Background The American College of Rheumatology Nomenclature for neuropsychiatric lupus provides case definitions for 19 neuropsychiatric syndromes seen in SLE with reporting standards and recommendations for laboratory and imaging tests.
Objectives To test the new ACR case definitions in a cohort of 527 SLE previously analysed for neuropsychiatric lupus.
Methods Only patients with definite SLE (ACR > = 4) were included. The 19 case definitions established by ACR including diagnostic criteria, important exclusions and methods of ascertainment were utilised for the cohort. Clinical, serologic and imaging data were retrospectively reviewed. The secondary neuropsychiatric manifestations were excluded. A short neuropsychological test battery for the diagnosis of cognitive deficits, and specific tests and questionnaire for headache, mood and anxiety disorders were realised in 40 patients from the cohort and compared to normals. Patients under 14 or older than 65 were not included in this evaluation.
Results Seizures was observed in 39 patients, psychosis in 28, polyneuropathy in 20, acute confusional state in 15, cerebrovascular disease in 13, cranial neuropathy in 8, mononeuropathy in 7, myelopathy in 6, chorea in 4, asseptic meningitis in 2, Guillan- Barré, demyelinating syndrome and myasthenia gravis in one case each. Autonomic disorders and plexopathy were not observed.
Specific tests applied in 40 patients revealed 75% of cognitive disturbs,75% of mood disorders and 70% of anxiety disorders. Headache occurred in 62.5%.of these patients.
Conclusion The ACR nomenclature and case definitions for neuropsychiatric lupus is useful for facilitate clinical research, in special multicentric studies and series with a great number of patients. Headache was a common symptom in SLE, however some conditions such as plexopathy or autonomic disorders were rare. Specific battery tests and questionnaire are necessary for cognitive disturbs, mood and anxiety disorders diagnostic but they are not practical for routine testing because are time consuming.
ACR AD HOC Comitte on neuropsychiatric lupus nomenclature. The American College of Rheumatology nomenclature and case definition for neuropsychiatric syndromes. Arthritis Rheum. 1999;42(4):599–608
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