Appendix A: Case Definitions for Neuropsychiatric Syndromes in Systemic Lupus Erythematosus
Acute Confusional State
Disturbance of consciousness or level of arousal characterized by reduced ability to focus, maintain, or shift attention, and accompanied by disturbances of cognition, mood, affect, and/or behavior. The disturbances typically develop over hours to days and tend to fluctuate during the course of the day. They include hypo- and hyperaroused states and encompass the spectrum from delirium to coma.
NB: "Acute Confusional State" is equivalent to "delirium" defined in DSM-IV and ICD-9 as an observable state of impaired consciousness, cognition (including perception), mood, affect, and behavior. The definitions of delirium in DSM-III, DSM-III-R, and DSM-IV have been tested for reliability, and the committee wanted a definition that would conform to ICD-9, WHO, and DSM-IV.
Neurologists often use "encephalopathy" where psychiatrists use "delirium" to describe the same clinical state. Encephalopathy is defined in neurologic texts as a diffuse cerebral dysfunction associated with a disturbance in consciousness, cognition, and mood, affect, and behavior. It implies a physiologic etiology and is usually used with descriptors of various metabolic disorders.
"Organic brain syndrome" is not recommended for usage since there is better studied terminology.
Acute confusional states are generally accompanied by cognitive deficits. If cognitive deficits are the only CNS manifestations, the illness should be recorded as "Cognitive Dysfunction."
Diagnostic criteria:
Disturbance of consciousness or level of arousal with reduced ability to focus, maintain, or shift attention, and one or more of the following developing over a short period of time (hours to days) and tending to fluctuate during the course of the day:
A. Acute or subacute change in cognition that may include memory deficit and disorientation.
B. A change in behavior, mood, or affect (e.g., restlessness, overactivity, reversal of the sleep/wakefulness cycle, irritability, apathy, anxiety, mood lability, etc.)
Exclusions:
- Primary mental/neurologic disorder not related to SLE.
- Metabolic disturbances (including glucose, electrolytes, fluid, osmolarity)
- Substance or drug-induced delirium (including withdrawal)
- Cerebral infections
NB: Preexisting cognitive deficits are not an exclusion. If acute confusional state is superimposed on preexisting cognitive deficits, diagnose both.
Associations:
- Marked psychosocial stress
- Corticosteroid use
- Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome
Ascertainment:
Disturbed consciousness: Clinical observation, mental status, and neurologic examination.
Cognitive function: Mental status examination, including instruments such as the Mini Mental Status Examination (32). For more detailed assessment, see Cognitive Dysfunction category.
Mood and behavioral dysfunction: Clinical observation, history by patient and others, standardized instruments (e.g., Hospital Anxiety and Depression Scale) (35).
Determine from the individual or from informants the impact of disturbance on daily life, previous occupational and social functioning.
Record:
- Basic descriptors
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