Infantile multiple system atrophy with cytoplasmic and intranuclear glioneuronal inclusions

Neuronal intranuclear inclusion disease and juvenile Parkinsonism

“…26,37 The inclusions in the present case were immunoreactive for ubiquitin, a protein that is thought to play an important part in the breakdown and disposal of abnormal cellular proteins. 39 Similar findings have previously been reported for neuronal inclusions seen in NIID 27,32,36 and in a number of neurodegenerative disorders, including the polyglutamine expansion disorders, PD, and Alzheimer's disease, suggesting abnormal protein aggregates are important components of these inclusions. 39 In summary, this case with levodopa-responsive JP complicated by motor fluctuations and dyskinesias was associated with widespread intraneuronal inclusions consistent with NIID.…”