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Case 2

46y female with acute bilateral facial muscle weakness, ptosis, dysarthria, followed by bilateral upper limb weakness. Blurred vision but no other sensory symptoms. No spasticity, decrease deep tendon reflexes.

Vitals: Normal

GCS: 15/15

Perceived

Weakness

Perceived weakness - The patient feels that more energy is required to exert a given amount of force, when in fact the actual muscle strength is normal.

Paresis

Paralysis is the loss of voluntary movement because of interruption of one of the motor pathways at any point from the cerebrum to the muscle fiber.

Paresis is a lesser degree of paralysis.

Clinical Findings

Strength - diminished to absent

Reflexes - increased*

Sensory - Variable

Tone - Increased (spasticity)

Gait - Spastic

Babinski - up going plantar

Clinical Findings

Strength - decreased (LE > UE)

Reflexes - decreased to absent

Sensory - Very common*

Tone - decreased to flaccid

Gait - Normal to unsteady*

Babinski - down going plantars

Other - Autonomic dysfunction

Clinical Findings

Strength - LE > UE > Oculobulbar

Reflexes - Normal*

Sensory - Normal

Tone - Normal

Babinski - down going plantar

Other - Muscle fatigue with repetition

Clinical Findings

Strength - Decreased (prox > dist)

Reflexes - Depends on severity

Sensory - Normal

Tone - Normal

Babinski - Down going plantar

Other - Tenderness on palpation*

- Acute monophasic illness provoked by previous infection

- Rapidly progressive secondary to autoimmune process

- Demyelinating / Axonal Polyneuropathy

Cardinal clinical features of GBS

- Progressive, symmetric muscle weakness

- Absent or depressed deep tendon reflexes.

- Respiratory failure in about 30 percent,

- Dysautonomia occurs in 70% of patients.

- Progresses over 2 weeks on average

- Facial weakness (50%) ocular muscles (15%)

- Often a prominent severe back pain

- Sensory disturbance in 80% (often mild)

- Has multiple variants (AIDP, Miller Fisher, AMAN)

Investigations

LP - Elevated protein count with normal WBC (albuminocytologic dissociation)

EMG/NCS - Evidence of either demyelinating or axonal neuropathy (depends on the variant)

Systemic Antibodies - GQ1b useful for Miller Fisher variant; otherwise of no clinical utility.

Prognosis

Clinical course with no treatment

- Progression of disease up to 2 weeks

- Plateau for 2 weeks

- Recovery 'weeks' to 'months'

Long term

- 85% complete recovery

- 10% delayed and incomplete recovery

- 5% die

- 10% recurrence

Botulism is a neuroparalytic syndrome resulting from the action of a neurotoxin elaborated by the bacterium Clostridium botulinum, .a heterogeneous group of gram-positive, rod-shaped, spore-forming, obligate anaerobic bacteria.

Cardinal clinical features of Botulism

- Acute onset of bilateral cranial neuropathies

- Symmetric descending weakness

- Absence of fever

- The patient remains responsive

- No sensory deficit, except for blurred vision

- Heart Rate normal to slow

- Five forms of botulism exist, including food borne botulism, infant botulism, wound botulism, adult enteric infectious botulism, and inhalation botulism

Investigations

Generally not applicable; clinical diagnosis

Non specific blood markers

spores and/or toxin can be isolated

Prognosis

Requires 1 - 3 months of hospitalization regardless of the type of botulism

Ventilator support in 20-30%

Long term

- 5 - 8% mortality

- 1% newborn mortality

- long term sequelae of perceived weakness 25%

?

Approach to Acute Weakness in Adults presenting to The Emergency Department

Case 1

32y male with progressive (6 days), bilateral muscle weakness (lower extremity > upper extremity), and paresthesia x 4 limbs, accompanied by absent deep tendon reflexes. Bladder retention.

Vitals: Normal

GCS: 15/15

Guillain Barre Syndrome (GBS)

Botulism

"I feel weak"

Treatment

Supportive Therapy

Intubate if impending respiratory failure

Analgesia for neuropathic pain (50%)

Plasma Exchange or IVIG

No role for steroids

Treatment

Supportive Therapy

Intubate if impending respiratory failure

Anti-toxin (Equine serum botulism antitoxin and Human-derived botulinum immune globulin)

Pen G if wound infection (metronidazole if Pen allergy

Manage accordingly

Step 1

Reversible

Causes

Step 1

Compromised

Reversible

Causes

Compromised

Perceived

Paresis

Assess ABC

Assess Level of Consciousness

Shock

Severe Metabolic

Severe Endocrine

Perceived

Paresis

Massive cerebral stroke

Thalamic Stroke/lesion

Brainstem stroke/lesion

Cord inflammation/compression

Guillen Barre Syndrome

Tick Paralysis

Organophosphate toxicity

Botulism

Myopathic Crisis

Assess ABC

Assess Level of Consciousness

Shock

Severe Metabolic

Severe Endocrine

Massive cerebral stroke

Thalamic Stroke/lesion

Brainstem stroke/lesion

Cord inflammation/compression

Guillen Barre Syndrome

Tick Paralysis

Organophosphate toxicity

Botulism

Myopathic Crisis

Normal

Step 2

Perceived

Correct underlying

Cause and revaluate

Perceived

Perceived Weakness vs Paresis

Correct underlying

Cause and revaluate

Perceived Weakness vs Paresis

UMN

Paresis

Cortical - often associated with aphasia, neglect, visual field deficit

Subcortical - often purely motor. Ataxia and 'shaking hand'.

Brainstem - associated with CN deficit (contralateral)

Cord - Rarely pure motor; often associated with sensory level and bowel/bladder dysfunction.

Step 3

Unilateral

Step 3

Unilateral

Unilateral vs Bilateral

Unilateral

Unilateral vs Bilateral

LMN

Physical Exam*

Bilateral

Physical Exam*

Bilateral

Radiculopathy

Plexopathy

Peripheral nerve injury/entrapment

Step 4

UMN

Step 4

UMN

Cortical / Subcortical - Should have been identified on Step 1.

Brainstem - associated with CN deficit (potentially life threatening)

Cord - Rarely pure motor; often associated with sensory level and cord level plegia +/- bowel / bladder signs.

UMN vs non - UMN

Cortical / Subcortical - Should have been identified on Step 1.

Brainstem - associated with CN deficit (potentially life threatening)

Cord - Rarely pure motor; often associated with sensory level and cord level plegia +/- bowel / bladder signs.

UMN vs non - UMN

Non - UMN

Step 5

Guillain Barre

Tick Paralysis

Yes

Step 5

Guillain Barre

Tick Paralysis

Yes

CSF - Normal

Normal sensory exam

Acute course

Variable (single limb to full)

Tick present on PE

CSF - Abnormal

Objective sensory deficit

Subacute course

Always bilateral

No ticks on PE

bladder/bowel common

LMN Signs - LE > UE, sensory symptoms, and dysautonomia

CSF - Normal

Normal sesnory exam

Acute course

Variable (single limb to full)

Tick present on PE

CSF - Abnormal

Objective sensory deficit

Subacute course

Always bilateral

No ticks on PE

bladder/bowel common

LMN Signs - LE > UE, sensory symptoms, and dysautonomia

*Consider Toxins:

Arsenic poisoning / hexane / Thallium / mercury / lead

*Consider Toxins:

Arsenic poisoning / hexane / Thallium / murcury / lead

No

Step 6

Yes

Step 6

Yes

Neuromuscular Junction

Disorders

*Early Ocular and Bulbar weakness

Fatiguibale on excertion

Proximal > distal weakness

*Early Ocular and Bulbar weakness

Fatigable on exertion

Proximal > distal weakness

No

Muscular Pathology

Step 7

Yes

Muscular Pathology

Step 7

Yes

Myositis (inflamatory vs viral)

Alcoholic myopathy

- Asses heart function *

Weakness Proximal > Distal +

No sensory signs +/- myalgia

Myositis (inflamatory vs viral)

Alcoholic myopathy

- Asses heart function *

Weakness Proximal > Distal +

No sensory signs +/- myalgia

Shock /

Relative ischemia

Cardiogenic

Obstructive

Volume depletion

Distributive

Impaired delivery

Psychogenic

Inflammatory

Infection

Cancer

Anxiety / Depression

Fibromyalgia

Chronic fatigue Syndrome

Conversion Disorder *

Malingering

Rheumatologic

Allergy

Metabolic/

endocrine

Hypothyroid

Adrenal

Azotemia

Sodium

Ethanol

Medications

Hyperthyroid *

Glucose *

Potassium *

Calcium

Phosphate

Magnesium

Ethanol

Medications

Acute

Weakness

Manage accordingly

Step 1

Reversible

Causes

Compromised

Paresis

Perceived

Assess ABC

Assess Level of Consciousness

Shock

Severe Metabolic

Severe Endocrine

Massive cerebral stroke

Thalamic Stroke/lesion

Brainstem stroke/lesion

Cord inflammation/compression

Guillen Barre Syndrome

Tick Paralysis

Organophosphate toxicity

Botulism

Myasthenic Crisis

Normal

Step 2

Perceived

Correct underlying

Cause and reevaluate

Perceived Weakness vs Paresis

UMN

Paresis

Cortical - often associated with aphasia, neglect, visual field deficit

Subcortical - often purely motor. Ataxia and 'shaking hand'.

Brainstem - associated with CN deficit (contralateral)

Cord - Rarely pure motor; often associated with sensory level and bowel/bladder dysfunction.

Step 3

Unilateral

Unilateral vs Bilateral

LMN

Physical Exam*

Bilateral

Radiculopathy

Plexopathy

Peripheral nerve injury/entrapment

Step 4

UMN

Cortical / Subcortical - Should have been identified on Step 1.

Brainstem - associated with CN deficit (potentially life threatening)

Cord - Rarely pure motor; often associated with sensory level and cord level plegia +/- bowel / bladder signs.

UMN vs non - UMN

Non - UMN

Step 5

Guillain Barre

Tick Paralysis

Yes

CSF - Normal

Normal sensory exam

Acute course

Variable (single limb to full)

Tick present on PE

CSF - Abnormal

Objective sensory deficit

Subacute course

Always bilateral

No ticks on PE

bladder/bowel common

LMN Signs - LE > UE, sensory symptoms +/- autonomic dysfunction?

*Consider Toxins:

Arsenic poisoning / hexane / Thallium / mercury / lead

No

Step 6

Yes

Neuromuscular Junction

Disorders

*Early Oculobulbar weakness

Fatigable on exertion

Proximal > distal weakness

No

Step 7

Yes

Muscular Pathology

Weakness Proximal > Distal +

No sensory signs +/- myalgia

"The Thinkers"

Cerebral Cortex

Subcortical tract

corona radiata

posterior limb of the internal capsule

Upper Motor

Neuron

Differential Diagnosis

Brain Stem

Ischemia

Hemorrhage

Tumor

Infections (Polio/ West Nile)

Inflammatory (MS / transverse myelitis)

Migraine - hemiplegic (rare)

Post-ictal (Todd's) paralysis

Oculobulbar - exit on the

contralateral side.

Decussation

Medulla

Spinal Cord

Transition of the UMN

to LMN - anterior horn

Muscular

Lower Motor

Neuron

Differential Diagnosis

Inflammatory Myopathy

Rhabdomyolisis

Differential Diagnosis

Radiculopathy

- Direct compression/impingement

- Lyme disease (late in disease)

Plexopathy

- Direct compression/impingement

- Tick Paralysis*

Neuropathy (poly/mono)

- Guillain Barre

- Diphtheria

- Arsenic / hexane / Thallium / mercury / lead / Tick exotoxin

- vasculitis/sarcoidosis

- Porphyria / paraneoplastic

Myofibril

Muscle fiber

Muscle fascicle

Muscle

Tendon

Neuromuscular

Junction

Differential Diagnosis

Botulism / Snake Venom

Hypocalcemia / Hypermagnesemia

Organophosphate / Carbamates poisoning

Myasthenia Gravis/ Myasthenic Crisis

Tick Paralysis* (LMN)

Ehab Eshtaya, MD

Objectives

Gain an algorithmic approach to 'acute weakness' as a presenting complaint in the Emergency Department

Basic understanding of the clinical presentation and differential diagnosis for the different components of the Neuromuscular pathway

Highlights of Guillain Barre Syndrome and Botulism as example of LMN and NMJ disorders.

Aknowledgments

Dr Fisher - Internal Medicine Staff

Dr Smyth - Neurology Staff

Dr Lefebvre - Emergency Medicine

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