Magnetic resonance imaging in spinocerebellar degenerative diseases (apropos of 8 cases)

The results of NMR imaging in 8 cases of spinocerebellar degenerative diseases (age 4 to 19 years) are presented. In Friedreich ataxia (5 cases), spinal atrophy was constant and often severe, and was associated with a moderate cerebellar and/or bulbar atrophy in 3 cases. In hereditary spastic paraplegia, the only finding was a mild spinal atrophy in 2 of the 3 cases.     

Magnetic resonance imaging of acute cerebellar ataxia: Report of a case with gadolinium enhancement and review of the literature

Abstract A 10-year-old girl exhibited severe cerebellar ataxia following acute enterocolitis, and was diagnosed as having acute cerebellar ataxia (ACA). MR1 of the brain in the acute stage revealed moderate swelling of the cerebellum and abnormal signal intensity enhanced with gadolinium in the cerebellar hemisphere. This is the first report of an ACA case with positive gadolinium enhancement. Cases of ACA with MRI abnormalities are reviewed and the clinical entity of ACA is discussed in association with autoimmune encephalitis,     

Post-infectious acute cerebellar ataxia in children

Acute cerebellar ataxia is a relatively common neurologic disorder among children. Our aim was to characterize the clinical picture, etiology, and prognosis of acute cerebellar ataxia. The medical records of all children with a diagnosis of acute cerebellar ataxia hospitalized in our center and Hasharon Medical Center from 1990 to 2001 were reviewed. The diagnosis of acute cerebellar ataxia was based on the following criteria: acute onset of ataxia with or without nystagmus; absence of known genetic predisposing factors, such as familial degenerative disorders; and absence of drug intoxication, bacterial meningitis, and metabolic disorders. Thirty-nine children were identified; 54% were male; mean age at presentation was 4.8 +/- 3.8 years. All patients were observed for at least 1 year. A prodromal febrile illness was noted in 74.4%: varicella, 31%; mumps, 20%; nonspecific viral infection, 15.4%; mycoplasma, 5%; Epstein Barr virus, 3%. Latency from the prodromal illness to the onset of ataxia was 8.8 +/- 7.4 days. The most common associated neurologic findings were nystagmus and dysmetria. Full gait recovery took less than 2 weeks on average, and the longest duration of neurologic signs was 24 days (mumps-related). Acute cerebellar ataxia in childhood is a self-limited disease. The recovery was faster than that reported in previous publications and was complete in all children without any neurologic sequelae. Imaging studies are needed only in atypical presentation or if there is no spontaneous improvement after 1 to 2 weeks.     

儿童抗谷氨酸脱羧酶65抗体相关自身免疫性脑炎二例并文献复习

目的探讨儿童抗谷氨酸脱羧酶65(GAD65)抗体相关自身免疫性脑炎的临床特点及预后。方法对2019年于首都医科大学附属北京儿童医院神经内科住院的2例抗GAD65抗体相关自身免疫性脑炎患儿的临床资料进行回顾性分析。并分别以“抗GAD65抗体”“脑炎”“癫痫”“共济失调”和“anti-GAD65 antibody”“encephalitis”“epilepsy”“cerebellar ataxia”为关键词,对中文期刊全文数据库、万方数据知识服务平台、生物医学文献数据库(Pubmed)建库至2020年1月的文献进行检索,选取临床资料完整的儿童病例,总结临床特点及预后。结果2例抗GAD65抗体相关自身免疫性脑炎的患儿均为女性,例1于4岁9月龄起病,以发热伴意识障碍为主要临床表现,头颅磁共振成像提示颅内弥漫性T2加权序列(T2WI)稍高信号,脑电图提示慢波。例2于6岁8月龄起病,以反复局灶性癫痫发作、记忆力减退、头痛为主要临床表现,头颅磁共振成像提示双侧海马T2WI高信号,脑电图提示累及颞区的异常放电。2例患儿均应用了甲泼尼龙及丙种球蛋白免疫治疗,短期症状均有所改善。分别随访6个月及1年,例1完全恢复,例2仍有局灶性癫痫。文献检索共收集6篇英文文献,报道了6例患儿,结合本组共8例。8例患儿中癫痫发作6例,记忆力下降4例,意识障碍、行为异常各3例,认知障碍、头痛各2例,自主神经功能障碍、共济失调、吞咽困难、失语各1例。急性期或亚急性期5例头颅磁共振成像异常,其中3例累及边缘系统,2例边缘系统外受累为主。慢性期3例出现海马萎缩或硬化。8例均加用免疫治疗,免疫治疗后所有患者均短期获得不同程度的改善。随访6个月至6年,3例边缘叶外脑炎预后良好,5例边缘叶脑炎预后欠佳,其中死亡1例,4例遗留局灶性癫痫。结论儿童抗GAD65抗体相关免疫性脑炎是一种罕见可治疗的疾病,包括边缘叶脑炎及边缘叶以外脑炎,以癫痫发作及记忆力下降为常见临床表现。早期诊断并积极免疫治疗可短期内改善症状。边缘叶脑炎慢性期易出现难治性癫痫,长期预后不佳。     

Two patients with acute rotavirus encephalitis associated with cerebellar signs and symptoms

Rotavirus, one of the major causes of severe gastroenteritis in children, occasionally causes central nervous system complications. Recently several patients with acute encephalitis/encephalopathy due to rotavirus associated with cerebellar signs and symptoms have been reported. The condition is characterized by disturbances of consciousness at onset and cerebellar signs and symptoms such as hypotonia, ataxia, dysmetria, and speech disorders, including mutism, slow speech, and dysarthria at convalescence. We report two patients (3-year-old girl, 2-year-old boy) who developed acute encephalitis due to rotavirus and showed cerebellar signs and symptoms. Both patients had characteristic history of consciousness disturbances subsequent to several days of diarrhea, vomiting and fever, and cerebellar symptoms such as hypotonia, ataxia, dysmetria, and speech disorders during the recovery period. Electroencephalography showed diffuse high-voltage delta wave activity in each patient. Brain magnetic resonance imaging showed cerebellar edema in the acute phase followed by cerebellar atrophy on follow-up images in both patients. In the first patient, diffusion-weighted images (DWI) revealed high signals at the left cerebellar peduncle region and apparent diffusion coefficient (ADC) maps showed decreased ADC values of the lesion in the acute phase. The first patient had dysmetria at 1-year follow-up. However, she had normal motor and cognitive functions and could lead her daily life without impairment. In the second patient, no further symptoms were apparent at 1-year follow-up. Acute encephalitis/encephalopathy due to rotavirus with cerebellar signs and symptoms might be diagnosed on DWI, by demonstrating decreased ADC values in acute phase.     

Acute infantile hemiplegia caused by cerebral ischemic infarction. Etiology, clinical features and investigations

In 19 children with acute infantile hemiplegia an ischemic cerebral infarct was found clinically and by serial computertomography. In 11 patients an angiography has been performed in addition. 9 of the children had chronic diseases which are known as predisposing factors for cerebrovascular disease (congenital heart disease in 7 and chronic renal failure with hypertension in 2). One child had a severe hypernatremic dehydration due to infantile diarrhea and in 1 child thrombosis of the internal carotid artery occurred 3 days after a perforating trauma of the soft palate. No obvious reason for the ischemic stroke could be evaluated in 8 children. The onset of symptoms was either acute or slowly progressive. An altered state of consciousness was present in 11 children. Hemiparesis was found in 18 patients (13 right, 5 left) accompanied by facial palsy in 12 and aphasia in 6. Seizures occurred in 6 patients. One patient with incomplete occlusion of a vertebral artery showed acute cerebellar ataxia. In children without predisposing factors the prevalence of girls was higher (2 : 6) and there was a history of a preceding acute febrile illness in 5 of 8 patients. Laboratory investigations showed polycythemia in 4 children with cyanotic heart disease and additional hypochromia in two. Blood sedimentation rate was increased in 6 out of 8 patients without a known predisposing factor. Cerebrospinal fluid (CSF) showed a slight increase of erythrocytes (36-88/cmm) in 4 children, in two others purulent CSF was obtained after the infarct had developed into a brain abscess. The etiology of ischemic stroke in childhood and the possibility of an inflammatory vascular disease are discussed.     

Episodic ataxia type 2 showing ictal hyperhidrosis with hypothermia and interictal chronic diarrhea due to a novel CACNA1A mutation

Autosomal dominant episodic ataxia type 2 (EA2) results from mutations of the CACNA1A gene. We describe EA2 with unusual features in a father and daughter with a novel CACNA1A mutation coding for Y248C. Both patients showed severe cerebellar atrophy in MRI and clinical signs of progressive spinocerebellar atrophy type 6. Most disabling were the very frequent episodes of ataxia with migraine (with aura in the father and without aura in the daughter) and nystagmus in our patients. Additionally, they suffered from ictal hyperhidrosis with acute hypothermia of the extremities. Lastly, the father presented with interictal chronic diarrhea not associated to a known primary gastrointestinal disorder. Both ictal hyperhidrosis and interictal diarrhea ameliorated upon acetazolamide intake, the typical treatment for EA2. The significance of these findings is discussed and the phenotype correlated to previously reported cases.     

The CSF-protein pattern in acute cerebellar ataxia of childhood and intracranial midline tumours

CSF-protein profiles of 25 children with acute cerebellar ataxia and of 39 children with intracranial midline tumours at diagnosis were examined by quantitative zone electrophoresis in agarose gel. The profiles were compared with those obtained from a control group of 86 cases, and those from 61 patients with aseptic meningitis and 40 children with bacterial meningitis. The data from the latter groups demonstrated the CSF-protein pattern of moderate or severe disturbance of the blood-CSF barrier (B-CSF-B), respectively. The children with acute cerebellar ataxia showed minor signs of a B-CSF-B impairment and no increase of -globulin. These findings point to a slight acute vascular lesion. CSF changes indicative of a moderate-to-severe dysfunction of the B-CSF-B occurred in the majority of the patients with cerebellar astrocytomas, pontine gliomas, tumours around the 3rd ventricle, and medulloblastomas. Therefore acute cerebellar ataxia can be differentiated from intracranial midline tumours in most cases by means of CSF-protein electrophoresis. A striking finding was that 12 out of 14 children with medulloblastomas revealed a marked increase of -globulin. Since in 5 of these cases oligoclonal -globulin could be detected, this finding means local immunoglobulin synthesis within the CNS. The marked increase of -globulin which almost exclusively occurred in association with medullo-blastomas allows their seperation from acute cerebellar ataxia and the other tumour groups. Quantitative agarose gel electrophoresis can be a complementary diagnostic test in children with acute ataxia and suspected of having a CNS infection, or in cases with a negative CT brain scan in which intracranial midline tumour is a likely possibility.     

Sequential MRI, SPECT and PET in respiratory syncytial virus encephalitis

We report on a 3-year-old girl with respiratory syncytial virus (RSV) encephalitis manifested by disturbance of consciousness, conjugate eye deviation, anuria, truncal ataxia and intention tremor. T2-weighted magnetic resonance imaging (MRI) showed hyperintense areas in the cerebellar cortex. No lesion was detected in the cerebral cortex, pons or spinal cord. The hyperintense areas in the cerebellar cortex diminished with recovery from the clinical manifestations and had resolved 2 months after onset. The MRI lesions in the cerebellum were considered to be due to oedema. SPECT and positron emission tomography (PET), performed 3 months after onset, disclosed areas of hypoperfusion and hypometabolism at the same sites. One year after onset, MRI showed mild atrophy of the cerebellum. Hypoperfusion on SPECT and hypometabolism on PET remained. Neuroimaging showed that ataxia and tremor in this case were the result of cerebellitis. The patient has no neurological deficit except for mild truncal ataxia. This patient is a rare example of RSV encephalitis. Received: 11 December 1997 Accepted: 3 July 1998     

Imaging considerations of central nervous system manifestations in pediatric patients with neurofibromatosis type 1

CT and MRI were used in a prospective study of the central nervous system (CNS) manifestations in 41 consecutive children with neurofibromatosis type 1 (NF-1). Gadolinium-DTPA was used in 15 patients. MRI was more effective than CT in delimiting the extension of the optic pathway glioma and in evaluating associated cerebral malformations. MRI visualized lesions generally undetected by CT, in the form of iso- or hyperintense foci with respect to the cerebral cortex in T2-weighted sequences. Well-delimited lesions of high signal intensity were observed in the globus pallidus (22 cases), the internal capsule (6 cases), corpus callosum (2 cases), anterior commissure (1 case) and semioval center (2 cases). Poorly defined hyper- or isointense areas were also observed affecting the cerebellar white matter (21 cases) and brain stem (17 cases). None of these lesions showed Gadolinium-DTPA enhancement, and were of no clinical significance. MRI has displaced CT in the initial diagnosis of patients with NF-1. Periodic annual MRI controls are only justified in patients with MRI changes to evaluate the progression or stabilization of the lesions.     

Acute encephalitis in Swiss children: aetiology and outcome.

Since published data on the course and prognosis of encephalitis in Central Europe is limited, we retrospectively evaluated 104 children with either acute strict sense encephalitis (n = 80) or acute cerebellar ataxia (n = 24) treated at the Department of Pediatrics, University of Bern, Switzerland, between 1980 and 1991. Of the 80 patients with strict sense encephalitis, four (5%) died acutely and 28 (36%) of 78 followed up had sequelae - eight patients with severe, six with moderate and 14 with mild sequelae. Young age and seizures were shown to correlate with poor outcome. Among the 24 patients with acute cerebellar ataxia, there was no fatal outcome and none developed severe residua, but six had mild and one had moderate sequelae. Initial cerebrospinal fluid white cell count was significantly higher in these children with sequelae compared with those without any sequelae after acute cerebellar ataxia.     

Myoclonic encephalopathy of infants or "dancing eyes syndrome". Report of 7 cases with long-term follow-up and review of the literature (cases with and without neuroblastoma).

We describe 7 children with myoclonic encephalopathy of infants (MEI). MEI is a clinical entity characterized by an acute or subacute onset of polymyoclonia, cerebellar ataxia and opsoclonus ("dancing eyes"). It occurs either spontaneously, following an infectiuos illness or in association with an occult neuroblastoma. It is likely that immunological factors play a role in the pathogenesis. Steroid therapy resulted in rapid dramatic improvement of the neurological symptoms in 4 cases. However, this initial response did not correlate with the eventual outcome. We reviewed the literature to compare 45 reported cases of MEI associated with a neuroblastoma with 48 children without such a tumor to identify possible differences in clinical presentation, response to steroid medication and long-term prognosis of the neurological syndrome. In this respect we found no differences. Impairment of motor, verbal or intellectual performance were reported in at least half the cases. Although an immediate and marked response to steroids occurs in many cases of both groups, it remains unclear whether the long-term outcome is favourably influenced by this medication. The two-year-survival rate (90%) in the neuroblastoma group and the percentage of mediastinal localisation of the tumor (49%) are much higher compared with neuroblastomas without MEI. The reasons for these remarkable differences are not known. Diagnostic, therapeutic and prognostic implications justify the separation of MEI from the more common and benign syndrome known as acute cerebellar ataxia of childhood.     

The use of muscle biopsy in the diagnosis of undefined ataxia with cerebellar atrophy in children

Childhood cerebellar ataxias, and particularly congenital ataxias, are heterogeneous disorders and several remain undefined. We performed a muscle biopsy in patients with congenital ataxia and children with later onset undefined ataxia having neuroimaging evidence of cerebellar atrophy. Significant reduced levels of Coenzyme Q10 (COQ10) were found in the skeletal muscle of 9 out of 34 patients that were consecutively screened. A mutation in the ADCK3/Coq8 gene (R347X) was identified in a female patient with ataxia, seizures and markedly reduced COQ10 levels. In a 2.5-years-old male patient with non syndromic congenital ataxia and autophagic vacuoles in the muscle biopsy we identified a homozygous nonsense mutation R111X mutation in SIL1 gene, leading to early diagnosis of Marinesco-Sjogren syndrome. We think that muscle biopsy is a valuable procedure to improve diagnostic assesement in children with congenital ataxia or other undefined forms of later onset childhood ataxia associated to cerebellar atrophy at MRI.     

儿童周期性呕吐附加症5例临床分析

目的 探讨儿童周期性呕吐附加症(CVS+)的临床特征和预后。方法 回顾性总结分析2011年10月至2014年2月在首都医科大学附属北京儿童医院神经内科和消化科诊断的5例CVS+患儿的临床特征、线粒体基因检测结果和预后。结果 5例CVS+病例,女4例,男1例。就诊年龄5.8~10.4岁;起病年龄2~7岁,病程2.4~6.7年。①2例体重<同年龄儿童P3,1例身高<同年龄儿童P3。②5例均以反复周期性呕吐为首发和主要症状,持续2~8 d,呕吐发作间期为1周至6个月;5例在起病时或起病后1~15个月合并其他神经系统异常,包括植物神经功能紊乱1例、焦虑和抑郁1例、癫和肌病3例、共济失调2例。5例均有母系家族病史,包括偏头痛4例、眼震3例、耳聋2例、运动不耐受3例。③2例呕吐期血气分析示代谢性酸中毒,呕吐缓解后恢复正常;2例呕吐期血乳酸增高,伴有代谢性酸中毒,呕吐间期持续存在; 4例呕吐期伴低钾血症、低钠血症或低氯血症,呕吐缓解后恢复正常;5例头颅MR平扫均显示脑萎缩,双侧基底节对称短T1信号1例,双侧基底节对称长T2信号1例,左侧枕叶长T2异常信号1例;4例EEG可见发作间期样放电。④4例行线粒体基因全测序,2例3243A>G热点突变,突变比例为65.6%和34.0%,考虑线粒体脑病伴乳酸中毒及卒中样发作;2例为16519T>C多态性。⑤5例呕吐发作期予对症治疗。3例予抗癫药物;4例随访12~21个月,2例呕吐缓解不明显,2例呕吐缓解6个月,3例辍学。结论 反复呕吐并伴其他神经系统疾病的患儿应注意CVS+的可能,可能是线粒体病的一种特殊临床表现。     

Non-progressive congenital ataxia with cerebellar hypoplasia in three families

AIM: Non-progressive ataxias with cerebellar hypoplasia are a rarely seen heterogeneous group of hereditary cerebellar ataxias. METHOD: Three sib pairs from three different families with this entity have been reviewed, and differential diagnosis has been discussed. RESULTS: In two of the families, the parents were consanguineous. Walking was delayed in all the children. Truncal and extremity ataxia were then noticed. Ataxia was severe in one child, moderate in two children, and mild in the remaining three. Neurological examination revealed horizontal, horizonto-rotatory and/or vertical nystagmus, variable degrees of mental retardation, and pyramidal signs besides truncal and extremity ataxia. In all the cases, cerebellar hemisphere and vermis hypoplasia were detected in MRI. During the follow-up period, a gradual clinical improvement was achieved in all the children. CONCLUSION: Inheritance should be considered as autosomal recessive in some of the non-progressive ataxic syndromes. Congenital non-progressive ataxias are still being investigated due to the rarity of large pedigrees for genetic studies. If further information on the aetiopathogenesis and clinical progression of childhood ataxias associated with cerebellar hypoplasia is to be acquired, a combined evaluation of metabolic screening, long-term follow-up and radiological analyses is essential.     

眼球阵挛-肌阵挛综合征的临床诊治分析

目的 总结眼球阵挛.肌阵挛综合征(OMS)的临床特征及治疗经验,以提高诊疗水平.方法 对北京大学第一医院儿科2006至2007年收治的6例OMS患儿的临床、实验室资料及治疗情况进行分析.结果 6例中男1例,女5例,发病年龄12～26个月(平均21个月),其中4例病前有前驱感染史.就诊时主要症状为眼球阵挛、肌阵挛、共济失调、睡眠障碍、行为改变(6例).1例尿检3-甲氧基-4-羟基-苦杏仁酸(VMA)阳性,4例患儿腹部B超提示肝脏轻度增大,3例脑电图背景活动减慢,所有患儿均未见癎样放电.头颅MRI检查1例示内侧纵束、顶盖脊髓束T2高信号,1例腹部CT示L3-4蛛网膜囊肿,余患儿胸腹部CT未见异常.6例均给予促肾上腺皮质激素(ACTH)治疗,急性期有效.2例在随访过程中复发.结论 OMS是一种罕见的神经系统疾病,临床表现为眼球阵挛,并伴肌阵挛、共济失调、睡眠障碍和行为改变,容易复发且神经系统后遗症明显.ACTH治疗有效.     

Cerebellar ataxia in pediatric patients with Langerhans cell histiocytosis

The pathogenetic mechanisms of the central nervous system (CNS) problems associated with Langerhans cell histiocytosis (LCH) are not well established. Effective treatment strategies for these CNS complications are not yet available, while diabetes insipidus, also associated with LCH, can be managed effectively. Three Japanese boys with LCH who developed cerebellar ataxia were evaluated. Similar pediatric cases from the literature are also discussed. All three patients initially developed multifocal LCH lesions during early childhood (age <3 years) that responded well to chemotherapy; however, two of the three patients later developed diabetes insipidus. Ataxia, associated with mild developmental delay, was noted in the patients between the ages of 4 to 8 years. Analysis of these three cases, along with previously reported cases, indicates that the median age of onset of LCH was 2.5 (range 0.1-6.5) years and the median age of onset of cerebellar lesions/ataxia was 7 (range 3.5-16.5) years. Although the incidence of cerebellar LCH involvement is low, delayed onset of CNS disease must be monitored during follow-up care of pediatric LCH patients. Brain magnetic resonance imaging is strongly recommended for early detection of cerebellar lesions, but it remains to be determined whether there are any therapeutic measures to prevent exacerbation of CNS disease.     

髓鞘少突胶质细胞糖蛋白抗体阳性的儿童中枢神经系统脱髓鞘疾病临床特点

目的探讨髓鞘少突胶质细胞糖蛋白(MOG)抗体阳性的儿童中枢神经系统(CNS)脱髓鞘疾病的临床特点及疗效。方法回顾性分析2017年3月至2019年2月在浙江大学医学院附属儿童医院神经内科住院的CNS脱髓鞘疾病患儿115例,其中MOG抗体阳性的CNS脱髓鞘疾病患儿28例,总结28例患儿临床特点。结果28例患儿中男10例,女18例,男女比例为1.00∶1.80;起病中位年龄7岁9个月。临床症状表现多样,主要包括头痛、呕吐、嗜睡等脑病症状(13/28例),视力下降(7/28例),脊髓症状(6/28例),共济失调、口齿不清等小脑症状(4/28例),抽搐(2/28例),颅神经症状(1/28例)。24例行脑脊液检查,其中10例(41.7%)白细胞数轻度升高,2例(8.3%)蛋白升高,6例(25.0%)MOG抗体阳性,24例脑脊液寡克隆区带均阴性。25例头颅磁共振成像(MRI)阳性,阳性率为89.3%,常见累及部位包括大脑白质(20/28例)、小脑(10/28例)、大脑半球灰质(9/28例)、丘脑/基底核区(6/28例)、脑干(6/28例)、视神经(5/28例)和胼胝体(4/28例)。28例患儿中,13例脊髓受累,累及颈髓10例,胸髓9例,腰髓5例,8例患儿脊髓累及≥3个节段的长节段脊髓病变。14例患儿进行视觉诱发电位检查,2例临床无视觉障碍表现的患儿存在亚临床视觉损害。患儿均接受大剂量甲泼尼龙冲击治疗,16例联合丙种球蛋白免疫调节治疗,急性期均临床症状缓解,随访中7例患儿病情复发,复发率为25.0%,复发患儿再次使用甲泼尼龙冲击治疗联合丙种球蛋白免疫调节治疗,临床症状可缓解。结论MOG抗体阳性的儿童CNS脱髓鞘疾病临床表型以急性播散性脑脊髓炎为主,脊髓以累及颈胸段为主。其治疗以糖皮质激素和丙种球蛋白为主,疗效显著,但易复发,复发后再次使用糖皮质激素和丙种球蛋白,疗效仍较好。     

重度高胆红素血症新生儿苍白球磁共振成像特征及其临床意义

目的探讨苍白球MRI信号改变与高胆红素血症的严重程度及其相关因素关系,为胆红素脑病诊断与预后判定提供客观依据。方法36例高胆红素血症新生儿（TSB〉342μmoL／L）在生后[10±6（2～34）]d接受头部MRI检查。场强1．5～3．0Tesla,扫描序列为T1WI,T2WI和DWI。2名不知被检者病史的放射科医师分析MRI结果。结果首次MRI有20例苍白球T1WI呈对称性高信号。有苍白球信号改变组的TSB、B／A及UCB均显著高于无改变组[（605．28±89．19）μmoL／L vs．（438．19±67．89）μmoL／L,（1．08±0．18）vs．（0．77±0．16）,（555．49±92．3）μmoL／L vs．（412．01±54．8）μmoL／L,P=0．000],所有MRI-DWI均未见信号改变;TSB在342．0～427．5μmoL／L者9例,未见苍白球信号改变,427．5～513．0μmoL／L者7例,有改变者3例,超过525．0μmoL／L 20例,有改变者17例,黄疸程度与苍白球信号改变有密切关系（χ^2=15．000,P=0．000）;15例ABE苍白球T1WI均呈对称性的高信号（χ^2=17．601,P=0．000）,同时3例T2WI苍白球也呈对称性稍高信号（TSB分别为,745．3μmoL／L,735．7μmoL／L,707．6μmol/L）。7日内入院的25例中,16例苍白球有改变,平均入院时间显著晚于9例无改变者[（121．5±39．9）h vs．（68．9±35）h,P〈0．03]。6例接受了第2次MRI,其中3例ABE有2例苍白球信号转为T2WI高信号,临床均表现脑瘫,另1例苍白球信号正常,但有听力异常;余3例非ABE患儿,2例苍白球信号转为正常,1例两次均无苍白球信号异常,目前发育正常。结论MRI T1WI苍白球对称性高信号,与高胆红素血症的严重程度及暴露时间密切关系,是新生儿ABE的重要表现特征。T1WI高信号转变为T2WI高信号可能提示预后不良。     

Suppurated pneumococcal meningitis in infants and children: complications and prognostic factors

This paper investigates 21 patients (16 infants and 5 children) with Pneumococcal meningitis, 15 of which presented with a severe form. Intracranial pressure (IP) monitoring was performed in 7 patients who all had severe (4) or mild (3) intracranial hypertension (IH). Twelve children had a favourable outcome, 7 had sequelae (3 severe) and 2 died from coning, one before monitoring, the second after exhibiting the highest IP and the lowest cerebral perfusion pressure of the series. The 3 children with severe sequelae had a severe form (2) or a mild but prolonged form (1) of IH. The 4 patients who recovered with moderate sequelae presented severe (1), mild IH (1) or were not monitored (2). There were 14 cases with neurological complications. In 8 patients, this seemed to be related to cerebral oedema and IH; coning caused the death of 2 of them; the 6 others exhibited lesions of cerebral oedema on CT-scan, isolated or associated with cerebral infarction or subdural effusion. In the 6 others patients, IP monitoring was not performed; there was no evidence of cerebral oedema on CT-scan; 2 exhibited cerebral infarction and 3 a moderate ventricular dilatation; 2 had hemodynamic problems from acute pneumococcemia in one case and neurovegetative disturbances associated with cerebral infarction in the second. Pneumococcus meningitis remains a severe disease. The prognosis of severe forms can be improved by IP monitoring since IH seems to be the most frequent mechanism of complication.