Annals ofthe Rheumatic 1992; 51: 117-119 117

CASE REPORTS Ann Rheum Dis: first published as 10.1136/ard.51.1.117 on 1 January 1992. Downloaded from

Organic with as an initial manifestation of systemic lupus erythematosus in an elderly woman M E Mavrikakis, L G Antoniades, J B Germanides, D Sotou, A Rassidakis

Abstract of consciousness, with responsiveness to intense This paper describes a rare case of organic painful stimulation, a medium ability to brain syndrome with psychosis and clinically communicate, and an inability to stand without transverse myelopathy, as initial manifesta- assistance. Sensory examination was impossible. tions of systemic lupus erythematosus in an Tendon jerk reflexes of the upper extremities elderly woman. The identification and were normal but those of the lower extremities evaluation of antibodies to ribosome P in the were absent on the right and depressed on the serum and cerebrospinal fluid may be of help left; plantar reflexes were both indifferent. in such cases for . The There was a loss of bowel and bladder sphincter patient was treated successfully with 30 mg control. These later clinical findings are prednisone daily. compatible with transverse myelopathy at a low level. Other findings were unremarkable. Systemic lupus erythematosus (SLE) attacks the central nervous system (CNS) in a way that PSYCHIATRIC EXAMINATION can lead to a wide spectrum of clinical mani- Psychiatric examination showed that the festations.' The neurological manifestations of was not due to or drug SLE are usually seen in combination with abuse. She showed impaired recent other clinical features and are rarely the primary and disturbed orientation, impaired level of sign of the .2 consciousness, and psychomotor nervousness In the elderly the clinical picture usually alternating with apathy. The sleep-wakefulness follows thepattern: serositis, arthritis, arthralgia, cycle was disturbed with predominant . myalgia; while renal disease hypocomplemen- She misrecognised faces and had , taemia, neuropsychiatric disturbances, alopecia, emotional disturbances, and anxiety and and Raynaud's phenomenon are uncommon.3 4 nervousness alternating with apathy. http://ard.bmj.com/ We describe a rare case of SLE in an elderly woman, in which the primary manifestation PHYSICAL EXAMINATION was organic brain syndrome with psychosis and Physical examination showed a severely ill clinically transverse myelopathy. This case overweight patient. Skin, conjunctiva, and highlights the difficulty in differential diagnosis mucous membranes were pale. Lungs, heart, between SLE and other diseases with a similar and alimentary tract were normal. clinical picture, emphasises the diagnostic value on September 24, 2021 by guest. Protected copyright. of antibodies to ribosome P, and provides an LABORATORY FINDINGS example of successful clinical management of Laboratory findings were as follows: haemo- CNS-SLE with relatively small doses ofsteroids. globin 90 g/l, packed cell volume 0-293, red It is reported that moderate doses, as low as 30 blood cell count 3- 14x 1012/1, white blood cell mg prednisone daily, may be quite effective in count 3 x 109/1 with normal differential, platelets some patients.5 253 x 109/1, erythrocyte sedimentation rate 90 mm/lst h (Westergren). Haemoglobin and Case cellular casts were obtained from the urine. A report 24 hour urine sample showed only traces of A woman aged 78 years was admitted to the clinic with the following symptomatology, protein. Department of according to information provided by her Serum potassium, sodium, calcium, phos- Clinical Therapeutics, phorus, urea nitrogen, creatinine, glucose, 'Alexandra' Hospital, family: for six months, numbness in the Athens Medical School extremities, with increasing pain and aspartate transaminase, alanine transaminase, difficulty lactic dehydrogenase, creatine kinase, y- M E Mavrikakis in walking; for four months, loss of bowel and L G Antoniades bladder sphincter control, and mental distur- glutamyltransferase, alkaline phosphatase, uric J B Germanides acid, iron, vitamin B-12, folate, CEA, a feto- D Sotou bances; for two months, temperature fluctua- tions of the order of 37-3 to 38°C, drowsiness, protein, triiodothyronine, thyroxine, thyroid A Rassidakis and ammonia were memory disturbances, loss of orientation, with stimulating hormone, all Correspondence to: within normal limits. Prothrombin time was Dr Myron E Mavrikakis, occasional agitation and delirium. There was Department of nothing unusual in the 100%. Total serum protein was 58 g/l. Protein Clinical Therapeutics, patient's personal electrophoresis gave Al 44-7%, 'Alexandra' Hospital, history or habits. a, 6-7%, a2= Vas Sofias 80 and Lourou, 13-8%, ,13 14 3%, y 20-1%. Widal's test, Wright's GR 115 28 Athens, Greece. test, monotest, blood cultures, and urine Accepted for publication NEUROLOGICAL EXAMINATION cultures were all negative. C reactive protein 1 October 1991 Neurological examination showed impairment was not present; Ra test, latex, and LE cell tests 18 Mavrikakis, Antoniades, Germanides, Sotou, Rassidakis

were negative; anti-DNA was 10-6 mg/l (normal Organic brain syndrome is the most common <5), C3 1-36 g/l (normal 0 7-1[76), C4 175 mg/l severe manifestation ofCNS-SLE and is charac- Ann Rheum Dis: first published as 10.1136/ard.51.1.117 on 1 January 1992. Downloaded from (normal 160-500); antinuclear antibodies, anti- terised by the abrupt or gradual onset of SSA/SSB, anti-Sm/RNP, anti-Scl70 were not memory impairment, loss of orientation, present; antibodies to ribosome P were present intellect, or judgment, apathy, irritability, or (+++ +); Coombs' test was negative, serum delirium. The symptoms may either be transient circulating immune complexes were 42-7 mmol/l and fluctuate with the activity of the disease or (normal <20), IgG 9-8 g/l, IgA 4-8 g/l, IgM there may be a slow but progressive deter- 2-7 g/l. ioration.' The cerebrospinal fluid showed normal Psychosis occurs in 10-50% of patients with general features and the following values were SLE,8 and usually coexists with organic brain obtained: pH 7 0, glucose 1-7 mmol/l, protein syndrome. Clinically, psychosis may appear in 290 mg/l, red blood cells 1-05 x 109/1, white (a) severely systemically ill patients; (b) patients blood cells 107/1, C3 30 mg/l, C4 50 mg/l, anti- with a milder clinical course, but in combination DNA 913 mg/l, antibodies to ribosome P with other neurological signs; (c) patients with strongly positive (+ + +), culture negative, or without other clinical indications and with or Koch's bacillus test negative. without other abnormal serological tests for Brain computed tomography showed ventri- SLE.' Our patient falls into this last category. cular dilatation indicative of cerebral atrophy The positive antibodies to ribosome P in the greater than expected for the patient's age. serum and cerebrospinal fluid have particular Magnetic resonance imaging showed cerebral diagnostic value as they may serve as an atrophy. An ocular examination gave negative additional serological index for the diagnosis of results. Results ofa skin biopsy were compatible SLE, given that they may be detected in the with collagen disease. serum even when there are no other positive Her family refused to allow further investi- immunological indices, such as anti-dsDNA gations and so examination of the spinal cord by (35% of patients with SLE with antibodies to computed tomography and magnetic resonance ribosome P did not have anti-dsDNA9). The imaging were not carried out. recent identification of antibodies to ribosome P in the serum and cerebrospinal fluid of patients Discussion with SLE emphasises the high correlation Organic brain syndrome may be due to a wide between psychosis resulting from SLE and variety of conditions6-for example, these antibodies. Bonfa et al noted that 18 of 20 such as or intracerebral abscess; patients with psychosis arising from SLE had metabolic illnesses such as uraemia or hepatic antibodies to ribosome P, whereas there was no failure with hyperammonaemia; disturbances of such finding either in patients with SLE and electrolyte balance such as hypo- or hyper- other CNS manifestations, patients with SLE natraemia, hypo- or hyperkalaemia; severe and intermittent behaviour abnormalities due to hypoxaemia; overuse of drugs such as atropine steroids, patients with psychosis but without http://ard.bmj.com/ or anticonvulsants, or withdrawal from bar- SLE, or in normal subjects.'0 Similar findings biturates or alcohol; space occupied lesions such were reported by Golombek et al. " The as neoplasms, haematoma, or even multiple mechanism through which the antibodies to cerebral arterial emboli; endocrine diseases, ribosome P are responsible for psychotic mani- mainly hyper- or hypothyroidism; cerebral festations of SLE is believed to be via the blood- atrophy. These were ruled out, firstly, by the brain barrier,'2 so that cytotoxins act by affect- clinical history, which contained no reference to ing the neurotransmitters or neurones. on September 24, 2021 by guest. Protected copyright. drug abuse, and, secondly, by the findings of Other laboratory findings supporting the the biochemical blood tests for metabolic diagnosis of SLE of the CNS were the low disturbances and endocrine diseases, by the concentrations of cerebrospinal fluid comple- findings from the cerebrospinal fluid concerning ment'3 and the degree of cerebral atrophy infections, and by the methods used to visualise shown by the cerebral computed tomography the brain, which eliminated the possibility of and magnetic resonance imaging examinations, space occupied lesions or vascular damage. which was greater than expected for the patient's In such cases, where the main causes of age.'4 organic brain syndrome, with or without The diagnosis of transverse myelopathy in psychosis, have been ruled out, one must look our patient was based on the typical clinical for other rare diseases to which the syndrome findings and supported by the low cerebrospinal might be attributable, such as SLE. In this fluid glucose concentration.' It is a rare com- particular patient the organic brain syndrome plication ofCNS-SLE, like other rarely reported with psychosis, after exclusion of other possible complications such as diabetes insipidus.'5 causes, was strongly suggestive of SLE, as the Treatment of SLE in the elderly is subject to proposed criteria of the American Rheumatism the same problems as treatment of the young, Association for the diagnosis of SLE7 were though the average needed may satisfied-namely, (a) haematological-leuco- be less in the elderly.6 The favourable penia, absolute lymphopenia; (b) immuno- response of the patient to relatively low doses of logical-antibodies to ribosome P (+ + +), anti- steroids (30 mg prednisone daily) is worth DNA (+). In the cerebrospinal fluid, antibodies noting. A clear clinical improvement was seen to ribosome P (+ + +), low concentrations of within five days, particularly in the patient's C3, C4; (c) neuropsychiatric manifestations; (d) mental state, but the clinical signs of transverse renal disease-persistent haematuria, cellular myelopathy did not improve to the same extent. casts. In conclusion, it is suggested that in cases of Organic brain syndrome with psychosis in SLE 119

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