1/35. 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 = person (Clic here for more details about this article) |
2/35. 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 = 6keywords = person (Clic here for more details about this article) |
3/35. 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 = 5keywords = person (Clic here for more details about this article) |
4/35. 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 = 5keywords = person (Clic here for more details about this article) |
5/35. stiff-person syndrome associated with cerebellar ataxia and high glutamic acid decarboxylase antibody titer.glutamic acid decarboxylase (GAD) is the main target of humoral autoimmunity in patients with insulin-dependent diabetes mellitus (IDDM) and stiff-person syndrome. We reviewed the case of a 46-year-old woman who had cerebellar ataxia before getting stiff-person syndrome and IDDM with high anti-GAD autoantibody titers. This was a rare case in which there were both the clinical symptoms of stiff-person syndrome and cerebellar ataxia. In western blot analysis her serum reacted with 65-kDa proteins from rat cerebellum, cerebral cortex, and spinal cord. autoantibodies to GAD may cause functional impairment of gamma-aminobutyric acid (GABA) neurons in the spinal cord as well as in the cerebellum.- - - - - - - - - - ranking = 7keywords = person (Clic here for more details about this article) |
6/35. 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 = 6keywords = person (Clic here for more details about this article) |
7/35. Stiff person syndrome and myasthenia gravis.association of stiff person syndrome, an immune related disorder of anterior horn cells and myasthenia gravis an endplate disorder with similar pathogenesis, is rare. This communication documents this association in the Indian literature for the first time.- - - - - - - - - - ranking = 5keywords = person (Clic here for more details about this article) |
8/35. 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 = 5keywords = person (Clic here for more details about this article) |
9/35. Progressive encephalomyelitis with rigidity presenting as a stiff-person syndrome.diagnosis criteria of stiff-person syndrome (SPS) include progressive, fluctuating muscular rigidity and spasms with normal neurological examination. The presence of unusual features such as prominent limb rigidity with segmental signs and contracture, evidence of brainstem dysfunction, profound autonomic disturbances, CSF pleiocytosis or MRI abnormalities in patients with SPS presentation allows to classify these patients as progressive encephalomyelitis with rigidity (PER). We report a 50 year-old woman suffering from severe painful spasms of abdominal wall and limb muscles. Neurological examination showed pyramidal signs. EMG disclosed continuous muscle activity with superimposed discharges. Treatment with high doses of diazepam and baclofen led to moderate improvement of generalised stiffness. However, the right arm became more rigid with oedema and vasomotor changes. Subsequently, bilateral nystagmus and internuclear opthalmplegia appeared. There was mild CSF pleiocytosis. Associated auto-immune thyroiditis was found with positive anti-microsome antibodies and decreased thyroid hormones. Search for profound neoplasm was negative. The patient had three subacute bouts then she improved with methylprednisolone. The initial clinical presentation mimicking a SPS with subsequent diffuse involvement of the central nervous system and a striking localisation of a severe rigidity to one arm allowed to suspect the diagnosis of PER. The relationship between SPS and PER remains unclear because of the rarity of these disorders. The observation reported in this paper gives evidence that both the disorders are probably two clinical presentations of the same pathogenic process.- - - - - - - - - - ranking = 5keywords = person (Clic here for more details about this article) |
10/35. stiff-person syndrome associated with oral isotretinoin treatment.We describe a patient with severe nodulocystic acne who developed disabling muscle stiffness and painful superimposed spasms of the neck, back and upper limbs 10 days after the onset of oral isotretinoin treatment. The muscle hyperactivity condition, which revealed the clinical and electromyographic features of the stiff-person syndrome, gradually resolved 2 weeks after drug withdrawal.- - - - - - - - - - ranking = 5keywords = person (Clic here for more details about this article) |
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