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Its Occurrence With Periventricular Diffuse Low Density on Computed Tomography Scan

Toshiko Okamoto, MD; Keisuke Mizuno, MD; Mitsuo Iida, MD; Itsuro Sobue, MD; Masakuni Mukoyama, MD

Arch Neurol. 1981;38(7):423-426.

Abstract
• Familial cases of progressive external ophthalmoplegia, deafness, generalized weakness, and hypogonadism were studied. A muscle biopsy specimen showed increased amounts of glycogen particles, lipid droplets, and mitochondria that frequently contained paracrystalline inclusion bodies between intramitochondrial and extramitochondrial membranes. Involvement of the CNS was suspected from a computed tomography scan that revealed diffuse, low-density deep cerebral white matter. Therapy with corticosteroids (prednisolone) was effective for recovery of muscular strength in the extremities. Possible involvement of the CNS in ophthalmoplegia-plus might be related to an abnormal metabolism of mitochondria.

Author Affiliations
From the First Department of Internal Medicine, School of Medicine, Nagoya University, Nagoya, Japan (Drs Okamoto, Mizuno, Iida, and Sobue), and the National Center for Nervous, Mental and Muscular Disorders, Tokyo (Dr Mukoyama).

Footnotes
Accepted for publication Oct 27, 1980.

Reprint requests to the First Department of Internal Medicine, School of Medicine, Nagoya University, Tsuramai-cho, 65, Showa-ku, Nagoya, Japan (Dr Okamoto).