1/122. Chronic hepatitis b and neurogenic muscle disease: case report.A 17 year-old boy with chronic hepatitis b who developed left-sided muscle wasting is reported. When other possible known diseases as the cause of the neurogenic muscle disease were excluded it was hypothesised that there was a relation between the chronic hepatitis b infection and the neurogenic muscle disease. An immunopathogenesis could be explained by the presence of HBsAg in the cerebral spinal fluid.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/122. Subaxial cervical synovial cyst presenting with myelopathy. Report of three cases.Synovial cysts occur infrequently in the spinal canal and are most often associated with degenerative facet joints. Despite the prevalence of degenerative spinal disease, symptomatic synovial cysts are extremely uncommon. There have been only two previously reported cases of subaxial degenerative synovial cysts of the cervical spine in patients who presented with a clinical picture of spinal cord compression. The authors report three additional patients treated for degenerative cervical synovial cysts who presented with myelopathy. In all three patients the cyst was successfully excised and a good clinical outcome achieved.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
3/122. Progressive myelopathy caused by dural arteriovenous fistula at the craniocervical junction--case report.A 68-year-old male presented an unusual dural arteriovenous fistula (AVF) located at the craniocervical junction. magnetic resonance imaging revealed dilated perimedullary veins around the spinal cord at C-1 and C-2 levels, as well as high intensity signals in the spinal cord on T2-weighted images. Vertebral angiography identified an AVF at the point where the right vertebral artery penetrates the dura. The fistula was a single and direct communication between the vertebral artery and the spinal vein. Surgical interruption of the fistula at its venous side resulted in prompt improvement of both motor and sensory signs and symptoms.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
4/122. Acute care pediatric electromyography.The recognition of uncommon pediatric motor unit disorders or unusual clinical presentations of common illnesses, such as guillain-barre syndrome (GBS), have increased the need for electromyography (EMG) in childhood critical care units. There are two different clinical sets, one appropriate to newborns and infants and the other to older children. Some illnesses that present as an acute floppy infant are not found in the differential diagnosis of motor unit disorders in the older child or adult. These include spinal muscular atrophy, postvaccine poliomyelitis, intrauterine GBS, infantile botulism, and severe myopathies, such as myotonia dystrophy, and some glycogen storage diseases. An appreciation of the neurophysiological maturational norms is essential to an effective pediatric EMG consultation for children ages 0-3 years. Additionally, the neuromuscular complications of extended intubation and sepsis in children are gaining broader recognition. An increased dialogue between clinical neurophysiologists and pediatric neurologists and intensivists in both neonatal and pediatric intensive care units is essential.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
5/122. Neuromuscular disorders presenting as congenital bilateral vocal cord paralysis.Congenital bilateral vocal cord paralysis (BVCP) can be associated with an underlying neuromuscular disorder, and may present before other features of the neuromuscular disorder become apparent. All infants less than 12 months of age presenting with BVCP between July 1987 and July 1999 at the Royal Children's Hospital, Melbourne, in whom a neuromuscular disorder was subsequently diagnosed were followed. Three children in whom BVCP was diagnosed soon after birth and before recognition of an underlying neuromuscular disorder were identified. All presented with upper airway obstructive symptoms at birth, had a diagnosis of bilateral abductor vocal cord paralysis made at awake flexible laryngoscopy, and had no underlying structural laryngeal abnormality on microlaryngoscopy and bronchoscopy. Two children required a tracheostomy, and 1 child was weaned from nasopharyngeal continuous positive airway pressure after 3 weeks. Subsequent neuromuscular symptoms were recognized between 4 months and 7 years later, leading to diagnoses of facioscapulohumeral myopathy, spinal muscular atrophy, and congenital myasthenia gravis. In each case, the prognosis for recovery from symptoms related to BVCP reflected that of the underlying neuromuscular disorder. This experience suggests that congenital BVCP may be a feature of an unrecognized neuromuscular condition. This possibility should be considered particularly in the presence of associated neurodevelopmental or neuromuscular dysfunction, or in cases in which BVCP is progressive.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
6/122. 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 = spinal (Clic here for more details about this article) |
7/122. 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 = 6keywords = spinal (Clic here for more details about this article) |
8/122. Cardiac involvement in the Kugelbert-Welander syndrome.Two cases of the Kugelberg-Welander syndrome (juvenile form of progressive spinal muscular atrophy) associated with cardiomyopathy and cardiomegaly are presented. The first patient, a 24 year old man, had atrial flutter with complete atrioventricular (A-V) block due to A-H block. echocardiography revealed an increase in the left atrial and right ventricular dimensions. The second patient was a 26 year old man whose electrocardiogram revealed an A-V junctional rhythm, deep Q wave in leads I, aVL and V5 to V6 and an RS pattern in lead V1. Histologic examination of the myocardium in Case 2 showed slight interstitial fibrosis. review of previously reported cases shows that (1) the atrium, the ventricular myocardium and A-V conducting tissue may be involved, and (2) atrial arrhythmias, A-V conduction disturbances and congestive heart failure may occur in the Kugelberg-Welander syndrome.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
9/122. Extrapyramidal system involvement in motor neuron disease.Three cases of motor neuron disease (MND), in which neuropathological findings were atypical, are reported. The first case manifested widespread and severe degeneration of the spinal cord, as in spinal fibrosis. Case 2 revealed severe degeneration of the pyramidal tract with many spheroids, which made it difficult to differentiate from primary amyotrophic lateral sclerosis. The last case revealed degeneration of the nigro-pallido-luysian system, even though no clinical manifestation of extrapyramidal and/or cerebellar symptoms had been noted throughout the clinical course. In MND, degeneration might occur in various locations other than the motor system.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
10/122. Neuromuscular disorders in severe acute respiratory syndrome.OBJECTIVE: To delineate and clarify neuromuscular disorders in patients with probable severe acute respiratory syndrome (SARS). DESIGN: Case series with follow-up ranging from 3 weeks to 2 months. SETTING: National taiwan University Hospital, Taipei. patients: We investigated 4 patients with SARS who had concomitant neuromuscular problems. A diagnosis of SARS was based on the demonstration of serum coronavirus antibodies. Clinical presentations, laboratory results, electrophysiologic findings, and follow-up conditions were determined. RESULTS: patients developed neuromuscular problems approximately 3 weeks after the onset of SARS. Two women experienced motor-predominant peripheral nerve disorders. A man developed myopathy and a third woman experienced neuropathy and myopathy. cerebrospinal fluid obtained from 2 patients with neuropathy disclosed normal protein content and the absence of pleocytosis and SARS coronavirus antibodies. Both patients with myopathy had elevated serum creatine kinase levels. A rapid clinical and electrophysiologic improvement was evident during follow-up examinations, with a good prognosis. CONCLUSIONS: The neuromuscular problems in patients with SARS are considered to be critical-illness polyneuropathy or myopathy, possibly coexistent. Further pathological and microbiological studies are necessary to determine the relationship between SARS coronavirus and neuromuscular problems.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
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