Myotonic dystrophy presenting as new-onset hand weakness and recurrent pneumonia in a patient with paraplegia: a case report

We describe a previously independent T11 paraplegic patient who had delayed-onset hand weakness and recurrent pneumonia caused by myotonic dystrophy. A man in his late thirties suffered a thoracic spinal cord injury (SCI) from a gunshot wound at the age of 17 years, with resultant T11 American Spinal Injury Association class A paraplegia. He lived independently until the age of 36 years when he was hospitalized multiple times for pneumonia. During a rehabilitation stay after one of the acute hospitalizations, the patient's hand weakness and diffuse muscular atrophy were noted. Electrodiagnostic testing was performed, which showed myotonic discharges. Genetic testing was consistent with myotonic dystrophy. This case shows the importance of considering causes of weakness that affect the population as a whole when evaluating a patient with SCI who presents with delayed-onset weakness.     

Spinal cord injury in an inner city hospital

During the first six years of existence of the rehabilitation unit in a black inner city major municipal hospital, 53 patients with spinal cord injury were admitted. A retrospective study of these patients sought answers to questions concerning etiology, patient characteristics, services provided and method of delivery, and advantages and disadvantages of rehabilitation in a community hospital. Findings revealed differences in causation of spinal cord injury between women and men and between patients with paraplegia and quadriplegia. tthe male paraplegic patients were the youngest; their life style led to spinal cord injury. Social factors such as inadequate housing, lack of transportation, and insufficient financial resources were deterrents to rehabilitation. An approach that emphasized consideration of all the psychosocial factors was developed. Evaluation and treatment were extended into the community. Lack of peer groups and multiphasic programs were the major disadvantages.     

一种罕见的运动神经元病亚型：散发性面肩肱型脊肌萎缩症一例并文献复习

目的 探讨面肩肱型脊肌萎缩症的临床表现和早期诊断.方法 对我院2006年收治的1例患者临床资料结合文献进行回顾性分析.结果 该患者为青年女性,隐袭起病,进行性进展.初期表现为选择性累及颜面、肩胛带肌群和上肢近端肌群,早期血浆肌肉酶谱正常,电生理检查和肌肉活检均提示神经源性损害.结论 面肩肱型脊肌萎缩症与肌营养不良性面肩肱型肌萎缩临床上极为类似,应早期进行电生理和肌肉活检检查协助确诊.     

Rehabilitation outcomes in complete C5 quadriplegia

Establishment of rehabilitation goals for spinal cord injury patients generally has been based on the degree of residual motor function. Despite extensive clinical experience with spinal cord injury rehabilitation, there have been no reports of the ability of C5 quadriplegic patients to perform self-care and mobility skills before and after rehabilitation. This study was designed to examine the rehabilitation outcomes of 63 patients with C5 complete quadriplegia, who completed an interdisciplinary inpatient rehabilitation program, using the 100-point modified Barthel Index as a means of rating functional status. There were statistically significant increases in the mean modified Barthel index scores from 7.1 on admission to 28.9 on discharge for the entire group of patients. The self-care subscore increased significantly from 6.5 on admission to 20.0 on discharge and the mobility subscore increased significantly from 0.5 on admission to 8.9 on discharge. Ability to perform self-care and mobility subscore tasks is described. This study documented significant improvements in function during comprehensive rehabilitation among patients with C5 spinal cord injury.     

颈髓损伤并四肢瘫痪患者出院后心理状况的调查分析

目的 调查颈髓损伤并四肢瘫痪患者出院后的心理状况,寻求相应康复对策.方法 应用症状自评量表(SCL-90)对88例出院后的颈髓损伤并四肢瘫痪患者进行调查.结果 出院后的颈髓损伤并四肢瘫痪患者的SCL各项得分均超出常模.结论 颈髓损伤并四肢瘫痪患者出院后的心理健康状况都不理想,应采取相应措施改变其心理状况.     

Assessing sexual functions of the adult with traumatic quadriplegia

The individual who sustains a spinal cord injury resulting in traumatic quadriplegia experiences a drastic alteration in his lifestyle from both physiological and psychological perspectives. The effects of spinal cord injury on a patient's sexuality will be explored according to Carrera's rule for optimum sexual functioning: all systems go, a positive self-image, and nurturing environment.     

Reflex sympathetic dystrophy in quadriplegia: case report

A patient with quadriplegia developed severe localized unilateral hand pain associated with clinical evidence of abnormal sympathetic hand activity. Diagnosis revealed reflex sympathetic dystrophy (RSD), which was resolved with conservative management. The association of RSD with spinal cord injury has been reported only rarely. Since few speculations about this condition have been made, we suggest that unexplained painful syndromes in the extremities of patients with spinal cord injury be carefully evaluated to rule out the possibility of RSD.     

Voice recognition device as a computer interface for motor and speech impaired people

A voice recognition device has been identified as an interface for personal computer control by patients presenting with high-level spinal cord injuries and mild dysarthria. The device enables a person to bypass the traditional keyboard and activate a computer through voice control. The user simply creates templates of a spoken vocabulary in computer memory. The computer then matches real-time spoken words to stored templates for activation. The system has been clinically tested with one 10-year-old boy who has C1-C2 quadriplegia and a 19-year-old man who sustained a C6 spinal cord injury and dysarthria secondary to head injury. Each patient created a vocabulary for computer storage to run educational software packages and games. Accuracy rates for computer speech recognition were measured in multiple practice sessions. Intelligibility of single words produced by the dysarthric speaker was measured also. Both patients activated computer programs through voice control. Recognition rates ranged from 45 to 60% for the first patient, and 79 to 96% for the second patient. A mean success rate for voice recognition across trials with both patients was approximately 80%. The device increased the dysarthric speaker's articulatory precision. Results indicate that the system might be appropriate for rehabilitation programs though further technologic refinement of the device would increase its effectiveness.     

Traumatic brain injury is under-diagnosed in patients with spinal cord injury.

OBJECTIVE: To investigate the occurrence and severity of traumatic brain injury in patients with traumatic spinal cord injury. DESIGN: Cross-sectional study with prospective neurological, neuropsychological and neuroradiological examinations and retrospective medical record review. PATIENTS: Thirty-one consecutive, traumatic spinal cord injury patients on their first post-acute rehabilitation period in a national rehabilitation centre. METHODS: The American Congress of Rehabilitation Medicine diagnostic criteria for mild traumatic brain injury were applied. Assessments were performed with neurological and neuropsychological examinations and magnetic resonance imaging 1.5T. RESULTS: Twenty-three of the 31 patients with spinal cord injury (74%) met the diagnostic criteria for traumatic brain injury. Nineteen patients had sustained a loss of consciousness or post-traumatic amnesia. Four patients had a focal neurological finding and 21 had neuropsychological findings apparently due to traumatic brain injury. Trauma-related magnetic resonance imaging abnormalities were detected in 10 patients. Traumatic brain injury was classified as moderate or severe in 17 patients and mild in 6 patients. CONCLUSION: The results suggest a high frequency of traumatic brain injury in patients with traumatic spinal cord injury, and stress a special diagnostic issue to be considered in this patient group.     

Biomechanical analysis of a posterior transfer maneuver on a level surface in individuals with high and low-level spinal cord injuries

OBJECTIVE: The purpose of this study was to determine the movement patterns and the muscular demand during a posterior transfer maneuver on a level surface in individuals with spinal cord injuries. DESIGN: Six participants with high-level spinal cord injury (C7 to T6) were compared to five participants with low-level spinal cord injury (T11 to L2) with partial or complete control of abdominal musculature. BACKGROUND: Developing an optimal level of independence for transfer activities figures among the rehabilitation goals of individuals with spinal cord injury. There has been no biomechanical study which specifically describes the posterior transfer maneuver. METHODS: Tridimensional kinematics at the elbow, shoulder, head and trunk, as well as surface electromyographic data of the biceps, triceps, anterior deltoid, posterior deltoid, pectoralis major, latissimus dorsi, trapezius and rectus abdominus muscles were recorded during the posterior transfer. To quantify the muscular demand, the electromyographic data were amplitude normalized to the peak value obtained from maximum voluntary contractions. The transfer was divided into pre-lift, lift, and post-lift phases for analysis. RESULTS: The duration of the lift phase was significantly shorter (P<0.05) for the high-level spinal cord injury (1.24; SD, 0.37 s) when compared to the low-level spinal cord injury (1.74; SD, 0.39 s). The patterns and magnitudes of the angular displacements were found similar between groups (P values: 0.45-0.98). However, the high-level spinal cord injury initiated the task from a forward flexed posture, whereas the low-level spinal cord injury adopted an almost upright alignment of the trunk. Higher muscular demands were calculated for all muscles among high-level spinal cord injury participants during the transfer when compared to the low-level spinal cord injury. However, only the anterior deltoid (high level=92.4%; low level=34.2%) and the pectoralis major (high level=109.8%; low level=25.6%) reached statistical significance during the lift phase.Conclusions. Participants with high-level spinal cord injury presented different movement characteristics and higher muscular demands during the posterior transfer than low-level spinal cord injury ones. This is probably to compensate for the additional trunk and upper limb musculature impairment. RELEVANCE: The findings of this study may help to develop guidelines of specific strengthening programs for the thoracohumeral, scapulothoracic and shoulder muscles designed to restore optimal transfer capacity in individuals with spinal cord injury. Furthermore, innovative rehabilitation programs targeting the ability to control the trunk could be beneficial for these individuals.     

Neoplastic vs. traumatic spinal cord injury: an inpatient rehabilitation comparison

OBJECTIVE: To compare demographics, injury characteristics, and functional outcomes of patients with neoplastic spinal cord compression with those with traumatic spinal cord injuries. DESIGN: A prospective 5-yr comparison was undertaken comparing 34 patients with neoplastic spinal cord compression with 159 patients with traumatic spinal cord injury. RESULTS: Patients with neoplastic spinal cord compression were significantly older, more often female, and unemployed than patients with traumatic spinal cord injury. Neoplastic spinal cord compression presented more often with paraplegia involving the thoracic spine, and injuries were more often incomplete compared with traumatic spinal cord injury. Patients with neoplastic spinal cord compression had a significantly shorter rehabilitation length of stay compared with those with traumatic spinal cord injury. The neoplastic group had significantly lower FIM change scores. Both groups had similar FIM efficiencies and discharge to home rates. CONCLUSIONS: Patients with neoplastic spinal cord compression have different demographic and injury characteristics but can achieve comparable rates of functional gains as their traumatic spinal cord injury counterparts. Although patients with traumatic injuries achieve greater functional improvement, patients with neoplasms have a shorter rehabilitation length of stay and comparable FIM efficiencies and home discharge rates.     

Functional improvement after pediatric spinal cord injury

OBJECTIVE: To describe the functional gain (FGain) with pediatric spinal cord injury inpatient rehabilitation and to identify the relationship of various factors to FGain in pediatric spinal cord injury inpatient rehabilitation. DESIGN: Retrospective chart review of a series of 91 children with spinal cord injury admitted from 1993 to 1998 in a freestanding rehabilitation hospital. Admission and discharge functional status were assessed with the Pediatric Functional Independence Measure (WeeFIM) instrument for children 7 yr. The outcome measure is the FGain (difference between the discharge and admission functional status). RESULTS: Significant gains in functional status were observed in all patients. FGain was not significantly related to age, sex, length of inpatient rehabilitation, pathogenesis, or completeness or neurologic level of injury. However, there was a trend for higher FGain for patients with incomplete spinal cord injury and traumatic spinal cord injury. CONCLUSIONS: Functional improvement occurs with pediatric spinal cord injury inpatient rehabilitation. There is a trend for higher FGain in patients with less severe injury and traumatic injury. The lack of relationship between FGain and length of inpatient rehabilitation suggests that a variety of other factors influence the relationship between FGain and length of inpatient rehabilitation.     

早期功能锻炼对脊髓损伤患者骨质疏松的预防作用

目的探讨早期肢体被动功能锻炼对脊髓损伤患者骨质疏松的预防作用。方法将26例外伤性脊髓损伤所致截瘫患者随机分为实验组和对照组,每组13例,实验组进行早期肢体功能锻炼,对照组按照外伤瘫痪患者护理常规进行。常规x线片观察两组患者锻炼前后（入组时和4个月后）腰椎及股骨近端的松质骨密度变化,同时分别检测两组患者锻炼前后（入组时和4个月后）血清碱性磷酸酶、血钙、尿Ca／Cr及尿羟脯氨酸含量。结果腰椎及股骨近端的松质骨密度实测值与入组时比较,锻炼后（4个月后）两组均有下降趋势,但均无统计学意义（P〉0．05）,两组间差异也无统计学意义（P〉0．05）。与入组时比较,锻炼后（4个月）实验组血清碱性磷酸酶无统计学差异（P〉0．05）,血钙、尿Ca／Cr及尿羟脯氨酸均有统计学意义（P〈0．05）;对照组血清碱性磷酸酶血钙、尿Ca／Cr及尿羟脯氨酸均无统计学意义（P〉0．05）。锻炼后（4个月）两组间除血清碱性磷酸酶差异无统计学意义（P〉0．05）,血钙、尿Ca／Cr及尿羟脯氨酸差异均有统计学意义（P〈0．05）。结论早期肢体功能锻炼对脊髓损伤患者骨质疏松具有一定的预防作用。     

创伤性脊髓损伤患者术后肢体功能康复训练方案的构建与应用

目的通过文献回顾和Delphi法以循证医学为基础构建标准化、创伤性脊髓损伤患者术后肢体功能康复训练方案。方法运用Delphi专家咨询法构建创伤性脊髓损伤患者术后肢体功能康复训练方案。2011年6-12月,选取上海市两所三级综合性医院骨脊柱科和骨创伤科住院的患者113例为研究对象,根据其住院单双号将其分为对照组和肢体功能康复训练方案组(简称方案组),比较两组患者肢体功能康复知识掌握程度和并发症的发生率。结果在肢体主动和被动锻炼的方法、疼痛的汇报和缓解方法、出院后各种并发症预防方法与重要性等方面,方案组患者掌握程度明显优于对照组,差异均有统计学意义(均P<0.05)。在关节活动范围(range of motion,ROM)缩小、便秘和腹胀等并发症的发生率上,方案组患者优于对照组,差异有统计学意义(P<0.05)。结论实施创伤性脊髓损伤患者术后肢体功能康复训练方案能提高脊髓损伤患者对肢体功能康复知识的认知水平,并且明显减少术后并发症的发生,促进了患者肢体功能的康复,提高了患者的生活质量。     

Cost-effectiveness of duplex ultrasound surveillance in spinal cord injury

OBJECTIVE: To determine cost-effectiveness of surveillance with duplex ultrasound for thromboembolic disease in individuals with acute traumatic spinal cord injury at admission to rehabilitation. DESIGN: In this retrospective sequential case series study, individuals with traumatic spinal cord injury admitted to our rehabilitation facility between July 1, 1988, and December 31, 1998, were identified. Cost at our institution for treatment of thromboembolic disease was tabulated in 2001-2002 dollar amounts. Using this cost information, in a statistical model founded on available medical evidence, analysis for cost-effectiveness was then performed. RESULTS: A total of 369 subjects met inclusion criteria. Based on the statistical model, the cost and estimated mortality from thromboembolic disease per subject when performing admission duplex ultrasounds was $449.28 and 0.016%, respectively. The estimated total cost per subject when not performing admission duplex ultrasounds was $136.29, with predicted mortality of 0.524% due to thromboembolic disease. Therefore, cost of one life saved when performing admission duplex ultrasound surveillance is calculated to be $61,542, with cost per life year gained between $1193 and $9050, depending on age at time of injury and severity of injury. CONCLUSION: Duplex ultrasound is a cost-effective tool for deep venous thrombosis surveillance in individuals with acute traumatic spinal cord injury admitted to rehabilitation programs.     

Early unplanned transfers from inpatient rehabilitation

OBJECTIVE: To identify characteristics of patients who transfer off inpatient rehabilitation to a surgical or medical unit before completion of their rehabilitation program. DESIGN: A retrospective 9-yr chart review of patients transferred off the rehabilitation unit at a regional level 1 trauma center due to medical complications. RESULTS: Of 3072 patient admissions, 250 (8%) were transferred to an acute medical or surgical unit, and 55 (22%) of those transfers were within 3 days. Of the 250 patients, 33% had traumatic brain injury, 23% had spinal cord injury, 24% had stroke, 2% had amputations, 18% were in other diagnostic groups, and 23% were >64 yrs of age. When patients transferred early, only 47% were ultimately discharged to home, compared with approximately 72% of all discharges. The most common reasons for early transfer in these patients were infection (22%) and pulmonary complications (14%). Risk factors for early discharge included age of >64 yrs, spinal cord injury, or amputation. CONCLUSION: Patients admitted to the inpatient rehabilitation unit who have spinal cord injuries, amputations, or are >64 yrs old may have more medical/surgical complications. More detailed study of this patient population may help reduce the number of early transfers off the inpatient rehabilitation unit.     

The order of reacquirement of activity of daily living functions in people with spinal cord injury during rehabilitation after initial medical treatment and its affecting factors.

The purpose of this study was to investigate the order of reacquisition of activity of daily living (ADL) functions during inpatient rehabilitation at our spinal cord injury unit following the individual's initial medical care and to investigate what initial medical and biological factors would affect the ADL functions which the individual with spinal cord injury could perform 14 weeks after the beginning of inpatient rehabilitation. Fifty-two patients with spinal cord injuries who were hospitalized in the rehabilitation unit after their initial medical care participated in this study. The participants' ADL abilities were measured using the Barthel Index every four weeks after hospitalization for a period of 26 weeks. The order of reacquisition of ADL functions included in the Barthel Index was analyzed. Multiple regression analysis was performed to analyze the initial medical and biological factors affecting ADL reacquisition 14 weeks after hospitalization. The order of reacquisition of the ADL functions was almost the same in each individual. The multiple regression equations of the Barthel Index score and number of ADL functions after 14 weeks were both significant and the explanatory variables in both equations included the patients' age, complications on the initial stage and traumatic brain injury as an associated injury with spinal cord injury. We concluded that the patient's score on the Barthel Index and what the patient could actually perform 14 weeks after hospitalization could be predicted from the patient's status at hospitalization in the rehabilitation unit. This predictable correlation can contribute to the rehabilitation management for spinal cord injuries.     

The choice to end life as a ventilator-dependent quadriplegic

A 17-year-old male sustained a C5/6 fracture dislocation and complete C5 quadriplegia in a diving accident. Three days later sensory and motor function deteriorated and he required mechanical ventilation. Surgical exploration found no cause and a fusion was done. Neurologic function stabilized after three weeks with a C1 sensory level, no neck movement, and slight weakness of the tongue. Patient and family were followed closely by the spinal cord injury rehabilitation team from onset of injury. The patient was transferred to the ventilator-dependent pediatric rehabilitation program after ten weeks. Bowel, bladder, skin, and nutritional management were stabilized and taught to his parents who remained with him constantly. Communication was achieved with a "talking tracheostomy." He learned to use "Sip-n-Puff" control for driving an electric wheelchair and for Morse code input to a computer. He was passive but cooperative during hospitalization. Eight months after injury he was discharged to his home, which had been modified to meet his needs. A computer word processor, environmental control unit, and modified van were obtained; nursing care was provided around the clock. The patient enrolled in a community college course. Soon after discharge he contacted an attorney to explore legal actions for ending his life, which he considered intolerable. After obtaining medical and psychiatric reports, a court order was issued, which established his legal competence and directed people taking care of him to follow his directions. A few weeks later, 25 months after his injury, he privately said goodbye to his family, asked to be disconnected from the ventilator, and died. Medical and legal issues raised by this case are discussed.     

Functional skills after spinal cord injury rehabilitation: three-year longitudinal follow-up

Reports that spinal cord injury (SCI) patients maintain or improve functional abilities after initial rehabilitation have been limited by small sample size, inadequate functional measures, cross-sectional assessment at only one point in time, and lack of detailed statistical analysis. This study evaluated the follow-up functional status levels of 236 patients with traumatic SCI who completed comprehensive inpatient rehabilitation and were also followed for three years. The sample population was 83% men, with a mean age of 27.0 years. The 100-point Modified Barthel Index (MBI) was used to assess functional abilities. The 75 patients with complete quadriplegia had mean MBI scores of 30.3 at rehabilitation discharge and 37.8 at three-year follow-up. The 53 patients with incomplete quadriplegia were discharged with a mean MBI score of 53.8, and scored 68.3 at three-year follow up. The mean MBI scores for the 66 patients with complete paraplegia were 72.0 at discharge and 75.6 at three-year follow-up. The 42 patients with incomplete paraplegia had mean MBI scores of 77.0 at discharge and 86.1 at three-year follow-up. Each of the four groups demonstrated stability or increases in the proportions of patients who were independent in performance of each of the 14 MBI component tasks from discharge to follow-up. The finding that SCI patients, in this large series, maintained or improved functional levels for three years after discharge is consistent with previous studies and is reassuring to rehabilitation providers.     

脊髓损伤后的体位性低血压

体位性低血压是指在直立体位下的血压的下降。体位性低血压常发生在高位脊髓损伤的患者中,影响患者的训练和康复。但到目前为止,其发生原因尚未完全明确。本文主要就脊髓损伤后体位性低血压的可能原因;典型症状、诊断和测量;治疗和预防进行综述。