Appendix A: Case Definitions for Neuropsychiatric Syndromes in Systemic Lupus Erythematosus

Cognitive Dysfunction

Significant deficits in any or all of the following cognitive functions: simple or complex attention, reasoning, executive skills (e.g., planning, organizing, sequencing), memory (e.g., learning, recall), visual-spatial processing, language (e.g., verbal fluency), and psychomotor speed. Cognitive dysfunction implies a decline from a higher level of functioning and ranges from mild impairment to severe dementia. It may or may not impede social, educational, or occupational functioning, depending on the function(s) impaired and the severity of impairment. Subjective complaints of cognitive dysfunction are common and may not be objectively verifiable. Neuropsychological testing should be done in suspected cognitive dysfunction, and its interpretation should be done with a neuropsychologist.

Diagnostic criteria:

1. Documented impairment in one or more of the following cognitive domains:
   1. Simple attention
   2. Complex attention
   3. Memory (e.g., learning and recall)
   4. Visual-spatial processing
   5. Language (e.g., verbal fluency)
   6. Reasoning/problem solving
   7. Psychomotor speed
   8. Executive functions (e.g., planning, organizing, and sequencing)
2. The cognitive deficits represent a significant decline from a former level of functioning (if known).
3. The cognitive deficits may cause varying degrees of impairment in social, educational, or occupational functioning, depending on the function(s) impaired and the degree of impairment.

Associations:

• Substance abuse
• Medication (steroids, sedatives)
• History of learning disabilities
• History of head injury
• Other primary neurologic and psychiatric disorders
• Metabolic disturbances, particularly uremia and diabetes
• Antiphospholipid antibody syndrome
• Coexisting emotional distress, fatigue, and pain.

Ascertainment:

1. Standardized neuropsychological tests to evaluate cognitive domains (79,80) include*:
   • Simple Attention: Ability to register and maintain information. Suggested tests include Digit Span (Forward), Continuous Performance Test.
   • Complex Attention/Executive Functions: Ability to manipulate information and switch mental set. Examples of tests: Trail Making Test (Part B), Digit Span (Backward), Paced Auditory Serial Addition Test, WAIS III Letter-Number-Sequencing, Stroop Color-Word Test (interference), Wisconsin Card Sorting Test, Category Test.
   • Memory: Ability to register, recall, and recognize information. Tested by presenting verbal and nonverbal stimuli which have to be reproduced immediately and after a delay and recognized in the context of other stimuli. Examples of tests: California Verbal Learning Test, Rey Auditory-Verbal Learning Test, Wechsler Memory Scale, Rey-Osterrieth Complex Figure Test Recall, Continuous Visual Memory Test
   • Visual-Spatial Processing: Ability to analyze, synthesize, and manipulate visual-spatial information. Tested by presenting visual patterns to be copied, reconstructed, reorganized, or reoriented. Examples of tests: Rey-Osterrieth Complex Figure Test Copy, WAIS-R/III Block Design, Judgment of Line Orientation, Facial Recognition Test, Hooper Visual Organization Test.
   • Language: Ability to comprehend, repeat, and produce oral and written material. Examples of tests: Controlled Oral Word Association Test, Thurstone Word Fluency Test, Animal Naming Test, Boston Naming Test, Boston Diagnostic Aphasia Examination (select subtests), Token Test.
   • Reasoning/Problem Solving: Ability to reason and abstract. Tests include: WAIS-R/III Comprehension, WAIS-R/III Similarities, Raven’s Progressive Matrices, WAIS-III Matrix Reasoning.
   • Psychomotor Speed: Test the ability to rapidly process and produce oral and written information. Appropriate tests include Trail Making Test (Part A), WAIS-R/III Digit Symbol Substitution Test, Stroop Color-Word Test (word-reading and color-naming speed), Simple Reaction Time.
   • Motor Function: Manual speed, dexterity, and strength. Tests include Finger Tapping, Grooved Pegboard, Grip Strength.
2. Estimate premorbid level of functioning using currently accepted methods, e.g., demographic data and/or scores on a vocabulary or word-reading test and/or best performance method. A significant discrepancy from premorbid estimate necessary to identify cognitive impairment.
3. Determine impact of cognitive dysfunction on social, educational and/or occupational functioning through interview with patients and significant others and standardized quality-of life-questionnaires such as the SF-36 (37).

Record:

• Basic descriptors with these modifications:
• Type of cognitive dysfunction: Describe areas of deficit.
• Severity: Mild, moderate, and severe dysfunction to be defined by the range of deficits, extent or degree of decline, and impact on everyday functioning. Severe cognitive dysfunction may be limited to a profound disturbance in a single cognitive domain, or it may involve multiple cognitive deficits that include memory and at least one other cognitive domain ("dementia"). Severe and moderate cognitive dysfunction include impairment in everyday functioning. Mild dysfunction may exist in the absence of significant impairment in everyday functioning.
• Duration: Period of time during which current cognitive problems exist as reported by self or by formal testing.
• Objective vs. Subjective Data: Patient reports of cognitive dysfunction should be categorized as "subjective - not tested" or "subjective - tested and not verified."

(References: 32, 34, 71-80)

* See also suggested battery in Appendix C.

To Index | Previous (Cerebrovascular Disease) | Next (Demyelinating Syndrome) | To References