1/56. Acute neuromuscular respiratory failure after ICU discharge. Report of five patients.OBJECTIVE: To describe a syndrome of acute neuromuscular respiratory failure (NM-ARF) caused by ICU-acquired acute myopathy and neuropathy. DESIGN: Case series. SETTING: General Regional University Hospital in Brescia, italy. patients: Five adult patients with NM-ARF after prolonged ICU stay and successful weaning from the ventilator and ICU discharge. INTERVENTIONS: None. MEASUREMENTS: Clinical signs of NM-ARF, electroneurography and electromyography (ENMG) of peripheral nerves and muscles, and functional assessment of respiratory muscles. RESULTS: NM-ARF was diagnosed at the time of (one case), or 1-3 days after, ICU discharge. Limb weakness alarmed the physicians, while the signs of the NM-ARF were initially undetected. In the first observed case the acute respiratory failure was near fatal, and necessitated ICU readmission, while in the other cases 2 weeks of aggressive chest physiotherapy permitted resolution of the respiratory failure. history, clinical course and ENMG indicated the diagnosis of critical illness myopathy and neuropathy (CRIMYNE). Three patients recovered fully, while two had persisting evidence of axonal polyneuropathy several months after the onset. CONCLUSIONS: Critically ill patients with prolonged ICU stay, sepsis and MOF are at great risk of developing CRIMYNE, which in turn may be responsible for NM-ARF. This latter complication may arise after resolution of the respiratory and cardiac dysfunctions and successful weaning from the ventilator. As NM-ARF may cause unplanned ICU readmission or even unexpected death, strict clinical surveillance and monitoring of respiratory muscle function is recommended after discharge to the general ward of patients with proven NM-ARF. Early intensive chest physiotherapy can resolve the condition.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
2/56. Reversibility of paraneoplastic bilateral diaphragmatic paralysis after nephrectomy for renal cell carcinoma.Bilateral diaphragmatic paralysis is usually caused by anatomic lesions of both phrenic nerves (e.g., after cardiothoracic surgery), generalized neurologic diseases (e.g., primary motor neuron disease, amyotrophic lateral sclerosis) or is without a known cause (idiopathic). We report a case of a patient with renal cell carcinoma complicated by an isolated bilateral diaphragmatic paralysis without clinical or electromyographic signs of other muscle or nerve involvement. There has been progressive, though till now partial, recovery of his vital capacity rising from 44% to 72% of predicted values, and maximal inspiratory pressures during the two years following the curative resection of his renal cell carcinoma. We believe this is the first report of a paraneoplastic bilateral diaphragmatic paralysis with actual recovery after tumour therapy.- - - - - - - - - - ranking = 0.33333333333333keywords = muscle (Clic here for more details about this article) |
3/56. Diaphragmatic spinal muscular atrophy with bulbar weakness.We present the clinical and histopathological features of a child affected by diaphragmatic spinal muscular atrophy. The child was born with mild distal arthrogryposis, mild hypotonia and developed marked diaphragmatic and bulbar muscle weakness in the first week of life. Electrophysiological and pathological investigations performed at presentation were not conclusive, while the investigations performed at 3 months showed a clear neurogenic picture. Genetic studies excluded involvement of the SMN gene, or of other genes located on chromosome 5q, confirming that this syndrome represents a different entity from typical proximal spinal muscular atrophy.- - - - - - - - - - ranking = 0.33333333333333keywords = muscle (Clic here for more details about this article) |
4/56. Hemidiaphragmatic paresis after interscalene supplementation of insufficient axillary block with 3 mL of 2% mepivacaine.Breathing difficulty, agitation, and confusion developed in a 55-year-old male, ASA classification group III with a non-small-cell lung cancer 10 min after interscalene supplementation of insufficient axillary block with 3 mL of 2% mepivacaine with adrenaline 5 microg mL(-1). After administration of thiopentone and suxamethonium the patient's trachea was intubated and the lungs were ventilated with oxygen-enriched air. The block was successful and surgery was conducted as scheduled. Radiographic monitoring of the lungs at the end of operation showed ipsilateral elevation of the diaphragm with reduced respiratory excursions. Postoperatively, the patient was somnolent and hypercapnic, but maintained satisfactory oxygenation while breathing spontaneously and was extubated. Both the temporal relationship of events and the regression of all symptoms within three hours suggest that 3 mL of mepivacaine with adrenaline injected into the interscalene space blocked the phrenic nerve and compromised diaphragmatic function, which precipitated the respiratory failure.- - - - - - - - - - ranking = 62.72913018753keywords = paresis (Clic here for more details about this article) |
5/56. Bilateral phrenic paralysis in a patient with systemic lupus erythematosus.Respiratory manifestations of systemic lupus erythematosus (SLE) are frequent. They include respiratory muscle abnormalities, which have been implicated in the pathogenesis of the "shrinking lung syndrome" (SLS). We report the case of a patient with this syndrome, in whom diaphragmatic paralysis due to demyelinating phrenic lesions was diagnosed at the same time as SLE. follow-up studies showed a favorable clinical and diaphragmatic outcome with corticosteroid therapy, but little change in spirometry. It is concluded that severe diaphragm palsy is possibly due to phrenic nerve lesions in SLE, and that the link between diaphragm dysfunction and the SLS is probably not a straightforward one.- - - - - - - - - - ranking = 0.33333333333333keywords = muscle (Clic here for more details about this article) |
6/56. Phrenic paresis--a possible additional spinal cord dysfunction induced by neck manipulation in cervical spondylotic myelopathy (CSM): a report of two cases with anatomical and clinical considerations.The clinical records of two male subjects with severe cervical spondylotic myelopathy (CSM) who developed respiratory insufficiency after the cervical manipulation involved in preoperative anesthetic intubation were examined. Their cervical imaging was analyzed with respect to the known anatomic relationships of the spinal phrenic nerve nuclei to the spondylotic compressive lesions in an attempt to provide the anatomic and pathologic rationales that may explain this phrenic paresis as a possible traumatic complication of severe CSM. Perusal of extant literature revealed extensive descriptions of CSM symptoms, but none had previously reported an associated neuromuscular weakness of the diaphragm. magnetic resonance imaging analyses indicated that the existing degree of upper cervical cord compression, when reinforced by the additional posterior and anterior pressures consequent to cervical spinal extension and flexion, could readily account for the functional impairment of phrenic nerve neuron cells and/or their efferent fibers. Thus, the anatomic relations of the phrenic nerve nuclear columns and their efferent tracts predispose them to interference by compressive lesions found in CSM, and undue manipulation of the cervical spine when advanced stenosis is known to be present should be recognized as a possible cause of cervical spondylotic myelopathic-phrenic paresis.- - - - - - - - - - ranking = 94.093695281295keywords = paresis (Clic here for more details about this article) |
7/56. Dyspnoea exaggerated in the supine position and during exertion--diagnostic challenge.The case of dyspnoea, exaggerated when in the supine position and during exertion, as a result of severe weakness of the diaphragm is reported. The aim of the study was to present a rare case of idiopathic bilateral diaphragmatic paresis (BDP) and to describe all the diagnostic procedures necessary to perform differential diagnostics. In order to establish the final diagnosis, chest radiography, haemodynamic evaluation of the circulatory system, ultrasonography, ultrasonocardiography, measurement of transdiaphragmatic pressures, scintiscanning of the lungs, spirometry, analysis of arterial blood gases, computed tomography of the thorax and external stimulation of the phrenic nerve were performed. The measurement of transdiaphragmatic pressure was crucial to establish and confirm the diagnosis of BDP, as only a small difference in gastric and oesophageal pressures during tidal breathing and inspiratory efforts was recorded. As no cause of diaphragmatic paresis was found, the case was classified as idiopathic. The final diagnosis of non-trauma related bilateral diaphragmatic weakness was generally delayed. In the case of the described patient, dyspnoea, the main symptom he was suffering from, was supposed to result from his congenital heart defect. We recommend that the suspicion of idiopathic diaphragmatic paresis should always be raised in patients suffering from respiratory failure of unknown origin. It is, however, necessary to perform extensive diagnostics to exclude the other causes of phrenic-diaphragmatic impairment. It's also necessary to consider all infections, injuries and surgical procedures within the thorax as possible causes of diaphragmatic paresis.- - - - - - - - - - ranking = 62.72913018753keywords = paresis (Clic here for more details about this article) |
8/56. Transient hemi-diaphragmatic paralysis following neck surgery: report of a case and review of the literature.Diaphragmatic paresis following trauma to the phrenic nerves is a rare complication after neck surgery. The resulting elevation of the ipsilateral hemi-diaphragm is diagnosed on post-operative chest radiography and may be confirmed by ultrasound or fluoroscopy. When unilateral, this may lead to respiratory, cardiac or gastrointestinal symptoms and atelectasis and pulmonary infiltrates on radiography. If nerve damage is bilateral a period of ventilation may be required.- - - - - - - - - - ranking = 15.682282546882keywords = paresis (Clic here for more details about this article) |
9/56. Congenital hypomyelination neuropathy in a newborn infant: unusual cause of diaphragmatic and vocal cord paralyses.We report a case of congenital hypomyelination neuropathy presenting at birth. The infant had generalized hypotonia and weakness. There was decreased respiratory effort along with a right phrenic nerve and left vocal cord paralyses. tongue fasciculations were present. Deep tendon reflexes were absent in the upper extremities and hypoactive (1 ) in the lower extremities. magnetic resonance imaging of the head revealed no intracranial abnormalities, including normal cerebral myelination. Nerve conduction study showed absence of motor and sensory action potentials in the hands when the nerves in the upper limbs were stimulated. A motor response could be elicited only in the proximal leg muscles. Needle electromyography study was normal in the proximal limb muscles, but showed active denervation in the distal muscles of the arm and leg. These findings were thought to be consistent with a length-dependent sensorimotor peripheral polyneuropathy of axonal type with greater denervation of the distal muscles. A biopsy of the quadriceps muscle showed mild variability in fiber diameter, but no group typing or group atrophy. The muscle fibers showed no intrinsic abnormalities. biopsy of the sural nerve showed scattered axons with very thin myelin sheaths. There was also a nearly complete loss of large diameter myelinated fibers. No onion bulb formations were noted. These findings were thought to be consistent with congenital hypomyelination neuropathy with a component of axonopathy. dna analysis for identification of previously characterized mutations in the genes MPZ, PMP22, and EGR2 was negative. Several attempts at extubation failed and the infant became increasingly ventilator-dependent with increasing episodes of desaturation and hypercapnea. He also developed increasing weakness and decreased movement of all extremities. He underwent surgery at 2 months of age for placement of a gastrostomy tube and a tracheostomy. He was discharged from the hospital on a ventilator at 6 months of age. The infant was 13 months old at the time of submission of this report. Although he appears cognitively normal, he remains profoundly hypotonic and is on a home ventilator. There was no evidence of progressive weakness. Congenital hypomyelination neuropathy is a rare form of neonatal neuropathy that should be considered in the differential diagnosis of a newborn with profound hypotonia and weakness. It appears to be a heterogeneous disorder with some of the cases being caused by specific genetic mutations.- - - - - - - - - - ranking = 2keywords = muscle (Clic here for more details about this article) |
10/56. Acute lead poisoning: Five cases resulting from self-injection of lead and opium.Five cases of acute lead poisoning resulted from the self-injection of lead and opium pills which were crushed, heated and suspended in water. Two of the five patients died of illnesses in which hepatic failure and reversible acute tubular necrosis were prominent features. One of these two had a severe neuropathy, with flaccid quadriplegia and respiratory paralysis. The other three patients had relatively minor symptoms but unequivocal biochemical evidence of lead toxicity. autopsy changes included hepatic degeneration with inclusion bodies, regenerating renal tubular epithelium and wasting of skeletal muscle. Hepatic lead content was extremely high in one case. chelation therapy in the other fatal case resulted in a fall in blood lead to within normal limits and a clinical improvement, which was terminated by massive haemorrhage from a ruptured innominate artery.- - - - - - - - - - ranking = 0.33333333333333keywords = muscle (Clic here for more details about this article) |
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