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Johannes P. W. F. Lakke, MD; Ebel J. Ebels, MD; Otto J. ten Thye, PhD, MD

Arch Neurol. 1967;16(3):227-231.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

SINCE Leigh's publication¹ in 1951, 32 cases have been reported of a peculiar fatal encephalomyelopathy, affecting children exclusively. The clinical syndrome consists of feeding difficulties, psychomotor retardation, and, later, disturbances in the state of consciousness, ophthalmoplegia, ataxia, and muscular weakness. Beginning insidiously, often the condition deteriorates rapidly, and the child dies from respiratory complications within a few months.

At autopsy there is bilateral, symmetrical degeneration of the brain stem, but at times the spinal cord is also affected. Glial and mesodermal proliferation is present in these regions, but the neurons are often well preserved. Numerous dilated small vessels are a conspicuous finding. The condition resembles Wernicke's encephalopathy. The most remarkable differences from Wernicke's encephalopathy are the absence of pathological changes in the mamillary bodies in nearly all cases and involvement of the spinal cord.

Cases have been reported from Europe,^1-10 The United States,^11-13 Canada,¹⁴ Japan, . . . [Full Text PDF of this Article]

Author Affiliations
Groningen, Netherlands

From the departments of neurology and pathology, Hospital San Pedro di Verona, Oranjestad, Aruba (Dr. Lakke and Dr. ten Thye); and from the Department of Pathology, University of Groningen (Dr. Ebels), Netherlands.

Footnotes
Submitted for publication Sept 28, 1966; accepted Nov 9.

Reprint requests to Department of Neuroradiology, University Hospital, Groningen, Netherland (Dr. Lakke).

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