Multiple Sclerosis, Emergency Medicine

Basics

Description

Pathophysiology: Recurrent episodes of CNS demyelination:
- Signs and symptoms depend on location of lesions and timing of demyelination
Multiple sclerosis (MS) occurs in distinct patterns:
- Relapsing recurring MS: 2 or more episodes lasting ≥24 hr separated by ≥1 mo
- Primary progressive MS: Slow or stepwise progression over at least 6 mo
- Secondary progressive MS: Initial exacerbations and remissions followed by slow progression over at least 6 mo
- Stable MS: No progression (without treatment) over at least 18 mo

Etiology

MS is a chronic demyelinating disease of CNS:
- Etiology is not well understood
Presumed to be T cell " �mediated autoimmune disease
There is evidence for a viral trigger
Plaques in white matter:
- Characterized by infiltrate of T cells and macrophages
Persons of northern European origin most often affected (in US)
Increased prevalence is seen moving away from equator

Diagnosis

Signs and Symptoms

Initial attacks usually ( � � �85%) represent single lesions, are abrupt in onset, and are seen in characteristic patterns (in order of decreasing frequency):
- Optic neuritis:
  - Eye pain exacerbated by movement progressing to visual loss
- Paresthesias (or changed sensory level) in 1 limb
- Limb (usually leg) weakness
- Diplopia:
  - Intranuclear ophthalmoplegia from lesion of medial longitudinal fasciculus
  - Results in unilateral or bilateral paralysis of adduction of eye on horizontal gaze
- Trigeminal neuralgia
- Urinary retention
- Vertigo
- Transverse myelitis:
  - Acute onset of motor and sensory findings at specific spinal cord level
  - Often associated with bladder or bowel incontinence
  - Can be early manifestation of MS
  - Unusual initial manifestations include psychosis, aphasia, etc.
  - Initial symptoms may be minor and only be recognized as due to MS in retrospect
Symptoms typically develop abruptly (minutes to hours) and last 6 " �8 wk
Most common in young women of Northern European descent:
- Increased risk in 1st-degree relatives
- Peak age: 30 yr
- Female-to-male ratio: 2:1
Pain is an uncommon symptom in MS:
- Exceptions: Trigeminal neuralgia, early optic neuritis

Physical Exam
Physical exam: Focused on "hard " � neurologic signs: � �

Focal neurologic deficits
Afferent pupillary defect
Internuclear ophthalmoplegia
Sensory level or sphincter disturbance (transverse myelitis)

Essential Workup

MS is suspected based on history and physical exam
Definitive diagnosis is not typically made in ED:
- Requires observation over time and confirmatory testing

Infection, especially with fever, is a common cause of MS exacerbation � �

Diagnosis Tests & Interpretation

Lab
CSF analysis: � �

CSF gel electrophoresis reveals "oligoclonal bands " � not present in serum
- Oligoclonal bands are found in ≥95% of patients with clinically definite MS

Imaging
MRI most useful imaging test: � �

Lesions appear as areas of high signal, in cerebral white matter or spinal cord on T2-weighted images
Abnormal in almost all patients who have clinically diagnosed MS
McDonald diagnostic criteria for MS include specific MRI findings
May also see plaques on CT (less sensitive)

Diagnostic Procedures/Surgery
Sensory-evoked potential testing: � �

Not an ED test

Differential Diagnosis

Signs and symptoms of MS are usually focal:
- Diffuse symptoms (seizures, syncope, and dementia) are seldom due to MS
Cerebrovascular accident and transient ischemic attack:
- Usually older patients with risk factors for atherosclerotic disease or atrial fibrillation
Systemic lupus erythematosus:
- CNS involvement usually in setting of known disease and usually nonfocal
Sarcoid:
- CNS manifestations usually with known disease and lung involvement
Lyme disease:
- May mimic MS
- Seek history of rash and tick exposure in geographic areas of high risk
- Lyme titers may aid in diagnosis
Psychiatric illness is diagnosis of exclusion
Postinfectious or postimmunization demyelination may mimic MS, usually in children
Guillain " �Barre is usually ascending and symmetric and progresses over hours to days
MS unlikely in patients with:
- Normal neurologic exam
- Abrupt hemiparesis (stroke)
- Aphasia (stroke)
- Pain predominating
- Very brief symptoms (seconds to minutes)
- Age <10 or >50 yr

Treatment

Initial Stabilization/Therapy

Fever in MS patients is treated aggressively because it can worsen the manifestations of MS � �

Ed Treatment/Procedures

Acute optic neuritis:
- High-dose parenteral steroids
- Oral steroids relatively contraindicated (has been reported to increase recurrence risk)
Exacerbations:
- High-dose IV methylprednisolone (up to 1 g/day) or other parenteral corticosteroid
- High-dose oral steroids are sometimes used (except for optic neuritis)
Symptomatic treatment:
- Spasticity: Baclofen, tizanidine
- Tremor: Clonazepam
- Urinary symptoms:
  - Diagnose and treat infection
  - Self-catheterization
  - Oxybutynin may promote continence
- Trigeminal neuralgia: Carbamazepine
- Fatigue, general weakness: Amantadine, methylphenidate, and modafinil have been used
- Depression: SSRIs are effective

Medication

Amantadine: 100 mg PO BID
Baclofen: 10 mg PO TID initially; may increase to 25 mg PO TID
Carbamazepine: 100 mg PO BID to 200 mg PO QID
Clonazepam: 0.5 mg/d PO, increase in 0.5-mg increments and up to 3 times a day
Methylprednisolone: 1 g IV daily (1st-line treatment)
Modafinil: 100 " �200 mg PO daily in AM
Oxybutynin: 5 mg PO BID to TID
Tizanidine: 2 " �8 mg PO TID

Follow-Up

Disposition

Admission Criteria

Acute exacerbation that requires IV therapy
Patients unable to care for themselves due to severity of their illness
Another condition requiring inpatient treatment cannot be effectively ruled out

Discharge Criteria

Suspected MS: Patients may be referred for outpatient evaluation if their general condition permits and other serious conditions requiring admission have been effectively ruled out
Complication of known MS: Discharge if effective outpatient treatment is available for complication or exacerbating factor

Followup Recommendations

Patients with suspected MS should be referred to their primary care provider or a neurologist for further evaluation � �

Pearls and Pitfalls

Signs and symptoms of MS are usually focal
Diffuse symptoms are rarely MS
Oral steroids are contraindicated
Treat fever in MS patients aggressively

Additional Reading

Balcer � �LJ. Clinical practice. Optic neuritis. N Eng J Med. 2006;354:1273 " �1280.
Burton � �JM, O 'Connor � �PW, Hohol � �M, et al. Oral versus intravenous steroids for treatment of relapses in multiple sclerosis. Cochrane Database Syst Rev. 2012;12:CD006921.
Courtney � �AM, Treadaway � �K, Remington � �G, et al. Multiple sclerosis. Med Clin North Am. 2009;93:451 " �476, ix " �x.
Filippi � �M, Rocca � �MA. MR imaging of multiple sclerosis. Radiology. 2011;259:659 " �681.
Frohman � �EM, Racke � �MK, Raine � �CS. Multiple sclerosis " � the plaque and its pathogenesis. N Engl J Med. 2006;354:942 " �955.
Leary � �SM, Porter � �B, Thompson � �AJ. Multiple sclerosis: Diagnosis and the management of acute relapses. Postgrad Med J. 2005;81:302 " �308.

Codes

ICD9

340 Multiple sclerosis � �

ICD10

G35 Multiple sclerosis � �

SNOMED

24700007 Multiple sclerosis (disorder)
230372003 Acute relapsing multiple sclerosis (disorder)
428700003 Primary progressive multiple sclerosis
425500002 secondary progressive multiple sclerosis (disorder)
426373005 relapsing remitting multiple sclerosis (disorder)

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