[Treatment of neurologic diseases with synthetic corticotropin (beta-1-24-corticotropin)].
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Adenoma, Chromophobe↗
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Results for “Multiple System Atrophy”
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Genetic approach to the nosology of retinal disorders.
During the last 20 years we have carried out, with the collaborators of our Institute, a series of genetic and epidemiologic investigations on tapetoretinal degenerations and allied diseases in Switzerland. Basing conclusions both on the results of this inquiry and on the findings in the literature, we give in this study a general survey of the chorioretinal abiotrophies and describe in particular their clinical and genetic aspects as well as their association with other congenital and hereditary anomalies. A nosologic classification is proposed which takes into account the clinical and genetic features of the systemic heredodegenerative conditions of the retina.
Abnormalities, Multiple↗
Activity of the hypothalamic-pituitary-adrenal axis in multiple sclerosis: correlations with gadolinium-enhancing lesions and ventricular volume.
The known interaction between the immune system and the hypothalamic-pituitary-adrenal axis led us to explore the interrelation between magnetic resonance imaging findings and the hypothalamic-pituitary-adrenal axis activity in 53 multiple sclerosis patients. The cortisol release induced by the dexamethasone-corticotropin-releasing hormone test was negatively associated with the presence and number of gadolinium-enhancing lesions and positively associated with ventricular size. This finding suggests a protective effect of the hypothalamic- pituitary-adrenal drive on acute lesional inflammation in multiple sclerosis, probably by limiting immune overshoot. In contrast, the nature of the correlation between hypothalamic-pituitary-adrenal hyperdrive and brain atrophy remains to be determined.
Adult↗
The route of nutritional support in the critically ill: physiological and economical considerations.
Although it generally is accepted that early enteral nutrition is of benefit to critically ill patients, there is little evidence to support this assertion. Nevertheless, enteral nutrition has many advantages over total parenteral nutrition (TPN), the latter being associated with several complications. Animal studies have shown that injury and infection can lead to gut atrophy and increased mucosal permeability. Translocation of bacteria and endotoxin in these animal models may initiate a systemic inflammatory response and cause multiple organ failure (MOF). Again, there is little direct evidence to suggest that similar mechanisms operate in humans. As a cause of MOF, simple splanchnic ischemia and reperfusion may be sufficient with no absolute requirement for translocation. In this setting, enteral nutrition may preserve splanchnic blood flow and prevent mucosal breakdown. Unfortunately there is a widespread misconception that gastric stasis, the absence of bowel sounds, and recent abdominal surgery preclude enteral feeding. There are few absolute contraindications to early enteral feeding and with motivated staff, the use of prokinetics, and the availability of jejunal feeding tubes, the majority of intensive care patients can be fed enterally. Enteral feeding is more cost effective than TPN, but TPN remains a common therapeutic intervention in the intensive care unit and represents a significant burden on health care budgets. Nutrition support teams have led to savings, particularly by identifying patients who have been inappropriately prescribed TPN and also by preventing excessive enteral feeding.
Animals↗
Progressive intracranial aneurysmal disease in a child with progressive hemifacial atrophy (Parry-Romberg disease): case report.
Intracranial aneurysms are uncommon in children, and their presence often leads to suspicion of a systemic connective tissue disorder. We describe the case of a young male patient with progressive hemifacial atrophy (Parry-Romberg disease) and multiple intracranial aneurysms, a previously undescribed association, and propose that a neural crest defect may be the underlying abnormality in this patient. At age 5 years, the patient was treated for a giant aneurysm of the left cavernous carotid artery with carotid ligation in the neck and a superficial temporal artery-middle cerebral artery bypass. At age 12 years, the patient was similarly treated for a giant aneurysm of the right cavernous carotid artery, which had progressed from a previously noted minute dilatation at age 5 years, with carotid ligation and a superficial temporal artery-middle cerebral artery bypass. At age 21 years, the patient was endovascularly treated for a de novo saccular aneurysm of the left posterior cerebral artery at the P1-P2 junction and a fusiform aneurysm of the distal left posterior cerebral artery. Various studies have suggested that the facial dermis, the subcutaneous tissues, and the skeleton, as well as the tunica media of the cervicocephalic arteries, all arise from neural crest cells, and a disorder of neural crest migration might explain the constellation of findings in this patient.
Adolescent↗
[Advances of surgery and radiation therapy of glioblastoma and metastatic tumor].
On the surgery for malignant gliomas, most cases are beyond the stage for desirable removal of tumors, because of the risk of damage to the normal function of surrounding brain tissue. The restriction of the surgical treatment has inevitably required postoperative radiation therapy. With a protocol aiming at removing tumors extensively and delivering high dose radiation to the tumor area, we treated 107 patients with cerebral glioblastoma. Wide removal of the tumor combined with intraoperative radiation therapy (IORT) was applied to expected resectable cases at the first surgery or at the second salvage surgery after conventional external radiation therapy. Thirty patients underwent extensive removal with IORT and demonstrated a 2-year survival rate of 60%. Fifty six patients were treated only by postoperative radiation therapy and a two-year survival rate was only 6.8%. The results apparently indicate that areas adjacent to the margin of almost complete removal should be irradiated with a sufficient dose to sterilize the remaining malignant remnants. As metastatic brain tumors without systemic metastasis seldom develop to multiple lesions, surgical removal followed by local radiation therapy would be ideal in order to prevent brain atrophy and dementia induced by whole brain irradiation. IORT would be also useful for its strong local effect to tumors and for shortening the hospital stay of patients.
Brain Neoplasms↗
Brain and spinal cord atrophy in multiple sclerosis.
Until recently, atrophy of the brain and spinal chord was thought to occur late in the course of multiple sclerosis (MS) or as a result of rare, fulminant disease. Now atrophy is known to occur early and likely indicates destructive and irreversible pathologic change that may be subclinical. Central nervous system atrophy from MS now can be measured accurately over short time intervals. Atrophy may become an important prognostic indicator in MS and is being evaluated as a treatment outcome measure in population studies, and possibly in the future, in individuals.
Atrophy↗
[Klotho gene and endothelial function].
The klotho gene, originally identified by insertional mutagenesis in mice, suppresses multiple aging phenotypes (e.g. arteriosclerosis, pulmonary emphysema, osteoporosis, infertility, skin atrophy). We have demonstrated that mice deficient for the klotho gene show endothelial dysfunction as manifested by an attenuated response of aortic relaxation in response to acetylcholine stimulation. Systemic nitric oxide production was also significantly reduced in klotho deficient mice. Oxidative stress was increased in klotho deficient mice. The klotho gene delivery improves multiple aging phenotypes. Our findings establish the basis for the therapeutic potential of klotho gene delivery in age-related diseases.
Aging↗
Regional brain atrophy is associated with physical disability in multiple sclerosis: semiquantitative magnetic resonance imaging and relationship to clinical findings.
OBJECTIVE: Brain atrophy may occur early in the course of multiple sclerosis (MS) and may be associated with disability. Brain magnetic resonance imaging (MRI) of 114 MS patients (group A) were analyzed for regional atrophy (vs age-/gender-matched controls) and T1 and T2 lesions using 4-point rating systems. Thirty-five separate patients (group B) were analyzed for cortical atrophy (ordinal scale), third ventricular width, and total T2 hyperintense lesion volume (computer assisted). In group A, regression modeling indicated that inferior frontal atrophy (P = .0003) and T2 lesions in the pons (P = .02) predicted physical disability (Expanded Disability Status Scale [EDSS] score). Secondary progressive (SP) versus relapsing patients were predicted by inferior parietal (P = .002), superior parietal (P = .006), temporal (P = .008), inferior frontal (P = .01), superior frontal (P = .01), cerebellum (P = .01), occipital (P = .01), and midbrain (P = .02) atrophy. SP patients were also predicted by total atrophy (P = .01) and third ventricular enlargement (P = .03) but not T1 or T2 lesions. In group B, the regression model predicting EDSS score included only superior frontal atrophy (r = 0.515, P = .002). Mean kappa coefficients of ordinal ratings were 0.9 (intraobserver) and 0.8 (interobserver). Ordinal ratings correlated well with quantitative assessments. The authors conclude that brain atrophy is closely associated with physical disability and clinical course in MS patients and can be appreciated using a semiquantitative MRI regional rating system.
Adult↗
[Disseminated lupus erythematosus with generalized arteritis, granular atrophy and multiple cerebral lesions].
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Arteritis↗
Multiple dermal grafts for hemifacial atrophy caused by lupus panniculitis.
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Adult↗
A community survey of neurological disorders in Saudi Arabia: the Thugbah study.
We report the findings of a total population survey of Thugbah community in the Eastern Province of Saudi Arabia (SA) to determine its point prevalence of neurological diseases. During this two-phase door-to-door study, all Saudi nationals living in Thugbah were first screened by trained interviewers using a pretested questionnaire (sensitivity 98%, specificity 89%) administered at a face-to-face interview. Individuals with abnormal responses were then evaluated by a neurologist using specific guidelines and defined diagnostic criteria to document neurological disease. The questionnaire was readministered blind by a neurologist to all those with abnormal responses and a 1-in-20 random sample of those without abnormal responses, respectively. The family members of an individual with an abnormal response were also screened to improve accuracy. A total of 23,227 Saudis (98% of the eligible subjects) were screened and those residing in Thugbah on the reference date (22,630) were used to calculate the point prevalence rates. Forty-two percent of those screened were in the first decade of life and only 1.5% were more than 60 years old. There were marginally more females (50.2%) than males (49.8%). Consanguineous marriages especially between first cousins were present in 54.6%. The demographic characteristics of Thugbah community were similar to those in other parts of SA. The overall crude prevalence ratio (PR) for all forms of neurological disease was 131/1,000 population. All subsequent PRs are per 1,000 population. Headache syndromes were the most prevalent disorder (PR 20.7). The PR for all seizure disorders was 7.60, and the epilepsies (6.54) were more frequent than febrile convulsions (0.84). Mental retardation, cerebral palsy syndrome, and microcephaly were common pediatric problems with PRs of 6.27, 5.30 and 1.99, respectively. Stroke, Parkinson's disease, and Alzheimer's disease were uncommon with respective PRs of 1.8, 0.27 and 0.22. Central nervous system (CNS) malformations (0.49) such as hydrocephalus and meningomyelocele were more prevalent than spinal muscular atrophy (0.13), congenital brachial palsy (0.13) and narcolepsy (0.04). Multiple sclerosis was rare (0.04). Osteoarthritis and low back pain syndromes were the main non-neurological conditions seen. The major medical diseases that may be neurologically relevant were diabetes mellitus, hypertension, and connective tissue disorders.(ABSTRACT TRUNCATED AT 400 WORDS)
Adolescent↗
Death ligands and autoimmune demyelination.
Death ligands induce apoptosis, which is a cell suicide program leading mainly to selective elimination of an organism's useless cells. Importantly, the dying cell is an active participant in its own demise ("cellular suicide"). Under physiological conditions, apoptosis is most often found during normal cell turnover and tissue homeostasis, embryogenesis, induction and maintenance of immune tolerance, development of the nervous system, and endocrine-dependent tissue atrophy. However, apoptotic processes have also been suggested to contribute to the pathology of the autoimmune demyelinating disease multiple sclerosis (MS) and its animal model, experimental autoimmune encephalomyelitis. Here, apoptosis plays a double role. On one hand, impaired apoptosis may result in increased numbers or persistence of activated myelinspecific T cells. On the other hand, local tissue damage involves apoptosis of oligodendrocytes and neurons, leading to the clinical symptoms. In this article, an overview is given of the current knowledge of the roles of apoptosis-mediating and immune regulatory death ligands of the tumor necrosis factor (TNF) family (TNF, lymphotoxin-beta, OX40L [CD134L], CD154 [CD40L], CD95L, CD70 [CD27L], CD153 [CD30L], 4-1BBL [CD137L], TRAIL, TWEAK, BAFF, GITRL) in the pathogenesis of MS and of their implications for related therapeutic strategies.
Animals↗
Retinal periphlebitis and retinitis in multiple sclerosis. I. Pathologic characteristics.
Eyes from 47 autopsy cases of multiple sclerosis were studied pathologically. Lymphocytic or granulomatous retinal periphlebitis was found in four cases (seven eyes); focal lymphocytic or granulomatous retinitis was present in three cases (five eyes). These findings were correlated with optic nerve changes (periphlebitis, neuritis, leptomeningitis, and atrophy), retinal venous sclerosis, uveitis, central nervous system involvement, clinical activity of disease at death, and with ultrastructural and immunopathological retinal findings.
Humans↗
Ocular findings in lissencephaly.
PURPOSE: To report our retrospective study of 20 cases with lissencephaly and describe ocular and visual abnormalities associated with this disorder. METHODS: Patients with lissencephaly were identified and classified into classic (type I) or cobblestone (type 2) lissencephaly on the basis of a review of clinical records and neuroimaging studies. Only patients examined by an ophthalmologist were included in the study. RESULTS: Only 1 patient had a normal ocular examination. Ocular abnormalities included optic nerve hypoplasia and atrophy, retinal dysplasia, retinal nonattachment, macular hypoplasia, anterior segment malformation, and strabismus. CONCLUSIONS: Ocular abnormalities in classic (type 1) lissencephaly are less severe. Central, steady, and maintained fixation may be present despite the presence of optic nerve hypoplasia, optic atrophy, macular hypoplasia, strabismus, or refractive errors. Retinal and anterior segment abnormalities were observed only in cobblestone (type 2) lissencephaly. These patients often have severe visual impairment because of retinal or cortical disease.
Abnormalities, Multiple↗
DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy and deafness) syndrome associated with myocardial disease.
A 30 year old man with DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy and deafness) syndrome associated with myocardial disease is reported. Echocardiographic study revealed a marked symmetric left ventricular hypertrophy. Histology of the endomyocardial biopsy specimen from the right ventricle showed severe glycogen deposition in the myocytes. This case may indicate that DIDMOAD syndrome is a hereditary systemic disease affecting multiple organs, including the myocardium.
Adult↗
Ocular manifestations of Donohue's syndrome.
INTRODUCTION: Donohue's syndrome, also known as Leprechaunism, is a rare autosomal recessive disease that manifests at birth with symptoms of endocrine dysfunction. Metabolic characteristics of the disease include postprandial hyperglycemia, fasting hypoglycemia, insulin resistance, hyperinsulinemia, and failure to thrive. The physical features most often associated with this condition include hypertrichosis, pachyderma, acanthosis nigricans, prominent genitalia, and elfin-like facial characteristics of prominent eyes, wide nostrils, thick lips, and large, low-set ears. Not only is this syndrome rare, but it often results in infant and early childhood mortality. The literature regarding ocular manifestations is limited. CASE REPORT: We present a case of a 29-year-old male with Donohue's syndrome and significant ocular findings including a subluxated mature cataract, retinal detachment, high myopia, and optic atrophy. DISCUSSION: These ocular sequelae are discussed with regard to the noted endocrine dysfunction and its effects on tissue development and growth.
Abnormalities, Multiple↗
Multiple congenital malformations of the face, nervous system and musculoskeletal system of pigs.
Multiple congenital malformations occurred in 21 of 26 pigs (81%) in 3 litters from 2 piggeries. Two of 26 pigs (7%) were mummified while 3 of 26 (11%) were normal. Cheiloschisis was seen in all malformed pigs and was accompanied by various combinations of palatoschisis, arhinencephaly, hypoplasia of the spinal cord, arthrogryposis of the pelvic limbs, polydactyly, renal lesions and cauliflower ears. Histological examination suggested that arthrogryposis was due to neurogenic muscular atrophy. The teratogen was not identified.
Abnormalities, Multiple↗