Neuropsychiatric symptoms of 10 children (8 girls and 2 boys, ages 7.5 to 17 years) with systemic lupus erythematosus were studied at the Children’s Hospital Los Angeles, CA. Psychiatric symptoms at presentation included withdrawal (2), confusion (4), disorganized behavior (3), and sleep disorder (4). Psychosis in 7 was manifested by auditory, visual, and tactile hallucinations, and persecutory or bizarre delusions. Mood was depressed (6) or apathetic, irritable, anxious, or manic. Affect was constricted or blunted (5), labile or flat. One-half the patients were suicidal, and all exhibited poor attention and concentration. Neurologic symptoms in 8 patients included seizures (7) with status epilepticus (1), optic neuritis (2) with transverse myelitis (1), peripheral neuropathy (2), and headache (2). CSF was normal except for increased pressure in 2. EEGs recorded in 8 were normal. Serum anti-neuronal antibodies were elevated at onset and declined with resolution of symptoms. Anti-phospholipid antibodies were positive in 4. MRI showed a pontine infarct in one of 9 studied. SPECT was abnormal in all 10 patients, showing multiple small cortical defects with reduced cerebral perfusion. Neuropsychiatric symptoms improved within 6 months, but chronic sequelae including neuropathy and cognitive problems were frequent. Psychosis and depression recurred after intervals of 2 and 5 years in 2 patients. [1]

COMMENT. Neuropsychiatric disorder (seizures or psychosis) is included among the diagnostic criteria for systemic lupus erythematosus (American College of Rheumatology 1997). Children with an acute onset of delirium, psychosis, confusion, depression, or mania, with or without seizures or other neurologic symptoms, should be investigated for systemic lupus erythematosus. Diagnosis may be confirmed by serum anti-neuronal antibody (ANAB) testing, and resolution of symptoms is correlated with reduction in ANAB levels. In the above study, SPECT was abnormal in all patients and remained abnormal at follow-up.

Cerebral vein thrombosis as a complication of SLE was reported in 3 girls admitted with persistent headache at the Hospital for Sick Children, Toronto (see Progress in Pediatric Neurology III, PNB Publ, 1997; 173-4). [2]

Autoimmune-mediated, childhood onset obsessive-compulsive disorder and tics are the subject of a review [3] from the National Institute of Mental Health, Bethesda, MD. In this subgroup of PANDAS there is a temporal association between Group A Beta Hemolytic Streptococcal infection and onset or exacerbation of symptoms. If a causal link can be confirmed, antibiotic treatment may be justified as an adjunct therapy of OCD and tics.

SPECT findings in early-onset OCD. Regional cerebral blood flow (rCBF) SPECT was measured in 13 early-onset (<10 years) and 13 late-onset (>12 years) adult OCD subjects and in 22 healthy controls, in a study at the University of Sao Paulo Medical School, Brazil. Early-onset OCD subjects showed decreased rCBF in the right thalamus, left anterior cingulate, and both inferior prefrontal cortices relative to late-onset cases (p<.0005), and severity of OCD symptoms corresponded with left orbitofrontal rCBF. The mechanisms involved in OCD may differ according to age at onset of symptoms. [4]