ride, movie, napping
*may be worse in evening and early in the night
-uncomfortable, antsy, electrical, creeping, painful, itching, pins and needles
-may be associated with?
-Foods (diet soda; aspartame. ETOH)