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disorders that occur with higher than expected incidence in patients with MS The retinal vascular sheathing is due to T-cell in ltration, identical to that in typical plaques, but this is an anomalous nding, since the retina usually contains no myelinated bers (Lightman et al) The treatment of optic neuritis is discussed further on Acute Myelitis (Transverse Myelitis) This is the common designation for an acutely evolving in ammatory-demyelinative lesion of the spinal cord, which proves in many but not all instances to be an expression of MS In this sense the myelitic lesion is analogous to that of optic neuritis The term transverse in relation to the myelitis is somewhat imprecise, implying that all of the elements in the cord are involved in the transverse plane, usually over a short vertical extent Instead, in MS, the spinal cord signs are asymmetrical and incomplete and involve only a part of the long ascending and descending tracts, ie, paraplegia and complete sensory loss are unusual Clinically, the illness is characterized by a rapidly evolving (several hours or days) symmetrical or asymmetrical paraparesis or paraplegia, ascending paresthesias, loss of deep sensibility in the feet, a sensory level on the trunk, sphincteric dysfunction, and bilateral Babinski signs The CSF shows a modest number of lymphocytes and increase in total protein but both may be normal early in the illness As many as one-third of patients report an infectious illness in the weeks preceding the onset of neurologic symptoms, in which case a monophasic postinfectious demyelinating disease rather than MS is the likely cause of the myelitis The MRI usually shows indications of focal demyelination in the spinal cord at the appropriate level and there may be enhancement with gadolinium infusion, but neither of these ndings is invariable The lesions, as shown in Fig 36-1 (right panel), are almost indistinguishable from those of postinfectious myelitis In those instances associated with existing MS, even if not previously symptomatic, MRI of the cerebral hemispheres will show lesions consistent with demyelination; the absence of such lesions, however, does not assure that the illness is monophasic and will not later turn out to be MS We have found that fewer than half the patients have evidence of an asymptomatic demyelinative lesion elsewhere in the nervous system or develop clinical evidence of dissemination within 5 years of the initial attack of acute myelitis (Ropper and Poskanzer) Not entirely in accord with our experience is the analysis of subgroups in a trial of interferon therapy conducted by Beck and colleagues (2002), in which the cumulative probability of developing MS after 2 years was similar after either optic neuritis or transverse myelitis Our sense has been that acute transverse myelitis is somewhat less often an initial expression of MS than is optic neuritis A special problem is presented by patients with recurrent myelitis at one level of the spinal cord but in whom no other signs of demyelinative disease can be found by careful clinical examination or MRI Some of them may even have oligoclonal bands in the CSF, which are commonly associated with MS (see further on) Enough cases of this limited nature have come to our attention to permit the conclusion that there is a recurrent form of spinal cord MS in which cerebral dissemination is infrequent (Tippet et al) It should be mentioned that isolated recurrent myelitis or myelopathy occurs also with lupus erythematosus, sarcoid, Sjogren syndrome, mixed connective tissue disease, and the antiphospholipid antibody syndrome or in the presence of other autoantibodies, as well as with dural and cord vascular stulas and arteriovenous malformations An analogous situation pertains in respect to some instances.

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of optic neuritis repeated attacks that remain con ned to the optic nerve Other aspects of transverse myelitis are discussed in Chap 44, Diseases of the Spinal Cord, and under Acute Disseminated Encephalomyelitis later in this chapter Other Patterns of Multiple Sclerosis (See also Variants of MS, page 780) Like the modes of onset cited above, other early manifestations of MS, in descending order of frequency, are unsteadiness in walking, brainstem symptoms (diplopia, vertigo, vomiting, etc), paresthesias or numbness of an entire arm or leg, facial pain often simulating tic doloreoux, and disorders of micturition Vertigo of central type is also a frequent initial sign of MS, but it more often appears in established cases Discrete manifestations such as hemiplegia, trigeminal neuralgia or other pain syndromes, facial paralysis, deafness, or seizures occur in a small proportion of cases Most often the disease presents with more than one of the aforementioned symptoms almost simultaneously or in rapid succession Another relatively isolated syndrome of note, occurring mainly in older women, is a slowly progressive cervical myelopathy with weakness and ataxia This is particularly dif cult to differentiate from cervical spondylosis Not infrequently a prominent feature of the disease is nystagmus and ataxia, with or without weakness and spasticity of the limbs a syndrome that re ects involvement of the cerebellar and corticospinal tracts Ataxia of cerebellar type can be recognized by scanning speech, rhythmic instability of the head and trunk, intention tremor of the arms and legs, and incoordination of voluntary movements and gait, as described in Chap 5 The combination of nystagmus, scanning speech, and intention tremor is known as the Charcot s triad But while this group of symptoms is often seen in the advanced stages of the disease, most neurologists would agree that it is not a common mode of presentation The most severe forms of cerebellar ataxia, in which the slightest attempt to move the trunk or limbs precipitates a violent and uncontrollable ataxic tremor, are observed among patients with long-standing MS The responsible lesion probably lies in the tegmentum of the midbrain and involves the dentatorubrothalamic tracts and adjacent structures Cerebellar ataxia may be combined with sensory ataxia, owing to involvement of the posterior columns of the spinal cord or medial lemnisci of the brainstem In most cases of this type the signs of spinal cord involvement ultimately predominate; in others, the cerebellar signs are more prominent Diplopia is another common presenting complaint It is due most often to involvement of the medial longitudinal fasciculi, producing an internuclear ophthalmoplegia (see page 236) The signs are characterized by paresis of the medial rectus on attempted lateral gaze, with a coarse nystagmus in the abducting eye; in MS, this abnormality is usually bilateral (unlike small pontine infarcts, which cause a unilateral internuclear ophthalmoplegia) As a corollary, the presence of bilateral internuclear ophthalmoplegia in a young adult is virtually diagnostic of MS Occasionally, internuclear ophthalmoplegia in one direction is combined with a horizontal gaze paresis in the other, although this one-and-a-half syndrome is more typical of brainstem stroke (see also page 236) Other palsies of gaze (due to interruption of supranuclear connections) or palsies of individual ocular muscles (due to involvement of the ocular motor nerves in their intramedullary course) also occur, but less frequently Additional manifestations of brainstem involvement include myokymia or paralysis of facial muscles, deafness, tinnitus, unformed auditory hallucinations (because of.