Fortschritte der Neurologie-Psychiatrie
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Fortschr Neurol Psychiatr · Jan 2015
Review[Myotonic dystrophies: clinical presentation, pathogenesis, diagnostics and therapy].
The autosomal-dominant myotonic dystrophies dystrophia myotonica type-1 (DM1, Curschmann-Steinert disease) and dystrophia myotonica type-2 (DM2, proximal myotonic myopathy (PROMM)), are, contrary to the non-dystrophic myotonias, progressive multisystem disorders. DM1 and DM2 are the most frequent of the muscular dystrophies. In both diseases the skeletal muscle is the most severely affected organ (weakness, wasting, myotonia, myalgia). ⋯ The higher the repeat expansion the more severe the DM1 phenotype. In DM2 severity of symptoms and age at onset do not correlate with the expansion size. Contrary to DM2, there is a congenital form and anticipation in DM1.
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Delirium and dementia as organically caused mental disorders exhibit similarities in the clinical and neurological sense and often occur together. The existence of one appears to increase the risk for the development of the other. Although delirium is a very common disorder especially among the elderly, it is often not recognized. ⋯ Prevention appears to be extremely important for which the treating personnel require a good knowledge of risk factors and their management. The occurrence of delirium among cases of alpha-synucleinopathies represents a special case. Both international and German guidelines on the management of delirium are available.