Death Anxiety in Huntington Disease: Longitudinal Heath-Related Quality-of-Life Outcomes.

Objective: Death anxiety, represented by the HDQLIFE™ Concern with Death and Dying (CwDD) patient-reported outcome (PRO) questionnaire, captures a person's worry about the death and dying process. Previous work suggests that death anxiety remains an unremitting burden throughout all stages of Huntington disease (HD). Although palliative interventions have lessened death anxiety among people with advanced cancer, none has yet to undergo testing in the HD population. An account of how death anxiety is associated with longitudinal changes to aspects of health-related quality of life (HRQoL) would help optimize neuropalliative interventions for people with HD. Methods: HDQLIFE collected PROs concerning physical, mental, social, and cognitive HRQoL domains and clinician-rated assessments from people with HD at baseline and 12 and 24 months. Linear mixed-effects models were created to determine how baseline death anxiety was associated with follow-up changes in HRQoL PROs after controlling for baseline death anxiety and other disease and sociodemographic covariates. Results: Higher baseline HDQLIFE CwDD is associated with 12- and 24-month declines in HDQLIFE Speech Difficulties, neurology quality of life (NeuroQoL) Depression, Suicidality, HDQLIFE Meaning and Purpose, and NeuroQoL Positive Affect and Well-being. Interpretation: Death anxiety may be a risk factor for worsening mental health and speech difficulty. A further prospective study is required to evaluate whether interventions on death anxiety or mental health generally can reduce declines in HRQoL for people with HD over time.

Disparities in Palliative Care Utilization Among Hospitalized People With Huntington Disease: A National Cross-Sectional Study.

BACKGROUND: People with Huntington's disease (HD) often become institutionalized and more frequently die away from the home setting. The reasons behind differences in end-of-life care are poorly understood. Less than 5% of people with HD report utilization of palliative care (PC) or hospice services, regardless of the lack of curative therapies for this neurodegenerative disease. It is unknown what factors are associated with in-patient specialty PC consultation in this population and how PC might be related to discharge disposition. OBJECTIVES: To determine what HD-specific (e.g., psychosis) and serious illness-specific factors (e.g., resuscitation preferences) are associated with PC encounters in people with HD and explore how PC encounters are associated with discharge disposition. DESIGN: We analyzed factors associated with PC consultation for people with HD using discharge data from the National Inpatient Sample and the Nationwide Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality. An anonymized, cross-sectional, and stratified sample of 20% of United States hospitalizations from 2007 through 2014 were included using ICD-9 codes. RESULTS: 8521 patients with HD were admitted to the hospital. Of those, 321 (3.8%) received specialty PC. Payer type, (specifically private insurer or other insurer as compared to Medicare), income, (specifically the top quartile as compared to the bottom quartile), mortality risk, D.N.R., aspiration pneumonia, and depression were significantly associated with PC in a multivariate model. Among those who received PC, the odds ratio (OR) of discharge to a facility was 0.43 (95% CI, 0.32-0.58), whereas the OR of discharge to home with services was 2.25 (95% CI 1.57-3.23), even after adjusting for possible confounders. CONCLUSIONS: Among patients with HD, economic factors, depression, and serious illness-specific factors were associated with PC, and PC was associated with discharge disposition. These findings have implications for the adaptation of inpatient PC models to meet the needs of persons with HD.

Meaning and purpose in Huntington's disease: a longitudinal study of its impact on quality of life.

OBJECTIVE: Previous work in Huntington's disease (HD) has shown that a sense of meaning and purpose (M&P) is positively associated with positive affect and well-being (PAW); however, it was unknown whether HD-validated patient-reported outcomes (PROs) influence this association and how M&P impacts PROs in the future. Our study was designed to examine if HD-validated PROs moderate the relationship between M&P and PAW and to evaluate if baseline M&P predicts 12- and 24-month changes in HD-validated PROs. METHODS: This was a longitudinal, multicenter study to develop several PROs (e.g., specific for the physical, emotional, cognitive, and social domains) for people with HD (HDQLIFE). The sample consisted of 322 people with HD (n = 50 prodromal, n = 171 early-stage manifest, and n = 101 late-stage manifest HD). A single, multivariate linear mixed-effects model was performed with PAW as the outcome predicted by main effects for M&P and several moderators (i.e., an HD-validated PRO) and interactions between M&P and a given PRO. Linear-mixed models were also used to assess if baseline M&P predicted HD-validated PROs at 12 and 24 months. RESULTS: Higher M&P was positively associated with higher PAW regardless of the magnitude of symptom burden, as represented by HD-validated PROs, and independent of disease stage. In our primary analysis, baseline M&P predicted increased PAW and decreased depression, anxiety, anger, emotional/behavioral disruptions, and cognitive decline at 12 and 24 months across all disease stages. INTERPRETATION: These findings parallel those seen in the oncology population and have implications for adapting and developing psychotherapeutic and palliative HD interventions.

The case of a 37-year-old male with trouble ambulating and incontinence.

A 37 year-old previously healthy man from Jamaica presented with 2-3 months of progressive trouble ambulating and incontinence. By 1 month prior to arrival he was wheelchair bound and unable to ambulate even with assistance. He started to wear a diaper for bladder and bowel incontinence. He also complained of painless numbness in his legs over the same period of time. His exam is notable for marked weakness and spasticity in his legs, with hyper-reflexia and clonus. He has a sensory level at the level of the umbilicus. An MRI shows a longitudinally extensive T2 signal change throughout the thoracic cord. His cerebrospinal fluid is mildly inflammatory. His HTLV-1 antibody test is reactive.

Paraneoplastic Peripheral Nervous System Manifestations of Renal Cell Carcinoma: A Case Report and Review of the Literature.

Neurologic symptoms secondary to a paraneoplastic syndrome may be the presenting manifestation of a previously undiagnosed cancer, and alertness to these syndromes may provide an opportunity for early detection and treatment of a cancer. Paraneoplastic weakness is a rare manifestation of renal cell carcinoma and may present with variable electrophysiological features. We present a case of a patient with progressive weakness, sensory changes, and urinary retention, with electrophysiological features suggestive of a complex peripheral nervous system syndrome. Ultimately, a renal cell mass was detected and resected, resulting in significant clinical improvement. We review the literature, cataloging the known neurologic syndromes and antibodies associated with renal cell carcinoma. This case highlights that paraneoplastic neurological disorders associated with RCC can take on many features and provides a resource to practitioners for early detection of a neurologic paraneoplastic syndrome arising from renal cell carcinoma.

Alternative treatment of restless legs syndrome: an overview of the evidence for mind-body interventions, lifestyle interventions, and neutraceuticals.

Conventional pharmacologic treatment of restless legs syndrome (RLS) may be limited in some people. Up to 65% of patients with RLS regularly use alternative practices for symptom relief. We reviewed the current clinical evidence, and we proposed physiologic basis for various alternative practices for RLS including mind-body interventions (conventional exercise, yoga, and acupuncture), non-pharmacologic lifestyle interventions (pneumatic compression devices [PCDs], light therapy, and cognitive-behavioral therapy [CBT]), and neutraceuticals (vitamins, valerian, and Chinese herbs). Based on the available evidence, regular physical activity should be recommended for the treatment of RLS symptoms. Oral iron supplementation should be considered for people with RLS who have low ferritin levels, although criteria to identify probable responders, and optimal formulations and durations of treatment are needed. Supplementation for low levels of vitamins E, C, and D could be considered, although evidence specifically in RLS is limited, and it is unclear if levels should routinely be checked in patients with RLS. Insufficient evidence exists for yoga, acupuncture, PCDs, near-infrared light therapy, CBT, valerian, or Chinese herbs, but preliminary studies on each of these suggest that high-quality randomized controlled trials may be warranted to support and verify the data presented.

Reversible palinacousis from intracranial metastases.

Palinacousis is an auditory illusion consisting of perseveration or echoing of an external auditory stimulus after it has ceased. This rare clinical symptom has been reported in ictal (seizure), postictal, and nonictal states, and causative lesions have been most consistently found in or near the temporal lobes. It is distinct from the auditory hallucinations seen in psychiatric illness. We report the case of a 61-year-old man who experienced several days of palinacousis while undergoing treatment for newly diagnosed metastatic lung adenocarcinoma. Palinacousis was presumed to be triggered by intracranial metastases near the auditory cortex. An electroencephalogram showed bilateral theta slowing over the left greater than right temporal lobes without epileptiform activity. Palinacousis remitted with corticosteroid and whole brain radiation therapy.

Diffusion weighted imaging in heroin-associated spongiform leukoencephalopathy.

BACKGROUND: Heroin-associated spongiform leukoencephalopathy (HASL) is a rare complication of heroin abuse. We report a case that highlights the increased sensitivity of diffusion weighted imaging (DWI) compared with T2-weighted imaging in the acute setting of HASL. METHODS: Single case report. RESULTS: A 36-year-old male with a history of heroin abuse (snorting) presented with a 3-day history of lethargy. MRI brain revealed restricted diffusion involving the globus pallidum and cerebral cortex bilaterally that was not seen on fluid-attenuated inversion recovery (FLAIR) images. The patient was diagnosed with acute HASL. Repeated MRI FLAIR at 3 months confirmed the development of atrophy and T2 hyperintensity in the subcortical white matter, consistent with leukoencephalopathy. Neurological exam at 3-month follow-up was nonfocal. CONCLUSIONS: Restricted diffusion, which likely corresponds to electron microscopic findings of fluid entrapment between the myelin lamellae, may be detectable earlier than changes on FLAIR. Clinicians should be aware of the neuroimaging findings of HASL and the increased sensitivity of MRI DWI over T2-weighted images in detecting HASL acutely.