After watching this lecture, learners will be able to: Describe the various etiologies of non-traumatic paralysis Illustrate the neuro exam for the paralyzed patient Recognize the signs and symptoms of acute peripheral neuropathies Explain the treatment of acute peripheral neuropathies

1. Non-Traumatic Paralysis
Nicholas E. Kman MD FACEP
Associate Professor of Emergency
Medicine

2. • Columbus Botulism Outbreak

3. Objectives
• Describe the various etiologies of non-
traumatic paralysis
• Illustrate the neuro exam for the paralyzed
patient
• Recognize the signs and symptoms of acute
peripheral neuropathies
• Explain the treatment of acute peripheral
neuropathies

4. Differential Diagnosis
• ALS
• Stroke
• GBS
• Botulism
• Bell’s Palsy
• Seizure (Todd’s Paralysis)
• Brain Tumor
• Psych Stuff
• Poliomyelitis
• Electrolyte Disturbance
• Myasthenia Gravis
• Migraine
• Aortic Dissection
• Spinal Stenosis/Cauda
Equina
• Myopathies/ Polymyositis
• Drugs (can cause
myopathy)
• Organophosphates
• Lyme Disease
• Tick Paralysis

5. • Hand PJ, Kwan J, Lindley RI, Dennis MS, Wardlaw JM. Distinguishing between
stroke and mimic at the bedside: the brain attack study. Stroke. 2006
Mar;37(3):769-75. Epub 2006 Feb 16. PubMed PMID: 16484610.

8. The Neuro Exam
• Mental Status Testing
• Higher Cerebral Function (Language)
• Cranial Nerves
• Sensory Examination
• Motor System
• Reflexes
• Cerebellar Testing
• Gait and Station

9. TABLE 146-1 Differentiating Central from Peripheral Nervous System Disorders
Central Peripheral
History Cognitive changes
Sudden weakness
Nausea, vomiting
Headache
Weakness confined to 1 limb
Weakness with pain associated
Posture- or movement-dependent pain
Weakness after prolonged period in 1
position
Physical examination
Reflexes Brisk reflexes (hyperreflexia)
Babinski sign
Hoffman sign
Hypoactive reflexes
Areflexia
Motor Asymmetric weakness of ipsilateral upper
and lower extremity
Facial droop
Slurred speech
Symmetric proximal weakness
Sensory Asymmetric sensory loss in ipsilateral
upper and lower extremity
Reproduction of symptoms with
movement (compressive neuropathy)
All sensory modalities involved
Coordination Discoordination without weakness Loss of proprioception

10. Focal Neuropathies

11. Neuromuscular Junction Disorders

12. Botulism Epidemiology
• Clostridium botulinum is anaerobic, gram-positive,
spore-forming bacilli found globally in soil.
• Clostridium spores are hardy and survive at wide
range of temperatures.
• Clostridia vegetate and produce botulinum toxin
in oxygen-poor, low-salt, low-sugar, and low-
acidity environments.
• Botulism is not contagious.
Villar RG, Elliott SP, Davenport KM. Botulism: The Many Faces of Botulinum Toxin and its Potential for Bioterrorism. Infect Dis Clin
N Am 2006; 20: 313-327.

13. Botulinum Toxin
• Toxin contains enzyme that blocks acetylcholine-
containing vesicles from fusing with terminal
membrane of motor neuron.
• Results in flaccid paralysis and death by
asphyxiation.
• 100,000 times more lethal than sarin and 15,000
times more lethal than VX.
• Single gram of inhaled crystalline toxin can kill
over 1 million people.
Osterbauer PJ, Dobbs MR. Neurobiological Weapons. Neurol Clin 2005; 23: 599-621.

14. Botulism
• Botulism is caused by Clostridium botulinum
toxin and occurs in 3 forms:
– Foodborne
– Wound
– Infantile.
• Foodborne botulism typically comes from
improperly preserved canned foods.

15. Infantile Botulism
• In infantile botulism, organisms arise
from ingested spores, often in
honey, and produce a systemically
absorbed toxin.
• Clinical features appear 6 to 48
hours after ingestion and may be
preceded by nausea, vomiting, and
diarrhea.
• Infants may present with
constipation, poor feeding, lethargy,
and weak cry.

16. Wound Botulism
• Should be considered in patients
with a wound or a history of IV
drug use.
• Mexico is frequently source of black
tar heroin- a thick gummy
substance that needs to be diluted.
• Most believe the source of
botulism is in “cutting agent”. Often
things like dirt and honey.

17. Botulism Diagnosis
• Look for the classic triad of botulism: symmetric,
descending flaccid paralysis with prominent
bulbar palsies; an afebrile patient; and a clear
sensorium.
• Remember the "4Ds" of the bulbar palsies:
– Diplopia
– Dysarthria
– Dysphonia
– Dysphagia
Arnon SS, Schechter R, Inglesby TV. Botulinum Toxin as a Biological Weapon. JAMA 2001; 285: 1059-1081.

18. Botulism Diagnosis
• Look for the classic triad of botulism: symmetric,
descending flaccid paralysis with prominent
bulbar palsies; an afebrile patient; and a clear
sensorium.
• Remember the "4Ds" of the bulbar palsies:
– Diplopia
– Dysarthria
– Dysphonia
– Dysphagia
Arnon SS, Schechter R, Inglesby TV. Botulinum Toxin as a Biological Weapon. JAMA 2001; 285: 1059-1081.

19. Botulism Diagnosis
• Rapidity and severity of paralysis depends
upon amount of toxin absorbed.
• Neuromuscular blockade is irreversible,
recovery can only occur when new motor
axons are generated (weeks to months in
adults).
• Depending on dose and route, symptoms from
botulinum toxin occur between 2 hours and 8
days.

20. Botulism Treatment
• Mainstay is supportive care with fluids,
nutrition and mechanical ventilation.
• Monitor airway!
• Supportive care supplemented with passive
immunization with equine or human antitoxin.
Botulism toxin binds irreversibly. Antitoxin
cannot reverse effects that have already
occurred, but can help stop disease
progression.
Arnon SS, Schechter R, Inglesby TV. Botulinum Toxin as a Biological Weapon. JAMA 2001; 285: 1059-1081.

21. Botulism Treatment
• Treatment includes respiratory support,
trivalent botulinum antitoxin 10 mL IV, and
admission.
• For infants, human botulism immunoglobulin
has been shown to decrease mechanical
ventilation requirements and length of
intensive care unit stays.

22. Quiz Time!
• The food science department decides to can its
own tomatoes for use at the Ohio State v.
Michigan Tailgate. You are working the next day
when college kids start to arrive with weakness.
• How many ventilators do we have at THE
OSUWMC?
– A. 1201
– B. 563
– C. 199
– D. 86
– E. 884

23. Quiz Time!
• The food science department decides to can its
own tomatoes for use at the Ohio State v.
Michigan Tailgate. You are working the next day
when college kids start to arrive with weakness.
• How many ventilators do we have at THE
OSUWMC?
– A. 1201
– B. 563
– C. 199
– D. 86
– E. 884

24. OSU Vents
Row Labels Count of Model
BIPAP Equipment 62
Respironics V60 62
Ventilator Adult 112
Avea 31
Puritan Bennett 840 81
Ventilator Adult HF 3
Sensormedics 3100B 3
Ventilator Adult Transport 18
Impact Eagle II 14
iVent 4
Ventilator Infant HF 4
Sensormedics 3100a 4
Grand Total 199

25. Myasthenia Gravis
Bhandari A, Adenwalla F. Mysterious falls and a
nasal voice. Lancet. 2007 Aug
25;370(9588):712. PubMed PMID: 17720023.

26. Myasthenia Gravis
• Myasthenia gravis is a relatively uncommon (but
most common disorder of neuromuscular
transmission).
• Age of onset is characterized by an early peak in
2nd & 3rd decades (female predominance) and a
late peak in the sixth to eighth decade (male
predominance).
• About 10 to 15 percent of those with myasthenia
gravis have an underlying thymoma.

27. Myasthenia Gravis
• Fluctuating degree and variable combination of
weakness in ocular, bulbar, limb, and respiratory
muscles.
• Weakness is result of an antibody-mediated, T-
cell dependent immunological attack directed at
proteins in the postsynaptic membrane of the
neuromuscular junction (acetylcholine
receptors and/or receptor-associated proteins).
• Diagnosis by clinical and serologic testing

28. The Ice Test
• Ice Test

29. The Ice Test
• Ice Test

30. Tensilon Test
• Edrophonium inhibits acetylcholinesterase-
prolongs Ach at junction.
• + test is elevation of eyelids 2-5 minutes post
administration.

31. Treatment
• Initial therapy for most patients with
myasthenia gravis (MG) is oral
anticholinesterase (ie, acetylcholinesterase
inhibitor) like pyridostigmine bromide.
• For adults: pyridostigmine 30 mg TID. The
dose is then titrated by its effect.
• Almost all adult patients require a total daily
dose of ≤960 mg, divided into four to eight
doses.

33. Back to our case…
• Fluctuating diplopia and ptosis are highly
characteristic of myasthenia gravis.
• + rest test result may increase the likelihood of
MG.
• Now clinician can confirm the diagnosis with
the acetylcholine receptor antibody test and
to refer this patient to a specialist (neurologist
or neuro-ophthalmologist).

34. Quiz
• A 45-year-old man has a 2-month history of fluctuating
double vision, a “droopy” right eye that improves with
rest, and a complaint that “food gets stuck halfway
down.”
• Your examination confirms severe right eyelid ptosis
that dramatically improves with rest. What is the next
best test to perform in the ED?
• A. Acetylcholine receptor antibody test
• B. Neurology Consult
• C. Bedside testing for fluctuating diplopia and ptosis
• D. MRI/MRA of the brain
• E. CDU TIA Protocol

35. Quiz
• A 45-year-old man has a 2-month history of fluctuating
double vision, a “droopy” right eye that improves with
rest, and a complaint that “food gets stuck halfway
down.”
• Your examination confirms severe right eyelid ptosis
that dramatically improves with rest. What is the next
best test to perform in the ED?
• A. Acetylcholine receptor antibody test
• B. Neurology Consult
• C. Bedside testing for fluctuating diplopia and ptosis
• D. MRI/MRA of the brain
• E. CDU TIA Protocol

36. JAMA, 4/20/2005

37. What diagnosis
do we share?

38. Acute Peripheral Neuropathies

39. Guillain-Barre’ Syndrome
• Guillain-Barré syndrome (GBS) is an acute
polyneuropathy characterized by immune-
mediated peripheral nerve myelin sheath or
axon destruction.
• It affects all ages and usually follows a viral or
febrile illness, Campylobacter jejuni infection,
or vaccination.
• Currently most frequent cause of acute flaccid
paralysis worldwide (post-poliomyelitis).

40. Guillain-Barre’ Syndrome
• ? Vaccines: CDC 2008: >15 million doses of
Menactra Meningococcal Vaccine have been
distributed. Vaccine Adverse Event Reporting
System (VAERS) has received 26 confirmed case
reports of GBS within 6 weeks of vaccination.
• H1N1 outbreak and vaccination did not cause
increase.
• Common misconception that GBS has a good
prognosis — but up to 20% of patients remain
severely disabled & 5% die, despite
immunotherapy

41. Guillain-Barre’ Syndrome
• Although numerous variants exist, the typical
presentation includes ascending symmetric
weakness or paralysis and loss of deep tendon
reflexes.
• Respiratory failure and lethal autonomic
fluctuations may occur.

42. Guillain-Barre’ Syndrome
• Cerebrospinal fluid (CSF) analysis typically
shows high protein and a normal cell count.
• Albuminocytologic dissociation-50-66% of
patients with GBS in the first week after the
onset of symptoms and ≥75% by 3rd week.

43. TABLE 146-2 Diagnostic Criteria for Classic Guillain-Barré Syndrome
Required
Progressive weakness of more than 1 limb
Areflexia
Suggestive
Progression over days to weeks
Recovery beginning 2 to 4 weeks after cessation of progression
Relative symmetry of symptoms
Mild sensory signs and symptoms
Cranial nerve involvement (Bell’s palsy, dysphagia, dysarthria, ophthalmoplegia)
Autonomic dysfunction (tachycardia, bradycardia, dysrhythmias, wide variations in blood pressure,
postural hypotension, urinary retention, constipation, facial flushing, anhydrosis, hypersalivation)
Absence of fever at onset
Cytoalbuminologic dissociation of cerebrospinal fluid (high protein and low white cell count)
Typical findings on electromyogram and nerve conduction studies

44. TABLE 146-2 Diagnostic Criteria for Classic Guillain-Barré Syndrome
Required
Progressive weakness of more than 1 limb
Areflexia
Suggestive
Progression over days to weeks
Recovery beginning 2 to 4 weeks after cessation of progression
Relative symmetry of symptoms
Mild sensory signs and symptoms
Cranial nerve involvement (Bell’s palsy, dysphagia, dysarthria, ophthalmoplegia)
Autonomic dysfunction (tachycardia, bradycardia, dysrhythmias, wide variations in blood pressure,
postural hypotension, urinary retention, constipation, facial flushing, anhydrosis, hypersalivation)
Absence of fever at onset
Cytoalbuminologic dissociation of cerebrospinal fluid (high protein and low white cell count)
Typical findings on electromyogram and nerve conduction studies
NEJM 2012: Albuminocytologic dissociation is present in no more than 50% of patients with the
Guillain–Barré syndrome during the first week of illness, although this percentage increases to
75% in the third week.

45. Guillain-Barre’ Syndrome Treatment
• Initial treatment includes respiratory support,
admission to a monitored setting, and
neurologic consultation.
• Both IV immunoglobulin and plasmapheresis
shorten the time to recovery.

46. • n engl j med 366;24 june 14, 2012

47. • n engl j med 366;24 june 14, 2012

48. Bell’s Palsy
• Bell’s palsy causes seventh cranial nerve
dysfunction, and patients may complain of
facial weakness, articulation problems,
difficulty keeping an eye closed, or inability to
keep food in the mouth on one side.
• Physical examination findings demonstrate
weakness on one side of the face, including
the forehead, and no other focal neurologic
findings.

49. Bell’s Palsy
• If muscle strength is retained in the forehead,
the lesion most likely is central (ie, in the
brainstem or above); this would exclude Bell’s
palsy, and CT of the head is indicated.

50. Bell’s Palsy
• Treatment with corticosteroids increases the
frequency of complete recovery.
• The dose of prednisone is 1 milligram/ kg per
day PO for 7 days.
• There is no benefit from antiviral medications,
either alone or in addition to steroid therapy.
• Patients should apply lacrilube to prevent
corneal drying at night. Close follow-up with a
neurologist or ENT specialist is indicated.

51. Bell’s Palsy
• Ear should be inspected for ulcerations
caused by cranial herpes zoster
activation (Ramsey-Hunt syndrome).
• As opposed to classic Bell’s palsy, is
treated with both steroids (prednisone
1 milligram/kg per day PO for 7 days)
and antivirals (famciclovir 500 mg PO
TID for 7 days or valacyclovir 1 gram
PO TID for 7 days).

52. Take Home Pearls
• Classic triad of botulism: symmetric, descending
flaccid paralysis with prominent bulbar palsies; an
afebrile patient; and a clear sensorium.
• In GBS, typical presentation includes ascending
symmetric weakness or paralysis and loss of deep
tendon reflexes.
• In MG, hallmark is a fluctuating degree and
variable combination of weakness in ocular,
bulbar, limb, and respiratory muscles.

53. Take Home Pearls
• In Bell’s Palsy, there is no benefit from antiviral
medications, either alone or in addition to
steroid therapy.

54. References
• Hand PJ, Kwan J, Lindley RI, Dennis MS, Wardlaw
JM. Distinguishing between stroke and mimic at
the bedside: the brain attack study. Stroke. 2006
Mar;37(3):769-75. Epub 2006 Feb 16. PubMed
PMID: 16484610.
• Bhandari A, Adenwalla F. Mysterious falls and a
nasal voice. Lancet. 2007 Aug 25;370(9588):712.
PubMed PMID: 17720023.
•

55. Thanks and Questions