Dados do Trabalho

Título

REVERSIBLE POSTERIOR LEUKOENCEPHALOPATHY SYNDROME IN A PEDIATRIC PATIENT

Apresentação do caso

C.S.Y.A., ten years old, female, previous diagnosed with panniculitis-like subcutaneous T-cell lymphoma, Systemic Lupus Erythematosus (SLE) and lupus nephritis secondary to arterial hypertension. She presented digestive hemorrhage due to a perforated duodenal ulcer and mucosal laceration in the distal esophagus. In the follow up, she presented three episodes of clonic seizures and a report of headache with nocturnal awakening one hour before the onset of the crisis. She was admitted to the emergency room convulsing, requiring the use of antiseizure treatment to control the crisis. The electroencephalogram showed disorganized and symmetrical electrical activity, composed of slow waves in the theta-delta range, with a predominance of delta, irregular, medium amplitude, diffusely distributed and beta rhythm around 20 to 25 Hz predominating in frontal from slow moderator to severe base. Magnetic resonance imaging (MRI) presented extensive vasogenic edema in both posterior cerebral hemispheres (parieto-occipital lobe), thalamus and pons, suggesting Posterior Reversible Encephalopathy Syndrome (PRES).

Discussão

Subcutaneous T-cell lymphoma panniculitis-like is a subtype of primary cutaneous lymphoma, a rare disease, representing less than 1% of all cutaneous T-cell lymphomas, which may be associated with rheumatologic diseases such as systemic SLE, a chronic autoimmune inflammatory disease with clinical variability in terms of severity. PRES in patients with SLE was first described in 2006 and its pathogenesis is multifactorial. PRES is a clinical radiographic syndrome of heterogeneous etiologies that are grouped together because of similar findings on neuroimaging studies. The typical clinical syndrome includes headache, visual symptoms and seizures. Typical MRI findings are consistent with vasogenic edema in the subcortical white matter and are predominantly localized to the posterior cerebral hemispheres.

Comentários finais

A wide variety of conditions have been implicated as causes. Autoimmune diseases (such as SLE) are often associated with PRES due to side effects as hypertension with autoregulatory failure or immunosuppressive therapy used during treatment.

Referências (se houver)

1. Alehan F, Erol I, Agildere AM, et al. Posterior leukoencephalopathy syndrome in children and adolescents. J Child Neurol 2007; 22:406.
2. Chen TH, Lin WC, Tseng YH, et al. Posterior Reversible Encephalopathy Syndrome in Children: Case Series and Systematic Review. J Child Neurol 2013; 28:1378.
3. Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology and outstanding issues. Lancet Neurol 2015; 14:914.
4. Leroux G, Sellam J, Costedoat-Chalumeau N, et al. Posterior reversible encephalopathy syndrome during systemic lupus erythematosus: four new cases and review of the literature. Lupus 2008; 17:139.
5. Mak A, Chan BP, Yeh IB, et al. Neuropsychiatric lupus and posterior reversible leukoencephalopathy syndrome: a challenging clinical dilemma. Rheumatology (Oxford) 2008; 47:256.

Fonte de Fomento (se houver)

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Declaro não estar submetido a qualquer tipo de conflito de interesse junto aos participantes ou a qualquer outro colaborador, direto ou indireto.

Área

Manifestações neurológicas das doenças sistêmicas