At the completion of our behavioural analysis, the progression of demyelination and remyelination was quantified using immunohistochemistry for MOG, which correlates well with ultrastructural measurements of remyelination (Lindner et al., 2008).
A comparison of prion disease progression by behavioural, biochemical and anatomical methods surprisingly revealed no difference in disease progression in mice with or without α-synuclein (Asuni et al., 2010).
These models of HD are characterized by early onset and fast progression of behavioural deficits (later also by motor dysfunction) and presence of intranuclear polyglutamine (polyQ) inclusions (4 weeks postnatally), but without the evident reduction of neurons even in aged mice [ 16, 19].
There was no evidence of an association between disease progression and any behavioural factors (including ART knowledge, social capital, social support, relationship characteristics, and internalized stigma).
Several independent studies have shown that active and passive immunisation approaches were effective in reducing the burden of neurofibrillary tangles (NFTs) in the brain, slowing the progression of the behavioural phenotype or delaying the onset of motor function decline and weight loss in mouse models of tau tangle pathology [ 13- 19].
With the progression of the disease, behavioural symptoms increase in frequency and severity [ 16], which poses an increased burden on family and caregivers [ 17, 18].
Even within the high-grade group of tumours, behavioural diversity in terms of disease progression, survival and response to therapy is high.
The temporal progression of both electrical and behavioural seizures from 2 to 28 weeks post-injury have previously been described (D'Ambrosio et al., 2004, 2005).
Measuring HRQoL is as important as measuring disease severity, progression, symptom response, cognition and behavioural disturbance when assessing the impact of disease and determining proper intervention in the treatment and management of dementia.
Although it is accepted that CLN6 mice are phenotypically normal until 8 months of age, this study represents the first rigorous behavioural analysis of CLN6 mice throughout disease progression.
