Dementia in a patient with non-paraneoplastic limbic encephalitis associated with relapsing polychondritis

Relapsing Polychondritis with Encephalitis: A Case Report and Literature Review

“…It should be noted that repeated MRI was required to obtain these findings in some cases (5,8), including our case. The brain lesions in such cases became obvious on MRI images obtained several weeks after the onset of the initial neurological symptoms.…”

“…Although CNS involvement due to vasculitis is rare, RP has been reported to be associated with aseptic meningitis, meningoencephalitis, encephalitis, and ischemic stroke (3). To our knowledge, there have been 18 cases of RP-associated meningoencephalitis and encephalitis demonstrated clinically and on neuroimaging in the English literature (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19). The clinical features of the previously reported cases and the present case are summarized in Table. Among these cases, the age of onset was between 29 and 73 years (mean, 55 years).…”

“…Of the 18 patients treated with high-dose steroids, including mPSL pulse therapy, 11 showed significant improvements in their neurological symptoms (from within one week to three months), four demonstrated no improvements in symptoms and three died five to 18 months after disease onset. In two of the cases in which the patients died (8,12), more intensive and/ or timely treatment was likely required.…”

Meningoencephalitis or meningitis in relapsing polychondritis: Four case reports and a literature review

“…Associated neurological disorders included impaired cognitive function, memory loss, disorientation, expressive and receptive aphasia, dullness, acalculia, personality changes, euphoria, confabulation, hallucination, aggressive and abusive behaviour, disturbances of consciousness, confusion, somnolence, delirium, akinetic mutism, sleep inversion, insomnia, generalised tonic-clonic seizure, focal seizure, unstable gait, impaired visual acuity, papilledema, ptosis, diplopia, unilateral facial weakness, hearing loss, tinnitus, nystagmus, hemiplegia, and urinary incontinence ( Table 2). CSF tests revealed pleocytosis in 26 patients, and two patients 18,22 had no results. Seventeen patients, including our Patients 1, 2 and 4, showed pleocytosis with a predominance of mononuclear cells, including lymphocytes and monocytes in the CSF.…”

Perivasculitic Panencephalitis with Relapsing Polychondritis: An Autopsy Case Report and Review of Previous Cases

“…Stewart et al (13) reported extensive cerebral and systemic vasculitis to be the cause of CNS involvement in patient with RP. In contrast, other authors have reported inflammatory changes not specific to vessels in patients presenting with limbic encephalitis or meningoencephalitis associated RP (7,(14)(15)(16)(17). In these autopsy cases, diffuse vasculitis and/or perivascular infiltration were observed, with gliosis in the leptomeninges and brain parenchyma of the limbic area and basal ganglia, etc.…”