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Multiple Sclerosis, Emergency Medicine

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Multiple Sclerosis, Emergency Medicine

Basics

Description

Physical exam: Focused on "hard " пїЅ neurologic signs: пїЅ пїЅ

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

Sensory-evoked potential testing: пїЅ пїЅ

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

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

  • 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 moPrimary progressive MS: Slow or stepwise progression over at least 6 moSecondary progressive MS: Initial exacerbations and remissions followed by slow progression over at least 6 moStable MS: No progression (without treatment) over at least 18 mo
  • Signs and symptoms depend on location of lesions and timing of demyelination
  • 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 " пїЅmediated 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
  • Etiology is not well understood
  • Characterized by infiltrate of T cells and macrophages

Diagnosis

Signs and Symptoms

  • Initial attacks usually ( пїЅ пїЅ пїЅ85%) 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 lossParesthesias (or changed sensory level) in 1 limbLimb (usually leg) weaknessDiplopia:Intranuclear ophthalmoplegia from lesion of medial longitudinal fasciculusResults in unilateral or bilateral paralysis of adduction of eye on horizontal gazeTrigeminal neuralgiaUrinary retentionVertigoTransverse myelitis:Acute onset of motor and sensory findings at specific spinal cord levelOften associated with bladder or bowel incontinenceCan be early manifestation of MSUnusual 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 " пїЅ8 wk
  • Most common in young women of Northern European descent:Increased risk in 1st-degree relativesPeak age: 30 yrFemale-to-male ratio: 2:1
  • Pain is an uncommon symptom in MS:Exceptions: Trigeminal neuralgia, early optic neuritis
  • 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 fasciculusResults 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 levelOften associated with bladder or bowel incontinenceCan be early manifestation of MSUnusual initial manifestations include psychosis, aphasia, etc.Initial symptoms may be minor and only be recognized as due to MS in retrospect
  • Eye pain exacerbated by movement progressing to visual loss
  • Intranuclear ophthalmoplegia from lesion of medial longitudinal fasciculus
  • Results in unilateral or bilateral paralysis of adduction of eye on horizontal gaze
  • 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
  • Increased risk in 1st-degree relatives
  • Peak age: 30 yr
  • Female-to-male ratio: 2:1
  • Exceptions: Trigeminal neuralgia, early optic neuritis
  • 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
  • Requires observation over time and confirmatory testing

Diagnosis Tests & Interpretation

  • CSF gel electrophoresis reveals "oligoclonal bands " пїЅ not present in serumOligoclonal bands are found in ≥95% of patients with clinically definite MS
  • Oligoclonal bands are found in ≥95% of patients with clinically definite MS
  • 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)
  • 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 MSSeek history of rash and tick exposure in geographic areas of high riskLyme titers may aid in diagnosis
  • Psychiatric illness is diagnosis of exclusion
  • Postinfectious or postimmunization demyelination may mimic MS, usually in children
  • Guillain " пїЅBarre is usually ascending and symmetric and progresses over hours to days
  • MS unlikely in patients with:Normal neurologic examAbrupt hemiparesis (stroke)Aphasia (stroke)Pain predominatingVery brief symptoms (seconds to minutes)Age <10 or >50 yr
  • Diffuse symptoms (seizures, syncope, and dementia) are seldom due to MS
  • Usually older patients with risk factors for atherosclerotic disease or atrial fibrillation
  • CNS involvement usually in setting of known disease and usually nonfocal
  • CNS manifestations usually with known disease and lung involvement
  • May mimic MS
  • Seek history of rash and tick exposure in geographic areas of high risk
  • Lyme titers may aid in diagnosis
  • 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

Ed Treatment/Procedures

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

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 " пїЅ200 mg PO daily in AM
  • Oxybutynin: 5 mg PO BID to TID
  • Tizanidine: 2 " пїЅ8 mg PO TID

Follow-Up

Disposition

  • 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
  • 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

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 пїЅ пїЅLJ. Clinical practice. Optic neuritis. N Eng J Med. 2006;354:1273 " пїЅ1280.
  • Burton пїЅ пїЅJM, O 'Connor пїЅ пїЅPW, Hohol пїЅ пїЅM, et al. Oral versus intravenous steroids for treatment of relapses in multiple sclerosis. Cochrane Database Syst Rev. 2012;12:CD006921.
  • Courtney пїЅ пїЅAM, Treadaway пїЅ пїЅK, Remington пїЅ пїЅG, et al. Multiple sclerosis. Med Clin North Am. 2009;93:451 " пїЅ476, ix " пїЅx.
  • Filippi пїЅ пїЅM, Rocca пїЅ пїЅMA. MR imaging of multiple sclerosis. Radiology. 2011;259:659 " пїЅ681.
  • Frohman пїЅ пїЅEM, Racke пїЅ пїЅMK, Raine пїЅ пїЅCS. Multiple sclerosis " пїЅ the plaque and its pathogenesis. N Engl J Med. 2006;354:942 " пїЅ955.
  • Leary пїЅ пїЅSM, Porter пїЅ пїЅB, Thompson пїЅ пїЅAJ. Multiple sclerosis: Diagnosis and the management of acute relapses. Postgrad Med J. 2005;81:302 " пїЅ308.

See Also (Topic, Algorithm, Electronic Media Element)

  • Cerebrovascular Accident
  • Guillain " пїЅBarre Syndrome
  • Lyme Disease

Codes

ICD9

ICD10

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)