Magnetic Resonance Imaging

MRI in patients with APBD shows extensive, often ill-defined abnormalities within the cerebral and cerebellar white matter (Figs. 16.1 and 16.2). The abnormalities are most prominent in the periventricular region and tend to spare the U fibers and the corpus callosum (Fig. 16.1). The signal change is often most pronounced within the periventricular white matter, gradually becoming more normal in the deep white matter, and merging with normal white matter in the U fibers. In other patients, however, the white matter abnormalities are better demarcated (Figs. 16.1 and 16.2). In later stages, the corpus callosum and U fibers

Polyglucosan

Fig. 16.1. FLAIR images in a 52-year-old female patient with APBD. Polyglucosan bodies were found in the sural nerve biopsy. There are confluent periventricular white matter abnormalities with relative sparing of the deep white matter and

U-fibers, where only small foci of abnormal signal are seen. Note the cerebellar and brain stem involvement. Courtesy of Dr.B.Barcelos da Nobrega,Department of Radiology,School of Medicine, Federal University of Goias, Brazil

Fig. 16.1. FLAIR images in a 52-year-old female patient with APBD. Polyglucosan bodies were found in the sural nerve biopsy. There are confluent periventricular white matter abnormalities with relative sparing of the deep white matter and

U-fibers, where only small foci of abnormal signal are seen. Note the cerebellar and brain stem involvement. Courtesy of Dr.B.Barcelos da Nobrega,Department of Radiology,School of Medicine, Federal University of Goias, Brazil

Involvement Fibers Diseases

Fig. 16.2. FLAIR images in the 49-year-old sister of the patient in Fig. 16.1. Her disease is radiologically more advanced.There are confluent signal abnormalities in the periventricular white matter with some additional focal spots. The U fibers are relatively preserved. The corpus callosum has become thin. The

Fig. 16.2. FLAIR images in the 49-year-old sister of the patient in Fig. 16.1. Her disease is radiologically more advanced.There are confluent signal abnormalities in the periventricular white matter with some additional focal spots. The U fibers are relatively preserved. The corpus callosum has become thin. The temporal white matter is prominently involved. Note the cerebellar and brain stem involvement. Courtesy of Dr. B. Barcelos da Nobrega, Department of Radiology, School of Medicine, Federal University of Goias, Brazil become involved as well (Fig. 16.2). The cerebral white matter may be decreased in volume with widening of the lateral ventricles and subarachnoid spaces and thinning of the corpus callosum. The frontal lobe tends to be involved more seriously, with respect to both the white matter abnormalities and the atrophy. Within the brain stem signal abnormalities may be seen in the long tracts (Figs. 16.1 and 16.2). The cerebellum, brain stem, and spinal cord may be atrophic.

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Responses

  • jemima
    What are foci of abnormal signal within deep periventricular white matter tracts?
    8 years ago

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