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

Clinical and necropsy findings in 11 patients with sickle cell anemia (SS) indicate that intracranial hemorrhage (IH) is a delayed sequela of the same vasculopathy that causes cerebral infarction during childhood. Evidence of prior cerebral infarction during childhood included hemiparesis, seizures, an episode of coma, or mental retardation. Computerized tomography (CT) scans showed cerebral infarcts with lucent areas and dilated ventricles or cerebral atrophy. CT or magnetic resonance imaging (MRI) scans after the intracranial hemorrhage demonstrated intraventricular or intracerebral hemorrhages. Angiography or autopsy in seven patients showed widespread vascular occlusion and narrowing of arterial vessels. Moyamoya with internal carotid artery occlusion was identified in two cases. At the time of the IH, three patients were being treated with prophylactic transfusion regimens. We hypothesize that the central nervous system vasculopathy progresses over time and that arterial narrowing in both large and small vessels secondary to endothelial hyperplasia is followed by neovascularization and hemorrhage. Recognition of this pattern of delayed intracranial hemorrhage following cerebral infarction should encourage more intensive evaluation aimed at developing rational interventional therapy prior to a terminal intracranial hemorrhage.
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PMID:Delayed intracranial hemorrhage following cerebral infarction in sickle cell anemia. 213 21

Patients with Sturge-Weber disease with epilepsy refractory to medical therapy have been reported to develop slowly progressive neurological deficits and ultimately become moderately or severely disabled. We studied six patients with Sturge-Weber syndrome including its incomplete form. Three out of six patients with Sturge-Weber syndrome revealed evolution of calcified angioma on computed tomography. All of the three cases developed medically intractable seizures. Total and/or subtotal hemispherectomy was performed for these three cases. The surgery was effective for controlling seizures in all three cases except one with infantile spasm with hypsarrythmia on electroencephalogram who is still on anticonvulsant. Although the unremitting deterioration in mental retardation and hemiparesis was not effectively prevented by the surgery possibly because the timing of surgery was delayed in one case, the surgery not only stopped the frequent medically-intractable seizures, but also dramatically prevented the psychomotor deterioration in the other case. Although the role of surgical treatment for the patients with Sturge-Weber syndrome remains poorly defined, one can expect excellent results if the indications for surgery are carefully analyzed and hemispherectomy is performed on an individual basis.
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PMID:Sturge-Weber disease: operative indications and surgical results. 228 77

As pediatric cardiac surgical techniques have improved in recent years, mortality rates have dropped and attention has turned to residual morbidity, especially neurologic sequelae. Although the majority of children undergoing open-heart surgery for correction of congenital heart defects apparently emerge with no adverse consequences, a small percentage suffer permanent neurologic injury (seizures, motor disorders). Another small and not well-defined population may be left with disorders of higher cortical function, such as mental retardation or learning disabilities. A survey of six major pediatric cardiac surgery units in North America was undertaken in 1988-1989 to ascertain current approaches to the detection and management of neurologic sequelae of pediatric open-heart surgery. All units reported seeing a small but definite incidence of postoperative neurologic symptoms, including alterations of consciousness, seizures, and localized abnormalities such as hemiparesis or delayed choreoathetoid syndromes. Postoperative neuroimaging procedures have shown a disturbing incidence of hypoxic-ischemic encephalopathy, unsuspected cerebral atrophy, and subdural hematomas. Pathogenesis may include factors related to preoperative brain anomalies and/or hypoxic insults, altered cerebral blood flow and metabolism during hypothermic cardiopulmonary bypass with or without total circulatory arrest, embolization, and low cardiac output states postoperatively. Further studies are needed to examine the mechanisms of injury and to develop techniques to minimize the occurrence of these sequelae, as they may be associated with life-long neurologic disability and reduced quality of life.
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PMID:Neurologic sequelae of open-heart surgery in children. An 'irritating question'. 230 48

It is well known that when Sturge-Weber syndrome manifests with seizures in early infancy, hemiparesis develops early, seizures become intractable, and motor weakness and mental retardation are progressive. In North America and Europe, early surgical intervention is recommended in such cases. However, neurosurgical management of Sturge-Weber syndrome has not been reported in Japan. The authors describe a 4-month-old boy with Sturge-Weber syndrome accompanied by intractable seizures who was successfully treated by a two-stage hemispherectomy. Two years postoperatively he remains free of seizures and is active, although his psychomotor development is moderately retarded. Surgical treatment of Sturge-Weber syndrome, including long-term results, is discussed in detail.
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PMID:Surgical treatment of Sturge-Weber syndrome--case report. 247 71

A 5-year-old boy with the epidermal nevus syndrome and hemimegalencephaly is reported. He had pigmented nevi on the forehead and neck, and hemihypertrophy of the body from the birth. He developed intractable seizures, mental retardation, and right hemiparesis. His seizure pattern changed from early infantile epileptic encephalopathy to infantile spasms at 2 months of age. Electroencephalograms showed a suppression-burst pattern in the neonatal period, subsequently changing to hypsarrhythmia. Computerized tomography of the brain disclosed slight dilatation of the posterior horn of the lateral ventricle at the age of 2 months. Later, hemimegalencephaly with calcification on the left side of the brain was noted. Histological examination of the pigmented nevus on the neck showed it to be an acanthosis nigricans-like lesion. Clinical differences between tuberous sclerosis and epidermal nevus syndrome with hemimegalencephaly are discussed.
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PMID:Epidermal nevus syndrome with hemimegalencephaly: a clinical report of a case with acanthosis nigricans-like nevi on the face and neck, hemimegalencephaly, and hemihypertrophy of the body. 275 Oct 68

Cleidocranial dysostosis (CCD) is a rare congenital disorder characterized by the heredity, the disturbance of the ossification of the skull and clavicles, and dental anomaly. The entity of CCD was established by Marie and Sainton in 1898. In Japan about 150 cases have been reported since Haneda's first report in 1933. Recently we experienced a rare case of CCD associated with the temporal arachnoid cyst. The patient was a 61-year-old male who had suffered from mild spastic paresis of the left upper extremity since his childhood. One morning he suddenly noticed motor weakness of the left upper and lower extremities and was transferred to our hospital. On admission we observed the left hemiparesis (MMT 3/5), the left central type facial palsy, and the left long tract signs. Physical examination disclosed frontal bossing, depression of the forehead, sloped shoulders, cone-shaped thorax, and thoracic scoliosis. Plain skull radiograph showed persistent metopic suture and frontal fontanelle, many wormian bones around coronal and lambdoid sutures. Plain radiographs of the systemic bones also showed typical features of CCD such as dysplasia of the lateral third of the bilateral clavicles, deformities of the cervical vertebral bodies, thoracic scoliosis, and wide symphysis. CT scan disclosed the right putaminal hemorrhage, the right temporal arachnoid cyst, enlargement of the right middle fossa, thinning of the temporal bone adjacent to the arachnoid cyst. It also showed the atrophy of the right cerebral peduncle and midbrain. Surgical treatment was performed to remove the hematoma and release the cyst. Several neurological disorders associated with CCD have been reported such as epilepsy, mental retardation, spastic paresis etc.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of cleidocranial dysostosis associated with arachnoid cyst]. 343 33

A 34-year-old male developed consciousness disturbance; semicoma, on December 15, 1984, and diagnosed as a thalamic hemorrhage perforating into the ventricular system after a computed tomographical (CT) examination. Angiography revealed an arteriovenous malformation (AVM) in the right posterior thalamic region, which was fed by a posteromedial choroidal artery. He was transferred to our neurosurgical clinic on February 18, 1985. On admission, he was alert, however, disorientation and slight mental retardation were seen as well as Parinaud's sign. Mild left hemiparesis was also detected with equivocal hypesthesia on the left lower limb. The AVM was subtotally removed on March, 12, via transventricular approach after right parietooccipital craniotomy. Consciousness disturbance (drowsy) and left hemiplegia developed after the operation, however, these deteriorations were transient, recovering to the preoperative or better status by 2 weeks after the operation. Postoperative repetitive examinations of the sensory perceptibility of various modalities revealed remarkable disturbance or complete loss of perception in joint and vibration senses (0-3/10 compared to the healthy left side). Touch sensation was also severely deteriorated (0-3/10) on the affected extremities. The disturbances in these modalities of the sensation did not show any trend to improve until the time of discharge on 57th postoperative day. On the other hand, pain and temperature sensations were less remarkably disturbed (5-8/10), and with tendency of gradual improvement. Estimation of the range of lesion by the CT scan with projecting on the Schaltenbrand & Bailey's atlas revealed that the nucleus ventralis caudalis, centre-median nucleus and pulvinar thalami were involved.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Dissociative sensory disturbance after removal of an arteriovenous malformation in the posterior thalamic region]. 379 Mar 63

The Sturge-Weber syndrome is characterized by the presence of a leptomeningeal hemangioma variably associated with a facial nevus flammeus, seizure disorder, mental retardation, hemiparesis, homonymous hemianopsia, glaucoma, and other signs and symptoms. The relationship between the cutaneous manifestations and the other features of this disorder was studied in 35 patients. We observed that all patients with the facial nevus flammeus had involvement of the upper eye lid or forehead. Bilateral cutaneous involvement was common and when present, was often associated with extensive lesions of the trunk and extremities. The distribution of the cutaneous lesions appeared to be unrelated to that of the trigeminal nerve. A subgroup of patients, the syndrome's "forme fruste," were identified. There appeared to be no relationship between the presence and extent of the cutaneous lesions and the observed seizure disorder, difficulty of seizure control, intellectual deficit, hemiparesis, homonymous hemianopsia, and intracranial calcification. Notably, the "forme fruste" patients appeared to be spared from glaucomatous involvement and from choroidal hemangioma. We will also discuss other aspects of this syndrome.
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PMID:The cutaneous manifestations of Sturge-Weber syndrome. 622 7

Cerebral computerized tomography (CT) was obtained in 37 children with congenital hemiparesis to document the type of cerebral anomalies found in this group, correlate them with various features of the neurological handicap and clinical history and determine the prognostic and practical value of this examination in a newly diagnosed case. The CT-findings were: 1. normal CT: 4 children (11%); 2. unilateral ventricular enlargement: 9 children (24%); 3. others (cortical and subcortical lesions): 24 children (63%). In this latter group, special patterns were found: a) focal ischemic lesions: 7 cases; b) cystic porencephaly: 2 cases; c) ventricular deformities with hydrocephalus: 7 cases; d) varia: 8 cases. The children with normal CT or unilateral ventricular enlargement had mild or moderate hemiparesis without epilepsy or mental retardation. The third group comprised the more severe cases but the prognosis was very variable. The different types of CT-lesions, their possible origin and neuropathological correlates are described and compared with two previous studies. The variety of lesions found in this series and the relative prognostic value of the CT-Scan justify this examination in a newly diagnosed case.
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PMID:Congenital hemiparesis: the spectrum of lesions. A clinical and computerized tomographic study of 37 cases. 666 95

This study presents the results of neurological assessments of a representative sample of seven-year-old Swedish children with perceptual, motor and attentional deficits and their controls (N = 141). Among those children diagnosed as suffering from minimal brain dysfunction (MBD), the majority showed neurodevelopmental deviations indicative of the "clumsy child syndrome'. However, 20 per cent had slight signs of choreoathetosis, diparesis, hemiparesis or ataxia. Other associated neurodevelopmental deviations are considered. The correlation between neurological findings and background factors is discussed: the MBD group had higher scores for various "organic' background factors than the comparison children, and the children with neurological syndromes had somewhat higher scores than the remaining children with MBD. The difficulties in distinguishing MBD from mental retardation, cerebral palsy and childhood psychoses is clearly illustrated. Long-term follow-up will indicate the prognostic significance of these findings.
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PMID:Perceptual, motor and attentional deficits in seven-year-old children: neurological and neurodevelopmental aspects. 687 93


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