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

Successful cortical recordings of somatosensory-evoked potentials (SEPs) to posterior tibial nerve (PTN) stimulation were obtained in 21 (87.5%) for P1 and 22 (91.7%) for N1 of 24 infants who were followed up for at least 3 years and had a normal outcome. There were linear decreases with increasing post menstrual age in both P1 and N1 peak latency. Of the four cases with diplegia later, three showed definite abnormalities, no responses and delayed latency in PTN SEPs respectively, however, the other case showed normal responses. Of the three cases with mental retardation, two showed relatively long latency and borderline responses respectively, and the other case showed normal responses. As the pathway of PTN SEPs traverses the periventricular area of the brain likely to be affected by ischemic lesions in premature infants, abnormalities in the responses might indicate a later motor disorder.
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PMID:Cortical somatosensory evoked potentials to posterior tibial nerve stimulation in newborn infants. 887 48

Foix-Chavany-Marie syndrome (FCMS) is characterized by facio-linguo-masticatory diplegia in the absence of limb weakness. The most common cause is a cortical lesion resulting from a stroke but a congenital form has been reported. We present the case of a 53-year-old man who was admitted to hospital with worsening dysphagia which was know to have been present together with anarthria and facial palsy, since birth. He demonstrated features of FCMS with pseudobulbar palsy and unaffected reflexes and automatic responses. Cranial CT and MRI scans showed bilateral opercular lesions of CSF intensity in continuity with the lateral ventricles. We conclude that this case of static FCMS for over 50 years may represent a 'pure' form of congenital FCMS with motor symptomatology and unaccompanied by mental retardation or epilepsy.
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PMID:Pure congenital Foix-Chavany-Marie syndrome. 935 33

Congenital bilateral perisylvian syndrome (CBPS) is a recently described, neuronal migration disorder, characterized by pseudobulbar palsy, epilepsy and mental retardation and bilateral perisylvian dysplasia. A 15-year-old boy was diagnosed with CBPS according to the typical clinical, and magnetic resonance imaging (MRI) features. The patient was suffering from atypical absence seizures, repeating daily in spite of antiepileptic drug therapy, since age 7 years. He had also experienced rare generalized tonic-clonic seizures and complex partial seizures. Neurological examination showed severe restriction of tongue movements, severe dysarthria, dysphagia, facial diplegia, mild pyramidal signs and moderate mental retardation. A computed tomographic (CT) scan demonstrated bilateral perisylvian enlargement. The diagnosis was corrected with MRI after six years. Frequent irregular generalized spike and wave abnormalities and focal sharp and slow waves over the posterior regions of both hemispheres were shown by electroencephalograms (EEG). The patient was treated with Na-Valproate, carbamazepine and lamotrigine but did now show any significant change in seizure frequency in the eight-year follow-up period. Intractable seizures, mental retardation and particularly congenital pseudobulbar palsy suggest this congenital entity. Those patients who exhibit these typically clinical features, must have MRI.
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PMID:A clinically recognizable neuronal migration disorder: congenital bilateral perisylvian syndrome. Case report with long-term clinical and EEG follow-up. 953 Sep 47

Multiple system organ failure after cardiac surgery in children is a severe complication with unknown mid- and long-term sequelae. We therefore evaluated 11 children (aged 20-126 mo, median: 67 mo) having survived multiple system organ failure after cardiac operations for congenital cardiac defects in a cross-sectional follow-up study 12-76 mo (median: 32 mo) after surgery. Clinical and laboratory examinations included cardiac, pulmonary, renal, hepatic, neurological and psychological function tests. All patients had adequate cardiac function. Lung mechanics were abnormal in three children and glomerular renal function was abnormal in two patients. Slight elevation of gamma-glutamyl transpeptidase and coagulation factor deficiency was present in six and seven patients, respectively (five of whom had undergone the Fontan operation). Severe neurological sequelae such as diplegia (n = 1) and mental retardation (n = 1) were observed in two patients. In addition, five children presented delayed motor, graphomotor and/or speech development. Two children were found to have abnormal intelligence. We conclude that with the exception of neurological impairment, mid-term sequelae of multiple system organ failure after cardiac surgery in children are mild. However, longer follow-up using an appropriate control group is mandatory.
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PMID:Mid-term follow-up after multiple system organ failure following cardiac surgery in children. 1059 26

Sleep starts, also called hypnagogic or hypnic jerks, are bilateral, sometimes asymmetric, usually single, brief body jerks that coincide with sleep onset. We describe sleep starts occurring repetitively in three epileptic children with spastic-dystonic diplegia and mental retardation. Repetitive sleep starts began at age 18 months in two children and at 9 months in the third. All three children had had feto-neonatal asphyxia; two presented with spastic and one with dystonic tetraparesis. One had West syndrome and two had partial motor seizures in the first year of life. Seizures were controlled in all three patients by antiepileptic drug therapy. Video/EEG recordings of all the children during the afternoon nap revealed clusters of sleep starts during the transition between wakefulness and sleep. Cluster lasted 4-15 min and comprised from twenty to twenty-nine contractions. The EEG counterpart of the event sometimes showed an arousal response, at times inducing complete awakening. Repetitive sleep starts should be recognized and clearly differentiated from epileptic seizures, especially if they appear in epileptic subjects. In neurologically compromised patients, they could represent an intensification of an otherwise normal event, due to the lack of strong inhibitory influence of the pyramidal tract resulting from the pyramidal lesion.
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PMID:Repetitive sleep starts in neurologically impaired children: an unusual non-epileptic manifestation in otherwise epileptic subjects. 1093 35

This article deals with the clinical profile of children with cerebral palsy and epilepsy, and to study the clinical predictors of response to anti-epileptic drugs. It is a prospective hospital based follow-up study. All the children who presented with cerebral palsy and history of seizure (other than neonatal seizures) over a period of one year were included. Seizures were classified according to ILAE classification. An EEG was obtained in all cases. Neuroimaging was done in all patients. Eighty-five patients were studied and followed for minimum of 12 months. Perinatal factors accounted for 62 (72.3%) cases. The motor deficits seen were quadriparesis (n = 64), hemiplegia (n = 12) and diplegia (n = 9). Associated mental retardation was seen in 80.9% patients with quadriparesis. A predominance of generalised epilepsy was seen with generalised tonic clonic seizures (32.9%) followed by mycolonic seizures (30.6%) and localisation related epilepsy (24.7%). The patients with quadriparesis were more likely to have generalised epilepsy and 52.4% of them required two or more anti-epileptic drugs for control of seizures. Patients with hemiplegia had localisation related epilepsy in 83.3% of cases. On multivariate analysis presence of quadriparesis, microcephaly, mental retardation and myoclonic epilepsy were found to predict the poor response to AED. Epilepsy in patients with cerebral palsy is of severe nature and difficult to control. Presence of quadriparesis, mental retardation and myoclonic seizures was predictive of poor response to anti- epileptic drugs.
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PMID:Epilepsy in children with cerebral palsy. 1128 76

Primary varicella-zoster infection is very common during childhood and few patients develop complications. The most frequent complications are bacterial infection of the lesions, laryngitis and varicella pneumonia. In the nervous system it can produce encephalitis and especially cerebellitis. We describe a case of primary varicella-zoster induced rhabdomyolysis in a 5-year-old girl with mental retardation, microcephalia and mild diplegia who, in the context of varicella infection, presented extreme muscular weakness and prostration. Blood and urine tests showed high creatine phosphokinase concentrations and myoglobinuria. The patient received aggressive intravenous hydration. Evolution was favorable with no renal failure. Rhabdomyolysis can produce life-threatening complications such as renal failure, intravascular disseminated coagulation and hyperkaliemia. The disease can be precipitated by alcohol ingestion, compression injury and generalized seizures. Infectious etiology is less common. Few reports have been published on primary varicella-zoster induced rhabdomyolysis but, because creatine phosphokinase concentrations are not routinely performed in varicella infection, very mild cases might have been under-diagnosed. Despite its rarity, this disease should be considered in cases of infection, since early treatment with hyperhydration can prevent complications.
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PMID:[Varicella induced rhabdomyolysis]. 1157 48

Cystic periventricular leukomalacia refers to necrosis of the white matter in a characteristic distribution dorsal and lateral to the external angles of the lateral ventricles in preterm infants. The pathogenesis includes either hypoxic-ischaemic lesions resulting from impaired perfusion at the vascular border zones or the role of intra-amniotic infection with toxic effects of endotoxins and cytokines on oligodendrocytes. This overview illustrates the pathogenic theories, risk factors, diagnosis by cranial ultrasonography, and the actual classification. Cystic periventricular leukomalacia is the most severe and frequent cause of cerebral palsy in preterm infants and is almost constantly associated with serious subsequent neuromotor impairments such as diplegia or tetraplegia. Dependant on site and extension of the cysts additionally visual impairments, seizure disorders, hearing impairments, mental retardation, and microcephaly are observed.
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PMID:[Cystic periventricular leukencephalomalacia]. 1186 81

To study the spectrum of epilepsy in children with cerebral palsy, 105 consecutive children with cerebral palsy and active epilepsy, between 1 and 14 years of age, were studied prospectively. A detailed history and examination, electroencephalography (EEG), and computed tomography (CT) were done in all cases. The social quotient was assessed using the Vineland Social Maturity Scale. A retrospective cohort of 452 cases of cerebral palsy was studied to find the prevalence of epilepsy in cerebral palsy. A control group of 60 age-matched children with cerebral palsy but no epilepsy was also studied for comparison of the social quotient. Of the 105 children, 65 were male, 40 of 105 (38%) had a history of birth asphyxia. The mean age of onset of seizures was 18.9 months; 64 (60.95%) had seizure onset before 1 year of age. Children with myoclonic seizures (P < .05) and infantile spasms (P < .01) had seizure onset significantly early in life. Generalized seizures were the most common, followed by partial seizures, infantile spasms, and other myoclonic seizures. Seizures were controlled in 45 (58.1%) children, and polytherapy was required in 40 children. EEG and CT abnormalities were seen in 70.5% and 61% of the children. Seizure control was achieved in 74% of the patients with a normal to borderline social quotient compared with 48.7% with a social quotient less than 70. Social quotient values had a positive correlation with age of onset of seizures (P < .01) and with better control of seizures (P < .01). Of the cohort of 452 children, 160 (35.4%) had epilepsy. The maximum incidence (66%) was seen in children with spastic hemiplegia, followed by quadriplegia (42.6%) and diplegia (15.8%). Epilepsy in cerebral palsy is seen in about one third of cases; it is often severe and difficult to control particularly in children with mental retardation.
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PMID:Epilepsy in children with cerebral palsy. 1273 42

Bilateral hand skill assessment with a computerised version of the Peg Moving Task, and neuropsychological testing, were performed in 30 children aged 7 to 8 years with spastic cerebral palsy (CP) and without mental retardation, diplegia (n = 10), right hemiplegia (n = 10), or left hemiplegia (n = 10), and in 30 controls. Compared to controls: (i) 30% of the hemiplegic children showed impairment of the unaffected hand and 70% of the diplegic children showed impairment in both hands; (ii) children with CP were impaired only in oral repetition and in visual-motor tasks. Results of neuropsychological testing were not significantly different between the three groups of children with CP. Right minus left asymmetry in hand skill was not related to neuropsychological testing; however, degree of impairment of the right hand was associated with phonological and metaphonological skills, and of the left hand with visuospatial and counting performance. Hand skill was related to the ability to perform many daily living manual activities. It is concluded that impairment of hand function, rather than the side of the more affected hand, is related to neuropsychological deficits in children with cerebral palsy.
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PMID:Manual skill, hand skill asymmetry, and neuropsychological test performance in schoolchildren with spastic cerebral palsy. 1584 32


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