Multiple sclerosis
Background
- CNS myelin destruction causes variable motor, sensory, visual and cerebellar dysfunction[1]
Types
- Relapsing/remitting (most common)
- Relapse (days-months) followed by remission
- Secondary progressive
- Relapses and partial recoveries occur, but disability does not fade away between cycles
- Primary progressive
- Symptoms progress slowly and steadily without remission
- Progressive relapsing
- Similar to primary progressive but with superimposed flares
Clinical Features
- Classic patient has multiple presentations for neuro symptoms of different areas of pathology
- Patient often has resolution of the earlier symptoms
- Symptoms worsen with increases in body temperature, classically after hot showers (Uhthoff's phenomenon[2])
- Muscle/sensory signs:
- Lower extremity weakness usually worse than upper extremity
- Upper motor neuron signs:
- Hyperreflexia
- Positive Babinski
- Decrease in proprioception/pain/temperature sensation
- Lhermitte sign
- Electric shock sensation radiating down back into arms/egs from neck flexion
- If the discomfort is severe, carbamazepine or gabapentin may be beneficial for some patients.
- Optic neuritis
- Initial sign in 30% of patients
- Vision loss (usually unilateral) often preceded by retrobulbar pain
- Blurred vision
- Afferent pupillary defect is suggestive of optic neuritis
- Nystagmus
- Diplopia
- Internuclear ophthalmoplegia
- Abnormal eye adduction bilaterally and horizontal nystagmus
- Convergence (both eyes center medially) is preserved
- Dysautonomia
- Urinary retention (increased risk of UTI/pyelo)
- Constipation or incontinence
- Sexual dysfunction (males)
Differential Diagnosis
Weakness
- Neuromuscular weakness
- Upper motor neuron:
- CVA
- Hemorrhagic stroke
- Multiple sclerosis
- Amyotrophic Lateral Sclerosis (ALS) (upper and lower motor neuron)
- Lower motor neuron:
- Spinal and bulbar muscular atrophy (Kennedy's syndrome)
- Spinal cord disease:
- Infection (Epidural abscess)
- Infarction/ischemia
- Trauma (Spinal Cord Syndromes)
- Inflammation (Transverse Myelitis)
- Degenerative (Spinal muscular atrophy)
- Tumor
- Peripheral nerve disease:
- Neuromuscular junction disease:
- Muscle disease:
- Rhabdomyolysis
- Dermatomyositis
- Polymyositis
- Alcoholic myopathy
- Upper motor neuron:
- Non-neuromuscular weakness
- Can't miss diagnoses:
- ACS
- Arrhythmia/Syncope
- Severe infection/Sepsis
- Hypoglycemia
- Periodic paralysis (electrolyte disturbance, K, Mg, Ca)
- Respiratory failure
- Emergent Diagnoses:
- Symptomatic Anemia
- Severe dehydration
- Hypothyroidism
- Polypharmacy
- Malignancy
- Aortic disease - occlusion, stenosis, dissection
- Other causes of weakness and paralysis
- Acute intermittent porphyria (ascending weakness)
- Can't miss diagnoses:
Evaluation
- CSF
- Elevated protein and gamma-globulin (increased oligoclonal bands)
- MRI
- Multiple lesions in supratentorial white matter, paraventricular area, spinal cord
- MRI with GAD of brain (+/- spine) and orbits (if optic neuritis suspected)
- CBC, Chemistry
- Urinalysis
Management
- Fever must be reduced to minimize weakness associated with elevated temperature
Infection Treatment
- Antibiotics or surgical control of any infectious sources
Steroids and Immunomodulators
- High-dose steroid therapy for relapses in the form of oral or intravenous methylprednisolone (1000 mg)[3]
- Suppression therapies
- IFN B, Glatiramer, Estriol (usually not in ED)
Disposition
- Hospitalization indicated for:
- Any disease exacerbation associated with significant morbidity
- IV antibiotics or steroid therapy required
- Depression and significant risk of suicide
See Also
References
- ↑ Multiple sclerosis: a practical overview for clinicians. British Medical Bulletin, Volume 95, Issue 1, September 2010, Pages 79–104. https://doi.org/10.1093/bmb/ldq017
- ↑ Flensner G, et al. "Sensitivity to heat in MS patients: a factor strongly influencing symptomology-an explorative survey". BMC Neurol. 2011. 11:27.
- ↑ Le Page et al. Oral versus intravenous high-dose methylprednisolone for treatment of relapses in patients with multiple sclerosis (COPOUSEP): a randomised, controlled, double-blind, non-inferiority trial. Lancet. 2016 Jan 23;387(10016):340. https://core.ac.uk/download/pdf/52993687.pdf