[Cherry red spot without secure evidence of sphingolipidosis (author's transl)].

The occurence of bilateral cherry red spot without neurological disorders is a rare event. The reported case is a girl aged 14 years, whose only complaint was slight deterioration of vision. No secure enzymatic defects was found on examination of leucocytes. Though the cherry red spot is hardly to be explained in another way than by storage in optic ganglion cells. Static perimetry revealed a paracentral depression of contrast sensitivity.