MRI findings in acute disseminated encephalomyelitis following varicella infection in an adult

There are no abnormal magnetic resonance imaging (MRI) findings in children with acute disseminated encephalomyelitis (ADEM) after varicella infection. Here, we report abnormal MRI findings in an adult, namely: hyperintense signal in the right medullary olive, the right side of the pons, the left middle cerebellar peduncle, the dorsal aspect of both thalami and the periventricular white matter of both cerebral hemispheres. The patient's condition improved after 1 week of corticosteroid treatment. Four months later, MRI of the brain showed almost complete resolution and there were no further attacks of motor weakness at 18-months follow-up.     

Acute disseminated encephalomyelitis in adults: a reappraisal of clinical, CSF, EEG, and MRI findings

Objectives To establish an adequate definition of acute disseminated encephalomyelitis (ADEM) in adults, based on our clinical observations of a case-series. Methods Over a period of three years 10 adult patients with a para- or postinfectious disseminated (diffuse or multifocal) syndrome of the CNS fulfilling predefined strict criteria for the diagnosis of ADEM were encountered and systematically followed. Results The age ranged from 21 to 62 years, two were men. MRI was normal in 5 patients and only mildly abnormal in the remaining patients. CSF was normal in 5 patients and mildly abnormal in the remainder, EEG was abnormal in 7/8 patients. All patients survived and were followed over a period of 30 months (range: 8 to 48 months). Nine patients were left with some residual defects, consisting most often of a mild cognitive impairment. Conclusions The EEG as an investigation of brain function can be crucial in establishing the organic nature of disease. MRI is important to exclude other diffuse or multifocal encephalopathies. However, in contrast to previous reports in the literature abnormal MRI should not be considered mandatory in adult ADEM. Difficulties in the diagnosis of ADEM are discussed and the importance of clinical and paraclinical findings for establishing the diagnosis is outlined. Received: 20 April 2001, Received in revised form: 19 July 2001, Accepted: 25 July 2001     

急性播散性脑脊髓炎的临床特点

目的探讨急性播散性脑脊髓炎（ADEM）的临床特点。方法对3例ADEM患者的临床表现、辅助检查、治疗及预后等进行回顾性分析。结果 3例ADEM中2例有前驱感染史,1例有手术史,首发症状分别为意识障碍、头晕和癫癇发作,头颅磁共振（MRI）见颅内多发长T1、长T2信号,呈环形或脑回样强化,累及双侧大脑、小脑和脑干,以双侧侧脑室旁和丘脑常见,经糖皮质激素治疗后病情迅速缓解。结论 ADEM是一种急性炎性脱髓鞘疾病,认识该病的临床及MRI特点可使患者得到及时诊治,改善预后。     

Fulminant acute disseminated encephalomyelitis in children

Acute disseminated encephalomyelitis (ADEM) is a typically monophasic inflammatory demyelinating disease of the central nervous system with a favorable outcome. However, 2% of ADEM involves acute hemorrhagic leukoencephalitis (AHLE), which is a fulminant and hyperacute variant of ADEM with a poor outcome and high mortality. There are limited case reports of fulminant ADEM including AHLE in children. Herein, we report two pediatric cases of fulminant ADEM. Both cases had a rapid deterioration of consciousness, repetitive seizures, and brain edema on neuroimaging, in addition to atypical neuroradiological findings on magnetic resonance imaging (MRI), a reversible splenial lesion in case 1, and bilateral frontal and occipital cortical lesions in case 2. Both cases were treated with early high-dose methyl-prednisolone and immunoglobulin, while therapeutic hypothermia was also initiated in case 2 after the patient exhibited a decerebrate posture and irregular breathing pattern. Both cases had a favorable outcome. Further case reports on pediatric fulminant ADEM are required to clarify the various clinical types, and to examine the efficacy of various treatment modalities for fulminant ADEM and AHLE in children.     

MRI and proton MR spectroscopy in acute disseminated encephalomyelitis

Introduction Acute disseminated encephalomyelitis (ADEM) is one of a group of demyelinating disorders of the central nervous system (CNS). It is said to be attributed to an overshooting immunologic response following an infection or vaccination. The clinical course and type of manifestation is heterogeneous. The early application of corticosteroids has been shown to be beneficial to outcome; thus, an early diagnosis is highly desirable.Methods The potential diagnostic value of advanced MR techniques such as proton MR spectroscopy and diffusion-weighted imaging (DWI) was investigated in two paediatric patients with ADEM, one of whom had a remitting and relapsing clinical course and presented with additional cranial nerve involvement. Proton MR spectroscopy revealed typical signs of acute demyelination, such as increased macromolecules, not found in other forms of non-necrotising pathology.Conclusion The addition of proton MR spectroscopy and DWI adds to the diagnostic power of MRI in the setting of post-infectious demyelinating disorders of the CNS or ADEM and may obviate the need for biopsy.     

急性播散性脑脊髓炎、多发性硬化及视神经脊髓炎脑深部灰质病灶MRI影像比较

目的 探讨并比较急性播散性脑脊髓炎(ADEM)、多发性硬化(MS)及视神经脊髓炎(NMO)脑深部灰质病灶的MRI影像学特征. 方法 自2004年8月至2012年10月在中山大学附属第三医院神经内科住院的ADEM、MS、NMO患者共353例,筛选出其中MRI显示有脑深部灰质病灶者95例(包括ADEM 12例,MS 60例,NMO 23例),对这些病灶的大小、数量、部位等特征进行分析. 结果 3组患者丘脑、尾状核、苍白球受累的病例数比例比较差异均无统计学意义(P=0.154,P=0.438,P=0.697).ADEM组壳核受累的病例数比例明显高于MS组、NMO组,差异有统计学意义(P=0.002,P=0.013).NMO组下丘脑受累的病例数比例则明显高于ADEM组、MS组,差异有统计学意义(P=0.033,P=0.001).ADEM组丘脑的病灶直径明显大于NMO组,差异有统计学意义(P=0.027),但和MS组相比差异无统计学意义(P=0.116),而MS组和NMO组丘脑的病灶直径比较差异亦无统计学意义(P=0.209).3组尾状核、壳核、苍白球、下丘脑的病灶直径比较差异均无统计学意义(P＞0.05).3组的病灶分布对称性比较差异无统计学意义(P=0.335). 结论 丘脑受累对于ADEM和MS的鉴别诊断可能意义不大,壳核受累可能是将ADEM区别于MS和NMO的一个鉴别点,下丘脑受累是NMO的特异性表现.病灶直径大小在这三种疾病的鉴别诊断中价值不大.     

Acute disseminated encephalomyelitis associated with hepatitis B virus reinfection – Consequence or coincidence?

Acute disseminated encephalomyelitis (ADEM) is an idiopathic inflammatory demyelinating disease of the CNS that is particularly difficult to differentiate from the first episode of multiple sclerosis. ADEM typically occurs as a post-infectious phenomenon, and usually presents a monophasic episode, but also includes recurrent and multiphasic forms. We report a case of ADEM associated with hepatitis B virus (HBV) reinfection. After steroid and IV immunoglobulin treatment, neurologic symptoms were improved. We suppose that the HBV reinfection was the cause of ADEM, but possible pathogenetic mechanism is still obscure.     

Acute disseminated encephalomyelitis following nephropathia epidemica

Acute disseminated encephalomyelitis (ADEM) is an acute monophasic inflammatory and demyelinating disease of the central nervous system (CNS) occurring days to weeks after a virus infection or vaccination. Nephropathia epidemica (NE) is a haemorrhagic fever with renal syndrome caused by Puumala virus, with endemic regions in Europe, especially Scandinavia and Western Russia. We describe a case of severe nephropathia epidemica requiring dialysis, followed by severe CNS symptoms caused by ADEM. To our best knowledge this is the first case in the literature in which NE caused ADEM.     

Post-infectious encephalitis with anti-galactocerebroside antibody subsequent to Mycoplasma pneumoniae infection

Galactocerebroside (Gc) is a major component of myelin in both the peripheral and central nervous systems. Although it is regarded as an important glycolipid hapten of myelin in rabbit experimental allergic neuritis (EAN), its role in human demyelinating diseases is not known. We studied three post-infectious encephalitis (PIE) patients related to Mycoplasma pneumoniae infection. All three of three patients with encephalitis and M. pneumoniae infection were positive for Gc antibodies (100%), while 25% of 32 M. pneumoniae-in-fected patients without neurological disease were positive, and 3.8% of 52 healthy controls. This indicates anti-Gc antibody is induced by M. pneumoniae infection. One of the PIE patients, who had extraordinary high titer antibody to Gc, showed an extensive, diffuse white matter demyelination and poor recovery. Since circulating anti-Gc antibody induces central nervous system demyelination in animals with elevated antibody titers and disruption of the blood-brain barrier, anti-Gc antibody may have an important function in the increased demyelination in PIE patients after M. pneumoniae infection.     

Chlamydia pneumoniae-associated ADEM.

Inflammatory demyelinating diseases are a common cause of neurologic disability in young adults, and usually the cause is unknown. We describe a case of acute disseminated encephalomyelitis (ADEM) associated with Chlamydia pneumoniae infection. An 18-year-old previously healthy women, with a one-week history of coryzal illness, was admitted because of progressive headache, dizziness, and a left-sided hemiparesis. MR imaging of the brain and brainstem showed typical signs of ADEM. The diagnosis was established by PCR Chlamydia pneumoniae DNA positivity in a tracheal swab and by increasing titres of Chlamydia IgM antibody. The patient was treated with doxycycline and steroids and recovered completely. Apart from therapeutic implications, this case may contribute to our understanding of demyelinating diseases of the central nervous system.     

Colloid cysts in children, a clinical and radiological study

Introduction Colloid cysts are uncommon in children. They occur more frequently in young adults.Methodology This is a retrospective study on all pediatric patients (≤18 years) with colloid cysts treated surgically in our institute between 1977 and 2005.Results A total of 43 patients with colloid cyst were treated surgically. Seven pediatric patients (16% of the total number of patients) were identified. Patients’ ages ranged from 6 to 18 years (mean of 14.1±4.67). They all presented clinically with symptoms and signs of increased intracranial pressure. Radiologically, the cyst size ranged from 10 to 25 mm (mean of 16.1±5.2). All patients had either diagnostic computed tomography (CT) or magnetic resonance imaging (MRI), or both. The colloid cysts appeared hyperdense on CT in five patients (83%) and isodense in one (six available studies). On MRI, all (five available studies) lesions were hyperintense on T2-weighted images. All seven patients had complete cyst resection. Either transcortical (five patients) or transcallosal approaches (two patients) was used in the surgical resection. There was no mortality. One patient developed a delayed cerebrospinal fluid leak related to hydrocephalus, and another patient developed a delayed postoperative seizure. The radiological findings supported the aggressive clinical scenario of these lesions in the pediatric group of patients.Conclusion In conclusion, colloid cysts can occur in children. They are associated with more aggressive clinical and radiological patterns. Early surgical intervention is highly recommended.     

儿童急性播散性脑脊髓炎21例临床分析

目的分析儿童急性播散性脑脊髓炎(acute disseminated encephalomyelitis,ADEM)的临床特点及辅助检查特点,以提高对此病的早期诊断及治疗。方法对2008-01—2013-05我院21例ADEM患儿的临床表现、辅助检查、治疗经过及预后进行回顾性分析。结果 21例患儿中有前驱感染病史者17例(80%),接种疫苗史2例。起病方式呈急性或亚急性起病,首发症状多样。MRI主要表现为白质受累。13例患儿脑脊液检查异常(62%),主要表现为细胞数及蛋白轻度升高。所有病例予以大剂量甲泼尼龙,12例合用大剂量免疫球蛋白,病情大多明显好转。结论儿童ADEM临床症状多样,结合MRI、脑脊液检查等多种辅助检查有助于及时明确诊断,患儿预后较好。     

Behavioral and Neuroanatomical Phenotypes in Mouse Models of Autism

In order to understand the consequences of the mutation on behavioral and biological phenotypes relevant to autism, mutations in many of the risk genes for autism spectrum disorder have been experimentally generated in mice. Here, we summarize behavioral outcomes and neuroanatomical abnormalities, with a focus on high-resolution magnetic resonance imaging of postmortem mouse brains. Results are described from multiple mouse models of autism spectrum disorder and comorbid syndromes, including the 15q11-13, 16p11.2, 22q11.2, Cntnap2, Engrailed2, Fragile X, Integrinβ3, MET, Neurexin1a, Neuroligin3, Reelin, Rett, Shank3, Slc6a4, tuberous sclerosis, and Williams syndrome models, and inbred strains with strong autism-relevant behavioral phenotypes, including BTBR and BALB. Concomitant behavioral and neuroanatomical abnormalities can strengthen the interpretation of results from a mouse model, and may elevate the usefulness of the model system for therapeutic discovery.

Electronic supplementary material

The online version of this article (doi:10.1007/s13311-015-0360-z) contains supplementary material, which is available to authorized users.     

Differential response to corticosteroid therapy of MRI findings and clinical manifestations in spinal cord sarcoidosis

Spinal cord sarcoidosis is a rare disorder whose natural history and therapeutic outcome are not fully known. We examined four patients with spinal cord sarcoidosis both clinically and radiologically, particularly in relation to corticosteroid treatment. The initial manifestation was cervical myelopathy in three and uveitis in one. All four patients progressed slowly until corticosteroid therapy was initiated. The cervial spine was involved in all patients. Magnetic resonance imaging (MRI) showed spinal cord swelling with T2-weighted high intensity and linear leptomeningeal and patchy or diffuse intramedullary enhancement with gadolinium diethylene triaminepentaacetic acid. With corticosteroid therapy, dramatic improvement was seen on MRI, including disappearance or marked reduction of swelling and enhancement. Plasma levels of angiotensin-converting enzyme (ACE) were also markedly improved. In contrast, the clinical symptoms were little improved in one patient, unchanged in two, and rather worsened in one patient. Recurrence was seen on MRI at the maintenance dose in all four patients, without any dramatic change in clinical manifestation. MRI findings and plasma ACE are well correlated with active leasion of the spinal cord sarcoidosis, providing a useful marker for recurrence, but do not parallel the clinical manifestations. Received: 14 December 1999, Received in revised form: 25 February 2000, Accepted: 12 March 2000