Parkinsonism as an initial manifestation of rheumatoid meningitis

Abstract

Rheumatoid meningitis (RM) has been considered to be an extremely rare complication of rheumatoid arthritis (RA), which itself is very common [1]. However, due to better access to MRI, RM has been increasingly diagnosed. More than 30 cases diagnosed by MRI and pathology have been reported [2–4]. In most cases, onset occurred after the age of 50 in both sexes. The clinical symptoms and signs and the MRI findings were those of non-specific chronic leptomeningitis [5]. Of interest, there were no associations between the onset and activity of RM, the severity and activity of synovitis, the duration of RA or the way it treated [3]. We have described here for the first time a patient with pathologically proven RM the initial symptom of which was parkinsonism. A 68-year-old Japanese man with a 10-year history of RA treated successfully with prednisolone (6 mg/day) was referred to a regional hospital because of a bilateral resting tremor predominating on the right side, progressive gait instability that began by hesitation and bradykinesia. He was diagnosed as having Parkinson’s disease, but his symptoms did not respond to levodopa. He soon developed a progressive cognitive dysfunction, accompanied by a slight fever, followed 2 months later by urinary incontinence. In spite of more than 3 months of treatment with antibiotics, antiviral agents and antimycotics, his condition gradually deteriorated and he was transferred to our university hospital. On admission, he was apathetic and bedridden. Disuse atrophy was generalized, and he had severe bilateral swanneck deformities of his fingers due to RA. His face was mask-like, and his verbal responses were very slow with muted monotonous voice. His Mini-Mental State Examination (MMSE) score was 11. Bradykinesia was remarkable, and rigidity was seen in the neck and all extremities. Resting tremor was observed intermittently in both hands. Laboratory data suggested inactive RA. Cerebrospinal fluid (CSF), analyzed after lumbar puncture, was clear, and pressure was normal. The cell count was slightly increased (13/mm), but there were no atypical cells. Protein levels were increased (75 mg/dL), but glucose levels were normal. Results of cell cultures performed repeatedly, immunological investigations, a polymerase chain reaction (PCR) to detect microorganisms, and cytological investigations of the CSF were all negative. No malignancy was detected by a whole body CT scan with enhancement. Brain MRI with contrast enhancement (Fig. 1a, b) revealed a diffuse meningeal lesion. Secondary hydrocephalus was also observed (Fig.1c). A biopsy of the leptomeninx together with adjacent brain parenchyma from the right frontal lobe revealed foci of necrotic tissue in the subarachnoid space surrounded by chronic inflammatory cells consisting of numerous lymphocytes, plasma cells, a few neutrophils and scattered giant multinucleated cells (Fig. 1e, f). No inflammation was seen in the dura mater and parenchyma per se. These pathological findings associated with the negative laboratory data were consistent with a diagnosis of RM. Y. Hayashi M. Namekawa (&) Department of Neurology, Jichi Medical University School of Medicine, 3311-1 Yakushiji, Shimotsuke, Tochigi 329-0498, Japan e-mail: mnamekaw@jichi.ac.jp