[The interdisciplinary approach to the rehabilitation of the patients presenting with congenital atresia of the external auditory canal and the concomitant microtia]. / Mezhdistsiplinarnyi podkhod k reabilitatsii patsientov s vrozhdennoi atreziei naruzhnogo slukhovogo prokhoda i mikrotiei.

The objective of the present study was to develop and introduce into the clinical practice the method for the combined aesthetic and functional rehabilitation of the patients presenting with congenital atresia of the external auditory canal (CAEAC) and the concomitant microtia. A total of 8 patients at the age from 6 to 21 years with unilateral CAEAC and microtia were given the surgical treatment. During the intervention, atresia was resolved using the trans-mastoid approach, tympanoplasty of autofasciae and ossiculoplasty making use of the partial titanium prosthesis and the placement of cranial osteointegratable titanium implants. At the second stage of the surgical intervention the 3D silicone prosthesis of the auricle shaped on an individual basis were used. The long-term follow-up observations have demonstrated the stable formation of the tube of the external auditory canal, with the bone-air interval amounting to 15-20 dB. The auricular prosthesis was the mirror image of the natural ear and completely concealed the congenital defect.

Purinergic mechanisms in neuroinflammation: An update from molecules to behavior.

The principle functions of neuroinflammation are to limit tissue damage and promote tissue repair in response to pathogens or injury. While neuroinflammation has utility, pathophysiological inflammatory responses, to some extent, underlie almost all neuropathology. Understanding the mechanisms that control the three stages of inflammation (initiation, propagation and resolution) is therefore of critical importance for developing treatments for diseases of the central nervous system. The purinergic signaling system, involving adenosine, ATP and other purines, plus a host of P1 and P2 receptor subtypes, controls inflammatory responses in complex ways. Activation of the inflammasome, leading to release of pro-inflammatory cytokines, activation and migration of microglia and altered astroglial function are key regulators of the neuroinflammatory response. Here, we review the role of P1 and P2 receptors in mediating these processes and examine their contribution to disorders of the nervous system. Firstly, we give an overview of the concept of neuroinflammation. We then discuss the contribution of P2X, P2Y and P1 receptors to the underlying processes, including a discussion of cross-talk between these different pathways. Finally, we give an overview of the current understanding of purinergic contributions to neuroinflammation in the context of specific disorders of the central nervous system, with special emphasis on neuropsychiatric disorders, characterized by chronic low grade inflammation or maternal inflammation. An understanding of the important purinergic contribution to neuroinflammation underlying neuropathology is likely to be a necessary step towards the development of effective interventions. This article is part of the Special Issue entitled 'Purines in Neurodegeneration and Neuroregeneration'.

Congenital brain infections.

Pediatric congenital intracranial infections are a group of different and important entities that constitute a small percentage of all pediatric infections. The causal factors and clinical presentations are different in children compared with adults. They require early recognition because delay diagnosis and initiation of treatment may have catastrophic consequences. Despite improvements in prenatal screening, vaccine safety, and antibiotics, infections of the central nervous system remain an important cause of neurological disabilities worldwide. This article reviews the most common congenital infections and their imaging findings.

MR imaging of neonatal brain infections.

Infections of the brain in the postnatal period differ from those in older children as a result of a combination of distinct epidemiologic factors in general, and immaturity of neonatal brain and immunologic host response in particular. It has been recognized that clinical and neurologic signs are often nonspecific, sometimes scarce, and seldom correlate with the extent of neuroimaging findings, thus warranting an early MR imaging examination in the course of the disease, enabling rapid therapy institution and better clinical outcome. This article reviews most of postnatal pathogen agents involved in neonatal brain infections, related physiopathology, and neuroimaging findings.

Neuroimaging of pediatric central nervous system cytomegalovirus infection.

Cytomegalovirus (CMV) is a ubiquitous virus that usually results in asymptomatic or clinically benign infection. However, there are two groups of patients whose response to CMV infection is much more severe: those who are infected during fetal development and those who are immunocompromised. Although the manifestations of these types of infection differ, both often result in substantial neurologic sequelae. Imaging plays a key role in the diagnosis of both congenital and acquired CMV infection. Neurologic findings of congenital CMV infection include intracranial calcification, migrational abnormalities, cerebral and cerebellar volume loss, ventriculomegaly, and white matter disease. The presence of these findings in children with neurodevelopmental delays is suggestive of congenital CMV infection, even if the child was asymptomatic at birth. Certain imaging features also may indicate future neurologic deficits in symptomatic infants. Acquired CMV infection is potentially deadly in immunocompromised patients such as those infected with human immunodeficiency virus or with acquired immune deficiency syndrome and those with a history of solid organ or bone marrow transplantation. Imaging findings of acquired CMV infection often are nonspecific; however, they may indicate a need for further serologic analysis to determine if CMV infection is present. Early recognition and treatment of central nervous system CMV infection is vital for effective treatment, and familiarity with the imaging findings of this common infection is important for accurate diagnosis.

Aicardi syndrome mimicking intrauterine hydrocephalus.

Congenital enlargement of the cerebral ventricles is now commonly recognized in utero due to the availability of high resolution prenatal ultrasonography. It is important to distinguish between congenital hydrocephalus and ventricular enlargement due to malformations, infections or other destructive processes because these disorders can have markedly different prognoses. We report an infant diagnosed with Aicardi syndrome in the newborn period based on brain MRI and ophthalmological findings after she was referred for evaluation of hydrocephalus seen on fetal ultrasound. Aicardi syndrome most commonly comes to medical attention because of seizures later in infancy.

An unusual manifestation of a neonatal Chlamydia infection.

A 25-day-old boy was admitted to hospital because of pneumonia and additionally developed symptoms of encephalitis. The immune fluorescence test for Chlamydia trachomatis in tracheal fluids was positive. Furthermore, ligase chain reaction for C trachomatis was positive in the cerebrospinal fluid. The antibiotic regimen was changed to erythromycin intravenously. C trachomatis encephalitis is rare in neonates and may result from a defect in the alternative pathway of complement activation which was the case in this patient.

Central nervous system infections in the pediatric population.

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.

Activation of the innate immune system in murine congenital Toxoplasma encephalitis.

A murine model of congenital Toxoplasma encephalitis (CTE) was established in NMRI mice following prenatal infection with a low dose of Toxoplasma gondii (DX strain). Histopathologically, the disease exhibited the key features of human CTE including foci of necrosis, intracerebral calcifications, and ventriculitis. The inflammatory response in the brain of infected animals was predominantly mediated by macrophages and granulocytes with additional participation of astrocytes. These findings indicate that the prenatal Toxoplasma infection closely parallels human CTE, but differs significantly from adult toxoplasmosis.

[Severe forms of herpes simplex in newborn infants]. / Tiazhelye formy gerpes-virusnoi infektsii u novorozhdennykh.

Generalized herpetic infection in the newborn runs its course in association with cerebral lesions as well as with injuries to the lungs, heart and liver. The infection diagnosis should take into account the mother's epidemiological anamnesis, the clinical manifestations in the child and the results of the simultaneous use of several methods (fluorescent antibody test, IEA, virological test). Of paramount importance is the simultaneous examination of the mother. Of no less importance is echoencephalography, namely the detection of cysts in white matter of the frontal lobes.

[Clinical picture of congenital Cytomegalovirus infection in infants in their first year of life]. / Klinicheskaia kartina vrozhdennoi tsitomegalovirusnoi infektsii u detei pervogo goda zhizni.

A study was made of the clinical picture of congenital cytomegaloviral infection in 40 children of the first year of life. The diagnosis was established on the basis of demonstration in all the patients of IgM antibodies to cytomegalovirus (IgM anti-CMV) by EIA with the aid of the commercial test systems. Besides, 81% of the children were found to have cytomegalic-specific cells in urine, 17.6% in saliva, and 72.5% demonstrated IgG anti-CMV (in accordance with EIA). The majority of the patients (70%) were born premature. The most typical clinical signs of congenital cytomegaloviral infection were adynamia, jaundice, liver and CNS injury, prenatal hypotrophy; 45% of the children had developmental abnormalities and stigmas of dysembryogenesis. In 97.5% of the mothers, the pregnancy ran a pathological course. The disease history often pointed to miscarriages (10%) and death of the children due to intrauterine infection in the early times after birth (15%).

An unusual CT presentation of congenital cerebral toxoplasmosis in an 8 month-old boy with AIDS.

We report on a 8-month-old boy with AIDS, born of an asymptomatic mother with positive HTLV-III serology. He was hospitalized in the Intensive Care Unit because of anemia, fever and hepatosplenomegaly. Chest X-ray showed pneumonia and subsequent blood cultures were positive for Candida albicans. After 3 days of Amphotericin B treatment, the patient was transferred to Infectious Disease Department. After 30 days of hospitalization, the patient developed a rapid neurological impairment evolving into coma. CT scan showed a round, ring-shaped low density lesion with hyperdense and enhancing haemorrhagic centre in the left basal ganglia and a smaller hypodense lesion on the right. There was also evidence of cortical atrophy and mild ventricular dilatation. Such lesions are more commonly described in children with AIDS and congenital cytomegalic inclusion virus (CMV) encephalitis. In this case toxoplasma cysts were shown microscopically reinforcing the contention that in patients with AIDS, toxoplasma gondii infection may occur with atypical manifestation.

Cytomegalovirus encephalopathy in an infant with congenital acquired immuno-deficiency syndrome.

A female infant born pre-term to a HIV seropositive mother presented at birth with seropositivity for HIV and CMV viruria. At five months of age she developed an AIDS-related complex. Six months later she died from rapidly progressive diffuse encephalopathy. Post mortem examination revealed generalized CMV infection. Neuropathological examination showed a nodular encephalitis with occasional cytomegalic cells containing characteristic CMV inclusion bodies. There was no evidence of HIV encephalitis; immunostaining for HIV antigen (gp 41) was negative. Opportunistic infections in infants with congenital AIDS are the exception. To our knowledge, only one case of CMV encephalitis in an infant with congenital AIDS has been reported previously. In that case, as in the present one, a reactivation of a congenital CMV infection is likely.

Echogenic vasculature in the basal ganglia of neonates: a sonographic sign of vasculopathy.

The vessels that supply the basal ganglia and thalami are not normally conspicuous on the cranial sonograms of neonates. Twelve neonates with abnormally echogenic or "bright" vessels on cranial sonograms were studied. Records of these 12 patients were reviewed and were correlated with the neuropathologic findings available in four. The clinical diagnoses were cytomegalovirus infection (five patients), rubella (two patients), congenital syphilis (one patient), and trisomy 13 syndrome (three patients). No diagnosis was made in one infant. At neuropathologic examination, perforating medium-sized arteries to the basal ganglia and thalami had thickened hypercellular walls, with deposits of amorphous basophilic material in three infants. Results of computed tomography and radiography of brain sections were normal in these areas. Sonography is helpful in detecting early noncalcific inflammation and mineralization in vasculitis. Although nonspecific, these findings should alert the physician to the possibility of congenital infection or chromosomal abnormality.

The evolution of neonatal herpes encephalitis as demonstrated by cranial ultrasound with CT correlation.

Three neonates with herpes encephalitis had serial cranial ultrasound and CT studies performed during their hospitalization. Initially, subtle changes of diffuse brain edema were present but with no ventricular compression. A second phase revealed progressive brain edema with ventricular compression. A third phase with enlarging ventricles indicates developing encephalomalacia. Occasionally cyst formation may be noted within the cerebral white matter, a phenomenon initially more readily evident by ultrasound.

Liquefactive necrosis in Coxsackie B encephalitis.

Coxsackieviruses may cause serious illness in infants and children, specifically myocarditis and meningoencephalitis. Central nervous system lesions have been characterized as inflammatory in nature with mononuclear cell infiltration, neuronophagia, and glial nodule formation largely confined to the brain stem and spinal cord. We present two infants with documented Coxsackie B virus infection who also had widespread multifocal areas of liquefaction necrosis unassociated with inflammation. Such areas of bland necrosis, especially in the cerebrum, are unusual in Coxsackie B virus infection, and may provide the morphological substratum of permanent neurologic impairment in children who survive.