| 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. ( info) |
2/150. Autosomal dominant muscle cramp syndrome in a Japanese family. OBJECTIVES: To identify the clinical, electrophysiological, histological, and genetic characteristics of a Japanese family with a muscle cramp syndrome. methods: Fourteen patients (eight men, six women) were studied in four generations of a single family. Electrophysiological examinations were performed in four cases and muscle and nerve biopsies were performed on the propositus. RESULTS: The mode of inheritance seemed to be autosomal dominant. The cramps occurred during both exertion and at rest, and during sleep. Electromyographic examination indicated a neurogenic aetiology. There was a decreased number of large myelinated fibres in the sural nerve, and fibre type grouping in the quadriceps femoris muscle biopsy. CONCLUSIONS: The autosomal dominant muscle cramp syndrome in this family is probably caused by a polyneuropathy. ( info) |
3/150. Exertional myalgia syndrome associated with diminished serum ammonia elevation in ischemic exercise testing. A 36-year-old man with chronic severe exertional myalgias had a normal serum lactate elevation and diminished serum ammonia elevation on an ischemic forearm exercise test (IFET). The IFET is commonly performed in the evaluation of patients with complaints of exertional myalgias, cramps, and rhabdomyolysis. The finding of a normal serum lactate elevation and a diminished serum ammonia elevation after ischemic exercise is usually considered indicative of myoadenylate deaminase deficiency. However, myoadenylate deaminase activity was normal in this man's muscle biopsy specimen. This case suggests that a diminished serum ammonia elevation in the IFET is not always indicative of myoadenylate deaminase deficiency, a disorder of ammonia production. A diminished serum ammonia elevation in the IFET could also reflect an impairment of net ammonia efflux from muscle into blood. ( info) |
4/150. Superficial femoral artery branch avulsion after severe muscle cramping. Avulsion of a muscular branch of a major artery without a history of major trauma has not been reported to our knowledge. Occasionally, blunt and even minor trauma can result in injuries that seem out of proportion to the level of injury. We report a case of an avulsed muscular branch of the superficial femoral artery in a patient with recent thigh cramping. This injury is likely related to the intense tetany the patient described having before he came to the hospital. ( info) |
5/150. Clinical and pharmacological profile in a clenbuterol epidemic poisoning of contaminated beef meat in italy. Long-acting beta adrenergic agonists, such as clenbuterol accumulate in the liver, but not meat of treated farm animals, and result in epidemic poisonings in consumers. We describe an outbreak of poisoning in 15 people, following the consumption of meat. Clinical symptoms (distal tremors, palpitations, headache, tachipnoea-dyspnoea, and also moderate hyperglycaemia, hypokalemia and leucocytosis) were seen in nine hospitalised patients, starting about 0.5-3 h after poisoning, and disappearing within 3-5 days later. clenbuterol was found in the urine of all the symptomatic patients, at higher levels than pharmacokinetic computing (mean level 28 ng/ml, 36 h after ingestion), based on the levels found in the meat (1140-1480 ng/g edible tissue). Thus, epidemic poisoning can be produced following the consumption of contaminated meat. The need for a better definition of pharmaco- and toxico-kinetics, not only for drugs ingested as parent drug, but also when ingested as residues with animal tissues, is recommended. ( info) |
6/150. The stiff man syndrome: a psychiatric disease? Although the Stiff Man Syndrome has been traditionally viewed as a neurologic disorder, a number of recent articles have documented a pattern of familial, interpersonal, and intrapsychic stress which suggests that this disease may be, to a considerable degree, psychosomatic in origin. The present paper reviews several recent articles and focuses on a single case study. Although the medical diagnosis was earlier confirmed, an in-depth psychiatric evaluation and extensive psychological assessment showed many psychopathological concomitants associated with the severe muscle spasm and stiffness. A follow-up after about a year of group and individual psychotherapy showed that the symptoms were still in remission, and that the patient was functioning personally and occupationally on a significantly improved level. ( info) |
7/150. McArdle's disease in childhood: report of a new case. McArdle's disease (glycogenosis type V) is an inherited glycogen storage disease characterized clinically by myalgia, cramps and sometimes myoglobinuria, triggered by exercise. The onset of exercise intolerance is usually in late childhood or adolescence and diagnosis is exceptionally established during infancy. We report the case of a 6-year-old girl who had been complaining of aching muscles for a long time, and who presented after a near-drowning incident, with extensive muscle necrosis, probably secondary to myophosphorylase deficiency-induced cramps. These unusual manifestations led to the diagnosis of this rare disorder. We compare the clinical findings of this case to nine previous reports. This highlights the heterogeneous spectrum of this disease in childhood and supports the distinction of three clinical pictures in childhood: a neonatal form rapidly fatal, a milder form with congenital myopathic symptoms and a benign classical form with myalgia, cramps and pigmenturia. ( info) |
8/150. Specific muscle EMG biofeedback for hand dystonia. Currently available therapies have only limited success in patients having hand dystonia (writer's cramp). We employed specific muscle EMG biofeedback (audio feedback of the EMG from proximal large muscles of the limb that show abnormally high activity during writing) in 10 of 13 consecutive patients (age, 19-62 years; all males) with a duration of illness from 6 months to 8 years. In three patients, biofeedback was not applicable due to lack of abnormal EMG values. Nine patients showed dystonic posture during writing and had hypertrophy of one or more large muscles of the dominant hand. The remaining four patients showed either involvement of small muscles or muscle wasting. Ten patients were given four or more sessions of EMG audio biofeedback from the proximal large limb muscles, which showed maximum EMG activity. They also practiced writing daily with the relaxed limb for 5 to 10 min. Nine patients showed improvement from 37 to 93% in handwriting, alleviation of discomfort, and pain (assessed on a visual analogue scale). One patient did not show any improvement. Thus EMG biofeedback improved the clinical and electromyographic picture in those patients with hand dystonia who showed EMG overactivity of proximal limb muscles during writing. This specific type of EMG biofeedback appears to be a promising tool for hand dystonia and might also be applied to other types of dystonias. ( info) |
9/150. A woman with leg cramps and rash. A 68-year-old woman presented with generalized weakness and a rash on her lower extremities. The weakness began one week earlier, shortly after treatment for leg cramps and two days before the rash appeared. She had also had blood-streaked sputum a few days before admission. There was no history of bleeding diathesis, nose bleeding, hematuria, vasculitis, muscle or joint aches, cinchonism, or exposure to tuberculosis. She denied fever, chills, night sweats, leg pain or swelling, chest pain, or abdominal pain. ( info) |
| quinine is still frequently used by practitioners for the treatment of nocturnal leg cramps, despite the lack of food and Drug Administration (FDA) approval. We report the 15th case of quinine-induced hemolytic-uremic syndrome (HUS) in the medical literature. The likely mechanism by which quinine induces HUS is via quinine-dependent antibodies to blood cellular constituents. These antibodies likely cause endothelial damage and the resultant nephropathy, microangiopathic hemolytic anemia, and thrombocytopenia that define HUS. Although there is no set guideline for the treatment of quinine-induced HUS, most authorities consider plasmapheresis as the standard of care, especially in severe cases. Our patient required the longest known treatment duration (16 plasmapheresis treatments over a 37-day period) for disease resolution. The prognosis of quinine-induced HUS is excellent, with no deaths reported in the literature. ( info) |