伴有胼胝体压部可逆性病灶的临床症状轻微的脑炎/脑病1例报道并文献复习

目的探讨伴有胼胝体压部可逆性病灶的临床症状轻微的脑炎/脑病的临床特点、治疗及预后、发病机制。方法报道1例伴有胼胝体压部可逆性病灶的临床症状轻微的脑炎患者的诊治过程,总结其临床特征并复习相关文献。结果患者表现为发热、头痛、呕吐等非特异性脑膜炎症状,合并出现抗利尿激素分泌不当导致的低钠血症,头颅MRI检查发现胼胝体压部有可逆性的DWI高信号的孤立病灶,经抗病毒及对症治疗,临床痊愈。结论伴有胼胝体压部可逆性病灶的临床症状轻微的脑炎/脑病以头颅MRI上发现胼胝体压部可逆性病灶为特点,临床症状轻微,容易合并低钠血症,预后良好,病因及发病机制尚不清楚。     

A case of clinically mild encephalitis with a reversible splenial lesion (MERS) after mumps vaccination

We describe for the first time an 8-year-old male patient who demonstrated clinically mild encephalitis with a reversible splenial lesion after mumps vaccination. He suffered from transient hallucinations, nuchal rigidity, and inappropriate antidiuretic hormone secretion syndrome. On the 5th day of admission, his head MRI showed symmetrical high-signal-intensity lesions on T2, FLAIR, and diffusion-weighted images in the splenium of the corpus callosum and in the periventricular white matter, while an apparent diffusion coefficient map showed reduced diffusion. The images were not enhanced by gadolinium. Follow-up MRI on the 16th day of admission revealed none of these abnormalities. His serum IgM and IgG antibodies against the mumps virus were positive according to an enzyme immunoassay. Mumps Torii vaccine strain was isolated from the patient’s cerebrospinal fluid. Previous reports demonstrated that transient delirious behavior, the syndrome of inappropriate antidiuretic hormone secretion, and good prognosis were the main clinical features of mild encephalitis with a reversible splenial lesion. This case shows that mild encephalitis with a reversible splenial lesion could occur after mumps vaccination.     

A splenial lesion with transiently reduced diffusion in clinically mild encephalitis is not always reversible: A case report

The MR imaging finding of a reversible splenial lesion with transiently reduced diffusion has been reported in patients with clinically mild encephalitis/encephalopathy, leading to a new clinical-radiological syndrome, clinically mild encephalitis/encephalopathy with a reversible splenial lesion. We recently experienced a 3-year-old boy with clinically mild encephalitis with a splenial lesion exhibiting transient reduced diffusion on admission. He recovered completely with no particular treatment within 2 weeks. Though the splenial lesion decreased in size, it was detected for over 5 months in T2-weighted imaging. It is suggested that a splenial lesion with transiently reduced diffusion in clinically mild encephalitis/encephalopathy is not always reversible, and could result in gliosis.     

成人伴胼胝体压部可逆性病变的轻度脑炎/脑病临床特点分析

目的探讨成人伴胼胝体压部可逆性病变的轻度脑炎/脑病(mild encephalitis/encephalopathy with a reversible splenial lesion,MERS)的病程特点、影像学表现及实验室生化结果,并对其进行临床分析。方法通过回顾性分析2013年1月-2015年12月就诊于中国医科大学附属盛京医院并临床确诊为MERS的成人患者7例,对其临床特点、影像学、脑脊液化验资料、治疗及预后进行分析。结果 7例患者中,4例男性,3例女性,均有发热表现,其他表现有意识障碍、头痛、呕吐、抽搐、视物双影。4例有明确前驱感染史,分别为病毒、细菌、支原体、结核,通过针对应用相应抗感染药物,14~60 d临床症状缓解。所有病变(包括胼胝体压部及脑白质病变)在T1WI为等、稍低或低信号,在T2WI及Flair成像为稍高或高信号,在DWI为高信号,ADC为低信号,增强扫描均没有强化效应,于发病后第14~45天复查头部MRI提示病灶完全消失。7例MERS患者CSF表现与原发病相关,无明显特征性改变。结论成人MERS是一组新型临床影像学综合征,其病理生理学机制尚不完全明确,表现多样,有特征性影像学表现,病程有自限性,针对不同病因予以针对性治疗,预后较好。     

Mild encephalitis/encephalopathy with reversible splenial lesion (MERS) due to dengue virus

A 14-year-old girl presented with encephalopathy, delirium and ophthalmoplegia following a 3 day history of high-grade fever. Brain MRI on day 6 of illness showed diffusion restricted ovoid lesion in the splenium of corpus callosum. Dengue virus encephalitis was diagnosed with positive PCR for dengue virus type-2 in both serum and cerebrospinal fluid. She made a complete recovery from day 10 of illness. Repeat brain MRI on day 12 of illness showed resolution of the splenial lesion. Serial diffusion tensor imaging (DTI) showed normal fractional anisotropy values on resolution of splenial lesion indicating that MERS was likely due to transient interstitial oedema with preservation of white matter tracts. This is the first reported case of MERS following dengue virus infection. It highlights the usefulness of performing serial DTI in understanding the underlying pathogenesis of MERS. Our case report widens the neurological manifestations associated with dengue infection and reiterates that patients with MERS should be managed supportively as the splenial white matter tracts are reversibly involved in MERS.     

Encephalopathy with a reversible splenial lesion is associated with hyponatremia

We have encountered several patients with clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) associated hyponatremia. In order to better understand this phenomenon, Na levels were evaluated in a series of patients with MERS. Na was 131.8+/-4.1 mmol/l (mean+/-SD, range 121-140) in 30 patients with MERS; 138.3+/-2.7 mmol/l (range 134-144) in age-matched 21 patients with upper respiratory infection; 136.6+/-2.5 mmol/l (range 132-140) in nine patients with other type of encephalopathy; and 136.2+/-2.6 mmol/l (range 132-140) in 17 patients with febrile seizures. Twenty-five of the thirty patients with MERS had Na<136 mmol/l. There were significant differences between the Na levels of patients with MERS and those with other groups. It is not possible, from the clinical perspective, to completely separate MERS from hyponatremic encephalopathy or to rule out hyponatremia as a contributing factor of MERS.     

Oxidative stress in patients with clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS)

We examined oxidative stress markers, tau protein and cytokines in the cerebrospinal fluid (CSF) in six patients with clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS). In the CSF, 8-hydroxy-2'-deoxyguanosine (8-OHdG) and hexanoyl-lysine adduct levels increased over the cutoff index in four and one out of six MERS patients, respectively. The CSF IL-6 and IL-10 levels were increased in three out of six patients, two of which had extended lesion of the cerebral white matter. The CSF value of tau protein, marker of the axonal damage, was not increased, and neuron specific enolase (NSE) in the CSF was not increased. The increased 8-OHdG levels in the CSF, DNA oxidative stress marker, in four MERS patients, suggesting involvement of oxidative stress in MERS. MERS is occasionally accompanied with hyponatremia, although our patients lacked hyponatremia. It is possible that the disequilibrium of systemic metabolism including electrolytes may lead to facilitation of oxidative stress and reversible white matter lesion in MERS. The increase of cytokine production seems to be involved in the distribution of lesions in MERS.     

The first pediatric case of mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) associated with Neisseria meningitidis

BackgroundMild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is a clinic-radiological syndrome characterized by neurological deficiencies and reversible magnetic resonance imaging findings in the splenium of the corpus callosum. Usually, it has a good prognosis, and patients recover without any sequelae. A viral infection has caused most MERS cases, and bacterial agents have rarely been reported as a cause of MERS.Case reportA 5-month-old male was admitted to the hospital with fever, poor feeding, decreased activity and groaning. He had focal seizures and required mechanical ventilation. A lumbar puncture was performed, and nucleic acid amplification tests (NAATs) of cerebral spinal fluid was positive for non-typeable serogroup of Neisseria meningitidis. Cranial magnetic resonance imaging (MRI) performed on day 3 of admission showed T2 hyperintensity and diffusion restriction at the splenium of corpus callosum (SCC). Diffusion restriction in the occipital horn of the left ventricular compatible with empyema was also obvious. According to the findings on cranial MRI, this clinical course was diagnosed as MERS associated with meningitis. His fever resolved in one week, cefotaxime was discontinued after 14 days, and the patient completely recovered. A follow-up MRI performed after three weeks of admission showed complete resolution of the signal intensity changes in the SCC and the occipital horn of the left lateral ventricle.ConclusionTo the best of our knowledge, this is the first report of MERS associated with Neisseria meningitidis in children. Bacterial agents as a cause of MERS should be kept in mind, and we should avoid unnecessary treatment strategies due to the good prognosis of MERS in children.     

Comparison of mild encephalopathy with reversible splenial lesion with and without acute focal bacterial nephritis

ObjectiveClinically mild encephalitis/encephalopathy with a reversible lesion (MERS) is characterized by reversible lesions with transiently-reduced diffusion in the splenium of the corpus callosum on magnetic resonance imaging. Recently, cases of MERS with accompanying acute focal bacterial nephritis (AFBN) have been reported in children. This study aimed to clarify the clinical features of MERS with AFBN.MethodsA retrospective study of patients with MERS was conducted at Nagano Children’s Hospital, Japan, from April 2013 to March 2018. The clinical signs and laboratory findings of MERS patients with AFBN (AFBN group) and without AFBN (non-AFBN group) were measured and compared.ResultsOf 12 patients diagnosed as having MERS, 3 were also found to have AFBN. Seven of the 9 patients without AFBN were associated with infectious agents, including rotavirus and influenza viruses. No patient received steroids or intravenous immunoglobulin therapy, and none displayed neurological sequelae. Serum C-reactive protein (CRP) levels were significantly higher in the AFBN group than in the non-AFBN group (14.7 mg/dL versus 0.8 mg/dL, P = 0.009). AFBN group patients were also significantly older (97 months versus 27 months, P = 0.018) and experienced significantly less frequent seizures (33% versus 100%, P = 0.045). The mean duration of neurological symptoms was significantly longer in the AFBN group than in the non-AFBN group (4 days versus 1.7 days, P = 0.013).ConclusionsPediatric patients with AFBN often present with non-specific findings, such as fever and abdominal pain. Pediatricians should be aware of the possibility of AFBN in the clinical setting of MERS, particularly when the patient exhibits inexplicably high CRP.     

Isolated brainstem involvement in posterior reversible encephalopathy syndrome: a case report and review of the literature

Posterior reversible encephalopathy syndrome (PRES) is a clinical–radiological syndrome, reversible vasogenic edema predominantly within parieto-occipital regions. However, isolated brainstem involvement in PRES has been rarely reported, little is known about its clinical manifestations, radiological features and outcomes. We reported a case with PRES with only brainstem involvement and performed a systematic review of published cases. Twenty-four cases, together with our case, were included in the analysis. Mean age was 43?years and 63% were males. Hypertension (50%), nephropathy (25%) and chemotherapy (21%) were the major risk factors. All patients except two had acute hypertension and 50% of patients had renal dysfunction at onset. The most common symptoms were altered consciousness (46%) and headache (46%), Seizure was only presented in 21% of patients. All patients except two were treated with antihypertension. Most patients recovered to their neurological baseline. Clinicians should recognize this unique variant finding in PRES. which always affects males with severe hypertension, especially combined with renal dysfunction. Antihypertensive treatment is the most widely used therapy. Outcome is usually well.     

Surgery for intractable epilepsy after severe encephalopathy with reversible splenial lesion and new onset hippocampal lesion associated with parechovirus

We describe a case of severe encephalopathy with reversible splenial lesion associated with parechovirus, followed by intractable temporal lobe epilepsy (TLE), which was improved by epilepsy surgery. A 3-year-old girl was admitted because of fever, consciousness disturbance and generalized tonic clonic seizure. Her seizure lasted for four hours. Fluid-attenuated inversion recovery (FLAIR) showed a hyperintensity in the splenium of the corpus callosum. Electroencephalogram (EEG) demonstarated continuous diffuse epileptic activity represented by synchronous and rhythmic high-amplitude spikes and waves, which led to the diagnosis of status epilepticus. Her consciousness was improved with fosphenytoin, midazolam and methylprednisolone pulse after 3 days. Seven days later, FLAIR hyperintensity in the splenium of the corpus callosum was disappeared; however, a hyperintensity in the right hippocampus was detected. Since the stool examination was positive for parechovirus, her final diagnosis was reversible splenial lesion syndrome (RESLES) associated with parechovirus. At age 8, she experienced epigastric sensation and consciousness disturbance once a week. Based on the scalp EEG and radiological findings, she was diagnosed with intractable right TLE. We performed a right selective amygdalohippocampectomy and anterior temporal disconnection at 10 years of age. One year and 3 months after surgery, she was seizure free. To our knowledge, this is the first report of severe febrile epilepticus status.with RESLES associated with parechovirus, followed by intractable TLE, which was resolved by epilepsy surgery.     

Chikungunya infection presenting as mild encephalitis with a reversible lesion in the splenium: a case report

Chikungunya fever is an Aedes mosquito-transmitted infection caused by chikungunya virus, an RNA virus in the family Togaviridae. The disease is characteristically manifested as fever, arthralgia, and/or rash. Various neurological manifestations like meningoencephalitis, myelitis, and myeloneuropathy have been mentioned in various reports. We present a rare case of chikungunya fever presenting with mild encephalitis with a reversible lesion of the splenium (MERS), which showed complete clinical and radiological recovery.     

Sellar neuroblastoma mimicking a pituitary tumour: Case report and review of the literature

Neuroblastomas of the sellar region are exceedingly rare. Only 2 cases have previously been reported. Management of these tumours depends on the tumour's primary site, the patient's age and histopathological features. We are reporting the case of a 43-year-old woman who developed progressive bitemporal hemianopsia and visual loss, accompanied by amenorrhea and hyponatremia. Laboratory findings revealed a slightly elevated prolactin level. Cranial MR-imaging displayed an intrasellar and suprasellar lesion with a maximum diameter of 2.5 cm that was suspicious for a pituitary adenoma or tuberculum sellae meningioma. The tumour was approached via a pterional trepanation. Intraoperatively, the tumour was highly vascularized and adhesive to the optic chiasm, the floor of the third ventricle, the hypothalamus and the hypophyseal stalk. Postoperatively, vision improved and prolactin dropped to normal values, but hyponatremia persisted. Histopathological examination revealed a neuroblastoma with strong positivity for synaptophysin and chromogranin, MAP-2 protein and NeuN-antigen in the immunohistochemistry. No pituitary hormone receptors were expressed. The MIB-1 labelling index was positive in 5% of the cell nucleoli. In the further course, the patient underwent radiotherapy of the neuroaxis.A brief review of the literature is presented.     

Seizures in encephalitis: Predictors and outcome

BackgroundSeizures are common in encephalitis but there is paucity of comprehensive studies evaluating predictors of seizures.AimTo evaluate the frequency and predictors of seizures in encephalitis patients and its effect on outcome.MethodsIn a prospective hospital based study, the patients with encephalitis were evaluated clinically and presence of seizure, its type and duration were noted. Patients’ consciousness was assessed by Glasgow coma scale (GCS) score and neurological findings were recorded. Blood count, serum chemistry, electroencephalography (EEG), cranial MRI and CSF examination were done. The diagnosis of encephalitis was based on ELISA and PCR and grouped into herpes, Japanese, dengue and nonspecific encephalitis. Hospital mortality and 3-month outcome were noted.Results148 patients with encephalitis whose median age was 26 (range 1–75) years were included. Seizures occurred in 63 (42.6%) patients; 18 of whom had status epilepticus. Seizures were more common in herpes (75%) followed by Japanese (54%) encephalitis. The predictors of seizure in encephalitis were age, GCS score and cortical involvement on MRI. 61% children had seizures compared to 36.6% adults and 53.3% with cortical involvement on MRI had seizure compared to 14.3% without. Seizures were not related to mortality but associated with poor outcome.ConclusionIn encephalitis, seizures occur in 42.6% patients especially in children with low GCS score and having cortical involvement on MRI.     

Mild encephalitis/encephalopathy with a reversible splenial lesion associated with respiratory syncytial virus infection in infants

Journal of NeuroVirology - Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is a clinicoradiologic syndrome typically characterized by transient mild encephalitis or...     

Granulomatous amebic encephalitis: a review and report of a spontaneous case from Venezuela

Granulomatous amebic encephalitis (GAE), or meningoencephalitis due to Acanthamoeba spp. and leptomyxid ameba are uncommon CNS infections that generally occur in immunocompromised hosts. We describe a case of GAE caused by Balamuthia mandrillaris previously designated as a leptomyxid ameba, in an apparently healthy 14-year-old Venezuelan boy. This case was characterized by sudden onset of seizures, focal neurologic signs and by a prolonged clinical course (from November 1992 to March 1993). Neuroimaging studies showed cerebral hypodense lesions in cerebral hemispheres, brain stem and cerebellum. Microscopically, we found a chronic granulomatous inflammatory reaction with necrotizing angiitis, large numbers of amebic trophozoites and few cysts in perivascular spaces and within necrotic CNS tissue. The amebas were identified as B. mandrillaris based on their immunofluorescence reactivity with the anti-B. mandrillaris serum. So far, 30 cases of GAE due to B. mandrillaris have been recognized in humans, two in AIDS patients. No visceral involvement by free-living amebas or any other significant abnormality was observed. This patient developed spontaneous GAE, but it remains possible that an undiagnosed abnormality in cell-mediated immunity or a deficient humoral immune response may explain the susceptibility of this patient to this opportunistic infection.Supported in part by the Pathology Education and Research Foundation (PERF) of the Department of Pathology, University of Pittsburgh     

富亮氨酸胶质瘤失活1蛋白抗体阳性相关免疫脑炎1例临床分析并文献复习

目的 报道富亮氨酸胶质瘤失活1蛋白(LGI1)自身抗体阳性免疫脑炎1例,结合文献分析,以提高临床医师对该病的认识。方法 结合文献分析该LGI1抗体阳性相关免疫脑炎患者的临床资料,并分析疗效。结果 中年男性急性起病,以肢体麻木、意识丧失、精神症状、癫痫间发作、四肢不自主运动为主要临床表现并伴低钠血症。脑脊液细胞、生化、免疫均正常,肿瘤相关抗原未见异常,副肿瘤抗体阴性,影像学检查排除颅内肿瘤。脑电图检查示右侧额颞导多量阵发性慢波异常及棘慢综合波。双侧海马区冠状位FLAIR序列提示双侧高信号,血清及脑脊液LGI-1抗体均(++)。结论 LGI1自身抗体阳性免疫脑炎以颞叶癫痫间作、精神异常为主要临床表现,常伴有低钠血症,病灶累及颞叶内侧为主,海马冠状位FLAIR序列常为高信号,免疫调节治疗及大剂量激素效果显著。