Rosen & Barkin's 5-Minute Emergency Medicine Consult (461 page)

CODES

203.00 Multiple myeloma, without mention of having achieved remission

BASICS

• 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
    

• 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

• 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: Focused on “hard” neurologic signs:

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

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

CSF analysis:

• CSF gel electrophoresis reveals “oligoclonal bands” not present in serum
  
  • Oligoclonal bands are found in ≥95% of patients with clinically definite MS
    

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)
  

Sensory-evoked potential testing:

• Not an ED test
  

• 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–Barré 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

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

• 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
    

• 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

• 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
  

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

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