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Deep gray matter involvement in children with acute disseminated encephalomyelitis
P A Baum1, A J Barkovich, T K Koch
1Department of Radiology, University of California San Francisco.
Insights
Deep gray matter involvement is common in children with acute disseminated encephalomyelitis (ADEM). This finding on MRI does not rule out ADEM and may help differentiate it from multiple sclerosis.
Area of Science:
- Pediatric neurology
- Neuroradiology
- Neuroimmunology
Background:
- Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating disease.
- Understanding the neuroimaging characteristics of ADEM is crucial for diagnosis and differentiation from other neurological disorders.
Purpose of the Study:
- To determine the frequency, distribution, and extent of deep gray matter lesions in pediatric patients diagnosed with ADEM.
- To analyze MRI findings in the deep gray matter, white matter, and cerebral cortex in ADEM.
Main Methods:
- Retrospective review of MRI examinations from 10 pediatric patients diagnosed with ADEM.
- Recording locations of abnormal T2 signal in cerebral and cerebellar cortices, white matter, and deep gray matter nuclei.
- Comparing pre- and postcontrast images to assess lesion enhancement.
Main Results:
- Six of 10 patients showed T2 prolongation in deep gray matter structures, including caudate nuclei, globus pallidus, putamina, and thalami.
- White matter lesions were present in 9 of 10 patients, predominantly in subcortical regions, corona radiata, and centrum semiovale.
- Cortical involvement was seen in 4 patients; spinal cord lesions were identified in one patient with myelopathy.
Conclusions:
- Deep gray matter involvement is a frequent finding in pediatric ADEM.
- The presence of T2 prolongation in deep gray matter does not exclude an ADEM diagnosis.
- Thalamic involvement in ADEM may aid in distinguishing it from the initial presentation of multiple sclerosis.
Purpose:
To review the frequency, distribution, and extent of deep gray matter disease in children with acute disseminated encephalomyelitis.
Methods:
The MR examinations of 10 patients, who were discharged with the clinical diagnosis of acute disseminated encephalomyelitis between 1986 and 1992, were retrospectively reviewed. Locations of abnormal signal in the cerebral and cerebellar cortices, white matter, and deep gray matter nuclei were recorded. Precontrast and postcontrast images were compared, when available, to assess degree of enhancement (if any).
Results:
Six patients had foci of prolonged T2 relaxation in the deep gray matter, ranging in size from less than 1 cm to 4 cm. The caudate heads were involved in 4 patients, caudate body in 3, globus pallidus in 3, putamina in 3, and thalami in 4. In 1 patient, the thalami were involved nearly symmetrically, with mild mass effect. Asymmetric subcortical white matter involvement was present as well. Prolonged T2 relaxation was present within the cerebral cortex in 4 patients and was associated with subcortical white matter abnormality in 3 and more central white matter disease in 1. Nine of 10 patients demonstrated foci of T2 prolongation in white matter, most commonly involving the subcortical region, corona radiata, and centrum semiovale. Three patients also had periventricular foci. Of the 3 patients receiving gadolinium, one showed no enhancement. Two of the patients showed enhancement of some but not all lesions. One patient, who had normal brain MR findings and symptoms of myelopathy, underwent spine MR which demonstrated focal linear areas of T2 prolongation in the spinal cord at levels C-1 to C-2 and T-6.
Conclusion:
Involvement of deep gray matter was common in our small series. The finding of T2 prolongation in these structures does not preclude the diagnosis of acute disseminated encephalomyelitis in the proper clinical setting. Because thalamic involvement is reported to be rare in multiple sclerosis, it may prove useful in distinguishing between acute disseminated encephalomyelitis and the initial presentation of multiple sclerosis.