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Non-progressive leukoencephalopathy with bilateral anterior temporal cysts: a case report and review of the literature

      Abstract

      A newly described disease is characterized by anterior bilateral temporal lobe cysts associated with multilobar leukoencephalopathy and a non-progressive clinical course. We report a patient with bilateral anterior temporal lobe cystic changes associated with a non-progressive neurological disorder, microcephaly, spasticity, mental retardation, and sensorineural deafness. From the literature, 12 other patients have shown a similar phenotype. The common neuroradiological findings in these patients have been bilateral anterior temporal lobe cystic changes and non-progressive leukoencephalopathy. By contrast, variability in the clinical phenotype has been observed, ranging from severe neuromotor handicap with mental retardation and microcephaly to spasticity in the lower limbs associated with normal cognitive function. The pathological basis of the defect remains to be defined.

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      References

        • van der Knaap M.S.
        • Barth P.G.
        • Stroink H.
        • van Nieuwenhuizen O.
        • Arts W.F.
        • Hoogenraad F.
        • Valk J.
        Leukoencephalopathy with swelling and a discrepantly mild clinical course in eight children.
        Ann Neurol. 1995; 37: 324-334
        • van der Knaap M.S.
        • Leegwater P.A.
        • Konst A.A.
        • Visser A.
        • Naidu S.
        • Oudejans C.B.
        • Schutgens R.B.
        • Pronk J.C.
        Mutations in each of the five subunits of translation initiation factor eIF2B can cause leukoencephalopathy with vanishing white matter.
        Ann Neurol. 2002; 51: 264-270
        • Anlar B.
        • Yalaz K.
        • Saatci I.
        Bilateral temporal cysts in a case of subacute sclerosing panencephalitis.
        Eur J Paediatr Neurol. 2003; 7: 81-84
        • Olivier M.
        • Lenard H.G.
        • Aksu F.
        • Gartner J.
        A new leukoencephalopathy with bilateral anterior temporal lobe cysts.
        Neuropediatrics. 1998; 29: 225-228
        • De Stefano N.
        • Balestri P.
        • Dotti M.T.
        • Grosso S.
        • Mortilla M.
        • Morgese G.
        • Federico A.
        Severe metabolic abnormalities in the white matter of patients with vacuolating megalencephalic leukoencephalopathy with subcortical cysts. A proton MR spectroscopic imaging study.
        J Neurol. 2001; 248: 403-409
        • Deonna T.
        • Roulet E.
        • Vallée L.
        • Thalman H.
        • Blumberg A.
        • Maeder P.
        Bilateral temporal cysts, focal leukoencephalopathy, and non progressive encephalopathy in three children: a special entità? (Abstract).
        Dev Med Child Neurol. 1999; 40: 18
        • Gomes A.L.
        • Vieira J.P.
        • Saldanha J.
        Non-progressive leukoencephalopathy with bilateral temporal cysts.
        Eur J Paediatr Neurol. 2001; 5: 121-125
        • Wassmer E.
        • Barrat L.
        • Green S.H.
        • Whitehouse W.P.
        Siblings with development delay, mild spasticity and subcortical cysts: a further leukoencephalopathy?.
        Eur J Paediatr Neurol. 2001; 5: 169-173
        • Battini R.
        • Bianchi M.C.
        • Tosetti M.
        • Guzzetta A.
        • Cioni G.
        Leukoencephalopathy with bilateral anterior temporal lobe cysts: a further case of this new entity.
        J Child Neurol. 2002; 17: 773-776
        • Sener R.N.
        Leukoencephalopathy with patchy lesions, temporal cystic degeneration, and normal head size.
        Comput Med Imaging Graph. 2003; 27: 87-91
        • Barkovich A.J.
        • Lindan C.E.
        Congenital cytomegalovirus infection of the brain: imaging analysis and embryologic considerations.
        Am J Neuroradiol. 1994; 15: 703-715