Megalencephalic Leukoencephalopathy with Subcortical Cyst: A Case Report

Authors

  • K Gangadhar Department of Radiodiagnosis and imaging, Institute of Medical Sciences, Banaras Hindu University, Varanasi,
  • S Patwari Department of Radiodiagnosis and imaging, Institute of Medical Sciences, Banaras Hindu University, Varanasi
  • A Verma Department of Radiodiagnosis and imaging, Institute of Medical Sciences, Banaras Hindu University, Varanasi
  • - Kaviyarasy Department of Radiodiagnosis and imaging, Institute of Medical Sciences, Banaras Hindu University, Varanasi

DOI:

https://doi.org/10.3126/njr.v2i2.7691

Keywords:

Macrocephaly, Megalencephalic leukoencephalopathy, Subcortical cysts

Abstract

Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare disease first described by van der Knaap et al, in 1995. MLC is a relatively new entity of neurodegenerative disorder of autosomal recessive inheritance characterized by infantile onset macrocephaly, cerebral leucoencephalopathy, mild neurological symptoms and an extremely slow course of functional deterioration. The degree of macrocephaly is variable and can be as much as 4-6 SD above the mean. Almost all patients have seizures from an early age. Some patients have died in their 2nd and 3rd decades but few may live till fourth decade. We report a case of 5-year-old girl diagnosed to have this disease.

Nepalese Journal of Radiology; Vol. 2; Issue 2; July-Dec. 2012; 76-80

DOI: http://dx.doi.org/10.3126/njr.v2i2.7691

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Published

2013-03-03

How to Cite

Gangadhar, K., Patwari, S., Verma, A., & Kaviyarasy, .-. (2013). Megalencephalic Leukoencephalopathy with Subcortical Cyst: A Case Report. Nepalese Journal of Radiology, 2(2), 76–80. https://doi.org/10.3126/njr.v2i2.7691

Issue

Vol. 2 No. 2 (2012)

Section

Case Reports