Neuro: Disorders of Motor Function Flashcards
Parkinson’s Disease About + Etiology
Idiopathic, chronic, progressive degenerative disorder of CNS
motor, neuropsychiatric manifestations
50+ in age
2nd most common neurodegenerative disorder
Parkinson’s s/s
tremors bradykinesia (chew, swallow, speak) muscle rigidity postural instability flat affect
Conditions Causing Secondary Parkinsonism
head trauma toxins metabolic disorders infections multiple strokes in basal ganglia atypical antipsychotics&antiemetics
Parkinson’s Causes
90% Idiopathic
10% Genetic
Increased RF for Parkinson’s
increased age (most significant) male exposure to pesticides, metals family history of PD genetic mutations
Decreased RF for Parkinson’s
cigarette smoking
caffeine intake
high blood urate levels
Parkinson’s Pathogenic Mechanisms
proteolytic stress (too many proteins, Lewy bodies) oxidative stress (too little gluutathione, increase in iron, reactiev oxygen species) mitochnodrial dysfunction (decreased mitochondrial activity by 30-40%) inflammation (overactive microglia, excess production of neurotoxic factors)
Parkinson’s Manifestations
insidious onset
Motor: tremor in resting hands/lips/chin/legs/mouth, regidity from increased muscle stiffness/tone, cogwheeling, bradykinesia, postural instability
Nonmotor: fatigue/sleep disturbances, olfactory dysfunction, pain, autonomic dysfunction
Neuropsychiatric: cognitive dysfunction, dementia, psychosis, hallucinations, mood disorders, depression, anxiety
Parkinson Disease Stages
Stage 1 (mild s/s on one side only, changes in posture/locomotion/facial expression) Stage 2 (bilateral symptoms/minimal disability, posture&gait effected) Stage 3 (significant slowing of body movements, early impairment of equilibrium on walking/standing, generalized dysfunction moderately severe) Stage 4 (severe symptoms, rigidity & bradykinesia, can still walk to a limited extent, unable to live alone, tremor may be less than in earlier stages) Stage 5 (cachectic stage, invalidism, cannot stand or walk, requires constant care)
Parkinson’s TX
pharmacologic replenishment w/ dopaminergic drugs dopamine agonists anticholinergic agents amantadine monoamine oxidase inhibitors catecol-o-methyltransferase inhibitors ablation surgery deep brain stimulation complementary & supportive therapies
Dopamine Agonists (ex/Moa/Se/Monitoring/Teaching/Preg)
levodopa/carbidopa
increases biosynthesis of dopamine in nerve terminals
uncontrolled/purposeless movements, involuntary movements, loss of appetite, n/v
monitor LFTs, contraindicated in narrow-angle glaucoma
discontinue gradually
C
Anticholinergic Agents (Moa/ex/Use/Se/monitoring/teaching/Preg)
block acetylcholine to inhibit overactivity
benztropine
tx early stages of Parkinson’s
dry mouth, blurred vision, photophobia, urinary retention, constipation, tachycardia, glaucoma
watch liver disease, severe constipation, blockage of urinary tract
monitor for hypotension/tachycardia
avoid alcohol
C
Multiple Sclerosis
chronic inflammatory demyelinating degenerative disorder of CNS inflammatory autoimmune diease lesions throughout CNS progressive disease unknown/no cure
MS RF
age gender ethnicity geography r/t latitude genetics
MS Pathogenesis
development of plaques
demyelination which causes conduction blocks and axonal degeneration
atrophy of gray matter (r/t cognition)
MS Development
initial: clinically isolated syndrome
Relapsing-remitting multiple sclerosis (RRMS) [stable for 10-20 years]
Secondary Progressive MS (SPMS) [gradual worsening may have occasional relapses, minor remissions, plateaus]
Primary Progressive MS (PPMS) [progressive deficits w/ occasional plateaus, temp improvements or acute relapses]
MS s/s
sensory symptoms in extremities/face visual loss double vision slurred speech weakness vertigo gait/balance disturbances bowl&bladder problems neuropathic pain spasticity bilateral internuclear opthalmoplegia fatigue cognitive dysfunction heat sensitivity sexual dysfunction
MS DX
CNS lesion disseminated in time & space
MRI (McDonald criteria/plaques present)
Blood tests to rule out other disorders
evoked potentials recording timing of CNS response to stimuli
EEG
lumbar puncture
MS TX
immunomodulatory therapy amantadine/modafinil for fatigue spasticity physical therapy routine stretching program medications intramuscular botulinum toxin nerve blocks intrathecal baclofen pump placement
Immune-modulating Drugs (Moa/use/SE/monitoring/teaching/preg)
uknonwn, interferes with cell-binding
reduces severity of MS symptoms, decreases lesions
flushing, chest pain, weakness, infection, pain, nausea, joint pain, anxiety, muscle stiffness
liver fx, depression, suicidal ideation
rotation of injection sites/use new needles
C
Amyotrophic Lateral Sclerosis
Lou Gehrig’s Disease
progressive neurodegenerative disease
causes weakness/disability/death w/in 3-5 years
Amyotrophic Lateral Sclerosis RF/Etiology
age/family history
no direct mechanism known
similar to prion disease/malignancy
motor axons die by wallerian degneration
Amyotrophic Lateral Sclerosis Pathways
oxidative stress mitochonodrial dysfunction defect of axonal transport abnormal growth factor expression excitotoxicity
Amyotrophic Lateral Sclerosis s/s
insidious onset slowly progressive, painless weakness in one or more body parts upper motor neuron s/s lower motor neuron s/s bulbar dysfunction (CN 9/10/11/12) frontotemporal executive dysfunction
Amyotrophic Lateral Sclerosis DX
based primarily on s/s electromyography nerve conduction studies MRI/CT of brain & spinal cord muscle or nerve biopsy genetic blood tests world federation of neurology diagnostic algorithm
Amyotrophic Lateral Sclerosis survival rate/TX
3 years
riluzole
noninvasive ventilation
percutaneous endoscopic gastrostomy tube feeding
meds to relieve symptoms/maximinze remaining function
multidisciplinary approach to care
Amyotrophic Lateral Sclerosis Stages
1 (symptom onset involvement of first body region)
2A (diagnosis 35% thru)
2B (Involvement of second body region 38% thru)
3 (involvement of thirst body region 61% thru)
4A (Need for gastrostomy/feeding tube 77% thru)
4B (Need for noninvasive ventilation 80% thru)
Huntington Disease
progressive, incurable neurodegenerative disease of brain
causes uncontrolled involuntary movements, dementia, behavior changes
onset ages 35-44
primary causes of death (19 years after diagnosis) pneumonia&cardiovascular disease
Huntington Disease Etiology/Pathogenesis
autosomal dominant inheritanges (HTT) chromosome 4
leads to neuronal cel death
excitotoxicity oxidative stress impaired metabolism opoptosis
Huntington Disease Pathology
gross atrophy in caudate nucleus & putamen w/ selective neuronal loss & gliosis
neuronal loss in cerebral cortex
varying degrees of atrophy in other areas in midbrain & cerebellum
shrinkage of brain in olum
numerous biochemical defects
Huntington Disease Severity
Grade 0: no detectable histologic neuropathology/no gross striatal atrophy
Grade 1: neuropathologic changes detected microscopically by w/out gross atrophy
Grade 2: striatal atrophy present, caudate nucleus remains convex
Grade 3: striatal atrophy more severe, caudate nucleus flat
Grade 4: striatal atrophy most severe, medial surface of caudate nucleus concave
Huntington’s Manifestations
involuntary movements chorea parkinsonian features akinetic-rigid syndrome dysarthria dysphagia abnormal eye movements tics myoclonus short-erm memory loss impaired intellectual function dementia psychiatric manifestations irritability moodiness depression OCD untidiness antisocial apathy
Huntington Disease DX
genetically proven family history
clinical presentation
MRI/CT to measure brain atrophy
referral to neurologist who specializes in HD
Huntington Disease TX
reduce symptoms & improve quality of life
tetrabenazine for chorea
antidepressant or antipsychotic medications
levodopa/dopamine agonist
Seizure Activity Definition
abnormal/uncontrolled electrical discharges within brain
epilepsy
seizures occurring chronically
Seizure Diagnosis
lab tests (cbc, bmp, cmp, urine culture, lumbar puncture) EEG Lead level, toxicology screening CT san MRI w/ angiography
Seizures Infant Causes
congenital anomoloies
perinatal brain injury
metabolic imbalances
Seizures Late Childhood
CNS infections
neuro degenerative disorders
inherited epilepsies
Seizures Adult Causes
trauma
cerebrovascular disorders
neoplastic disease
Seizure s/s
loss of conscious awareness of environment
varying patterns of muscular rigidity/relaxation
aura
Status epilepticus
life-threatening
enhanced/sustained electrical activity over 30 minutes or more
Febrile Seizures
acute illness, rapid temperature rise above 102.2
tonic-clonic activity (1-2 min)
rapid return to conciousness
Partial Seizures (Focal Seizures)
contained w/in limited area of brain
simple: hallucinations/intense emotions/twitching
complex: typically w/ aura, postictal confsion
Intractable Seizures
refractory (can happen even w/ optimal medical management)
require referral to epilepsy center
Absence (petit mal)
seizures last few seconds most often in children
s/s: stare into space, no response to verbal, fluttering eyelids, jerking
Tonic-clonic (grand mal)
aura, tonic (intense muscle contractions) alternate with clonic (relaxation muscles) postictal state (disoriented & deep sleep)
Tonic Phase
15-60 seconds muscle rigidity sudden loss of consciousness pupils fixed/dilated increased metabolic demans hypoxia urinary/bowel incontinence
Clonic Phase
60-90 seconds
alternating muscle contraction/relaxation in extremities
hyperventilation
eyes roll back/forth at mouth
Postictal Period
decreased level of consciousness/sleepy
quiet/relaxed breathing
gradual regaining of consciousness
Seizure Meds & Contraceptives
decrease efficacy of oral contraceptives
Eclampsia
severe HTN disorder of pregnancy
characterized y seizures/coma/perinatal mortality
Seizure Medications
initial low dosing gradually increased newer meds have fewer side effects may double risk of suicidal behavior GABA-potentiating drugs
Barbituarates
controll seizures
SE: dependence drowsiness, vitamin deficiencies, laryngospasm
monitor liver/kidney function
report pregnancy immediately
at risk for bleeding, drowsiness, bone pain
Preg Cat D
Phenobarbital (class/use/admin/SE/Contra/interx)
Long acting Barbiturate
used for management of seizures
IM may cause local inflammatory response
IV (rarely used) can cause tissue necrosis
controlled substance (can cause dependence)
drowsiness
vitamin deficiencies
OVERDOSE: respiratory depression, CNS depression, coma, death
Severe uncontrolled pain, pre-existing cNS depression, glaucoma, prostatic hypertropy
don’t mix w/ alcohol/CNS depressants
may increase metabolism of other medications reducing effectiveness
Benzodiazepines (use/se/monitoring/teaching/overdose/preg)
short-term seizure control similar to barbiturates but safer drowsiness/dizziness drug-abuse potential contra for narrow-angle glaucoma respiratory depression w/ other CNS depressants rebound seizures if discontinued abruptly D
Flumazenil
antidote to benzodiazepines
not usually given if taking benzos for a reason chronically
Diazepam (class/admin/se/contra/interx)
anti-seizure drug
benzo
oral better, if IV (assess RR Q5-15 min, have airway equipment ready)
IV: hypotension, muscular weakness, tachycardia, resp depression
INJ: avoid in shock/coma/depressed vital signs/newborns
PO: avoid in infants/narrow-angle glaucoma/within 14 days of monoamine oxidase inhibitor therapy
don’t combine w/ alcohol
levodopa/barbiturates decrease efficacy
if taken with phenytonin increases toxicity
Hydantoin Meds (use/se/monitoring/teaching/preg)
desensitize sodium channels tx epilepsy except absence seizures CNS depression, gingival hyperplasia (excessive gum growth) skin rash dysrhthmias hypotension fatal hepatoxicity serum drug levels, signs of toxicity, blood dyscrasias, bleeding disorders, liver/kidney fx need routine labs report hypoglycemia/pregnancy D
Phenytoin (class/use/se/bbw/contra/inx)
hydantoin antiseizure can cause dysrhythmias bradycardia ventricular fibrillation severe hypotension hyperglycemia headache nystagmus ataxia confusion slurred speech nervousness twitching insomnia peripheral neuropathy (long-term) agranulocytosis rashes exfoliative dermatitis SJS connective tissue reactions watch cardiac monitoring during/after IV rash/sinus/bradycardia/heart block w/ tricyclic antidepressants = seizure
Valproates (class/use/admin/se/bbw/contra/interx)
phenytoin-like drug antiseizure don't crush/chew ER sedation/drowsiness GI upset prolonged bleeding time visual disturbances muscle weakness tremor psychomotor agitation BMS weight gain abdominal cramps rash alopecia pruritus photosensitivity erythema multiforme fatal hepatoxicity pancreatitis/tertogenic effects liver disease/bleeding dysfunction/pancreatitis/congenital metabolic disorders don't give w/ warfarin (bleeding) or alcohol increases w/ phenobarbital
Succinimides (use/se/monitoring/teaching/preg)
tx absence seizures impaired mental/physical abilities psychosis/extreme mood swings dizziness HA lethargy fatigue ataxia sleep disturbances caution admin w/ phenothiazines/anti-seizure meds/antidepressants don't abruptly withraw watch mental status C
