1/46. neurophysiology of orthostatic tremor. Influence of transcranial magnetic stimulation.A 74-year-old patient suffers from painful muscle cramps when he stands since 30 years. He has no visible tremor but 16 Hz burst activity on EMG, indicating orthostatic tremor. Previous diagnosis was hysteria, stiff person syndrome or dystonia. This shows that EMG during standing should be part of the examination of patients with stiff muscles or muscle cramps. tremor was not strictly orthostatic. It appeared in back muscles while sitting, when the patient supported a weight with outstretched arms. Phase between muscles differed between normal standing and standing on heels. Subthreshold transcranial magnetic stimulation modulated timing of the tremor bursts and inhibited them at higher intensity stimulation.- - - - - - - - - - ranking = 1keywords = cramp, muscle (Clic here for more details about this article) |
2/46. Long-term remission of refractory stiff-man syndrome after treatment with intravenous immunoglobulin.The stiff-man syndrome is a rare neuromuscular disorder characterized by progressive rigidity, stiffness, and intermittent spasm of axial and extremity muscles. Its etiology is unknown. Different therapeutic regimens have been used with variable success. We present a case of refractory stiff-man syndrome, in which the symptoms were successfully controlled by the administration of intravenous immunoglobulin (IVIg). This case gives evidence that IVIg can be a safe and an efficient treatment of refractory stiff-man syndrome. The exact indication for and the cost-effectiveness of IVIg in the treatment of this rare entity remain to be determined.- - - - - - - - - - ranking = 0.0096369315725031keywords = muscle (Clic here for more details about this article) |
3/46. Double filtration plasma exchange and immunoadsorption therapy in a case of stiff-man syndrome with negative anti-GAD antibody.We report the effects of double filtration plasma exchange and immunoadsorption therapy which were performed for a case of stiff-man syndrome even though the patient was negative for anti-glutamic acid decarboxylase (GAD) antibody. The patient underwent a course of four double filtration plasma exchanges, which resulted in marked clinical improvement. Painful muscle cramps disappeared and muscle stiffness reduced within a day after the first plasma exchange. The patient's improvement continued, but his condition declined again about ten months after plasma exchange. Immunoadsorption therapy was then performed, and this treatment was also effective.- - - - - - - - - - ranking = 0.49518153421375keywords = cramp, muscle (Clic here for more details about this article) |
4/46. Motor evoked potentials in a case of stiff-man syndrome: a longitudinal study.The clinical and neurophysiological findings in a patient with a typical stiff-man syndrome and their three-year evolution are described. The patient had high titers of anti-glutamic acid decarboxylase antibodies in both serum and cerebrospinal fluid. magnetic resonance imaging (MRI) of brain and spinal cord was normal. transcranial magnetic stimulation (TMS) revealed a distinctive motor evoked potential (MEP) pattern in proximal lower limb muscles consisting of markedly increased MEP amplitudes and MEP/M ratios, reduced excitability thresholds, and absent silent period. However, MEP latencies, central and peripheral conduction times and amplitudes obtained by magnetic spinal root stimulation were normal. Treatment with benzodiazepine and baclofen normalized both the clinical picture and the MEP values. TMS may be useful both as a diagnostic tool and to monitor the response to drug treatment.- - - - - - - - - - ranking = 0.011864339912188keywords = muscle, limb (Clic here for more details about this article) |
5/46. Stiff-person like syndrome in a patient with multiple pituitary hormone deficiencies.A patient with long-standing, occult pituitary insufficiency, who developed painful muscle stiffness and superimposed spasms, closely resembling stiff-person syndrome, was described. Complete resolution of neuromuscular symptoms with hormone replacement in this case, as well as in a previously reported one, led to the suggestion that a syndrome like stiff-person could represent a rare consequence of multiple pituitary hormone deficiencies.- - - - - - - - - - ranking = 0.0096369315725031keywords = muscle (Clic here for more details about this article) |
6/46. Focal stiff-person syndrome.stiff-person syndrome (SPS) is a disorder of motor function characterized by rigidity of axial musculature and fluctuating painful spasms, which are often induced by startle or emotional stimuli. Neurophysiological studies have demonstrated the presence of continuous motor unit activity in muscle at rest, with abnormally enhanced extereoceptive reflexes. Although criteria for the diagnosis of SPS were proposed, several variants of this syndrome have been described before. In this communication, we report the case of a patient with a focal form of SPS. A 39-year-old woman developed progressive instability in her gait, spasms and stiffness restricted to both legs. The electromyographic examination showed continuous motor unit activity of the affected muscles at rest. Moreover, high anti-GAD antibodies titers were found in CSF and serum. Clinical symptoms, electrophysiological and immunological profiles suggest a focal form of SPS. Clinical and immunological findings indicate that SPS is a heterogeneous disease, suggesting the need to redefine its diagnostic criteria. Definition of the range of clinical expression and immunological profiles could be important for the clinical management of these patients.- - - - - - - - - - ranking = 0.019273863145006keywords = muscle (Clic here for more details about this article) |
7/46. plasma exchange in stiff-man syndrome.Stiff-man syndrome (STS) is a rare neurological disorder characterized by involuntary axial and proximal limb rigidity and continuous motor unit activity on electromyography (EMG). autoantibodies to glutamic acid decarboxylase (GAD) present in 60% of the patients are implicated. We report on the use of plasma exchange (PE) in 2 patients with STS whose serum and cerebrospinal fluid were negative for GAD autoantibodies. One patient showed minimal clinical improvement following PE while the second reported subjective improvement, but not any different from that with medications. Based on the results of PE in our patients, it seems that those who are autoantibody negative are less likely to respond. Whether a more aggressive approach to PE will be beneficial remains speculative.- - - - - - - - - - ranking = 0.0022274083396845keywords = limb (Clic here for more details about this article) |
8/46. Stiff person syndrome.This communication considers a patient with episodic muscle spasms. Pharmacological intervention and electrophysiological studies provided adequate evidence confirming the diagnosis of stiff person syndrome. Electrophysiological studies in present communication similar to earlier reports, confirmed once again the autonomous state of the anterior horn cell in this disease process.- - - - - - - - - - ranking = 0.0096369315725031keywords = muscle (Clic here for more details about this article) |
9/46. stiff-person syndrome associated with invasive thymoma: a case report.We report a case of a 40-year-old female with continuous muscle stiffness and painful muscle spasms. The symptoms worsened over a two-week period after onset. Electrophysiological examinations revealed continuous muscle discharge, which was markedly reduced by intravenous administration of diazepam. High levels of anti-glutamic acid decarboxylase (GAD) antibodies were detected in both serum and cerebrospinal fluid, suggesting that the patient suffered from stiff-person syndrome. Steroid pulse therapy and immunoadsorption therapy alleviated the clinical symptoms and decreased the anti-GAD antibody titer. A chest CT revealed the presence of an invasive thymoma. Neither anti-acetylcholine receptor (AChR) antibodies nor symptoms of myasthenia gravis (MG) were observed. The patient underwent a thymectomy and postoperative radiotherapy. These treatments further alleviated the clinical symptoms. The present case is the first that associates stiff-person syndrome with invasive thymoma, and not accompanied by MG. The autoimmune mechanism, in this case, may be triggered by the invasive thymoma.- - - - - - - - - - ranking = 0.028910794717509keywords = muscle (Clic here for more details about this article) |
10/46. Atypical low back pain: stiff-person syndrome.stiff-person syndrome was diagnosed in a patient with chronic low back pain. The diagnosis of this rare neurological condition rests mainly on the clinical findings of axial and proximal limb rigidity, increased lumbar lordosis often accompanied with pain, and normal neurological findings apart from brisk deep tendon reflexes. electromyography of the lumbar paraspinal muscles shows motor unit firing at rest with normal appearance of the motor unit potentials. Titers of antibody to glutamic acid decarboxylase are elevated. diazepam is the treatment of reference. Physical therapy can substantially improve quality of life.- - - - - - - - - - ranking = 0.011864339912188keywords = muscle, limb (Clic here for more details about this article) |
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