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PubMed2002

Neurotrophin-4 and glial cell line-derived neurotrophic factor in cerebrospinal fluid from meningitis/encephalitis patients.

The neurotrophin-4 and glial cell line-derived neurotrophic factor levels were measured in cerebrospinal fluid from 61 patients with bacterial meningitis, viral meningitis, or encephalitis, and other diseases by means of two-site enzyme-linked immunoassay. Elevated cerebrospinal fluid levels of neurotrophin-4 were demonstrated in four of the 11 patients with bacterial meningitis, and seven of the 23 patients with viral meningitis or encephalitis. None of the other patients demonstrated elevation of the neurotrophin-4 level in cerebrospinal fluid. The neurotrophin-4 levels in cerebrospinal fluid were correlated with the numbers of total and mononuclear cells in patients with viral meningitis/encephalitis. In patients with bacterial meningitis, three of the four patients with elevated neurotrophin-4 levels exhibited persistent abnormalities on computed tomography, and one revealed transient subdural effusion. On the other hand, none of the seven patients without neurotrophin-4 elevation had persistent computed tomography abnormalities, and five patients demonstrated transient computed tomography abnormalities. The glial cell line-derived neurotrophic factor levels were below the detection limit, or only slightly higher than the detection limit, in the patients with or without central nervous system infections. Although the precise roles of neurotrophin-4 and glial cell line-derived neurotrophic factor in central nervous system infections remain to be determined, neurotrophin-4 might play a neuroprotective or immunomodulatory role in central nervous system infections.

Adolescent↗

PubMed2002

Psychomotor development in children with early diagnosed giant interhemispheric cysts.

Systematic studies of cognitive development in individuals with congenital giant interhemispheric cysts (GIC) without neurosurgical intervention are rare. However, data from such studies are of great importance in deciding whether or not to perform cerebral shunting in otherwise asymptomatic patients. In this study, psychomotor function was examined in 13 children (three females, 10 males; mean age at follow-up: females 1 year 6 months, males 3 years 2 months; age range: females 13 months to 4.2 years, males 10 months to 10 years) all diagnosed with GIC during the neonatal period after indicative foetal ultrasound. Eight patients received neurosurgical treatment due to increased intracerebral pressure during the first year of life. Psychomotor development was evaluated using Griffith's Psychomotor Developmental Scales, the Sniders-Oomen Non-verbal Intelligence Scale for Young Children and the Kaufmann Assessment Battery for Children. The majority of patients showed normal intelligence, irrespective of the presence or absence of neurosurgical intervention. Evidence was found that the cognitive outcome might be related to an association of GIC with agenesis of the corpus callosum or additional congenital cerebral malformations, such as gyration anomalies. Main postoperative complications were chronic subdural effusions with spastic cerebral (hemi)-paresis; were present in three patients. In view of the risk of severe postoperative neurological complications and a comparable psychomotor development in patients without neurosurgical treatment, recommendations for early neurosurgical intervention in otherwise asymptomatic patients with a foetal or neonatal diagnosis of GIC should be viewed with caution.

Age Factors↗

PubMed1997

Evaluation of hearing loss with auditory brainstem responses in the early and late period of bacterial meningitis in children.

The hearing function of 50 children with bacterial meningitis was evaluated at the second and 10th days, and eight weeks after admission with auditory brain system responses (ABR) to investigate whether meningitis causes hearing loss. Normal values were obtained in all tests from both ears of 24 patients (48 per cent). Twelve patients (24 per cent) had temporary, and seven (14 per cent) patients had persistent mild degree hearing loss. Severe hearing loss was detected bilaterally in five (10 per cent) patients and unilaterally in two (four per cent) patients. Patients, with other complications such as subdural effusion, convulsion, brain oedema and paralysis were found to have a higher incidence of hearing loss. We observed that patients treated with dexamethasone had 7.7 per cent persistent hearing loss, 11.6 per cent mild hearing loss, 34.6 per cent transient hearing loss, but in the group who did not receive dexamethasone there was 19.2 per cent persistent hearing loss, 15.3 per cent mild hearing loss and 11.6 per cent transient hearing loss. There were other significant differences between the two groups in restoration of normal body temperature, the CSF/plasma glucose concentration ratio was elevated, CSF (cerebro-spinal fluid) protein concentration was decreased and the cell count in the CSF was decreased in the dexamethasone group, significantly more than the group who were not receiving dexamethasone. The hearing loss tended to be more frequent among younger children.

Acute Disease↗

PubMed2004

Neuroradiological findings in glutaric aciduria type I (glutaryl-CoA dehydrogenase deficiency).

This article summarizes the magnetic resonance imaging features of glutaric aciduria type I (GA I) based on the cases presented at the 3rd International Workshop on Glutaryl-CoA Dehydrogenase Deficiency together with a review of previously reported neuroimaging characteristics of GA I. Previous reports have focused on characteristic findings, such as basal ganglia injury and frontotemporal atrophy or hypoplasia, subdural effusions and white-matter disease. Most of these findings have been demonstrated in symptomatic children, i.e. after manifestation of acute encephalopathic crises. In contrast, prospective investigations in presymptomatically diagnosed children are rare. Since more recent investigations have highlighted CNS changes in patients without encephalopathic crises, systematic prospective investigations of neuroradiological findings in this disease are indispensable for a better understanding of this disease. Based on these findings a suggestion for a MRI protocol is presented, supporting a standardized evaluation of patients with GA I.

Amino Acid Metabolism, Inborn Errors↗

PubMed1999

Spontaneous intracranial hypotension.

Spontaneous intracranial hypotension is a rare benign and usually self-limited condition of unknown aetiology. Four cases are presented that illustrate how the MRI features may support a diagnosis suggested on clinical grounds. The MRI findings include diffuse symmetric smooth dural thickening and enhancement, not only at the cranial level, but also involving the cervical spinal dura, subdural effusions and downward displacement of the cerebellar tonsils.

Female↗

PubMed1999

Salmonella meningitis and its complications in infants.

OBJECTIVE: To review the presenting features, complications and outcome of infants with Salmonella meningitis. METHODOLOGY: Retrospective review of all cultures of cerebrospinal fluid positive for bacteria in children below 12 years of age, processed at the Department of Medical Microbiology, University of Malaya Medical Centre, Kuala Lumpur from 1973 to 1997. Records of all cases positive for Salmonella species were retrieved and studied. RESULTS: Thirteen infants aged 3 days to 9 months with Salmonella meningitis were included. The median age of onset of symptoms was 4 months. The clinical and laboratory features were similar to other causes of bacterial meningitis. Salmonella enteritidis was the commonest serotype isolated. Nine infants developed fits, six of which were difficult to control. Other complications noted were hydrocephalus (five), subdural effusions (four), empyema (three), ventriculitis (two), intracranial haemorrhage and cerebral abscess (one each). The use of ampicillin and/or chloramphenicol and inadequate duration of therapy resulted in recrudescence or relapse in five infants. The overall mortality was 18%. The presence of empyema, intracerebral abscess, ventriculitis, hydrocephalus, and intracranial haemorrhage were associated with adverse neurodevelopmental sequelae or death. More than half of those who survived had normal long-term outcome. CONCLUSION: Infants who developed neurological complications as a result of Salmonella meningitis had significant mortality and adverse long-term neurodevelopment outcome.

Anti-Bacterial Agents↗

PubMed1999

The syndrome of spontaneous intracranial hypotension.

The authors report four cases of headache and other symptomatology related to the syndrome of intracranial hypotension. They were seen in a routine clinical practice over the past 3 years. The clinical features, magnetic resonance imaging (MRI) findings, and follow-up of these patients are described. Review of the prior literature on the topic is also included. All four patients presented with orthostatic headache syndrome. Three of the four demonstrated diffuse leptomeningeal thickening and enhancement on MRI studies. One subsequently developed a subdural effusion. One patient demonstrated downward displacement of the posterior fossa initially, which resolved on follow-up MRI scanning. Possible pathophysiologies of the syndrome are discussed.

Adult↗

PubMed2001

Twenty colloid cysts--comparison of endoscopic and microsurgical management.

The management of colloid cyst remains controversial, evaluation of the competing methods seems to be necessary. We report on our experience with colloid cysts in the last decade: ten were managed solely endoscopically, 10 were resected microsurgically (9 via a transcortical/transventricular, 1 via a transcallosal approach). The outcome in the endoscopic group was excellent in 9 cases and unsatisfying in 1 case (recurrence). In the microsurgical group we achieved a good outcome in 5 of 10 cases, a fair outcome in 4 cases and 1 lethal outcome (caused by pulmonary embolism). Complications in the endoscopic group: one intraoperative bleeding, 1 stitch granuloma, 1 mispuncture of the ventricle, and 1 meningitis. Complications in the microsurgical group: 1 subdural effusion, 1 flap infection, 1 mild hemiparesis, 1 transient impairment of consciousness and 1 pulmonary embolism. Mean operative time and length of hospitalization of the endoscopic group were clearly shorter than in the microsurgical group: 91 min versus 267 min time of surgery, 5.1 days versus 18.9 days of hospitalization. Complete resection was achieved in 8 of 10 cases of microsurgery, and in 3 of 10 cases in endoscopy. Endoscopic management results in lower costs and superior patients' comfort. The reduced number of total resections in the endoscopic group may lead to a higher recurrence rate in long-term follow-up, which might be a serious disadvantage of endoscopy. However, more experience in the endoscopic techniques may result in a higher rate of total resection of colloid cysts.

Adult↗

PubMed1996

Clinical course, early diagnosis, treatment, and prevention of disease in glutaryl-CoA dehydrogenase deficiency.

BACKGROUND: Glutaryl-CoA dehydrogenase deficiency (GDD) is a recessively inherited neurometabolic disorder associated with encephalopathic crises and severe extrapyramidal symptoms. Treatment regimens including glucose and electrolyte infusions during acute illnesses, oral carnitine supplementation and/or a low-protein or lysine-restricted diet have been recommended, but their efficacy has been documented only on an anecdotal basis. SUBJECTS AND METHODS: We conducted a retrospective analysis of 57 patients with proven GDD-relating appearance and severity of neurological disease to age and clinical status at diagnosis, glutaric acid levels in body fluids, and different treatment regimens. RESULTS: Thirty-six patients were diagnosed after the onset of neurological disease (symptomatic group), twenty-one before (presymptomatic group). Carnitine levels were found to be reduced in all patients at diagnosis. In the symptomatic group, macrocephaly had been present around birth and was followed by rapid postnatal head growth in 70% of the children. The patients often showed symptoms such as hypotonia, irritability, and jitteriness followed by an acute encephalopathic crisis occurring on average at 12 months of age. Common neuroimaging findings included frontotemporal atrophy, subependymal pseudocysts, delayed myelination, basal ganglia atrophy, chronic subdural effusions and hematomas. In four patients the latter two findings were initially misinterpreted as resulting from child abuse. Other important misdiagnoses in older siblings who were affected and went undiagnosed include postencephalitic cerebral palsy, dystonic cerebral palsy and sudden infant death syndrome. Metabolic treatment did not convincingly improve the neurological disease, although it may have prevented further deterioration. Symptomatic treatment with baclofen or benzodiazepines was effective in reducing muscle spasms. Children in the presymptomatic group were diagnosed because of familiarity for the disease (n = 13), macrocephaly and/or additional minor neurological signs in infancy (n = 6), or acute encephalopathy, which was fully reversible after prompt treatment (n = 2). After diagnosis, all children were treated with oral carnitine, fluid infusion during intercurrent illnesses and, in addition, a diet was started in 13 of the 21 children. All 21 children except one (born prematurely at 31 weeks) have continued to develop normally up to now. Mean age at report is 6.3 years with a range from 6 months to 14.8 years. In older patients, the neuroradiological changes, present in infancy as in the symptomatic patients, became less prominent and in one girl disappeared. CONCLUSIONS: In presymptomatic children with GDD, the onset of neurological disease can be prevented by vigorous treatment of catabolic crises during illnesses together with carnitine supplementation. The importance of dietary therapy remains unclear and needs further evaluation. The potential treatability of GDD calls for increased attention to early presenting signs in order to recognize the disorder and to initiate treatment before the onset of irreversible neurological disease.

Adolescent↗

PubMed1993

Control of hydrocephalus by endoscopic choroid plexus coagulation--long-term results and complications.

Endoscopic choroid plexus coagulation has been used for the control of hydrocephalus regularly at this unit for the past 20 years. 156 of these operations have been performed on 116 patients and the aim of this study was to assess the rate of long-term control of hydrocephalus and the complications of the procedure. Data have so far been found for 98 patients with a median age at operation of 5 months (range 1 week-30 years). After a mean follow-up period of 10.5 years there were 32 (33%) patients continuing without a ventricular shunt. One patient developed papilloedema and required ventricular shunting 16 years after choroid plexus coagulation. There were no deaths resulting from operation. 5 patients developed post-operative meningitis and 3 patients had post-operative infections of implants (2 shunts and 1 reservoir). No cases of post-operative meningitis have occurred since vancomycin and gentamicin have been added to the solution used to perfuse the ventricles after operation (28 cases). Other complications included post-operative fits (2), respiratory arrest in a premature baby (10, low-pressure state (1), blocked or leaking external ventricular drain (4), drain displacement (4), subdural effusion (1) and per-operative minor ventricular bleeding forcing abandonment of the procedure (2).

Adolescent↗

PubMed1997

The importance of the dual-switch valve for the treatment of adult normotensive or hypertensive hydrocephalus.

Since the beginning of 1995 the new hydrostatic dual-switch valve (DSV) was implanted in 35 adult patients with hydrocephalus of different etiology. 26 patients suffered from normotensive hydrocephalus (10 idiopathic and 16 symptomatic), and 9 patients from hypertensive hydrocephalus of various origin. The first 21 cases of this cohort were compared in a randomized study with a comparable group of 21 hydrocephalic patients who received a conventional differential-pressure (DP-) valve. The clinical status and CT were assessed prior to shunting, 14 days and 3 and 6 months after the operation. The reduction of ventricular size was evaluated by the measurement of the Evans Index. The CT follow-up in the DSV group was characterized by an only minimal (14) or only slight (16) reduction of ventricular size in the vast majority of cases. A comparison of 21 patients with a DSV and the patients with DP valves, evaluated by measuring the reduction of the Evans Index, revealed a distinctly higher percentage of significant regressions in the DP valve collective, without doubt due to chronic overdrainage. The overall clinical result of our 35 patients with a DSV was excellent and good in 31 patients, but the outcome seems to be more dependent on the preshunt damage of the brain than on hydrocephalic aspects. A neglegible incidence of subdural effusions in the DSV group compared to 11 cases in the DP valve collective reflects the ability of the DSV to prevent overdrainage. The capability of the DSV to maintain the IVP within physiological limits after shunting, especially in the upright position, is documented by a comparison with possible unphysiological IVP variations in other valve constructions, which depend on the level of implantation, subcutaneous pressure or CSF flow through the valve.

Cerebrospinal Fluid Shunts↗

PubMed1993

Parasomnia with rhythmic movements manifesting as nocturnal tongue biting.

The case of a healthy 2-year-old girl with repeated nocturnal tongue biting as a result of rhythmic movements of the jaw associated with body rocking in non-REM sleep is described. Parasomnias manifesting with rhythmic, stereotyped movements of the head, trunk and extremities are well described in healthy children. The term rhythmic movement disorders (RMD) was introduced for these repetive movements in sleep which may appear as head banging (jactatio capitis), body rocking or leg rolling. Severe injuries including fractures, subdural effusions and eye injures are reported. Repeated tongue injuries have not been described as a consequence of RMD. The differential diagnosis from nocturnal seizures is crucial to avoid overtreatment of this benign albeit dramatically presenting condition.

Bites, Human↗

PubMed2004

Cranial computed tomography in purulent meningitis of childhood.

The cranial computed tomography (CT) findings of 48 children with purulent meningitis were examined, prospectively, to determine the importance of cranial CT findings on the prognosis of childhood meningitis, in a developing country. The age of children ranged from 2 months to 13 years. Of 48 patients, 29 (60.5%) survived without sequelae, 13 (27%) survived with sequelae, and six (12.5%) died. Cranial CT was normal in 21 (43%) patients of 48 children with meningitis at admission. Abnormal CT findings were detected in 10, 11, and 6 children in the groups of survived without sequelae, survived with sequelae, and deaths, respectively, at admission (p <.05) We found that CT scan results were correlated with neurological signs (p <.05). At least one or more cranial CTs were was re-taken in children in whom the first CT revealed abnormal findings; we did not find a statistically significant difference for the follow-up CT findings between the groups (p >.05). Hydrocephalus and subdural effusion were the commonest abnormal CT findings. In conclusion, our findings showed that cranial CT may safely be used to detect intracranial complications of meningitis in childhood and the ratio of sequelae and death were more common in children with abnormal cranial CT than those of normal cranial CT findings. Additionally, there was a positive correlation between CT scan results and neurological signs.

Adolescent↗

PubMed1998

Origin of chronic subdural haematoma and relation to traumatic subdural lesions.

The origin of chronic subdural haematoma (CSDH) and the pathogenesis of subdural hygroma (SDG) are still controversial issues. These issues and relationships between these traumatic subdural lesions are discussed. The origin of CSDH is usually a SDG, although a few cases are caused by acute subdural haematomas (ASDH). Subdural hygroma is produced by separation of the dura-arachnoid interface, when there is sufficient subdural space. When the brain remains shrunken, the SDG remains unresolved. Any pathologic condition inducing cleavage of tissue within the dural border layer at the dura-arachnoid interface can induce proliferation of dural border cells with production of neomembrane. In-growth of new vessels will follow, especially along the outer membrane, then bleeding from these vessels occurs. These unresolved SDGs become CSDHs by repeated microhaemorrhage from the neomembrane. Although most victims with ASDH underwent surgery or died, some patients could be managed conservatively. Since the ASDH is usually absorbed within a few weeks, only a very few ASDHs become CSDHs, when there is a sufficient potential subdural space. Chronic subdural haematoma can arise from ASDH, but more commonly from SDG. Such transformation, or development of a new subdural lesion, is a function of the premorbid status and the dynamics of absorption and expansion.

Acute Disease↗

PubMed1984

A comparison of ampicillin-cefotaxime and ampicillin-chloramphenicol in childhood bacterial meningitis: an experience in 55 patients.

Ampicillin-cefotaxime was tested as initial therapy of presumptive bacterial meningitis in 55 children greater than or equal to 2 months of age at our hospital. During the first year of this ongoing trial, 11 patients, 10 whose CSF yielded ampicillin-resistant Haemophilus influenzae type b (MIC greater than 16 mg/l, beta-lactamase +) and one, indole-negative proteus (MIC 4 mg/l), were begun on ampicillin-cefotaxime and then continued on cefotaxime alone. All did well clinically except one who convulsed briefly but recovered without sequelae. The cefotaxime MICs/MBCs of the beta-lactamase-positive H. influenzae isolates (less than or equal to 0.007 to 0.03/less than or equal to 0.007 to 0.12) and the proteus isolate (0.03/0.12) were significantly lower than chloramphenicol MICs/MBCs (0.25 to 1.0/0.5 to 1.0 and 8/greater than 16). We followed 44 other children with meningitis due to ampicillin-sensitive organisms who were treated with ampicillin or penicillin after 1 or 2 days of ampicillin-cefotaxime. Aetiological agents included ampicillin-sensitive H. influenzae (25), pneumococci (9), meningococci (8), Strept. MG (1) and Listeria monocytogenes (1). 40/44 recovered uneventfully. There were 4 neurological complications: the streptococcal meningitis sustained a brain abscess and the three others were motor incoordination (sensitive haemophilus), hearing loss and subdural effusion (2 pneumococci). There were no deaths. 18/48 children managed initially with ampicillin-chloramphenicol during the same 12-month period one year earlier had significant neurological complications and/or sequelae and there was one death; aetiological agents included sensitive H. influenzae (30), pneumococci (9), ampicillin-resistant haemophilus (5), meningococci (3) and pneumococci plus strept. MG (1). The two groups were comparable except for the number of resistant haemophilus and meningococcal strains and underlying disease more frequent in the ampicillin-cefotaxime group. A significant reduction of neurological morbidity (5/55 or 9.1% vs. 18/48 or 37.5%:P less than 0.001) was therefore associated with the ampicillin-cefotaxime schedule in the initial treatment of proven bacterial meningitis. A prolonged hospitalization (greater than 15 days) was less frequent (P less than 0.01) in the ampicillin-cefotaxime group (3/55 or 5.5% vs. 13/48 or 27.1%). The results of the trial to date are considered to be very promising.

Adolescent↗

PubMed1995

Cranial sonography in pyogenic meningitis in neonates and infants.

Cranial sonography was performed in 34 infants with proven meningitis and with clinical suspicion of its complications, utilizing the patent anterior fontanelle. Twenty-five patients showed various sonographic findings which included ventriculomegaly, echogenic sulci, subdural effusion, ventriculitis, infarction/cerebritis, cerebral oedema, porencephalic cyst, etc. Sonography is safe and cost-effective in diagnosis of complications, and management of pyogenic meningitis in infants.

Brain Diseases↗

PubMed1987

Radionuclide scintigraphy of the brain and ultrasound studies in tubercular meningitis.

The present study was undertaken to assess the usefulness of brain scintigraphy and ultrasound evaluation of changes described in tuberculous meningitis. Brain scans were done with Tc-99m DTPA in 127 cases of tuberculous meningitis (TBM) and ultrasound studies in 41 cases with open anterior fontanelles. The data show that 54/127 (42.5%) of cases had abnormal brain scans, of which 33/54 had cerebrovascular infarcts and 14/54 had tuberculomas, while the remaining cases had either subdural effusions or generalized increased activity. The cerebrovascular infarcts were more frequent in patients with acute onset of TBM with associated hemiplegias. The ultrasound studies indicated that 80% of all cases of TBM had a mild to severe dilatation of the ventricles. About 55% of cases had a dilatation of only the lateral ventricles while the remaining cases had a block of aqueduct or beyond the fourth ventricles. Increased intracranial pressure changes were associated with a higher incidence of severe ventricular dilatation. Noninvasive detection methods in the study of TBM are important not only from the point of view of diagnosis but also to understand and evaluate the pathological changes occurring in vivo during the disease process and to judge the progress and response to treatment. A combination of ultrasound and brain scans offers diagnostic accuracy comparable to CT. Apart from cost, the larger radiation doses resulting from CT studies preclude its routine use in pediatric subjects.

Brain↗

PubMed2001

Review of the use of somatosensory evoked potentials in the prediction of outcome after severe brain injury.

OBJECTIVE: Review the predictive powers of somatosensory evoked potentials (SEPs) in severe brain injury. DATA SOURCES: Publications in the scientific literature, manual review of article bibliographies, and questioning workers in the field. STUDY SELECTION: Studies addressing the prediction of outcome after severe brain injury using SEPs. DATA EXTRACTION: To determine the outcome of patients with either normal or bilaterally absent SEPs as categorized using the Glasgow Outcome Scale into favorable outcomes (good or moderate disability) or unfavorable outcomes (severe disability, vegetative, or dead). Studies were included if they were in English and allowed the determination of outcomes for all patients with normal or bilaterally absent SEPs. Papers were not considered if subjects were neonates, consisted of abstracts where all necessary details were unavailable, were case reports or duplications of other published studies, or dealt only with brain dead subjects. DATA SYNTHESIS: For all studies (n = 44), positive likelihood ratio, positive predictive value, and sensitivity were 4.04, 71.2%, and 59.0%, respectively, for normal SEPs (predicting favorable outcome) and 11.41, 98.5%, and 46.2%, respectively, for bilaterally absent SEPs (predicting unfavorable outcome). Summary receiver operating characteristic curve analysis detected a cut-off criterion effect for only blinded studies of bilaterally absent SEPs. Twelve patients (12/777) were identified with bilaterally absent SEPs who had favorable outcomes. These false positives are typically pediatric patients or have suffered traumatic brain injuries. We suggest criteria for the use of bilaterally absent SEPs in the prediction of poor outcome, which include absence of focal lesions, subdural or extradural fluid collections, and no decompressive craniotomy in previous 48 hrs. Using these criteria the data suggest that the false-positive rate is <0.5% for bilaterally absent SEPs. CONCLUSIONS: SEPs are powerful predictors of outcome, particularly poor outcome, if patients with focal lesions, subdural effusions, and those who have had recent decompressive craniotomies are excluded.

Adult↗