No abstract

The objective of this study was to determine the prevalence of blepharospasm (BSP), with and without apraxia of eyelid opening (AEO), in patients with parkinsonism, cervical dystonia (CD), and essential tremor (ET). BSP, with or without AEO, is associated with parkinsonism. There have been several reports of BSP in other dystonic conditions, but few looked at the incidence of BSP in ET patients. This study included 659 patients of which 357 had parkinsonism (276 idiopathic Parkinson’s disease (IPD) and 81 atypical parkinsonism (57 progressive supranuclear palsy; 11 multiple system atrophy 13 corticobasal degeneration)), 274 had ET, 22 had CD, and 6 had spinocerebellar ataxia. Our results indicate that BSP (with or without AEO) was more prevalent in atypical parkinsonism (6 out of 81, 7.41%) than IPD (9 out of 276, 3.26%). The study also followed 10 (of the 28) patients with BSP to screen for the development of other movement disorders – of these, 2 developed Parkinson’s disease. We conclude then that BSP is common in parkinsonism and that BSP is more prevalent in atypical parkinsonism. We also conclude that BSP is not a common feature in ET patients (0 out of 274 patients reported BSP symptoms).

Background: Parkinson's disease (PD) is commonly characterizedby its motor symptoms such as resting tremor, rigidity, bradykinesia and postural instability; however, some of the most debilitating symptoms of this disease are non-motor ones such as dementia and sialorrhea (drooling). Drooling is caused by impaired swallowing and it can have a significant impact on the quality of life. However, it is still unclear whether cognitive dysfunction could exacerbate drooling. We wanted to examine if any relationship existed between drooling and dementia in PD patients. Identifying the correlation will aid physicians to screen and initiate early management of drooling in the course of PD. This can possibly lead to improvements in the quality of life in these patients. Methods: In this retrospective study, we investigated the prevalence of drooling in 314 PD patients and further compared the difference in the prevalence of drooling in patients with dementia and without dementia. In addition, we studied the impact of gender on drooling in this patient population. Results: Our results show that a significant correlation exists between drooling and dementia in our sample of PD patients. Furthermore, in males, the correlation between the prevalence of drooling and dementia was found to be clinically significant as compared to the female population. Conclusion: Our findings suggest that drooling is a major concern in the course of PD and should therefore be addressed early and more aggressively in patients with dementia.

Locked-in syndrome (LIS) is a rare neurologic disorder rendering an individual quadriplegic and anarthric with preserved self-awareness and normal if not near-normal cognition. A lesion to the ventral pons causes the classic form of LIS, and patients can typically interact with their environment with eye/eyelid movements. LIS patients may live for years with preserved quality of life (QoL) and cognitive function, but with severe disability. However, medical providers and family often underestimate the patient's QoL, and choose less aggressive care. Prompt assessment of decisionality in LIS patients is challenging, but it must be done to allow these patients to participate in their care. We present the case of a 54-year-old man with LIS. The medical team recommended comfort measures, but the family advocated involving the patient in goals of care discussions. The patient was determined to be decisional during the acute hospitalization, and he elected for life-prolonging care. This case emphasizes the importance of unbiased shared decision making, but also the importance of utilizing a practical framework to assess the decision-making capacity in these patients. We provide a suggested approach to determining decision-making capacity in similar cases or conditions.