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Spontaneous cerebrospinal fluid otorrhea is very rare. Because of nonspecific symptoms, it is usually diagnosed when complications such as meningitis occur. Cerebrospinal fluid leak may be caused by cochlea malformation, which permits nonphysiological communication between subarachnoid space and tympanomastoid cavity. Nearly 20% of congenital sensorineural hearing loss is connected with inner ear bone malformation. We present a case of 40-year-old man suffering since early childhood from recurrent meningitis and right ear deafness, caused by congenital internal ear malformation. For many years, patient with sensorineural hearing loss had not undergone diagnostic radiology; computed tomography scans of the temporal bone had not been performed. Developing meningitis in early childhood was regarded as the reason for deafness.

Meningitis occurs frequently in neonates and can lead to a number of acute, severe complications and long-term disabilities. An early diagnosis of neonatal meningitis is essential to reduce mortality and to improve outcomes. Initial clinical signs of meningitis are often subtle and frequently overlap with those of sepsis, and current haematologic tests do not distinguish sepsis from meningitis. Thus, lumbar puncture (LP) remains the gold standard for the diagnosis of meningitis in infants, and this procedure is recommended in clinical guidelines. Nevertheless, in clinical practice, LP is frequently deferred or omitted due to concerns regarding hypothetical adverse events or limited experience of the performer. Future studies should assess whether a combination of clinical findings and select haematologic tests at disease onset can identify those neonates with the highest risk of meningitis who should undergo LP. Furthermore, clinicians should be convinced that the actual benefits of an early diagnosis of meningitis far outweigh the hypothetical risks associated with LP.

Objectives: The treatment of multiloculated hydrocephalus is a difficult problem in pediatric neurosurgery. Definitive treatment is surgical, yet the approach remains controversial. The author has therefore reviewed his results with endoscopic cyst fenestration (ECF) in the management of this disease., Methods: The author presents the largest series to date of 24 patients with multiloculated hydrocephalus who were treated endoscopically. The group included 10 boys and 14 girls with a mean age of 12.5 months. Uniloculated hydrocephalus was not included in this study because it is a different entity that would be better studied separately., Results: Neonatal meningitis was the most common cause (in 9 patients), followed by intraventricular hemorrhage (in 6 patients), postoperative gliosis (in 6 patients), and multiple neuroepithelial cysts (in 3 patients). Multiplanar magnetic resonance images made early diagnosis possible and are indicated if the computed tomography scan shows disproportionate hydrocephalus. Surgical treatment included ECF (in 24 patients), endoscopic revision of a malfunctioning preexisting shunt (in 6 patients), placement of a new shunt (in 15 patients), and third ventriculostomy (in 3 patients). The ECF was easily performed in all cases through devascularization of the cyst wall by coagulation to prevent recurrence. The results are encouraging with improvement of hydrocephalus in 18 patients (75%). The need for shunt insertion was avoided in 3 patients (12.5%). Endoscopy reduced shunt revision rate from 2.9 per year before fenestration to 0.2 per year after fenestration. During the overall mean follow-up period (30 months), repeated ECF was necessary in 8 patients (33%). Six (75%) of these 8 patients had already undergone shunt treatment before endoscopy. Endoscopic complications were minimal (2 cerebrospinal fluid leaks and 2 minor arterial hemorrhages), and there were no deaths (0%)., Conclusions: An ECF procedure is recommended in the treatment of multiloculated hydrocephalus because it is effective, simple, minimally invasive, and associated with low morbidity and mortality rates.

Chronic infantile neurological cutaneous and articular (CINCA) syndrome is a severe chronic inflammatory disease of early onset, characterized by cutaneous symptoms, central-nervous-system involvement, and arthropathy. In the present study, we report, in seven unrelated patients with CINCA syndrome, distinct missense mutations within the nucleotide-binding site of CIAS1, a gene encoding cryopyrin and previously shown to cause Muckle-Wells syndrome and familial cold urticaria. Because of the severe cartilage overgrowth observed in some patients with CINCA syndrome and the implications of polymorphonuclear cell infiltration in the cutaneous and neurological manifestations of this syndrome, the tissue-specific expression of CIAS1 was evaluated. A high level of expression of CIAS1 was found to be restricted to polymorphonuclear cells and chondrocytes. These findings demonstrate that CIAS1 missense mutations can result in distinct phenotypes with only a few overlapping symptoms and suggest that this gene may function as a potential inducer of apoptosis.

Central nervous system infections and sequelae in the pediatric population may present differently depending on the patient's stage of development. Infections contracted from conception through the neonatal period may affect central nervous system formation, whereas infections acquired later tend to cause parenchymal destruction. This article discusses congenital, focal parenchymal, extra-axial, and diffuse parenchymal infections and their effect on the developing brain.

Specific virulence factors associated with the pathogenesis of Escherichia coli strains causing neonatal meningitis (ECNM), such as the K1 capsular polysaccharide, the S fimbriae, and the Ibe10 protein, have been previously identified. However, some other yet unidentified factors are likely to be involved in the pathogenesis of ECNM. To identify specialized unique DNA regions associated with ECNM virulence, we used the representational difference analysis technique. The genomes of two strains belonging to nonpathogenic phylogenetic group A of the ECOR reference collection were subtracted from E. coli strain C5, isolated from a case of neonatal meningitis. Strain C5 belongs to the phylogenetic group B2 as do the majority of ECNM. We have isolated and mapped 64 DNA fragments which are specific for strain C5 and not found in nonpathogenic strains. Of these clones, 44 were clustered in six distinct regions on the chromosome. The sfa and ibe10 genes were located in regions 2 and 6, respectively. A group of genes (cnf1, hra, hly, and prs) known to be present in a pathogenicity island of the uropathogenic strain E. coli J96 colocalized with region 6. The occurrence of these DNA regions was tested in a set of meningitis-associated strains and in a control group composed of non-meningitis-associated strains belonging to the same B2 group. Regions 1, 3, and 4 were present in 91, 82, and 81%, respectively, of the meningitis strains and in 40, 13, and 47% of the control strains. Together, these data suggest that regions 1, 3, and 4 code for factors associated with the ability of E. coli to invade the meninges of neonates.

Neonatal meningitis has two closely related features: the mechanism of infection and the nature of the pathogen. When transmitted from mother to foetus, the infection is mainly caused by one of three microorganisms: Streptococcus group B, Escherichia coli or Listeria monocytogenes. It may occur before birth, in which case meningitis is of early onset and has a rather poor prognosis. When it occurs later, the infection is a pathological consequence of physiological bacterial colonization, and its symptoms and prognosis are those of post-natal meningitis. Post-natal infections are facilitated by a pre-existing pathology or by prematurity. The responsible organisms (Gram-positive or Gram-negative bacteria, or yeasts) are often multiresistant. Advances in biology provide increasingly clearer explanation of the cerebral complications that determine the medium- and long-term prognosis. The variety of organisms and their frequent resistance to antibacterials make it necessary to use antibiotics that possess an exceptionally broad spectrum.

We report a case of neurosyphilis of the spinal cord in a 38-year-old-man presenting with urinary incontinence and Argyll-Robertson sign. Anamnesis revealed the congenital origin of the disease. Urodynamics findings were consistent with a conus medullaris involvement and CSF study revealed a specific lymphocytic meningitis. A specific spinal arteritis was found by angiography. A quarterly 15 to 20 days course of intravenous penicillin was then instituted during 2 years. A specific treatment of urinary symptoms resulted in complete disappearance of sphincter dyssynergia. Antibiotic therapy was followed by clinical improvement and regression of most of the CSF abnormalities.

The characteristic neuroradiological features of multiloculated hydrocephalus following neonatal meningitis and ventriculitis in five infants are presented. The role of ventriculography and computerized tomography in detecting multiloculated and enlarged ventricles and paraventricular cavities, as well as complete and incomplete septa, is emphasized for early diagnosis and surgical management. CT scan also provides clues indicating the presence of thin but not visualized septa. It is suggested that post-meningitic hydrocephalus should be repeatedly followed-up for detection of multiloculated transformation. Probably this entity occurs less uncommonly than was thought in the past. The CT scan appears the most helpful and safe procedure for the serial evaluation, but ventriculography may be usefully combined with it in order to plan the most suitable operative approach for fenestration of membranes.

Congenital infection is uncommon and the cause of only a small proportion of handicap seen in children but some infections may be preventable or even treatable. As an example, the congenital rubella syndrome first described in the 1940s is preventable by use of the vaccine but cases still occur. It is hoped that with the introduction of the measles, mumps, rubella immunization for young children, rubella will become as rare in the UK as it is in the USA. Cytomegalovirus is now a more common cause of handicap than rubella but no vaccine has been developed. Although antiviral drugs are available for herpes simplex virus and vaccinia, infection mortality in the newborn is high, even following the use of these agents; many HSV infections in the newborn arise following primary and asymptomatic maternal infections so that treatment may start late in the course of the illness. The obstetrician needs to understand the natural history as well as possible investigations available for congenital infections. There may be warning signs which require action, such as herpetic lesions in the genital tract of the mother. Less specific abnormalities during pregnancy, such as intra-uterine growth retardation and spontaneous onset of preterm labour, may point to congenital infection. This chapter describes both antenatal and postnatal management of the major congenital infections. We have included recent research data that should influence clinical practice; studies on HSV which suggest that, for women with a history of recurrent infection, routine viral culture of the genital tract at the end of pregnancy is unnecessary; reports from both the USA and the UK that rubella immunization performed inadvertently during early pregnancy has not resulted in the congenital rubella syndrome. The chapter would not have been complete without a discussion of human immunodeficiency virus, of concern to the obstetrician and midwife. There is still much to be learned about the natural history of this infection in both the mother and infant.