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Il Giornale Italiano di Radiologia Medica 2019 Maggio-Giugno;6(3):213-9

DOI: 10.23736/S2283-8376.19.00176-1


language: English, Italian

A very rare case of pediatric Weston-Hurst Syndrome

Pierluigi LUPO 1 , Guglielmo STICCHI 2, Alfredo PAUCIULO 2, Fernando A. LUPO 2, Alberto GAETA 3, Marco ROSSI 4

1 Unit of Radiodiagnostics, Department of Diangostic Imaging, OO.RR. Ospedali Riuniti di Foggia, Foggia, Italy; 2 Unit of Neuroradiology, Vito Fazzi Hospital, ASL Lecce, Lecce, Italy; 3 Unit of Radiodiagnostics, Giovanni XXIII Hospital, A.U.O. Consorziale Policlinico di Bari, Bari, Italy; 4 Unit of Neurology, Giovanni XXIII Hospital, A.U.O. Consorziale Policlinico di Bari, Bari, Italy


Acute hemorrhagic leukoencephalopathy (AHLE) represents a serious and rare demyelinating disease in the ADEM group, characterized by a rapidly progressive acute inflammation of the white substance, often with a fulminant course. AW, 5-year-old, born at term, with normal psychomotor development, comes in PS for a reported convulsive critical episode preceded, a few weeks before, by fever and vomiting. Therefore, it executes brain CT with the finding of multiple areas of hypodensity above and below tentorial bilaterally. MRI showed multiple and widespread areas of altered signal of white matter, under- and supratentorial, iso-hypointense in T1 and hyperintense in T2, with contrast enhancement after MdC, suggestive of infectious-inflammatory lesions. The infectivological evaluation and the parasitological tests gave a negative result against a leukocytosis with a prevalence of PMN and a not significant increase in PCR and PCT. The child was therefore treated with IV immunoglobulin therapy, methylprednisolone, acyclovir, antiepileptic and antibiotic therapy and subjected to repeated cycles of plasmapheresis. Subsequent MRI controls showed improvement of the iconographic findings with almost total resolution of the encephalic lesions to a control about 60 days from the onset. The case demonstrates the crucial role of MRI in the timely diagnosis of a rare case of Syndrome of Weston-Hurst pediatric with positive evolution.

KEY WORDS: Leukoencephalitis, acute hemorrhagic; Demyelinating diseases; Disease managemen

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