Muscular and cerebral involvement in a familiar mitochondrial disease.
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Biomedical subjects
Publications and source records attributed to M Lo Monaco.
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The clinical features and course of amyotrophic lateral sclerosis are discussed. The data on a series of 116 patients are compared with those of the literature. The following points emerge: 1) when the disease starts before the age of 50, the prognosis is often less poor than usual; 2) the forms with spinal, and especially cervical, onset appear to be less rapid than bulbar forms; 3) in 20% of the patients survival is over 5 years. There may be some unknown factor that increases the resistance of some subject to the disease.
Median, ulnar, radial and common peroneal nerve somatosensory evoked potentials (SEPs) were studied in 17 patients suffering from cervical spondylotic myelopathy. Median, ulnar and common peroneal nerve SEPs were abnormal in 41%, 71% and 100% of cases respectively. Abnormalities of the scalp far-field P14 evoked by upper limb stimulation correlated with joint and touch sensation impairment, but not with radiological findings. Therefore, P14 may be a reliable marker of dorsal column impairment in cervical spondylotic myelopathy. The analysis of the cervical N13 response, which was recorded using a cephalic reference electrode, did not give any further information. Common peroneal nerve SEP abnormalities were found in all our patients, but they were obviously of no value in identifying the cervical spine as the site of lesion.
The successful management of 2 cases of rare myopathies, who underwent hemithyroidectomy, is here reported. Anaesthesia was induced with TPS and fentanyl, and maintained with isoflurane. Neuromuscular blockade was achieved by atracurium; neuromuscular monitoring by Neurostar-Medeleck was performed. This intraoperative monitoring allowed a quick recovery without complications.