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Cree leukoencephalopathy: neuroimaging findings
I A Alorainy1, Y G Patenaude, A M O'Gorman
1Department of Medical Imaging, Montreal Children's Hospital, Quebec, Canada.
Insights
Cree leukoencephalopathy shows distinct imaging patterns on CT and MR, with diffuse white matter abnormalities and sparing of specific brain regions. This helps in diagnosing this rare neurological condition.
Area of Science:
- Neurology
- Radiology
- Pediatric Neurology
Background:
- Cree leukoencephalopathy is a rare genetic neurological disorder.
- Accurate diagnosis relies on characteristic neuroimaging findings.
Purpose of the Study:
- To detail the computed tomographic (CT) and magnetic resonance (MR) imaging features of Cree leukoencephalopathy.
- To aid in the identification and diagnosis of this condition.
Main Methods:
- Retrospective review of medical records and neuroimaging (CT and MR) in 12 infants.
- Diagnosis confirmed clinically or by autopsy.
Main Results:
- CT revealed diffuse, symmetric white matter hypoattenuation in cerebral and cerebellar regions.
- MR imaging showed hyperintensity in white matter, internal capsule, corpus callosum, and globus pallidus on T2-weighted images.
- Caudate nucleus and putamen were consistently spared in all imaging modalities.
Conclusions:
- Cree leukoencephalopathy presents with characteristic symmetric and diffuse white matter and brainstem involvement.
- Imaging findings, particularly the sparing of caudate nucleus and putamen, are key diagnostic indicators.
Purpose:
To describe the computed tomographic (CT) and magnetic resonance (MR) imaging findings in Cree leukoencephalopathy.
Materials And Methods:
The authors retrospectively reviewed the medical records and neuroimaging studies in 12 infants with Cree leukoencephalopathy (CT in 12 infants, MR in six). The diagnosis was established clinically in six patients and at autopsy in the other six.
Results:
At CT, extensive, diffuse, and symmetric hypoattenuation was seen in the cerebral and cerebellar white matter in all 12 patients. Hypoattenuation was also seen in the corpus callosum in 11 (92%), internal capsule in 10 (83%), globus pallidus in nine (75%), brainstem in nine (75%), and thalamus in four (33%). The caudate nucleus and putamen were spared. On T2-weighted MR images in six patients, the cerebral and cerebellar white matter, including the subcortical arcuate fibers, was hyperintense as were the internal capsule, corpus callosum, corticospinal tracts, and globus pallidus. The thalamus was affected in four (67%) patients, pons in five (83%), and medulla in four (33%). The caudate nucleus and putamen were not affected.
Conclusion:
Cree leukoencephalopathy causes striking symmetric and diffuse involvement of the cerebral and cerebellar white matter and brainstem with sparing of the caudate nucleus and putamen.