Test #1 Flashcards
Huntington’s Disease
Cerebral and caudate nucleus atrophy -> GABA and acetylcholine deficiency
Chronic, progressive chorea w/ impulsive and antisocial behavior from dopamine surplus
Dx: MRI (caudate atrophy), PET (caudate metabolic abnormalities), Genetic testing - gold standard
Huntington’s Disease Treatment
Goal: Downregulate dopamine, suppress chorea
Neuroleptic and Tetrabenazine to suppress and breakdown Dopamine
Anticonvulsants: Clonazepam, Valproic Acid
Antipsychotic: Risperidone, Olanzapine
Antidepressants: Fluoxetine, Sertraline, Nortriptyline (TCA)
Huntington’s Treatment Side Effects
Hyper-excitability, fatigue, restlessness
Antipsychotic SE mimic signs of Parkinson’s - dull facies, tardive dyskinesia
Essential Tremor
Most common tremor cause - inherited
Bilateral, occurs w/ action, constant frequency w/ variable amplitude
Have to r/o Parkinson’s - should be only abnormal thing on exam
Relieved by ETOH
Essential Tremor Treatment
Propanolol - 1st line (Atenolol w/ asthma/bronchospasm)
Mysoline/Gabapentin - anticonvulsants
Parkinson’s Disease
Progressive neurodegenerative disorder -> substantia nigra breakdown causes dopamine deficiency
TRAP, fixed facial expressions, Myerson’s sign (repeated tapping of the nose causes blinking), Lewy bodies
Onset usually after 50 years old
Tremor @ rest, disappears during sleep, cog-wheel/rachet-like motions
TRAP
Tremor - resting and postural = unilateral, @ rest
Rigidity = increased resistance to passive movement, unilateral -> bilateral
Akinesia (Bradykinesia) = difficulty/slow initiation movements, get “frozen or stuck” - huge fall risk
Postural instability = late stage, lean forward w/ shuffling gait, prone to falling backwards
- All DTRs intact w/ no weakness
Parkinson’s Therapeutic Treatment
Depression: SSRI
Hallucination: Decrease Sinemet, Zyprexa
Orthostatic HOTN: TED hose, slow rising
Sexual dysfunction: Viagra, Dopamine agonist
Constipation: Cease causative medication; Reglan is CI - Dopamine antagonist
Deep Brain Stimulation
No more effective than highest med dose, no SE
Use in pts w/ drug-induced dyskinesias who lack any complicating med/psych conditions
Pulse generator at the STN and Thalamus - replace every 5 years
Restless Leg Syndrome/Wittmaack-Ekbon’s Syndrome
Uncontrollable urge to move limb to stop uncomfortable/painful/odd sensation - mot common in legs
Often have varicose veins, less common among Asian pop
Always get a CBC to r/o iron deficiency anemia
Causative agents of RLS
Meds: Anti-nausea, H2 Blocker, antihistamines, SSRI/anti-psych
Food: Diet soda/aspartame, ETOH
RLS Treatment
Tx underlying cause, OTC Ibuprofen, baths/massages, warm/cool packs
Pramipexole, Ropinirole, Sinemet, Lyrica (w/ Parkinson’s)
Gabapentin, Opioids
Parkinson’s Treatment
GOAL: Restore dopamine activity, manage SE of therapy
Selegiline (MAO-B) may slow progression w/ early therapy
All other meds replenish Dopamine or block Acetylcholine/GABA
Apomorphine (NMDA) is used only for emergent freezing instances
Levodopa/Carbidopa (Sinemet)
Gold standard, generally 1st line (>70 yo, dementia)
Levodopa = dopamine precursor, Carbidopa = prevent peripheral breakdown
Best for rigidity and slowness, less for tremor/balance/gait
Short acting, high doses cause dyskinesias
Sinemet side effects and contraindications
SE: Vivid dreams, hallucinations, HOTN, Dyskinesia
Wearing off effect - after 4-6 yrs, gets progressively worse
- initial bradykinesia, tremor before next dose
CI: MAOI, psychotics, angle-closure glaucoma, history of melanoma
Use caution w/ cardiac dx, PUD
Monoamine Oxidase-B Inhibitors (MAO-B)
Selegiline/Rasagiline = stop dopamine breakdown, penetrate BBB
May slow progression if given @ early onset in young pt
1st line for mild dx, also to decrease Sinemet wearing off effect
MAO-B side effects and contraindications
SE: Insomnia, Jitteriness, Dyskinesias, Increases Sinemet SE
CI: TCA, SSRI, Demerol
Caution: Liver impairment, cardio/CV dx, seizure, hypothyroidism, DM, psych disorders
Dopamine Agonists
Older, Ergo derivatives: Bromocriptine
Newer, synthetic: Pramipexole, Ropinirole
Stimulate dopamine receptors in substantia nigra
- Improve akinesia, postural instability
1st line in young pts w/ moderate symptoms
Dopamine agonist side effects and contraindications
SE: poorly tolerated - drowsiness/sleepiness, HA, constipation, nightmare/psychosis/dyskinesias
CI: psychotic illnesses, recent MI, PUD
Avoid ergo derivatives in pts w/ PVD
Apomorphine (Apokyn)
Emergent only, NMDA agent
Treats episodes of freezing/hypermobility
Give SQ, expensive
SE: N/V, yawning, dyskinesia, sedation, dizziness
- give w/ antiemetic that is not Zofran/Kytril
Catechol-O-Methyltransferase Inhibitors (COMT-I)
Entacapone (Comtan, Tolcapone (Tasmar)
Inhibit enzyme that metabolizes levodopa in periphery
Only use w/ Sinemet - improves wearing off effect
SE: Happen immediately, poorly tolerated - dyskinesias, confusion/hallucinations, urine discoloration, cramps, N/D, HA, insomnia
Amantidine
Antiviral, MOA unknown, Adjunct only
Use for early mild sx, short-lived
No effect on tremor
SE: sedation, vivid dreams, dry mouth, depression
Caution w/ renal dysfunction
Anticholinergic Acetylcholine-blocking drugs
Trihexyphenidyl, Benzotropine
Target Acetylcholine to prevent dopamine inhibition
Primarily for tremor, helps w/ rigidity - no effect on akinesias
SE: CNS and systemic, SE usually outweigh any benefit
- CV, IOP, AMS
CI: BPH (causes retention), obstructive GI, angle-closure glaucoma
COMT uses
Try to improve on-off syndrome
Take off if not effect in a few weeks due to SE
Epidural hematoma
Most commonly skull fx - high force trauma
Arterial blood from venous sinus or dural artery
Dyperdense biconcave, respects suture lines
Acute presentation
Subdural Hematoma
Venous blood - from venous plexus
Cresent shaped, doesn’t respect suture lines
Low force trauma
Insidious presentation - worsening HA over days
Subarachnoid Hemorrhage
Below arachnoid/Within the brain
Arterial blood from circle of Willis - aneurysm rupture or high-force trauma
Acute presentation - thunderclap HA
Glasgow Coma Scale components
Eye Opening - 4 points
Best Verbal Response - 5 points
Best Motor Response - 6 points
GCS 13-15 = Mild TBI
GCS 9-12 = Moderate TBI
GCS <8 = Severe TBI
GCS 3 = Totally unresponsive, lowest possible score
GCS Eye opening
Spontaneous = 4
Response to verbal command = 3
Response to pain = 2
No eye opening = 1
GCS best verbal response
Oriented = 5
Confused = 4
Inappropriate words = 3
Incomprehensible sounds = 2
No verbal response = 1
GCS best motor response
Obeys commands = 6
Localizing response to pain = 5
Withdrawal response to pain = 4
Flexion to pain = 3
Extension to pain = 2
No motor response = 1
Seizure and Types
Sudden, excessive disorderly discharge of neuronal activity in brain
- Sudden, transient
- Motor/sensory/autonomic/psychic manifestations
- Temporary alteration of systemic arousal
- Often manifests as convulsions; different kinds of seizures
Seizure disorder/Epilepsy
Recurrent seizures without any immediate treatable cause such as hypoglycemia or ETOH withdrawl
Convulsion
Rapid contraction and release of muscle causing an uncontrollable shake
Epilepsy
Occurrence and reoccurrence of seizures without know or correctable cause
Can get strange sensations/emotions/behavior -> convulsions, spasms, LOC
Idiopathic or primary generalized epilepsy usually present by puberty
Seizures after 20 yo -> usually focal process/metabolic derangement
Cancer, stroke, degenerative brain disorders cause seizures later in life
Epilepsy Pathophysiology
A. Complex gene mutations or environmental factors = abnormal connections
B. Hypersensitive neurons have sudden, violent depolarizations
-Temperature, electrolyte imbalances set off
C. Epilectogenic neurons fire more often and intensly w/ greater amplitude than normal
Provoked vs Unprovoked Seizures
Provoked = triggered by provoking factors in healthy brain
- metabolic, ETOH, drugs, high fever
Unprovoked = occur with persistent brain pathology
-repeat seizures may be similar or different, get a complete H&P after initial seizure to r/o other conditions
Sudden unexpected death (SUDEP)
Sudden, nontraumatic, nondrowning death of a person w/ epilepsy
Happens in refractory epilepsy or poorly controlled seizure disorder
Seizure phases
Prodrome (1st phase) = hours/days before seizure
-deja vu, smell/sounds/taste, fear, dizzy, HA, nausea
Aura = 1st seizure sx, beginning of seizure
Middle “ictal phase” = from Aura to end of seizure
- awareness lost, confused/daydreaming, can’t talk/swallow
Ending “postictal” = recovery can be immediate or takes minutes/hours
-sleepy, slow to respond, confused, HA, nausea
Loss of consciousness
Complete/partial unawareness or lack of response to sensory stimuli
Induced by hypoxia, metabolic or chemical depressants, brain pathology, trauma
Focal Seizures
Occur on one side of the brain
Occur w/ and w/out consciousness impairment
w/out: Jacksonian March (motor), Todd’s Paralysis, sensory, autonomic, psychic
w/: pt unresponsive, may evolve from w/out
- most common, arise from temporal lobe - pt appears to be “daydreaming”
- 30 seconds to 1 minutes w/ confusion and tired 15 min postictal
Jacksonian March
Motor focal seizure w/out consciousness impairment
Starts at fingers and works its way up limb
Abnormal activity in primary motor cortex
Also get sudden head & eye movement, tingling, numbness, lip smacking, and muscle spasms
Todd’s Paralysis
Focal seizure w/out impaired consciousness - Postictal state
Temporary (30 min to 36 hours), unilateral paralysis of limb
Have to r/o stroke
Generalized Onset Seizures
Disturbed consciousness, bilateral cerebral cortex malfunction
Absence, Atypical, Myoclonic, Atonic, Febrile, Tonic-Clonic, and Secondary seizure types
Can develop from focal seizures
Absence seizure
“petit mal seizure”
Looks like daydreaming, disturbed consciousness w/o convulsions
Typically in childhood
Abrupt onset and termination w/ brief impairment ~10 seconds
May have mild tonic-clonic, or atonic components; no postictal
Atypical Absence Seizures
Lapse in awareness w/ gradual onset and resolve
Autonomic features and muscle tone lost
Often occurs in mentally impaired kids
Doesn’t respond well to AED therapy