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PubMed2001

[A high aged case of herpes simplex viral encephalitis associated with progressive cerebral white matter lesion].

An 80-year-old male without abnormal past medical history presented with coma, general seizures, and fever subsequent to abnormal behavior. The pressure of the cerebrospinal fluid(CSF) elevated(13.5-20.5 cm H2O), and CSF examination revealed pleocytosis with predominant mononuclear cells(80-879/mm3) and elevated protein level(32-130 mg/dl). DNAs of herpes simplex virus(HSV) type 1 and 2 in CSF were not confirmed by polymerase chain reaction method in the acute phase. The HSV(type 1) antibody(HSV-1 Ab) ratio of serum to CSF(= [serum HSV-1 Ab]/[CSF HSV-1 Ab]) was 0.98 and HSV-1 Ab index(= [CSF HSV-1 Ab]/[serum HSV-1 Ab] divided by [CSF albumin]/[serum albumin]) was 62.4. Initial fluid attenuated inversion recovery(FLAIR) (TR/TE/TI = 6,882/110/1,700 msec) axial magnetic resonance(MR) imaging showed hyperintensity in the subfrontal area, inferomedial portions of the temporal lobes, cingulate gyri, and insular cortices bilaterally. Meningoencephalitis caused by HSV-1 was diagnosed based on the values of HSV-1 Ab ratio of serum to CSF(less than 20), of HSV-1 Ab index(larger than 1.91), and the findings of MR imaging. Diffuse white matter lesions manifesting hyperintensity on FLAIR imaging in the bilateral frontal and temporal lobes close to the affected cortices developed approximately six weeks after the onset despite administration of antiviral agent and steroid. The lesion extensively involved the white matter of the bilateral frontal and temporal lobes finally. The initial value of myelin basic protein(MBP) in CSF was 0.9 ng/ml (normal value: less than 4 ng/ml). Subsequent measurement of MBP in CSF about two, six weeks, two, three, and six months after the onset showed a marked increase of 233.9 ng/ml followed by a gradual decrease of 25.4 ng/ml, 18.4 ng/ml, 7.4 ng/ml and 4.3 ng/ml, respectively. Therefore, demyelination of the lesion in the cerebral white matter was suggested by the chronological change in FLAIR imaging and MBP in CSF.

Aged↗
PubMed2002

[Growth and morphogenesis of the craniofacial bones. Applications in orthodontics. The concepts of J. Delaire].

The present report about J. Delaire's lecture summarizes his approach to: the nature and physiology of the membranous sutures, the "neural" and "adaptive" growth of the cranial base, the "central" and "cortical" development of the maxillary, the role of the premaxillary "skeletal entity" in the development of the anterior part of the face, the "bifocal" development of the mandible, the architectural balance of the craniofacial set (leading to the author's cephalometric analysis).

Cephalometry↗
PubMed2002

Schizophrenia and bipolar disorder: similarities in pathogenic mechanisms but differences in neurodevelopment.

Over the past 100 years, the Kraepelinian classification of psychoses has dominated our approach to schizophrenia and bipolar disorder. However, controversy as to the nature of the illnesses--whether they can be viewed as completely distinct, essentially the same, or occupying different points along a psychosis spectrum--has intensified in recent years. This paper reviews the evidence for these differing opinions, examining both the commonalities between the two diseases and the distinctions. A genetic propensity towards psychotic disorders is widely acknowledged; more recent studies suggest a considerable overlap in genetic susceptibility to schizophrenia and bipolar disorder. The influence of early environmental effects, such as obstetric complications, on schizophrenia is also established but little such evidence exists for bipolar disorder. Structural abnormalities of the brain of developmental origin as well as neuropsychological deficits have been clearly identified in schizophrenia but less evidence has been found in bipolar disorder. The most plausible explanation is that one or more susceptible genes are shared between schizophrenia and bipolar illness, and can be thought of as predisposing individuals to psychosis, perhaps by producing a dysregulation of the dopaminergic response to stress. Other genes and environmental factors are likely to have more specific effects and contribute to producing the patterns that psychiatrists recognize as 'classical' schizophrenia and mania. In particular, genes involved in early cortical development and early neurodevelopmental insults causing developmental impairment may put individuals on a trajectory towards schizophrenia rather than bipolar illness.

Adult↗
PubMed2003

[Brain function peculiarities in premature babies and infants with perinatal encephalopathy and cerebral palsy].

Eighty five children with perinatal encephalopathy and cerebral palsy have been studied. EEG analysis showed functional imbalances of cortical and subcortical structures. A delay of cortical electrogenesis development and cortical functional activity were reduced, particularly in its sensomotor areas and left hemisphere with insufficient efferent impulse flow into underlying structures and predominance of electrical subcortical and stem structure activity. The changes discovered may relate directly to psychomotor retardation.

Brain↗
PubMed2004

MR imaging and spectroscopic study of epileptogenic hypothalamic hamartomas: analysis of 72 cases.

BACKGROUND AND PURPOSE: Reports of MR imaging in hypothalamic hamartomas associated with epilepsy are few, and the number of patients studied is small. We aimed to detail the relationship of hypothalamic hamartomas to surrounding structures, to determine the frequency and nature of associated abnormalities, and to gain insight into mechanisms of epileptogenesis. METHODS: We systematically examined MR imaging studies of 72 patients with hypothalamic hamartoma and refractory epilepsy (patient age, 22 months to 31 years). A dedicated imaging protocol was used in 38 cases. Proton MR spectroscopy of the hypothalamic hamartoma was performed for 19 patients and compared with the metabolite profile of the thalamus in 10 normal children and the frontal lobe in 10 normal adults. RESULTS: Compared with normal gray matter, hypothalamic hamartomas were hyperintense on T2-weighted images (93%), hypointense on T1-weighted images (74%), and had reduced N-acetylaspartate and increased myoinositol content shown by MR spectroscopy. Hypothalamic hamartomas always involved the mammillary region of the hypothalamus, with attachment to one or both mammillary bodies. Intrahypothalamic extension (noted in 97%) tended to displace the postcommissural fornix and hypothalamic gray matter anterolaterally, such that the hypothalamic hamartomas nestled between the fornix, the mammillary body, and the mammillothalamic tract. Larger hamartoma size was associated with central precocious puberty. Associated findings of questionable epileptic significance included anterior temporal white matter signal intensity abnormalities (16%) and arachnoid cysts (6%). Malformations of cortical development were observed in only two patients, and hippocampal sclerosis was not observed. CONCLUSIONS: Hypothalamic hamartomas can be readily distinguished from normal hypothalamic gray and adjacent myelinated fiber tracts, best appreciated on thin T2-weighted images. MR imaging and spectroscopy suggest reduced neuronal density and relative gliosis compared with normal gray matter. Associated epileptogenic lesions are rare, supporting the view that the hypothalamic hamartoma alone is responsible for the typical clinical features of the syndrome. The intimate relationship to the mammillary body, fornix, and mammillothalamic tract suggests a role for these structures in epileptogenesis associated with hypothalamic hamartomas.

Adolescent↗
PubMed2004

[Diagnosis of epilepsy].

0.5-1% of the population suffers from epilepsy, while another 5% undergoes diagnostic evaluations due to the possibility of epilepsy. In the case of suspected epileptic seizures we face the following questions: Is it an epileptic seizure? The main and most frequent differential-diagnostic problems are the psychogenic non-epileptic seizures ("pseudo-seizures") and the convulsive syncope, which is often caused by heart disorders. Is it epilepsy? After an unprovoked seizure, the information on recurrence risk is an important question. The reoccurrence is more possible if a known etiological factor is present or the EEG shows epileptiform discharges. After an isolated epileptic seizure, the EEG is specific to epilepsy in 30-50% of cases. The EEG should take place within 24 hours postictally. If the EEG shows no epileptiform potentials, a sleep-EEG is required. What is the cause of seizures? Hippocampal sclerosis, benign tumors, and malformations of the cortical development are the most frequent causes of the focal epilepsy. Three potentially life-threatening conditions may cause chronic epilepsy: vascular malformations, tumors, and neuroinfections. The diagnosis in theses cases can usually be achieved by MRI, therefore, MRI is obligatory in all epilepsies starting in adulthood. The presence of epileptogenic lesion has a prognostic significance in treatment. If the MRI shows a circumscribed lesion then the pharmacological treatment will likely to be unsuccessful, while surgery may result in seizure freedom. The new and quantitative MRI techniques, such as volumetry, T2-relaxometry, MR-spectroscopy, and functional MRI play a growing role in the epilepsy diagnosis.

Brain Neoplasms↗
PubMed2005

Temporal lobe epilepsy in children: overview of clinical semiology.

Temporal lobe epilepsy in children has been less well studied compared to that seen in adults. Whereas hippocampal sclerosis is the most common etiology for the adult temporal lobe epilepsy syndromes, tumors and malformations of cortical development are more commonly seen in children. Differences in seizure semiology are also apparent. Temporal lobe epilepsy in very young infants may exhibit prominent motor manifestations reminiscent of extra-temporal seizures. These motor manifestations however decrease with increasing age and are less abundant in adults. Automatisms, which are commonly seen in temporal lobe epilepsy in all age groups, are simple at a younger age and become increasingly more complex and discrete with age. Several case studies, illustrated on the video are included in this review. They highlight the differences in temporal lobe seizure semiology between children and adults. [Published with video sequences].

Adolescent↗
PubMedDate not supplied

MR findings in acute and chronic coccidioidomycosis meningitis.

PURPOSE: To characterize MR findings in acute and chronic coccidioidomycosis meningitis and relate the imaging features to the clinical course. METHODS: We reviewed MR scans and clinical findings of 12 patients with coccidioidal meningitis. RESULTS: Patients with active or untreated disease were found to have hydrocephalus and intense enhancement of the cervical subarachnoid space, basilar, sylvian, and interhemispheric cisterns on postcontrast MR scans. Focal parenchymal signal abnormalities suggesting ischemia or infarction were common. Abnormal MR enhancement decreases during therapy, although patients develop cortical and/or brain stem atrophy. CONCLUSIONS: Widespread cisternal and cervical subarachnoid meningeal involvement is common in coccidioidal meningitis. Serial contrast MR imaging reflects the effects of therapy in patients with coccidioidal meningitis.

Acute Disease↗
PubMed2006

Status epilepticus in pediatric practice: neonate to adolescent.

SE in the pediatric age group presents challenges in diagnosis and management. There is need for renewed consensus on the temporal definition of SE, both clinical and electrographic. SE in children exhibits an age-dependent vulnerability, with genetic predisposition and etiology as determinants of susceptibility. Nonepileptic phenomena may mimic SE. Clinical and electrographic SE in neonates are relatively rare, while serial (clinical and electrographic) and repetitive seizures are more common. Neurometabolic disease, chromosomal disorders, and abnormalities of cortical development are important etiological considerations. Abrupt discontinuation of or an aberrant response to AEDs can also precipitate SE. Metabolic perturbations and toxins can further aggravate the situation. Clinical and experimental data suggest that the longer a seizure lasts, the more difficult it becomes to control, and that seizures can have immediate and long-term adverse consequences on the immature and developing brain. Hence, treatment (usually with a benzodiazepine) should be started early in the clinical course. A trial of pyridoxine, biotin, or folinic acid should be considered in the appropriate clinical setting (e.g., neonates or young infants, in particular). Phenytoin/fosphenytoin and phenobarbital remain important treatment options. Pentobarbital and midazolam are preferred choices in the management of RSE. Once metabolic causes are excluded, children with RSE should be evaluated for surgical treatment early in the clinical course. Clinical guidelines based on best available evidence have to be periodically reviewed. The clinical consequences and management of electrographic SE, especially in the neonate, have to be addressed. Guidelines for continuous (video) EEG monitoring are needed to facilitate this task. AEDs that do not have an adverse effect on the developing brain have to be developed. Our review suggests a continuing need for prospective studies into all aspects of SE in the pediatric age group.

Adolescent↗
PubMed2006

How depth of anesthesia influences the blood oxygenation level-dependent signal from the visual cortex of children.

BACKGROUND AND PURPOSE: Functional MR imaging (fMRI) is playing an important role in investigations of cortical development and maturation. Functional MR imaging in young children or infants frequently involves measuring a clinical population under sedation or anesthesia. We examined the effect of depth of anesthesia on the extent and amplitude of the blood oxygen level-dependent (BOLD) response. METHOD: We performed BOLD-based fMRI on a visual stimulus flickering at 8 Hz at sevoflurane concentrations of 0.5 minimum alveolar concentration (MAC), 0.75 MAC, and 1.0 MAC, on 16 children at least 5 years of age. We determined the extent of activation by counting the number of activated voxels and assessed the change in the local deoxyhemoglobin concentration by comparing DeltaR2*. RESULTS: The number of activated voxels of the positive BOLD response was higher at 0.75 MAC than at 0.5 MAC or 1.0 MAC. The magnitude of their mean DeltaR2* steadily declined as the level of sevoflurane was increased from 0.5 MAC to 1.0 MAC. The extent of activation of the negative BOLD response declined progressively from 0.5 MAC to 1.0 MAC. The magnitude of their mean amplitude of the DeltaR2* did not change with sevoflurane concentrations. The change in the extent of activation and the magnitude of DeltaR2* when the concentration of sevoflurane increased from 0.5 MAC to 0.75 MAC was due to its vasodilative property. The change in the extent of activation and the amplitude of DeltaR2* following the increase in the concentration of sevoflurane from 0.75 MAC and 1.0 MAC was due to its anesthetic property. This was the case for both the positive and negative BOLD response. CONCLUSIONS: Careful adjustment of anesthetic depth can be used advantageously when performing BOLD-based fMRI measurements in children.

Adolescent↗
PubMed2006

Status epilepticus in a patient with fragile X syndrome: electro-clinical features and peri-ictal neuroimaging.

Fragile X syndrome (Fra-X) is a common cause of mental retardation that can be associated with partial epilepsy characterized by a variety of electro-clinical features. A wide spectrum of interictal activities are reported, although no data regarding ictal EEG activity have as yet been published. Drug-resistant seizures are uncommon, and the occurrence of clustering seizures or status epilepticus has only been reported anecdotally. We describe a Fra-X patient with refractory partial epilepsy related to a malformation of cortical development who experienced a partial status epilepticus that was well documented by video-EEG and MRI. We report the electro-clinical features and peri-ictal neuroimaging data.

Adolescent↗
PubMed1991

Distribution and fine structural analysis of undifferentiated cells in the primate subependymal layer.

The subependymal layer (SEL) of the postnatal marmoset, a simian primate, has been investigated by histological and electron microscopic techniques. Although well documented in rodents, little is known about this layer in primates. The distribution of the SEL in marmosets is most extensive at birth around the anterior lateral ventricles, where the layer is generally 5-10 cells deep; however, there is considerable regional variation. With age the size of the SEL decreases dramatically, becoming very poorly demarcated in adult animals. Ultrastructurally, many subependymal cells in neonatal and young brains display the features of undifferentiated cells, although neurons and glia are also present. Cells displaying features intermediate between astrocytes and undifferentiated SEL cells are occasionally encountered. In adults undifferentiated cells are seen rarely and the former SEL is composed primarily of glial and neuronal processes. Thus the layer in primates probably represents a site of continued cellular differentiation in the postnatal brain and as such must play an important role in the final stages of cortical development.

Aging↗
PubMedDate not supplied

[Handedness, cerebral dysfunction and dyslexia].

A frequency distribution of handedness and its relations with minor signs of neurological dysfunction was studied in a group of 56 dyslexic children and 56 good readers. An unusual frequency of 18% left-handed children in the dyslexic group and the high frequency of soft signs in this lefthanded children, is in concordance with recent hypothesis about the dysgenesic brain origin of dyslexia and lefthandedness, postulated by Galaburda, Geschwind and others. They suggest a distorted cortical development of the brain areas related with linguistic functions, with subsequent expression on verbal language reading ability and handedness.

Brain↗
PubMedDate not supplied

Local blood-brain barrier penetration following systemic contrast medium administration. A case report and an experimental study.

The present study was initiated by a severe complication in a patient with renal dysfunction who developed cortical blindness and weakness of her left extremities 30 hours following renal and abdominal angiography. To evaluate the impact of prolonged high serum concentrations of contrast medium (CM) this clinical situation was simulated in a laboratory model using sheep with elevated serum levels of contrast medium maintained for 48 hours. The experimental data did not support the theory that the prolonged exposure to high circulating levels of contrast medium (4 ml/kg body weight of meglumine diatrizoate 60%) is sufficient alone to cause penetration of the blood-brain barrier.

Aged↗
PubMedDate not supplied

Malignant migraine: the syndrome of prolonged classical migraine, epilepsia partialis continua, and repeated strokes; a clinically characteristic disorder probably due to mitochondrial encephalopathy.

We present ten patients with classical or common migraine of increasing severity accompanied by seizures of multiple patterns and increasing severity leading to episodes of epilepsia partialis continua. Long lasting deficits associated with hypodense lesions on CT and abnormal signals on MRI, then developed. Cortical blindness, cortical deafness and dementia were common. Five of the patients died in 1-10 years. Some of these patients had markers of mitochondrial disease (ragged red fibers and serum lactate elevation) and others with the same clinical picture did not. This group of patients indicates that mitochondrial encephalopathy may exist without evidence of myopathy, that the clinical syndrome is characteristic and that it should suggest the diagnosis even in the absence of muscular abnormalities.

Adolescent↗
PubMedDate not supplied

Thymus histogenesis in C3H mice.

The histogenesis of the mouse thymus was studied by means of light and electron microscopy in an attempt to clarify the simultaneous development of stromal and lymphoid cell populations. On the twelfth day of embryonic life, the thymus primordium was composed principally of undifferentiated epithelial cells and some lymphoblasts. In the developing cortical regions, lymphoblasts accumulated rapidly, stretching the epithelial cells which became stellate in shape. The latter contained multivesicular bodies and, from the sixteenth day on, also typical clear vacuoles. Medullary regions were prefigurated as soon as day 13 by several areas wherein lymphoblasts were sparse and epithelial cells were closely associated, with numerous desmosomes and abundant tonofilaments. On the sixteenth day, some epithelial cells in these regions were differentiated into globular cells, or formed Hassall's corpuscles and intra- or extracellular cysts. On the seventeenth day, the presence of interdigitating cells in the medullary areas completed cortico-medullary differentiation. On the eighteenth day, small cortical thymocytes differentiated and the thymus possessed all characteristics of an adult thymus. Thus, at birth, the histogenesis of the mouse thymus was achieved and the only further modification consisted in a gain of weight.

Age Factors↗
PubMed1987

[Aneurysms of the infrarenal aorta and visceral cancer. Therapeutic problems].

The association of an abdominal aortic aneurysm (AAA) and a long-standing or progressing cancer is a frequent finding: 14 cases among the 112 infrarenal aortic aneurysms treated by one of us (J.C.) are discussed in this report. The marked predominance in bronchial and ORL epithelioma (50%) is explained by the common pathogenic factors of these neoplasms and atheroma. Surgical treatment is difficult because of the potentially lethal character of the two lesions: it must allow for size and possible progressive nature of aneurysm and prognosis of the neoplasm as defined by the TNM classification. Detection of an AAA in a patient with a history of neoplasm means that the opportunity for aortic surgery is dependent of therapeutic control (or otherwise) of the neoplastic disease and therefore frequently the length of follow up period after therapy. When detection of the AAA and neoplasm is simultaneous, the aneurysm progressing or ruptured, surgical complications leave little choice with regard to operative strategy. In 3 cases, simultaneous treatment of an AAA and a neoplasm was possible, particularly in the case of Grawitz tumors of cortical development without pyelocaliceal invasion. In most patients, separate operative stages are necessary in order to ensure asepsis of AAA surgery. Aneurysmal occlusion with an extrafocal shunt can allow one-stage surgery when aneurysm and neoplasm are equally menacing.

Aged↗
PubMed1986

Neuronal types of the cerebral cortex of the adult chicken (Gallus gallus). A Golgi study.

The telencephalic cortex of adult chicken (Gallus gallus) consists of two anatomical regions: the medial cortex (or hippocampal cortex) and the dorsal cortex (or parahippocampal cortex) which we have denominated "dorsomedial cortex" (DMC) as a whole. The dorsomedial cortex shows, with Nissl's technique, two main layers of lamination, besides the ependymal layer: plexiform layer and granular layer. In the plexiform layer, there is a sub-layer called periventricular. In Golgi impregnations, the plexiform layer of the DMC is filled with neurons of horizontal type and fibers. The granular layer of the medial region is mainly occupied with pyramidal and bipyramidal neurons, as well as some short-axon and long-axon multipolar neurons. In the granular layer of the dorsal region basically long-axon multipolar neurons are found and, to a lesser extend, short-axon multipolar ones are also seen. In the periventricular sub-layer of all the DMC are located the periventricular neurons. In general, we can say that the chicken DMC is a poorly developed cortical structure.

Animals↗