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Query: UMLS:C0018991 (hemiplegia)
 3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present our analysis of 44 patients with alternating hemiplegia of childhood. The clinical course usually consisted of three phases. The first was dominated by abnormal eye movements and dystonic episodes, the second by hemiplegic spells and psychomotor regression, and the third by persistent developmental delay and fixed neurologic deficits. The age of onset was 0-54 months (mean = 7.9 +/- 13 months). The presenting signs included abnormal ocular movements in 65%, dystonia in 60%, and hemiplegia in 32%. Patients with an early onset of the disorder and an early appearance of hemiplegic spells faired the poorest developmentally. Developmental delay was present in 91%, ataxia in 68%, choreoathetosis in 50%, and seizures in 18%. Laboratory investigations suggested mitochondrial abnormalities and cerebrovascular dysfunction in several patients. Numerous therapies were largely ineffective. Flunarizine reduced the duration, severity, and frequency of the hemiplegic attacks in 78%. Patients who received flunarizine did not differ developmentally from those who did not. Our data suggest that flunarizine does not adversely affect and may favorably influence the outcome in patients with alternating hemiplegia of childhood. Additionally, the occurrence of autosomal-dominant cases of the syndrome, although rare, suggests that, in addition to mitochondrial dysfunction, genetic factors may be important.
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PMID:Alternating hemiplegia of childhood: clinical manifestations and long-term outcome. 1102 Jun 38

Georges Gilles de la Tourette's contribution to neurology goes beyond the description of the neurological disorder named after him. On December 28, 1885, he defended his doctoral thesis devoted to "gait in the diseases of the nervous system, studied by the method of imprints". In collaboration with Albert Londe, he worked for two years in Charcot's department on "a simple method applicable to both healthy and unhealthy patients", establishing the scientific and modern basis of functional exploration of human gait. The purpose was to "record the modifications of the gait and to fix them permanently, using suitable devises to guarantee not only rigorous comparison, but also to prevent observers, making use of the same method, from disputing or canceling the results completely independent of the experimenter himself". Georges Gilles de la Tourette defined the various characteristic parameters and provided the normal reference values in males and females, determining the physiological asymmetry of steps. He described spastic gait, shaking palsy, and locomotor ataxia. He distinguished between disorders of nervous control and related joint diseases. He also classified gait disorders occurring during hemiplegia. Modern development of kinetic, kinematic and biomechanical studies is a good illustration of the current relevancy of Gilles de la Tourette's contribution.
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PMID:[Georges Gilles of Tourette, pioneer of gait analysis in the nervous system diseases]. 1131 91

A previously healthy 50-year-old man developed aseptic meningoencephalitis with clinical manifestations including fever, headache, seizure, Wernicke aphasia, right hemiplegia, and blindness in the left eye. One and one-half months after remission of meningoencephalitis, marked ataxia and psychiatric symptoms became apparent. Magnetic resonance imaging revealed multiple new lesions involving the basal ganglia, thalamus, white matter, and cerebellum. Despite these developments, cerebrospinal fluid findings continued to improve except for excessive content of myelin basic protein. Within 2 weeks, steroid therapy dramatically resolved the ataxic symptoms and disseminated lesions.
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PMID:Acute disseminated encephalomyelitis following aseptic meningoencephalitis. 1153 55

The aim of this research was to identify, analyze and classify disorders in behavior which occur in the use of mirrors in patients with right cerebral damage presenting left visual spatial hemiplegia. This work was based on models of visual information processing. Seven controls and eleven patients with right cerebral damage performed a test involving grasping of an object using only specular information from a conventional mirror and then from an inverted mirror. The controls grasped up all the cubes straight away. They only experienced minor difficulty with the inverted mirror, mainly in relation to lateral displacement. The patients revealed a variety of behaviors: 1) searching for and trying to seize the object in the conventional and/or inverted mirror, 2) inversion of the paralysed side (left versus right) in the inverted mirror or the appearance of a visual spatial hemiplegia, 3) modifications in the order of grasped (from right to left, from left to right, or at random), 4) directional anomalies in the horizontal plane linked (or not) with disorders in the use of the anteroposterior space. The results of this study confirm that the patients have abnormal behavior in mirror spaces. While the characteristics of this behavior shows analogies with those described in the non-recognition of objects and/or defects in the processing of visual information for localising objects in space, they can be disassociated from them, and constitute separate syndromes. Specific terminology and taxonomy for the clinical forms of mirror agnosia and specular agnosia, of mirror paralysis and specular paralysis, and of specular ataxia are proposed.
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PMID:[Disorders of the processing of spatial information in patients with right cerebral lesions and left hemi-neglect]. 1291 75

The recent discovery of heterozygous de novo mutations in the glial fibrillary acidic protein (GFAP) gene as the cause of infantile and juvenile Alexander disease has shed new light on the long-standing debate whether the adult subtype has the same etiology as infantile and juvenile Alexander disease. A 40-year-old man presented with subacute left hemiplegia and ataxia. Cranial MRI revealed disseminated patchy white matter changes involving the corpus callosum, basal ganglia and brainstem. CSF investigation demonstrated elevated total protein but was otherwise normal. Mutation analysis of the GFAP gene was performed in the patient, his mother and healthy brother. A novel heterozygous mutation in exon 4, 681G-->C, predicting an amino acid substitution E223Q in the rod region of GFAP was detected in the patient and his mother but not in his healthy brother or 150 control chromosomes. We conclude that the patient is actually afflicted with Alexander disease. Mutation analysis of GFAP should be considered in patients with remitting neurological deficits, disseminated white matter lesions and absence of inflammatory CSF changes.
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PMID:A novel GFAP mutation and disseminated white matter lesions: adult Alexander disease? 1294 15

Herpes Simplex Encephalitis (HSE) is an uncommon but severe disease with high mortality and morbidity. The major clinical manifestations are deteriorating consciousness with confusion, drowsiness or coma, altered behaviour, convulsions and a variety of neurological signs (hemiplegia, aphasia, ataxia, etc.). An uncommon complication of HSE is Kluver Bucy syndrome (KBS), characterized by hyperorality, bulimia and changes in emotional behaviour. Neuroimaging studies frequently show an involvement of the temporal lobes and limbic areas. Another uncommon complication of HSE is central diabetes insipidus as a result of herpes simplex infection of the hypothalamus. We report two pediatric cases of HSE complicated with Kluver Bucy syndrome and central diabetes insipidus.
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PMID:[Kluver Bucy syndrome and central diabetes insipidus: two uncommon complications of herpes simplex encephalitis]. 1527 70

We report a longitudinal, prospective, multicentre cohort study designed to measure the outcomes of gastrostomy tube feeding in children with cerebral palsy (CP). Fifty-seven children with CP (28 females, 29 males; median age 4y 4mo, range 5mo to 17y 3mo) were assessed before gastrostomy placement, and at 6 and 12 months afterwards. Three-quarters of the children enrolled (43 of 57) had spastic quadriplegia; other diagnoses included mixed CP (6 of 57), hemiplegia (3 of 57), undiagnosed severe neurological impairment (3 of 57), ataxia (1 of 57), and extrapyramidal disorder (1 of 57). Only 7 of 57 (12%) could sit independently, and only 3 of 57 (5%) could walk unaided. Outcome measures included growth/anthropometry, nutritional intake, general health, and complications of gastrostomy feeding. At baseline, half of the children were more than 38D below the average weight for their age and sex when compared with the standards for typically-developing children. Weight increased substantially over the study period; the median weight z score increased from -3 before gastrostomy placement to -2.2 at 6 months and -1.6 at 12 months. Almost all parents reported a significant improvement in their child's health after this intervention and a significant reduction in time spent feeding. Statistically significant and clinically important increases in weight gain and subcutaneous fat deposition were noted. Serious complications were rare, with no evidence of an increase in respiratory complications.
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PMID:Gastrostomy tube feeding in children with cerebral palsy: a prospective, longitudinal study. 1570 29

Familial hemiplegic migraine (FHM) is an autosomal dominant disorder characterized by transient hemiplegia followed by migraine headache, and recently approximately half of FHM families have been elucidated to be caused by mis-sense mutations in P/Q-type Ca channel alpha(1)-subunit gene (CACNA1A). This subunit forms channel pore and is implicated in the regulation of membrane excitability as voltage sensor, therefore FHM is thought to be channelopathy. The CACNA1A gene is causative of episodic ataxia type-2 and of spinocerebellar atrophy type 6. Moreover, FHM with cerebellar ataxia is only associated with the mutation in CACNA1A, dysfunction of the calcium channel may cause cerebellar degeneration. New genotype and phenotype have been reported, more reports and analyses are expected.
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PMID:[Familial hemiplegic migraine]. 1577 64

The aim of this cross-sectional study was to determine the prevalence of cerebral palsy (CP) among children in Turkey between the ages of 2 and 16 years. Samples were selected from cities, towns, districts, and villages using the cluster sampling method; 41,861 children were selected. Data was collected by parental interview and physical examination. One hundred and eighty-six children were identified with CP. The prevalence of CP was determined as 4.4 per 1,000 live births and included postnatally acquired CP. Origin of CP was classified as prenatal in 49 (26.6%), perinatal/neonatal in 34 (18.5%), postnatal in 11 (5.9%), and unclassifiable in 90 participants (48.9%; data was unobtainable for two individuals). Type of CP was diplegia in 39.8% of children, hemiplegia in 28%, tetraplegia in 19.9%, ataxia in 5.9%, and dyskinetic in 6.4%. Prenatal factors were seen more frequently in the groups with a high socioeconomic status while perinatal factors were encountered more often in those with a low socioeconomic status (p<0.05). Place of residence and sex had no significant effect on the prevalence of CP (p>0.05). This cross-sectional study shows that the prevalence of CP in Turkey is higher than that in developed countries but the aetiology is probably similar. Although the high prevalence of CP in Turkey could originate from an increased level of obstetric and neonatal problems, the lack of a possible aetiological factor in approximately half the children suggests that the high level might also be due to other factors, such as genetic disorders.
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PMID:Prevalence of cerebral palsy in Turkish children between the ages of 2 and 16 years. 1670 Sep 29

The Manual Ability Classification System (MACS) has been developed to classify how children with cerebral palsy (CP) use their hands when handling objects in daily activities. The classification is designed to reflect the child's typical manual performance, not the child's maximal capacity. It classifies the collaborative use of both hands together. Validation was based on the experience within an expert group, a review of the literature, and thorough analysis of children across a spectrum of function. Discussions continued until consensus was reached, first about the constructs, then about the content of the five levels. Parents and therapists were interviewed about the content and the description of levels. Reliability was tested between pairs of therapists for 168 children (70 females, 98 males; with hemiplegia [n=52], diplegia [n=70], tetraplegia [n=19], ataxia [n=6], dyskinesia [n=19], and unspecified CP [n=2]) between 4 and 18 years and between 25 parents and their children's therapists. The results demonstrated that MACS has good validity and reliability. The intraclass correlation coefficient between therapists was 0.97 (95% confidence interval 0.96-0.98), and between parents and therapist was 0.96 (0.89-0.98), indicating excellent agreement.
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PMID:The Manual Ability Classification System (MACS) for children with cerebral palsy: scale development and evidence of validity and reliability. 1678 Jun 21


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