Cases reported "Muscle Hypotonia"

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1/4. Monopharmacologic general anaesthesia with sevoflurane in paediatric patient with prader-willi syndrome.

    prader-willi syndrome (PWS) is a genetic disease caused by a loss of paternal genes located in chromosome 15. Children affected by this syndrome often have preterm delivery; during childhood the hallmarks are: severe infantile hypotonia and feeding problems. Afterward, neurologic manifestations, endocrine signs and dysmetabolic abnormalities are usually seen together with craniofacial manifestations and musculoskeletal abnormalities. obesity causes sleep abnormalities including sleep apnea. The case we present is of a 5 year old child (CA) scheduled for strabismus surgery. The child has a lot of typical (PWS) signs. A number of anaesthesiologic problems are associated with (PWS). Some of them relate to obesity, others to facial dysmorphism. Moreover, the syndrome may give a prolonged and exaggerated response to every sedative drug. P.W.S. is also characterized by thermoregulatory disorders. sleep apnea occurs often. Considering all these problems, we planned a monopharmacologic anaesthesiologic procedure using sevoflurane.
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2/4. Lumbosacral plexopathy following regional paracervical block anaesthesia.

    We report a 35 year old lady who developed lumbosacral plexopathy immediately following regional paracervical block anaesthesia for medical termination of pregnancy. There was only partial recovery at one year. The neurological deficit and its mechanisms are discussed.
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3/4. Prolonged neuromuscular blockade with atracurium.

    During general anaesthesia with oxygen, nitrous oxide and enflurane, a 29-year-old woman received a total of 105 mg (1.78 mg X k-1) of atracurium over a 2.5 hour period. The neuromuscular blockade could not be completely reversed with neostigmine and mechanical ventilatory support was necessary for three hours postoperatively. The patient received succinylcholine without unusual sequelae before and after this episode. This is the first report of a patient who exhibited prolonged weakness after receiving atracurium.
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4/4. mitochondrial myopathies: an unusual cause of hypotonia in infants and children.

    Children frequently undergo muscle biopsy for the workup of hypotonia under general anaesthesia which poses unique risks in patients with undiagnosed muscle disease. mitochondrial myopathies are a relatively newly recognized cause of myopathy and multisystem disease in both adults and children. The diagnosis is complex. In addition to causing myopathy, there are metabolic derangements present in some cases that may be life-threatening. We present three cases of children with hypotonia where the diagnosis was suspected in two patients, and confirmed in the third. The question of whether patients with mitochondrial myopathies are at increased risk for developing malignant hyperthermia is discussed.
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