Magnetic resonance imaging in neuronal ceroid lipofuscinosis

Neuronal ceroid lipofuscinosis is a group of neurodegenerative disorders characterized by accumulation of lipofuscin and/or ceroid within the tissues of the body. These entities are manifest by visual, intellectual, and motor deterioration as well as recurrent seizures. Computed tomography has been shown to demonstrate changes of cerebral atrophy in more severely affected patients. Seven patients with neuronal ceroid lipofuscinosis were examined with both computed tomography and magnetic resonance imaging, and the results were correlated with the clinical severity of the disorder. Two less severely affected patients had normal results on computed tomography and magnetic resonance imaging studies. Varying degrees of cerebral atrophy were seen in the remaining five patients with both computed tomography and magnetic resonance imaging. Severity of atrophy correlated with the severity of disability in these patients. Abnormal white matter was seen in the two most severely affected patients only with magnetic resonance imaging. Although the findings in patients with neuronal ceroid lipofuscinosis were nonspecific, the increased sensitivity of magnetic resonance imaging for subtle white matter abnormalities over computed tomography may prove helpful in monitoring the progression of this rare disorder.