| myasthenia gravis (MG) characteristically involves ocular, bulbar, and proximal extremity muscles. Distal extremity muscles are typically spared or less prominently involved. The authors performed a retrospective chart review of MG patients treated at two university-based neuromuscular clinics. From a total population of 236, nine patients (3%) had distal extremity weakness exceeding proximal weakness by at least one Medical research Council grade during their illness. hand muscles, particularly finger extensors, were involved more frequently than were distal leg and foot muscles. ( info) |
2/892. myasthenia gravis and polymyositis as manifestations of chronic graft-versus-host-disease. myasthenia gravis and polymyositis are each a rare manifestation of immune dysregulation in chronic graft-versus-host disease (cGVHD). We report a 4-year-old boy with idiopathic acquired aplastic anemia who developed myasthenia gravis 22 months and polymyositis 69 months after an allogeneic BMT (5/6 matched, MLC-nonreactive). The occurrence of both syndromes in one patient is unique. Autoimmune dysfunction may be associated with the development of cGVHD as demonstrated by the high incidence of prior aplastic anemia in BMT patients presenting with myasthenia gravis and polymyositis. Recognition of these neurologic manifestations is important in the diagnosis and treatment of cGVHD. ( info) |
3/892. Cryptococcal prostatic abscess in an immunocompromised patient: a case report and review of the literature. A case of cryptococcal prostatic abscess in a 65-year-old Chinese man with immunosuppression from treatment of myasthenia gravis is presented. The patient was diagnosed to have cryptococcaemia when he presented with fever and urinary symptoms. Further investigations confirmed cryptococcal meningitis and imaging studies showed a hypodense lesion in the prostate. This proved to be an abscess and it was deroofed transurethrally. histology of the prostatic tissue revealed the presence of cryptococcus. The prostate can be a site of persistent cryptococcal infection and may take the form of an abscess. It should be drained transurethrally to prevent relapse. ( info) |
4/892. Congenital myasthenia gravis: clinical and HLA studies in two brothers. Two brothers with congenital myasthenia gravis are described. In both, ptosis and ophthalmoplegia responded poorly to oral anticholinesterase therapy and to thymectomy. The brothers had two different HLA haplotypes and neither had the HLA-A1-B8-DW3 haplotypes which are commonly associated with myathenia gravis in adult-onset cases. ( info) |
| myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction caused by antibodies against the nicotinic acetylcholine receptor (AChR-Ab). We report a 16-year-old girl with severe MG who showed a poor response to plasma exchange and tryptophan-linked polyvinylalcohol gel immunoadsorption. Further improvement of muscle strength and decline of AChR-Ab could be achieved after initiation of protein-A immunoadsorption (PA-IA). maintenance therapy with PA-IA and intravenous pulses of cyclophosphamide resulted in a stabilisation of the disease, with a complete remission during the follow-up period of six months. We suggest that PA-IA may be valuable and safe in the management of patients with severe MG, and maintenance therapy with PA-IA and cyclophosphamide may prevent serious and potentially life-threatening relapses of the disease. ( info) |
6/892. Development of myasthenia gravis after interferon alpha therapy. Interferon (IFN) alpha is now used in the treatment of some malignant diseases and chronic viral hepatitis. There have been several reports of development of autoantibodies and autoimmune diseases or the deterioration of preexisting disorders in patients under treatment. We enclose a case of myasthenia gravis (MG) which developed after six weeks of treatment as fluctuating bilateral ptosis, intermittent diplopia, and mild weakness of limb and neck muscles. A test dose of edrophonium chloride was administered, resulting in improved muscle strength. Elevated anti acetylcholine receptor (AChR) antibody titer was found. Single fiber electromyography showed an increased jitter from extensor digitorum communis, frequently accompanied by transmission blocking. Repetitive electric 3 Hz stimulation of the abductor pollicis brevis muscle, revealed an abnormal decrement of 28% in compound motor action potential. myasthenia gravis was diagnosed and the patient was given pyridostigmine, immunoglobulines and prednisone with benefit. Six months latter he developed an acute myasthenic crisis with severe respiratory failure and high anti AChR antibody titer. IFN-alpha can induce MG or simply manifests a preexisting subclinical disease, but otherwise its therapeutic efficacy in MG has been shown in experimental and clinical studies. Autoimmune mechanisms, as the release of different cytokines as IFN, by immunocompetent cells, may be involved in the pathogenesis of both MG and chronic active hepatitis. Autoantibody production against postsynaptic membrane structures by IFN-alpha could be the underlying pathophysiology. ( info) |
7/892. myasthenia gravis presenting with dysphagia and postoperative ventilatory failure. We report a case of myasthenia gravis presenting to the department of otolaryngology with acute dysphagia on two separate occasions over a one-year period. diagnosis of myasthenia gravis was made when the patient developed ventilatory failure after his second general anaesthetic for rigid oesophagoscopy. Our patient required emergency transfer to the intensive therapy unit for ventilation. He improved after treatment with corticosteroids, anticholinesterase and immunosuppressive medications. Our case was unusual in that cricopharyngeal spasm causing dysphagia and significant aspiration was demonstrated by a barium swallow and this was completely resolved after treatment of the myasthenia gravis. ( info) |
| In a myasthenia gravis patient with profound ophthalmoplegia who was given edrophonium during forward fixation or horizontal/vertical saccades, horizontal macrosaccadic oscillations occurred that were conjugate, 2 to 3 Hz, and of peak-to-peak amplitude up to 50 degrees. The intersaccadic interval averaged 129.5 msec as measured by electro-oculography. eye closure or the use of Frenzel goggles markedly reduced the amplitude and frequency of the macrosaccadic oscillations. This short latency is similar to that of a corrective saccade, and suggests that edrophonium induces macrosaccadic oscillations through a non-visually guided reflex saccade by temporarily unmasking the adaptively increased saccadic gain to overcome myasthenic ocular paresis. ( info) |
| A nephrotic syndrome has been observed rarely in association with thymoma. In most of the reported cases, it occurs when the thymoma is in remission; histological examination generally shows minimal change disease. We report a case of a 43-year-old man presenting with minimal lesion nephrotic syndrome at the time of the diagnosis of thymoma and myasthenia gravis, which persists despite remission of the thymoma. The role of a disorder of T-cell function and of circulating cytokines is discussed. ( info) |
10/892. Can immunoadsorption plasmapheresis be used as the first choice therapy for neuroimmunological disorders? The subjects were 31 patients in whom immunoadsorption plasmapheresis (IAPP) was performed as the first choice therapy for primary or recurrent neuroimmunological disorders. The clinical manifestations before and after IAPP and the use of corticosteroids were investigated in the present study. IAPP was clinically effective in all patients. The corticosteroids (CSs) administration was begun or CSs were increased after completion of IAPP in 11 patients. IAPP was performed as the first choice therapy, and favorable results were obtained in patients with guillain-barre syndrome and Miller-Fisher syndrome. IAPP alone was also effective in a patient with lupoid sclerosis. When IAPP was used alone in 2 patients with chronic inflammatory demyelinating polyradiculoneuropathy, it completely eliminated the clinical manifestations, but the symptoms recurred about 2 months later. Therefore, although IAPP could be performed as the first choice therapy for many neuroimmunological disorders, subsequent therapies should be carefully investigated. ( info) |