Progressive myelopathy due to meningeal thickening in shunted patients: description of a novel entity and the role of surgery

INTRODUCTION: Spinal cord compression due to meningeal thickening is a rare occurrence in shunted patients. Because of the long delay to clinical onset, this complication has not been identified as yet. AIMS: We report on nine cases of shunt-related progressive myelopathy due to meningeal thickening (SPMMT). MATERIALS AND METHODS: We reviewed our database of shunted children, for cases having developed progressive tetraparesis due to cervical meningeal thickening. RESULTS: We identified nine observations of SPMMT, eight of these with hydrocephalus due to neonatal meningitis; the last case had Dandy-Walker malformation shunted at birth and suffered postoperative meningitis. The age of clinical onset of myelopathy was between 6 and 20 years (median 12.8). All patients presented with slowly progressive walking difficulties with falls and no spinal pain. Magnetic resonance imaging (MRI) showed typically a thickened dura mater with collapse of the arachnoid space, compensatory expansion of the epidural fat, and T2 hyperintensity in the spinal cord. We operated on seven patients for surgical decompression and arachnoidolysis: One died postoperatively because of shunt malfunction, and two others died later of complications of tetraplegia. Three patients were aggravated after surgery, three experienced partial improvement, but one of these subsequently deteriorated again. CONCLUSION: SPMMT appears to be a novel and well-defined clinical and pathological entity; its pathological and radiological features are stereotyped; however, the diagnosis is delayed because of the slow pace of the disease. Although surgical decompression may be the only option, its results were poor in our experience; earlier surgery might improve this grim prognosis.     

Lymphocyte subclasses and function in patients with optic neuritis in childhood with special reference to multiple sclerosis

Ten patients with childhood optic neuritis (5 with a single attack of ON and 5 with later MS) were studied at various stages of the disease. Lymphocyte count and function were analysed in the peripheral blood of all patients, 3 repeatedly, and in one they were also analysed in the CSF. T-lymphocytes counts were normal in all but 2 MS cases who had high counts. In acute stages the T4/T8 ratio were high in 1/3 determinations, in recovery low in 2/2 determinations, and in stable stages normal in 6/8 determinations. Lymphocyte function, measured by PHA, ConA and PWM stimulation, was normal in all but one. One patient showed significantly higher T-cell percentages and a high number of stimulated lymphocytes in CSF but a lower count of suppressor cells than in the blood. We found no abnormalities specific to MS nor to childhood MS or to disease activity stage. Rather than peripheral blood, it would seem more worthwhile to study CSF to clarify the pathogenesis of ON and MS.     

Neurotransmitters in cerebrospinal fluid reflect pathological activity

The excitatory transmitters glutamate and aspartate become toxic whenever their extracellular levels are increased because of neuronal, glial and endothelial impairment. Taurine, a volume-regulating amino acid, is released upon excitotoxin-induced cell swelling. Our aim was to investigate if glutamate and aspartate in cerebrospinal fluid (CSF) reveal neuropathology in neurological patients, and if taurine unmasks glutamate-mediated toxicity. Glutamate and aspartate are doubled in viral meningitis, acute multiple sclerosis (MS) and myelopathy compared with control subjects and patients with peripheral facial nerve palsy. These levels do not coincide with a disturbed blood–brain barrier, as estimated by the albumin ratio, are independent of their precursors (glutamine, asparagine) and are not associated with cell lysis. Taurine is significantly increased in meninigitis, acute MS, and myelopathy, suggesting glutamate-mediated toxicity. Analysis of transmitters in lumbar CSF can be used to identify patients with cerebral and spinal pathology who might benefit from specific receptor-modulating agents.     

脑型肌酸激酶同功酶在脑血管病中的意义 Ⅱ.急性脑血管病患者血清和脑脊液中CKBB变化及与CT片上病损大小的关系

用改良的ELISA检测25例正常对照组和32例急性脑血管病患者的血清和脑脊液(CSF)中的脑型肌酸激酶同功酶BB(creatine kinase isoenzyme BB)浓度。32例急性脑血管病患者CSF CKBB平均水平为16.62±8.3ng/ml,明显高于对照组(7.5—4.8ng/ml)。发病24h内CSFCKBB轻微增高,24～48h达高峰,以后下降,7天左右尚未恢复正常。病初CSF CKBB水平明显高于恢复期。9例高血压性脑出血的血肿出血量(按CT片上血肿大小计算)与患者CSF CKBB有密切关系(r=0.8127,P<0.01)。血肿体积(X)与CSF CKBB浓度(y)的回归方程y—7.945±0.872X。     

Relative risks of ventriculostomy infection and morbidity

Summary Ventricular catheter placement is a common procedure for the management of increased intracranial pressure. Hypotheses regarding the etiology of infection of catheters center on two alternative assumptions: 1) contamination leading to infection occurs at the time of catheter insertion, implying that catheter duration has minimal effect on infection risk; and 2) infection of catheters derives from catheter contamination after insertion, suggesting that duration of catheter use may significantly affect infection risk.We have studied the relative complication rate of ventricular catheter insertions using a retrospective approach (n=161 patients and 253 catheter insertion procedures). The overall infection rate was 4.1%, but the daily infection hazard increased exponentially with time, to a maximum daily rate of 10.3% by day 6 of catheter insertion. This increasing risk appears most consistent with the second hypothesis. The risk of non-infectious complications was 5.6%, including hemorrhagic occurrences and misplacement severe enough to require a new catheter insertion. The daily hazard of infection approximately equalled the non-infectious risk of routine catheter replacement by day 5.Additional prospective data on the daily risk of CSF infection and the appropriateness of antibiotic prophylaxis either at the time of ventricular catheter insertion or continued through the catheter's-presence may be required to both definitively identify which hypothesis of infection risk is correct and whether antibiotics can significantly ameliorate this risk.     

60例急性病毒性脑炎的临床分析

目的　评价急性病毒性脑炎的诊断与治疗。方法　收集 1993～ 2 0 0 1年 6月的住院病案 60份 ,就临床症状、神经体征、周围血白细胞计数和分类、脑脊液、脑电图、脑CT检查 ,以及单用激素组和激素 +抗病毒毒药组之间的疗效关系进行分析。结果　本组病毒性脑炎可分为六种临床类型 :精神异常 ;持续性剧烈头痛伴呕吐 ;强直 -阵挛性抽搐 ;持续性意识障碍 ;脑神经损害、失语、偏瘫 ;小脑性共济失调。前三型最为常见 ,并且三型之间的临床现象有相互穿插出现的现象。经统计学处理 ,单用激素组与激素 +抗病药物组之间的疗效无显著性差异。结论　急性病毒性脑炎为一种自限性疾病 ,但在没有病毒学检查的情况下 ,尚应和急性播散性脑脊髓炎或多发性硬化作鉴别     

Neurochemical Findings in the Cerebrospinal Fluid of Schizophrenic Patients with Tardive Dyskinesia and Neuroleptic-Induced Parkinsonism

Abstract: Monoamine and their acid metabolites were determined in the CSF of 18 drug-treated chronic schizophrenic patients with the symptoms of tardive dyskinesia and neuroleptic-induced Parkinsonism (Parkinsonism). Six healthy volunteers were used as the control group.
The norepinephrine (NE) levels were found to be significantly higher in the patients with tardive dyskinesia than in the controls. Furthermore, elevated CSF NE levels were also observed in the patients with Parkinsonism. Epinephrine (E) and Dopamine (DA) were not present in the CSF of the control group, whereas measurable levels of DA could be detected in 4 out of 9 and E was found in 8 out of 9 patients with tardive dyskinesia. The mean concentration of HVA was slightly but not significantly elevated in the patients with tardive dyskinesia and Parkinsonism. The mean values of CSF 5-HIAA were all within the normal range in both patient groups. From the above results, it was suggested that abnormal adrenergic activity rather than abnormal dopaminergic activity may play an important role as a mechanism in the etiopathogenesis of extrapyramidal disorders. Furthermore, in the patients with Parkinsonism, CSF neurochemical observations were similar to those of the patients with tardive dyskinesia in this study. It may help to explain the clinical coexistence of tardive dyskinesia and neuroleptic-induced Parkinsonism.     

“Occult” hydrocephalus in children

The authors describe 32 children between 2 and 15 years of age who had hydrocephalus that was only clinically manifest late in life. The clinical picture of these children did not suggest an obvious increase in intracranial pressure; instead, the presenting signs were rather nonspecific and included macrocrania, mild psychomotor retardation, unsteady gait, increased muscle tone and deep tendon reflexes in the lower limbs, impaired ocular movement, epilepsy, and endocrine dysfunction. Their histories suggest the possible causes of the ventricular dilation in about one third of the cases were: perinatal hemorrhage, leptomeningitis, neurofibromatosis, and untreated aneurysm of the great vein of Galen. In 20 patients, however, no positive anamnestic findings were reported. CT scan revealed triventricular dilation in more than half of the cases; tetraventricular dilation was present in 6 patients, and biventricular dilation in the remaining subjects. All children underwent CSF shunting, which resulted in complete recovery in all but 2 cases. The most frequently recorded surgical complication was postoperative subdural effusion (7 subjects), which required surgical treatment in only 2 cases.Presented at the 15th Annual Scientific Meeting of the International Society for Pediatric Neurosurgery, New York, 1987     

GM and KM allotypes in eight tribal populations of madyha Pradesh and Orissa, India

Summary Serum samples from eight endogamous Indian tribal populations of Madhya Pradesh (Dhurwa, Halba, Bhatra, Muria, Maria) and Orissa (Deshia Khond, Binjhal, Kisan) with a total of n=731 unrelated individuals were typed for G1M (1,2,3,17), G3M (5,10,11,13,14,15,16,21,26), and KM (1). In seven of these populations five different GM haplotypes were found:GM*1,17;21,26; GM*1,17;10,11,13,15,16; GM*1,2,17;21,26; GM*1,3;5,10,11,13,14,26; andGM*3;5,10,11,13,14,26. In the Kisan sample the haplotypeGM*1,2,17; 21,26 is absent. The intergroup variability in the distribution of these haplotypes is considerable and statistically highly significant. The reasons for that can be attributed to the ethnohistory and to the genetic isolation of these eight endogamous tribal populations. The GM haplotype distribution pattern of all these groups is quite different from that of the non-tribal populations of India, whereas it is in good agreement with that of the so far tested other tribal populations from India. This can be explained by different origin and history of the Indian tribal and non-tribal populations. In the KM system, too, remarkable variability is seen in the distribution of phenotype and allele frequencies among the eight tribal populations under study.     

Substance P given intrathecally at the spinal T9 level increases adrenal output of adrenaline and noradrenaline in the rat

Administration of 10 micrograms of substance P intrathecally to the spinal T9 level of the adult rat, anaesthetized with urethane, provoked an increase in free catecholamines in plasma taken from the inferior vena cava. Adrenaline levels at 1 min after administration were 154.8 +/- 10.8% (mean +/- SE; n = 11) of preadministration levels and noradrenaline levels were 153.5 +/- 11.8% of preadministration levels. Differences between the values of free catecholamines in animals given substance P vs those given vehicle only were statistically significant at 1 and 10 min postinjection, but not at 30 min. Administration of a substance P analogue with central antagonistic properties 15 min before substance P was given prevented expression of the effects of substance P. These results suggest that substance P may be an excitatory chemical mediator of synaptic transmission in spinal pathways controlling adrenal medullary output. Thus dysfunction of substance P mechanisms may underlie some animal models of hypertension and may be involved in some cases of essential hypertension in man as well as in autonomic dysfunction associated with some neurological entities.