In addition to a well-described posterior root ganglionopathy and sensory neuritis, an inflammatory myelitis is associated with Sjögren syndrome. In most instances, the patient has had overt symptoms of Sjögren disease including, the sicca complex, and in others, the association has been established through serologic testing or by the finding of inflammatory infiltration of minor salivary glands (obtained by biopsy). 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. The 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 demyelinating myelitis. The spinal fluid formula has also varied but generally does not contain oligoclonal bands. Treatment with prednisone and cyclophosphamide or methotrexate has been suggested and was seemingly successful in several of our patients.
A review of Sjögren myelopathy and peripheral neuropathy has been given by one of the authors with
Berkowitz. There is little pathologic material on which to judge the association, but the presence of other inflammatory lesions of the central and peripheral nervous system in Sjögren disease makes the existence of myelitis plausible. Antibody tests (anti-SS-A [Ro] and SS-B [La]) and possibly a biopsy of the minor salivary glands (at the junction of mucosa and epidermis of the lower lip) are justified in patients with unusual myelopathies or in those with sicca symptoms; however, screening in this manner of all cases that otherwise suggest MS or postinfectious myelitis may be excessive. This subject is also reviewed in Chap. 35, in relation to multiple sclerosis.
There is also the rare occurrence of nondescript myelitis with scleroderma as mentioned above (systemic sclerosis). There it is emphasized that many cases are of the longitudinally extensive type. The authors of most reports acknowledge the difficulty 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.
Behçet Disease Myelopathy (See Also Chap. 33)
This vascular and inflammatory disease is usually considered in the context of cerebral venous thrombosis and other forms of stroke but, in addition to the typical findings of orogenital ulcers and uveitis, it has varied manifestations in the nervous system in about 5 percent of cases (neuro-Behçet’s syndrome). Among these is a myelopathy that may relapse like MS and may occur at single or multiple sites in the cord or simulate neuromyelitis optica in causing a longitudinally extensive lesion. The lesions may take up gadolinium on MRI and a peculiar imaging “bagel sign” has been described by Uygunoglu and colleagues, consisting of central hypointense area with a hyperintense rim. The clinical syndrome and imaging changes are said to resolve with glucocorticoids.
Paraneoplastic Myelitis (See Also Chap. 30)
A subacute necrotic myelitis developing in conjunction with bronchogenic carcinoma was first brought to notice by Mancall and Rosales in 1964. Several dozen cases have since been recorded in association with lymphomas and carcinomas, but the disease must be rare. Actually, in cancer patients, intramedullary metastasis, quite infrequent to begin with, is more common as a cause of intrinsic myelopathy and, of course, a compressive lesion is far more frequent than either of these conditions.