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Central Nervous System Systemic Lupus Erythematosus Mimicking Progressive Multifocal Leucoencephalopathy

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Central Nervous System Systemic Lupus Erythematosus Mimicking Progressive Multifocal Leucoencephalopathy 1152 Annals ofthe Rheumatic Diseases 1992; 51: 1152-1156 CASE REPORTS Ann Rheum Dis: first published as 10.1136/ard.51.10.1152 on 1 October 1992. Downloaded from Central nervous system systemic lupus erythematosus mimicking progressive multifocal leucoencephalopathy Brian R Kaye, C Michael Neuwelt, Stuart S London, Stephen J DeArmond Abstract count, chemistry panel, and urine analysis. A The case is reported of a patient with central test for antinuclear antibodies was positive at a nervous system systemic lupus erythematosus 1:80 dilution. Antibodies to double stranded (SLE) with features of progressive multifocal DNA were negative and serum complement leucoencephalopathy (PML) seen clinicaily concentrations were normal. and by magnetic resonance imaging. A brain The patient did well until June 1983 when biopsy sample showed microinfarcts. The use she again developed pleuritic chest pain and of magnetic resonance imaging and IgG swelling of her hands. At this time an anti- synthesis rates in evaluating central nervous nuclear antibody test was positive at a 1:320 system lupus, the co-occurrence of SLE and dilution with a speckled pattern. Antibodies to PML, and the differentiation of these entities ribonucleoprotein, Sm, SS-A, and SS-B were by magnetic resonance imaging and by histo- absent. Her complete blood count and urine logy are considered. analysis were normal. In November 1985 she developed daily fevers, (Ann Rheum Dis 1992; 51: 1152-1156) fatigue, and malar rash. Her packed cell volume was 0 34 and a direct Coombs' test was positive. The central nervous system manifestations of A diagnosis of SLE was made and she was systemic lupus erythematosus (SLE) are treated with hydroxychloroquine (400 mg/day). protean. Strokes, seizures, acute psychosis, One month later she had a brief psychotic depression, focal neurological impairment, and reaction. subtle psychological abnormalities are among In February 1986 she developed mouth ulcers the myriad of neurological findings in patients and erythema on her fingertips. She was noted with SLE. One finding in patients with SLE is to have slowing of her mentation. At that time progressive multifocal leucoencephalopathy her white blood cell count had decreased to http://ard.bmj.com/ (PML).1-5 Magnetic resonance imaging has 4 4x 106/1, her packed cell volume was 0-31, and been used for the ongoing evaluation of central her erythrocyte sedimentation rate was 52 mm/ nervous system lupusl' and PML," perhaps hour (Westergren method). She had 3+ blood increasing our rate of identification of these two on a urine dipstick test. She was treated with diseases when they occur concomitandy. prednisone (10 mg/day), which was subsequently We report here the case of a patient with SLE increased to 20 mg/day. who had neurological findings and magnetic In March 1986, after a trip to Hawaii, she on September 28, 2021 by guest. Protected copyright. resonance imaging abnormalities that mimicked developed a fever to 40°C, difficulty concen- PML. A brain biopsy sample was taken from trating, dysarthria, and a tremor of her left the patient while she was still alive, providing hand. Antibodies to double stranded DNA, pathological correlation with the magnetic direct Coombs' test, and antibodies to extract- resonance imaging scan. This case shows the able nuclear antigen were negative; total need to use several different diagnostic tech- haemolytic complement concentrations were East Bay Rheumatology in evaluating central nervous system normal. An of her cerebral spinal fluid Medical Group, niques analysis Oakland and manifestations of SLE. It also reinforces the gave a white blood cell count of 0 03 x 106/1 with San Leandro, need to consider serious brain disease in 91% lymphocytes, 7% monocytes, and 2% and Departnent of patients with SLE showing subtle psychological polymorphonuclear leucocytes, and a negative Medicine, University of California, abnormalities. herpes simplex virus antibody test. The IgG San Francisco, CA, USA synthesis rate in the cerebrospinal fluid was B R Kaye 0-46 g/24 hours (normal value less than 0 033 C M Neuwelt Case report g/24 hours), cerebrospinal fluid protein 500 g/l Oakland, CA, USA S S London A 35 year old white female speech pathologist (normal less than 450 g/l), and cerebrospinal presented in December 1982 with one month's fluid glucose 4-1 mmol/l. Her peripheral white Department of Pathology, history of pleuritic chest pain and an erythe- blood cell count was 31lx106/1 with normal University of California, matous urticarial rash following a laparotomy serum C3 and C4 concentrations. An initial San Francisco, CA, USA for a ruptured appendix. Two and a half months magnetic resonance imaging scan of her brain S J DeArmond previously she had delivered a healthy infant. showed a confluent high signal intensity on Correspondence to: Dr B R Kaye, Her pregnancy had been marked by poly- proton density and T2 weighted images within The Arthritis Center, arthralgias. Physical examination revealed only the centrum semiovale and the coronal radiata 5700 Telegraph Avenue, Oakland, CA 94609, USA. a fine erythematous, urticarial rash on her consistent with demyelination (fig 1). An electro- Accepted for publication chest, hands, and feet. Initial laboratory encephalogram showed a mild, generalised, 12 December 1991 evaluation included a normal complete blood non-specific abnormality that was consistent Central nervous system SLE mimicking multifocal leucoencephalopathy 1153 Ann Rheum Dis: first published as 10.1136/ard.51.10.1152 on 1 October 1992. Downloaded from Fire I Axial T2 weightedspin echo imagesfrom the March 1986 magnetic resonance imaging brain scan showing subtle confluent abnormaly increased signal intensity affecting theparietal and occipital centrum semiovale and corona radiata bilaterally. Scan parameters: 1-5 T; axialSE5 mm, TR 2500 ms, TE 80 ins, 2 NEX, 256x256 matrix. http://ard.bmj.com/ 2 Axial echo 1986 resonance brain scan showing Figure T2 weighted spin imagesfrom the3June magnetic imaging on September 28, 2021 by guest. Protected copyright. progression ofthe abnormally increased signal intensity ofthe cerebral hemispheres which now affects thefrontal lobes. There is also interval increase in the calibre ofthe lateral and third ventricles reflecting central cerebral atrophy. Scan parameters: 15 T; axial SE 5 mm, TR 2500 ms, TE 80 ms, 2 NEX, matrix 256x 128. with lupus cerebritis. The dose of prednisone was forgetful and was having difficulty perform- was increased and then gradually tapered over ing her job as a speech pathologist. She was the next few months when her neurological leucopenic (white blood cell count 2 4x 106/1) and mental status improved. and anaemic (packed cell volume 0-327, direct In June 1986 she was again having difficulty Coombs' test negative). She received one dose concentrating and showed emotional lability. of cyclophosphamide (750 mg) intravenously She had mild gait ataxia and a decrease in and methylprednisolone (100 mg) intravenously immediate recall of memory on examination. A each day for three days. Her condition continued three month follow up magnetic resonance to deterioriate over the next few months. imaging scan of the brain showed progressive In December 1986 she developed a shuffling demyelination predominately within the frontal gait, further difficulty with her memory, centrum semiovale and coronal radiata. In confusion, and increased emotional lability. She addition the lateral and third ventricles showed was disoriented with respect to time and had striking enlargement, reflecting central cerebral difficulty with simple calculations. She showed atrophy (fig 2). An electroencephalogram mild cogwheel rigidity and a subtle positional performed in August 1986 showed a greater tremor. Cerebrospinal fluid analysis showed generalised non-specific abnormality than that 0-005 x 106 white blood cells/l, normal protein performed in March 1986. and glucose concentrations, and a negative In October 1986 the patient became severely Venereal Disease Research Laboratory (VDRL) psychotically depressed and required admission and coccidioiodomycosis agglutination titre. to a hospital psychiatric ward. In addition she The IgG synthesis rate was again increased at 1154 Kaye, Neuwelt, London, DeArmond Ann Rheum Dis: first published as 10.1136/ard.51.10.1152 on 1 October 1992. Downloaded from http://ard.bmj.com/ Figure 3 A andB: axial T2 weighted spin echo imagesfrom thefourth magnetic resonance imaging brain scan (performed in December 1986) showing significant diffuse increase in demyelination throughout the cerebral hemispheres in addition to a significant increase in the ventricular size reflectingprogressive central cerebral atrophy. Scan parameters: 1 5 T; axial SE 5 mm, TR 2500 ms, TE 80 ms, matrix 256x 128, 2 NEX. C and D: axial T2 weighted spin echo imagesfrom thefourth magnetic resonance imaging brain scan (performed in December 1986) showing progressive demyelination affecting the posterior limbs ofthe internal capsules bilaterally as well as midbrain. Scan parameters: 1 5 T; axial SE 5 mm, TR 2500 ms, TE 80 ms, matrix 256x 128, 2 NEX. on September 28, 2021 by guest. Protected copyright. 0-357 g/24 hours. Routine, mycobacterial, and The rapid progression of the neurological fungal cultures of the cerebrospinal fluid were disease raised the possibility that the patient negative. Antibodies to double stranded DNA might have PML, which cannot be treated. were slightly increased at 20% (Farr assay, Consequently before using
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