A clinically isolated syndrome: A challenging entity

Abstract

Acute isolated neurological syndromes, such as optic neuropathy or transverse myelopathy, may cause diagnostic problems since they can be the first presentations of a number of diseases such as multiple sclerosis (MS) and collageneous tissue disorders. In the present study, particular systemic lupus erythematosus (SLE) and primary Sjogren syndrome (pSS) patients, who were followed up with the initial diagnosis of possible MS with no evidence of collagen tissue disorders for several years, are described. Five patients with the final diagnosis of SLE and five pSS patients are evaluated with their neurologic, systemic and radiologic findings.Over several years, all developed some systemic symptoms like arthritis, arthralgia, headache, dry mouth and eyes unexpected in MS. During the regular and close follow-up laboratory evaluations of vasculitic markers revealed positivity, leading to the final definite diagnosis of SLE or pSS. Patients with atypical neurological presentation of MS, a relapsing remitting clinical profile, or lack of response to the regular MS treatment should be evaluated for the presence of a connective tissue disease. Various laboratory tests, such as cerebrospinal fluid findings, autoantibodies profile, markers, cranial and spinal magnetic resonance imaging, can be helpful for the differential diagnosis. Lack of response to the regular multiple sclerosis treatment, even increasing rate of relapses can force the clinician for the differential diagnosis. In particular cases an accurate diagnosis can only be made after close follow-up.

Frequency of Autoantibodies and Connective Tissue Diseases in Chinese Patients with Optic Neuritis

“…[6], [7]. ON with autoimmune diseases present a relapsing remitting clinical profile, or lack of response to the regular glucocorticoid treatment [8]. The long-term visual prognosis is more severe in chronic relapsing inflammatory optic neuritis (CRION) patients and neuromyelitis optica-immunoglobulin G (NMO-IgG)-positive patients [9].…”

CNS inflammation other than multiple sclerosis: How likely is diagnosis?

“…None of the patients with a diagnosis of CIS converted to a diagnosis of CIDOMS in this study, but this may need longer follow-up, as illustrated by a recent case series. 3 However, categorization of low-risk CIS with CIDOMS and high-risk CIS with MS maintains nondiagnosis as the most common outcome in patients with CIDOMS (16 out of 78, i.e., 21%), and maintains patients with CIDOMS as still more resource-intensive than patients with MS (data not shown).…”

The Impact of Optic Nerve Disorders on Sleep Wake

“…A monophasic typical ON with duration at least 24 hours is known as a CIS, which is an episode of CNS demyelination with features analogous to an attack of MS, but which fails to fulfil the McDonald criteria of dissemination in space and time for MS (352). A CIS may transpire to be an isolated event or may progress to RRMS (359), and may present with unilateral or bilateral ON (360,361). The CSF biomarker homebox protein HOXB3 has been found to be associated with progression of CIS to MS within 5 years (362), and diffusion tensor imaging of the brain combined with brain volumetry has been found to be predictive for development into clinically definite MS within 2 years (363).…”