Lecture 6 - Neuromuscular Diseases Flashcards
The type of neuropathy a patient has depends on the ___________
type of fibers affected
are they myelinated or unmyelinated fibers?
Are they A Delta, A beta, …..?
You’re more likely to have a ______ injury than an injury at the plexus
nerve root/nerve injury
What is ALS?
Does it have UMN or LMN degeneration?
Who is more commonly affected?
Progressive fatal disease that has degen of both UMN and LMN
More men than women
10% familial
Average life expectancy after diagnosis: 3 years
What is a common way of diagnosing ALS?
You see atrophy of tongue muscles, fasciulations of tongue muscles
T or F: ALS also causes cognitive problems
F, no cognitive probems
C9orf72 ALS vs SOD1 ALS
SOD1 is a rare type that is actually treatable
C9orf72 is the common form that is not treatable
What are the most common symptoms of ALS?
Fatigue
Muscle stiffness
SOB
muscle cramps
increased saliva
How is the west nile virus transmitted
Mosquito bites
note: peak transmission between july-october
T or F: most cases of west nile virus are asymptomatic
T, 80% asymptomatic
20% have a fever
Less than 1% are neuroinvasive
What is acute flaccid paralysis
Usually after viral illness
acute onset and rapid progression of asymmetric flaccid weakness and hypoactive/absent reflexes, respiratory insufficiency, bowel and bladder problems
What is poliomyelitis
Virus that is usually aymptomatic
minor disease- flu like symptoms
major disease- CNS invasion (less than 1% fo cases)
Has been eradicated in north america
What is the diagnostic criteria for post-polio syndrome?
History of paralytic poliomyelitis
period of partial or complete recovery
gradual onset of progressive and presistent muscle weakness
symptoms for +1 year
exclude other causes
Radiculopathy is most common in what nerve root?
L5
Symptoms of radiculopathy
Pain, numbness, tingling in dermatome
weakness in myotome
reduced reflexes
__% of cervical radiculopathies due to herniated disc improve without surgical intervention
95
note: surgery is need if: Not responding to PT, significant weakness
How do you differentiate between radiculopathy and lower motor neuron lesion?
Radiculopathy is in a dermatomal distribution AND shooting pain down neck
If you’ve got more than one nerve distribution affected by radiculopathy in one arm or leg, what is a possible cause?
Plexopathy
What are the signs and symptoms of plexopathy? What is plexopathy most commonly caused by?
typically painful, restricted to single limb, more widespread than single nerve root
most commonly casued by trauma, tumor, or thoracic outlet syndrome
What is mononeuropathy
what are the signs and symptoms
what is it caused by?
Neuropathy of single peripheral nerve
Signs and symptoms: restricted to anatomic distribution of one nerve
etiology: usually compression, entrapment, trauma
What is polyneuropathy?
generalized process affecting (many) peripheral nerves
signs and symptoms: depending on which nerves are involved
commonly isometric and length dependent weakness, sensory loss, loss of DTR
stocking/glove distribution
hundreds of reasons you could have this
How do you characterize polyneuropathy?
Is it sensory, motor, or autonomic?
is it large or small fibers?
demyelinating or axonal?
hereditary or accquired?
What nerves are myelinated?
Which ones arent?
Myelinated: Motor, sensory A-Beta A-Delta, Some autonomic
Unmyelinated: Sensory C fibers, some autonomic
Most myelinated fibers are _____ diameter and mainly __________
large
mainly motor
What is the main kind of neuropathy we can treat w/ medication (neurologists can)
demyelinating
What are the suggestive features that someone has demyelinating neuropathy (polyneuropathy)
weakness without atrophy (at first)
length independent
patchy
asymmetric
What are the suggestive features that tell you it’s an axonal problem vs a demyelinating problem?
Suggestive features of an axonal problem:
Distal > proximal
Legs > arms (longer axons more susceptible)
muscle wasting early (demyelinating doesnt have early muscle wasting)
What are two common causes of axonal problems?
Diabetes (most common)
Alcohol (second most common)
What are 2 examples of “small” nerve fibers
Sensory nerves (specifically pain and temp)
Autonomic nerves
Patient’s with small fiber neuropathy will experience what symptoms?
Autonomic dysfunction
allodynia
neuropathic pain
discoloration of skin, reduced sweating, hairloss
reduced ability to feel pain, hot/cold
what are examples of large diameter nerve fibers?
Motor Nerves
Sensory nerves that carry pressure/vibration/proprioception
Patients that have “large fiber neuropathy” will present w/…..
Numbness, tightness, balance problems
“walking on foam/cobblestone” (no proprioception)
Orthostatic hypotension
arrythmias
severe constipation, ED, Urinary Retention
abnormal sweating
early satiety, light headedness w/ meals
These are signs of what kind of problem?
Autonomic neuropathy
What are 2 of the most common causes of polyneuropathy?
- Diabetes
- Alcohol
Others: Thyroid disease, vitamin deficiency, systemic problems, infections
What is an example of a rapidly progressing inflammatory demyelinating polyneuropathy?
What is an example of a slowily progressive inflammatory demyelinating polyneuropathy
Rapid: Acute inflammatory demyelinating polyneuropathy (AIDP) or Guillain Barre syndrome.
-progression ends in majority by 4 weeks
Slow: Chronic inflammatory demyelinating polyneuropathy (CIDP)
-Weakness progresses for over 2 months
What are common causes of Acute inflammatory demyelinating polyneuropathy (AIDP)
preceding illness, surgery, vaccination in some
How does Acute inflammatory demyelinating polyneuropathy (AIDP) progress?
- Tingling paresthesia w/ little sensory loss
- severe radicular back pain
- Weakness (mild to complete quadriplegia)
How is Acute inflammatory demyelinating polyneuropathy (AIDP) usually diagnosed with an exam?
Exam:
-symmetric weakness,
-facial weakness
-sensory loss variable
-areflexia in most after 1 week
note: also diagnosed w/ CSF and EMG
What is the progression of Guillain Barre syndrome
Monophasic
How do you treat Guillain Barre syndrome?
Supportive care: monitor/treat respiratory failure, autonomic dysfunction
-plasma exchange and IVIG (anti immune system drug)
NO STEROIDS
Rehab starts early
at the start of demyelinating conditions you do not see muscle __________, but you do see muscle ___________
atrophy
weakness
Who is normally affected by Chronic inflammatory demyelinating polyneuropathy (CIDP)?
Male > female
Average onset 50+
Usually relapsing-remittent or can be progressive
How does Chronic inflammatory demyelinating polyneuropathy (CIDP) progress?
slowily over months
Motor > sensory
symmetric proximal and distal weakness which progresses to atrophy
hypo or areflexive
sensory loss mainly in legs, rarely disabling
What condition is slowily progressive
with symmetric weakness in proximal and distal muscles
hypo/areflexive
CIDP
What is the normal length dependent pattern in polyneuropathy
stocking/glove
How does diabetic neuropathy usually present?
Distal symmetric sensory polyneuropathy
“stocking/glove”
usually affects LE/UE
slowily progressive
no weakness
autonomic problems
diabetic neuropathy affects what % of diabetics
65%
What condition is associated w/ large weight loss
begins abruptly
begins w/ sharp aching pain asymmetrically in hip and thigh, spreads to leg and foot
pain -> progressive weakness of thigh and knee -> weakness progresses distally
often becomes bilateral
Diabetic lumbosacral radiculoplexus neuropathy AKA diabetic amyotrophy
note: usually affects DM2 in middle or older age
Hereditary neuropathies:
Charcot-marie-tooth disease
Hereditary sensory autonomic neuropathy
amyloid
HNPP
These commonly have an insidious onset when??
Under 20 years old
What condition presents w/
distal weakness and atrophy
length dependent sensory loss
foot deformities: pes cavus, pes plannus, hammer toe
abscent or decreased reflexes
chronic slow progression
Charcot-Marie-Tooth-disease
What can clue you in that a demyelinating condition is hereditary
delayed early milestones
slow progression
foot deformities, injuries, surgeries
family history
hip problems/socliosis
abscence of positive sensory symptoms but prominent sensory loss on exam
Charcot Marie Tooth disease clinical phenotypes:
Early onset:
Childhood onset:
Late onset:
Early onset: delayed onset of walking (>15 months)
Childhood onset: Normal onset of walking
slow runner as a child
develops symptoms between 5-20
most common presentation
late onset group:
20+
Supportive treatment for Charcot Marie Tooth disease
PT and OT
orthotics
screen for scoliosis and hip dysplasia
surgery for skeletal and soft tissues abnormalities
Lambert eaton myasthenic syndrome and botulism are forms of __________ disorders
myasthenia gravis is a ___________ disorder
Presynaptic
Post synaptic
How can you differentiate myasthenia gravis from MS?
Pt improves w/ rest
What disease presents as:
Flucuating/fatiguable (can test fatigue w/ eye muscles)
asymmetric ptosis/diplopia
difficulty chewing
dysarthra,dysphasia, nasal speech
hoarseness
head drop
limb weakness
Myasthenia gravis
Who does Myasthenia gravis mainly affect?
Younger woman and older men
What are common triggers for Myasthenia gravis
Emotional Upset
Viral Illness/systemic illness
heat or hyperthyroidism
surgery
menstral cycle
drugs
What drugs make Myasthenia gravis worse?
D-Penicillamine
Botox
Magnesium Salts
IFN
Succinylcholine
How to diagnose Myasthenia gravis?
Tensilon test- The test involves injecting the drug Tensilon (edrophonium chloride) intravenously into a patient’s arm or hand, and then observing their response to repetitive physical movements:
Ocular cooling - Their eye works better after u put a cold pack on it because cold makes the NM junction work better
antibody panel serum
EMG
Symptoms of Myasthenia gravis
fatigue
exercise intolerance
weakness
myalgia
cramps
stiffness
atrophy
How do you identify myopathy on a physical exam
Usually proximal weakness
-identify w/ GOWER’S SIGN (they have to get up from the ground using their hands to post) \
DTR can be normal or slightly reduced
Polymyositis, dermatomyositis, and inclusion body myositis are all examples of…
inflammatory myopathies
How do polymyositis and dermatomyositis usually present?
Subacute/chronic onset of proximal weakness
variable pulmonary and cardiac involvement
usually involve a malignancy (cancer)
autoimmune disorder
HIV
Dermatomyositis: Inflammation of the skin aswell, theyre all red and inflamed in different places
What supplementation is important for bonehealth for patients w/ dermatomyositis
Calcium and vitamin D
Who most commonly gets inclusion body myositis?
50+ men
How does inclusion body myositis usually present?
Proximal leg and distal arm weakness (finger flexors and quad)
asymmetric
muscle atrophy
dysphagia
sometimes associated w/ autoimmune disease
note: 56% need an AD after 7 years (20% WC)
