Revision 8 for 'Megalencephalic leukoencephalopathy with subcortical cysts'All Revisions - View changeset
Van der Knapp disease
Van der Knapp disease refers to a rare diffuse leukoencephalopathy associated with cystic degeneration of the white matter of the brain.
Patients typically present with megalencephaly during the 1st year of life
It is sometimes classified as a megalencephalic leuckoencepalopathy.
It is thought to carry an autosomal recessive inheritance 4.
Elevated levels of glycine has been reported in CSF 1.
Shows a megalencephalic brain with bilateral cystic lesions of CSF intensity particularly affecting the anterior temporal lobes. There can also be wide diffuse signal abnormality and features of swelling affecting white matter.
It is named after Marjo van der Knapp - Dutch physician 4