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Brain stem atrophy in Joseph disease: a morphometric study using two-dimensional (area) measurement by computed
Y Fukutani1, K Katsukawa, Y Ishizaki
1Division of Neuropsychiatry, National Sanatorium Hokuriku Hospital, Toyama, Japan.
Journal of Neurology
|July 1, 1991
Summary
Computed tomography (CT) revealed significant pontine atrophy and fourth ventricular enlargement in Japanese patients with Joseph disease. These findings suggest CT morphometry aids in diagnosing this neurological condition.
Area of Science:
- Neurology
- Radiology
- Neuroscience
Background:
- Joseph disease is a rare, inherited neurodegenerative disorder.
- Previous studies suggest infratentorial abnormalities in Joseph disease.
Purpose of the Study:
- To investigate infratentorial structural changes in Japanese patients with Joseph disease using computed tomography (CT).
- To evaluate the utility of CT morphometry in the clinical diagnosis of Joseph disease.
Main Methods:
- Morphometric analysis of the infratentorial region using two-dimensional CT measurements.
- Calculation of brain-stem index, fourth ventricular index, and cerebellar index.
- Comparison of measurements between eight Japanese patients with Joseph disease and 16 healthy controls.
Main Results:
- Significant decrease in the brain-stem index (indicating pontine atrophy) in patients compared to controls (P < 0.01).
- Significant increase in the fourth ventricular index (indicating fourth ventricular dilatation) in patients compared to controls (P < 0.01).
- No significant difference in the cerebellar index, but increased visible sulci in the cerebellum of patients.
Conclusions:
- CT morphometry demonstrates severe pontine atrophy and fourth ventricular dilatation in Joseph disease.
- Mild cerebellar involvement is suggested by increased sulcal visibility.
- CT-based morphometric evaluation is a potentially valuable tool for the clinical diagnosis of Joseph disease.