Neuropsychiatric Manifestations

Neuropsychiatric sequelae of pediatric SLE occur due to disease involvement in the central and periph eral nervous systems. Prevalence rates of neuropsy-chiatric manifestations vary widely and range from 20% to 95%; these variable rates may be due to lack of standard definitions in the literature and to the frequency of lupus-associated headaches (Benseler and Silverman 2007). Common neuropsychiatric sequelae include headaches, psychiatric manifestations (e.g., anxiety disorders, mood disorders, psychosis, cognitive dysfunction, and acute confusional state), cerebrovascular disease, seizures, and choreiform movements (Benseler and Silverman 2007).

Prevalence rates and patterns of neuropsychiatric sequelae are variable and evidenced by studies of pe-diatric patients with SLE. A retrospective chart review of pediatric SLE patients revealed that 44% of youth had neuropsychiatric occurrences (Steinlin et al. 1995). Nearly one-half of these youth experienced psychiatric manifestations, with the most common type of psychosis presenting as hallucinations and paranoia. Other reported psychiatric manifestations include severe depression and cognitive impairment or emotional lability. Additional observed manifestations included seizure, cerebrovascular accident, headache, and choreiform movements. In contrast, a differential pattern of neuropsychiatric occurrences was revealed in a retrospective chart review of pedi-atric patients with SLE (Olfat et al. 2004). For example, approximately 22% of youth had occurrences, with the most common manifestation being headache, observed in more than one-half of the youth. Fifty percent of youth also reported psychiatric symptoms consisting of depression, confusion, visual hallucinations, memory loss, and/or psychosis. Other observed neuropsychiatric manifestations included seizure, cerebrovascular accident, coma, transverse myelitis, and chorea.

A focus on psychiatric manifestations of SLE is warranted given the frequency in this population. Psychosis occurs in 12%-40% of youth with SLE and is best differentiated from idiopathic schizophrenia by the type of symptoms experienced (Benseler and Silverman 2007). The most common symptom is visual hallucinations, frequently of a threatening nature. Benseler and Silverman (2007) suggested that SPECT scans also may be helpful in differentiating whether psychosis can be attributed to SLE or idiopathic schizophrenia. Other psychiatric manifestations include mood disorders, most commonly depression, which may be organic in nature or a secondary reaction to the challenges and stressors of living with a chronic disease (Benseler and Silverman 2007).

One mechanism implicated in CNS involvement and SLE in adults includes the presence of antiphos-pholipid antibodies (aPL), although it is actually unclear whether aPL is associated with CNS involvement in youth (Harel et al. 2006). A retrospective cohort study was conducted with pediatric SLE patients to examine the association between aPL and neuropsychiatric manifestations (Harel et al. 2006). Approximately 34% of patients had neuro-psychiatric manifestations, with the most common being seizures. Other manifestations included headache, mood disorder, cognitive dysfunction, cere-brovascular accident, and psychosis. Within this cohort, a 70% prevalence of aPL was observed; however, the relationship with neuropsychiatric manifestations was weak, with the exception of cere-brovascular accident, suggesting that there may be a different mechanism for the occurrence of neuro-psychiatric sequelae in pediatric populations.

In the clinical setting, psychiatric consultations frequently are requested to assist in differentiating CNS lupus flares from corticosteroid-induced psychosis. Most of the guidance for this differential diagnosis in pediatric patients comes from case reports reported in the literature. Nonetheless, corti-costeroid-induced psychosis is believed to be rare and may be ruled out if occurring in the presence of common features of CNS lupus flares such as headache, confusion, and concentration difficulties while also demonstrating uncommon features of neuro-psychiatric SLE such as mania, head-banging, and excessive crying (Benseler and Silverman 2007).

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