NEUROLOGIC

EMERGENCIES

Nina Bates, BSN, RN

Revised by

Elena Stock, BSN, RN

Reference: Sheehy’s Manual of Emergency Care, 7th Edition

Neurologic Assessment in the ED

• Level of Consciousness

• Most reliable indicator of change in patient’s neuro status

• 2 components:

• Arousal (responsiveness)

• AVPU

• Awareness

Glasgow Coma Scale

Three components:

• Eye opening

• Verbal Response

• Best Motor Response

Glasgow Coma Scale

Eye opening

• 4: Spontaneous: eyes open without stimulation

• 3: To verbal (not necessarily to command)

• 2: To pain

• 1: None

Verbal Response

• 5: Oriented x4

• 4: Confused, but use of language is correct

• 3: Inappropriate words

• 2: Incomprehensible words

• 1: None- no words/sounds despite stimulation

Glasgow Coma Scale

Best Motor Response

• 6: Obeys commands

• 5: Localizes to pain

• 4: Withdraws from pain

• 3: Abnormal Flexion

• 2: Extension

• 1: Flaccid

Glasgow Coma Scale

• 15-13: Normal- mildly altered

• 12-9: Moderate abnormality

• 8 or Less: Severely altered (comatose)

AEIOU-TIPS for AMS

• A: Alcohol

• E: Epilepsy (seizure), environmental

• I: Insulin (too much, too little)

• O: Oxygen (hypoxia, hyperoxia)

• U: Uremia (metabolic disorders)

• T: Trauma, toxicity, tumor

• I: Infection, ischemia

• P: Psych, poisoning

• S: Stroke, syncope

SELECTED EMERGENCIES

COMA

• Prolonged state of unconsciousness

• Swift action to preserve life and brain function

• Initial work up is critical to establish correct diagnosis and treatment

Common Causes of Coma in the ER

• Trauma (primary or secondary)

• Cerebrovascular disease (hemorrhagic or ischemic stroke, dissection)

• Intoxications (ETOH, recreational drugs, medications, environmental toxins, CO2)

• Infections (bacterial, viral, fungal)

• Metabolic derangements

• Postictal state

Initial Interventions

• Evaluate A, B, C’s and c-spine (if concerned for trauma)

• Assess LOC (AVPU)

• IV access

• Meds:

• Glucose, BG <70, give D50

• Thiamine 100 mg IV (prevent Wernicke’s

encephalopathy)

• Narcan IV

• Fluids

• Labs

• Radiology

Details Are Important

• Time course of unconsciousness

• Abrupt vs. gradual onset

• Medications

• Toxin exposure

• Social history

• Past medical/surgical history

• Neurological exam findings

• Abnormal labs

• Results of Head CT, LP or EEG if available

• Response to treatments given

CAUSES OF COMA

STRUCTURAL

FOCAL EXAM

• Asymmetric pupillary

abnormalities

• Unilateral weakness, paralysis

• Aphasia, dysarthria

CAUSES

• Stroke, trauma, tumor

NONSTRUCTURAL

NON FOCAL EXAM

• Progressive, gradual onset

• Symmetric cranial nerve findings

CAUSES

Medication overdose, ETOH, illicit

drug use, toxin exposure, CNS

infections, Metabolic

encephalopathy

Key Elements for Handoff

• History of Present Illness

• Clinical presentation/Neurological exam

• Relevant PMH/PSH

• Brain imaging results

• Treatment given thus far

Case Study #1

• A 42 year old female presents to the emergency via

squad, after being found unresponsive by her daughter.

Last known well 2200 the night.

• On initial assessment GCS is 9, and she appears be

protecting her airway. Her pupils are 4mm PERRL, but

has a right gaze deviation. She will open her eyes to

sternal rub for a few seconds, moans and mumbles some

words . She does not follow commands, and moves

extremities spontaneously, except her RUE is noted to be

weaker. A stroke alert was called at this time.

1. What immediate interventions?

Case Study #1

• VS: HR 86, RR 18, BP 149/86, Temp 100.1, Spo2 98% on room air

• Non contrast head CT: no acute pathologies

• CTA: no large vessel occlusion

• CT perfusion: no perfusion deficit

• ABG PH 7.25, PCO2 28, HCO3 19, sats 97%

• Labs: Lactate 4.5. CBC, and Chem7 unremarkable

• Past medical history provided by daughter: • Stroke 2011

• Alcohol abuse

• Anxiety- takes xanax tid every day

• There are many possible causes of this patient being obtunded.

• Presented: RUE weakness, right gaze deviation, and elevated lactate.

• 1. What are your thoughts?

• 2. What further work up interventions?

Courtney Kresge ACNP-BC,CCRN

• Huff, S.J.,Stevens, R.D., Weingart, S.D.,(2012). Emergency Neurological

Life Support: Approach to the Patient with Coma. Neurocritical Care Society,

DOI 10.1007/ s12028-012-9755-4 2012

• Varelas, P. N., Graffagino, C., (2013), Metabolic Encephalopathies and

Delirium. Neurocritical Care Society Practice Update. Retrieved from

www.neurocriticalcare.org/sites/.../ 22.MetabolicEncephalopathies.final

MENINGITIS/ ENCEPHALITIS

Bacterial Meningitis and Viral Encephalitis

• Severity of clinical signs and symptoms may predict

prognosis

• Early interventions for maintaining airway, hemodynamic

instability, and early initiation of anti-infective is critical

• Pathogens

• Bacteria- *Emergency

• Viruses- *HSV encephalitis

• Fungi

• Parasites

Facts

• Annual incident of

bacterial meningitis

• 4-6 cases per 100,000

• Encephalitis is less

common than

meningitis

• Literature suggest 48%

of patients will present

within the first 24 hours

of onset of symptoms

Signs and Symptoms

• Encephalitis

• Focal neurological findings/Cortical disturbances

• New seizure onset

• Extremity weakness

• Aphasia

• Meningitis

• 44% of patients presented with classic triad

• Fever

• Stiff neck

• Mental status changes

• 95% of patients had diagnosis if two of classic triad is

present with headache

Physical Exam

A positive sign is when clinician extends the knee from a flexed 90

degree and produces pain posterior thigh, or lower back

Physical Exam

A positive sign is when patient is supine and the neck is passively

flexed and triggers a flexion of hips and knees

Other Causes of Fever and Headache

• Viral infections without meningitis

• Cerebral abscess

• Severe sepsis

• Sinusitis, tooth abscess, tonsillitis

• Non-infectious conditions: SAH, cerebral venous

thrombosis, pontine hemorrhage

BACTERIAL

• More severe, can lead to

serious complications

• Transmitted via respiratory

droplets and secretions

VIRAL (aseptic)

• Usually mild, self limited

• Not usually contagious

Initial Assessment

• Airway, breathing and circulation evaluated immediately

• Vital signs

• Temperature >100.4F or <96.8F

• Blood pressure

• Heart rate

• Respiratory rate

• Oxygen saturation

• Glasgow Coma Scale

Initial Interventions

• History, examination

• IV access-minimum of two 18 gauges

• POC Glucose and INR, CBC, chemistries, blood cx,

lactate

• IV resuscitation to treat shock

• If bacterial meningitis is presumed, immediate

administration of dexamethasone followed by empiric

antibiotic coverage

• Expedite transfer for Head CT

• Prep for LP after CT is reviewed

• If suspected meningococcus, exposure prophylaxis

CT Prior To LP

• CT will be needed prior if:

• Patients with clinical presentation suggesting

meningitis/encephalitis

• Enough uncertainty of intracranial process

• If show mass/lesion that explains condition/symptoms

• Abort LP

Antibiotic Coverage

• Should be given immediately according to sepsis

guidelines

• After obtaining blood cultures

• Do not wait for LP or CT

• Each hour of delay in the septic patient, mortality is

increased.

Antibiotic Coverage

Community acquired

• Haemophilus influenzae, Neisseria meningitidis,

and Streptococcus pneumoniae

• Rocephin

• Vancomycin

• Acyclovir

• Ampicillin- Listeria monocytogenes

Age >55, Immuncompromised, Alcoholics

• Healthcare or Hospital acquired- ECF, HD, recent hospitalizations

MRSA and Pseudomonis

• Vancomycin

• Cefepime

Antibiotic Prophylaxis

• Recommended for close contacts of a patient with invasive

meningococcal disease to prevent secondary cases.

• Should be initiated within 24 hours after the index patient is identified

• Prophylaxis given >2 weeks after exposure has little value

• Antibiotic regimens for prophylaxis include:

• Rifampin 600mg orally twice daily for two days

• Ciprofloxacin 500mg orally as a single dose

• Ceftriaxone 250mg as a single intra-muscular injection

Normal Lumbar Puncture Results

• Opening pressure <20 cm H2O

• <5 WBC cells/mm3

• NO RBC

• NO xanthochromia (from the Greek xanthos = yellow)

• Protein <50 mg/dl

• CSF glucose >2/3 serum glucose

• Gram stain negative

• LP -> Normal -> Evaluate other infectious sourse

Abnormal LP Results

Very High CSF White Cells

• Bacterial Meningitis

• WBC 100-1000

• RBC normal

• Protein High

• Glucose Low

• Gram stain (+)

Elevated WBC and NO RBC

• Viral Meningitis

• WBC 10-100

• NO RBC

• Protein normal

• Glucose normal

• Gram stain (-)

Abnormal LP Results

Elevated WBC and RBC

• Herpes Encephalitis

• WBC 100s

• RBC 10-100 or High

• Protein normal or

elevated

• Glucose normal

• Gram stain (-)

Elevated RBC or Xanthochromia

• SAH

• WBC normal

• RBC High

• Xanthochromia

• Protein normal to elevated

• Glucose normal

• Gram stain (-)

Case Study #2

• 26 year old female presents to the emergency department via squad after having a witnessed seizure. She is lethargic but arouses to verbal stimuli, and complaining of a 10/10 headache. There is no one present to give a detailed history

• Vital signs: BP 96/62, RR 28, Temp 101F, and Spo2 98% on room air. On exam she has no nuchal rigidity, negative Kernig’s, and Brudzinski sign. Remaining exam unremarkable

• Labs: WBC 16K, NA 130, create 0.9, BUN 10. POC glucose 80 and INR 1.2. Remaining labs were unremarkable.

• What is her possible diagnoses?

• Does she need a head CT prior to an LP?

• Do you think it is appropriate to start antibiotics at this time?

Case Study #2

• Head CT results do not show any lesions or evidence of increased

ICP. LP was performed with the following CSF cell count.

• Elevated opening pressure

• WBC- 1,200 cells 60 % neutrophils

• Protein -70 mg/dL

• Glucose- 35 mg/dL

• Sent for Gram Stain and culture

• 1. What is this patient’s likely diagnosis?

• 2. List some nursing interventions you would want to execute within

the first hour.

STATUS EPILEPTICUS

SE is a Medical Emergency

• Life threatening medical and neurological

emergency

• The more prolonged the status, the more

intractable it is to treatment

• The more prolonged the status, the poorer the

prognosis for recovery

Definition of Status Epilepticus

• > 5 minutes of continuous clinical and/or electrographic seizure activity OR recurrent seizure activity without recovery between seizures

• Convulsive SE with rhythmic jerking of extremities

• Non-convulsive SE with seizure activity seen on EEG but no clinical findings of seizure observed

• Refractory SE is that which does not respond to standard treatment regimens with adequate initial dose benzodiazepine and one antiepileptic drug

Scope of the Problem

• 1st described in 7th century BC

• Effective management remains challenging

• ~ 1 million seizure related ER visits per year • 20% of ED visits for neurological problems

• 200,000 US patients/year status epilepticus

• 30 day mortality of generalized convulsive SE is 19-27%

• Prolonged seizures associated with higher mortality and worse clinical outcomes

Claasen et al. 2015. Neurocritical Care. ENLS SE

Non-convulsive Status Epilepticus

• 8-20% of critically ill patients

• NCC: 18-34% of patients with cEEG monitoring have non-convulsive seizures (NCS) and 10% have non-convulsive status epilepticus (NCSE)

• Medical and surgical ICUs: 8-10% of patients with cEEG monitoring have NCSE

• Up to 48% of comatose patients after convulsive SE have NCS or NCSE

• ~ 20% of patients go from convulsive SE to NCSE after initial treatment

Causes of Status Epilepticus

• Acute

• Metabolic dysfunction

• Infections

• CNS infections

• Stroke

• Head trauma

• Drug toxicity or

withdrawal

• Noncompliance with

AEDs

• Hypoxia

• Cardiac arrest

• Chronic

• Pre-existing epilepsy

• AED withdrawal

• Chronic alcohol abuse

• CNS tumor

• Remote CNS

pathology

• Organ failure

• Illicit drug use

Status Epilepticus by Etiology

Cook et al NCC 2012

Pediatric Causes of SE

• # 1 is systemic infection

• Congenital anomalies

• Anoxia

• Metabolic problems

• Antiepileptic drug withdrawal

• CNS infection

• Trauma

Drugs that Lower the Seizure Threshold

• Theophylline (bronchodilator)

• Carbapenems (ATB)

• Fluoroquinolones (Cipro,

Levaquin)

• Isoniazid (TB ATB)

• Antidepressants (Bupropion,

TCAs)

• Accumulation of drugs with renal failure

• Metronidazole

• Meperidine (Demerol)

• Tramadol

• Lithium

• Clozapine (antipsychotic)

• Cyclosporine (immunosuppressive)

• Sympathomimetic (Neo,

Levo, Dopamine)

• Organophosphate (base of

many insecticides0

Electrolyte Abnormalities and Seizure

• Hyponatremia

• Risk of cerebral edema and herniation

• Increased CNS neuronal irritability

• Hypocalcemia

• Increased neuromuscular excitability and tetany

• CNS neuronal irritability

• Hypomagnesemia

• Magnesium stabilizes neuronal membranes

Phases of SE • Phase I

• First 30 minutes

• Convulsive tonic clonic seizure

• Increased autonomic activity

• Hypertension, hyperglycemia, hyperpyrexia

• Increased sweat and salivation

• Phase II

• Minor twitching, loss of clinical exam

• Decreased cerebral autoregulation

• Decreased cerebral blood flow

• Increased ICP

• Systemic hypotension

Abou-Khalil. 2013. Vanderbilt

Complications of SE

• Hyperthermia

• Lactic acidosis

• Rhabdomyolysis

• Acute kidney injury

• Neurogenic pulmonary

edema

• Hyperkalemia

• Arrhythmias

• Aspiration

• Impaired ventilation

• Hypertension

• Hypotension

• Hyper/hypoglycemia

• Intracranial

hypertension

• Altered cerebral

perfusion

• Permanent

neurological damage

Diagnosis

• Diagnostics should not

delay control of

seizures

• H & P

• O2 sat

• Vital signs including

temp

• Blood glucose

• CBC

• Chemistry panel,

magnesium, calcium

• ABG

• Lactic acid

• CPK

• Toxicology screen

• AED levels

• Neuroimaging

• Lumbar puncture in

febrile patients

• EEG

History

• Seizure description and course

• Onset and duration

• Observed signs

• Tonic clonic movements, gaze deviation, automatisms,

impaired consciousness

• Autonomic signs: HTN, tachycardia, pupillary dilation

• Past medical history

• History of prior seizure disorder

• Medication history

• AEDs and compliance

• New medications

• Social history

• Illicit drug or ETOH use or withdrawal

Physical Exam

• LOC

• Cranial nerves

• Motor skills

• Reflexes

• Sensory exam

• Cerebellar exams

• Mental status

• Focal exam findings

EEG

• 24 hour EEG is gold standard

• 30 minute spot EEG misses ~ 2/3 non-convulsive seizures and ½

clinical seizures

• 24 hour EEG detects 95% seizures in non-comatose and 80% in

comatose patients

• Video EEG to distinguish EEG abnormality from artifact

Indications for EEG with SE

• Recent clinical seizure or SE without return to baseline >

10 minutes

• Coma, including post cardiac arrest

• Epileptiform activity or periodic discharges on initial 30

minute EEG

• Intracranial hemorrhage including TBI, SAH, ICH

• Duration of cEEG for minimum of 48 hours for suspected

non-convulsive seizures in patients with altered mental

status

Brophy et al NCC 2012

Differential Diagnosis

• Status epilepticus or non-convulsive status epilepticus

• Post-ictal state

• Movement disorder

• Herniation

• Limb-shaking TIA

• Psychiatric disorder

• Toxic metabolic disorders

Psychogenic Non-Epileptic Seizure

• “Pseudo seizure” or “Conversion Disorder”

• Non-epileptic spell simulating SE

• Not intentionally produced or feigned

• Conversion of emotional pain to physical symptoms

• Differs from malingering or factitious disorders

Peters. Vanderbilt

Goals of Treatment

• Airway protection and supportive care

• Rapid termination of seizure activity

• Prevention of seizure recurrence

• Manage precipitating cause(s)

• Manage complications

Response to Treatment

• If treatment of GCSE initiates within 30 minutes of seizure

onset, 80% respond to 1st AED given

• When treatment initiates 2 hours or more after seizure

onset, 40% respond to 1st AED given

Benzodiazepines-1st Line Therapy

• Lorazepam (Ativan) preferred benzodiazepine

• Duration of action 4-14 hours

• Higher initial response rate

• 4 mg IV, may repeat in 5-10 minutes

• Diazepam (Valium) duration of action 20 minutes

• 0.15 mg/kg IV to max of 10 mg/dose, may repeat every 5 minutes

• May give rectally

• Midazolam (Versed)

• May be given IV, IM, nasally, or buccally

• 0.2 mg/kg IM to max of 10 mg dose

2nd Line Therapy

• Phenytoin (Dilantin)/Fosphenytoin (Cerebyx)

• 20 mg PE/kg IV (or IM), may give additional 5 mg/kg

• Complicated by hypotension and cardiac conduction disturbances

• Infuse 50 mg/min

• Valproic Acid (Depacote)

• 20-40 mg/kg IV, may give additional 20 mg/kg

• Phenobarbital: 20 mg/kg IV, may give additional 5-10

mg/kg

• Levetiracetam: 1000-3000 mg IV

• Lacosamide: 200-400 mg IV

Treatment for Refractory SE

• Intubate

• Midazolam (Versed) infusion 0.2 mg/kg IV. Repeat 0.2-0.4 mg/kg boluses Q 5 minutes until seizure stopped. Maintenance 0.05-2.0 mg/kg/hr

• Propofol infusion 20 mcg/kg/min, titrate up to 200 mcg/kg/min.

• Pentobarbital infusion • Load 5 mg/kg IV up to 50 mg/min

• Repeat 5 mg/kg boluses until seizure stops

• Maintenance 0.5 – 10 mg/kg/hr

• Ketamine

• Therapeutic hypothermia • Seizure control and neuroprotective

Super-Refractory SE

• Seizures resistant to 3rd line therapy

• Generally poor outcome

• Control seizures with pentobarbital infusion

Initial Pre-hospital Therapy

• Early intervention most effective

• Benzodiazepines

• Airway adjuncts and O2

• IV or IO access

ED 0-5 Minutes

• Obtain VS including temperature

• Ensure open airway—administer O2 if needed

• Establish IV access

• Assess for cardio-respiratory compromise

• Brief exam to observe seizure activity and focal

neurological signs

• Fingerstick glucose

• Examine for evidence of head trauma

• Draw baseline bloodwork and toxicology screen

• 1st dose benzodiazepine, repeat in 5-10 min if seizures

continue

ED 10-45 Minutes • Additional dose lorazepam

• Loading dose AED

• Monitor EKG, pulse, BP, respiratory status while infusing drugs

• Evaluate respiratory status • Consider intubation if compromised airway or gas

exchange or suspected intracranial HTN

• Administer dextrose if hypoglycemic

• Volume resuscitation and/or vasopressor support if indicated

• Foley catheter

• Consider EEG

• Consider neuroimaging

Initial Hospital Therapy

• Continue ABCs

• Optimize blood glucose

• Continue benzodiazepines until seizure controlled

• If not controlled, add 2nd line agent

Manno. Neurohospitalist. 2011.

Outcomes Convulsive SE

• Mortality of CSE

• At hospital discharge 9 – 21 %

• At 30 days 19 - 27 %

• Morbidity of CSE

• Severe neuro or cognitive sequelae 11 – 16 %

• Deterioration in functional status 23 – 26 %

• Factors associated with worse outcomes

• Underlying etiology, development of SE in hospitalized patient,

older age, impaired consciousness, duration of SE, at onset focal

signs, medical complications, insufficient therapy

• Mortality rate for pediatric patients 3 - 11 %

Outcomes of NCSE and RSE

• Mortality in NCSE

• At hospital discharge 18 – 52 %

• At 30 days 65 %

• Mortality in RSE

• At hospital discharge 23 – 61 %

• At 3 months 39 %

References

• Brophy et al. 2012. Guidelines for the evaluation and

management of status epilepticus. Neurocritical Care Journal

17: 3-23.

• Brophy. 2015. Management of status epilepticus: Breaking

bad. Pharmacotherapy of Neurocritical Care Webcast series.

http://www.neurocriticalcare.org/news/new-release-

pharmacotherapy-neurocritical-care-series-pons-presentation

• Claassen et al. 2015. Emergency neurological life support:

Status epilepticus. Neurocritical Care Journal. Published

online.

• Laccheo et al. 2015. Non-convulsive status epilepticus and

non-convulsive seizures in neurological ICU patients.

Neurocritical Care Journal 22: 202-211.

Kay Goodall CNP

Neurocritical Care Service

Riverside Methodist Hospital

NEUROMUSCULAR EMERGENCIES

Neuromuscular Diseases

• Myasthenia Gravis

• Guillain-Barre syndrome

• Tetanus

• Envenomation –Snakes (Northern Copperhead,

Eastern Massasauga Rattlesnake, and the Eastern

Timber Rattlesnake), Spider ( Brown Recluse and

Black widow), Scorpions (rare but can be found in

Ohio), Sharks (Just seeing if you’re still listening).

• Organophosphorus poisoning – Insecticides,

herbicides, and Sarin gas

Case Study

• A patient presents to the emergency department after

having diarrhea, fever and body aches that lasted for two

weeks. He states, “I think I have a flu”. Current symptoms

include tingling in both hands and weakness in his legs.

Based on the medical history and current symptoms,

which of neurological disorder is he likely to be diagnosed

with?

• A. Myasthenia Gravis

• B. Guillain-Barre syndrome

• C. Multiple Sclerosis

• D. Parkinson's Disease

Case Study

• The correct answer is B • Rationale: Guillain-Barre syndrome (GBS), myasthenia gravis, and multiple

sclerosis are all autoimmune disorders. GBS occurs following viral or

bacterial illness, such as influenza, mononucleosis, or pneumonia. Clinical

manifestations include numbness and tingling in the hands and feet, along

with weakness in the lower extremities that ay quickly progress to paralysis

during acute phase.

• Myasthenia gravis symptoms include muscle weakness, spurred speech and

ptosis (ocular weakness) often triggered by emotional stress, menstruation,

bright sunlight, viral infection, and medications such as Cipro, beta-blockers,

and statins.

• Symptoms of multiple sclerosis include muscle cramping, bowel, bladder and

sexual dysfunction, ataxia, and tremors which manifest months or years

apart.

• Signs and symptoms of Parkinson disease include having resting tremor, gait

difficulty, reduction of sense of swell and forgetfulness.

Recognition and Triage

• What is the differential? Life threatening to trivial

• Is it non-traumatic, spinal cord, brain, infection,

psych?

• Do I need to resuscitate or pontificate?

• Start with the basics; A,B,C,D

• Airway compromise can be from weakness of

oropharyngeal muscles or the diaphragm.

• Be vigilant when assessing for facial droop, trouble

swallowing, weak cough, or poor ventilatory effort

Why is Neuromuscular Disease a

Concern?

• What are you assessing in your patient?

•Airway… And then?

•Breathing… And then?

•Circulation… And then?

•Disability

Respiratory Failure

• Are there secretions, facial drooping, or dysphasia?

• Can your patient talk to you without getting short of

breath?

• Are they breathing fast and shallow using accessory

muscles?

• Are they SOB while supine, does it get better when they

stand?

• Hallmark sign not to miss! The diaphragm and respiratory muscles

may be too weak to over come the weight of the chest wall, neck,

and abdominal contents.

Respiratory Failure - Testing

• Can they count to 20 in one breath?

• What is the resting respiratory rate?

• On going testing would include a Force Vital Capacity

(FVC), Negative inspiratory force (NIF) or Maximum

Expiratory Pressure (MEP)

• Pulse oximetry and ABGs - Although they are good tests

they show late signs of changing status.

Treatment

Myasthenia Gravis

• “Chronic auto immune disease mediated by acetylcholine

receptor(Ach) antibodies that act at the neuromuscular

junction.” (McCance, Huether, Brashers, Rote, 2010, pg.

639)

• Usually insidious onset.

• Fatigue following exertion, resolves with rest but reoccurs

with exertion. Due to the destruction of receptor sites for

the ACh on the postsynaptic membrane.

• The eventual destruction of the receptor sites cause nerve

transmission to be decreased.

• Cause is unknown.

Myasthenia Gravis

• Occurs around 20 to 30 years of age

• More frequently in females

• May have a familial connection

• Many commonly prescribed drugs (antibiotics,

psychotropic and antidysrhythmic drugs) can precipitate

an event

Clinical Manifestations

• Eyelid drooping, extra-ocular muscles weak, flaccid facial muscles, diplopia

• “Nasal speech with impaired bulbar reflexes (tongue, pharynx, larynx).

• Neck and proximal limb weakness.”

• Flower, Wainwright, Caulfield, (2015).

Testing

• CT scan r/o thymoma

• Electromyogram (EMG)

• Acetycholine receptor antibody testing, muscle specific

tyrosine kinase (MuSK)

• Edrophonium testing (Tensilon test) – prevents break

down of acetylcholine reducing muscle weakness. Edrophonium

2 mg + Edrophonium 8 mg IV. => increased muscle srenth with MG. Administer atropine if symptoms not improved

(cholinergic chrisis)

Treatment • Pyridostigmine or Neostigmine (for symptom relief not for

acute phase necessarily)

• Close observation in ICU

• NIFs (negative inspiratory force) and FVC (forced expiratory volume) frequently measured

• IVIG and/or Plasmapheresis (PLEX)

• Thymectomy if warranted

• Avoid anticholinesterase and aminoglycoside medication

• Steroids can be used at a low dose with long term goals of immunosuppression but not for acute phase.

• Caution with antiarrhythmic, neuromuscular blocking agents, certain antibiotics (aminoglycosides), thyroid hormones, and phenytoin to name a few.

Myasthenia Crisis

• Respiratory failure in the setting of MG 2/2 respiratory

muscle weakness and oropharyngeal weakness

• Life threatening, rapid decompensation due to the

pathophysiology of the disease.

• May be preceded by infection (caution with antibiotics as

C-diff can be devastating), pregnancy, medication

changes including anticholinesterase and prednisone.

• Can last two weeks.

Guillain – Barre syndrome

• Acquired acute inflammatory demyelinating or axonal polyneuropathy with four subtypes: • Acute inflammatory demyelinating polyneuropathy (AIDP)-

ascending paralysis with typically distant start, early sensory symptoms and loss of deep tendon reflexes (DTRs)

• Acute motor axonal neuropathy (AMAN) – acute progressive weakness with no sensory impairment

• Acute motor and sensory axonal neuropathy (AMSAN) – ascending paralysis

• Fisher syndrome (FS) –ophthalmoparesis, areflexia, and ataxia

• Muscles innervated by the damaged nerves may atrophy and experience denervation. If the cell body survives it may regenerate peripheral nerve and recover. If the cell body does not survive then no regeneration is possible and may lead to permanent damage.

• (McCance, Huether, Brashers, Rote, 2010, pg. 636)

GBS

Tetanus

• Caused by neurotoxin tetanospasmin, elaborated by C tetani.

• Spores of the bacteria can be found in soil. Once introduced in a

wound the vegetative bacteria can produce a protease that stops

necessary proteins from facilitating neurotransmitter release.

• Tetanospasmin interferes with neurotransmission at spinal synapses

of inhibitory neurons. As a result, minor stimuli results in uncontrolled

spasms and exaggerated reflexes.

• (Papadakis & McPhee, 2013, pg. 1429)

Clinical Manifestations • Pain, tingling, numbness at site of inoculation

• Spasticity of muscles near site of inoculation.

• Jaw, neck stiffness, dysphagia, and drooling. Spasms of the jaw muscles (trismus) or facial muscles, rigidity and spasms of the abdominal, back or neck muscles.

• Painful tonic convulsions, spasms of the glottis and respiratory muscles causing ASPHYXIA.

• Oh by the way, the patient is awake and alert through the whole illness!

Treatment

• Prevention with tetanus and diphtheria toxoid.

• Human tetanus immune globulin within 24 hours of

presentation.

• Reduce stimuli, bed rest.

• Full ventilatory support, sedation, paralysis.

• PCN to eradicate toxin-producing organisms.

Questions

References

Flower, O., Wainwright, M., Caulfield, A.F., (2015). Emergency Neurological Life

Support: Actue Non-traumatic Weakness. Neurocritical Care Society.

DOI 10.1007/s12028-015-0160-7

McCance, K., Huether, S., Brashers, V., Rote, N., (2010). Pathophysiology: The

biologic basis of disease in adults and children (6th ed). Philadelphia,

PA. Elsevier

Papadakis, M., & McPhee, S., (2013). Current medical diagnosis & treatment

2013 (52ed). McGraw-Hill. New York, NY.

ENLS. 2017. OhioHealth Neuroscience Symposium.

ENS’s CEN Online Review. 2017.

Strokes

Head Trauma