Misc Neuro Disorders Flashcards
Multiple Sclerosis
- what is this?
- most common in who?
- dx at what age?
- most commonly seen in what regions?
What: body makes abys and wbc against proteins in the myelin sheath, this results in inflammation and injury to the sheath and nerves. The result is scarring eventually damage can slow or block the nerve signals that control muscle coordination, strength, sensation, and vision.
- brain, spinal cord, and optic nerve
- Relapsing-remitting*
Most common in women
Dx between ages 30-50yo
Not found between +40 to - 40 degrees (around the equator), found in northern latitudes; western european lineage
*increased risk if HLA-DR2 gene
Causes of MS
- environmental factors
- genetics
- viruses/bacteria; epstein barr
Signs & Sx of Multiple Sclerosis
- weakness, numbness, tingling, or unsteadiness in a limb “Lhermittes Sxs”
- unilateral visual impairment
- fatigue
- spastic paraparesis
- diplopia
- disequilibrium
- sphincter disturbance
- dysarthria
- mental disturbance
Signs:
- optic neuritis
- ophthalmoplegia
- nystagmus
- spasticity or hyperreflexia
- babinski sign
- absent abd reflexes
- labile or change in mood
- *UMNL**
MS
-describe onset in younger and older pts
-what are the types?
young=subacute or acute sx
older=insidious
Types:
-Benign = no disability, return to normal between attacks
-relapsing-remitting*= interval months to years after initial episode before new sx develop or original ones reoccur. (get progressively worse, but only after attack)
Secondary progressive: steady deterioration occurs, unrelated to acute relapses
Primary progressive: steady increase in disability w/o attacks
How do you dx MS?
-what is definitive dx?
- MRI w/ contrast= gold standard
- LP if you dont see anything on MRI and you still have high suspicion, looking for protein, lymphocytosis, elevated IgG, myelin abys, oligoclonal bands
Clinical Dx:
-2 or more exacerbations greater than 1-2mo apart that sx take longer than 24hrs to resolve.
Definiative dx:
-intermittent or progressive CNS sx with TWO OR MORE CNS white matter lesions in an appropriately aged patient
What scale is used to measure disease progression of MS?
-Kurtzke Explanded Disability Status Scale
MS
- Tx
- -minimally ill
- -exacerbations
- -rest of people
- -symptomatic
- minimally affected pts:
- -no tx
- -encourage healthy lifestyle
- -PT
Rest of people:
-Corticosteroids; mainstay for acute exacerbations = reduce inflamm, improve nerve conduction
- immune modifiers; IFN 1a, IFN 1b, Glatiramer acetate
- immunosuppressents; clyclophosphomide(Cytoxan), imuran (azathioprine)
IVIG can reduce clinical attack rates
Tysabri (Natalzumab); decreases movement of immune cells from the blood stream across the BBB into the brain and spinal cord.
*only used if cannot tolerate any other therapies…we dont ever use this.
symptomatic tx
-spasticity: dantrolene, baclofen
- fatigue: provigil, amantadine
- bladder problems: cholinergics (Bethanechol
- depression- antidepressants
Cerebral Palsy
- what is this?
- when is this commonly dx?
- cause
What: abnormalities in parts of the brain that control muscle movements
Commonly dx 18mo-3years
Causes:
- damage to young developing brain, can occur:
- -during pregnancy; infections, insufficient perinatal oxygen, prematurity, Rh incompatibility
–during birth: asphyxia during labor and delivery
–after birth: brain injuries occuring in the first 2 years of life; infections such as meningitis, head injuries (trauma, child abuse)
What are the 4 major classifications to describe the different movement impairments of cerebral palsy?
All of these are characterized by what?
- Spastic
- Athetoid
- Ataxic
- mixed
Characterized by abnormal muscle tone, posture, reflexes, motor development, and coordination.
Spastic Cerebral Palsy:
-presentation
- *most common**
- hypertonic
- spastic hemiplegia (one side)
- spastic diplegia (legs)
- spastic quadriplegia
- spasticity of the limbs
Athetoid or Dyskinetic Cerebral Palsy:
-presentation
- have trouble holding themselves upright, steady position for sitting or walking
- requires a lot of concentration to get their hand to a certain spot
- may not be able to hold onto things
- mixed muscle tone (hypertonic, hypotonic)
Ataxia Cerebral Palsy
-presentation
- hypotonia and tremors
- writing, typing, using scissors may be difficult
- problems with balance while walking
Signs of Cerebral Palsy in Newborns and older children?
Newborns: floppy movements, unable to roll over, unable to sit crawl, or walk, birth defects such as irregular spine, small jawbone, or small head
older children:
- walking with one foot or leg dragging
- walking on the toes
- muscle tone that is either too stiff or too floppy
Cerebral Palsy:
-sx
seizures, spasms, involuntary movements, speech or communication disorders, hear or vision impairments, cognitive disabilities, learning disabilities, behavioral disorders
Cerebral Palsy
-dx
- may take months to years
- clinical, parental observations, medical hx, PE, screening tests
Evaluate childs muscle tone; floppy or stiff
Check reflexes; persistence of primative reflexes:
- moro reflex (startling)
- walking reflex
- rooting reflex
- sucking reflex
- tonic neck reflex
- palmar grasp
- babinkski
See if baby has developed handedness by 12mo (most dont develop this until after 12mo)
CT, MRI, US of brain may be done….though they may not be helpful
What are some secondary consequences of Cerebral Palsy?
-orthopedic deformities such as scoliosis and hip dislocation
- shafts of bones are often thin (gracile)
- metaphyses are enlarged (ballooning)
- shorter in height
- bones may grow at different lengths (one leg longer than the other)
Cerebral Palsy
- prognosis
- tx
brain damage does not progress, but symptoms DO progress
Tx: cannot be cured -PT, OT, ST -Drugs to control seizures, relax muscle spams, allieviate pain -surgery to correct anatomical abnormalities or release tight muscles -braces/orthotics -Wheelchair, rolling walkers -communication aids
Amyotrophic Lateral Scerlosis
- aka
- what
- pathophysiology
- what do these patients usually die of?
aka: Lou Gehrigs Dz
What;
-fatal neuromuscular dz characterized by progressive muscle weakness resulting in paralysis
Patho:
- attacks nerve cells in the brain and spinal cord. Motor neurons which control movement of voluntary muscles deteriorate and eventually die, once these are dead the brain can no longer initiate and control muscle movement, these muscles weaken and deteriorate.
- degeneration of the upper and lower motor neurons.
- bowel and bladder sphincters and eye movement are usually spared.
Usually die of PNA or respiratory failure, generally within 2-5 years of dx
ALS
- common at what age?
- what gender is most affected?
- cause
- earliest sx
Can strike at any age, most between 40-70yrs
More common in men
Cause:
- unknown, may have genetic component (5%)
- may be associated with veterans & repeated concussions
Sx:
- weakness, muscle atrophy
- twitching, cramping, stiffness of muscles
- slurred and nasal speech
What are the two types of onset in ALS?
Dx of ALS
75% Limb onset
25% bulbar onset; difficulty speaking, slurred nasal speech, difficulty swallowing
Dx:
- no test can provide definitive dx of ALS; clincal
- must have signs and sx of both upper and lower motor neuron damage that cannot be attributed to other causes
EEG, EMG/Nerve Conduction Velocity, MRI
ALS patients may develop frontotemporal dementia, have mild problems with word generation, attention, and decision making, T/F?
True
ALS Tx
Riluzole (Rilutek)
- may offer neuroprotective effects, though it does not reverse the damage already done to the motor neurons.
- lengthens survival by several months
-BIPAP for ventilatory support if resp muscles weakened
Wernicke-Korsakoff Syndrome
- cause
- who gets this?
- characterized by?
Cause: deficiency of Thiamine (B1)
Who: chronic alcoholism
Characterized: confusion, amnesia, confabulation
Confabulation = memory disturbance, production of fabricated or distorted memories about oneself or the world w/o the intention to deceive.
other features:
- weakness
- Paralysis of EOM
- Nystagmus
- Ataxia
- peripheral neuropathy
- diplopia
Dx of Wernicke-Korsakoff Syndrome
sx
CBC (r/o infection)
Coags (r/o bleeding disorder)
serum/urine drug test
liver enzymes
CT (r/o head trauma)
MRI** will show acute lesions of Wernickes
^^diagnostic^^
