Characterized by chronic inflammation and selective destruction of CNS myelin; peripheral nervous system is spared. Pathologically, the multifocal scarred lesions of multiple sclerosis (MS) are termed plaques. Etiology is autoimmune, with susceptibility determined by genetic and environmental factors. MS affects >350,000 in the United States and 2.5 million worldwide; onset is often in early to middle adulthood, and women are affected three times as often as men.
Onset may be abrupt or insidious. Some pts have symptoms that are so trivial that they may not seek medical attention for months or years. Recurrent attacks of focal neurologic dysfunction lasting weeks or months and followed by variable recovery, are typical; some pts initially present with slowly progressive neurologic deterioration. Symptoms often transiently worsen with fatigue, stress, exercise, or heat. Manifestations include weakness and/or sensory symptoms, visual difficulties, abnormalities of gait and coordination, urinary urgency or frequency, and abnormal fatigue. Motor involvement can present as a heavy, stiff, weak, or clumsy limb. Localized tingling, “pins and needles,” and “dead” sensations are common. Optic neuritis produces monocular blurring of vision, especially in the central visual field, often with associated retro-orbital pain accentuated by eye movement. Involvement of the brainstem may result in diplopia, nystagmus, vertigo, or facial pain, numbness, weakness, hemispasm, or myokymia (rippling muscular contractions). Ataxia, tremor, and dysarthria may reflect disease of cerebellar pathways. Lhermitte’s symptom, a momentary electric shock–like sensation evoked by neck flexion, indicates disease in the cervical spinal cord. Diagnostic criteria are listed in Table 190-1; MS mimics are summarized in Table 190-2.
TABLE 190-1DIAGNOSTIC CRITERIA FOR MULTIPLE SCLEROSIS (MS) |Favorite Table|Download (.pdf) TABLE 190-1DIAGNOSTIC CRITERIA FOR MULTIPLE SCLEROSIS (MS)
|Clinical Presentation ||Additional Data Needed for MS Diagnosis |
|2 or more attacks; objective clinical evidence of 2 or more lesions or objective clinical evidence of 1 lesion with reasonable historical evidence of a prior attack ||None |
|2 or more attacks; objective clinical evidence of 1 lesion || |
Dissemination in space, demonstrated by
• ≥1 T2 lesion on MRI in at least 2 out of 4 MS-typical regions of the CNS (periventricular, juxtacortical, infratentorial, or spinal cord)
• Await a further clinical attack implicating a different CNS site
|1 attack; objective clinical evidence of 2 or more lesions || |
Dissemination in time, demonstrated by
• Simultaneous presence of asymptomatic gadolinium-enhancing and nonenhancing lesions at any time
• A new T2 and/or gadolinium-enhancing lesion(s) on follow-up MRI, irrespective of its timing with reference to a baseline scan
• Await a second clinical attack
|1 attack; objective clinical evidence of 1 lesion (clinically isolated syndrome) || |
Dissemination in space and time, demonstrated by:
For dissemination in space
• ≥1 T2 lesion in at least 2 out of 4 MS-typical regions of the CNS (periventricular, juxtacortical, infratentorial, or spinal cord)
• Await a second clinical attack implicating a different CNS site
For dissemination in ...