Myasthenia gravis in old age. A retrospective study of 58 patients

The course in 58 cases of myasthenia gravis in subjects over 50 years of age is reported. They total about 30% of cases in all age-groups. 36.2% of the patients died after an average period of illness of 3.4 years. 58.6% initially showed only ocular symptoms and in 58.8% of these the myasthenia was likewise located only in the eyes in the further course of the illness. The prognosis of ocular myasthenia is very good if the myasthenic process does not extend to other muscle groups in the first or second year of illness. 41.2% of primary ocular myasthenia turned into other types, in most cases a generalized type, and these had a poor prognosis. 69.2% of these patients died after an average period of illness of 1.5 years due to myasthenia-induced complications. Cases of myasthenia which initially are of the generalized type, in which the average duration is only 2.7 years until death, and to some extent the bulbar types, also have a poor prognosis. Ocular myasthenic cases generally need only small doses of cholinesterase inhibitors. Some patients had no medical treatment because there was no subjective or objective need for it. Several patients were given doses of cholinesterase inhibitors which were too large; excessive doses are to be avoided because of the proven negative influence of cholinesterase inhibitors on the myoneural system. It is recommended that types of myasthenia with a poor prognosis be treated early with corticoids, ACTH or azathioprin, if the cholinesterase inhibitors have proved ineffective or it their effect is unsatisfactory. Thymectomy is advisable only in patients under 60 years of age.     

Recurrence of thymoma and myasthenia gravis after 19 years. A case report

Myasthenia gravis developed in a 51-year-old woman in 1956; she was found to have a thymoma. Complete remission of the myasthenia gravis occurred within 18 months of thymomectomy. In 1975, myasthenia gravis recurred in association with recurrence of the thymoma. Remission of the myasthenic state once more followed the thymomectomy. The asymptomatic period of 18 years is, to our knowledge, the longest reported interval for recurrence of a thymoma-associated myasthenia gravis; 15 years is the longest reported interval for recurrence of a thymoma.     

Immunoadsorption therapy for myasthenia gravis

The results of a multicentre trial were analysed to evaluate the efficacy of immunoadsorption therapy for severe generalised myasthenia gravis. Twenty patients with myasthenia gravis who were concurrently receiving high dose prednisolone and azathioprine therapy were treated with an affinity-type adsorbent, using tryptophan-linked polyvinyl alcohol gel (IM-TR), according to a standardised treatment protocol. The 20 patients received five adsorption treatments within a period of 10 days. In 11, pronounced improvement of myasthenic weakness was seen and long-term remission was maintained. The treatment was especially effective in patients with thymic hyperplasia. Circulating acetylcholine receptor (AChR) antibodies were reduced by about 60% by treating one plasma volume. There was no difference in the rate of removal of the AChR antibodies between patients with thymic hyperplasia and patients with thymoma. No serious complications occurred during 100 procedures. It was concluded that the immunoadsorption therapy with IM-TR is useful in controlling symptoms in patients with severe myasthenia gravis who are otherwise unresponsive.     

Effect of tensilon on the behavior of optokinetic nystagmus in myasthenia

In 14 myasthenics tensilon test was performed together with electronystagmographic recording of optokinetic nystagmus. The mean amplitude of deflections and the mean frequency of nystagmus were calculated before and after intravenous administration of tensilon 10 mg. The results were compared with those obtained in a control group. The test was positive in 11 myasthenics: the amplitude and frequency of nystagmus beats increased and extinction at the end of the test was absent or diminished. In the control group the test was negative, with the exception of 3 cases in which a slight increase in amplitude and frequency was observed. The authors stress the advantages and disadvantages of tensilon test and state that it is diagnostically valuable in myasthenia and myasthenic syndromes in subjects sensitive to the drug.     

Total hip arthroplasty with cement in patients who have rheumatoid arthritis. A minimum ten-year follow-up study

One hundred and six consecutive total hip arthroplasties with cement were performed by one surgeon, at least ten years before the time of the present clinical and radiographic review, in seventy-five patients who had adult-onset rheumatoid arthritis. Two patients (three hips) were lost to follow-up. Seven (7 per cent) of the remaining 103 hips were revised. The revisions were performed because of infection (three hips), dislocation (two hips), or aseptic loosening (two hips). Of the ninety-eight hips that were not lost to follow-up or revised because of infection or dislocation, eight (8 per cent) had radiographic loosening of the acetabular component and two (2 per cent) had radiographic loosening of the femoral component. Although the prevalence of radiographic loosening of the acetabular component was four times greater than the prevalence of radiographic loosening of the femoral component, the prevalence of revision because of aseptic loosening of the acetabular component was identical to that for the femoral component (one component each). These results compared favorably with those of total hip arthroplasty with cement, performed by the same surgeon, for the treatment of other diagnoses. Loosening of the acetabular component was significantly associated with a younger age at the time of the index operation (p = 0.03) and with acetabular osteolysis (p = 0.0006). Of forty-eight hips in thirty-two patients who survived for at least ten years, 96 per cent (forty-six hips) were considered by the patients to have a satisfactory result. At the time of the latest follow-up, twenty-four (75 per cent) of the patients had no pain in the hip. Although eighteen patients (56 per cent) could walk without support at a minimum of ten years after the operation, we found that the functional results for patients who had rheumatoid arthritis were inferior to those observed for patients who had had a total hip arthroplasty with cement, performed by the same surgeon, for the treatment of other diagnoses.     

Treatment of infection with débridement and retention of the components following hip arthroplasty

Forty-two patients (forty-two hips) who had an infection following a hip arthroplasty were managed with open débridement, retention of the prosthetic components, and antibiotic therapy. After a mean duration of follow-up of 6.3 years (range, 0.14 to twenty-two years), only six patients (14 per cent) -- four of nineteen who had had an early postoperative infection and two of four who had had an acute hematogenous infection -- had been managed successfully. Of the remaining thirty-six patients, three (7 per cent of the entire group) were being managed with chronic suppression with oral administration of antibiotics and thirty-three (79 per cent of the entire group) had had a failure of treatment. All nineteen patients who had a late chronic infection were deemed to have had a failure of treatment. Débridement had been performed at a mean of six days (range, two to fourteen days) after the onset of symptoms in the patients who had been managed successfully and at a mean of twenty-three days (range, three to ninety-three days) in those for whom treatment had failed. Débridement with retention of the prosthesis is a potentially successful treatment for early postoperative infection or acute hematogenous infection, provided that it is performed in the first two weeks after the onset of symptoms and that the prosthesis previously had been functioning well. In our experience, this procedure has not been successful when it has been performed more than two weeks after the onset of symptoms. Retention of the prosthesis should not be attempted in patients who have a chronic infection at the site of a hip arthroplasty as this approach universally fails.     

Complications and long-term results of ankle arthrodeses following trauma

A review of sixty patients who had undergone ankle fusion for post-traumatic arthritis revealed that thirty-five (58 per cent) had the procedure performed within the first year after injury. A total of forty-eight complications occurred in twenty-nine (48 per cent) of the patients. Frequent complication were infection (23 per cent), non-union (23 per cent), inadequate surgical alignment or early loss of position (15 per cent), malunion (12 per cent), and delayed union (7 per cent). The lateral transfibular approach had the highest incidence of complications, and a two-incision approach using the Charnley compression apparatus was the procedure with the fewest complications. Forty-one patients were followed for an average of 7.5 years after operation. Of these, thirty-four (83 per cent) were satisfied with the procedure. Examination of thirty of the forty-one patients at an average of 7.3 years after surgery revealed virtually no subtalar motion but motion of 13 degrees at Chopart's joint. With shoes, patients had a near-normal gait. The roentgenograms revealed a minimum amount of degenerative arthritis at Chopart's joint, which may worsen with time. Varus or valgus angulation of the hind part of the foot was associated with a greater degree of symptoms in the subtalar area as well as the middle of the foot. The neutral position in varus-valgus angulation as well as dorsiflexion-plantar flexion was the optimum position for both men and women. The results of the procedure did not deteriorate with time.     

Long-term results of total hip replacement in young patients who had ankylosing spondylitis. Eighteen to thirty-year results with survivorship analysis

We determined the long-term results of total hip replacement in a series of young patients who had ankylosing spondylitis. Between 1966 and 1978, forty-three Charnley low-friction arthroplasties were performed in twenty-four patients who had an average age of 28.8 years (range, nineteen to thirty-nine years) at the time of the operation. There were seventeen men and seven women. The average duration of follow-up for the series was 22.7 years (range, one month [a perioperative death] to 30.3 years). Four patients (seven hips) had died an average of 12.6 years (range, one month to 18.7 years) after the operation. The twenty surviving patients had clinical and radiographic follow-up until the time of writing or until both of the original components had been revised. All patients had substantial relief of pain and improvement of function and the range of motion of the joint. Twenty-one patients (88 per cent; thirty-nine hips) were completely free of pain (6 points, according to the scale of Merle d'Aubigne and Postel), and the remainder had only slight discomfort (5 points). Ten acetabular components and one femoral component were revised because of aseptic loosening, and one patient had a revision of both components because of late deep infection. Three additional femoral components were revised during a revision operation for a loose acetabular component. Although the femoral components were not loose, they had been in place for more than ten years and it was thought likely that the bearing surface was damaged. The average time to revision was 13.3 years (range, 4.0 to 20.3 years). At an average of 22.7 years, thirty-eight (88 per cent) of the original femoral components and thirty-two (74 per cent) of the original acetabular components remained in situ. The average annual rate of acetabular wear was 0.12 millimeter for the entire series. Only six hips (14 per cent) had minor heterotopic ossification, and none of the hips had clinically important ossification (class III or IV according to the system of Brooker et al.). To our knowledge, the present report describes the largest series of total hip arthroplasties, with the longest duration of follow-up, in young patients who had ankylosing spondylitis. Survivorship analysis with use of the Kaplan-Meier method revealed that the probability of survival of the femoral component (with 95 per cent confidence intervals) was 91 per cent (83 to 99 per cent) at twenty years and 83 per cent (72 to 94 per cent) at thirty years. The probability of survival of the acetabular components was 73 per cent (61 to 84 per cent) at twenty years and 70 per cent (57 to 83 per cent) at thirty years. The probability that both components would survive was 91 per cent (82 to 100 per cent) at ten years, 73 per cent (61 to 84 per cent) at twenty years, and 70 per cent (57 to 83 per cent) at thirty years. The Charnley low-friction arthroplasty provided consistently good long-term results, with a low rate of complications and revisions, in this group of young patients.     

Primary carcinoma of the gallbladder. The role of adjunctive therapy in its treatment

Of forty-three patients with carcinoma of the gallbladder discovered ih a twenty-five year period (during which 10,349 patients were diagnosed as having cholelithiasis), eighteen patients (42 per cent) had no obvious tumor outside of the gallbladder at the time of operation, nineteen patients (44 per cent) had local spread of the disease, and six patients (14 per cent) had abdominal carcinomatosis or distant metastasis. In the twenty-one patients who underwent surgical therapy for cure of their disease, the five year survival rate was 33 per cent. The more extensive surgical procedures (other than cholecystectomy alone) did not significantly increase survival. Neither the duration of the symptoms nor the pathologic type of the tumor altered the eventual outcome. No patients with tumor outside the gallbladder at the time of operation survived longer than two years. Compared to those who did not receive it, the fifteen patients treated postoperatively with adjunctive therapy (radiation therapy or chemotherapy or both) lived longer and also were significantly better palliated when tumor outside of the gallbladder was found at the time of operation. From these findings, the routine use of adjunctive therapy is recommended in all patients with disease outside of the gallbladder and serious consideration should be given to its use in all patients found to have carcinoma of the gallbladder.     

Effect of seminal plasma on implantation rates

Of 2774 consecutive, prospectively documented fractures of the distal radius, 225 (8 per cent) occurred as sports injuries, mainly in young men. Soccer produced the greatest number of wrist fractures with 112 cases (50 per cent). Skiing, dancing and rugby caused 12 per cent, 9 per cent and 7 per cent of all sporting wrist fractures respectively. Skiing, horseriding and dancing consistently resulted in more complex fractures. In soccer, synthetic pitches increased the likelihood of a fracture following a fall by a factor of five. Twelve per cent of fractures required further treatment because of instability leading to redisplacement. The complication rate was 14 per cent with the majority being cases of malunion (12 per cent). Of the 131 patients who returned a questionnaire, 72.5 per cent had returned to their original sport. This was influenced mainly by the patient's age and pre-injury standard of competition.     

Free revascularized jejunal loop repair following total pharyngolaryngectomy for carcinoma of the hypopharynx: report of 90 patients

Ninety patients with carcinoma of the hypopharynx underwent pharyngolaryngectomy and reconstruction with a jejunal free autograft. Fifty-five patients had primary surgery and 35 salvage surgery for recurrence after radiotherapy. Following primary surgery 28 patients had postoperative radiotherapy and 27 did not. Complications occurred in 51 per cent of patients, the most common being necrosis of the jejunal graft (19 per cent); 12 per cent developed significant stenosis and 4 per cent died in the perioperative period. Eleven per cent of patients developed a fistula. The total number of complications diminished as the experience of the unit increased. Median follow-up was 4.9 years. Of patients treated with primary surgery, 48 per cent developed primary site recurrence (at 3 years) and 53 per cent neck node recurrence (at 5 years). The tumour-specific 5-year survival rate for all patients was 42 per cent. Following primary surgery 28 per cent survived for 5 years and after salvage surgery the rate was 59 per cent. Positive resection margins and extensive neck disease adversely affected survival (P = 0.02 and P = 0.001 respectively). The free revascularized jejunal graft is a safe and predictable method of repair following total pharyngolaryngectomy.     

Myasthenia gravis: recognition of a human autoantigen at the molecular level

The symptoms of myasthenia gravis are primarily or exclusively due to an autoimmune response against the muscle nicotinic acetylcholine receptor (AChR) and this has been the object of intensive investigations for almost 20 years. A detailed picture at the molecular level of the interaction of this autoantigen with the key elements involved in the autoimmune response, such as anti-AChR antibodies, the T-cell receptor and restricting major histocompatibility complex molecules, is now emerging for both human myasthenia gravis and its experimental model, experimental autoimmune myasthenia gravis. Here, Maria Pia Protti and colleagues focus on the molecular interactions occurring in human myasthenia gravis and summarize recent information on pathogenic mechanisms of the autoimmune response, and the structure of epitopes recognized by B cells and CD4+ T cells of myasthenic patients on the AChR molecule.     

Progression of the curve in boys who have idiopathic scoliosis

The prevalence of curve progression was evaluated in 210 boys who had idiopathic scoliosis. A minimum age of eight years, a deformity of at least 10 degrees, and radiographic follow-up of one year or progression of the curve within the first year of follow-up were the criteria for inclusion in the study. Of the 210 patients, sixty-eight (32 per cent) had progression of 10 degrees or more. Four of the five patients who had had an initial curve of 50 degrees or more subsequently had a spinal arthrodesis. The risk of progression was significantly greater for patients who were at an earlier Risser stage (p < 0.002) and for those who were younger (p < 0.005). The risk of progression was also greater for patients who had had a larger curve at the time of presentation; of the sixty-three boys for whom the Risser grade was 1, 2, 3, 4, or 5 when they were first seen and who had a curve of 25 degrees or more, twenty (32 per cent) had progression, compared with only two (5 per cent) of the thirty-eight who had a Risser grade of 1, 2, 3, 4, or 5 and a curve of 24 degrees or less. Of the thirty-four patients for whom the Risser grade was 4 when they were first seen, five (15 per cent) had progression.(ABSTRACT TRUNCATED AT 250 WORDS)     

Prognosis of myasthenia gravis: a retrospective study of 380 patients

The 9139 follow-up records of 438 myasthenia gravis (MG) patients were reviewed. Excluding those patients who were diagnosed 5 or more years after symptom onset (n = 37) and those who experienced only oculomotor symptoms throughout follow-up (n = 21), there were 380 patients. A survival analysis approach was used to assess the influence of prognostic factors on the following endpoints: (a) stable complete remission, (b) complete remission of at least 6 months and (c) pharmacological remission of at least 6 months. Early diagnosis was associated with a better prognosis with respect to all endpoints. Thymectomy also improved the prognosis but only for those patients without thymoma. Later MG onset was associated with a higher tendency to achieve pharmacological remission.     

T helper cell recognition of muscle acetylcholine receptor in myasthenia gravis. Epitopes on the gamma and delta subunits

We tested the response of CD4+ cells and/or total lymphocytes from the blood of 22 myasthenic patients and 10 healthy controls to overlapping synthetic peptides, 20 residues long, to screen the sequence of the gamma and delta subunits of human muscle acetylcholine receptor (AChR). The gamma subunit is part of the AChR expressed in embryonic muscle and is substituted in the AChRs of most adult muscles by an epsilon subunit. The delta subunit is present in both embryonic and adult AChRs. Adult extrinsic ocular muscles, which are preferentially and sometimes uniquely affected by myasthenic symptoms, and thymus, which has a still obscure but important role in the pathogenesis of myasthenia gravis, express the embryonic gamma subunit. Anti-AChR CD4+ responses were more easily detected after CD8+ depletion. All responders recognized epitopes on both the gamma and delta subunits and had severe symptoms. In four patients the CD4+ cell response was tested twice, when the symptoms were severe and during a period of remission. Consistently, the response was only detectable, or larger, when the patients were severely affected.     

Effects of alternate-day prednisone therapy on respiratory function in myasthenia gravis

To determine the effects of alternate-day prednisone therapy on respiratory function in myasthenia gravis, eight patients were evaluated during "days on" and "days off" prednisone. The patients were treated with long-term (up to three years), high-single-dose, alternate-day oral prednisone. After patients had been controlled with alternate-day prednisone they had no episodes of acute respiratory insufficiency or myasthenic crises. Although a small reduction in respiratory function during the day off prednisone was seen in some patients, the change was not statistically significant for the group and was probably physiologically unimportant in most patients. The data indicate that in myasthenic patients who have no underlying lung disease respiratory function is not significantly compromised by administering the prednisone on alternate days.     

Frequency of N. gonorrhoeae, T. vaginalis, and C. albicans in female venereological patients. A one-year study

The frequency of Neisseria gonorrhoeae, Trichomonas vaginalis, and Candida albicans has been studied over a period of one year in women attending a venereal diseases clinic. A total of 1,347 women were investigated, all coming from the same catchment area. Gonorrhoea was established at the first visit in 506 patients (38 per cent.), who constituted 97.5 per cent. of the total number of cases of gonorrhoea. Trichomonas vaginalis was found in 272 (20 per cent.) and Candida albicans in 233 (17 per cent.). 176 patients (13 per cent.) had more than one pathogen. Of the patients attending, 22 per cent. (292 women) were so-called "named contacts". The frequency of gonorrhoea established at the first visit in these patients (64 per cent.), was significantly higher, but the frequency of symptoms did not differ from that in other gonorrhoea patients. The number of asymptomatic cases was so large that a single compulsory examination is undoubtedly very useful from the epidemiological point of view, but the value of repeated specimen collections for gonorrhoea is debatable. Complications of gonorrhoea were observed in 29 patients (6 per cent.) at the first visit.     

Clinical course and remission rate in patients with early rheumatoid arthritis: relationship to outcome after 5 years

OBJECTIVE: To investigate the clinical course in early rheumatoid arthritis (RA) patients followed prospectively, to relate course to outcome after 5 yr, and to try to identify prognostic features. METHODS: A total of 183 patients with definite RA and a mean disease duration of 11 months were included. Of these, 75% were rheumatoid factor (RF) positive; 85% carried the shared epitope, 32% on both alleles. Most patients were assessed every 6 months. Disability was evaluated with the Health Assessment Questionnaire (HAQ) and radiographic findings according to Larsen. Remission was defined in two ways: with the American Rheumatism Association (ARA) criteria and as 'no arthritis at least at one follow-up visit'. RESULTS: Twenty per cent achieved ARA-defined remission periods of at least 6 months duration; 21 were spontaneous and 18 drug induced. Average length of remission was 20.5 months. The remission periods constituted 7% of follow-up for all patients. Another 36% achieved remission according to the second definition. All 56% were considered to have a relapsing-remitting disease pattern, in contrast to the remaining 44% with a persistent disease pattern. More patients with persistent disease were treated with disease-modifying anti-rheumatic drugs (DMARDs) and had also received a larger number of different drugs. Outcome after 5 yr regarding disability, joint inflammation and joint damage was worse for patients with persistent disease. Neither ARA-defined remission nor disease pattern could be accurately predicted. CONCLUSIONS: Long-term ARA-defined remission was rare, constituting 7% of follow-up for the entire cohort. For those 20% achieving remission, this period represented 34% of their follow-up. A total of 56% had a relapsing-remitting disease pattern and 44% had a persistent disease pattern. This classification had prognostic implications with persistency being a bad prognostic sign.     

Acetylcholine receptor antibodies in the Lambert-Eaton myasthenic syndrome

Two patients were initially diagnosed with myasthenia gravis with elevated titers of acetylcholine receptor antibodies. Features including weakness that normalized with sustained contraction, areflexia, autonomic symptoms, and low-amplitude baseline compound muscle action potentials with abnormal increments following brief exercise and high-frequency repetitive stimulation, however, suggested that these patients had Lambert-Eaton myasthenic syndrome. One patient had antibodies directed against presynaptic calcium channels, confirming the diagnosis. The second patient was seronegative for these antibodies but had elevated titers of antistriated muscle antibodies. This shows that serologic studies can conflict with clinical and electrodiagnostic findings in patients with Lambert-Eaton syndrome. These cases also point out that acetylcholine receptor antibodies are not necessarily diagnostic of myasthenia gravis in patients with Lambert-Eaton syndrome. Instead, these antibodies could represent a nonpathogenic epiphenomenon.