Relevance of sleep quality on caregiver burden in Parkinson's disease.

Parkinson's disease (PD) is a neurodegenerative disorder which affects the quality of life of patient and their family. Sleep disorders appear in 80-90% of PD patients and have a great impact on the PD well-being. We examined the relationship of patients' sleep quality and depression on burden, mood, quality of life, and quality of sleep of their caregivers. A multicenter, regional (Veneto), observational, cross-sectional study that included 55 patient-caregiver pairs was conducted. Patients were assessed using Parkinson's Disease Sleep Scale (PDSS) and Epworth Sleepiness Scale (ESS) for sleep disorders, Beck Depression Inventory (BDI) as a measure of depression, and Parkinson's Disease Questionnaire (PDQ-39) as a measure of quality of life. Caregivers were evaluated by the Caregiver Burden Inventory (CBI) a measure of burden, BDI, SF-36 Health Survey as measures of HRQoL, and Medical Outcomes Study-Sleep Scale (MOS-SS) for quality of sleep. CBI, HRQoL, MOS-SS, and BDI scores displayed no association with patients' age, cognition (Mini Mental State Examination (MMSE) and Frontal Assessment Battery (FAB)), disease duration, and Hoehn and Yahr (H&Y), and UPDRS III scales whereas were significantly correlated with patients' quality of sleep, depression, and quality life. CBI and HRQoL were also associated respectively with patients' ESS and L-dopa daily dose. This study underscores the presence of a significant relationship between patient and caregiver quality of life. Interestingly, sleep quality and depression rather than motor disability best predicted caregivers' well-being.

Microglial and Neuronal TDP-43 Pathology in Anti-IgLON5-Related Tauopathy.

A novel neuronal tauopathy, mainly confined to hypothalamus and brainstem tegmentum, has recently been reported in patients with autoantibodies to the neuronal cell-adhesion molecule IgLON5. We describe a patient with anti-IgLON5 syndrome, who presented with dysautonomia and sleep disorder, followed by subacute dementia. Postmortem brain examination disclosed neuronal tau pathology prevailing in the hippocampus, amygdala, and locus coeruleus, in addition to microglial/neuronal TDP-43 pathology, with overexpression of aberrantly phosphorylated forms and neurotoxic truncated fragments, in basal ganglia, nucleus basalis, thalamus, and midbrain. These findings suggest that neurodegeneration in anti-IgLON5 syndrome might also occur via a microglia-triggered non-cell autonomous pathway.

Accuracy of diagnostic criteria for sporadic creutzfeldt-jakob disease among rapidly progressive dementia.

Rapidly progressive dementia (RPD) is a rare presentation of different neurological disorders characterized by cognitive impairment leading to loss of functional independence within 24 months or less. The increasing recognition of treatable non-prion causes of RPD has made the differential diagnosis with sporadic Creutzfeldt-Jakob disease (sCJD) of crucial importance. We therefore assessed the frequency of different etiologies of RPD and evaluated the accuracy of newly proposed diagnostic criteria for sCJD. Clinical records of patients with RPD referred to Memory Clinic between 2007 and 2012 were retrospectively analyzed. The accuracy of diagnostic criteria for sCJD was evaluated by: a) MRI images in DWI and FLAIR sequences; and (b) CSF 14-3-3 protein. In addition, CSF total tau protein level was also assessed. Final diagnosis was obtained after a 1-year follow-up or after autopsy. Among 37 patients with RPD, the most frequent causes were non-prion diseases, either untreatable (38%) or potentially treatable (32%), thus leaving sCJD as a less frequent cause (30%). DWI images had a sensitivity of 73% and specificity of 96%, while FLAIR yielded a very low sensitivity (40%). CSF 14-3-3 protein had a sensitivity of 100%, but a very low specificity (43%). The strongest independent predictor of sCJD diagnosis was the CSF tau level (p = 0.002) (91% sensitivity, 83% specificity). Treatable causes of RPD are as frequent as sCJD and a rapid differential diagnosis is mandatory. We suggest that DWI images and CSF analysis combining 14-3-3 and total tau protein determination hold the best informative diagnostic values.