Neuro 2 study cards Flashcards
Myasthenia Gravis: hereditary or Autoimmune disorder?
Autoimmune disorder
Define myopathy
neuromuscular disorders in which the primary symptom is muscle weakness due to dysfunction of muscle fibers.
What is Myasthenia Gravis
Neuromuscular disease of the neuromuscular junction, Skeletal m. weakness that worsens with activity and improves with rest.
Symptoms of Myasthenia Gravis?
- Diplopia (double vision)
- Facial weakness
- Ptosis- (eyelid drooping)
- SOB
- Weakness in neck, UE, LE
- Fatigue
Interventions fro Myasthenia Gravis?
- Interval training
- Fatigue intervention/ conservation
- Physical and behavioral training
- Resistance, Aerobic, and balance.
Precautions of Myasthenia Gravis?
• MG Crisis
o Medical emergency where breathing stops and the individual requires ventilation.
o Not always related to exercise .
Limb Girdle Muscular Dystrophy (LGMD): hereditary or Autoimmune disorder?
Hereditary
What does Limb Girdle Muscular Dystrophy (LGMD) affect?
Affects proximal muscles, Some may have cardiomyopathy
Early stages of Limb Girdle Muscular Dystrophy presents what symptoms? (early and late)?
- Early stage: changes in walking, hands on knees to transition.
- Late Stage: may require a w/c
Interventions for Limb Girdle Muscular Dystrophy?
- Resistance training
- Aerobic training
- Work more on functional tasks
Age for male and women to get Myasthenia Gravis?
Women< 40 yrs
men >60 yrs
Fascioscapularhumeral Muscular Dystrophy % of type 1 vs type 2?
type 1 : 95%
type 2: 5%
Fascioscapularhumeral Muscular Dystrophy: hereditary or Autoimmune disorder?
hereditary
When do symptoms come about (age) for Fascioscapularhumeral Muscular Dystrophy?
before 20
Number one symptom or presentation of Fascioscapularhumeral Muscular Dystrophy?
Mask-like appearance
Interventions for Fascioscapularhumeral Muscular Dystrophy?
- Pulmonary function test
- Speech therapy
- Hamstring and trunk muscles early onset
- Maintain flexibility
- Manage pain
- Orthotics
- Gait aid
- w/c
are thick or thin myelin faster?
Thicker the faster conduction
are thick or thin myelin slower?
thin is slower conduction
Shwann cells make?
Myelin in the PNS
oligodendrocytes make?
Myelin in the CNS
How is Bell’s palsy acquired?
after a viral infection
What does Bells palsy affect?
Half of the face
What nerve does bells palsy affect ?
CN 7 (facial nerve)
What percent of Bell’s palsy patients have a full recover?
71%
How does Trigeminal Neuralgia present?
Really bad facial pain: brief, intense bursts of pain within a distribution of pain.
How does one get Trigeminal Neuralgia?
compression of trigeminal nerve (CN5)
What is Poliomyelitis? what causes it?
Epidemic in the 1950s
Caused by a virus that is highly infectious especially in children <5 y.o
Symptoms of Poliomyelitis?
- Fever
- Fatigue
- Headache
- Vomiting
- Stiffness of the neck
- Pian in the limbs
What is Postpolio Syndrome (PPS)
New neuromuscular symptoms that occur decades after recovery
Who develops Postpolio Syndrome (PPS) ?
¼ to ½ with childhood polio develop PPS.
What symptoms do Postpolio Syndrome (PPS) have?
Deterioration in the LE, predispose individuals to overuse of UE m. for compensation.
Interventions for Postpolio Syndrome (PPS)?
Non-exhaustive exercises. General body conditioning Lifestyle modifications/ energy conservation Compensation Prevention
Charcot-Marie-Tooth Disease: Hereditary or autoimmune?
Hereditary motor and sensory neuropathy.
What does Charcot-Marie-Tooth Disease symptoms look like?
Distal limb muscle wasting and weakness,
abnormalities in DTRs and skeletal deformity.
Begins with affecting ankle DF and EV (peroneal n.)
Which type of Charcot-Marie-Tooth Disease is axonal?
- CMT1: Dysmyelinating
- CMT2: Axonal
- CMT3: Dysmyelinating
- CMT4: Dysmyelinating or axonal
- CMT5: Dysmyelinating
Diabetic Peripheral Neuropathy (DPN) Affect what cells?
Schwann cells and neurons
Diabetic Peripheral Neuropathy (DPN) is under what classification?
metabolic
Guillain-Barre Syndrome (GBS) & AIDP Acute Inflammatory Demyelinating Polyneuropathy presents?
most common cause of rapidly evolving motor paresis, paralysis, and sensory deficits.
Guillain-Barre Syndrome (GBS) & AIDP Acute Inflammatory Demyelinating Polyneuropathy is motor or sensory?
either motor and sensory
or
Just motor
Name the peripheral nervous system disorders?
o Myasthenia Gravis o Limb Girdle Muscular Dystrophy o Fascioscapularhumeral Muscular Dystrophy o Bell’s Palsy o Trigeminal Neuralgia o Poliomyelitis o Postpolio Syndrome (PPS) o Charcot-Marie-Tooth Disease o Diabetic Peripheral Neuropathy o Guillain-Barre Syndrome (GBS)/ AIDP
ALS- Amyotrophic Lateral Sclerosis is most common in men or women?
Men
When does ALS typically present? (age)
40+
Define: ALS- Amyotrophic Lateral Sclerosis
Progressive NM disease characterized by degeneration and eventual death of upper motor neurons and LMN causing weakness of the limb, respiratory, and bulbar musculature.
Pathology of ALS
Dysfunction of the EAAT2
Loss of
brainstem CN nuclei and
anterior horn cells in spinal
cord
For GBS/AIDP what do we want to avoid?
fatigue exercises
When would you see foot deformities?
Charcot foot deformities
What percent of people with DM will develop Diabetic peripheral neuropathy?
50%
Who are most likely to get ALS
White males
ALS scale to diagnose
EI Escorial Criteria
Presence of all three:
o LMN degeneration by exam.
o UMN degeneration by exam
o Progression of the motor syndrome within a region or to other regions by Hx and exam.
ALS survival rate for 3, 5 and 10 years
3 years: 50%
5 years: 20%
10 years: 10%
What are negative progonistic factors for ALS?
- Negative prognostic factors:
- Bulbar onset
- Weight loss
- Cognitive impairment
- Impaired respiratory function
what are the two staging outcomes we use for ALS? and score ranges
King’s Clinical Staging (0-5)
&
MITOS Functional Staging (0-5)
For ALS what gets affected first distal or proximal extremities?
Distal to proximal
Weakness, low tone (hypotonia) and decreased/absent reflexes are signs of what?
LMN lesion
Hyperrelexia would be result of?
UMN lesion
Clonus, babinski, Hoffmans is a ___ lesion?
UMN lesion
Largest gene in the body?
Dystrophin
Whats apraxia?
It’s when you can’t do learned movements on command
true or false: you can do a prenatal screen for Duchenne’s muscular dystrophy
False
What can you do a prenatal screen for?
SMA
What is the 2nd most common type of dementia
Vascular dementia
What are the cardinal signs of PD?
TRAP • T: tremor: resting. • R: rigidity • A: Bradykinesia (Akinesia) • P: Postural instability
What type of tremor is associated with PD
resting tremor
Age and percentage of boys that lose ambulation with DMD?
age 10-12: >70% of boys lose ambulation
What age do boys present Prominent muscle weakness with DMD ?
5 years old
Signs of SMA (spinal muscular atrophy)
Smart Kind they can slip through your arms hand tremor tongue fassiculations
What part of the brain does Parkinson’s affect?
Substantia nigra (in the basal ganglia)
most common form of hereditary ataxia in the US?
Fredrick’s Ataxia
What part of the brain is damaged With ATAXIA
Cerebellum
Are the impairments from guillaine barre permanent?
NO
GB symptoms
Tingling
numbness
weakness
starting distal to proximal
Name the two Subcortical Dementias?
- Dementia with Lewy bodies (DLB)
2. Huntington’s disease
Scales used for dementia?
global deterioration scale 1-7
GEMS
Can you do blood work tests for Parkinson’s?
NO
medications for PD
dopamine replacement and deep brain stimulator
how long do deep brain stimulators work for?
3-5 years
How long does Dopamine replacement for PD last?
5 years
whats the gold standard diagnostic test thing for PD?
UPDRS (united parkinsons disease rating scale)
Whats the 2nd used scale diagnostic test thing for PD?
Hoehn and Yahr
Parkinson’s has too much or too little dopamine?
not enough
Which disease is characterized by choreic (dance) movements?
HUNTINGTONS
what stages of Hoehn and Yahr involve unilateral deficits ?
1, 1.5 and 2
Stocking glove pattern neuropathy is associated with that?
Diabetes
Why is MS considered an autoimmune disease ? what attacks what ?
T cell and B cells are overproduced and attack oligodendrocytes.
What chemical imbalance causes HD?
Chromosome 4 produced a protein called huntingtin which caused neuronal degeneration and death.
Whats the most common type of MS ?
Relapsing-remitting
What the most effective way to manage heat with MS?
Spray bottle with fan
what a cardinal sign of ATAXIC DYSARTHRIA?
“scanning speech” slurred “drunk”
Where does PICA come off of ?
vertebral artery
Where does AICA come off of ?
basilar artery
Where does most of the blood supply in brain come from?
Superior cerebellar A
Where is the big dentate nucleus located?
cerebellum
Can someone with stage 4 Huntington’s live independently ?
No
Most primitive (vestibular nuclei) is in the ?
BRAINSTEM
blank percent of MS patients will require an aid to walk within blank years?
50% within 10 years
Whats the best type of MS to get?
relapsing-remitting
Where is MS more common?
In US and Europe, places where there is less sunlight (VITAMIN D)
What type of medication is used for MS
corticosteroids
What is the number one important thing to ppl with MS?
Walking is generally the most important function to people. (70%)
Biggest symptom for ppl with MS?
fatigue
If someone can walk 500 meters With MS what general range EDSS are they on?
0-3.5: Mild Disability
If someone can walk 100-500 meters With MS what general range EDSS are they on
4-5.5: Moderate Disability
If someone cant walk 100 meters Without assistance with MS what general range EDSS are they on.
6+
Name some non motor signs of Parkinson’s ?
Olfactory loss, sleep dysfunction, autonomic dysfunction, hallucinations, dementia
Older age, lower BMI, cigarette smoking, being a professional athlete, and repeated head injuries are all risk factors for what ?
ALS (lou gertis)
Most important intervention for patients with dementia
exercises
What progresses faster: postural instability and gait disorder or tremor predominant PD
PIGD
What is the gold standard for measuring PD impairments ?
UPDRS (higher the score the worse)
Is ALS an UMN or LMN disease ?
Both
Most ppl with MS will not be working within ____ years?
10
A scared look is a characteristic of what disease ?
Progressive Supranuclear Palsy (PSP)
PD plan of care, emphasize what 4 things
Flexibility, strength, aerobic conditions and task specific training?
Describe what happens with SMA if the novel treatment isn’t given
mm atrophy until eventual respiratory/cardiac failure
SMA’s novel treatment lets them?
gene therapy: lives a full life
What is a common gait abnormality in patients with CMT
stomping
True or false: there is novel treatment for CMT
False
Describe common gait abnormalities in DMD
Extended, closed-packed and trendelenberg
Too much or too little dystrophin in DMD?
little
True or false: SMA is an autosomal recessive disease?
true
Which is most common type of CMT: dysmyelinating or axonal ?
dysmyelinating
Lewy body and huntingtons are what types of dementia
subcoritcal
What type of memory is best to train w dementia ? implicit or explicit
implicit
What type of memory do you train with cerebellar disorders? implicit or explicit
explicit
Are fruits and vegetables helpful in protecting yourself against dementia?
true
What serious medical complication comes from bell’s palsy ?
eye can dry out
Whats the difference between stroke and bells palsy sx?
Bell’s palsy is complete paralysis, Stroke can raise their eyebrows.
Explain why a pes cavus happens with CMT?
Something to do with the DF/EV losing control first. (peroneal n damage)
Whats dysmetria?
Impaired ability to scale movement distance
A cerebellar tremor is also called what kind of tremor?
action tremor
When do Fredricks ataxia symptoms start?
5-15 YO
Are Focal lessions better or worse prognosis
better
idiopathic disease? (no known cause)
multiple systems atrophy (MSA)
DO blood supply zones and functional zones correlate?
No
Name the functional zones
Cerebrocerebellum
Spinocerebellum
Vestibulocerebelum
What is the largest functional zone?
cerebrocerebellum
What is cerebrocerebellum zone?
Largest and in charge of planning and initiating voluntary movement
What is spinocerebelar zone?
comparator, regulation of muscle tone and coordinates skilled voluntary movement, postural tone
Vestibularcerebellum where is the nuclei
in brain stem
What does vestibularcereebelum do?
balance, eye movement VOR
How do we treat cerebellar disorders implicit or explicit learning?
explicit
What is deep in the cerebellum?
Fastigial nucleus anf Dentate nucleus
both nucleus
Where is vestibular nuclei?
brainstem
where are purkinji cells
white matter
What can you do to test Dysmetria?
heel to shin, finger to nose test
Scale for ataxia
BARS, SARA, ICARS