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43 Cards in this Set

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Neuromuscular Junction Disorder Symptoms
-Purely motor, esp. proximal, w/ fatigue.
-Often abnl eye movement & ptosis (variable diplopia & ptosis)
-Bulbar musculature-dysphagia, dysarthria--Neuro emergency!
-Muscle tone-normal or reduced
-Reflexes-nl or reduced
What are the tests for Neuromuscular Junction Disorder?
-Special maneuver--prolonged (2 minute) upgaze or arm elevation
-Nerve conductions: usually nl
-Repetitive nerve conductions: decremental response.
-Needle EMG examination: nl
What are three examples of NMJ disorders?
Myasthenia Gravis, Botulism, Myasthenic Syndrome
What are the two types of myopathy?
Inherited--Muscle Dystrophy,
Acquired --Polymyositis, Toxic Myopathy.
Describe Duchenne Muscular Dystrophy
-Mutation in gene for Dystrophin protein.
-Amt of dystrophin determines severity
-X-linked recessive Xp21.
-Gower's sign
-30% w/ new mutation,
-Proximal > Distal wkness of arms & legs, symmetric.
-Onset 3-5 yrs-wkness
-Unable to walk by 13 y/o
-Calf hypertrophy, contractures, scoliosis
-Cardiomyopathy--cause of death.
-Mental retardation
-CRK-very high.
How do you diagnose & treat Duchenne Muscular Dystrophy?
-Diagnose: Muscule biopsy, DNA testing.
-Steroids: may prolong walking 2 yrs.
Describe the clinical presentation of Myasthenia Gravis.
Presents in 2 ways:
1. Ocular MG: ptosis, extraocular muscles (purely ocular in 20%,most start w/ this & generalize)
2. Generalized: bulbar, extremities (weakness, swallowing problems)
-Fatigue w/ repeated activity
-Proximal>distal wkness
-Dysphagia-->Myasthenic crisis--Neuro emergency!!
Describe the pathophysiology of Myasthenia Gravis.
Classic autoimmune d/o.
-Ab's to Ach receptor (Generalized--90% w/ Ab's, Ocular: 50-70% w/ Ab's)
-May be assoc w/ Thymus tumor
What are the ways to diagnose MG?
-Tensilon Test (short acting cholinesteric inhibitor, rarely causes heart block but use EKG)
-Repetitive nerve stimulation-->decremental response
What is the treatment for MG?
-Myasthenic crisis--plasma exchange, IV immunoglobulin.
-Cholinesterase inhibitors--Pyridostigmine (Mestinon)
-Immunosuppressant agents: attacks root cause of MG.
-Ocular MG--? need immunosuppreants (if only ocular, don't use)
-Thymectomy-may improve control, needed for thymus tumor.
Describe Myasthenic Syndrome (Lambert-Eaton Syndrome)
-Legs weaker than arms;
-Fatigability, improves w/ brief exercise.
-Occasional ptosis.
-Autonomic dysfxn--dry mouth & eyes
-Reflexes absent, appears w/ exercise.
-Ab to voltage gated Ca channel
-Exercise increases intracellular Ca.
-Presynaptic disorder (absent reflexes, M. syndrome before synapse, and MG after synapse)
-May be assoc w/ neoplasm, precedes CA >80%, esp w/ small cell lung CA, higher chance of resp failure.
-Tx: immunosuppression, treat CA.
Describe Sensory neuronopathy & what conditions its associated with.
-Dorsal root ganglia neurons are affected.
-Prominent sensory loss: very poor position sense, areflexia.
-Viral: Sjogren's syndrome.
-Paraneoplastic-anti-Hu antibody, assoc w/ small cell lung CA.
In which 4 conditions would you give IV Immunoglobulin and how does it work?
Acutely ill patient w/ Guillain-Barre syndrome, Myasthenia Gravis, Chronic Demyelinating Polyneuropathy, Dermatomyositis.
-Pooled, purified, sterilized Ab's, work by blocking antibodies, reducing complement.
-Easily given, widely available, rare side effects, short onset of action.
Describe Polyneuropathy.
-Symmetric, length dependent, worse in legs than arms, worse distally;
-Sensory, motor, autonomic.
-Nerve pain;lancinating, burning, paresthesias.
-Allodynia: neutral stimuli are painful (ex. bed sheets hurt).
-Asymmetric: mononeuritic multiplex.
Describe Polymyositis.
Endoymsial inflammation (around muscle FIBER). Its a type of inflammatory myopathy: symmetric, proximal>distal wkness, dysphagia, posterior neck wkness (hold up head from behind), quads very weak, age: >20, fatigue, anorexia, muscle pain. Slightly inc risk of malignancy (lung, breast), Assoc w/ autoimune, Thyrotoxicosis, arhythmias, muscle bx important b/c justifies use of immunosuppressants, EMG w/ small MUPs w/ inc insertional activiity.
What are the 5 signs of Acute cord Lesion?
Ascending weakness, Hypotonia, Hyporeflexia or areflexia, Babinski sing not reliable, reduced rectal sphincter tone
What does and Acute cord lesion resemble?
An acute peripheral neuropathy - "spinal Shock"
Define Myopathy? What is the effect on Motor system and what is the effect on sensory system?
Muscle pain with movement, palpation. Motor: Normal, hyper-, atrophy, normal or reduced muscle tone, proximal weakness> distal, Dysphagia possible, eye movemnt normal, fce normal.
Sensory: Normal
Reflexes: Normal or reduced
What is the time course of a acute vs chronic myopathy?
Acute (toxic, infectoins) or chronic (inflammatory, inherited)
WHat do you see on an EMG and a Needle EMG for a myopathy?
EMG- Normal Sensory nerves
- normal or reduced motor amplitude
- NEEDLE EMG: increased spontneous activity (indicates abnormality), Small MUP and increased recruitment (both indicate myopathy)
What are the three types od Peripheral Neuropathy
-Axonal or Demyelinatin
-Acute or chronic
-Critical care neuropathy and Myopathy
What is Beckers MD
Its a type of inherited myopathy
New mutations are rare
Onset >7 yrs, still walking past 13 (unlike Duchene MD)
Proximal> Distal
Calf hypertrophy, contracturee, scoliosis
Cardiomyopathy, mental retardation
CPK- very high
What is Statin Induced Myopathy? aka Lipid Lowering Agents Myopathy
-1 to 6/10,000
- Older females with renal or liver disease, DM, debilitated
- Starts after 2-3 mo of starting the drug
- Early- resting oain, worse with exertion
- Late- weakness, myoglobinuria
-CPK- normal to high
- Recovery- Days to months after stoppin (pts can restart the drug for short time then stop again)
What is Botulism?
- WHat agent causes it
- What is the MOA of the Toxin
- What effects does it have?
- What is its Txt?
It is a NMJ disorder
- prod. by Clost. botulinum (gram + bacilli, anaerobic, spores)
-7 types (A to G)- A & B (medical uses)
-Degrades a specific SNARE protein in the axon terminal- nNT vesicles cannot bind with cell membrane (cant release ACH)
-Presynaptic defect
- 18-38 hrs latency
-Weakness- diffuse, symmetric, proximal worse than distal
- Bulbar - Dysphagia and dysarthis
-Ptosis, extraocular muscle weakness
-Reflexes reduced
-Autonomic-dilated pupils , dec HR, dec BP
-Txt- supportive, antitoxin
WHat is CIDP? Chronic Inflammatory Demylinating Polyneuropathy
What does the EMG show?
What is its treatment?
-SLow progressive weakness> moths
-Proximal and distal weaknes
-Sensory Loss- not prominent
-Abscent reflexes
-EMG: SLowed conduction + blocks
-Treatment: Long term immunosuppressant agents
What are two types of Acute inflammatory Polyneuropathy (AIP) and what is an example of the "classic demyelinating disease"
- Can be either Axonal or demyelinating
- Guillian -Barre syndrome
What are the 2 types of GBS?
Decribe each type.
Two types are Demyelinating or Axonal

Demyelinating: distal paresthesias, weakness: progressive "ascending", Weakness more than sensory loss, Abscent reflexes early, CN VII- upper and lower face

AXONAL type- worse prognisi, slow recover, Campylobacter jejuni coinfecton, Quadriplegia in 30%, Mechanical ventilaton in 1/3, NEUROLOGIC EMERGENCY- watch resipiration function
What do you see on Lab studies for GBS
EMG: NL for 1st 7-10 days
-Nerve conduction study- demyelnating -slowed conduction
Axonal- Reduced amplitude
-Needle EMG- normal first 3 weeks
-CSF- inc protein, NL WBC
- If elevated CSF whit cell- ?HIV
What would you get is the ulner N. is entrapped/Lesioned in the following locations: Axilla, Elbow, Forearm, wrist? What are the symptoms? How do you diagnose it? How do you treat it?
-Axilla- cruches, usually with median n.
-Elbow- Cubital tunnel syndrome (2nd most common nerve compression site, leprosy)
-Forearm- trauma, hematoma,shunts
- Writst- Guyon's cannal (Hamate and pisiform)-0 distal to this- sensation spared
Symptoms- parathesis and numbness in ulnar n sensory distribustion
-DX- EMG examination
Tx: Cubital Tunnel- ant. transposition
What is the sensory innervation of ulnar nerve?
C8-T1 and covers the pinky and half of 4th finger on each side of the hand.
Describe Carpel Tunnel Syndrome
F>M, middle aged.
-Most common nerve entrapment syndrome.
-Intermittent palmar finger/hand paresthesias
-Wakes up pt @ night or on waking up
-Better after flicking wrist.
-Tinel's sign--tap wrist
-Phalen's test--forced wrist flexion x 30 sec
-EMG: slowed conductions across wrist
-Tx: splinting, surgery.
What is the sensory innervation of median nerve (C6-T1)?
Tips of fingers on dorsum (2-4), palmar surfaces of your hand (thumb thru 1/2 of fourth).
What are the causes of foot drop?
Damaged muscles: Tib Ant, Pero Long, TibPos, GasSol.
-If peroneal N cause-->TibAnt & PerLon involved.
-If Sciatic N-->All muscles involved.
-If L5, Tib Ant, PerLon, Tib Pos involved.
What is the fxn of PerLon?
Foot down & out
What is the fxn of Gas Sol?
Down like on Gas Peddle
What is the fxn of Tib Ant?
Picks foot up
What is the fxn of Tib Posterior?
Down and In.
What are some causes of lumbosacral radiculopathy?
-Immune-elevated sed rate.
-Diabetic amyotrophy (severe pain in anterior thigh, knee reflex decreases)--actually upper lumbar plexitis.
-Radiation to pelvis.
-Cancer or hemorrhage in pelvis.
-Lyme disease, vasculitis, heroin.
Describe the symptoms and causes of Polymyelitis.
Causes: -viral infection w/ an enterovirus, major illness in 10% infected & paralytic dz in 50% w/ major illness; Legs >arms >bulbar, may have acute dysautonomia. Post-polio syndrome after >15 yrs (suddenly develop worsening symptoms)
What are the symptoms of radiculopathy?
-Pain in N distribution, esp sharp shooting.
-wkness & absent reflexes in N distribution.
-Rarely w/ sensory abnormalities
-In legs, radiates past knees (+straight leg raising test)
-Dx: MRI:anatomical study, loss for compressive N lesions.
-EMG: physiologic study.
-Sensory: ALWAYS NL in radic;
-Needle EMG: best @ localizing.
-Paraspinal muscles affected.
What is the tx for radiculopathy?
Conservative management vs. surgery.
What is Amyptrophic lateral Sclerosis (ALS) aka Lou gehrig's disease) and what is its Hallmark?
-Weakness esp in distal extremisties
-Hallmarck- Combined upper and lowe motor neuron symptoms and signs
- Also get hyperreflexia and increased tone combined with atrophy and fasciculations
-often in same limb
- Cranial nerves involved- tongue fasciculations
- Paraspinal muscle involved- EMG
-Overall survival 3-4 yrs
a) C4 is sensory nerve root for-
b) C6 is sensory nerve root for-
c) C7 is sensory nerve root for-
d) C8 is sensory nerve root for-
e) L1 is sensory nerve root for-
f)L2 is sensory nerve root for-
g)S2 is sensory nerve root for-
C4- Cape
C6- rdial forearm + thumb
C7- Middle finger
C8- Ulnar forearm + thumb
L1- inguinal region
L2- Anterior thigh
S2- coccygeal- target around anus