Lobar Form Research Articles

Alobar holoprosencephaly: A case report

Introduction: Holoprosencephaly (HPE) is a rare brain malformation, which results from a cleavage defect of the prosencephalon. Three forms have been described as: alobar, semi-lobar, and lobar forms. Case Report: We report a rare case of holoprosencephaly, diagnosed at the maternity of the Ibn Rochd Hospital Center in Casablanca. Conclusion: Holoprosencephaly is secondary to a cleavage anomaly of the prosencephalon, the diagnosis is based on echotomography, computed tomography (CT) scan and nuclear magnetic resonance imaging (MRI). It is important to perform a karyotype to look for a chromosomal anomaly. Holoprosencephaly, although rare, should be recognized and diagnosed; minor forms are likely to benefit from medical replacement therapy or ventricular shunting.

Abstract 125: Enlarged Perivascular Spaces In The Centrum Semiovale Are Associated With Incident Late-life Depression: Framingham Heart Study

Introduction: Perivascular spaces visible on brain MRI (PVS) are considered cerebral small vessel disease (CSVD) markers, possibly reflecting impaired brain fluid and metabolite clearance, and could contribute to depression in old age. This study aims to determine the association of PVS with prevalent and incident depression in the community-based Framingham Heart Study (FHS). Methods: FHS participants free of stroke and dementia with available Center for Epidemiological Studies Depression (CES-D) scale and PVS ratings in the basal ganglia (BG) and centrum semiovale (CSO) were included. Depression was defined as CES-D score≥16 or use of antidepressants. PVS were rated on a scale from I-IV in the BG and CSO using a validated method, with grades III-IV representing high PVS burden. A mixed score was created to describe high burden in the BG and/or CSO (0=no high ePVS burden, 1=high burden in the BG, 2=high burden in the CSO, 3=high burden in both regions). Logistic regression analysis was used to assess the association between PVS in the CSO, BG, and mixed regions and prevalent and incident depression. Models adjusted for age, sex, FSH cohort, time interval between CES-D score and MRI measurements, body mass index, smoking status, prevalent hypertension and diabetes. Results: Among 3815 participants included (mean age 57±14, male 47%), incident depression was observed in 19% over the follow up period ranging from 8 to 15 years subsequent to PVS rating. In multivariable adjusted analyses, high burden PVS in the CSO was associated with incident depression (OR 1.75 [1.18, 2.60], p=0.005). In the mixed score, concurrent high burden PVS in both regions (OR 2.10 [1.13, 3.91], p=0.02) and high burden in the CSO only (OR 1.65 [1.05, 2.58], p=0.03) were associated with incident depression. PVS were not associated with prevalent depression. Conclusion: In conclusion, high PVS burden was associated with incident depression in a community-based sample free of stroke and dementia. The association was predominantly driven by high burden of CSO PVS, suggesting that lobar forms of CSVD may play role in late life depression, possibly reflecting disrupted neuroanatomic circuits involved in mood control, and differing from BG PVS.

Sonography of fetal holoprosencephaly: a guide to recognize the lesser varieties

Background Alobar holoprosencephaly (HPE) is easily detected during a first-trimester screening examination, conversely, recognizing the lesser varieties may be difficult even in the second trimester. Objectives To describe the imaging findings of a cohort of fetuses with holoprosencephaly (HPE) and to elucidate the appearances of the different anatomical varieties. Materials and methods We reviewed medical records and stored images of pregnant women referred to our clinic because of a diagnosis or the suspicion of various forms of HPE. We reported the imaging characteristics, the presence of other associated anomalies, magnetic resonance findings, karyotype and autoptic examinations when available. Results Alobar forms show great distortion of normal brain anatomy, with a single ventricle detectable during the first trimester of pregnancy. Extracerebral, face and karyotype abnormalities are often associated. In semilobar and lobar forms the septum pellucidum is typically absent in axial planes, with fused frontal horns, while posterior fossa is often normal. At multiplanar neurosonogram, anomalies involving corpus callosum and cortex development can be detected. Face abnormalities are mild in lobar forms: receding forehead, various degrees of hypotelorism and the presence of a single central maxillary incisor are reported. Conclusions The alobar forms are detectable since the first trimester, with a peculiar single ventricle and extremely frequent extracerebral and karyotype abnormalities. The semilobar and lobar forms are more challenging and the diagnosis is easily missed in a mid-trimester screening exam unless a careful evaluation of both cavum septi pellucidi and frontal horns as well is conducted.

The incidence of Mycoplasma pneumoniae in community acquired pneumonia among children: one centre study

Background: Mycoplasma pneumoniae (M. pneumoniae) is an important cause of pediatric community acquired pneumonia (CAP). Aim: The aim of this study was to determine the incidence of M. pneumoniae in pediatric community acquired pneumonia and to determine the most frequent clinical findings of M. pneumoniae CAP. Methods: A total of 83 pediatric CAP patients were subjected to history taking, clinical examination, chest X-ray, CBC, CRP and serum antimycoplasma pneumonia IgM and IgA by ELISA. Results: Twenty-nine (34.9%) out of 83 patients were positive for M. pneumoniae Ig M while 2 (3.4%) patients were positive for M. pneumoniae Ig A. There were more infection (54%) in age group (5-9 years; p value = <0.001). M. pneumoniae pneumonia infected patients were presented with cough (29/29; 100%), fever (29/29; 100%), malaise (18/29; 43.8%), headache (16/29; 33.8%), wheeze (21/29; 52.5%), chest discomfort (13/29; 44.8%), sore throat (13/29; 46.4%), rhinitis (8/29; 27.5%) and pharyngitis (6/29; 24%). The most frequent X ray findings in M. pneumoniae pneumonia was air space pneumonia (71%); segmental more than lobar form (p-value = < 0.0001). Conclusion: The findings of this study highlight the clinical significance of M pneumoniae in pediatric community acquired pneumonia.

To the question of the lobar form of holoprocephalus

An overview of the literature data on holoprozencephaly is given. It is considered a classic look on this congenital pathology. The opinions of modern researchers are given. The reasons for the large mosaicity of the echographic and radiological images of the same variant of the anomaly are analyzed. It is concluded that holoprozencephaly does not consist of three stable neuroanatomical forms, but is a continuous spectrum of anomalous development of proencephalon without clear boundaries, starting with the most severe variants and ending with microscopic forms.

Hepatic Steatosis: Etiology, Patterns, and Quantification

Hepatic steatosis can occur because of nonalcoholic fatty liver disease (NAFLD), alcoholism, chemotherapy, and metabolic, toxic, and infectious causes. Pediatric hepatic steatosis is also becoming more frequent and can have distinctive features. The most common pattern is diffuse form; however, it can present in heterogenous, focal, multinodular, perilesional, perivascular, subcapsular, and lobar forms. Focal steatosis and fat sparing can occur because of the presence of veins of Sappey, pancreaticoduodenal vein, and aberrant right and left gastric veins, which drain into the liver as third inflow. Hypersteatosis and multinodular forms can mimic metastasis in patients with cancer. Perilesional fat can be seen in insulinoma. Recent introduction of proton-density fat fraction enabled easy and reproducible quantification of hepatic fat. Follow up of patients with NAFLD can be performed for the assessment of treatment response using proton-density fat fraction as biomarker. Multiecho gradient-echo techniques also simultaneously calculate T2* maps, which is important to rule out coexisting hepatic iron overload. NAFLD can progress to steatohepatitis (nonalcoholic steatohepatitis), which can result in cirrhosis. Magnetic resonance (MR) elastography and functional evaluation with Gd-EOB-DTPA are becoming important for monitoring this process. Hepatocellular carcinoma can develop in patients with NAFLD, which is usually a large tumor with necrotic center. In the future, fatty acid maps obtained by MR imaging may allow more detailed analysis of steatosis. MR imaging is superior to ultrasonography and computed tomography for comprehensive evaluation of steatosis.

Ultrasound Monitoring and Age Sonographic Characteristics of Community-Acquired Pneumonia in Children

The aim of the investigation was to study ultrasound semiotics of community-acquired pneumonias in children of different age, and its dynamics in the course of treatment. Materials and Methods. Pneumonia semiotics study and dynamic monitoring of the disease have been carried out on the basis of chest ultrasound of 154 children divided into 4 age groups: 14 infants (9.1%) from birth to 3 months; 60 children (39.0%) from 3 months to 3 years of age; 49 children (31.8%) from 4 to 7 years of age; 31 children (20.1%) from 7 to 18 years of age. During the hospital stay the children underwent ultrasound at least three times: on admission to determine pneumonia volume and localization; on day 3–5 or day 5–7 to assess the dynamics of sonographic picture and the necessity of etiotropic therapy change; before discharge to evaluate residual effects and determine the follow-up strategy. Ultrasound revealed zones of airness impairment of various size in the pulmonary tissue of all children. Pneumonia semiotics presented extensive and lobar forms of pneumonia. An extensive type occupies a segment or a lobe, and looks like a delta-shaped air-free area with fuzzy contours and heterogeneous structure, with an air echobronchogram, and occurs in children of older age. Lobar pneumonia presents small rounded subpleural infiltrate up to 15 mm with a homogeneous hypoechogenic structure, and is typical of newborns and younger children. The size and echostructure of apneumatic (air-free) area were the assessment criteria of pneumonia regression. Conclusion. Chest ultrasound is an informative radiologically safe technique for diagnosing and dynamic control of pneumonia in children of different age, which enables timely assessment of etiotropic therapy efficacy.

Holoprosencephaly: a survey of the entity, with embryology and fetal imaging.

Structural malformations of the brain are an important cause of childhood mortality and morbidity, with the latter having long-term financial and psychosocial implications for the affected child and family. Holoprosencephaly (HPE) is a severe brain malformation characterized by abnormal cleavage of the prosencephalon in the 5th gestational week. Aprosencephaly and atelencephaly occur earlier because of failure in the formation of the prosencephalon and telencephalon, respectively. The HPE holoprosencephaly spectrum classically includes alobar, semilobar, and lobar forms, although there are no clear-cut defining features. The middle interhemispheric variant (MIH), also known as syntelencephaly, is classified as a variant of HPE holoprosencephaly with midline interhemispheric fusion. Other conditions sometimes included in the spectrum of HPE holoprosencephaly include septo-optic dysplasia (SOD); "minimal" HPE holoprosencephaly , which is associated with subtle craniofacial malformations and mild developmental delay; and microform HPE holoprosencephaly , which by definition excludes brain involvement. The focus of this article will be on the spectrum of findings visible in fetal manifestation of the HPE holoprosencephaly spectrum. Brain embryology; the imaging characteristics, epidemiology, and embryology of HPE; and the more common associated anomalies, particularly those of the face ("the face predicts the brain") are reviewed. Recognition of these anomalies is important for accurate parental counseling, since the prognosis is poor but not invariably lethal; children with the milder forms may live well into their teens with severe developmental delays, endocrine dysfunction, and disrupted homeostasis. Available data on outcome in surviving children are summarized. Illustrative fetal ultrasonographic and magnetic resonance images are presented with clinical, autopsy, and postnatal imaging correlation.

Syntelencephaly, a lesser known variant of holoprosencephal

Holoprosencephaly has been traditionally classified in to three types: alobar, semi lobar and lobar forms. A lesser known variant of holoprosencephaly, known as Midline Interhemispheric Fusion (MIH) variant was described in 1993. We present a case of MIH along with a review of imaging findings and review of literature, correlating the differences in presentation with the underlying differences in neuroanatomy.

Neuropathologic research strategies in holoprosencephaly.

Hypotheses are presented to explain the pathogenesis of several clinical features of holoprosencephaly, and neuropathologic approaches to testing these hypotheses are suggested. The traditional morphologic classification of holoprosencephaly into alobar, semilobar, and lobar forms is grades of severity, and each occurs in all of the genetic mutations known. Of the four defective genes identified as primary in human holoprosencephaly, three exhibit a ventrodorsal gradient of expression (SHH, SIX3, and TGIF) and one a dorsoventral gradient (ZIC2). But, in addition to the vertical axis, genes expressed in the neural tube also may have rostrocaudal and mediolateral gradients in the other axes. These other gradients may be equally as important as the vertical. If the rostrocaudal gradient extends as far as the mesencephalic neuromere, it may interfere with the formation, migration, or apoptosis of the mesencephalic neural crest, which forms membranous bones of the face, orbits, nose, and parts of the eyes, and may explain the midfacial hypoplasia seen in many, but not all, children with holoprosencephaly. This rostrocaudal gradient also causes noncleavage of the caudate nucleus, thalamus, and hypothalamus and contributes to the formation of the dorsal cyst of holoprosencephaly, which is probably derived from an expanded suprapineal recess of the 3rd ventricle with secondary dilation of the telencephalic monoventricle and at times may produce a unique transfontanellar encephalocele. The extent of the mediolateral gradient may explain the severe disorganization of cerebral cortical architecture in medial parts of the forebrain and normal cortex in lateral parts, including the radial glial fibers. This preserved lateral cortex may explain why some children with holoprosencephaly have better intellectual function than expected and may also be important in the pathogenesis of epilepsy, by contrast with malformations such as lissencephaly, in which the entire cerebral cortex is involved. Epilepsy in some, but not all, cases also may be related to the sequential maturation of axonal terminals in relation to the neurons they innervate. Diabetes insipidus is a complication in a majority of patients; other neuroendocrinopathies occur less frequently. Secondary down-regulation of the OTP gene or of downstream genes such as BRN2 or SIM1 may result in failure of terminal differentiation of magnocellular neurons of the supraoptic and paraventricular hypothalamic nuclei. Disoriented radial glial fibers or abnormal ependyma may allow aberrant migration of neuroepithelial cells into the ventricle. A new classification of holoprosencephaly is needed to integrate morphologic and genetic criteria.

Holoprosencephaly (Lobar Form) Associated With Bilateral Vocal Cord Palsy

There have been many causes associated with bilateral vocal cord palsy, both congenital and perinatal. Until now, the main congenital causes of bilateral vocal cord palsy have been associated with meningomyelocele, Arnold-Chiari malformation, and hydrocephalus. We report a patient with bilateral vocal cord palsy associated with the lobar form of holoprosencephaly. In this case, bilateral vocal cord palsy was caused by the disruption of the cortical laryngeal motoneurons. Neonatal stridor must be carefully evaluated by the neonatologist to exclude severe cerebral anomalies.

The CT angiogram sign.

The CT angiogram sign has been described as a finding in the lobar form of bronchoalveolar cell carcinoma (1). Bronchoalveolar cell carcinoma represents a subgroup of adenocarcinoma and accounts for approximately 5% of all bronchogenic carcinomas in most series, although a recent increase in its prevalence to as high as 20.3% has been reported (2). Histopathologically, bronchoalveolar cell carcinoma can be separated into two basic morphologic types—mucinous and Clara cell or type II pneumocyte—and two spread pattern types—tumors with aerogenous spread and those without aerogenous spread. The mucinous tumors account for approximately 20%–30% of all bronchoalveolar cell carcinomas (3,4) and are characterized by tall, mucus-filled columnar cells. The mucinous tumors tend to spread aerogenously, infiltrating along the preexisting normal framework of the lung (ie, lepidic growth). Abundant secretion of mucin positive for periodic acid–Schiff reagent that fills the alveoli and small airways is more characteristic of the mucinous tumors; however, minimal amounts of this mucin can be seen in the nonmucinous tumors. Four clinical manifestations have been reported: (a) a single nodule, (b) multiple nodules, (c) a single consolidation, and (d) multiple lobar consolidations. Single and multiple lobar consolidations are more common in the mucinous tumors. Bronchoalveolar cell carcinoma in a patient with alveolar consolidation remains difficult to diagnose clinically and radiologically. Bronchorrhea, with up to liters of mucus discharged per day in some cases, was once considered a standard clinical finding; however, it is seen in a minority of patients. Bronchorrhea was found in 5% of the patients with bronchoalveolar cell carcinoma examined by Miller et al in 1978 (5). Some patients present for radiologic evaluation of a nonresolving alveolar consolidation that was initially diagnosed as pneumonia. Without underlying causes of hemorrhage, edema, or infection, persistent alveolar consolidation is suggestive of bronchoalveolar cell carcinoma. An incidental asymptomatic mass is another common manifestation. The CT angiogram sign was initially described as a specific sign of lobar bronchoalveolar cell carcinoma; specificity can be as high as 92.3% (1). Recently, the results of several retrospective studies have challenged the specificity of the CT angiogram sign; the CT angiogram sign was reported to be seen in both benign and malignant entities, including bronchoalveolar cell carcinoma, pneumonia, pulmonary edema, obstructive pneumonitis due to central lung tumors, lymphoma, and metastasis from gastrointestinal carcinomas (6–14). In cases of obstructive pneumonitis and primary pulmonary lymphoma, investigators have suggested that it is the relative difference between the attenuation of the pulmonary vessels

Vibration of Hemi-Ellipsoidal Axisymmetric Domes Submerged in Water

Ten thin-walled domes, of hemi-ellipsoidal form, were vibrated in air and while partially and fully submerged in water. The domes varied in shape from oblate ones of aspect ratio (AR) 0.25 to prolate ones of aspect ratio 4. The fundamental modal patterns for the oblate domes tended to be of axisymmetric form, while the fundamental modal patterns of the hemispherical and prolate domes tended to be of asymmetric or lobar form. The theoretical analysis was carried out by the finite element method, where the motion of the shell structure was represented by three different types of element and the motion of the water was represented by a solid annular element. Comparison between theory and experiment was found to be good.

Investigations on the Pneumonia Diagnosis in early Childhood with special Reference to Serotherapy

SummaryIn order to be able to evaluate tbe influence of serum on pneumonia in very young‐ children it is necessary to know from what form of pneumonia the child under consideration suffers, as the different forms of bronchopneumonia have different prognoses.On comparison of the X‐ray with the clinical picture (Fig. I) the bronchopneumonias can be divided into 2 main groups: (I) Primitive pneumonia: (a) hilufugal, b) miliary, c) primary abscess formation). This form is characterized by a great tendency of dissemination in the lung‐ field issuing from the hilus, following the configuration of vessels and bronchi fan‐like outward towards the periphery. (II) The transitionary form: (a) the focal, b) the marginal, c) the partly lobar, and d) the lobar form). It is characterized by a tendency to focal limitation of the process located to different places in the lung. The tendency to form a focus is considered an expression of an allergic defensive process.Fig. I shows that the primitive forms are most frequently met with in children below 2 years of age, that the focal transitionary form is most frequent in children of from 2—4 years, and that the lobar and cronpous forms are most frequent in children above 4 years.

THE STREPTOCOCCUS EPIDEMIC AT CAMP ZACHARY TAYLOR, KY.

It was expected that the acute respiratory diseases would share with meningitis the responsibility for the morbidity and mortality of the Army camps. It was, however, the pneumococcus from which it was thought the greatest number of infections would come, so that the history of the acute infections of Camp Zachary Taylor was as surprising as it was interesting. Pneumonia in its lobar form, due to the pneumococcus, appeared early in the autumn months and has continued at the rate of about fifty cases a month ever since, a summary of which is given in the accompanying tabulation. The field was soon occupied by the streptococcus, which did not confine itself to the respiratory tract, but extended its malign influence to practically every tissue of the body, appearing, indeed, in the form of an epidemic infective agent. Chronologically, measles appeared after the pneumococcic pneumonias, typical in character, moderate in severity

CLINICAL LECTURE on BRONCHO-PNEUMONIA, to Show that Pneumococcal Pneumonia in a Child takes the Lobular and not the Lobar Form: Given at St. Bartholomew's Hospital

CLINICAL LECTURE on BRONCHO-PNEUMONIA, to Show that Pneumococcal Pneumonia in a Child takes the Lobular and not the Lobar Form: Given at St. Bartholomew's Hospital