1/19. 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.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/19. quinine induced coagulopathy--a near fatal experience.A 67 year old man presented to his local dentist for restorative treatment. He stated he was fit and well and denied taking any medications. When he was given an inferior alveolar nerve block, excessive bleeding was noted at the injection site and the dentist advised the patient see an oral and maxillofacial surgeon. An appointment was made for the patient but he did not attend. Three days later, he presented with evidence of massive deep haemorrhage to the point of airway compromise. He underwent hospital admission, early intubation, intensive care for nine days and hospitalization for six weeks. The cause of his bleeding was a severe thrombocytopoaenia, induced by chronic ingestion of quinine. He was self-medicating with this to relieve muscular cramps. Despite this experience, the patient continued to deny that quinine was the cause of his problem and that he had failed in his obligations to advise the dentist of his drug history. dentists need to be alert to the risk that patients may not reveal their true medical history. There are, however, obligations on the dentist to ensure the accuracy of information the patient gives and to ensure that patients whom they believe are at risk follow their advice. Teamwork and skillful airway management prevented this patient's demise.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
3/19. Axillary injection of botulinum A toxin in a patient with muscle cramps associated with severe axillary hyperhidrosis.Muscle cramps may be caused by fluid and salt loss induced by diffuse or focal hyperhidrosis. Recent reports have described the efficacy of botulinum, toxin in the treatment of primary focal hyperhidrosis. Botulinum toxin inhibits sweating by blocking exocytosis of acetylcholine from presynaptic cholinergic nerve terminals. We report the case of a patient who complained of frequent muscle cramps associated with unusually severe axillary hyperhidrosis. We used botulinum toxin to treat the excessive focal sweating presuming that it would also reduce the muscle cramps. A total dose of 200 MU of botulinum A toxin (Dysport) per axilla markedly reduced sweating and cramps. The beneficial effect started four days after the injection and it was still present five months later. Treatment was repeated in the sixth month with analogous results. No side-effects were observed and no compensatory sweating occurred.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
4/19. Repetitive nerve stimulation and muscle membrane excitability: case report and review.Few muscle disorders can be diagnosed by repetitive nerve stimulation (RNS). Decreasing compound muscle action potentials (CMAP) on high frequency RNS is recorded in muscle channelopathies, and particularly in sporadic and recessive congenital myotonia. In this myopathy, decreasing CMAP after exercise test and RNS are the most sensitive electrophysiological in detecting muscle membrane dysfunction and are considered highly informative even in mildly symptomatic patients. We report on a patient with excercise-induced diffuse muscle cramps and myalgia; muscle biopsy and laboratory investigations were normal. Decreasing CMAP on high frequency RNS suggested muscle membrane conduction anomalies and, though clinical and electrical myotonia was not detected, the neurophysiological finding raised the suspicion of congenital myotonia and addressed to molecular investigation.- - - - - - - - - - ranking = 2.5keywords = nerve (Clic here for more details about this article) |
5/19. Medial compartment syndrome of the foot: an unusual complication of spine surgery.STUDY DESIGN: Descriptive case report. OBJECTIVES: To report the case of a child with medial compartment syndrome of the foot following posterior spinal instrumentation and fusion. SUMMARY OF BACKGROUND DATA: No previous study has reported medial compartment syndrome of the foot following spinal surgery. methods: A 15-year-old female with progressive idiopathic scoliosis was taken for posterior instrumentation and fusion. The patient had a history of severe postexertional cramping in the feet following athletics. Surgery progressed uneventfully and the patient was continuously monitored with somatosensory-evoked potentials, which showed no changes. In the recovery room, the patient complained of severe cramping in one foot that was similar to her postexertional cramping. This was lessened with massage and ketorolac. Soreness continued in the foot into postoperative day one and then increased overnight. On the morning of postoperative day 2, pressure in the medial compartment was found to be 97 mm Hg and she was taken for fasciotomy, which found necrosis of the abductor hallucis muscle, and all other compartments of the foot were normal. RESULTS: At the 6-month follow-up, the patient is doing well with no known sequelae. CONCLUSION: This was a very rare case of medial compartment syndrome of the foot following spine surgery. We believe that the patient had a predisposition, whether neurologic or vascular, toward cramping in the foot and that this activity was stimulated by the nerve stimulation during the evoked potential monitoring. Although the patient had thoracic epidural analgesia after surgery, it was not felt to have contributed to the development or result of the compartment syndrome. We strongly advocate for checking patients feet and legs during surgery for overactivity and stress the need for a high index of suspicion for compartment syndrome for unexplained pain after surgery.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
6/19. Hereditary neuropathy with liability to pressure palsies (HNPP) in a toddler presenting with toe-walking, pain and stiffness.The typical clinical presentation of hereditary neuropathy with liability to pressure palsies is an adult-onset recurrent, painless monoparesis. Electrophysiological abnormalities--decreased nerve conduction velocities and delayed distal latencies--can be detected even in asymptomatic patients. We describe a toddler, who presented with asymmetric toe walking, painful cramps and stiffness in the legs. He had calf hypertrophy, brisk tendon reflexes and bilateral Babinski signs and the electrophysiological examination was normal. The unlikely diagnosis of hereditary neuropathy with liability to pressure palsies was reached 5 years later, when the boy started to complain of episodic numbness and weakness in the upper extremities. His father, paternal aunt and grandmother had similar symptoms, but they had never been investigated. The typical 1.5 Mb deletion on chromosome 17p11.2-12 was found in our patient and his affected relatives.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
7/19. tocainide therapy in muscle cramps and spasms due to neuromuscular disease.Significant improvement in symptoms of disabling cramps and muscle spasms was obtained in 9 patients with motor neuron diseases, tetany, and myotonic disorders who were treated with tocainide, a lidocaine analog. No significant side effects were observed except for light-headedness and fatigue in 1 patient, who also showed slight prolongation of intraventricular conduction time. tocainide is useful in treating disabling muscle spasms and cramps associated with conditions characterized by neuromuscular irritability. This effect is probably based on stabilization of the membrane potential at various levels (motor neurons, peripheral nerve, or muscle fiber membrane).- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
8/19. Incapacitating peripheral neuropathy as a manifestation of chronic graft-versus-host disease.A 35-year-old caucasian man developed mild and transient signs of chronic graft-versus-host disease (GVHD) 5 months after bone marrow transplantation. At 16 months he presented with painful cramps in hands, feet, and truncal muscles. Electrophysiological studies revealed generalized sensory neuropathy. There was occasional fasciculation and high frequency motor units during involuntary muscle contractions, and agonist/antagonist cocontractions of peripheral muscle groups (non-continuous). A sural nerve biopsy showed schwann cells and macrophages with myelin debris, and Schwann cell stacks with collagen pockets. The patient's symptoms responded to treatment with glucocorticoids and azathioprine. We propose that this patient's incapacitating polyneuropathy was a major manifestation of chronic GVHD, suggesting that the peripheral and possibly central nervous systems can be targets of chronic GVHD.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
9/19. The syndrome of continuous muscle fibre activity: light and electron microscopic studies in muscle and nerve biopsies.Histological and ultrastructural studies were performed on nerve and muscle biopsy specimens from two patients with the syndrome of continuous muscle fibre activity. The characteristics of muscle biopsies were as follows. By light microscopy, internal nuclei were present in many of the fibres. By electron microscopy many fibres contained filamentous bodies and subsarcolemmal aggregates of mitochondria embedded in the peripheral zone of cytoplasm, and occasional mitochondria with disorganized or branched cristae were larger than normal. Biopsies of sural nerves revealed a decreased number of myelinated fibres, clusters of small myelinated fibres, and evidence of active axonal degeneration such as disintegrated myelin segments and degenerated axon components, as well as loss of axonal contents. With the present biopsy findings, it is suggested that the pathological process of this syndrome affects peripheral nerves as well as muscles.- - - - - - - - - - ranking = 3.5keywords = nerve (Clic here for more details about this article) |
10/19. Myoadenylate deaminase deficiency in children.Myoadenylate deaminase (MADA) is an enzyme which participates in the purine nucleotide cycle necessary for energy production in human skeletal muscle. Approximately 35 patients with deficiency of this enzyme have been reported; one-half experienced their initial difficulties in childhood. Children with "primary" MADA deficiency typically have symptoms including muscle cramps, stiffness, and post-exercise myalgia and weakness. In "secondary" MADA deficiency, the clinical findings have been variable with delayed motor development, hypotonia, cardiomyopathy, delayed speech development, and generalized weakness. In most cases creatine kinase determinations, nerve conduction velocity studies, and routine muscle histopathology have been normal. diagnosis has been established by demonstrating an absence of MADA activity by either direct muscle enzyme assay or histochemical staining. In this report we describe a 12-year-old boy with primary MADA deficiency and contrast his symptoms with those of previously described pediatric patients.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
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