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Acute encephalopathy with bilateral thalamotegmental involvement in infants and children: imaging and pathology
A Yagishita1, I Nakano, T Ushioda
1Department of Neuroradiology, Tokyo Metropolitan Neurological Hospital, Japan.
Insights
Acute encephalopathy in infants and children can affect the thalami and brain stem, often leading to severe outcomes. Imaging reveals symmetric lesions, with T1 shortening in the thalami indicating petechiae.
Area of Science:
- Pediatric Neurology
- Neuroradiology
- Neuropathology
Background:
- Acute encephalopathy is a severe neurological condition affecting children.
- Bilateral thalamotegmental involvement is a specific pattern observed in some cases.
- Understanding imaging and pathological features is crucial for diagnosis and prognosis.
Purpose of the Study:
- To investigate the imaging and pathologic characteristics of acute encephalopathy with bilateral thalamotegmental involvement in infants and children.
- To correlate imaging findings with neuropathologic results.
- To elucidate the potential causes and outcomes of this condition.
Main Methods:
- Retrospective study of five Japanese children (11-29 months old).
- Utilized CT imaging, MRI examinations, and autopsy in one case.
- Analyzed lesion distribution, evolution, and specific MRI signal changes.
Main Results:
- Encephalopathy affected thalami, brain stem tegmenta, and white matter.
- Autopsy revealed necrosis and edema without inflammation, with thalamic petechiae and congestion.
- CT/MRI showed symmetric focal lesions, evolving over time.
- MR imaging demonstrated T1 shortening in the thalami, suggestive of petechiae.
- Prognosis was poor, with significant mortality and severe sequelae.
Conclusions:
- The described encephalopathy may represent a postviral or postinfectious brain disorder.
- Thalamic T1 shortening on MRI is a potential indicator of petechiae.
- Bilateral thalamotegmental involvement in pediatric acute encephalopathy carries a poor prognosis.
Purpose:
To investigate the imaging and pathologic characteristics of acute encephalopathy with bilateral thalamotegmental involvement in infants and children.
Methods:
Five Japanese children ranging in age from 11 to 29 months were studied. We performed CT imaging in all patients, 10 MR examinations in four patients, and an autopsy in one patient.
Results:
The encephalopathy affected the thalami, brain stem tegmenta, and cerebral and cerebellar white matter. The brain of the autopsied case showed fresh necrosis and brain edema without inflammatory cell infiltration. Petechiae and congestion were demonstrated mainly in the thalamus. CT and MR images showed symmetric focal lesions in the same areas in the early phase. These lesions became more demarcated and smaller in the intermediate phase. The ventricles and cortical sulci enlarged. MR images demonstrated T1 shortening in the thalami. The prognosis was generally poor; one patient died, three patients were left with severe sequelae, and only one patient improved.
Conclusions:
The encephalopathy might be a postviral or postinfectious brain disorder. T1 shortening in the thalami indicated the presence of petechiae.