感染后脑炎(附26例报告)

目的根据最新版国际疾病分类神经学分册(ICD-NA),感染后脑炎是一个独立的疾病,本文报告26例.方法采用美国国家和地区流行病学家委员会(CSTE)和疾病控制和预防中心(CDC)制定的统一标准,对我院近5年收治的符合CSTE和CDC诊断标准的感染后脑炎进行分析.结果该组感染后脑炎病例均有前驱感染史,以精神异常、意识障碍为主要临床表现,激素治疗有效.结论根据本文资料,感染后脑炎在我国作为独立疾病存在,约占脑炎病人的10%.     

Postvaccinial encephalomyelitis without cutaneous vaccination reaction

A 5-year-old girl developed a progressive febrile neurological illness consisting of right focal seizures, right hemiparesis, and stupor evolving over a period of six weeks. During the month preceding the onset of her illness she had received two apparently unsuccessful smallpox vaccinations without a skin lesion. Elevation of cerebrospinal fluid gamma globulin and findings on brain biopsy were consistent with postinfectious encephalitis, and a simultaneous increase in serum vacinia antibody titer suggested that the illness was postvaccinial encephalitis. Clinically, the child developed a severe extrapyramidal movement disorder during the acute phase followed by nine months of stuporous unresponsiveness, yet subsequently made a substantial recovery.     

Postinfectious immune-mediated encephalitis after pediatric herpes simplex encephalitis

We report a 3-year-old patient who presented a secondary acute neurological deterioration clinically characterized by a partial Kluver-Bucy syndrome, 1 month after the onset of herpes simplex encephalitis. This episode is unlikely due to continuation or resumption of cerebral viral replication but might be related to an immune-inflammatory process. In children, postinfectious immune-mediated encephalitis occurring after HSE are usually clinically characterized by choreoathetoid movements. This type of movement disorder was, however, not observed in this patient. On the basis of this case and a review of the literature, we hypothesize the existence of a spectrum of secondary immune-mediated process triggered by herpes simplex virus cerebral infection ranging from asymptomatic cases with diffuse white matter involvement to secondary acute neurological deteriorations with or without extrapyramidal features.     

Spinal fluid lymphocytes from a subgroup of multiple sclerosis patients respond to mycobacterial antigens

Thirty-one patients with large, focal cerebral demyelinating lesions are reported. Twenty-four patients had solitary lesions and 7 had multiple foci, the latter apparently of identical age. The lesions presented clinically and radiologically as brain tumors (gliomas or metastases) or as multiple cysts. Six patients were older than 57 years (2 in their 70s) at the onset of their symptoms. The demyelinating nature of the lesions was established through biopsy in each patient and all improved significantly after corticosteroid therapy. Three patients developed additional lesions during the follow-up periods ranging from 9 months to 12 years consistent with the course of multiple sclerosis. Twenty-eight patients did not develop additional lesions. These included 6 patients with multiple lesions at the onset. In 1 of the patients, the first symptoms developed 10 days after receiving vaccination against influenza. Two patients had concomitant malignancy (chronic monomyelogenous leukemia and retroperitoneal seminoma respectively) and 1 patient developed immunoblastic sarcoma in the opposite hemisphere after biopsy diagnosis and steroid treatment of her demyelinating lesion. Tumor-like masses of demyelination may occupy an intermediate position between multiple sclerosis and postinfectious/postvaccination encephalitis. The clinical course (history of vaccination in one instance, acute onset, good response to corticosteroids, no clinical or radiological evidence of new lesions in the great majority of patients) favored postinfectious/postvaccination encephalitis. Lesion size however greatly exceeded that of the small foci of perivenous demyelination seen in typical postinfectious/postvaccination encephalitis and tended to present as space-occupying masses.     

Pre- and post-encephalitic neuropsychological profile of a 7-year-old girl

Our understanding of the neuropsychological effects of encephalitis has largely come from studies in which the level of premorbid functioning is estimated. Moreover, data on the neuropsychological effects of encephalitogenic pathogens other than the herpes simplex virus (HSV) are scant. We present the case of a 7-year-old girl who had intellectual and language assessment seven months prior to the onset of non-HSV encephalitis (possible aetiology: Mycoplasma pneumoniae), and again post-encephalitis. MRI post-illness demonstrated basal frontal, temporal and (limited) parietal damage. Details of speech, psychological and neuropsychological assessments were also documented. Pervasive changes were apparent in the domains of personality, behaviour, emotionality, attention, executive function, speech, language and memory. The patient's profile appears to differ most from that seen following typical HSV encephalitis with respect to marked executive and attentional difficulties. Her deficits appear to reflect both her acute basal brain injury and more diffuse insult, probably caused by postinfectious encephalitis.     

Rhythmic involuntary movement of the neck in a patient with brainstem encephalitis]

A 36-year-old Japanese woman was admitted to our hospital, because of facial palsy, ophthalmoplegia, cerebellar ataxia, and rhythmic myoclonus of the neck. About a few weeks before admission, she developed symptoms of common cold and general fatigue. Her laboratory data were unremarkable, and her CSF was normal. Serum levels of antibodies to gangliosides were within normal limits. Her MRIs of the brain and neck were normal. Both somatosensory evoked cortical potential study and auditory evoked brainstem response study were normal. She was diagnosed as postinfectious brainstem encephalitis, and the administration of corticosteroid was started. After the treatment, her symptoms improved. To our knowledge, it is rare to see myoclonus to the neck without palatal tremor in patients with brainstem encephalitis.     

Encephalitis.

Encephalitis is an acute infection of brain parenchyma characterized clinically by fever, headache, and an altered level of consciousness. There may also be focal or multifocal neurologic deficits, and focal or generalized seizure activity. This article discusses the clinical presentation, diagnosis, and treatment of herpes simplex virus (HSV) encephalitis, the arthropodborne viral encephalidities, Rocky Mountain spotted fever, viral encephalitis in the immunocompromised patient, and postinfectious encephalomyelitis.     

Expanding the Spectrum of MERS Type 2 Lesions,a Particular Form of Encephalitis

We report on a 13-year-old boy who presented with signs suggestive of encephalitis and in whom magnetic resonance imaging revealed lesions in the genu and splenium of the corpus callosum and symmetrical lesions bilaterally in the center semiovale. This clinical-radiologic entity was previously reported in the literature and was given the acronym MERS type 2 (mild encephalitis with reversible splenial) lesion. The clinical, radiologic, and biochemical characteristics of the patient with MERS type 2 lesions presented in this article show some differences with those in previously reported patients. His clinical recovery was particularly slow, cerebrospinal fluid was abnormal, and on magnetic resonance imaging the typical time course of MERS type 2 lesions resolving through a phase of solitary lesions in the splenium of the corpus callosum, the so-called type 1 lesions, was not seen. He is also the first patient in whom mycoplasma pneumoniae was found to be associated with MERS type 2 lesions. These findings further expand the spectrum of MERS type 2 lesions. The question raises whether the MERS type 2 lesion represents a new type of encephalitis or a particular radiologically recognizable subtype of postinfectious encephalitis. In the article, previously reported patients with MERS type 2 lesions are reviewed.     

Measles Virus in the Brain

Measles virus can give three different forms of infections in the central nervous system. These are acute postinfectious encephalitis, acute progressive infectious encephalitis, and subacute sclerosing panencephalitis (SSPE). The postinfectious acute disease is interpreted to reflect an autoimmune reaction. The acute progressive form of brain disease, also referred to as inclusion body encephalitis, reflects a direct attack by the virus under conditions of yielding cell-mediated immunity. The late progressive form of encephalitis (SSPE) has been extensively analyzed. Recent molecular genetic studies have unravelled a range of mechanisms by which a defective expression of either the matrix, the fusion, or the hemagglutinin proteins may lead to viral persistence in brain cells under conditions not allowing identification by immune surveillance mechanisms. Many aspects of virus-cell interactions have been examined by use of explant cultures of neuronal cells of human and animal origin. Some of the findings are reviewed. Experimental animals, in particular rodents, have been used to establish systems in which phenomena, pivotal to the evolution of acute as well as persistent measles virus infections in the brain, can be studied. A wide range of potentially important mechanisms has been highlighted and is discussed. More recently, mice with genetic defects in immune functions were used to evaluate consequences as to initiation and dissemination of virus infection in the brain.     

Movement disorders in adult-onset measles encephalitis

There are three different neurological complications of measles infections in the brain: acute postinfectious encephalitis, acute progressive infectious encephalitis, and subacute sclerosing panencephalitis. The diagnosis of measles encephalitis (ME) is established when supported by the clinical picture, mainly of juvenile onset, and confirmed by the presence of cerebrospinal measles antibodies. Although ME is clinically characterized by progressive behavioral and mental deterioration associated with myoclonus, prior reports have suggested that adult-onset may have atypical features. We describe a 28 year-old immunocompetent man, admitted into the hospital due to a rapid motor and cognitive decline after an episode of fever and gastroenteritis. His neurological examination was significant for cognitive impairment, cervical dystonia, spontaneous and action induced myoclonus, choreiform movements, parkinsonism and ataxic gait. He was diagnosed of acute postinfectious ME based on the presence of elevated intrathecal synthesis of measles antibodies in his CSF, and a lymphocytic infiltrate of perivascular distribution without viral inclusions, with PCR negative for measles from brain biopsy. The patient continued to deteriorate to an akinetic mutism state, dying a few weeks later. Adult-onset ME is an entity rarely seen in the Western world. Although myoclonus is the most common movement disorder related to juvenile-onset ME, ataxia and other dyskinesias such as chorea, dystonia, and parkinsonism, can result from this infection when presenting in adult life.     

Postinfectious leukoencephalitis complicating Mycoplasma pneumoniae infection

Neuropathological findings in a patient with fatal neurological complications due to infection with Mycoplasma pneumoniae were similar to those seen in postinfectious encephalitis and acute hemorrhagic leukoencephalitis. This case supports the hypothesis that immune mechanisms play a role in the pathogenesis of neurological symptoms during mycoplasmal infections.     

Soluble IL-2 receptors in acute and subacute encephalitis

Elevated levels of soluble interleukin-2 receptors were present in the serum from patients with acute primary and postinfectious encephalitis and subacute sclerosing panencephalitis. In addition, soluble interleukin-2 receptors were detected in the cerebrospinal fluid from patients with acute primary encephalitis. Their presence in the cerebrospinal fluid was not explained by damage to the blood-brain barrier and our data attest to their local origin. This suggests that it may be possible in certain neurological diseases to detect cerebral T-lymphocyte activation through a specific marker in the cerebrospinal fluid.     

Postinfectious encephalomyelitis with localized basal ganglia involvement

The diagnosis of postinfectious encephalomyelitis with symmetric lesions in the basal ganglia was confirmed by magnetic resonance imaging in 2 patients. A 7-year-old patient experienced severe dystonia and hyperreflexia; magnetic resonance imaging demonstrated bilateral lesions in the putamina and basis pontes. The other patient, a 2-year-old female, manifested hypotonia, facial grimacing, and athetosis. Symmetric lesions in the globus pallidus and substantia nigra were demonstrated by imaging studies. The nature and monophasic course of illness in these 2 patients, as well as the symmetric involvement of specific regions of the basal ganglia, may result from an immune-mediated postinfectious demyelinating process.     

The spectrum of postinfectious encephalomyelitis

The medical records of 52 consecutive patients diagnosed with postinfectious encephalitis/encephalomyelitis during the period from 1980 to 1998, including 29 males and 23 females, were reviewed. These patients were divided into three groups according to their clinical and neurodiagnostic characteristics: (1) group I: postinfectious encephalitis, 38 patients; (2) group II: acute disseminated encephalomyelitis (ADEM), 13 patients; (3) group III: multiphasic disseminated encephalomyelitis (MDEM), one patient. Fever, headache/vomiting, seizure and disturbance of consciousness were common clinical features in all patients, while pictures of pyramidal, extrapyramidal, brainstem, and spinal cord lesions were more often found in the group II and group III patients than in the group I patients. Magnetic resonance imaging (MRI) revealed abnormal lesions in six (60%) of ten group I patients, but all group II (n=7) and group III (n=1) patients who received MRI study showed abnormal signals in various regions of the brain including the cerebral hemisphere, basal ganglia, brainstem and cerebellum. Patients with ADEM and MDEM had a longer clinical course and more neurological sequelae than group I patients. This study demonstrates the breadth of the clinical spectrum of postinfectious encephalomyelitis. Thorough clinical observations and appropriate neurodiagnostic studies such as MRI are crucial for the diagnosis.     

Long-term persistence of intrathecal viral antibody responses in postinfectious diseases of the central nervous system and in Rett syndrome

Twenty patients: seven with herpes simplex virus encephalitis (HSVE), six with other severe central nervous system (CNS) infections and 7 with Rett syndrome were studied to determine whether they showed any intrathecal synthesis of virus-specific or total IgG in CNS. The study of the postinfectious patients was made a mean of 20 years after the primary infection in childhood. Four of seven patients with HSVE had an elevated IgG index and four showed intrathecal viral antibody production which was both specific (against HSV) and non-specific. One patient with congenital syphilis and one with tuberculotic meningitis showed non-specific intrathecal viral antibody synthesis. In three of seven patients with Rett syndrome intrathecal antibody production was observed. The clarification of the mechanism of polyclonal immunoactivation in postinfectious diseases would be of interest since similar persistent immunoactivation is a common feature in multiple sclerosis. In Rett syndrome the immunoactivation may also have pathogenetic significance.     

Movement disorders in association with herpes simplex virus encephalitis in children: a review

Movement disorders have been reported in association with herpes simplex virus (HSV) encephalitis in adults and children. The cause of this complication is unclear, although a number of proposed aetiologies have been suggested, including inadequate antiviral therapy^1–3, a side effect of anticonvulsants⁴, or a possible postinfectious mechanism^5–8. A child who presented with severe chorea associated with relapse of HSV encephalitis prompted us to review the clinical features, management, and outcomes of this condition.     

Quantitative CSF PCR in Epstein-Barr virus infections of the central nervous system

Acute Epstein-Barr virus (EBV) infection of the central nervous system (CNS) is associated with meningoencephalitis and other neurological syndromes and with CNS lymphomas (CNSLs). Diagnosis is based on serological studies and more recently on detection of EBV DNA in cerebrospinal fluid (CSF) by polymerase chain reaction (PCR). We measured EBV DNA by quantitative PCR and EBV mRNA by RT-PCR in the CSF in patients with EBV-associated neurological disorders. EBV was identified as the cause of CNS infection in 28 patients: 14 with CNSL, 10 with encephalitis, and 4 with postinfectious neurological complications. CSF analysis showed that patients with CNSL had high EBV load (mean +/- standard error of 4.8 +/- 0.2 log(10) DNA copies/ml) and low leukocyte counts (22 +/- 7 cells/microl); encephalitis was characterized by high EBV load (4.2 +/- 0.3 log(10) DNA copies/ml) and high leukocyte counts (143 +/- 62 cells/microl); and patients with postinfectious complications showed low EBV load (3.0 +/- 0.2 log(10) DNA copies/ml) with high leukocyte counts (88 +/- 57 cells/microl). Lytic cycle EBV mRNA, a marker of viral replication, was identified in 10 CSF samples from patients with CNSL and encephalitis. These studies demonstrate the utility of quantitative CSF PCR and establish the presence of lytic cycle EBV mRNA in CSF of patients with EBV-associated neurological disease.     

Acute cerebellar swelling in varicella encephalitis

A 4-year-old male developed encephalitis 2 weeks after the onset of varicella. During his evaluation neuroradiologic procedures documented cerebellar edema and demyelination. Cerebrospinal fluid titers confirmed varicella encephalitis. To our knowledge, this patient is the first reported with focal cerebellar edema, an expected finding, with a clinical varicella infection as documented by computed tomography and magnetic resonance imaging.     

Relapsing Anti-NMDAR Encephalitis after a gap of eight years in a girl from North-East India

It has been just 7 years since the discovery of anti-NMDAR encephalitis as distinct immune-mediated encephalitis and we have such cases being reported from our country. Herein, we describe a case of a 13-year-old girl who had relapsing encephalitis consisting of multiple types of difficult-to-control seizures, abnormal behavior, language disintegration, memory loss and abnormal movements eight years after the first clinical attack. In 2005, when she was 5 yearsold, anti-NMDAR encephalitis was not yet discovered and she was provisionally diagnosed as a case of viral encephalitis. During her second attack in 2013, antibodies against NMDAR were demonstrated by immunofluoresence in serum (1:10). This is the first report from our country of a case of relapsing anti-NMDAR encephalitis of such a long duration, successfully treated by immunotherapy.     

Measles virus and associated central nervous system sequelae

Worldwide, measles remains one of the most deadly vaccine-preventable diseases. In the United States, enrollment in the public schools requires that each child receives 2 doses of measles-containing vaccine before entry, essentially eliminating this once endemic disease. Recent outbreaks of measles in the United States have been associated with importation of measles virus from other countries and subsequent transmission to intentionally undervaccinated children. The central nervous system complications of measles can occur within days or years of acute infection and are often severe. These include primary measles encephalitis, acute postinfectious measles encephalomyelitis, measles inclusion body encephalitis, and subacute sclerosing panencephalitis. These measles-associated central nervous system diseases differ in their pathogenesis and pathologic effects. However, all involve complex brain-virus-immune system interactions, and all can lead to severe and permanent brain injury. Despite better understanding of the clinical presentations and pathogenesis of these illnesses, effective treatments remain elusive.