Progressive multifocal leukoencephalopathy without overt immunosuppression.

Progressive multifocal leukoencephalopathy (PML) is a central nervous system disease caused by the human polyomavirus 2 that usually occurs in a setting of immunodeficiency. PML without overt immunosuppression is considered a rare occurrence but has been described in multiple previous case reports and series. Its prevalence, overall frequency, and prognosis are largely unknown. This is a single-center retrospective review of all University of Florida cases with the ICD10 PML diagnosis code (A81.2). PML without overt immunosuppression was defined as absence of human immunodeficiency virus (HIV) infection, hematological malignancy, immunomodulatory/-suppressive medications, autoimmune conditions with a propensity for PML (sarcoidosis, systemic lupus erythematosus). Cases that did not fulfill criteria for clinically or histologically definite PML were excluded. Of 52 patients with the ICD10 code A 81.2, 17 fulfilled definite diagnostic criteria for PML. Overt immunosuppression was identified in 15/17 (88.2%) cases (10/17 (58.8%): human immunodeficiency virus; 5/17 (29.4%): immunomodulatory/-suppressive medication). Two/seventeen (11.8%) cases were consistent with PML without overt immunosuppression. Possible contributing factors were a preceding dog bite and mild hypogammaglobulinemia M (39 mg/dL) in case 1 and significant alcohol use without evidence for liver disease in case 2. Both cases were fatal within 6 (case 1) and 2 (case 2) months. The results suggest that PML without overt immunosuppression may be more common than previously described. Therefore, PML should be considered even in the absence of overt immunosuppression if clinical and radiographic findings are suggestive of the diagnosis.

Applying the Care and Communication Bundle to Promote Palliative Care in a Neuro-Intensive Care Unit: Why and How.

Background: Delivery of palliative care in neurointensive care units (neuro-ICUs) can be inconsistent, often due to absence of formal care triggers. The Care and Communication Bundle (CCB) of Quality Indicators provides a standardized process to deliver effective palliative care services in ICUs, but application of these indicators in this setting has not yet been systemically assessed. Objectives: To evaluate the fit of a CCB in the neuro-ICU through a novel scoring system and identify barriers to adherence. Design: CCB standards for a neuro-ICU were delineated. Assessment of documented indicators and barriers was conducted through electronic medical record retrospective review. Setting/Subjects: A 30-bed neuro-ICU in a large Academic Medical Center in the Southeastern United States. Chart reviews were conducted for 133 critically ill neurology and neurosurgery patients who expired between November 2018 and January 2020. Results: Results demonstrate moderate adherence to CCB standards, including excellent consistency in establishment of patient-centered communication and referral to supportive services (e.g., social work, spiritual support). Identified areas for improvement include documentation of patient and family involvement in care process (i.e., advance directive completion, interdisciplinary team meetings). Conclusions: Application of the CCB in the neuro-ICU is useful for examining adherence to time-based triggers of palliative care standards. The novel scoring system offers opportunities to motivate improvement and reduce variation in palliative care integration.

Visual hallucinations, thalamocortical physiology and Lewy body disease: A review.

One of the core diagnostic criteria for Dementia with Lewy Bodies (DLB) is the presence of visual hallucinations. The presence of hallucinations, along with fluctuations in the level of arousal and sleep disturbance, point to potential pathological mechanisms at the level of the thalamus. However, the potential role of thalamic dysfunction in DLB, particularly as it relates to the presence of formed visual hallucinations is not known. Here, we review the literature on the pathophysiology of DLB with respect to modern theories of thalamocortical function and attempt to derive an understanding of how such hallucinations arise. Based on the available literature, we propose that combined thalamic-thalamic reticular nucleus and thalamocortical pathology may explain the phenomenology of visual hallucinations in DLB. In particular, diminished α7 cholinergic activity in the thalamic reticular nucleus may critically disinhibit thalamocortical activity. Further, concentrated pathological changes within the posterior regions of the thalamus may explain the predilection for the hallucinations to be visual in nature.

Auditory system dysfunction in Alzheimer disease and its prodromal states: A review.

Recent findings suggest that both peripheral and central auditory system dysfunction occur in the prodromal stages of Alzheimer Disease (AD), and therefore may represent early indicators of the disease. In addition, loss of auditory function itself leads to communication difficulties, social isolation and poor quality of life for both patients with AD and their caregivers. Developing a greater understanding of auditory dysfunction in early AD may shed light on the mechanisms of disease progression and carry diagnostic and therapeutic importance. Herein, we review the literature on hearing abilities in AD and its prodromal stages investigated through methods such as pure-tone audiometry, dichotic listening tasks, and evoked response potentials. We propose that screening for peripheral and central auditory dysfunction in at-risk populations is a low-cost and effective means to identify early AD pathology and provides an entry point for therapeutic interventions that enhance the quality of life of AD patients.