Acute encephalopathy as the presenting symptom in Henoch-Schoenlein purpura: A case report

Aims: Henoch-Schonlein purpura is an acute, self-limited vasculitis syndrome characteristically associated with skin, joint, renal and gastrointestinal manifestations. We report a patient with the rare complication of neurologic involvement in Henoch-Schoenlein purpura presenting with signs of acute encephalopathy. Case report: An 8-year-old boy was hospitalised due to severe dehydration because of gastroenteritis. On hospitalisation, he presented with somnolence, horizontal nystagmus, paresis of the left leg, dysarthria and anisocoria. By cerebrospinal fluid analysis and cranial CT infectious or bleeding complication could be excluded. The EEG showed diffuse slowing of background activity. In the course of symptomatic treatment, symptoms improved. After two days in hospital, scrotal petechiae in combination with abdominal pain led to the diagnosis of Henoch-Schoenlein purpura. Treatment with prednisone (2mg/kg/d) was initiated. Five days later, a prolonged status epilepticus complicated the course. Neuroradiologically, diffuse alteration of signal intensity in the occipital and parietal cortex was present (T2w) suggesting ischemic and oedematous lesions. Acute encephalopathy of unknown origin associated with progressive renal involvement led to histopathological analysis of renal tissue diagnosing fulminant IgA nephropathy. The boy was successfully treated with methylprednisolon pulse therapy, followed by cyclophosphamide (once monthly for 6 months) combined to daily low-dose prednisolon leading to neurological and renal remission (follow-up period: 6 months). Cortical lesions normalized as shown by repetitive brain MRI. Conclusion: Acute encephalopathy is a rare complication in Henoch-Schoenlein Purpura. Invasive histopathological investigation enabled diagnosis and specified treatment. Brain imaging reveals unspecific findings similar to vascular-ischemic lesions.