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Reversible splenial lesion syndrome associated with encephalitis/encephalopathy presenting with great clinical heterogeneity.

Zhu Y, Zheng J, Zhang L, Zeng Z, Zhu M, Li X, Lou X, Wan H, Hong D - BMC Neurol (2016)

Bottom Line: The initial MRI features showed isolated ovoid or extending SCC lesions with homogeneous hyperintense on diffusion weighted imaging (DWI) and decreased apparent diffusion coefficient (ADC) values.Fractional anisotropy map revealed the decreased value of SCC lesion in a severe case with poor prognosis.Reversible signal changes on DWI and ADC are identified in all patients, but fractional anisotropy values can be decreased in severe patient with neurological sequelae.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, the First Affiliated Hospital of Nanchang University, Yong Wai Zheng Street 17#, Nanchang, 330006, P. R. China.

ABSTRACT

Background: Reversible splenial lesion syndrome (RESLES) is a disorder radiologically characterized by reversible lesion in the splenium of the corpus callosum (SCC). Most of patients with RESLES associated with encephalitis/encephalopathy were identified in Japanese population, but almost no Chinese patients were diagnosed as RESLES associated with encephalitis/encephalopathy.

Methods: Possible patients with reversible isolated SCC lesions were retrieved from January 2012 to July 2015 using keyword "restricted diffusion and isolated SCC lesion" in MRI report system from a large academic center. The clinical, laboratory and radiological data were summarized.

Results: A total of 15 encephalitis/encephalopathy patients (9 males and 6 females) were identified with a reversible isolated SCC lesion. Except for 13 patients with fever symptom, 8 patients also had cold symptoms before the onset of neurological symptoms. The neurological symptoms included headache, vertigo, seizure, disturbance of consciousness, and delirious behavior. Thirteen patients completely recovered within 1 month, but 2 patients who were subjected to mechanical ventilation had persistent neurological deficits. The initial MRI features showed isolated ovoid or extending SCC lesions with homogeneous hyperintense on diffusion weighted imaging (DWI) and decreased apparent diffusion coefficient (ADC) values. The follow-up MRI revealed that isolated SCC lesions with diffuse restriction disappeared at 10 to 32 days after the initial MRI study. Fractional anisotropy map revealed the decreased value of SCC lesion in a severe case with poor prognosis.

Conclusions: RESLES associated with encephalitis/encephalopathy is a reversible syndrome with an excellent prognosis in most patients, while a few patients required ventilator supporting at the early stage might have severe neurological sequelae. Reversible signal changes on DWI and ADC are identified in all patients, but fractional anisotropy values can be decreased in severe patient with neurological sequelae.

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The cerebral MRI features of case 7. MRI showed an isolated SCC lesion with slight hyperintense on T2WI (a), slight hypointense on T1WI (b), hyperintense on DWI (c, arrow), and obvious decreased ADC values (d). At 32 days after first MRI, second MRI showed abnormal signals of the SCC completely disappearing on T2WI (e), T1WI (f), DWI (g) and ADC (h)
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Fig2: The cerebral MRI features of case 7. MRI showed an isolated SCC lesion with slight hyperintense on T2WI (a), slight hypointense on T1WI (b), hyperintense on DWI (c, arrow), and obvious decreased ADC values (d). At 32 days after first MRI, second MRI showed abnormal signals of the SCC completely disappearing on T2WI (e), T1WI (f), DWI (g) and ADC (h)

Mentions: A 16-year-old boy with the complaints of fever and headache for 5 days, delirious behaviors for 3 days, and consciousness disturbance for 1 day was admitted to the hospital on February 12, 2014. The boy initially had fever, cough, and headache. After 3 days, the patient presented with severe headache, vomiting, and irritability, so antiviral drug (acyclovir) was administrated. After 4 days, he had consciousness disturbance, but no seizures were observed in the disease course. Physical examination on admission revealed comatose state (GCS: 8), slight limb movements responsive to pain stimulus, and negativity of bilateral pathological reflex. On admission, cerebral MRI showed an isolated lesion in the central SCC with slight hyperintense on T2WI (Fig. 2a), slight hypointense on T1WI (Fig. 2b), hyperintense on DWI (Fig. 2c), and apparent decreased ADC values (Fig. 2d). A lumbar puncture revealed a mild elevation of cell counting (15 cells/uL) with normal protein and glucose content. Oligoclonal bands were negative in serum and CSF. The quantity of MBP was 3.3ug/L in CSF. Intrathecal IgG synthesis rate was 0.61 mg/24 h. Laboratory examination revealed serum chemistry, thyroid function, serum ammonia, tumor biomarkers, ENA polypeptide spectrum, and ANCA were normal. At 1 day after admission, patients experienced more severe disturbance of consciousness, low blood pressure (70/50 mmHg), and breathing difficulty with hypoxemia and acidosis, so mechanical ventilation was administrated immediately. Meanwhile, the patient was administrated with methylprednisolone (1000 mg/d), IVIG (2 g/kg), antiviral (acyclovir), and antibiotics (vancomycin). At 10 days after admission, the patient had stable vital signs, while he was still in comatose state. At 32 days after admission, second MRI showed abnormal signals of the SCC completely disappearing on T2WI (Fig. 2e), T1WI (Fig. 2f), DWI (Fig. 2g) and ADC (Fig. 2h). After 7 months follow-up, the patient was in a condition of agrypnocoma, nasogastric tube feeding, and bedridden.Fig. 2


Reversible splenial lesion syndrome associated with encephalitis/encephalopathy presenting with great clinical heterogeneity.

Zhu Y, Zheng J, Zhang L, Zeng Z, Zhu M, Li X, Lou X, Wan H, Hong D - BMC Neurol (2016)

The cerebral MRI features of case 7. MRI showed an isolated SCC lesion with slight hyperintense on T2WI (a), slight hypointense on T1WI (b), hyperintense on DWI (c, arrow), and obvious decreased ADC values (d). At 32 days after first MRI, second MRI showed abnormal signals of the SCC completely disappearing on T2WI (e), T1WI (f), DWI (g) and ADC (h)
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4835842&req=5

Fig2: The cerebral MRI features of case 7. MRI showed an isolated SCC lesion with slight hyperintense on T2WI (a), slight hypointense on T1WI (b), hyperintense on DWI (c, arrow), and obvious decreased ADC values (d). At 32 days after first MRI, second MRI showed abnormal signals of the SCC completely disappearing on T2WI (e), T1WI (f), DWI (g) and ADC (h)
Mentions: A 16-year-old boy with the complaints of fever and headache for 5 days, delirious behaviors for 3 days, and consciousness disturbance for 1 day was admitted to the hospital on February 12, 2014. The boy initially had fever, cough, and headache. After 3 days, the patient presented with severe headache, vomiting, and irritability, so antiviral drug (acyclovir) was administrated. After 4 days, he had consciousness disturbance, but no seizures were observed in the disease course. Physical examination on admission revealed comatose state (GCS: 8), slight limb movements responsive to pain stimulus, and negativity of bilateral pathological reflex. On admission, cerebral MRI showed an isolated lesion in the central SCC with slight hyperintense on T2WI (Fig. 2a), slight hypointense on T1WI (Fig. 2b), hyperintense on DWI (Fig. 2c), and apparent decreased ADC values (Fig. 2d). A lumbar puncture revealed a mild elevation of cell counting (15 cells/uL) with normal protein and glucose content. Oligoclonal bands were negative in serum and CSF. The quantity of MBP was 3.3ug/L in CSF. Intrathecal IgG synthesis rate was 0.61 mg/24 h. Laboratory examination revealed serum chemistry, thyroid function, serum ammonia, tumor biomarkers, ENA polypeptide spectrum, and ANCA were normal. At 1 day after admission, patients experienced more severe disturbance of consciousness, low blood pressure (70/50 mmHg), and breathing difficulty with hypoxemia and acidosis, so mechanical ventilation was administrated immediately. Meanwhile, the patient was administrated with methylprednisolone (1000 mg/d), IVIG (2 g/kg), antiviral (acyclovir), and antibiotics (vancomycin). At 10 days after admission, the patient had stable vital signs, while he was still in comatose state. At 32 days after admission, second MRI showed abnormal signals of the SCC completely disappearing on T2WI (Fig. 2e), T1WI (Fig. 2f), DWI (Fig. 2g) and ADC (Fig. 2h). After 7 months follow-up, the patient was in a condition of agrypnocoma, nasogastric tube feeding, and bedridden.Fig. 2

Bottom Line: The initial MRI features showed isolated ovoid or extending SCC lesions with homogeneous hyperintense on diffusion weighted imaging (DWI) and decreased apparent diffusion coefficient (ADC) values.Fractional anisotropy map revealed the decreased value of SCC lesion in a severe case with poor prognosis.Reversible signal changes on DWI and ADC are identified in all patients, but fractional anisotropy values can be decreased in severe patient with neurological sequelae.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, the First Affiliated Hospital of Nanchang University, Yong Wai Zheng Street 17#, Nanchang, 330006, P. R. China.

ABSTRACT

Background: Reversible splenial lesion syndrome (RESLES) is a disorder radiologically characterized by reversible lesion in the splenium of the corpus callosum (SCC). Most of patients with RESLES associated with encephalitis/encephalopathy were identified in Japanese population, but almost no Chinese patients were diagnosed as RESLES associated with encephalitis/encephalopathy.

Methods: Possible patients with reversible isolated SCC lesions were retrieved from January 2012 to July 2015 using keyword "restricted diffusion and isolated SCC lesion" in MRI report system from a large academic center. The clinical, laboratory and radiological data were summarized.

Results: A total of 15 encephalitis/encephalopathy patients (9 males and 6 females) were identified with a reversible isolated SCC lesion. Except for 13 patients with fever symptom, 8 patients also had cold symptoms before the onset of neurological symptoms. The neurological symptoms included headache, vertigo, seizure, disturbance of consciousness, and delirious behavior. Thirteen patients completely recovered within 1 month, but 2 patients who were subjected to mechanical ventilation had persistent neurological deficits. The initial MRI features showed isolated ovoid or extending SCC lesions with homogeneous hyperintense on diffusion weighted imaging (DWI) and decreased apparent diffusion coefficient (ADC) values. The follow-up MRI revealed that isolated SCC lesions with diffuse restriction disappeared at 10 to 32 days after the initial MRI study. Fractional anisotropy map revealed the decreased value of SCC lesion in a severe case with poor prognosis.

Conclusions: RESLES associated with encephalitis/encephalopathy is a reversible syndrome with an excellent prognosis in most patients, while a few patients required ventilator supporting at the early stage might have severe neurological sequelae. Reversible signal changes on DWI and ADC are identified in all patients, but fractional anisotropy values can be decreased in severe patient with neurological sequelae.

Show MeSH
Related in: MedlinePlus