Moderate Intellectual Impairment Research Articles

Reaching across the disability divide: The case for collaboration with the disability community to construct a robust informed consent process around prenatal screening and diagnosis

Reaching across the disability divide: The case for collaboration with the disability community to construct a robust informed consent process around prenatal screening and diagnosis

Cerebral blood flow in chronic toxic encephalopathy in house painters exposed to organic solvents

Cerebral blood flow (CBF) was studied in 11 controls and 9 house painters occupationally exposed to organic solvents for a mean of 22 years. They had mild to moderate intellectual impairment, and no or only minor cerebral atrophy was seen in a CT-scan of the brain. The 133Xe inhalation technique was used and the flow was calculated from the initial slope of the 133Xe wash out curve (ISI). ISI averaged 36.8 ml/100 g/min in the painters and 45.4 ml/100 g/min in the controls, representing a significant difference (P less than 0.05). The reduced CBF in these painters with slight to moderate intellectual impairment might be due to limited neuronal loss or to permanently decreased metabolism of the neurones.

Epilepsy associated with a cerebellar arachnoid cyst: seizure control following fenestration of the cyst

The role of the cerebellum in the pathogenesis of seizures remains controversial. Cerebellar origin of seizures, albeit rare, has been described in the literature in association with intrinsic lesions of the cerebellum. We present a unique case of a patient with medically intractable, secondary generalized epilepsy, associated with a superior cerebellar quadrigeminal arachnoid cyst. A 9-year-old child presented with medically refractory secondary generalized epilepsy associated with recurrent headaches since 6 months of age. The child also had moderate intellectual impairment and autism. On the magnetic resonance imaging (MRI) of the head, he was noticed to have a small superior cerebellar arachnoid cyst in the quadrigeminal area that had increased in size slightly. Interictal electroencephalograph (EEG) was unable to localize the site of the epilepsy. Neurological examination was unremarkable. A suboccipital craniotomy and supracerebellar infratentorial approach to the cyst was performed at 9 years of age. Intraoperative electrocorticography (ECOG) demonstrated epileptic activity from the cerebellar tissue adjacent to the cyst. The cyst was fenestrated, and the cyst wall was sent for histology. Seizure control improved dramatically after fenestration of the cyst. This case provides strong evidence that, albeit rare, the cerebellum may be a source of epileptic activity due to compression by a lesion in the posterior fossa. Hence, in cases with intractable epilepsy of unknown supratentorial source, the differential diagnosis should include a posterior fossa lesion. The finding of a posterior fossa lesion in such cases, even if it is small and appears benign, should precipitate a discussion about the possible relationship between the posterior fossa lesion and the epilepsy.

Smooth mosaics

Lissencephaly (LIS) is a diffuse cortical malformation caused by an incomplete neuronal migration disorder in early brain development.1 There is a continuum of structural alterations ranging from complete agyria and thickened cortex to variable extent of agyria or pachygyria, and subcortical band heterotopia (SBH) as the mildest form.2 Severe phenotypes include marked mental retardation, therapy refractory epilepsy, and cerebral palsy leading to reduced life expectancy whereas milder phenotypes encompass less severe epilepsy and mild to moderate intellectual impairment. A majority of patients with a clinical and neuroradiologic manifestation of the lissencephaly spectrum harbor a mutation either in the LIS1 gene or in the doublecortin ( DCX or XLIS ) gene.3 Classic LIS (type I) is associated with mutations in the LIS1 gene whereas patients with typical SBH are prone to carry mutations in the X-chromosomal DCX …

Spirometry is Affected by Intelligence and Behavior in Duchenne Muscular Dystrophy

Children with Duchenne muscular dystrophy (DMD) have progressive respiratory muscle weakness. Spirometry monitors progress, but is effort-dependent. Intelligence quotients (IQ) average one standard deviation below normal, and behavioral disturbance is common. Our aim was to assess if impaired intelligence or behavior influences spirometry in children with DMD, and if computerized visual incentives (CVI) are beneficial. Forty-seven boys with DMD, of mean age 12.6 years (range, 6-19), were recruited. Full-scale, performance, and verbal IQ, and parent-and-teacher-reported oppositional behavior scores, were recorded. Each was divided into moderate, mild, and no impairment groups. A randomized crossover design was applied to performing spirometry with or without CVI first. A Jaeger Masterscope (version 4.60) was used. Linear regression defined the relationship between spirometry and both IQ and behavior scores. The paired Student's t- test compared spirometry performed with and without CVI for the overall group and subgroups. Boys with DMD can adequately perform spirometry. There is an association between %FEV1 and %FVC with full-scale (r = 0.50, P = 0.002; r = 0.49, P = 0.003, respectively), performance (r = 0.68, P < 0.0005; r = 0.68, P < 0.0005, respectively) and verbal (r = 0.39, P = 0.043; r = 0.36, P = 0.037, respectively) IQ, but not with parent (P = 0.77, P = 0.70, respectively) or teacher (P = 0.90, P = 0.90, respectively)-reported oppositional behavior scores. The effect of CVI was significant in those with moderate full-scale (P = 0.03), performance (P = 0.002), and verbal (P = 0.02) intellectual impairment, and moderately severe teacher-reported oppositional behavior (P = 0.02). In conclusion, spirometry results are related to intelligence in DMD. Using CVI improves the FVC obtained in those with moderate intellectual or behavioral impairment.

What influences physiotherapy use by children with cerebral palsy?

To investigate factors that influence the frequency of physiotherapy currently used by a population of children with moderate to severe cerebral palsy (CP). A survey using a postal questionnaire was sent to 212 parents of children with moderate to severe CP. The families were identified from a geographically defined case register of children with CP in Northern Ireland (the Northern Ireland Cerebral Palsy Register). Eighty-five per cent of parents responded. One-third of parent responses regarding their child's use of physiotherapy were validated with their child's physiotherapist and the level of agreement was high. Ninety-four per cent (169/180) of children received conventional physiotherapy from a statutory source during the school term. Of these, 61% (104/169) used 'intense' levels of physiotherapy (defined as at least twice a week). A higher proportion of intense users were children with severe CP compared with moderate CP (69% vs. 47%; P < 0.01); with moderate intellectual impairment (IQ </= 70 > 50) compared with severe (IQ </= 50) or no intellectual impairment (IQ > 70) (81% vs. 64% vs. 39% respectively; P < 0.01) and at schools for physical disability (PD) compared with severe learning disability (SLD) or mainstream (MS) schools (82% vs. 66% vs. none respectively; P < 0.001). After controlling for severity of motor impairment and the presence and severity of intellectual impairment, children with CP at MS schools used on average significantly less physiotherapy compared with children with similar levels of motor impairment at PD schools (P < 0.001). Children with CP in MS schools use less physiotherapy compared with children with similar levels of disability in special schools. Organizing services around special schools may limit the degree to which children with CP and other disabilities are successfully integrated into MS education.

Patterns of service use among people with learning disabilities discharged from long-stay hospital care in Northern Ireland

AbstractObjectives: Continuing deinstitutionalisation has led to growing concern about the availability and accessibility of services for people with learning disabilities transferring to community living. This study was undertaken in order to assess the configuration of services in terms of availability and uptake for people with learning disabilities who have left long-stay hospital care and to identify gaps or barriers to service provision within the unique integrated health and social services structure in Northern Ireland.Method: The ‘keyworkers’ of 195 people – most of whom were aged 40-59 years with a diagnosis of moderate intellectual impairment – were interviewed by a researcher one year after discharge using the Service Interview.Results: While a wide range of generic and specialist services was available, ‘packages’ of care consisted largely of public sector services (eg. GPs, chiropodists and social workers) and relied, to some extent, on the type of community accommodation. Although services appeared well co-ordinated in terms of care reviews and keyworker arrangements, 40% of people required more one-to-one support particularly in areas related to integration. However, services were perceived by care staff to be satisfactoryConclusions: The development of community care has been slower in Northern Ireland than elsewhere and a large proportion of resources remain tied up in hospital care. However, existing community-based services appear to be addressing individual needs. Some former patients, though, may have been subject to transinstitutionalisation in the sense that their choice of accommodation was restricted mainly to large private sector homes and work and daytime opportunities were insufficient to facilitate integration. Service planners and providers need to give further consideration to the likely effects of different forms of rehabilitation, reprovision and resettlement and to be aware that the pattern of service provision is likely to be different for the more dependent cohorts of people who leave hospital in the future.

Multiple system atrophy: a review of 203 pathologically proven cases.

We report the clinicopathological features of 203 cases of pathologically proven multiple system atrophy (MSA) from 108 publications up to February 1995. The majority of patients showed symptoms in their early fifties, and men were more commonly affected than women (ratio of 1.3:1). Most patients suffered from some degree of autonomic failure (74%). Parkinsonism was the most common motor disorder (87%), followed by cerebellar ataxia (54%) and pyramidal signs (49%). The response to levodopa was poor in most patients, but there was a subgroup with a good response, who also often developed axial levodopa-induced dyskinesias. Other characteristic features included severe dysarthria, stridor, and, in a few patients, contractures and dystonia (antecollis). Mild or moderate intellectual impairment occurred in some cases, but severe dementing illness was most unusual. The main pathological change comprised cell loss and gliosis in the putamen, caudate nucleus, external pallidum, substantia nigra, locus ceruleus, inferior olives, pontine nuclei, cerebellar Purkinje cells, and intermediolateral cell columns of the spinal cord. However, other neuronal populations were also involved to varying degrees, such as the thalamus, vestibular nucleus, dorsal vagal nucleus, corticospinal tracts, and anterior horn cells. Characteristic glial and/or neuronal cytoplasmic inclusions were identified in all cases in which they were sought, irrespective of clinical presentation. Akinesia correlated with the degree of nigral and putaminal cell loss, whereas rigidity was related only to the later. Tremor was unrelated to cell loss at any site. Ataxia correlated with the degree of olivopontocerebellar atrophy. Pyramidal signs were associated with pyramidal tract pallor. Our analysis also confirmed an association of postural hypotension with intermediolateral cell column degeneration.

Nosology of spastic tetraplegic cerebral palsy: clinical review of fifty cases.

The aim of this study is to contribute to the definition of tetraplegic cerebral palsy (TCP) and to verify the classification criteria currently used by reviewing the clinical and neurological aspects of 50 children having non-progressive encephalopathy with neurological involvement of 4 limbs (symmetric 4-limb type, side-asymmetric type, upper-limb dominated type with or without dystonic traits). All severe diplegic patients, i.e. less upper than lower limb involvement and patients with dystonic hyperkinetic syndrome without spastic features were excluded. The data were supplied by the hospital records, evolution of motor performance and the presence or absence of epilepsy, visual problems, language disorders, intellectual impairment. All the children underwent: neurological examination, functional assessment, cognitive evaluation. Severe motor impairment was found in 80% of the patients, whose clinical characteristics led to a diagnosis of severe TCP or "true TCP" according to Hagberg, whereas 20% of them showed mild to moderate impairment with a more favorable prognosis. This group of patients complied with the classification of Michaelis who defined the quadrispastic CP types more broadly and delineated five subgroups also including cases with milder involvement (side-dominated, three-limb dominated tetraparesis). The intellectual assessment showed that, in the severely affected patients, only a small percentage had severe intellectual impairment (IQ less than 50, 37.5%), which contrasts with published data on this pathology, while the majority of the subjects showed moderate (25%) or mild intellectual impairment (32.5%) or normal cognitive function (5%).(ABSTRACT TRUNCATED AT 250 WORDS)

Neuropsychological sequelae of carbon monoxide toxicity at eleven-year follow-up

Abstract This is a case study of a man who experienced accidental carbon monoxide (CO) poisoning and who developed neurological sequelae which led to his psychiatric hospitalization 4 years later. Skull x-rays, EEG, brain scan, and CT scan were all within normal limits at the time of hospitalization, but the patient demonstrated moderate intellectual impairment, including marked visual/spatial deficits, as well as other neuropsychological difficulties. A follow-up evaluation 11 years after the poisoning indicated moderate improvement, however, the visual/spatial deficits were relatively unimproved. The authors concluded that it is important to evaluate the cognitive impairment of CO victims over time: that the apparent diffuse neurological impairment of these patients may, in reality, be a stable and discrete pattern of neurological impairment; and that CO victims who appear to have “totally recovered”; may, to the contrary, have lingering neurological impairments which are not immediately obvious without...

Congenital muscular dystrophy and cerebral CT scan anomalies: Results of a collaborative study of the Société de Neurologie Infantile

We present the results of a collaborative study on the association of congenital muscular dystrophy with central nervous system anomalies revealed by CT scan investigation of 10 patients. In seven children, an abnormal hypodensity of the cerebral white matter is found; in four of these patients, this radiological anomaly is either isolated, or associated with a moderate intellectual impairment; in one case, severe mental retardation and ocular changes had occurred; in the other two cases, the muscular disease was progressing slowly, in association with microcephaly, epilepsy, and moderate mental retardation. Three children were afflicted with a severe early encephalopathy and congenital muscular dystrophy, and presented signs of cortical and subcortical atrophy on CT scan. Two of these patients corresponded to different types of cerebro-ocular dysplasia-muscular dystrophy syndromes, and the third patient of Fukuyama's congenital muscular dystrophy. These observations are discussed and compared with those reported in the literature. The authors emphasize the need to investigate possible cerebral CT scan anomalies in congenital muscular dystrophies, and to look for muscular changes in some prenatal encephalopathies.

Brain Damage Due to Penicillin

Two young adults suffered anaphylactic shock after administration of penicillin. One patient received an intramuscular injection while the other ingested a tablet. Upon recovery from shock, signs and symptoms of an acute brain syndrome, including disorientation and confusion, were present. This was followed by a persistent chronic brain syndrome. Nine months after the episode of shock, one patient showed moderate intellectual impairment with constriction of interests and confabulation. The other patient, 15 months after being in shock, was severely impaired with disorientation, very poor memory, and was unable to adequately take care of her home or child. Other psychiatric disturbances due to penicillin do not generally have such serious sequelae. It seems quite probable that many other patients who recover from anaphylactic shock resulting from penicillin suffer some degree of brain damage due to

The fairfield block substitution test for measuring intellectual impairement

1. The description, development, and standardization of The Fairfield Block Substitution Test are reported. 2. The methods of administering, scoring and interpreting are stated. 3. Norms are established for three levels of intellectual impairment. 20 points or more—no evidence of intellectual impairment (normals) 16 to 20 points—moderate intellectual impairment (schizophrenics, deteriorated alcoholics) 0 to 16 points—severe intellectual impairment (organics) 4. The Fairfield Block Substitution Test depicts intellectual impairment due to functional or organic defects.