1/42. Computer modeling of the pathomechanics of spastic hip dislocation in children.Spastic muscles about the hip cause subluxation, dislocation, and lead to acetabular dysplasia. Spastic hip disease occurs when the muscles about the hip exert forces that are too high or in the wrong direction or both. To determine the role of the hip forces in the progression of spastic hip disease and the effect of both muscle-lengthening and bony reconstructive surgeries, a computerized mathematical model of a spastic hip joint was created. The magnitude and direction of the forces of spastic hips undergoing surgery were analyzed preoperatively and postoperatively to determine which procedure is best suited for the treatment of spastic hip disease. The muscle-lengthening procedures included (a) the adductor longus, (b) the psoas, iliacus, gracilis, adductor brevis, and adductor longus, and (3) the psoas, iliacus, gracilis, adductor brevis, adductor longus, semimembranosus, and semitendinosus. The bony reconstructive and muscle-lengthening procedures included (a) lengthening the psoas, iliacus, gracilis, adductor brevis, adductor longus, semimembranosus, and semitendinosus combined with changing femoral neck anteversion from 45 to 10 degrees , (b) lengthening of the psoas, iliacus, gracilis, adductor brevis, adductor longus, semimembranosus, and semitendinosus combined with changing neck-shaft angle from 165 to 135 degrees , and (c) lengthening of the psoas, iliacus, gracilis, adductor brevis, adductor longus, semimembranosus, and semitendinosus combined with changing femoral neck anteversion from 45 to 10 degrees and neck-shaft angle from 165 to 135 degrees . Results show that a child with spastic hip disease has a hip-force magnitude 3 times that of the a child with a normal hip in the normal physiologic position. Based on this mathematical model the best to normalize the magnitude of the hip-joint reaction force, the muscles to be lengthened should include the psoas, iliacus, gracilis, adductor brevis, and the adductor longus. To normalize the direction of the hip force, the extremity should be positioned in the normal physiologic position. The impact of decreasing the femoral anteversion or femoral neck-shaft angle or both had little additional effect on the direction or magnitude of hip forces.- - - - - - - - - - ranking = 1keywords = impact (Clic here for more details about this article) |
2/42. A patient with cerebral palsy whose mother had a traffic accident during pregnancy: a diffuse axonal injury?A 16-year-old girl had spastic cerebral palsy (CP) with triplegia and focal epilepsy. The patient's past history included her mother's lower abdominal trauma caused by a traffic accident at the 7th month of gestation. brain examination with magnetic resonance imaging (MRI) revealed encephalomalacia at the bilateral parieto-temporal lobes and the left caudate nucleus, segmental narrowing of the splenium of the corpus callosum, dilatation of the left lateral ventricle and an abnormally high intensity at the right posterior portion of the internal capsule. These findings might indicate a diffuse axonal injury (DAI), but not an asphyxic brain damage. In this patient, CP might be caused by an intrauterine DAI when her mother was involved in the accident.- - - - - - - - - - ranking = 15.874291973518keywords = epilepsy (Clic here for more details about this article) |
3/42. cerebral palsy and pyruvate dehydrogenase deficiency: identification of two new mutations in the E1alpha gene.Pyruvate dehydrogenase (PDH) complex deficiency, a common cause of congenital lactic acidosis, is mostly due to mutations in the X-linked gene coding for the E1alpha subunit of the complex. We have studied two unrelated girls presenting a static encephalopathy with spastic quadriplegia, microcephaly and seizures and in one girl, hypocalcaemia, a new finding in PDH complex deficiency. PDH deficiency was diagnosed in adolescence and both girls had low PDH complex activity in muscle but normal amounts of all subunits on Western blotting, and a normal lactate/pyruvate ratio in blood and CSF. mutation analysis of the E1alpha gene at the cDNA or dna level revealed an arginine to histidine substitution at amino acid position 288 (R288H) in the girl with hypocalcaemia and a 12 bp insertion, predicting a four amino acid duplication at the c-terminal end of the protein in the second girl. They both carried a normal and a mutated E1alpha gene and X-inactivation studies showed skewed patterns. CONCLUSION: mutation identification in pyruvate dehydrogenase complex deficiency remains important especially for the determination of the recurrence risk and for reliable genetic counselling in couples with an affected child.- - - - - - - - - - ranking = 6.7487765921604keywords = seizure (Clic here for more details about this article) |
4/42. Repetitive sleep starts in neurologically impaired children: an unusual non-epileptic manifestation in otherwise epileptic subjects.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.- - - - - - - - - - ranking = 13.497553184321keywords = seizure (Clic here for more details about this article) |
5/42. Reduced accommodative function in dyskinetic cerebral palsy: a novel management strategy.A 9-year-old boy with dyskinetic cerebral palsy secondary to neonatal encephalopathy is described. He presented with blurring of near vision which had begun to impact on his school work. Objective assessment of accommodation showed that very little was present, although convergence was almost normal. The near-vision symptoms were completely removed and reading dramatically improved with the provision of varifocal spectacles. Varifocal lenses provide an optimal correction for far, intermediate (i.e. for computer screens), and near distances (i.e. for reading). Managing this type of patient with varifocal spectacles has not been previously reported. It is clearly very important to prescribe an optimal spectacle correction to provide clear vision to optimize learning.- - - - - - - - - - ranking = 1keywords = impact (Clic here for more details about this article) |
6/42. Improvement of sleep apnea in a patient with cerebral palsy.Intrathecal baclofen (ITB) can reduce spasticity in adults and children with cerebral palsy. Benefits of ITB therapy include improved Ashworth scores, activities of daily living, and mobility. The impact of ITB therapy on sleep apnea in patients with cerebral palsy has not been reported. This case report describes a 29-yr-old female with mixed spastic athetoid quadriparetic cerebral palsy with dystonia, gross motor function IV, who had sleep apnea, requiring nightly continuous positive airway pressure. She received ITB with the goal to improve her wheelchair positioning and decrease her excessive movements. After the initiation of the ITB, reduction of her spasticity and dystonia was noted, as well as improvement of her sleep apnea. This case suggests that ITB therapy may improve respiratory function through reduction of respiratory muscle spasticity.- - - - - - - - - - ranking = 1keywords = impact (Clic here for more details about this article) |
7/42. DDAVP-associated hyponatremia.More than 250,000 patients with nocturnal enuresis have been treated with DDAVP in the united states since 1989. It adequately controls nocturnal enuresis in over half of patients with significant improvement in their quality of life. Although the overall incidence of adverse effects associated with treatment of nocturnal enuresis with DDAVP is low, it is not a benign drug particularly when used in patients at extreme of age. A review of the literature and the present case demonstrate that the potential risk factors for hyponatremia following administration of DDAVP include hepatic disease, surgery, stress, pain, renal disorder, excessive fluid intake, and increased dose of DDAVP. Potentially serious side effects of DDAVP administration such as hyponatremia and seizure may be prevented by close monitoring of serum electrolytes, urine output, as well as fluid restriction and avoidance of solutions with low-sodium content.- - - - - - - - - - ranking = 6.7487765921604keywords = seizure (Clic here for more details about this article) |
8/42. Extraction as a treatment alternative follows repeated trauma in a severely handicapped patient.Handicapped patients with protruding maxillary incisors are prone to repeated dental trauma. A 13-year-old girl with cerebral palsy, severe mental retardation and seizure disorder was referred to our department for restoring the traumatized anterior teeth. Despite drug combination, the frequency of seizure attack was around 10 times a month. The oral examination showed multiple caries, gingival hyperplasia, class II malocclusion with 14 mm overjet and deep overbite. During the first 3 years of a 7-year follow-up period, six episodes of anterior tooth trauma due to seizure attack occurred. The trauma-related treatment performed included endodontic therapy, multiple composite restorations, apical repositional flap, and finally extraction of all four upper incisors with fabrication of a semi-fixed band-retained denture. The denture restored normal overbite and overjet with improved esthetics. For 4 years following the fabrication of denture, no trauma occurred to the anterior teeth in later seizure attacks. Considering inadequate control of seizure disorder, little ability of the patient to receive comprehensive orthodontic treatment, poor prognosis of restorations, and possible future injuries, the removal of non-functional, nonesthetic, trauma-susceptible incisor teeth can be justified as an alternative to tooth preservation.- - - - - - - - - - ranking = 299.93303092905keywords = seizure disorder, seizure (Clic here for more details about this article) |
9/42. Pseudostatus epilepticus in childhood.Pseudostatus epilepticus in childhood has not been well reported in the literature. We describe the clinical presentation and management of a 9-year-old child with well-controlled epilepsy who presented in a prolonged period of pseudoseizures. intensive care management over a number of weeks with multiple high-dose antiepileptic drugs, anesthesia, and ventilation at a tertiary care pediatric center was performed before the diagnosis of pseudostatus epilepticus was made. Initiation of family counseling and behavior therapy after diagnosis of the nonepileptic nature of the protracted paroxysmal events with video telemetry in our pediatric epilepsy unit was followed by remission. The patient reported herein illustrates the risks of iatrogenic morbidity that may result from a delay in the diagnosis of pseudoseizures and pseudostatus epilepticus in childhood.- - - - - - - - - - ranking = 45.246137131356keywords = epilepsy, seizure (Clic here for more details about this article) |
10/42. Neuromuscular scoliosis: causes of deformity and principles for evaluation and management.scoliosis is commonly associated with a variety of neuromuscular disorders including conditions affecting upper and lower motor neurons as well as myopathies. Contained herein is a discussion of the spectrum of neuromuscular disorders that have been associated with scoliosis and related spinal deformities. Management, including surgical treatment in such patients, is summarized including indications, expectations, and impact on trunk balance, pulmonary function, and appearance.- - - - - - - - - - ranking = 1keywords = impact (Clic here for more details about this article) |
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