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DISEASES OF SPINAL CORD, PERIPHERAL NERVE, AND MUSCLE
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matory cerebral hemorrhages There were multiple occlusions of small vessels surrounding the spinal cord and a mild vasculitis (Ropper et al) Polyarteritis nodosa and necrotizing arteritis only rarely involve the spinal cord Treatment None of the treatments offered in our experience has made a noticeable difference in this disease, but some authors have the impression that high-dose corticosteroids, cyclophosphamide, or plasma exchange may have been bene cial in individual cases and we continue to attempt these in our patients Myelitis (Myelopathy) with Connective Tissue Disease A rapidly evolving or subacute myelopathy in association with systemic lupus erythematosus must always be considered in the differential diagnosis of demyelinative myelitis It is presumed to arise from a microvasculitis of the spinal cord Propper and Bucknall presented such a case and reviewed 44 others in which a patient with lupus erythematosus developed a transverse myelitis over a period of days There was back pain at the level of sensory loss (the cases we have seen have been painless) Pleocytosis and elevation of CSF protein were characteristic The MRI revealed a segmental swelling of the spinal cord Postmortem examinations of similar cases have disclosed widespread vasculopathy of small vessels with variable in ammation and myelomalacia, and, rarely, a vacuolar myelopathy Some but not all cases also have an antiphospholipid antibody; the relationship of these antibodies to the myelopathy and to microvascular occlusion is uncertain (see also page 735) The incidence of lupus myelopathy is not known, but one such case is admitted to our service every several years Also mentioned here is the rare occurrence of nondescript myelitis with scleroderma (systemic sclerosis) The authors of most reports acknowledge the dif culty in distinguishing between the myelopathies of various connective tissue diseases There may be some response to corticosteroids and other immunosuppressive medications Myopathy and neuropathy, particularly trigeminal neuritis, are more common manifestations of scleroderma Sjogren Syndrome with Myelopathy In addition to the well described posterior root ganglionopathy and sensory neuritis, an in ammatory myelitis has been associated with Sjogren syndrome In most instances, the patient has had overt symptoms of Sjogren syndrome, namely the sicca complex, and in others, the association has been established through serologic testing or by the nding of a characteristic in ammatory in ltration of minor salivary glands (from which a biopsy has been taken on the lip) In many reported cases the myelopathy has simulated the myelitis of MS, even to the extent of including episodes of optic neuritis as in the cases described by Williams and colleagues and by de Seze and coworkers This putative Sjogren myelitis has been in different cases acute, chronic, or relapsing and displayed MRI changes in the cord that would otherwise be considered to be postinfectious or demyelinative myelitis The spinal uid formula has varied but generally does not contain oligoclonal bands Treatment with prednisone and cyclophosphamide or chrlorambucil has been suggested The authors cannot comment authoritatively on this entity There is little pathologic material on which to judge the association but the presence of other in ammatory lesions of the central and peripheral nervous system in Sjogren cases makes the existence of a myelitis plausible Antibody tests (anti-SS-A and SS-B) and possibly a biopsy of the minor salivary glands (at the junction of mucosa and epidermis of the lower lip) are justi ed in older women
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with unusual myelopathies or those with sicca symptoms; however, screening of all cases that otherwise suggest MS may not be practical Whether our ndings relative to the insensitivity of the serologic tests in patients with Sjogren disease and polyneuropathy also hold true for those with myelopathy is not known (see page 1141) Paraneoplastic Myelitis A subacute necrotic myelitis developing in conjunction with a bronchogenic carcinoma was rst brought to notice by Mancall and Rosales in 1964 Several dozen cases have since been recorded, some in association with lymphomas, but the diseaes must be rare Actually, in cancer patients, intramedullary metastasis is more common as a cause of intrinsic myelopathy than is paraneoplastic disease and, of course, a compressive lesion is far more frequent The clinical syndrome consists of a rapidly progressive painless loss of motor and then sensory tract function, usually with sphincter disorder Imaging studies demonstrate an area of T2 signal change in the cord or may be normal This is in distinction to the nodular enhancing appearance of an intramedullary metastasis or of extradural metastatic disease with cord compression The larger problem of metastatic compression of the cord from the epidural space is discussed in a later part of the chapter The CSF may contain a few mononuclear cells and a slightly increased protein, or it may be normal The lesions are essentially of necrotic type and respect neither gray nor white matter, but the latter is more affected There is little or no evidence of an infectivein ammatory or ischemic lesion, for the blood vessels, apart from a modest cuf ng with mononuclear cells, are normal No tumor cells are visible in the CSF, meninges, or spinal cord tissue, and no virus has been isolated Unlike the situation in most of the paraneoplastic neurologic disorders, there are no diagnostic markers by way of antineural antibodies In particular, this myelopathy does not seem to be a component of the anti-Hu associated encephalitisneuropathy spectrum In some cases of paraneoplastic myelopathy, the degenerative changes are more chronic, con ned to the posterior and lateral funiculi and they are often associated with a diffuse loss of cerebellar Purkinje cells This latter syndrome has a disproportionately high association with ovarian carcinoma but has been observed with carcinoma of other types and with Hodgkin disease All of the reported cases of these types have ended fatally Steroids and plasmapheresis were of no clear value A special but rare variety of anterior horn cell destruction resembling motor neuron diseases is known to occur rarely with certain lymphomas; it is discussed with the paraneoplastic syndromes in Chap 31 Subacute Spinal Neuronitis Whitely and colleagues have drawn attention to a rare but distinctive form of encephalomyelitis of unknown cause, characterized clinically by tonic rigidity and intermittent myoclonic jerking of the trunk and limb muscles and by painful spasms of these muscles evoked by sensory or emotional stimuli In the late stages of the disease, signs of brainstem involvement may appear which are usually progressive over a period of several weeks, months, or a year or longer; consciousness is preserved However, the cases we have observed remained con ned to several contiguous spinal segments, usually the upper abdomen and lower thorax The CSF may be normal or show a mild lymphocytosis and increase in protein content This is probably the same disorder that had been described earlier by Campbell and Garland under the title of subacute myoclonic spinal neuronitis and
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