Final NEURO Degenerative Flashcards
Explain the mechanism of Huntington Disease
Results in altered Huntington protein that damages neurons
What is Huntington disease? Genetic
Inherited disease
Autosomal dominant gene
Carried on Huntington gene (chromosome 4)
When does Huntington manifests?
Does not usually manifest until 3rd to 5th
In Huntington’s Disease there is
Progressive atrophy of brain
Particularly in caudate nucleus
Huntington’s Disease and neurotransmitters
Depletion of GABA in the basal nuclei
Reduced levels of ACh in the brain
How is huntington’s disease diagnosed?
DNA analysis for the protein mutation
Huntington’s disease Cure?
No cure
Supportive treatments
Huntington’s disease Treatment: Medication use?
Tetrabenazine – suppresses hyperkinetic movements (central monoamine depleting agent)
Huntington Disease Anesthesia Considerations: Airway
Dysphagia & ↑ risk of aspiration
Huntington Disease Anesthesia Considerations: Depolarizing agents
Caution with Succinylcholine- ↑ sensitivity
Huntington Disease Anesthesia Considerations: NONDepolarizing agents
↑ ↑ ↑ sensitivity to nondepolarizing muscle relaxants
– titrate carefully
Huntington Disease Anesthesia Considerations: Prepare for
Prepare pt. and family for poss. Post-op. Ventilation
Explain the mechanism of Myasthenia Gravis
Auto-antibodies attack ACh receptors @ NMJ
Skeletal muscle weakness (without atrophy)
Ocular & facial muscles usually affected first
Explain the mechanism of Myasthenia Gravis
Auto-antibodies attack ACh receptors @ NMJ
Skeletal muscle weakness (without atrophy)
Ocular & facial muscles usually affected first
What are the signs and symptoms of Myasthenia Gravis?
PSW CD
Ptosis, diplopia Slurred speech, dysphagia, aspiration Weakness in arms & legs Chronic muscle fatigue Difficulty breathing
DIAGNOSIS Myasthenia Gravis Diagnostic tests:
Electromyography
Serum antibody test
Edrophonium (acetylcholinesterase inhibitor) temp. reversal
Treatment of Myasthenia Gravis : Medications primary
Anticholinesterase agents
Pyridostigmine
Treatment of Myasthenia Gravis : Steroids
Glucocorticoids
Suppress immune system
Myasthenia Gravis: Surgery
Thymectomy (70% of cases have thymus hyperplasia)
Treatment of Myasthenia Gravis: Antibodies
Plasmaphoresis (Removal of antibodies from the blood)
Myasthenia Gravis – Anesthesia : What can aggravate Myasthenia?
Aminoglycosides (aggravate MG)
For Myasthenia, When is Post-op. ventilation required?
DPDV
Disease with duration > 6 yrs.
Presence of COPD
Daily dose of pyridostigmine > 750mg
Vital capacity < 2.9 L
Do all patients with Myasthenia gravis require Post op ventilation?
NO
Myasthenia Gravis – Anesthesia
Pts may be resistant to
succinylcholine
Myasthenia Gravis avoid preop
Avoid opioids pre-op. (respiratory effects)
Myasthenia Gravis and IV anesthetic?
Use short-acting IV anestheic for induction
Consider intubation without muscle relaxants
Myasthenia Gravis and NDNMB
↑ sensitivity to nondepolarizing muscle relaxants – if used, initial dose should be titrated to response according to
peripheral nerve stimulator.
Myasthenia Gravis: Atracurium and vecuronium
potency is increased twofold compared to response in normal pt.
For Myasthenia Gravis, If maintenance requires nondepolarizing drugs,
decrease intial dose by half to two thirds
Your patient has recent changes that include loss of arm swings when walking and absence of head rotation when turning the body. A loss of facial expression, and decreased emotional response is also noted. what would these signs indicate?
Parkinson’s Disease
Loss of facial expression, and decreased emotional response
Parkinson’s Disease
Mechanism of the Disease PARKINSON’S: what is it?
Progressive degeneration of the substantia nigra in the
midbrain
Parkinson’s and location of dopamine production
Substantia nigra normally produces Dopamine which
controls unwanted movements
Parkinson’s and Dopamine
↓ Dopamine leads to unopposed firing of LMNs & tremor
Parkinson’s and CLASSIC Triad of major signs:
Rigidity (first in neck muscles)
Akinesia
Tremor
Treatment of Parkinson’s (DAA SPOT)
Dopamine replacement therapy with Levodopa w/
decarboxylase inhibitor (prevents peripheral conversion)
Anticholinergic drugs
Amantadine
Speech and language pathologist
PT
OT ( Improve balance, coordination)
What is the role of Decarboxylate inhibitor in Parkinsons?
decarboxylase inhibitor (prevents peripheral conversion)
Medication management for the patient with Parkinson’s undergoing surgery? When should it be administered?
Parkinson Disease – Anesthesia Mgmt.
Maintain Levodopa peri-op.
- Oral levodopa can be administered ~20 min. before inducing anesthesia
Anesthesia considerations with Parkinson’s Interruption of levodopa?
Interruption of therapy can lead to muscle rigidity interfering with lung ventilation
Important anesthesia considerations for Parkinson’s
What medication to avoid?
Avoid Butyrophenones - antagonize effects of dopamine
ex. droperidol, haloperidol
Hemodynamics instability for the patient with parkinson’s disease ?
Hypotension & cardiac arrhythmias possible during
anesthesia
Your patient is a 27 year old who states that she has been experiencing, numbness, tingling, and burning that started in legs and later included her arms. She has also begun to notice some loss of coordination and vision disturbances. Her MRI Appears below? What does it indicate?
Multiple Sclerosis
Tests that can be ordered to diagnosis MS? (MECE)
MRI for diagnosis and monitoring
Evoked potential tests (VER)
CSF (antibodies, abnormal cells)
EEG if seizures present
Seizures and Multiple Sclerosis
Increase incidence of Seizures in MS patiens
Explain the mechanism of Multiple Sclerosis : Progressive
Progressive autoimmune demyelination of CNS neurons &
Cranial Nerves
Explain the mechanism of Multiple Sclerosis loss of
Loss of myelin interferes with conduction of impulses
Affects motor, sensory, & autonomic fibers
Occurs in diffuse patches in the nervous system
Course of MS
Course variable: subacute w/ relapse and remission or
chronic and aggressive
MS Cure? and what is the role of treatment options
No cure. Treatments control symptoms and slow progression.
MS medications for treatments?
Corticosteroids
Interferon β
Low-dose Methrotrexate
PT, OT
MS Anesthesia Considerations:and the Use of SPINAL ANESTHESIA? which one do not cause that effect?
Spinal anesthesia known to cause Post-op. exacerbations
Epidural and peripheral blocks usually do not
MS and GA
General anesthesia usually tolerated well
MS and SUCC
Avoid succinylcholine - ↑ risk of hyperkalemia
MS and Steroids
Continue steroids peri-op.
Amyotrophic Lateral Sclerosis (ALS) - AKA
Lou Gehrig disease
ALS cause and genetic
No identified cause
Genes on various chromosomes have been linked to the
disease
ALS Mechanism of disease
Progressive degenerative disease affecting upper motor
neurons in the cerebral cortex and lower motor neurons in
brainstem and spinal cord (at anterior horns)
Inflammation and cognition in ALS
No indication of inflammation around the nerves
Cognition unimpaired
Amyotrophic Lateral Sclerosis (ALS) Anesthesia Considerations: GETA
General anesthesia associated with exaggerated ventilatory depression
ALS and SUCC
Avoid Succinylcholine (↑ risk of hyperkalemia)
ALS and NDNMB –> There is
prolonged response to nondepolarizing muscle relaxants
ALS and Regional: and why?
Avoid regional anesthesia – exacerbates disease symptoms
ALS and EPIDURAL
Epidural anesthesia ok
Causes of Alzheimer’s : Progressive
Progressive cortical atrophy
Pathophysiology of Alzheimer’s BCD
Beta-amyloid plaques & neurofibrillary tangles form destroy neuron microtubules
Disrupt neurotransmitter function
↓ choline acetyltransferase = ↓ ACh levels
Most common neurodegenerative disease
Alzheimer’s Disease
Alzheimer’s is responsible for
Responsible for 40-80% of all cases of dementia
Affected with Alzheimer’s
Affects ~20% of pts. over 80
Alzheimer’s Specific cause
unknown
Alzheimers cause unknown but repetitive
Repetitive DNA sequences on different chromosomes have been associated with AD
ALzheimer’s and diagnosis (PCP)
PET scan exhibits areas of ↓ cerebral blood flow
CT demonstrates ventricular dilation & cortical atrophy
Postmortem examination of brain tissue
Alzheimer’s detection other tests
Peanutbutter detection Test
Explain why cholinesterase inhibitors are used to treat Alzheimer’s Disease?
Because there is decrease ACH in Alzheimer’s. Cholinesterease break down ACH. Cholinesterase inhibitors prevent further breakdown of ACh
Why is glycopyrrolate a preferred anticholinergic for AD patients?
It does not cross BBB
AD – Anesthesia Considerations
Preoperative sedation
usually avoided - may aggravate dementia & precipitate postop. confusion.
AD Pts. Taking cholinesterase inhibitors and SUCC
may have prolonged duration of action with succinylcholine.
AD and NDNMB
Resistance to nondepolarizing muscle relaxants with
acetylcholinesterase inhibitors.
AD is preferred anticholinergic - doesn’t cross
BBB.
Glycopyrrolate
Atropine and scopolamine
Atropine & scopolamine may cause increased confusion
Your patient has a distorted sense of reality. See CT , what are the abnormalities seen? What do they indicate? Common changes include Schizophrenia are REEAD
–Reduced gray matter in temporal lobes
–Enlarged third and lateral ventricles
– Excessive dopamine secretion
Indicate Schizophrenia
Schizophrenia has both
positive and negative symptoms.
Schizophrenia Positive symptoms
exaggeration of normal behavior with hallucinations & delusions
Schizophrenia Negative symptoms
Reflect decline in normal function with apathy, flattened affect
changes in hygiene & appearance, and occupational or social withdrawal.
Schizophrenia Tx of positive symptoms
Dopamine receptor blockers (D2 & D4)
Improve the ‘positive’ symptoms
Schizophrenia Tx of negative symptoms
“Atypical” serotonin receptor blockers (5-HT2A)
Improve the ‘negative’ symptoms
Schizophrenia – Anesthesia Mgmt.
–Be mindful of the effects of antipsychotic medications
->Prolonged QT interval can lead to torsades de pointes
–sedation that may lessen anesthetic requirements
– seizures
Schizophrenia – anesthesia Mgmt. Hepatic enzymes and hypotension?
↑ hepatic enzymes
postural hypotension caused by alpha adrenergic
blockade
What is ECT used for ?
ECT - Electroconvulsive therapy may be used in severe
depression unresponsive to medications.
Used of ECT include
For treatment of medically resistant depression, schizophrenia or bipolar disorder w/ suicidal tendencies
ECT is Performed
under anesthesia
During ECT , small
Small electric currents used to induce brief small seizures
ECT does what?
Effectively reverses certain mental illnesses
ECT CV effects
10-15 second stimulation of the parasympathetic nervous
system produces bradycardia & hypotension followed by hypertension and tachycardia that lasts for
several minutes as a result of sympathetic nervous system
activation.
Other side effects of ECT
Other side effects: ↑ intragastric pressure ↑ ICP ↑ IOP ↑ cerebral blood flow Memory impairment (most common long-term effect)
Other side effects of ECT
↑ intragastric pressure ↑ ICP ↑ IOP ↑ cerebral blood flow Memory impairment (most common long-term effect)
2 most common causes of death related to ECT:
Cardiac dysrhythmias & MI
ECT – Anesthesia Mgmt.
Glycopyrrolate
IV 1-2 min. before induction decreases excess salivation & bradycardia
ECT – Anesthesia Mgmt. Beta Blocker
Esmolol 1 mg/kg IV just prior to induction can attenuate
tachycardia & HTN
ECT – Anesthesia Mgmt. HTN
HTN can be ameliorated w/ nitroglycerine
ECT and Methohexital
Methohexital (0.5-1.0 mg/kg IV) is standard for induction
Has rapid onset & recovery
Short duration of action and minimal anticonvulsant effects
ECT beside methohexital
Propofol is alternative but has anticonvulsant effect
ECT – Anesthesia Mgmt. Succinylcholine and dose
Succinylcholine IV promptly after induction reduces risk of potentially dangerous muscle contractions/bone fxs
Dose of 0.3 to 0.5 mg/kg IV sufficient to attenuate contractions but still permit visual confirmation of seizure activity
ECT and anesthesia mgmt. EEG
most reliable way to confirm seizure
ECT and anesthesia mgmt: Use of Tourniquet
Alternatively, a tourniquet can be applied to a limb
before administration of Succ. – subsequent tonic/clonic
movement of limb provides evidence that seizure has
occurred.
ECT and Anesthesia Management: Pacemakers and defibrillators
Pacemakers/defibrillators are shielded and not affected
by ECT, however – external magnet should be available
Ensure capability of converting pacemakers to asynchronous modes if malfunction does occur
ECT and Cardioverter/defibrillators
should be turned off prior to ECT and reactivated afterward
ECT Methods to monitor Pacemaker function PPPE
External MAGNET SHOULD BE AVAILABLE
What is Neuroleptic Malignant Syndrome?
.
A rare complication of antipsychotic drug treatment that is
potentially fatal and is hypothesized to be caused by
central nervous system depletion of dopamine.
Neuroleptic Malignant Syndrome usually occurs ?
Usually occurs within the first few weeks of treatment, or
when there is an increase in the dose of the drug
Causes of death in NMS: CCRH
–CHF
–RF
–cardiac dysrhythmias
–hypoventilation
Neuroleptic Malignant Syndrome Signs/Symptoms: (SHRD)
Severe muscle rigidity (may require mechanical ventilation)
Hyperpyrexia (pts. have ↑ susceptibility to developing MH)
Rhabdomyolysis
Dehydration & myoglobinuria (can lead to renal failure)
Shy-Drager Syndrome aka
Multiple System Atrophy (MSA) a.k.a. “Shy-Drager Syndrome”
Shy-Drager Syndrome Develops in
50s
Shy-Drager Syndrome there is
Chronic degeneration of the ANS w/ ↓ Norepi levels
Characterized by severe orthostatic hypotension & urinary
involvement (urgency/incontinence)
Multiple System Atrophy (MSA) or SHY DRAGER – Anesthesia Considerations- ANS and CV
↓ ANS / Cardiovascular responses with change in body
position, positive airway pressure & acute blood loss
Avoid what drugs with Multiple System Atrophy (MSA) aka. Shy DRAGER ; monitor what?
Avoid drugs with negative inotropic effects
Closely monitor systemic BP
BP correction for Multiple System Atrophy (MSA) Aka shy drager
Prompt correction of hypotension w/ crystalloid or colloid
Phenylephrine is preferred direct-acting vasopressor
Administer small doses initially
Alpha receptors and MSA:
α-receptors are up-regulated in MSA 2o to ANS chronic
denervation and produce exaggerated response to small
doses of the drug
MSA aka SHY drager
Continuous infusion of phenylephrine may be used to maintain BP during GA
MSA aka SHY DRAGER – Anesthesia Considerations
Volatile anesthetics
can diminish C.O. & contractility leading to exaggerated hypotension
Absent with MSA
Compensatory vasoconstrictive/tachycardic responses absent
MSA aka shy drager, treat with bradycardia
Atropine or glycopyrrolate to treat bradycardia
MSa aka shy drager “ NDNMB
Vecuronium preferred muscle relaxant
Has less effect on systemic circ.
MSA aka Shy drager: Adjust
propofol or thiopental dose & rate according to pt’s
↓ compensatory responses
Risk factors for Parkinson’s (AGE)
Risk Factors:
Age
Genetic associations
Exposure to manganese in welders has been identified
ALS loss of UMN leads to (US ) “ think US”
Loss of upper motor neurons
Spastic paralysis & hyperreflexia
Decreased muscle tone and reflexes Progressive muscle weakness and loss of fine motor coordination Stumbling and falls are common. Death occurs due to respiratory failure
ALS loss of LMN leads to (FL) “ think FL”
Flaccid paralysis (decrease muscle tone and reflexes) Damage to Lower motor neurons
ALS there is
Progressive muscle weakness and loss of fine
motor coordination
Stumbling and falls are common.
ALS death occurs due to
Death occurs due to respiratory failure.
ALS and Autonomic NS
Autonomic dysfunction : Orthostatic hypotension and Resting tachycardia
ALS sx where first
Skeletal muscle weakness w/ fasciulations
Intrinsic muscles of the Hands first
Myasthenia Gravis affected first
Ocular & facial muscles usually affected first
Myasthenia Gravis population affected
Women 20 – 30 yrs. most often
Men > 60 yrs.
What are the 4 subtypes of schizophrenia (DCUP)
Disorganized
Catatonic
Undifferentiated
Paranoid
Depression
Most common psychiatric disorder
characteristics associated with major depression:
Depression : Suicide rate
15% suicide rate among pts. with major depression
Depression Unipolar
Diagnosis based on biologic factors or personal characteristics
Depression Bipolar
Alternating periods of depression and manic episodes
Depression distinction ?
length and degree of the disturbances in mood
distinguish depression from ordinary grief and sadness
Treatments: Psychotherapy Antidepressant drugs that:
increase norepinephrine activity
Antidepressant medications:
SSRIs (ex. zoloft, prozac)
SNRIs (ex. cymbalta, effexor)
Tricyclics (ex. amitriptyline, nortriptyline)
MAOIs
Spondylosis aka
Spinal Stenosis
Transverse osteophytes compress cord in canal and/or
nerve roots at intervertebral foramina.
Ankylosing Spondylitis and HLA
90% of pts. are HLA-B27 positive
Ankylosing Spondylitis Causes:
Decreased mobility of vertebral column
Ankylosing Spondylitis And systemic manifestation : EYE
Conjunctivitis/Uveitis
Unilateral w/ visual impairment, photophobia & eye pain
Ankylosing Spondylitis And systemic manifestation: LUNGS
Apical cavitary lesions with fibrosis & pleural thickening in the lung (mimics TB)
Ankylosing Spondylitis And systemic manifestation: SPINE and RESP
T-spine involvement = ↓ chest wall compliance and
↓ vital capacity
Ankylosing Spondylitis And systemic manifestation: CV
Aortic regurgitation from fibrosis may also be present
Ankylosing Spondylitis Anesthesia Considerations Stiffened/deformed C-spine may cause_____
difficult intubation- Awake fiberoptic intubation may be neede
Ankylosing Spondylitis Restrictive lung disease results in
↓ vital capacity
Ankylosing Spondylitis Anesthesia Considerations : SVR
Sudden increases in systemic vascular resistance poorly
tolerated if aortic regurgitation present
Ankylosing Spondylitis Anesthesia Considerations : Neuraxial
Regional anesthesia may be difficult 2o to limited jt. mobility
Epidural or spinal anesthesia is acceptable alternative to
general
