Cranial CT in the Sjögren-Larsson syndrome

Cranial computed tomography was performed on five patients with the Sjögren-Larsson syndrome (SLS). No specific morphological abnormalities were found, nor was there any indication of a reduction of brain mass. This result is in keeping with the findings on gross examination of brain specimens of subjects with SLS but not with some previously reported PEG findings. The lack of agreement is discussed. The slowly progressive destruction of neuronal substance in the course of the chronic, degenerative process in the brain and spinal cord is obviously not paralleled by a corresponding loss of brain mass nor does it result in any alteration of the shape of the brain. The probable tissue decomposition was not striking enough in our five patients to cause attenuation changes which can be detected by CT. Neuroradiological examinations, including CT, are of no practical value at present for the diagnosis, therapy or prognosis of the disease.