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Alexander Disease, Rosenthal Fiber Encephalopathy

Smith ABS - MedPix (2008)

View Article: MedPix Image - MedPix Case

Affiliation: Uniformed Services University

ABSTRACT

Diagnosis: Alexander Disease, Rosenthal Fiber Encephalopathy

History: Six month old baby boy with intermittent vomiting for 7 weeks Now listless. Parents say his head is too big.

Findings: • Low T1 Signal involving the periventricular white matter and high T1 signal involving the periventricular rim. • Increased T2 signal int the involved white matter and the basal ganglia • On post-contrast imaging there is intense enhancement of the basal ganglia

Ddx: • Canavan disease: Diffuse involvement of the brain, with the subcortical U fibers involved early • Megaloencephalic leukoencephalopathy with subcortical cysts: Diffuse white matter involvment (including subcortical U fibers), but spares the basal ganglia • Mucopolysaccharidoses: Prominent Virchow Robin spaces, and patchy, periventricular white matter signal abnormality • Alexander's Disease

Dxhow: Imaging and Autopsy

Exam: Head circumference greater than 95%.

No MeSH data available.


High signal is present within the white matter of the frontal lobes and the basal ganglia
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MPX1613_synpic45596: High signal is present within the white matter of the frontal lobes and the basal ganglia


Alexander Disease, Rosenthal Fiber Encephalopathy

Smith ABS - MedPix (2008)

High signal is present within the white matter of the frontal lobes and the basal ganglia
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=MPX1613&req=5

MPX1613_synpic45596: High signal is present within the white matter of the frontal lobes and the basal ganglia

View Article: MedPix Image - MedPix Case

Affiliation: Uniformed Services University

ABSTRACT

Diagnosis: Alexander Disease, Rosenthal Fiber Encephalopathy

History: Six month old baby boy with intermittent vomiting for 7 weeks Now listless. Parents say his head is too big.

Findings: • Low T1 Signal involving the periventricular white matter and high T1 signal involving the periventricular rim. • Increased T2 signal int the involved white matter and the basal ganglia • On post-contrast imaging there is intense enhancement of the basal ganglia

Ddx: • Canavan disease: Diffuse involvement of the brain, with the subcortical U fibers involved early • Megaloencephalic leukoencephalopathy with subcortical cysts: Diffuse white matter involvment (including subcortical U fibers), but spares the basal ganglia • Mucopolysaccharidoses: Prominent Virchow Robin spaces, and patchy, periventricular white matter signal abnormality • Alexander's Disease

Dxhow: Imaging and Autopsy

Exam: Head circumference greater than 95%.

No MeSH data available.