[Globoid cell leukodystrophy (Krabbe's disease)]. / Globoidnokletochnaia leikodistrofiia Krabbe.

ABSTRACT

One case of Krabbe's disease was found for 25 years among 40500 newborns; this concerned a girl who died from the aspiration pneumonia at the age of 8.5 months. A number of typical one- and multinuclear globoid cells were observed in white matter of the large hemispheres and brain stem. Brain was atrophied by 27%. Thrombosis of some brain vessels was noted microscopically in the zone affected, this being attributed to the cytomegalovirus angiotoxic effect. The presence of polynuclear leukocytes in the nervus trigeminus branches is considered to be an equivalent of the brain globoid cells that phagocytize the products of the myelin destruction in the peripheral nerves.