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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 2. MRI revealed an isolated SCC lesion with obvious hyperintense on DWI (a, arrow), decreased ADC values (b), decreased fractional anisotropy value (c), and normally projecting direction of white matter fibers (f). At 16 days after initial MRI, second MRI showed the abnormalities of SCC nearly disappeared on DWI (d) and ADC (e)
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Fig1: The cerebral MRI features of case 2. MRI revealed an isolated SCC lesion with obvious hyperintense on DWI (a, arrow), decreased ADC values (b), decreased fractional anisotropy value (c), and normally projecting direction of white matter fibers (f). At 16 days after initial MRI, second MRI showed the abnormalities of SCC nearly disappeared on DWI (d) and ADC (e)

Mentions: A 23-year-old man with the complaints of fever for 6 days, disturbance of consciousness for 4 h was admitted to the hospital on March 14, 2012. The patient initially presented with cold symptoms including fever, nasal congestion, and pharyngalgia 6 days ago. At the day of admission, he had temperature up to 39 °C, severe headache, and vomiting. He suddenly lost consciousness and experienced a seizure at that night. The symptom of seizure completely stopped after 2–3 min, but coma was persistent. Physical examination on admission revealed comatose state (Glasgow Coma Scale, GCS: 7), cyanosis, stiff neck, and no voluntary movements. The emergent therapy included mechanical ventilation, antiviral (acyclovir), and antibiotics (cefoperazone). At 1 day after admission, he can detach from the ventilator, but still was in comatose state. Cerebral MRI revealed an isolated lesion in the splenium of corpus callosum with slight hyperintense on T2 weighted images (T2WI), slight hypointense on T1 weighted images (T1WI), no enhancement on contrast images, obvious hyperintense on diffusion weighted imaging (DWI) (Fig. 1a), and decreased apparent diffusion coefficient (ADC) values (Fig. 1b). Fractional anisotropy map revealed mildly decreased value of SCC lesion (Fig. 1c), but with normally projecting direction of white matter fibers (Fig. 1f). At 4 days after admission, the patient experienced several seizures again, and became deep comatose (GCS: 5). The patient was administrated with carbamazepine (400 mg/d), methylprednisolone (1000 mg/d), and intravenous immunoglobulin (IVIG) (2 g/kg). The symptoms of seizure gradually disappeared, and the temperature returned to normal limits, while disturbance of consciousness was persistent. Laboratory examination revealed serum chemistry, blood routine, thyroid function, serum ammonia, tumor biomarkers, extractable nuclei antigen (ENA) polypeptide spectrum, and anti-neutrophil cytoplasmic antibody (ANCA) were normal. A lumbar puncture revealed a mild lymphocytic pleocytosis (20 cells/μL) with normal protein and glucose contents. Oligoclonal bands were negative in serum and CSF. The quantity of myelin basic protein (MBP) was 3.8ug/L (normal <4ug/L) in CSF. Intrathecal IgG synthesis rate was 0.55 mg/24 h (normal <0.7 mg/24 h). The etiological procedure found a positive PCR for influenza A in nasopharyngeal swabs, while other extensive microbiological workups were negative. At 17 days after admission, second MRI showed the abnormalities of SCC nearly disappeared on DWI (Fig. 1d) and ADC (Fig. 1e). Spinal cord MRI was normal. At 45 days, he was transferred back to local hospital for nursing care. After 1 year follow-up, the patient was in vegetative state, nasogastric tube feeding, and bedridden.Fig. 1


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 2. MRI revealed an isolated SCC lesion with obvious hyperintense on DWI (a, arrow), decreased ADC values (b), decreased fractional anisotropy value (c), and normally projecting direction of white matter fibers (f). At 16 days after initial MRI, second MRI showed the abnormalities of SCC nearly disappeared on DWI (d) and ADC (e)
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

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

Fig1: The cerebral MRI features of case 2. MRI revealed an isolated SCC lesion with obvious hyperintense on DWI (a, arrow), decreased ADC values (b), decreased fractional anisotropy value (c), and normally projecting direction of white matter fibers (f). At 16 days after initial MRI, second MRI showed the abnormalities of SCC nearly disappeared on DWI (d) and ADC (e)
Mentions: A 23-year-old man with the complaints of fever for 6 days, disturbance of consciousness for 4 h was admitted to the hospital on March 14, 2012. The patient initially presented with cold symptoms including fever, nasal congestion, and pharyngalgia 6 days ago. At the day of admission, he had temperature up to 39 °C, severe headache, and vomiting. He suddenly lost consciousness and experienced a seizure at that night. The symptom of seizure completely stopped after 2–3 min, but coma was persistent. Physical examination on admission revealed comatose state (Glasgow Coma Scale, GCS: 7), cyanosis, stiff neck, and no voluntary movements. The emergent therapy included mechanical ventilation, antiviral (acyclovir), and antibiotics (cefoperazone). At 1 day after admission, he can detach from the ventilator, but still was in comatose state. Cerebral MRI revealed an isolated lesion in the splenium of corpus callosum with slight hyperintense on T2 weighted images (T2WI), slight hypointense on T1 weighted images (T1WI), no enhancement on contrast images, obvious hyperintense on diffusion weighted imaging (DWI) (Fig. 1a), and decreased apparent diffusion coefficient (ADC) values (Fig. 1b). Fractional anisotropy map revealed mildly decreased value of SCC lesion (Fig. 1c), but with normally projecting direction of white matter fibers (Fig. 1f). At 4 days after admission, the patient experienced several seizures again, and became deep comatose (GCS: 5). The patient was administrated with carbamazepine (400 mg/d), methylprednisolone (1000 mg/d), and intravenous immunoglobulin (IVIG) (2 g/kg). The symptoms of seizure gradually disappeared, and the temperature returned to normal limits, while disturbance of consciousness was persistent. Laboratory examination revealed serum chemistry, blood routine, thyroid function, serum ammonia, tumor biomarkers, extractable nuclei antigen (ENA) polypeptide spectrum, and anti-neutrophil cytoplasmic antibody (ANCA) were normal. A lumbar puncture revealed a mild lymphocytic pleocytosis (20 cells/μL) with normal protein and glucose contents. Oligoclonal bands were negative in serum and CSF. The quantity of myelin basic protein (MBP) was 3.8ug/L (normal <4ug/L) in CSF. Intrathecal IgG synthesis rate was 0.55 mg/24 h (normal <0.7 mg/24 h). The etiological procedure found a positive PCR for influenza A in nasopharyngeal swabs, while other extensive microbiological workups were negative. At 17 days after admission, second MRI showed the abnormalities of SCC nearly disappeared on DWI (Fig. 1d) and ADC (Fig. 1e). Spinal cord MRI was normal. At 45 days, he was transferred back to local hospital for nursing care. After 1 year follow-up, the patient was in vegetative state, nasogastric tube feeding, and bedridden.Fig. 1

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