Leukoencephalopathy with Bilateral Anterior Temporal Lobe Cysts

Abstract

Recently, several reports describing patients with a nonprogressive clinical course, increased signal in the cerebral white matter, and cystic changes in the anterior temporal lobes on magnetic resonance imaging (MRI) have appeared. To date, 25 patients with this very distinctive condition have been described. We report four new cases of this newly recognized entity. All have been identified primarily because of the distinctive MRI features consisting of the very unusual anterior temporal lobe cystic changes. The clinical features are characterized by severe, disabling, but nonprogressive mental and motor retardation. Magnetic resonance spectroscopy has shown increased myo-inositol and decreased N-acetylaspartate in the cerebral white matter. This is a distinctive, probably genetic, condition with characteristic neuroimaging and clinical features. In the appropriate clinical situation, the neuroimaging features are diagnostic. (J Child Neurol2006; 21:419—422; DOI 10.2310/7010.2006.00092).

Get full access to this article

View all access options for this article.

References

Oliver M. , Lenard HG , Aksu F. , Gartner J. : A new leukoencephalopathy with bilateral anterior temporal lobe cysts. Neuro-pediatrics 1998;29:225—228.

Deonna T. , Roulet E. , Vallee L. , et al: Bilateral temporal cysts, focal leucodystrophy, and non-progressive encephalopathy in three children: A special entity, abstract? Dev Med Child Neurol 1999;40:A18.

Gomes AL , Vieira JP , Saldanha J. : Non-progressive leukoencephalopathy with bilateral temporal cysts. Eur J Paediatr Neurol 2001; 5:121—125.

Wassmer E. , Barrat L. , Green SH , Whitehouse WP : Siblings with developmental delay, mild spasticity and subcortical cysts: A further leukoencephalopathy? Eur J Paediatr Neurol 2001;5: 169—173.

Bertini R. , Bianchi MC , Tosetti M. , et al: Leukoencephalopathy with bilateral anterior temporal lobe cysts: A further case of this new entity. J Child Neurol 2002;17:773—776.

Sener RN : Leukoencephalopathy with patchy lesions, temporal cystic degeneration, and normal head size. Comput Med Imaging Graph 2003 ;27:87-91.

Grosso S. , Cerase A. , De Stefano N. , et al: Non-progressive leukoencephalopathy with bilateral anterior temporal cysts: A case report and review of the literature. Brain Dev 2005; 27:73—77.

Wolf NI , Harting I. , Boltshouser E. , et al: Leukoencephalopathy with ataxia, hypodontia and hypomyelination . Neurology 2005; 64:1461—1464.

Henneke M. , Preuss N. , Engelbrecht V. , et al: Cystic leukoencephalopathy without megalencephaly: A distinct disease entity in 15 children. Neurology 2005 ;64:1411—1416.

10.

Kaye EM : Update on genetic disoders affecting white matter. Pediatr Neurol 2001;24:11—24.

11.

Van der Knaap MS , Barth PG , Stroink H. , et al: Leukoencephalopathy with swelling and a discrepantly mild clinical course in eight children. Ann Neurol 1995;37:324—334.

12.

Schiffmann R. , Moller JR , Trapp BD , et al: Childhood ataxia with diffuse central nervous system hypomyelination. Ann Neurol 1994;35:331—340.

13.

Van der knaap MS , Leegwater PA , Konst AA , et al: 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.

14.

Espay AJ , Bodensteiner JB , Patel H. : Episodic coma in a new leukodystrophy. Pediatr Neurol 2002;26:139—142.

15.

Tatli B. , Ozmen M. , Aydinli N. , Caliskan M. : Not a new leukodystrophy but a congenital cytomegalovirus infection. J Child Neurol 2005;20:525—527.