lecture 3 GD- nueromuscular Flashcards
what are the 4 components of the peripheral nervous system
- neuron cell body
-peripheral processes
-neuromuscular junction
-mm
in the neuron cell body what is sensory and what is motor
dorsal root ganglion is sensory and anterior horn cell is motor
what type of fibers function as dull , aching , burning pain and temperature sensation
C
what type of fiber function as sympathetic preganglionic motor axons
B
what type of axon fiber functions as shap pain ,very light touch and temp sensation
ad
what type of axon fiber functions as touch, pressure , vibration and joint position sensory axons
aB
what type of axon fibers functions as large motor axons , mm stretch and tension sensory axons
Aa
what is a Progressive fatal disease associated with degeneration of upper and lower motor neurons
ALS
is ASL more common in men or women
men
what is the avery life expectancy of people with ALS
3 years from time of diagnosis
what are LMN signs of people with ALS
weakness, atrophy and fasciculations
what is UMN signs of ppl with ALS
pathologic spread fo reflexes , clonus
what is the most common genetic causes for ALS
C9orf2 hexanucleotide
what does C9orf72 hexanucleotide also cause
frontotmeporla dementia
what is the main symptom for LAS
fatigue and mm stiffness
(fatigue is the most tho)
what are the 3 FDA approved medications for ALS
• Riluzole
• Edaravone
• Tofersen
what is the symptom management fro faitgue in ALS patients
BIPAP, Modafinil
what is the symptom management for mood in ALS patients
SSRI’s
what is the symptom management for Pseudobulbarpalsy(PBA) in ALS patients
Nuedexta
what has been associated with an increased risk of ALS
intense physical activity
what is anarthropod-borne flavivirus
west nile virus
how does the west nile virus transmission occur
following a bit from an infected mosquito which got the virus after feeding on other hosts , mainly birds
when is the e peak transmission for west nile virus
july-octovober
are people with the west nile virus symptomatic or asymptomatic
80% are asymptomatic
what is the west nile fever
myalgia, headache, fever, and maculopapular rash)
Less than 1% of west nile virus cases are ____
neuroinvasive
what are the 3 things that can happen if the west nile virus is neuroinvasive
– Aseptic meningitis
-Meningoencephalitis(including brainstem encephalitis)
– Acute flaccid paralysis
acute flaccid paralysis is an acute onset and rapid progression of what 2 things
-asymmetric flaccid weakness and hypo active/absent reflexes
what other 2 things can happen with acute flaccid paralysis
-respiratory insufficiency
-bowel and bladder dysfucntion
Poliovirus is anRNA Enterovirus that belong to the ___ family
Picornavirus
Most poliovirus infections are ___,with viral growth limited to the ___
asymptomatic
gut
if someone had a minor disease of polio what would happen
flue like illness due to viremia
what is the diagnostic criteria for post polio syndrome
- History of paralytic poliomyelitis
- period of partial or complete recovery followed by interval of stable function
- gradual or sudden onset of progressive and persistent mm weakness
- symptoms for > 1year
- exclude other casues
what is the neurological examination of post polio syndrome
-LMN syndrome
- joint pain and cold intolerance may happen
t/f some people may have new neurologic problems unrealted to polio for post poli syndrome
T
each nerves root corresponds to a ___ and ___
dermatome and myotome
what is an area of skin supplied by a particular nevre root
dermatome
what is a myotome
mm inverneated by a particular nerve root
what are symptoms of radiculopathy
- pain , numbness and thingling in the corresponding dermatome
-weakens of mm supplied by the corresponding myotome - reduced reflex
if C7 root is causes neurologic S&S which disc is it compressed in
C6-C7
for the cervical spine it is always the one above the root
if a patient has pain in the low back , buttock , and later high and you found sensory finding in her lateral leg , dorosmedial foot and large toe and the motor finding were tight adduction , knee flexion and DF what root would u suspect is damaged and between what disc
L5 and between L4-L5
if your patient has pain in the medial forearm and hand and 5th digit and u find sensory finding at the medial forearm and hand and 5th digit and motor finding in the intricinc hand mm and a finger flexor change in reflex what nerve root would u suspect and between which disc
c8 and c7-t1
if your patient has pain int he dorsal forearm area and u find sensory changes int he 3rd and 4th fingers and motor fingers in arm extension , finger and wrist flexors and extensors and a reflex change in the triceps would root would u suspect and in between which disc
C7 and C6-C7
how do u treat radiculopthy
improve with time and pt
___ of cervical radiculopathies due to herniated disk improve without surgical intervention.
95%
when do u get sx for radiculopathy
– Not responding to PT
– Intractable pain
– Significant weakness
how do u differentiate between radiculopathy and lower motor neuron lesion
A – weakness
B – radicular pain in a dermatomal distribution
C – muscle atrophy
D – hyporeflexia
what is it called when Mutiple spinal nerve roots effected
polyradiculopathy
what are the S&S of polyradiculopathy
Motor symptoms predominate, variable degree of sensory loss, pain, generalized loss of DTRs
Inflammatory (Guillan-Barre, sarcoid, SLE, Sjogrens), DM, porphyria, Infectious (lyme, HIV, VZV, CMV, TB), neoplastic.. these are all etiologies of what
polyradiculopathy
what plexi is plexopathy effected
cervical or lumbosacral
what are S&S of plexopathy
pain , restricted to single limb , more widespread then single nerve or root
what are the casues/etiologies of plexopathy
trauma most common , malignant infiltration , thoracic outlet syndrome
what is Neuropathy of single peripheral nerve
mononeuropahty
what are the sings of mononeuropahty
restricted to anatomic distribution of one nerve
what is the causes of mononeuropahty
compression , entrapment , trauma
carpal tunnel and meralgia parasthetica are examples of what
mononeuropahty
what is carpal tunnel
median never compressed at the wrist
what is meralgia parasthetica
lateral femorla cutaneous compressed at inguinal ligament
what is polyneuropathy
generalized process affecting peripheral nerves
whar are signs of polyneuropathy
commonly symmetric , length dependent weakness, sensory loss, loss of DTRs (stocking glove distribution so from distal to proximal)
• Sensory, Motor, Autonomic
• Large fiber, small fiber
• Demyelinating, Axonal
• Hereditary, Acquired
this would characterize what
polyneuropathy
Most of the myelinated fibers are ____ diameter fibers
LARGE
what are suggestive features of demyelinating
- Weakness without atrophy
- Length independent
- Patchy
- Asymmetric
• Compression/stretch
• Guillain–Barresyndrome
• ChronicInflammatoryDemyelinatingPolyneuropathy(CIDP)
• Multifocal motor neuropathy
• Diphtheria
• Charcot – Marie – Tooth
• Hereditary neuropathy with liability to pressure palsies
• MAG-neuropathy
• Leukodystrophies
• Diabetes
these are all examples of what
Demyelinating
what are suggestive features of axonal demyelinating
– Distal>proximal
– Legs>arms(longer axons more susceptible)
– Muscle wasting early
*
Diabetes
AMAN / AMSAN (axonal GBS)
Toxic – vincristine, acrylamide, arsenic
Alcohol
Uremia
Collagen vascular diseases
Carcinoma
Amyloid
these are examples of what kind of neuropathy
axonal
small diameter never fibers that are sensory carry information about __ and ___
pain and temp
what are 2 nerves that are small diameter nerve fibers
sensory never and autonomic
- Reduced ability to feel pain and hot/cold temperature
- Allodynia
- Burning, electric current-like, neuropathic pain
- Discoloration of the skin, reduced sweating in the feet, loss of hair in the feet/legs
- Autonomic dysfunction
patients that present with this will have what type of nerupathy
small fiber
what are the 2 nevers that are large diameter nerve fibers
motor and sensory nerves
large diameter nerve fibers that are sensory nerves carry information about ___, ____ and ____
pressure , vibration and proprioception
neuropathy that affects the large fibers present with what
• Numbness, tightness, balance problem
• “walking on foam, cobblestone
autonomic neuropathy is an autonomic dysfunction that is ____
involuntary
what are the 5 S&S of autonomic neuropathy
- orthostatic hypotension
- arrthymias
-severe constipation , urinary retention and erectile dysfunction - sweating
- early satiety , lightheadedness with meals
– Diabetes or IGT(Impaired Glucose Tolerance)
– Thyroid disease (hypo or hyperthyroidism)
– Toxic (alcohol, chemotherapy agents, HIV medications, heavy metals)
– Vitamins and/or nutritional deficiencies (vitamin B12, B6, vitamin E, Copper, Zinc)
– Systemic Conditions (kidney problems/cancers/autoimmune)
– Infections: HIV, Syphilis, Lyme, Herpes
– Immune mediated (CIDP, GBS)
theses are major causes of what
polyneuropathies
inflammatory demyelinating polyneuropathies is a disorder that causes what
weakness, sensory loss and areflexia
what is a rapidly progressive inflammatory demyelinating polyneuropathy and when does pression end
acute inflammatory demyelinating polyneuropathy ( AIDP or guillain- barre syndrome)
progression ends by 4 weeks
what is a slowly progressive inflammatory demyelinating polyneuropathy and when does pression end
• Chronic inflammatory demyelinating
polyneuropathy (CIDP)
• Weakness progressing > 2 months
what is the progression for Acute Inflammatory Demyelinating Polyneuropathy
- tingling , parasthesia w little sensory loss
- severe radicualr back pain
- weakness
- legs and arms
-legs then arms
-arms to legs
-facial (most common)
-dysphagia
what does the exam for Acute Inflammatory Demyelinating Polyneuropathy (gullian barre syndrome) show
mostly symmetric weakness
- areflexia in most after 1 week
what is absence and what is elevated when doing a CSF with albuminocytologic dissociation in someone with Acute Inflammatory Demyelinating Polyneuropathy (gullian barres)
no WBC’s and elevated protein
what hasten recovery for people with guillain barre syndrome
plasma exchange and IVIG
T/F: steroids are beneficial for guillain barre syndrome
F
is Chronic Inflammatory Demyelinating Polyneuropathy more common in men or women
men
Chronic Inflammatory Demyelinating Polyneuropathy is _____ progressive sx over weeks–months
Slowly
does Chronic Inflammatory Demyelinating Polyneuropathy affect more motor or sensory
motor
is symmetric weakness proximal and distal more common or atrophy in Chronic Inflammatory Demyelinating Polyneuropathy
symmetric
where is sensory loss mainly from Chronic Inflammatory Demyelinating Polyneuropathy
legs
diabetic neuropathies is a ____ ____ pattern in polyneuropathy
length dependent
does biabetic neuropathies affect LE or UE first
LE (stocking glove sensory loss)
does diabetic neuropathies causes weakness ?
no
what is associated with less frequent and less severe neuropathy
tight glucose control
– Tricyclic antidepressants -> Cymbalta
– Gabapentin -> Lyrica
– Tramadol
– Topical capsacin
these al treat neuropathic pain in what
diabetic neuropathy
Diabetic Lumbosacral Radiculoplexus Neuropathy (DLRPN) aka Diabetic Amyotrophy is usually in type 1 or type 2 diabetes
2
**Diabetic Lumbosacral Radiculoplexus Neuropathy (DLRPN) aka Diabetic Amyotrophy is associated with ___ weight loss and beings ____
large and abruptly
Diabetic Lumbosacral Radiculoplexus Neuropathy (DLRPN) aka Diabetic Amyotrophy being with what
sharp or aching pain asymmetrically in the hip and thigh which can spread to the leg and foot
in Diabetic Lumbosacral Radiculoplexus Neuropathy (DLRPN) aka Diabetic Amyotrophy how does weakness progress
distally and can become bilateral
Insidious onset, indolent course, onset <20 years old is present in what
hereditary neuropathy
what is the term used to describe group genetic neuropathies
charcot marie tooth disease
what are 2 clinical manifestions of Charcot-Marie-Tooth Disease
distal weakness and atrophy and length dependent sensory loss
what foot deformities are present with Charcot-Marie-Tooth Disease
pes cavus , pes plannus , hammer toes
how are the reflexes in Charcot-Marie-Tooth Disease
absent or decreased
does Charcot-Marie-Tooth Disease progress slow or fast
slow
– Delayed early milestones
– Slow progression
– Absence of positive sensory symptoms but
prominent sensory loss on exam
– Foot deformities, injuries, surgeries
– Family history/consanguinity
– Hip problems/scoliosis
would this make u think hereditary or acquired
hereditary
what is the early onset of Charcot-Marie-Tooth Disease
-delayed onset of walking
-very slow CV
what is the childhood onset for Charcot-Marie-Tooth Disease
- normla walking
-slow runner as child - develop symptoms 5-20 years old
- most common
what are presynaptic disorder of the neuromuscular junction
– Lambert Eaton myasthenic syndrome
– Botulism
what is a **post synaptic **disorder of the neuromuscular junction
Myasthenia gravis
– Fluctuating, fatigable
– Asymmetric ptosis, diplopia
– Difficulty chewing
– Dysarthria, dysphagia, nasal speech
– Hoarseness
– Head drop
– Limb weakness
if someone presents with this int he clinic what do they have
myasthenia gravis
myasthenia gravis is a ___ disorder
autoimmune
what are 7 triggers fro MG
• Emotional upset
• Viral illnesses and other systemic illness, fever
• Heat
• Hypo or hyperthyroidism
• Surgery
• Menstrual cycle
• Drugs
– D-penicillamine
– IFN-α,
– Botox
– Succinylcholine
– Magnesium salts
these are drugs that should never be used for what
Myasthenia gravis
what test for u do for myasthenia gravis dianosis
tensilon test
what are negative signs for myopathies
• Weakness
• Fatigue
• Atrophy
• Exercise Intolerance
what are positive symptoms for myopathies
• Myalgia
• Cramps
• Contractures
• Hypertrophy
• Stiffness
• Myoglobinuria
what will we see for a physcial exam for myopathy
- proximal weakness
-sensation intact - DTR can be normal or little reduces
• MuscularDystrophies
• Congenital Myopathies/Muscular Dystrophies
• Metabolic/Mitochondri al
• Channelopathies
are these classification of hereditary or acquired myopathies
hereditary
what are acquired myopathies
• Toxic/DrugInduced
• Endocrine
• Inflammatory/Immune
• Associatedwith Systemic Illness
Polymyositis and dermatomyositis is a chronic onset of ___ weakness and has variable ___ and ___ invovlment
proximal
cardiac and pulmonary
Polymyositis and dermatomyositis can be
malignancy (eso dermatomyositis)
MTX and Azathioprine are apart of what treatment line for dermatomyositis
2nd line
what is the management for dermatomyositis
• Cancer Screening
• Bone Health while on steroids
– Calcium & Vitamin D
• Physical Therapy
what is the most frequent myopathy in patients >50 and more common in men
inclusion body myositis
is inclusion body myositis slow or fast progressive weakness
slowly
how will the weakness present in inclusion body myositis
proximal left and distal arm
-asymmetric
-mm atrophy
-dysphagia
