Abstract
The name Sträußler’s disease (StD) refers to the neuropathological substrate of the peculiar familial disease which originally had been described by Gerstmann et al. (5) in a family from Lower Austria. Later on further cases from the same sip, were reported (3, 9). Remarkable characteristics of the disease are:
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Genealogy: The family tree shows incidence of the disease in each of 5 generations. No defined or typical mode of heredity can be asserted.
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Clinical syndrome: The first symptom starting usually in the 5th decade is spinal ataxia followed by cerebellar signs and finally organic dementia. Duration of the disease is 2–10 years.
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Neuropathology: Gross examination of the CNS shows diffuse cortical atrophy, atrophy and sclerosis of the cerebellar vermis and slenderness of the spinal cord.
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© 1981 Springer-Verlag
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Seitelberger, F. (1981). Sträußler’s Disease. In: Jellinger, K., Gullotta, F., Mossakowski, M. (eds) Experimental and Clinical Neuropathology. Acta Neuropathologica Supplementum, vol 7. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-81553-9_97
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DOI: https://doi.org/10.1007/978-3-642-81553-9_97
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-540-10449-0
Online ISBN: 978-3-642-81553-9
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