Neurological Manifestations in Mixed Connective Tissue Disease: A Systematic Review and Narrative Synthesis

Mixed connective tissue disease (MCTD) is a rare autoimmune overlap syndrome. Neurological manifestations are underreported and not previously synthesised systematically. This review aims to identify and describe the spectrum, clinical presentation, diagnostic approaches, management, and outcomes of neurological manifestations in patients with MCTD reported in case reports and case series and observational studies. We did a systematic review and narrative synthesis with a total of 40 articles, out of which there were 34 case reports, one case series, two case-control studies, two cross-sectional comparative studies and one retrospective study. These articles together included a total of 149 patients. In the case reports and case series, the most common manifestation was trigeminal neuralgia, reported in 11, followed by Posterior reversible encephalopathy syndrome was reported in five cases, four cases of neuromyelitis Optica, two cases of transverse myelitis, two cases of encephalopathy, one case each of various forms of neuropathy and dropped head syndrome, subarachnoid haemorrhage, haemorrhagic stroke, sagittal sinus thrombosis, normal pressure hydrocephalus, arachnoiditis, aseptic meningitis and psychosis. To enhance patient outcomes, clinicians must continue to be on the lookout for a variety of Neuropsychiatric and Neuromuscular symptoms in MCTD.