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Acute necrotizing encephalopathy in Korean infants and children: imaging findings and diverse clinical outcome.

Kim JH, Kim IO, Lim MK, Park MS, Choi CG, Kim HW, Kim JE, Choi SJ, Koh YH, Yang DM, Choo SW, Chung MJ, Yoon HK, Goo HW, Lee M - Korean J Radiol (2004 Jul-Sep)

Bottom Line: On the follow-up images (n = 12), the brain lesions were reduced in extent for all patients, and generalized atrophy was seen in six patients.The significant prognostic factors identified in this study were the presence of hemorrhage (p = 0.009) and localized atrophy (p = 0.015).The presence of hemorrhage and localized tissue loss on MR images may suggest a poor prognosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Kangnam-gu, Seoul, Korea. jhkate.kim@samsung.com

ABSTRACT

Objective: The purpose of our study was to describe acute necrotizing encephalopathy in Korean infants and children, and we sought to evaluate the prognostic factors.

Materials and methods: Acute necrotizing encephalopathy was diagnosed in 14 Korean infants and children. We retrospectively analyzed the neuroimaging findings including the follow-up changes. The clinical course of the disease was graded, and we evaluated prognostic factors including age, serum level of the aminotransferase, hemorrhage, and localized atrophy of the brain.

Result: This encephalopathy predominantly affected the bilateral thalami (n = 14), pons (n = 12), and midbrain (n = 10) in a symmetrical pattern. Hemorrhage was observed in eight patients (57%). On the follow-up images (n = 12), the brain lesions were reduced in extent for all patients, and generalized atrophy was seen in six patients. Localized tissue loss was observed in five patients and a complete resolution occurred for one patient. All the patients survived and two recovered completely; mild (n = 6) to severe (n = 6) neurological deficits persisted in the remaining 12 patients. The significant prognostic factors identified in this study were the presence of hemorrhage (p = 0.009) and localized atrophy (p = 0.015).

Conclusion: Acute necrotizing encephalopathy in Korean patients showed the characteristic patterns of the post-infectious encephalopathy as described in the literature. The high survival rate and the relatively favorable clinical course observed for the present study suggest a more diverse spectrum of disease severity than was previously described. The presence of hemorrhage and localized tissue loss on MR images may suggest a poor prognosis.

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MRIs of a 10-month-old girl (patient 4) who was left with severe sequelae.A, B. Initial MR images obtained on the day following the hospital admission. T2-weighted axial images (A, B) show increased signal intensity in the thalami, the posterior internal capsule, putamen, periventricular white matter and the tegmen of the pons (arrows).C, D. Follow-up MR images obtained after one month of initial study. The T1-weighted image (C) shows shrunken thalami, localized low signal intensity that was similar to the cerebrospinal fluid in the periventricular white matter, and generalized atrophy of the cerebral hemispheres. The patches of bright signal intensity in the bilateral thalami (open arrows) suggest a subacute stage of hemorrhage. The localized bright signal intensity in the periventricular white matter and in the right thalamus on T2 weighted images (D) suggest the cystic evolution.
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Figure 1: MRIs of a 10-month-old girl (patient 4) who was left with severe sequelae.A, B. Initial MR images obtained on the day following the hospital admission. T2-weighted axial images (A, B) show increased signal intensity in the thalami, the posterior internal capsule, putamen, periventricular white matter and the tegmen of the pons (arrows).C, D. Follow-up MR images obtained after one month of initial study. The T1-weighted image (C) shows shrunken thalami, localized low signal intensity that was similar to the cerebrospinal fluid in the periventricular white matter, and generalized atrophy of the cerebral hemispheres. The patches of bright signal intensity in the bilateral thalami (open arrows) suggest a subacute stage of hemorrhage. The localized bright signal intensity in the periventricular white matter and in the right thalamus on T2 weighted images (D) suggest the cystic evolution.

Mentions: The neuroimaging findings are summarized in Table 3. The major involved sites were the thalami (n=14), pons (n=12), midbrain (n=10), and internal capsule (n=7) in a bilateral symmetrical pattern (Figs. 1, 2). The brainstem involvement was predominantly tegmental (Figs. 1B, 3B) in eight patients, and both the ventral and dorsal brainstems were involved for four patients. The temporal lobe (n=4), external capsule (n=4), cerebral deep white matter (n=3), cerebellum (n=2), putamen (n=2), frontal lobe (n=1), and caudate nuclei (n=1) were also involved in some patients. The observed lesions were initially edematous with T1 and T2 prolongation. There were hyperintense thalamic lesions noted on the T1-weighted images in eight patients on the initial images (n=5) or on the follow-up images (n=3, Fig. 1D); this suggested that there was a subacute stage of hemorrhage, which was also seen in the pons in one patient. These hemorrhagic lesions showed variable T2 signal intensity. Contrast enhancement was performed for eight patients and enhancement occurred in four of them, usually on the follow-up images (n=3).


Acute necrotizing encephalopathy in Korean infants and children: imaging findings and diverse clinical outcome.

Kim JH, Kim IO, Lim MK, Park MS, Choi CG, Kim HW, Kim JE, Choi SJ, Koh YH, Yang DM, Choo SW, Chung MJ, Yoon HK, Goo HW, Lee M - Korean J Radiol (2004 Jul-Sep)

MRIs of a 10-month-old girl (patient 4) who was left with severe sequelae.A, B. Initial MR images obtained on the day following the hospital admission. T2-weighted axial images (A, B) show increased signal intensity in the thalami, the posterior internal capsule, putamen, periventricular white matter and the tegmen of the pons (arrows).C, D. Follow-up MR images obtained after one month of initial study. The T1-weighted image (C) shows shrunken thalami, localized low signal intensity that was similar to the cerebrospinal fluid in the periventricular white matter, and generalized atrophy of the cerebral hemispheres. The patches of bright signal intensity in the bilateral thalami (open arrows) suggest a subacute stage of hemorrhage. The localized bright signal intensity in the periventricular white matter and in the right thalamus on T2 weighted images (D) suggest the cystic evolution.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2698159&req=5

Figure 1: MRIs of a 10-month-old girl (patient 4) who was left with severe sequelae.A, B. Initial MR images obtained on the day following the hospital admission. T2-weighted axial images (A, B) show increased signal intensity in the thalami, the posterior internal capsule, putamen, periventricular white matter and the tegmen of the pons (arrows).C, D. Follow-up MR images obtained after one month of initial study. The T1-weighted image (C) shows shrunken thalami, localized low signal intensity that was similar to the cerebrospinal fluid in the periventricular white matter, and generalized atrophy of the cerebral hemispheres. The patches of bright signal intensity in the bilateral thalami (open arrows) suggest a subacute stage of hemorrhage. The localized bright signal intensity in the periventricular white matter and in the right thalamus on T2 weighted images (D) suggest the cystic evolution.
Mentions: The neuroimaging findings are summarized in Table 3. The major involved sites were the thalami (n=14), pons (n=12), midbrain (n=10), and internal capsule (n=7) in a bilateral symmetrical pattern (Figs. 1, 2). The brainstem involvement was predominantly tegmental (Figs. 1B, 3B) in eight patients, and both the ventral and dorsal brainstems were involved for four patients. The temporal lobe (n=4), external capsule (n=4), cerebral deep white matter (n=3), cerebellum (n=2), putamen (n=2), frontal lobe (n=1), and caudate nuclei (n=1) were also involved in some patients. The observed lesions were initially edematous with T1 and T2 prolongation. There were hyperintense thalamic lesions noted on the T1-weighted images in eight patients on the initial images (n=5) or on the follow-up images (n=3, Fig. 1D); this suggested that there was a subacute stage of hemorrhage, which was also seen in the pons in one patient. These hemorrhagic lesions showed variable T2 signal intensity. Contrast enhancement was performed for eight patients and enhancement occurred in four of them, usually on the follow-up images (n=3).

Bottom Line: On the follow-up images (n = 12), the brain lesions were reduced in extent for all patients, and generalized atrophy was seen in six patients.The significant prognostic factors identified in this study were the presence of hemorrhage (p = 0.009) and localized atrophy (p = 0.015).The presence of hemorrhage and localized tissue loss on MR images may suggest a poor prognosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Kangnam-gu, Seoul, Korea. jhkate.kim@samsung.com

ABSTRACT

Objective: The purpose of our study was to describe acute necrotizing encephalopathy in Korean infants and children, and we sought to evaluate the prognostic factors.

Materials and methods: Acute necrotizing encephalopathy was diagnosed in 14 Korean infants and children. We retrospectively analyzed the neuroimaging findings including the follow-up changes. The clinical course of the disease was graded, and we evaluated prognostic factors including age, serum level of the aminotransferase, hemorrhage, and localized atrophy of the brain.

Result: This encephalopathy predominantly affected the bilateral thalami (n = 14), pons (n = 12), and midbrain (n = 10) in a symmetrical pattern. Hemorrhage was observed in eight patients (57%). On the follow-up images (n = 12), the brain lesions were reduced in extent for all patients, and generalized atrophy was seen in six patients. Localized tissue loss was observed in five patients and a complete resolution occurred for one patient. All the patients survived and two recovered completely; mild (n = 6) to severe (n = 6) neurological deficits persisted in the remaining 12 patients. The significant prognostic factors identified in this study were the presence of hemorrhage (p = 0.009) and localized atrophy (p = 0.015).

Conclusion: Acute necrotizing encephalopathy in Korean patients showed the characteristic patterns of the post-infectious encephalopathy as described in the literature. The high survival rate and the relatively favorable clinical course observed for the present study suggest a more diverse spectrum of disease severity than was previously described. The presence of hemorrhage and localized tissue loss on MR images may suggest a poor prognosis.

Show MeSH
Related in: MedlinePlus