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Paraplegia
Kinds of Paraplegia Selected AbstractsParaplegia associated with brucellosis involving the anterior lumbrosacral nerve rootsJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2003Umit Hidir Ulas Abstract We report the case of a 21-year-old man with paraplegia due to brucellosis involvement of lumbosacral anterior roots. Lumbosacral magnetic resonance imaging showed contrast enhancement of anterior roots and the anterior part of duramater. Conduction block was found at the level of the lumbosacral anterior roots by electrophysiological studies, including magnetic stimulation study. Wright agglutination, Rose Bengal tests and bacterial culture obtained from cerebrospinal fluid confirmed the diagnosis of neurobrucellosis. Oral administration of ceftriaxon with additional rifampin was effective, and after 3 months of treatment, laboratory data resolved and clinical signs partially improved. [source] Paraplegia associated with combined spinal-epidural anaesthesia caused by preoperatively unrecognized spinal vertebral metastasisACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 9 2002A. Kararmaz We describe a case of paraplegia following combined spinal-epidural anaesthesia. It was postoperatively determined that a tumour of the vertebrae which was compressing the spinal cord was responsible for this complication. We suggest that the pre-existing pathology of the spine must be borne in mind as a differential diagnosis of acute postoperative paraplegia. [source] Development and Experimental Identification of a Biomechanical Model of the Trunk for Functional Electrical Stimulation Control in ParaplegiaNEUROMODULATION, Issue 4 2008Ingenieur Michele Vanoncini ABSTRACT Objectives., Theoretic modeling and experimental studies suggest that functional electrical stimulation (FES) can improve trunk balance in spinal cord injured subjects. This can have a positive impact on daily life, increasing the volume of bimanual workspace, improving sitting posture, and wheelchair propulsion. A closed loop controller for the stimulation is desirable, as it can potentially decrease muscle fatigue and offer better rejection to disturbances. This paper proposes a biomechanical model of the human trunk, and a procedure for its identification, to be used for the future development of FES controllers. The advantage over previous models resides in the simplicity of the solution proposed, which makes it possible to identify the model just before a stimulation session (taking into account the variability of the muscle response to the FES). Materials and Methods., The structure of the model is based on previous research on FES and muscle physiology. Some details could not be inferred from previous studies, and were determined from experimental data. Experiments with a paraplegic volunteer were conducted in order to measure the moments exerted by the trunk-passive tissues and artificially stimulated muscles. Data for model identification and validation also were collected. Results., Using the proposed structure and identification procedure, the model could adequately reproduce the moments exerted during the experiments. The study reveals that the stimulated trunk extensors can exert maximal moment when the trunk is in the upright position. In contrast, previous studies show that able-bodied subjects can exert maximal trunk extension when flexed forward. Conclusions., The proposed model and identification procedure are a successful first step toward the development of a model-based controller for trunk FES. The model also gives information on the trunk in unique conditions, normally not observable in able-bodied subjects (ie, subject only to extensor muscles contraction). [source] Paraplegia as the presenting manifestation of extramedullary megakaryoblastic transformation of previously undiagnosed chronic myelogenous leukemiaAMERICAN JOURNAL OF HEMATOLOGY, Issue 2 2007Barbara J. Bryant Abstract Extramedullary tumors, also known as granulocytic sarcomas (GS), occur most frequently in acute myelogenous leukemia (AML). They may signal the onset of the accelerated phase of chronic myelogenous leukemia (CML) or the blastic transformation of a myeloproliferative disorder. Occasionally, a GS may be the presenting sign of undiagnosed AML, and rarely the presenting sign of undiagnosed CML or aleukemic leukemia. Paraplegia due to a spinal cord GS is an extremely rare presentation of undiagnosed leukemia. This is the first case report of paraplegia as the presenting manifestation of extramedullary megakaryoblastic transformation of previously undiagnosed CML. A 53-year-old woman reported back pain for 6 days, rapidly progressing to paraplegia. Physical examination noted a large abdominal mass and flaccid paralysis in both lower extremities. Spinal MRI revealed a T4,T6 vertebral mass causing spinal stenosis and cord compression. Tumor debulking and laminectomy were performed emergently. The tumor consisted of noncohesive blast cells. The CBC revealed a leukocyte count of 238,300/,l and a differential consistent with CML. Reexamination of the patient found that the abdominal mass was a giant spleen. Further immunohistochemical studies of the tumor were consistent with extramedullary acute megakaryoblastic blast transformation of CML. Although extramedullary blast crises herald the accelerated phases in ,10% of CML cases, megakaryoblastic blast transformation of CML accounts for less than 3% of these cases. The combination of acute paraplegia and megakaryoblastic transformation in a previously undiagnosed patient with CML is extremely rare and may pose a diagnostic dilemma. Am. J. Hematol. 2006. © Wiley-Liss, Inc. [source] Spastic Paraplegia, Optic Atrophy, and Neuropathy: New Observations, Locus Refinement, and Exclusion of Candidate GenesANNALS OF HUMAN GENETICS, Issue 3 2009Lúcia Inês Macedo-Souza Summary SPOAN is an autosomal recessive neurodegenerative disorder which was recently characterized by our group in a large inbred Brazilian family with 25 affected individuals. This condition is clinically defined by: 1. congenital optic atrophy; 2. progressive spastic paraplegia with onset in infancy; and 3. progressive motor and sensory axonal neuropathy. Overall, we are now aware of 68 SPOAN patients (45 females and 23 males, with age ranging from 5 to 72 years), 44 of which are presented here for the first time. They were all born in the same geographic micro region. Those 68 patients belong to 43 sibships, 40 of which exhibit parental consanguinity. Sixty-one patients were fully clinically evaluated and 64 were included in the genetic investigation. All molecularly studied patients are homozygotes for D11S1889 at 11q13. This enabled us to reduce the critical region for the SPOAN gene from 4.8 to 2.3 Mb, with a maximum two point lod score of 33.2 (with marker D11S987) and of 27.0 (with marker D11S1889). Three genes located in this newly defined critical region were sequenced, but no pathogenic mutation was detected. The gene responsible for SPOAN remains elusive. [source] Centrifugal Pump Support for Distal Aortic Perfusion During Repair of Traumatic Thoracic Aortic InjuryARTIFICIAL ORGANS, Issue 11 2002Joseph T. Walls Abstract: Paraplegia from ischemic injury of the spinal cord and renal failure from inadequate perfusion of the kidneys may occur from aortic cross-clamping during repair of traumatic thoracic aortic injuries. After Institutional Review Board approval, we retrospectively reviewed the charts of 26 patients surgically treated for traumatic transection of the descending thoracic aorta during a 14 year period (1987,2001), using centrifugal pump (Sarns) support for distal aortic perfusion. The study group comprised 19 males and 7 females, whose ages ranged from 15 to 69 years. For all but 1 patient, who fell from a flagpole, the injuries were incurred in motor vehicle accidents. Aortic cross-clamp time lasted between 5 to 78 min (median = 40 min). Mean arterial pressure ranged from 50 to 80 mm Hg (median = 70 mm Hg). All patients survived operation without developing paraplegia or renal failure. Distal centrifugal pump perfusion during repair of traumatic injury of the descending thoracic aorta is a valuable adjunct during surgical treatment and aids in preservation of spinal cord and renal function. [source] Fine-needle aspiration of brown tumor of bone: Cytologic features with radiologic and histologic correlationDIAGNOSTIC CYTOPATHOLOGY, Issue 2 2009Ph.D., Sasha Pavlovic M.D. Abstract We report the case of a 40-year-old man with tertiary hyperparathyroidism due to end stage renal disease who initially presented with acute-onset paraplegia, elevated serum parathyroid hormone, and multiple bone abnormalities, including a large extradural intraspinal mass seen by magnetic resonance imaging. In contrast with imaging features, fine-needle aspiration cytology showed numerous benign-appearing multinucleated osteoclast-type giant cells that are the characteristics of either brown tumor or benign giant cell tumor of bone. Sheets of mononuclear spindled stromal cells were also noted. A core-needle biopsy confirmed the diagnostic features of brown tumor of hyperparathyroidism. Diagn. Cytopathol. 2009. © 2008 Wiley-Liss, Inc. [source] Paediatric lap-belt injury: A 7 year experienceEMERGENCY MEDICINE AUSTRALASIA, Issue 1 2006Michael Shepherd Abstract Objective:, To highlight the injuries that result from lap-belt use and make recommendations for prevention, the recent experience of a regional paediatric trauma centre was reviewed. Methods:, Retrospective review of admissions to Starship Children's Hospital from 1996 to 2003, with significant injury following involvement in a motor vehicle crash, while wearing a lap-belt. Patients were identified from two prospectively collected databases and discharge coding data. Results:, In total, 19 patients were identified over the 7 year period. The morbidity sustained includes 15 patients with hollow viscus injury, 13 laparotomies, 7 spinal fractures, 2 paraplegia and 1 fatality. A total of 11 patients required laparotomy with a median delay of 24 h. Of patients in the present series, 58% were aged less than 8 years and thus were inappropriately restrained. Conclusions:, Lap-belt use can result in a range of life-threatening injuries or permanent disability in the paediatric population. The incidence of serious lap-belt injury does not appear to be decreasing. Morbidity and mortality could be reduced by the use of three-point restraints, age appropriate restraints and booster seats. [source] Hereditary spastic paraplegia or spinocerebellar ataxia?EUROPEAN JOURNAL OF NEUROLOGY, Issue 8 2009Not always as easy as it seems No abstract is available for this article. [source] Functional electrical stimulation in neurological disordersEUROPEAN JOURNAL OF NEUROLOGY, Issue 5 2008O. K. Sujith Functional electrical stimulation (FES) refers to electrical stimulation of muscles in order to improve the impaired motor function. This is achieved by activating skeletal muscles with constant frequency trains of stimulations. This method has been found useful in various neurological disorders like hemiplegia, foot drop and paraplegia including spinal cord injuries. The first half of this review focuses on the broad clinical applications of functional electrical stimulation, its mechanism of action and the complications of this mode of therapy. Advanced Parkinson's disease (PD) is characterized by marked slowing of gait and frequent freezing episodes. Medical and surgical treatments are often ineffective in managing freezing episodes. The second half of this review discusses briefly the gait abnormalities in PD and the available treatment options. The possible role of FES in improving gait in parkinsonism and the importance of future research in this direction are highlighted. [source] Screening of ARHSP-TCC patients expands the spectrum of SPG11 mutations and includes a large scale gene deletion,HUMAN MUTATION, Issue 3 2009Paola S. Denora Abstract Autosomal recessive spastic paraplegia with thinning of corpus callosum (ARHSP-TCC) is a complex form of HSP initially described in Japan but subsequently reported to have a worldwide distribution with a particular high frequency in multiple families from the Mediterranean basin. We recently showed that ARHSP-TCC is commonly associated with mutations in SPG11/KIAA1840 on chromosome 15q. We have now screened a collection of new patients mainly originating from Italy and Brazil, in order to further ascertain the spectrum of mutations in SPG11, enlarge the ethnic origin of SPG11 patients, determine the relative frequency at the level of single Countries (i.e., Italy), and establish whether there is one or more common mutation. In 25 index cases we identified 32 mutations; 22 are novel, including 9 nonsense, 3 small deletions, 4 insertions, 1 in/del, 1 small duplication, 1 missense, 2 splice-site, and for the first time a large genomic rearrangement. This brings the total number of SPG11 mutated patients in the SPATAX collection to 111 cases in 44 families and in 17 isolated cases, from 16 Countries, all assessed using homogeneous clinical criteria. While expanding the spectrum of mutations in SPG11, this larger series also corroborated the notion that even within apparently homogeneous population a molecular diagnosis cannot be achieved without full gene sequencing. © 2008 Wiley-Liss, Inc. [source] Genetic and clinical aspects of X-linked hydrocephalus (L1 disease): Mutations in the L1CAM geneHUMAN MUTATION, Issue 1 2001Sabine Weller Abstract L1 disease is a group of overlapping clinical phenotypes including X-linked hydrocephalus, MASA syndrome, spastic paraparesis type 1, and X-linked agenesis of corpus callosum. The patients are characterized by hydrocephalus, agenesis or hypoplasia of corpus callosum and corticospinal tracts, mental retardation, spastic paraplegia, and adducted thumbs. The responsible gene, L1CAM, encodes the L1 protein which is a member of the immunoglobulin superfamily of neuronal cell adhesion molecules. The L1 protein is expressed in neurons and Schwann cells and seems to be essential for nervous system development and function. The patients' gene mutations are distributed over the functional protein domains. The exact mechanisms by which these mutations cause a loss of L1 protein function are unknown. There appears to be a relationship between the patients' clinical phenotype and the genotype. Missense mutations in extracellular domains or mutations in cytoplasmic regions cause milder phenotypes than those leading to truncation in extracellular domains or to non-detectable L1 protein. Diagnosis of patients and carriers, including prenatal testing, is based on the characteristic clinical picture and DNA mutation analyses. At present, there is no therapy for the prevention or cure of patients' neurological disabilities. Hum Mutat 18:1,12, 2001. © 2001 Wiley-Liss, Inc. [source] Gain scheduling control of functional electrical stimulation for assisted standing up and sitting down in paraplegia: a simulation studyINTERNATIONAL JOURNAL OF ADAPTIVE CONTROL AND SIGNAL PROCESSING, Issue 5 2005Fabio Previdi Abstract This paper reports on a simulation study that concerns the design of a non-linear controller for the standing up and the sitting down of a paraplegic patient by means of functional electrical stimulation. The simulations refer to a specific experimental device developed at the Fondazione Don Gnocchi (Italy). This is a seesaw, with the patient on one side and a weight on the other side. The patient is seated so that its posture can be fully known in real-time by continuously monitoring the knee joint angle. By delivering a suitable electrical stimulation to the quadriceps muscles groups, the patient can be raised and made to sit via smooth movements. Hitherto, the only feedback control law, which has been implemented in this area, is based on a PID controller and usually provides poor tracking performances. Hence, in this work, a non-linear gain scheduling controller has been designed and tested in a series of simulation experiments. The controller is tuned following a gain scheduling strategy: a set of local linear quadratic controllers is designed using a set of linear tangent models. A global non-linear gain scheduled controller is then obtained via interpolation. The gain- scheduled controller is implemented following an advanced strategy that guarantees that the so-called linearization property holds. Copyright © 2004 John Wiley & Sons, Ltd. [source] Endoluminal repair of distal aortic arch aneurysms causing aorto-vocal syndromeINTERNATIONAL JOURNAL OF CLINICAL PRACTICE, Issue 10 2008J. P. Morales Summary Purpose:, We have evaluated the efficacy of endovascular repair of distal aortic arch aneurysms (DAAA) causing recurrent laryngeal nerve palsy. Material and methods:, Eight patients (five male and three female) with median age of 72 years (range: 59,80) presented with left recurrent laryngeal nerve palsy associated with DAAA. All patients were considered unfit for open surgery. The median aneurysm size was 5.9 cm (range: 5,7.3). Thirteen stents were deployed: eight Gore, four Endofit and one Talent. Epidural anaesthesia was used in all patients. The left subclavian artery was covered in all and the left common carotid in three who had a preliminary right to left carotid,carotid bypass. Routine follow-up (FU) was with computed tomography (CT) at 3,6 months and yearly thereafter. Results:, Exclusion of the aneurysm sac was achieved in all patients. Thirty-day mortality was 0%, with no paraplegia or stroke. Early complications included: rupture of the external iliac artery (one) and common femoral artery thrombectomy (one). One patient died of unknown cause at 17 months. The mean FU in the remaining seven patients was 21 months (range: 6,51). Aneurysm size decreased in five, was unchanged in one and increased in one. Three patients had improvement in voice quality postoperatively. One patient had a recurrent type 1 endoleak which was restented twice. No late deaths have occurred. Conclusion:, Though technically the procedures involved were more complicated, endovascular repair of DAAA causing aorto-vocal syndrome is safe and offers a realistic alternative to open surgery. Hoarseness of the voice can improve postoperatively and is associated with reduction in aortic sac diameter. [source] Cerebral epidural hematoma following cerebrospinal fluid drainage during thoracoabdominal aortic repairACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 9 2009Y. B. JEONG Cerebrospinal fluid (CSF) drainage is a common adjunct to thoracoabdominal aortic aneurysm (TAAA) repair. CSF drainage may improve perioperative spinal cord perfusion and thereby decrease the incidence of paraplegia or paraparesis. Complications of CSF drainage may arise. We present a case of cerebral epidural hematoma (EDH), possibly arising from excessive CSF drainage, during thoracoabdominal aortic repair. [source] Serial compression B-scan and Doppler sonography for the screening of deep venous thrombosis in patients with spinal cord injuriesJOURNAL OF CLINICAL ULTRASOUND, Issue 1 2010Alfried Germing MD Abstract Purpose To evaluate the usefulness of serial compression B-scan and Doppler sonography (US) in screening for deep venous thrombosis (DVT) of the lower extremities in patients with spinal cord injuries. Method Patients with paraplegia and tetraplegia due to spinal cord injuries were screened by a serial compression B-scan and Doppler US protocol for DVT of the bilateral lower extremities within the first 36 hours after admission, at day 7 and at day 21. In patients with DVT, a follow-up US examination was performed 3 weeks after diagnosis to assess thrombi distribution. Results Between January 2007 and March 2008, a total of 115 patients (75 males, 40 females), aged 19 to 85 years, were included. The first US examination documented a DVT in 44 cases (38.3%). After an initial negative scan, sonography after 7 days and 21 days showed DVT in 6 patients and 2 patients, respectively. Cumulative rate of DVT after the first 3 weeks was 45.2% (n=52). Follow-up US after 3 weeks in patients with DVT documented a complete recanalization in 19 patients (36.5%), no change in 12 patients (23.1%), and residual thrombi with partial recanalization in 21 patients (40.4%). Conclusion Our study supports the use of serial compression B-scan and Doppler US as a screening tool for DVT of the lower extremities in patients with spinal cord injuries early after injury. © 2009 Wiley Periodicals, Inc. J Clin Ultrasound, 2010 [source] The role of mitochondria in inherited neurodegenerative diseasesJOURNAL OF NEUROCHEMISTRY, Issue 6 2006Jennifer Q. Kwong Abstract In the past decade, the genetic causes underlying familial forms of many neurodegenerative disorders, such as Huntington's disease, Parkinson's disease, Alzheimer's disease, amyotrophic lateral sclerosis, Friedreich ataxia, hereditary spastic paraplegia, dominant optic atrophy, Charcot-Marie-Tooth type 2A, neuropathy ataxia and retinitis pigmentosa, and Leber's hereditary optic atrophy have been elucidated. However, the common pathogenic mechanisms of neuronal death are still largely unknown. Recently, mitochondrial dysfunction has emerged as a potential ,lowest common denominator' linking these disorders. In this review, we discuss the body of evidence supporting the role of mitochondria in the pathogenesis of hereditary neurodegenerative diseases. We summarize the principal features of genetic diseases caused by abnormalities of mitochondrial proteins encoded by the mitochondrial or the nuclear genomes. We then address genetic diseases where mutant proteins are localized in multiple cell compartments, including mitochondria and where mitochondrial defects are likely to be directly caused by the mutant proteins. Finally, we describe examples of neurodegenerative disorders where mitochondrial dysfunction may be ,secondary' and probably concomitant with degenerative events in other cell organelles, but may still play an important role in the neuronal decay. Understanding the contribution of mitochondrial dysfunction to neurodegeneration and its pathophysiological basis will significantly impact our ability to develop more effective therapies for neurodegenerative diseases. [source] Subacute Combined Degeneration Due to Copper DeficiencyJOURNAL OF NEUROIMAGING, Issue 4 2007Joseph M. Ferrara MD ABSTRACT There is growing clinical evidence supporting a connection between copper deficiency and subacute combined degeneration. While nearly half of patients with copper deficiency myelopathy exhibit MRI abnormalities, signal changes are often ill-defined in distribution. We report a patient with sensory ataxia and spastic paraplegia from copper deficiency whose MRI demonstrates abnormal signal restricted to the dorsal and lateral columns, providing clear radiological support of an association between hypocupremia and combined system degeneration. [source] Neuroimaging of Tuberculous Myelitis: Analysis of Ten Cases and Review of LiteratureJOURNAL OF NEUROIMAGING, Issue 3 2006Mohammad Wasay MD ABSTRACT We retrospectively reviewed the clinical and neuroimaging features of 10 patients with tuberculous myelitis. The most common presenting symptoms were fever (70%) and paraplegia (60%). Bladder and bowel symptoms were present in 90% patients. On MRI, the involvement of the cervical/thoracic segment of the spinal cord was most commonly seen (90%). The most consistent finding was hyperintense signals on T2-weighted MRI. T1-weighted images showed isointense (n= 5) and hypointense (n= 4) signals in the spinal cord lesions. Post-contrast enhancement was present in 6 patients, epidural enhancement in 4 patients, and cord swelling in 2 patients. We reviewed more than 250 published cases with the diagnosis of tuberculous myelitis and radiculomyelitis with special attention to MRI findings. It is predominantly a disease of the thoracic spinal cord. Most spinal cord lesions appear as hyperintense on T2 and iso- or hypointense on T1-weighted images. MRI findings in patients with spinal cord tuberculosis have both diagnostic and prognostic significance. Cord atrophy or cavitation and the presence of syrinx on MRI may be associated with poor outcome. [source] Hysterical paraplegia simulating acute transverse myelitis after general anesthesiaACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 3 2008A. B. S. Hobaika No abstract is available for this article. [source] Treatment of a prolapsed lumbar intervertebral disc in a ferretJOURNAL OF SMALL ANIMAL PRACTICE, Issue 10 2004D. Lu A seven-month-old, male ferret had acute paraplegia and radiographs showed signs of disc prolapse between the second and third lumbar vertebrae (L2/3). Hemilaminectomy was performed to decompress the spinal cord. Histological examination revealed that the extradural material was consistent with annulus fibrosus and the L2/3 articular facets were enlarged as a result of bone remodelling. The ferret became ambulatory one month postoperatively. Five months postoperatively, the ferret had normal posture with mild proprioceptive deficits in the pelvic limbs, and fusion of the L2 and L3 vertebral bodies. [source] Paraplegia associated with brucellosis involving the anterior lumbrosacral nerve rootsJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2003Umit Hidir Ulas Abstract We report the case of a 21-year-old man with paraplegia due to brucellosis involvement of lumbosacral anterior roots. Lumbosacral magnetic resonance imaging showed contrast enhancement of anterior roots and the anterior part of duramater. Conduction block was found at the level of the lumbosacral anterior roots by electrophysiological studies, including magnetic stimulation study. Wright agglutination, Rose Bengal tests and bacterial culture obtained from cerebrospinal fluid confirmed the diagnosis of neurobrucellosis. Oral administration of ceftriaxon with additional rifampin was effective, and after 3 months of treatment, laboratory data resolved and clinical signs partially improved. [source] New model for simultaneous heart and kidney transplantation in miceMICROSURGERY, Issue 2 2003Minghui Wang M.D. In clinical settings, combined heart and kidney transplantation results in a lower incidence of cardiac graft and renal graft rejection as compared to isolated heart and kidney transplantation. To study the phenomenon in an experimental setting, we developed a model of combined heart and kidney transplantation in mice. According to our technique, we kept the patch of the infrarenal aorta and inferior vena cava (IVC) as long as possible during the donor's kidney explantation, and then, after finishing the kidney implantation with conventional techniques, we anastomosed the innominate artery and pulmonary artery of the heart graft to the patch of the infrarenal aorta and IVC of the renal graft, respectively, instead of the recipient's aorta and IVC. With the use of our method, combined heart and kidney transplantation in mice can be performed to get enough suture room for the second graft, to avoid prolonging the occlusion time of the recipient's circulation, and to profoundly decrease postoperative complications such as paraplegia and mortality. Of the 14 recipients with combined heart-kidney isografting, 10 have been successful (71.4%), surviving over 100 days with normal function of both grafts, and with lack of change in histological appearance. This suggests that the technique is feasible and reliable. © 2003 Wiley-Liss, Inc. MICROSURGERY 23:164,168 2003 [source] Botulinum neurotoxin type A injections reduce spasticity in mild to moderate hereditary spastic paraplegia, Report of 19 casesMOVEMENT DISORDERS, Issue 2 2008Martin J. Hecht MD Abstract Hereditary spastic paraplegia (HSP) is characterized by lower extremity spasticity. Symptomatic therapy generally includes physical therapy and oral antispastic agents, in selected cases intrathecal baclofen. Because of the positive results in other treatments of spasticity, the use of botulinum neurotoxin type A (BoNT-A) might also be considered for patients with HSP. We report the effect of BoNT-A injections in 19 unselected patients with HSP treated by the members of the German Spasticity Education Group. In 17 patients, the modified Ashworth scale had improved by one point. In one patient, it improved by three points. Most of the patients reported reduction of spasticity. BoNT-A injections were continued in 11 of 19 patients (57.9%). All of the patients with continued injections had a good or very good global subjective improvement. Patients with less pronounced spasticity and patients with accompanying physical therapy tended to exhibit a better effect. Only four patients reported adverse effects which were increased weakness in three patients and pain in one patient. BoNT-A injections appear to reduce spasticity effectively and safely, especially in patients with mild to moderate spasticity. The preliminary results of our case series should encourage larger studies of BoNT-A injections in HSP. © 2007 Movement Disorder Society [source] Paraplegia associated with combined spinal-epidural anaesthesia caused by preoperatively unrecognized spinal vertebral metastasisACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 9 2002A. Kararmaz We describe a case of paraplegia following combined spinal-epidural anaesthesia. It was postoperatively determined that a tumour of the vertebrae which was compressing the spinal cord was responsible for this complication. We suggest that the pre-existing pathology of the spine must be borne in mind as a differential diagnosis of acute postoperative paraplegia. [source] Hereditary spastic paraplegia associated with dopa-responsive parkinsonismMOVEMENT DISORDERS, Issue 5 2006Federico Micheli MD Abstract A 47-year-old patient with hereditary spastic paraplegia and parkinsonian features is reported. Treatment with levodopa led to marked improvement in his neurological status and quality of life. However, several years later he developed motor fluctuations and dyskinesias. The latter promptly remitted with amantadine treatment. © 2006 Movement Disorder Society [source] Gene and protein expression associated with protein synthesis and breakdown in paraplegic skeletal muscleMUSCLE AND NERVE, Issue 4 2008Micah J. Drummond PhD Abstract Spinal cord injury reduces the rate of skeletal muscle protein synthesis and increases protein breakdown, resulting in rapid muscle loss. The purpose of this study was to determine whether long-term paraplegia would eventually result in a downregulation of muscle mRNA and protein expression associated with both protein synthesis and breakdown. After 10 weeks of spinal cord transection, soleus muscle from 12 rats (6 sham-control, 6 paraplegic) was studied for mRNAs and proteins associated with protein synthesis and breakdown using real-time polymerase chain reaction and immunoblotting techniques. Protein kinase B (PKB/Akt), ribosomal S6 kinase 1 (S6K1), and myogenin mRNA were downregulated, whereas muscle ring finger 1 (MuRF1) and phospho-forkhead transcription factor 4 (FoxO4) protein were increased in paraplegic rats. We conclude that gene and protein expression of pathways associated with protein synthesis are reduced, whereas some markers of protein breakdown remain elevated following chronic paraplegia. Clinical interventions designed to increase muscle protein synthesis may be helpful in preventing excessive muscle loss during long-term paraplegia. Muscle Nerve, 2008 [source] Bilateral Motor Cortex Stimulation for the Relief of Central Dysesthetic Pain and Intentional Tremor Secondary to Spinal Cord Surgery: A Case ReportNEUROMODULATION, Issue 4 2002Roberto Fabian Rodríguez MD Abstract Objectives. Our objective was to describe and analyze through a third party disinterested observer the results obtained by using motor cortex stimulation (MCS) for the treatment of central dysesthetic diffuse-distal type of paraplegic pain and intentional tremor secondary to the total removal of a cervical ependymoma. Design. Retrospective case report with discussion. Methods. A 69-year-old female, who after satisfactory removal of a cervical ependymoma, developed a central dysesthetic diffuse-distal type of paraplegic pain and intentional tremor associated with mild cerebellar deficit. Neurologic compromise became so intense that it prevented the patient from leading an independent lifestyle. Conservative treatments failed and a unilateral trial of MCS was performed. After a four-day satisfactory unilateral trial, a bilateral electrode, Resume II (Medtronic, Inc., Minneapolis, MN), was inserted through a small craniotomy and a dual-channel RF activated receiver was implanted. During the second month of follow-up an independent observer personally interviewed the patient and assessed results through a multimodal approach, encompassing several analog scales used to measure the different components of the painful experience; a daily life activities scale and drug intake. Results. Evoked painful phenomena were dramatically improved, but the steady component of pain was only moderately relieved. The patient's tremor improved to allow for the performance of simple movements such as independent eating. Conclusion. In this single case report MCS was extremely useful in eliminating almost all of the patient's pain-evoked phenomena. Both steady burning pain and tremor were also improved. This is only one case report and MCS warrants further investigation as to its utility in controlling central dysesthetic pain in paraplegia and postchordotomy dysesthesias. [source] Clinicopathological studies of peripheral neuropathy in Churg,Strauss syndromeNEUROPATHOLOGY, Issue 4 2002Toshiko Nagashima Clinicopathological studies were performed on the visceral organs and the sural nerve of a male patient with Churg,Strauss syndrome (CSS) in order to understand the mechanisms of peripheral nervous system damage. A 67-year-old man, with a 2-year history of bronchial asthma, developed acutely painful paraplegia and dyspnea. Laboratory data showed a leukocytosis, an elevated serum creatinine kinase (CK) and marked eosionophilia. Autoantibodies including p- and c-ANCA were negative. Electrophysiological studies revealed a severe sensory-motor neuropathy of multiple mononeuritis type. Steroid pulse therapy performed a day after biopsy of skin, muscle and sural nerve was effective in resolving his respiratory and neurological dysfunction but a perforation of an intestinal ulcer occurred which required surgical intervention. In the biopsied sural nerve and the surgically resected intestine and mesentery there was vasculitis with fibrinoid necrosis accompanied by numerous eosinophils and macrophages containing eosinophil cationic protein (ECP). These findings suggest that in addition to ischemic changes due to vasculitis some neurotoxic substances generated by the eosinophils may be involved in the development of neuropathy in CSS. [source] The extent of axonal loss in the long tracts in hereditary spastic paraplegiaNEUROPATHOLOGY & APPLIED NEUROBIOLOGY, Issue 6 2004G. C. DeLuca Hereditary spastic paraplegia (HSP) comprises a group of inherited neurodegenerative disorders with the shared characteristics of progressive weakness and spasticity predominantly affecting the lower limbs. Limited pathological accounts have described a ,dying back' axonal degeneration in this disease. However, the distribution and extent of axonal loss has not been elucidated in a quantitative way. We have studied post-mortem material from six HSP patients and 32 controls in detail. The population of axons was examined quantitatively in the corticospinal tracts from the medulla to the lumbar spinal cord and the sensory tracts from the lumbar to upper cervical spinal cord. Myelin and axon-stained sections were employed to estimate the notional area and axonal density, respectively, of both tracts. Our results indicate that in the corticospinal tracts there is a significant reduction in area and axonal density at all levels investigated in HSP compared to controls. In the corticospinal tracts, the ratio of medulla and lumbar total axonal number was significantly greater in HSP cases compared to controls suggesting more pronounced axonal loss in the distal neuraxis in HSP than in controls. The sensory tracts in HSP, in contrast, showed a significant reduction in area and axonal density only in the upper regions of the spinal cord. Similar to the corticospinal tracts, the ratio of lumbar and upper cervical cord total axonal number in the sensory tracts was increased in HSP cases compared to controls. These findings are consistent with a length-dependent ,dying back' axonopathy. Nerve fibre loss was not size-selective with both small and large diameter fibres affected. In HSP, axonal loss is widespread and symmetrical and its extent tract-specific. The characterization of the nature of axonal loss in HSP, where this is a primary phenomenon, may help the interpretation of axonal loss in conditions such as multiple sclerosis where the sequence of events is less clear. [source] | |||||||||||||||||||||||||||||||