Adult Polyglucosan Body Disease Associated With Lewy Bodies and Tremor

Abstract

To our knowledge, this is the first report of adult PGBD associated with Lewy bodies and levodopa-responsive tremor. Although polyglucosan bodies were seen in substantia nigra, it is most likely that our patient had coexisting Parkinson disease.

Increased glucose concentration results in reduced proteasomal activity and the formation of glycogen positive aggresomal structures

“…Polyglucosan bodies are considered to be lesser branched and insoluble forms of glycogen and are associated with several neurological disorders [14]. Intriguingly, neurodegenerative disorders such as Alzheimer’s disease, Parkinson’s disease and amyotrophic lateral sclerosis, in which the proteasomal function is known to be compromised, also have polyglucosan bodies [52–54]. Although the molecular and cellular mechanisms resulting in the genesis of polyglucosan bodies are not fully understood, the observations reported in the present study suggest a functional link between proteasomal function and polyglucosan bodies, and also indicate these two pathological processes could be linked to neurodegenerative disorders.…”

Increased glucose concentration results in reduced proteasomal activity and the formation of glycogen positive aggresomal structures

“…Polyglucosan bodies are considered to be lesser branched and insoluble forms of glycogen and are associated with several neurological disorders [14]. Intriguingly, neurodegenerative disorders such as Alzheimer’s disease, Parkinson’s disease and amyotrophic lateral sclerosis, in which the proteasomal function is known to be compromised, also have polyglucosan bodies [52–54]. Although the molecular and cellular mechanisms resulting in the genesis of polyglucosan bodies are not fully understood, the observations reported in the present study suggest a functional link between proteasomal function and polyglucosan bodies, and also indicate these two pathological processes could be linked to neurodegenerative disorders.…”

Neuronal glycogen synthesis contributes to physiological aging

“…The accumulation of alpha-synuclein, an aggregate-prone protein involved in Parkinson’s disease, has also been observed in PGBs (Trivedi et al . 2003; Uchida et al ., 2003; Krim et al ., 2005). In our hands, PGBs containing alpha-synuclein were found mainly in the hippocampus of aged wild-type brains and more ubiquitously in malin KO brains (Figs 2A and S6B).…”

Atypical parkinsonism combining α‐synuclein inclusions and polyglucosan body disease

“…Less common features included cerebellar syndrome,8 amyotrophic lateral sclerosis,9 and frontal dementia 10. Four cases presented with parkinsonism 10–13. In the case of Rifai and colleagues, there were signal abnormalities in the putamen (T2 hypointensity) 11.…”

Increased glucose concentration results in reduced proteasomal activity and the formation of glycogen positive aggresomal structures

“…Polyglucosan bodies are considered to be lesser branched and insoluble forms of glycogen and are associated with several neurological disorders [14]. Intriguingly, neurodegenerative disorders such as Alzheimer’s disease, Parkinson’s disease and amyotrophic lateral sclerosis, in which the proteasomal function is known to be compromised, also have polyglucosan bodies [52–54]. Although the molecular and cellular mechanisms resulting in the genesis of polyglucosan bodies are not fully understood, the observations reported in the present study suggest a functional link between proteasomal function and polyglucosan bodies, and also indicate these two pathological processes could be linked to neurodegenerative disorders.…”

Neuronal glycogen synthesis contributes to physiological aging

“…The accumulation of alpha-synuclein, an aggregate-prone protein involved in Parkinson’s disease, has also been observed in PGBs (Trivedi et al . 2003; Uchida et al ., 2003; Krim et al ., 2005). In our hands, PGBs containing alpha-synuclein were found mainly in the hippocampus of aged wild-type brains and more ubiquitously in malin KO brains (Figs 2A and S6B).…”

Atypical parkinsonism combining α‐synuclein inclusions and polyglucosan body disease

“…Less common features included cerebellar syndrome,8 amyotrophic lateral sclerosis,9 and frontal dementia 10. Four cases presented with parkinsonism 10–13. In the case of Rifai and colleagues, there were signal abnormalities in the putamen (T2 hypointensity) 11.…”

Increased glucose concentration results in reduced proteasomal activity and the formation of glycogen positive aggresomal structures

“…Polyglucosan bodies are considered to be lesser branched and insoluble forms of glycogen and are associated with several neurological disorders [14]. Intriguingly, neurodegenerative disorders such as Alzheimer’s disease, Parkinson’s disease and amyotrophic lateral sclerosis, in which the proteasomal function is known to be compromised, also have polyglucosan bodies [52–54]. Although the molecular and cellular mechanisms resulting in the genesis of polyglucosan bodies are not fully understood, the observations reported in the present study suggest a functional link between proteasomal function and polyglucosan bodies, and also indicate these two pathological processes could be linked to neurodegenerative disorders.…”

Neuronal glycogen synthesis contributes to physiological aging

“…The accumulation of alpha-synuclein, an aggregate-prone protein involved in Parkinson’s disease, has also been observed in PGBs (Trivedi et al . 2003; Uchida et al ., 2003; Krim et al ., 2005). In our hands, PGBs containing alpha-synuclein were found mainly in the hippocampus of aged wild-type brains and more ubiquitously in malin KO brains (Figs 2A and S6B).…”

Atypical parkinsonism combining α‐synuclein inclusions and polyglucosan body disease

“…Less common features included cerebellar syndrome,8 amyotrophic lateral sclerosis,9 and frontal dementia 10. Four cases presented with parkinsonism 10–13. In the case of Rifai and colleagues, there were signal abnormalities in the putamen (T2 hypointensity) 11.…”