1/23. Diaphragmatic spinal muscular atrophy with bulbar weakness.We present the clinical and histopathological features of a child affected by diaphragmatic spinal muscular atrophy. The child was born with mild distal arthrogryposis, mild hypotonia and developed marked diaphragmatic and bulbar muscle weakness in the first week of life. Electrophysiological and pathological investigations performed at presentation were not conclusive, while the investigations performed at 3 months showed a clear neurogenic picture. Genetic studies excluded involvement of the SMN gene, or of other genes located on chromosome 5q, confirming that this syndrome represents a different entity from typical proximal spinal muscular atrophy.- - - - - - - - - - ranking = 1keywords = spinal muscular atrophy, muscular atrophy, atrophy, spinal (Clic here for more details about this article) |
2/23. Cerebrospinal trypanosomiasis masquerading as pulmonary infectious disease in a 1-year-old boy.A 1-year-old boy with cerebrospinal trypanosomiasis presented with severe respiratory symptoms, hepatosplenomegaly and no neurological signs of trypanosomiasis. Agitation and high fever on the 2nd day in hospital prompted a lumbar puncture and trypanosomes were recovered from the cerebrospinal fluid.- - - - - - - - - - ranking = 0.0011427433076585keywords = spinal (Clic here for more details about this article) |
3/23. Postoperative use of a cough-assist device in avoiding prolonged intubation.Postoperative management following major surgery in patients with neuromuscular disorders associated with scoliosis is frequently complicated by sputum retention and ventilatory failure. This report demonstrates the successful perioperative management of an 11-yr-old boy with type II spinal muscular atrophy undergoing a single-stage posterior spinal fusion procedure. Use of an MI-E device was able to successfully treat sputum retention and avoid a tracheostomy.- - - - - - - - - - ranking = 0.16685712388461keywords = spinal muscular atrophy, muscular atrophy, atrophy, spinal (Clic here for more details about this article) |
4/23. Successful pregnancy in a patient with spinal muscular atrophy and severe kyphoscoliosis.pregnancy imposes a load on the respiratory system that is usually easily assumed because of alterations in the thoracoabdominal architecture. It is presumed that the respiratory mechanical disadvantage of severe kyphoscoliosis and the muscle weakness of spinal muscular atrophy impede these adaptations sufficiently to preclude a successful gestation. We report the case of a successful pregnancy in a woman with spinal muscular atrophy, severe uncorrected scoliosis, and the lowest spirometric values reported in the literature without the use of ventilatory support. This patient demonstrates that women with severe kyphoscoliosis and a profound ventilatory limitation can carry a successful pregnancy well into the third trimester without requiring full ventilatory support.- - - - - - - - - - ranking = 1keywords = spinal muscular atrophy, muscular atrophy, atrophy, spinal (Clic here for more details about this article) |
5/23. Cervical spinal cord hemorrhage secondary to neonatal alloimmune thrombocytopenia.Neonatal alloimmune thrombocytopenia with intracranial hemorrhage is a reported phenomenon. While most of the hemorrhages are noted to be either intraventricular or intraparenchymal, the authors describe the case of a fourth-ventricle hemorrhage with extension into the spinal column down the cervical spinal cord secondary to maternal anti-human platelet antigen (HPA-1a) antibody.- - - - - - - - - - ranking = 0.0011427433076585keywords = spinal (Clic here for more details about this article) |
6/23. A new extra-vertebral treatment model for incomplete spinal cord injuries.Advances made in recent times in spinal cord injury repair research will soon take us toward a cure in paraplegics. But what are the prospects for quadriplegics? Certain fundamental issues make treatment approaches to quadriplegia different and difficult. Injury at cervical region poses additional problems for any surgical intervention with life-threatening risks of i) endangering respiratory function, ii) cavitation, cysts, and syringomyelia formation extending cephalad to the injury, and iii) mid-lower cervical injuries, lower motor neuron death, and the resultant degeneration of brachial plexus axons would still leave the upper limbs denervated and paralyzed even as treatment procedures might successfully salvage the lower limbs. With these apparently insurmountable impediments in quadriplegic cord repair, it would be wise to turn to alternative treatment strategies. Conventional treatment models since the days of Ralph Gerard (1940) have all used intra-vertebral procedures. We present here a plausible extra-vertebral repair model suitable for incomplete cord injuries at cervical, thoracic, and lumbar levels. The procedure consists of identifying the extent of viable grey-white matter in the injured area and to utilize it efficiently as a "neural tissue bridge." Next, labile state is induced by using botulinum toxin/colchicine (Krishnan, 1983, 1991; Krishnan et al., 2001 a,b) and Ca channel blockers in the motorsensory nerve terminals of polisegmentally innervated skeletal muscles that "bridge" the injured cord segments. This would retrogradely induce a redundant state of intra-spinal growth of nerve terminals and new synaptic connections within those viable neural tissues, as well as promote effective relinking of the injured cord ends and enhance motor-sensory recovery.- - - - - - - - - - ranking = 0.0011427433076585keywords = spinal (Clic here for more details about this article) |
7/23. Long-term follow-up of home mechanical ventilation in young children with spinal cord injury and neuromuscular conditions.OBJECTIVES: To provide outcomes of two decades of experience in home ventilation of children with spinal cord injury and neuromuscular conditions. STUDY DESIGN: Data were collected through chart review and interviews on 39 children who had become ventilator-dependent before their 6th birthday; 23 children had neuromuscular diseases and 16 had spinal cord injuries. RESULTS: patients required an average of 0.7 rehospitalizations per year. There were 8 deaths. survival rates were 97% at 1 year, 97% at 3 years, 84% at 5 years, and 71% at 10 years. Thirty children attended school, 13 were in regular school (1 at university level), 5 were home-schooled, 5 were in special education schools, and 5 were in regular school with some special education classes. One graduated high school, and another graduated university and received a graduate degree. Three children had progressive weakness. Two gained significant muscle strength. CONCLUSIONS: Our experience showed that these patients can be discharged to home with low morbidity and mortality rates and successful reintegration into the community.- - - - - - - - - - ranking = 0.0011427433076585keywords = spinal (Clic here for more details about this article) |
8/23. Upper airway dysfunction in olivopontocerebellar atrophy.We report the findings in a patient known to have olivopontocerebellar atrophy who developed respiratory distress, inspiratory stridor, and maximum inspiratory and expiratory flow volume loops. Treatment with carbidopa-levodopa gave symptomatic relief.- - - - - - - - - - ranking = 0.0010627462546122keywords = atrophy (Clic here for more details about this article) |
9/23. Continuous subarachnoid analgesia in two adolescents with severe scoliosis and impaired pulmonary function.We report postoperative pain management of two adolescents after upper abdominal procedures, one with Hurler-Scheie syndrome and a second with Duchenne muscular dystrophy, and both had progressive spinal scoliosis with poor pulmonary function. A combined technique of subarachnoid and general anesthesia was used during surgery. Postoperative administration of small intermittent doses of subarachnoid morphine produced profound analgesia, which eliminated the need for systemic opioids, restored preoperative arterial oxygenation within 48 hours after the operation, and expedited postoperative recovery.- - - - - - - - - - ranking = 0.00019045721794308keywords = spinal (Clic here for more details about this article) |
10/23. Spondyloepiphyseal dysplasia congenita. Caesarean section under epidural anaesthesia.Spondyloepiphyseal dysplasia congenita is a rare condition with several features of concern to the anaesthetist. The patients are of extremely short stature and the presence of kyphoscoliosis may lead to significant respiratory impairment. Cervical vertebral body changes can result in spinal cord compression and laryngotracheal stenosis may be present. The management of such a patient presenting for elective Caesarean section under epidural anaesthesia is described.- - - - - - - - - - ranking = 0.00019045721794308keywords = spinal (Clic here for more details about this article) |
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