scholarly journals Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics

2013 ◽  
Vol 34 (6) ◽  
pp. E6 ◽  
Author(s):  
Sandeep Mittal ◽  
Monika Mittal ◽  
José Luis Montes ◽  
Jean-Pierre Farmer ◽  
Frederick Andermann
Keyword(s):  
Molecular Biology ◽  
Clinical Features ◽  
Clinical Course ◽  
Cognitive Behavioral ◽  
Gelastic Seizures ◽  
The Past ◽  
Hypothalamic Hamartomas ◽  
Epileptic Syndrome ◽  
Psychiatric Disturbances ◽  
Developmental Malformations

Hypothalamic hamartomas are uncommon but well-recognized developmental malformations that are classically associated with gelastic seizures and other refractory seizure types. The clinical course is often progressive and, in addition to the catastrophic epileptic syndrome, patients commonly exhibit debilitating cognitive, behavioral, and psychiatric disturbances. Over the past decade, investigators have gained considerable knowledge into the pathobiological and neurophysiological properties of these rare lesions. In this review, the authors examine the causes and molecular biology of hypothalamic hamartomas as well as the principal clinical features, neuroimaging findings, and electrophysiological characteristics. The diverse surgical modalities and strategies used to manage these difficult lesions are outlined in the second article of this 2-part review.

scholarly journals Hypothalamic hamartomas. Part 2. Surgical considerations and outcome

2013 ◽  
Vol 34 (6) ◽  
pp. E7 ◽  
Author(s):  
Sandeep Mittal ◽  
Monika Mittal ◽  
José Luis Montes ◽  
Jean-Pierre Farmer ◽  
Frederick Andermann
Keyword(s):  
Clinical Syndrome ◽  
Gelastic Seizures ◽  
Considerable Effort ◽  
Behavioral Outcome ◽  
Mammillary Bodies ◽  
The Past ◽  
Hypothalamic Hamartomas ◽  
Refractory Seizures ◽  
Hallmark Feature ◽  
Neurosurgical Techniques

Hypothalamic hamartomas are uncommon developmental heterotopic masses composed of an intermixed array of neurons, glia, and myelinated fibers closely associated with the mammillary bodies. Gelastic seizures, the hallmark feature of hypothalamic hamartomas, commonly present in early childhood. However, patients usually also display a disabling clinical syndrome, which may include various other types of refractory seizures with secondary generalization together with progressive cognitive, behavioral, and psychiatric dysfunction. The hamartoma itself has been unequivocally shown to be intrinsically epileptogenic. Over the past 2 decades there has been considerable effort to develop neurosurgical techniques to treat the epileptic syndrome effectively as well as to improve the neurocognitive and behavioral outcome.

scholarly journals Pediatric COVID-19 and the Factors That May Mitigate Its Clinical Course

2020 ◽  
Vol 10 (01) ◽  
pp. e137-e140
Author(s):  
Mosaad Abdel-Aziz ◽  
Nada M. Abdel-Aziz ◽  
Dina M. Abdel-Aziz ◽  
Noha Azab
Keyword(s):  
Clinical Features ◽  
Clinical Symptoms ◽  
Clinical Course ◽  
Clinical Manifestations ◽  
Gastrointestinal Symptoms ◽  
Angiotensin Converting Enzyme 2 ◽  
Compulsory Vaccination ◽  
Specific Factors ◽  
Novel Coronavirus ◽  
Radiographic Data

AbstractThe clinical manifestations of novel coronavirus disease 2019 (COVID-19) vary from mild flu-like symptoms to severe fatal pneumonia. However, children with COVID-19 may be asymptomatic or may have mild clinical symptoms. The aim of this study was to investigate clinical features of pediatric COVID-19 and to search for the factors that may mitigate the disease course. We reviewed the literature to realize the clinical features, laboratory, and radiographic data that may be diagnostic for COVID-19 among children. Also, we studied the factors that may affect the clinical course of the disease. Fever, dry cough, and fatigue are the main symptoms of pediatric COVID-19, sometimes flu-like symptoms and/or gastrointestinal symptoms may be present. Although some infected children may be asymptomatic, a recent unusual hyperinflammatory reaction with overlapping features of Kawasaki's disease and toxic shock syndrome in pediatric COVID-19 has been occasionally reported. Severe acute respiratory syndrome-coronvirus-2 (SARS-CoV-2) nucleic acid testing is the corner-stone method for the diagnosis of COVID-19. Lymphocyte count and other inflammatory markers are not essentially diagnostic; however, chest computed tomography is highly specific. Factors that may mitigate the severity of pediatric COVID-19 are home confinement with limited children activity, trained immunity caused by compulsory vaccination, the response of the angiotensin-converting enzyme 2 receptors in children is not the same as in adults, and that children are less likely to have comorbidities. As infected children may be asymptomatic or may have only mild respiratory and/or gastrointestinal symptoms that might be missed, all children for families who have a member diagnosed with COVID-19 should be investigated.

Autonomic and hormonal ictal changes in gelastic seizures from hypothalamic hamartomas

1998 ◽  
Vol 107 (5) ◽  
pp. 317-322 ◽  
Author(s):  
A Cerullo ◽  
P Tinuper ◽  
F Provini ◽  
M Contin ◽  
A Rosati ◽  
...  
Keyword(s):  
Gelastic Seizures ◽  
Hypothalamic Hamartomas

Varicella and Cortisone

PEDIATRICS ◽  
1956 ◽  
Vol 18 (1) ◽  
pp. 160-162 ◽  
Author(s):  
ROBERT J. HAGGERTY ◽  
R. CANNON ELEY
Keyword(s):  
Infectious Diseases ◽  
Clinical Course ◽  
Medical Center ◽  
Post Mortem ◽  
The Past ◽  
Vesicle Fluid ◽  
Heart Blood ◽  
Cortisone Therapy ◽  
Relationship Of

In the past 2 years we have seen 2 patients at the Children's Medical Center, who, while receiving cortisone therapy, developed varicella and died following a state of shock within a few days after the appearance of the vesicles. Post-mortem examinations of these patients revealed hemorrhagic vesicles throughout all viscera. In 1 case the virus of varicella was grown from vesicle fluid, heart blood, and lung. Since fatalities from varicella in childhood are so rarely encountered, even by physicians with vast experience in the field of infectious diseases, and since the clinical course of the infection in these two patients was so fulminating, the possible relationship of cortisone administration was raised.

scholarly journals Features of the clinical course of cardiovascular pathology on the background of thyrotoxicosis

2019 ◽  
Keyword(s):  
Clinical Course ◽  
Endemic Goiter ◽  
The Elderly ◽  
Early Complications ◽  
Cardiovascular Pathology ◽  
The Past ◽  
Cardiac Symptoms ◽  
Long Time ◽  
Frequent Absence

Relevance of the topicofthyroid diseases is duetothein crease in their number in the world and in Ukraine inparticular over the past 5 years, as well as the fact that the total frequency of various forms of thyroid pathology, even outside the zones of endemic goiter, is about 20% of the total morbidity. On the example of a clinical case, the question of diagnostics, management tactics and features of the clinical course of cardiovascular pathology on the background of thyrotoxicosis in the absence of adequate therapy for a long time are considered. Thyrotoxicosis leads to the occurrence of early complications of the cardiovascular system, which determine the further prognosis of the patient’s quality of life. The heart effects of Thyrotoxicosisare most dangerous for the elderly, and they (the heart effects of Thyrotoxicosis) often dominate the clinical picture in them (the elderly). Difficulties in their diagnosis are due, among other things, to the frequent absence of ophthalmopathy. When atrial fibrillation develops, the presence of cardiac symptoms may increase, indicating an accelerated development of heart failure. Therefore, the management of patients with cardiovascular disease on the background of thyrotoxicosis has its own peculiarities, which should be taken into account during treatment.

scholarly journals Modelling reaction kinetics inside cells

2008 ◽  
Vol 45 ◽  
pp. 41-56 ◽  
Author(s):  
Ramon Grima ◽  
Santiago Schnell
Keyword(s):  
Molecular Biology ◽  
Reaction Kinetics ◽  
Single Molecule ◽  
Predictive Accuracy ◽  
Reaction Pathway ◽  
Imaging Techniques ◽  
Deterministic Models ◽  
Non Invasive ◽  
The Past ◽  
Fundamental Length

In the past decade, advances in molecular biology such as the development of non-invasive single molecule imaging techniques have given us a window into the intricate biochemical activities that occur inside cells. In this chapter we review four distinct theoretical and simulation frameworks: (i) non-spatial and deterministic, (ii) spatial and deterministic, (iii) non-spatial and stochastic and (iv) spatial and stochastic. Each framework can be suited to modelling and interpreting intracellular reaction kinetics. By estimating the fundamental length scales, one can roughly determine which models are best suited for the particular reaction pathway under study. We discuss differences in prediction between the four modelling methodologies. In particular we show that taking into account noise and space does not simply add quantitative predictive accuracy but may also lead to qualitatively different physiological predictions, unaccounted for by classical deterministic models.

Clinical features of familial juvenile cases of moyamoya disease: analysis of patients treated in a single institute over a 28-year period

2013 ◽  
Vol 12 (2) ◽  
pp. 175-180 ◽  
Author(s):  
Maki Mukawa ◽  
Tadashi Nariai ◽  
Yoshiharu Matsushima ◽  
Kikuo Ohno
Keyword(s):  
Clinical Features ◽  
Moyamoya Disease ◽  
Mr Angiography ◽  
Posterior Cerebral Artery ◽  
Clinical Course ◽  
Intelligence Scale ◽  
Familial Cases ◽  
Sporadic Cases ◽  
Tokyo Medical ◽  
Disease Analysis

Object The authors compared the clinical features between familial and sporadic cases of moyamoya disease (MMD) by retrospectively analyzing data on patients with MMD registered in the database of Tokyo Medical and Dental University over a period of 28 years. Methods In total, 383 patients with hospital records at Tokyo Medical and Dental University from 1980 to 2007 were registered into the database. The data on all of these patients were retrospectively reviewed to clarify the occurrence of familial cases. Clinical features of child or adolescent patients (< 20 years of age) with MMD were compared between familial and sporadic cases in a subgroup of patients who were registered after 1995, initially diagnosed using MR angiography, and assessed using an intelligence scale. Results Familial occurrence was observed in 59 patients (15.4%) in 40 pedigrees. The clinical features of juvenile patients were analyzed in 124 patients, 22 (17.7%) of whom had familial histories. In comparison with the sporadic cases, patients with familial histories were significantly younger at onset (4.7 vs 6.6 years old), had significantly more cortical infarction (59.1% vs 25.5%), and had significantly more stenoocclusive lesions in the posterior cerebral artery (45.4% vs 24.5%). The rate of patients with intellectual disturbance (intelligence quotient < 75) was significantly larger in the familial cases (47.4%) than in the sporadic cases (17.8%). Conclusions This survey of the clinical features of familial MMD suggests that patients with familial MMD had a more serious clinical course in childhood than the sporadic MMD cases.

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