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Leukoencephalopathy with bilateral anterior temporal lobe cysts is a nonprogressive neurological disorder marked by intellectual deficit, spasticity and motor retardation associated with characteristic MRI findings of anterior bilateral temporal lobe cysts and multilobar leukoencephalopathy. So far, around 30 cases have been reported in the literature. Onset occurs in the first few months of life. Sensorineural deafness and microcephaly have also been reported. The etiology is unknown but an autosomal recessive mode of inheritance has been suggested.
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
No clinical trials have been registered for leukoencephalopathy with bilateral anterior temporal lobe cysts.
3 publications have been identified in PubMed for leukoencephalopathy with bilateral anterior temporal lobe cysts. Research spans Case Report / Case Series (100%).
V P (2025). [PMID: 39726909](https://pubmed.ncbi.nlm.nih.gov/39726909/). *Radiology case reports*. [Case Report / Case Series]
Retal H (2025). [PMID: 40255831](https://pubmed.ncbi.nlm.nih.gov/40255831/). *Cureus*. [Case Report / Case Series]
Algahtani H (2025). [PMID: 39481545](https://pubmed.ncbi.nlm.nih.gov/39481545/). *Encephalitis (Seoul, Korea)*. [Case Report / Case Series]
Data assembled from 3 of 12 sources · Last updated Sep 18, 2026, 9:43 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center