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Query: UMLS:C0025362 (mental retardation)
 15,878 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-five patients presenting basal ganglia calcification were assessed. This finding comprised 0.68% of all skull CT scan carried out during the period. Two patients were neurologically asymptomatic and 23 presented a variety neurological disorders--headache (7 patients), stroke (5 patients), extrapyramidal syndromes (2 patients), tumor (2 patients), epilepsy (1 patient), mental retardation (1 patient), dementia (1 patient), cranial trauma (1 patient), other neurological conditions (3 patients)--or were asymptomatic from the neurological point of view (2 patients). Findings in the CT scan other than the basal ganglia calcification were observed in 15 (60%) patients. There was a clinical-CT scan correlation in these cases but not in those in which the basal ganglia calcification was an isolated finding. This study highlights the fact that basal ganglia calcification is often a nonspecific finding on CT scan and that it may not be possible to establish a clinical-pathological correlation between them.
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PMID:[Basal ganglia calcification on computed tomography: clinical characteristics in 25 patients]. 1711 22

Hunter syndrome, or mucopolysaccharidosis type II, is an X-linked recessive disorder resulting from iduronate sulfatase deficiency. Typical manifestations include short stature, mental retardation, hydrocephalus, macroglossia and cardiac valvulopathy. We describe a 21-year-old patient who presented with acute ischemic stroke and evidence of cerebral embolization. The echocardiogram demonstrated thickened mitral and aortic valves as potential sources of emboli. We conclude that ischemic stroke secondary to cardioembolization is a potential complication of Hunter syndrome.
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PMID:Cerebral infarction in Hunter syndrome. 1705 60

Homocystinuria is an inherited metabolic disorder caused by severe deficiency of cystationine beta-synthase activity, resulting in the tissue accumulation of homocysteine (Hcy). Affected patients usually present many signs and symptoms such as seizures, mental retardation, atherosclerosis and stroke. The aim of this study is to evaluate in vivo and in vitro effects of Hcy using hippocampal slices from Wistar rats exposed to oxygen and glucose deprivation (OGD), followed by reoxygenation, an in vitro model of hypoxic-ischemic events. Neural cell injury was quantified by the measurement of lactate dehydrogenase (LDH) released from damaged cells into the extracellular fluid. The results showed that both in vivo and in vitro Hcy increased the LDH released to de incubation medium, suggesting an increase of tissue damage caused by OGD. This fact can be related with the high incidence of stroke in homocystinuric patients.
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PMID:Homocysteine increases neuronal damage in hippocampal slices receiving oxygen and glucose deprivation. 1710 28

Alcohol is detrimental to the developing brain and remains the leading cause of mental retardation in developed countries. The mechanism of alcohol brain damage remains elusive. Studies of neurological problems in adults have focused on alcohol's cerebrovascular effects, because alcoholism is a major risk factor for stroke and cerebrovascular injuries. However, few studies have examined similar cerebrovascular effects of fetal alcohol exposure. We examined the effect of chronic binge alcohol exposure during the second trimester on fetal cerebrovascular and metabolic responses to hypoxia in near-term sheep and tested the hypothesis that fetal alcohol exposure would attenuate cerebrovascular dilation to hypoxia. Pregnant ewes were infused with alcohol (1.5 g/kg) or saline intravenously at 60-90 days of gestation (full term = 150 days). At 125 days of gestation, we measured fetal cerebral blood flow (CBF) and oxygen metabolism at baseline and during hypoxia. Maternal blood alcohol averaged 214 +/- 5.9 mg/dl immediately after the 1.5-h infusion, with similar values throughout the month of infusion. Hypoxia resulted in a robust increase in CBF in saline-infused fetuses. However, the CBF response to hypoxia in fetuses chronically exposed to alcohol was significantly attenuated. Cerebral oxygen delivery decreased in both groups of fetuses during hypoxia but to a greater degree in the alcohol-exposed fetuses. Prenatal alcohol exposure during the second trimester attenuates cerebrovascular responses to hypoxia in the third trimester. Altered cerebrovascular reactivity might be one mechanism for alcohol-related brain damage and might set the stage for further brain injury if a hypoxic insult occurs.
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PMID:Binge alcohol exposure in the second trimester attenuates fetal cerebral blood flow response to hypoxia. 1734 36

Thyroid hormones are of primary importance for the perinatal development of the central nervous system, and for normal function of the adult brain. These hormones primarily regulate the transcription of specific target genes. They increase the cortical serotonergic neurotransmission, and play an important role in regulating central noradrenergic and GABA function. Thyroid deficiency during the perinatal period results in mental retardation. Hypothyroidism of the adults causes most frequently dementia and depression. Other less common clinical pictures include myxoedema coma, dysfunction of cerebellum and cranial nerves. Hypothyroidism also increases predisposition of stroke. Peripheral diseases frequently include polyneuropathy, carpal tunnel syndrome, myalgic state, and rarely myokymia. Nearly all the hyperthyroid patients show minor psychiatric signs, and infrequently psychosis, dementia, confusion state, depression, apathetic thyrotoxicosis, thyrotoxic crisis, seizures, pyramidal signs, or chorea occur. The peripheral complications may be indicated by chronic thyrotoxic myopathy, infiltrative ophthalmopathy, myasthenia gravis, periodic hypokalemic paralysis and polyneuropathy. Generalized resistance to thyroid hormone was confirmed in a number of patients with attention deficit-hyperactivity disorder. Significantly elevated antithyroid antibody titers characterize Hashimoto's encephalopathy. This condition is a rare, acute - subacute, serious, life threatening, but steroid-responsive, relapsing-remitting, autoimmune disease.
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PMID:[Some neurologic and psychiatric complications in endocrine disorders: the thyroid gland]. 1734 50

Nitric oxide (NO) and carbon monoxide (CO) synthesized from L-arginine by NO synthase and from heme by heme oxygenase, respectively, are the well-known neurotransmitters and are also involved in the regulation of vascular tone. Recent studies suggest that hydrogen sulfide (H(2)S) is the third gaseous mediator in mammals. H(2)S is synthesized from L-cysteine by either cystathionine beta-synthase (CBS) or cystathionine gamma-lyase (CSE), both using pyridoxal 5'-phosphate (vitamin B(6)) as a cofactor. H(2)S stimulates ATP-sensitive potassium channels (K(ATP)) in the vascular smooth muscle cells, neurons, cardiomyocytes and pancreatic beta-cells. In addition, H(2)S may react with reactive oxygen and/or nitrogen species limiting their toxic effects but also, attenuating their physiological functions, like nitric oxide does. In contrast to NO and CO, H(2)S does not stimulate soluble guanylate cyclase. H(2)S is involved in the regulation of vascular tone, myocardial contractility, neurotransmission, and insulin secretion. H(2)S deficiency was observed in various animal models of arterial and pulmonary hypertension, Alzheimer's disease, gastric mucosal injury and liver cirrhosis. Exogenous H(2)S ameliorates myocardial dysfunction associated with the ischemia/reperfusion injury and reduces the damage of gastric mucosa induced by anti-inflammatory drugs. On the other hand, excessive production of H(2)S may contribute to the pathogenesis of inflammatory diseases, septic shock, cerebral stroke and mental retardation in patients with Down syndrome, and reduction of its production may be of potential therapeutic value in these states.
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PMID:Hydrogen sulfide (H2S) - the third gas of interest for pharmacologists. 1737 2

A pilot study of the Agrabiah area in Al-Khobar was undertaken to field test study methodologies and identify possible limitations and constraints to a planned community survey for neurological disorders in the Eastern Province of Saudi Arabia. The survey used a pre-tested questionnaire administered by trained personnel to all subjects living within 50 blocks randomly selected from the 198 inhabited ones in the area. Subjects with abnormal responses on screening were then evaluated by neurologists using specific guidelines and criteria to establish the diagnosis of neurological disease. One thousand four hundred and eighty-five subjects (98.3% of all eligible subjects) were screened: 227 (15%) had abnormal responses. Of the 202 subsequently evaluated by neurologists, 178 had definite neurological disease. The overall crude prevalence rate (PR) per 1000 population for neurological morbidity was 120.5 (95% confidence limits [CL] 103.5 to 136.5). Headache syndromes (PR 99.7, CL 83 to 114.7) were common. The other common disorders were seizures (PR 10.2, CL 5.1 to 15.3), peripheral nerve disorders (PR 2.7), and stroke (PR 2.0). Mental retardation and cerebral palsy were the main pediatric problems with PRs of 1.4 and 0.7 respectively. Our results show that a community survey for neurological disorders is feasible in Saudi Arabia and the modified questionnaire was a good screening instrument (sensitivity 94.7%, specificity 96.8%). However, the findings on the pattern and prevalence of neurological disorders need to be viewed with caution, particularly against the background of the scope of the study and the small number of subjects assessed. Cultural practices, local time and social events, and climatic conditions significantly affected community participation and the coverage achieved by the study. These factors should be considered when planning community surveys in Saudi Arabia and other environments with similar sociocultural settings.
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PMID:Community survey of neurological disorders in Saudi Arabia: Results of the pilot study in Agrabiah. 1758 96

Congenital disorders of glycosylation type Ia (CDG-Ia) are the most common type of CDG and are characterized by liver dysfunction, coagulation disorders, mental retardation, hypotonia, cerebellar dysfunction, polyneuropathy, seizures, and stroke-like episodes. Stroke-like episodes occur in 40-55% of cases, but their etiology is not fully understood. Although it has been stated that an epileptic process may cause the stroke-like episodes, there is no clear evidence of ischemic stroke. Here, we describe two stroke-like episodes in a patient with CDG. We performed radiological studies during each episode and obtained two distinct magnetic resonance imaging (MRI) findings: one revealed an ischemic stroke, and the other demonstrated marked edema followed by focal necrosis. This is the first direct evidence of ischemic stroke, and we report that another process may affect the etiology in the same patient.
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PMID:Different neuroradiological findings during two stroke-like episodes in a patient with a congenital disorder of glycosylation type Ia. 1883 16

The anterior opercular or biopercular syndrome is a cortical pseudobulbar palsy due to bilateral lesions of the anterior brain operculum. It is characterized by preservation of reflex function and automatic activity, without mental impairment. Two cases are reported herein and the relevant literature reviewed. The first case was a 73-year-old female with a history of a stroke occurring seven years previously, without sequelae in the interim. She presented with sudden loss of consciousness. The neurological examination showed a right facial central palsy and anarthria, with reflex acts such as smiling, blinking and yawning, not elicited by commands; she also had a right hemiparesis and walking impairment. A brain CT scan showed an old ischemic infarction in the region of the right medial cerebral artery. Because the right motor involvement did not correlate with the findings of the initial CT scan, another CT scan two days later showed an acute brain infarction in the vicinity of the left medial cerebral artery. The second case was an 8-year-old girl with mental retardation and impairment of verbal development, caused by of biopercular pachygyria. Facio-pharyngo-glosso-masticatory diplegia and volitional selective palsy of the oro-facial muscles was seen in both patients. The neuropsychological assessment showed cognitive, emotional and social interaction impairment in both cases -as part of the frontal convexity syndrome in the first case and of mental retardation in the second. The two patients had difficulty in mastication and swallowing. The prognosis for recovery of verbal capacity is poor, although generally most patients recover the ability to swallow.
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PMID:[Biopercular syndrome: report of two cases and literature review]. 1871 20

The Dyke-Davidoff-Masson syndrome is characterized by various symptoms related to hemiatrophy of the cerebrum and hypertrophy of the ipsilateral calvarium and paranasal sinuses. Clinical findings include hemiparesis or hemiplegia, seizures and/or mental retardation. The present report discusses the very unusual case of a late-diagnosed Dyke-Davidoff-Masson syndrome in a 75-year-old body donor who had suffered a left-sided stroke associated with the internal carotid artery in the course of tonsillitis at the age of 5.
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PMID:Postmortal diagnosis of a Dyke-Davidoff-Masson syndrome in a 75-year-old woman---a case report. 1909 69


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