Psychiatric manifestations in posterior reversible encephalopathy syndrome

Case presentation: L.S.O., female, 15 years old, hospitalized for peaks of fever, anemia, positive direct coombs, hypocomplementemia and proteinuria >0.5 g/24h. Pulse therapy with methylprednisolone was prescribed for the hypothesis of systemic lupus erythematosus (SLE). Evolved with severe headache and convulsive crises presenting cortical, subcortical, posterior and bilateral hypodensity on cranial tomography. Phenobarbital 150mg/d was started, lamotrigine 25mg/d and due to the persistence of the seizures, phenytoin 300mg/day, valproic acid 1500mg/day and hydroxychloroquine 400mg/d were associated. She had positive antiphospholipid antibodies and, due to severe lupus activity, a high Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) 31 was verified. She was again treated with methylprednisolone and cyclosporine with maintenance of prednisone 60 mg and AAS 100 mg/d. Cerebral resonance angiography without alterations. During follow-up, the patient had SLEDAI 39 and was started on 20mg/d of citalopram and 4mg/d of clonazepam and did not experience new convulsive events and psychogenic crises.

Discussion: Posterior reversible encephalopathy syndrome (PRES) is diagnosed clinically and radiologically and is characterized by reversible subcortical vasogenic cerebral edema, with characteristic neuroimaging features¹. PRES has been attributed to many etiologies, including SLE and drug toxicity². It occurs in <1% of these patients, with a higher incidence in young people, with a SLEDAI Index ≥6 and associated comorbidities³. Clinical manifestations include seizures, encephalopathy, “confusion” and “altered mental function” ¹. A proposed mechanism of PRES in SLE patients is T cell activation resulting in the production of inflammatory cytokines, which may contribute to brain endothelial dysfunction. Cytotoxic drugs such as cyclosporine, often used to treat SLE and other inflammatory diseases, can also induce PRES ⁴. Psychiatric symptoms occurred before, during, or after the onset of PRES, which is consistent with evidence of psychiatric morbidities in neurological disorders. Despite the term reversible, residual infarctions and subsequent leukomalacia are recognized sequelae of PRES¹, supporting the likelihood of long-term psychiatric symptoms⁵.

Final comments: The diagnosis of PRES requires high clinical and imaging suspicion, and it is necessary to consider it as a rare differential diagnosis for acute changes in mental status.

 

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Artikel online veröffentlicht:
18. September 2023