Polyradiculopathy and/or myelopathy as verified by electrophysiological examination within 4 weeks from symptom onset were indicative of a more severe disease course and a greater likelihood of moderate to serious sequelae even after long-term rehabilitation. Older age at symptom onset seems to be associated with a less favourable outcome. Because of frequent long-term hospitalization with immobilization and invasive ventilation, secondary complications, such as ventilation associated pulmonary infections and decubiti, must be avoided.
This report could not conclude that the occurrence of cardiomyopathy is rare logically. Although an underlying pathophysiological cause was not obvious, it is proposed that in all MND patients a routine cardiological evaluation should be performed.
Since valosin-containing protein mutations were reported as a cause of hereditary inclusion body myositis associated with Paget's disease of the bone and frontotemporal dementia, many new mutations have been described in the last decade. We report on a 46-year-old German male with a progressive tetraparesis and autosomal dominant inheritance pattern. Echocardiography revealed a beginning dilated cardiomyopathy and laboratory analyses showed increased alkaline phosphatase. Decreased verbal memory and an impairment of concept building were observed on neuropsychological examination. Muscle biopsy demonstrated a myopathic pattern, rimmed vacuoles, CD8+ T-cell infiltrates and positive MHC1-muscle fibres. We found a heterozygote mutation in exon 5 of the valosin-containing protein gene (c.464G > T p.Arg155Leu), which until now has been described only in an Australian family. We describe here the first German case with the above-mentioned mutation causing inclusion-body myositis associated with Paget's disease of the bone and fronto-temporal dementia. Here, we recommend regular controls of cardiac and respiratory functions.
The diagnosis of intensive care unit acquired weakness (ICUAW) in the setting of neurological rehabilitation is steadily increasing. This is due to the fact that the intensive care of patients with sepsis or after cardiac or abdominal surgery is improving. A longer duration of respiratory weaning and comorbidities frequently complicate rehabilitation. Clinically, patients present with a flaccid (tetra) paresis and electrophysiological studies have shown axonal damage. Besides involvement of peripheral nerves, muscle can also be affected (critical illness myopathy) leading to ICUAW with inconstant myopathic damage patterns found by electrophysiological testing. Mixed forms can also be found. A specific therapy for ICUAW is not available. Early mobilization to be initiated on the intensive care unit and commencing neurological rehabilitation improve the outcome of ICUAW. This review highlights the current literature regarding the etiology and diagnosis of ICUAW. Furthermore, studies about rehabilitation and outcome of ICUAW are discussed.
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