Assessment of bulbar function in amyotrophic lateral sclerosis: validation of a self-report scale (Center for Neurologic Study Bulbar Function Scale).

BACKGROUND AND PURPOSE: Impaired bulbar functions of speech and swallowing are among the most serious consequences of amyotrophic lateral sclerosis (ALS). Despite this, clinical trials in ALS have rarely emphasized bulbar function as an endpoint. The rater-administered Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) or various quality-of-life measures are commonly used to measure symptomatic benefit. Accordingly, we sought to evaluate the utility of measures specific to bulbar function in ALS. METHODS: We assessed bulbar functions in 120 patients with ALS, with clinicians first making direct observations of the degree of speech, swallowing and salivation impairment in these subjects. Clinical diagnosis of bulbar impairment was then compared with ALSFRS-R scores, speech rate, time to swallow liquids and solids, and scores obtained when patients completed visual analog scales (VASs) and the newly-developed 21-question self-administered Center for Neurologic Study Bulbar Function Scale (CNS-BFS). RESULTS: The CNS-BFS, ALSFRS-R, VAS and timed speech and swallowing were all concordant with clinician diagnosis. The self-report CNS-BFS and ALSFRS-R bulbar subscale best predicted clinician diagnosis with misclassification rates of 8% and 14% at the optimal cut-offs, respectively. In addition, the CNS-BFS speech and swallowing subscales outperformed both the bulbar component of the ALSFRS-R and speech and swallowing VASs when correlations were made between these scales and objective measures of timed reading and swallowing. CONCLUSIONS: Based on these findings and its relative ease of administration, we conclude that the CNS-BFS is a useful metric for assessing bulbar function in patients with ALS.

A protocol for identification of early bulbar signs in amyotrophic lateral sclerosis.

The purpose of this project is to identify characteristics that may be of assistance in establishing the diagnosis and monitoring early progression of bulbar dysfunction in patients with Amyotrophic Lateral Sclerosis (ALS). Early identification of bulbar dysfunction would assist in clinical trials and management decisions. A database of 218 clinic visits of patients with ALS was developed and formed the basis for these analyses. As a framework for the description of our methodology, the Disablement Model [World Health Organization. WHO International classification of impairment, activity, and participation: beginner's guide. In: WHO, editor. Beta-1 draft for field trials; 1999] was utilized. Our data identified that the strongest early predictors of bulbar speech dysfunction include altered voice quality (laryngeal control), speaking rate, and communication effectiveness. A protocol for measuring these speech parameters was therefore undertaken. This paper presents the protocol used to measure these bulbar parameters.

Role of mitochondria in amyotrophic lateral sclerosis.

Neurodegeneration in amyotrophic lateral sclerosis (ALS) is characterized by the specific loss of central and peripheral motor neurons. While this pattern of neuronal demise gives rise to a distinct clinical syndrome, at the cellular and molecular level ALS pathology is similar to that seen in other neurodegenerative diseases. In particular, mitochondrial dysfunction in ALS is reminiscent of that observed in Alzheimer's and Parkinson's diseases. Mitochondria in persons with ALS demonstrate impaired electron transport, increased free radical generation, and an inability to adequately buffer cytosolic calcium shifts. These abnormalities are probably systemic and potentially due to mutation of mitochondrial DNA.

Mitochondria in sporadic amyotrophic lateral sclerosis.

Mitochondria are abnormal in persons with amyotrophic lateral sclerosis (ALS) for unknown reasons. We explored whether aberration of mitochondrial DNA (mtDNA) could play a role in this by transferring mitochondrial DNA (mtDNA) from ALS subjects to mtDNA-depleted human neuroblastoma cells. Resulting ALS cytoplasmic hybrids (cybrids) exhibited abnormal electron transport chain functioning, increases in free radical scavenging enzyme activities, perturbed calcium homeostasis, and altered mitochondrial ultrastructure. Recapitulation of defects previously observed in ALS subjects and ALS transgenic mice by expression of ALS mtDNA support a pathophysiologic role for mtDNA mutation in some persons with this disease.

Don't throw away the Austin Moore.

For the last 20 years, the authors' institution has treated displaced femoral neck fractures in the elderly with femoral head prosthetic replacement. The procedure has been accomplished utilizing a posterolateral approach, sectioning of the short external rotators, suction fitting of the acetabulum, and capsular repair. The post-operative management has been progressive ambulation and full weight bearing. The authors review the operative procedure, perioperative complications, and functional long-term follow-up in 173 elderly patients who had prosthetic replacement for femoral neck fractures. These were all done as primary procedures. One hundred pressfit Austin Moore procedures were performed in 97 patients from 1977 to 1981. Eighty bipolar proximal femoral replacements were performed in 76 patients from 1985 to 1987. These were each consecutive groups of patients and excluded were patients with pathologic fractures. There were four patients with spasticity in the Austin Moore group and four patients with spasticity in the bipolar group. Medical complications were similar in both groups. There were no deep infections in either group. Two of the three dislocations in the Austin Moore group were in patients with spasticity. Seven patients (7%) died within 3 months in the Austin Moore group and nine patients (11%) died within 3 months in the bipolar group. At 2-year follow-up, 24 patients (24%) died within 2 years in the Austin Moore group and 16 patients (20%) died in the bipolar group. At follow-up (mean, 26 months), there did not appear to be any statistically significant difference in the patients' Harris hip scores. The average hip score ranged from 55 to 92, with a mean of 76 in both groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Partial thickness rotator cuff tears: results of arthroscopic treatment.

Thirty-one patients with arthroscopically documented partial thickness rotator cuff tears treated by arthroscopic debridement of the lesion were retrospectively reviewed. The patients had had symptoms for an average of 20.5 months prior to surgery. Twenty-two of these 31 shoulders also had bursoscopy, with 18 having arthroscopic subacromial decompression. The results were graded by both the UCLA Shoulder Rating Scale and Neer's criteria. Twenty-six (84%) of the patients had satisfactory results with the remaining 5 (16%) patients having unsatisfactory results. A classification system for the size and location of partial thickness rotator cuff tears is presented. The lesion size did not affect the result. Repeat arthroscopy in three patients demonstrated no further deterioration of their rotator cuff. The results with and without subacromial decompression were similar. The need for subacromial decompression is best determined by the arthroscopic finding of a bursal side tear.

Instrumented measurements of laxity in patients who have a Gore-Tex anterior cruciate-ligament substitute.

Twenty patients who had substitution of the anterior cruciate ligament with a Gore-Tex synthetic ligament were evaluated preoperatively and postoperatively with the University of California at Los Angeles instrumented clinical-testing apparatus, which records anterior-posterior force versus displacement-response curves of the tibia with respect to the femur at 20 degrees of flexion of the knee. The mean age of the patients was thirty-three years (range, nineteen to fifty-four years). The duration of follow-up ranged from twenty-four to forty-four months (mean, thirty-one months). The mean preoperative difference in anterior laxity between the injured knee and the normal knee (4.5 millimeters with neutral rotation of the foot) was unchanged two years after the operation; at that time, all patients had an anterior laxity of the injured knee of more than eight millimeters, and 90 per cent had a difference in anterior laxity of more than two millimeters between sides. The mean values for anterior stiffness at fifty and 100 newtons of anterior force were unchanged after the operation, remaining at 40 to 50 per cent of normal levels. At 200 newtons, or 20.4 kilograms (forty-five pounds) of anterior force, the mean stiffness of the involved knee was 11 to 17 per cent greater than that of the normal knee. Clinically, there were improvements in both subjective and objective knee-rating scores. All but four patients had a reduction of at least one grade in the pivot-shift score; in thirteen, the pivot-shift sign was eliminated.(ABSTRACT TRUNCATED AT 250 WORDS)

Four to ten year followup of unreconstructed anterior cruciate ligament tears.

Both operative and nonoperative methods have been advocated for the treatment of ACL tears. However, the optimum management of this injury remains controversial. In the present study, patients treated nonoperatively were evaluated retrospectively 4 to 10 years after ACL tears were documented by arthroscopy and by mild to moderate pivot shifts under anesthesia. Forty-nine patients who underwent arthroscopic evaluation of the knee between 1976 and 1982 were found to have complete tears of the ACL. A mild to moderate pivot shift was present under anesthesia. One or both menisci were torn in two-thirds of the knees, requiring partial meniscectomies. The average age of the patients was 27 years (range, 16 to 46 years). Because of persistent disabling instability, 9 patients (18%) underwent late ACL reconstruction. The remaining 40 patients were evaluated at an average of 5.6 years after documentation of the tear (range, 4 to 10 years). At followup, 25 (62%) of the 40 patients had satisfactory subjective results. Eight of the patients (20%) had returned to their preinjury level of athletic activities without restrictions, and 10 (25%) functioned at the same level but with symptoms, some patients requiring bracing. Seventeen patients (43%) had diminished their level of sports activities, while 5 patients (12%) had given up all sports. Only 2 patients required late meniscectomies. On physical examination, 27 patients (87%) had pivot shifts. Instrumented laxity testing revealed an injured to normal difference of 3.1 mm with a 20 pound force. Radiographic studies were interpreted as normal in 35% of the knees, whereas 65% demonstrated mild degenerative changes.(ABSTRACT TRUNCATED AT 250 WORDS)

Synovial chondromatosis of the acromioclavicular joint. A case report.

Synovial chondromatosis involving the acromioclavicular joint occurred in a 20-year-old woman. Synovectomy including removal of loose bodies and resection of the distal end of the clavicle relieved symptoms. Synovial chondromatosis is a relatively rare disorder and seems not to have been reported in the acromioclavicular joint.

Anterior and posterior marginal fracture-dislocations of the distal radius. An analysis of the results of treatment.

Anterior and posterior marginal fractures of the distal end of the radius associated with dislocation of the carpus are rare injuries. The results of 20 patients with 12 anterior and eight posterior marginal fracture-dislocations of the distal radius were reviewed. Eleven patients had closed reduction and plaster cast immobilization, including three with external fixation, while nine patients required surgery and internal fixation. At a mean of 3.2 years, 40% were rated as excellent, 45% as good, 5% as fair, and 10% as poor. There was roentgenographic evidence of posttraumatic arthritis in 13 patients (65%). Major factors affecting the clinical results were accurate articular realignment and the presence of ipsilateral carpal injuries. There were no significant differences in results between anterior and posterior marginal fractures or between closed or operative methods of treatment when the radiocarpal articular surface was restored to less than 1 mm residual displacement. Restoration of articular congruency is the primary goal of management of these fractures.

Sonographic evaluation of the rotator cuff: correlation with arthroscopy.

High-resolution real-time sonography has been reported as a non-invasive means of evaluating the integrity of the rotator cuff. Fifty-two patients underwent both sonographic and arthroscopic evaluation of the rotator cuff. There were 42 men and 10 women with an average age of 47 years (range 28-71 years). The duration of shoulder pain averaged 24 months (range 3 months to 9 years). Thirty-five rotator cuff tears were noted arthroscopically. Twenty-seven of the tears were diagnosed by sonography for a sensitivity rate of 77%. Eleven of the 17 intact rotator cuffs were normal by sonography for a specificity rate 65%. The overall accuracy of sonography was 73% and the predictive value of a positive sonogram was 82%. Sonography appears to be a good initial screening examination in evaluating the integrity of the rotator cuff. It is noninvasive and relatively inexpensive. Technical limitations of this technique as well as the experience of the radiologist in performing and interpreting the examination play a major role in the accuracy of this test. As instrumentation improves and as experience in using this technique increases, we expect the accuracy of sonography in detecting tears of the rotator cuff to continue to improve.

The 40 Hertz auditory event-related potential: normal values and effects of lesions.

A model of the 40 Hz auditory event-related potential (40 Hz AERP) was developed and evaluated in a group of normal subjects and two patient groups. The model views the 40 Hz AERP as a combination of the brain-stem auditory evoked potential (BAEP) and a sinusoidal component presumed to arise from structures rostral to the brain-stem. Fourier analysis techniques were used to quantify changes in the sinusoidal component. The results obtained demonstrated that the phase of the sinusoidal component was quite stable in normal subjects, but was predictably altered as a consequence of thalamic or midbrain lesions. Lesions of the temporal lobe did not alter the phase of the sinusoidal component. These results were interpreted as being consistent with the model developed and suggesting a midbrain or thalamic origin for the 40 Hz sinusoid.