Non-Traumatic Paralysis

Nicholas E. Kman MD FACEP

Associate Professor of Emergency Medicine

• Columbus Botulism Outbreak

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

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

• 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.

The Neuro Exam

• Mental Status Testing

• Higher Cerebral Function (Language)

• Cranial Nerves

• Sensory Examination

• Motor System

• Reflexes

• Cerebellar Testing

• Gait and Station

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

Focal Neuropathies

Neuromuscular Junction Disorders

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.

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.

Botulism

• Botulism is caused by Clostridium botulinumtoxin and occurs in 3 forms:

– Foodborne

– Wound

– Infantile.

• Foodborne botulism typically comes from improperly preserved canned foods.

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.

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.

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.

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.

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.

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.

Botulism Treatment

• Treatment includes respiratory support, trivalent botulinum antitoxin 10 mL IV, and admission.

• For infants, human botulism immunoglobulinhas been shown to decrease mechanical ventilation requirements and length of intensive care unit stays.

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

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

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

Myasthenia Gravis

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

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.

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

The Ice Test

• Ice Test

The Ice Test

• Ice Test

Tensilon Test

• Edrophonium inhibits acetylcholinesterase-prolongs Ach at junction.

• + test is elevation of eyelids 2-5 minutes post administration.

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.

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).

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

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

JAMA, 4/20/2005

What diagnosis do we share?

Acute Peripheral Neuropathies

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).

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

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.

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.

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

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.

Guillain-Barre’ Syndrome Treatment

• Initial treatment includes respiratory support, admission to a monitored setting, and neurologic consultation.

• Both IV immunoglobulin and plasmapheresisshorten the time to recovery.

• n engl j med 366;24 june 14, 2012

• n engl j med 366;24 june 14, 2012

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.

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.

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.

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).

Take Home Pearls

• Classic triad of botulism: symmetric, descendingflaccid paralysis with prominent bulbar palsies; an afebrile patient; and a clear sensorium.

• In GBS, typical presentation includes ascendingsymmetric 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.

Take Home Pearls

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

References

• Hand PJ, Kwan J, Lindley RI, Dennis MS, WardlawJM. 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.

•

Thanks and Questions