Timothy C. Hain, MD Most recent update: August 29, 2009
DLB was first described in 1996, not very long ago. Lewy bodies are accumulations of alpha-synuclein. In this disease, accumulation of Lewy bodies is associated with a slowly progressive neurological deterioration.
DLB was intitially reported as accounting for about 15-25% of all dementia. There may be an incidence of DLB of about 0.1% in the general population.
Damage is thought to occur due to accumulation of Lewy bodies in the nervous system. A 6-stage system has been suggested beginning with lesions in the medulla and olfactory cortex, and with progression to the cortex. Lewy bodys may also be found in sympathetic and intramural ganglia (i.e. outside the CNS).
DLB is characterized by dementia associated with any two of the following three core features:
Autonomic failure may also accompany DLB ( Akaogi et al, 2009). Olfactory disturbances and autonomic failure may precede the motor symptoms and onset of dementia. Sweating disturbances and orthostatic hypotension are common.
Akaogi, Y., M. Asahina, et al. (2009). "Sudomotor, skin vasomotor, and cardiovascular reflexes in 3 clinical forms of Lewy body disease." Neurology 73(1): 59-65.
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