Brain MR imaging findings in two patients with Alpers' syndrome

We describe the magnetic resonance (MR) imaging findings in two patients with the clinical diagnosis of progressive neuronal degeneration of childhood with liver disease (Alpers' syndrome). One patient showed atrophy of both occipital lobes, and one patient showed high signal intensity in deep gray matter nuclei and diffuse atrophy. Although the imaging findings were nonspecific, they correlated well with the patients' clinical findings.