Progressive myoclonus ataxia: Time for a new definition?

Background: The clinical demarcation of the syndrome progressive myoclonus ataxia is unclear, leading to a lack of recognition and difficult differentiation from other neurological syndromes. Objectives: The objective of this study was to apply a refined definition of progressive myoclonus ataxia and describe the clinical characteristics in patients with progressive myoclonus ataxia and with isolated cortical myoclonus. Methods: A retro‐ and prospective analysis was performed in our tertiary referral center between 1994 and 2014. Inclusion criteria for progressive myoclonus ataxia patients were the presence of myoclonus and ataxia with or without infrequent (all types, treatment responsive) epileptic seizures. Inclusion criteria for isolated cortical myoclonus was the presence of isolated cortical myoclonus. Clinical and electrophysiological characteristics data were systematically scored. Results: A total of 14 progressive myoclonus ataxia patients (males, 7; females, 7), median age 14.5 years, and 8 isolated cortical myoclonus patients (males, 2; females, 6), median age 23.5 years, were identified. In 93% of the progressive myoclonus ataxia patients, ataxia started first (median 2 years) followed by myoclonus (4 years) and finally infrequent epilepsy (9.3 years), with a progressive course in 93%. In 64% of the progressive myoclonus ataxia patients, a genetic underlying etiology was identified, including 3 not earlier reported causative progressive myoclonus ataxia genes. In isolated cortical myoclonus patients, myoclonus started at (median) 12 years with progression over time in 63% and a single epileptic seizure in 1 patient. No genetic causes were identified. Conclusion: Using a refined definition, we could create a rather homogenous progressive myoclonus ataxia group. Patients with isolated cortical myoclonus have a different course and do not appear to evolve in progressive myoclonus ataxia. The refined progressive myoclonus ataxia definition is a successful first step toward creating a separate syndrome for both clinical practice and future genetic research. © 2018 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.     

Comparative outcomes of bladder cancer

OBJECTIVE: To compare the survival of women and men with transitional cell bladder cancer. METHODS: We used the Surveillance Epidemiology and End Results database to identify patients aged 35 years or older diagnosed with bladder cancer between 1991 and 2001 actively followed up. We excluded cases diagnosed by autopsy or death certificates and those of unknown race. We used Cox proportional hazard regression to analyze survival in patients with advanced disease. RESULTS: Of the 31,009 patients meeting eligibility criteria, 26.7% were women. Median age at diagnosis for women and men was 72 and 70 years, respectively. Regional disease was diagnosed in 20.3% of white women and 35.5% of African-American women, compared with only 17.6% of white men and 25.9% of African-American men (P < .001). Increased age, African-American race, and being female significantly increased the hazard of death (hazard ratio [HR] 1.037, 95% confidence interval [CI] 1,034-1.041; HR 1.402, 95% CI 1.187-1.656; and HR 1.842, 95% CI 1.158-2.931). CONCLUSION: Women with bladder cancer, particularly African-Americans, have shorter survival. This is partially explained by higher risk of diagnosis with poorly differentiated tumors, advanced stage, and advanced age. Women should be targeted for timely diagnosis. LEVEL OF EVIDENCE: II-2.     

Beta-papillomavirus infection and skin cancer

The development of highly sensitive PCR techniques and multiplex bead-based Luminex platforms has accelerated the search for a specific role of human papilloma viruses in the development of squamous cell carcinoma. Human papillomaviruses are most likely indirectly involved in this process by facilitating UV-related carcinogenesis via preventing UV-induced apoptosis or impairing DNA repair, but other mechanisms are also possible.     

Clinical management of salivary gland hypofunction and xerostomia in head-and-neck cancer patients: successes and barriers

The most significant long-term complication of radiotherapy in the head-and-neck region is hyposalivation and its related complaints, particularily xerostomia. This review addresses the pathophysiology underlying irradiation damage to salivary gland tissue, the consequences of radiation injury, and issues contributing to the clinical management of salivary gland hypofunction and xerostomia. These include ways to (1) prevent or minimize radiation injury of salivary gland tissue, (2) manage radiation-induced hyposalivation and xerostomia, and (3) restore the function of salivary gland tissue damaged by radiotherapy.     

Adherence to national guidelines for antiemesis prophylaxis in patients undergoing chemotherapy for lung cancer

BACKGROUND:

Nausea and vomiting (N/V) during chemotherapy can have profound clinical and economic consequences. Effective antiemetic agents are available for prophylaxis, but barriers may prevent their use. For this population‐based study, the authors assessed the rates of antiemetic prophylaxis use, and predictors of such use, among patients who were receiving platinum‐based chemotherapy for lung cancer between 2001 and 2007.

METHODS:

The authors searched the Texas Cancer Registry–Medicare‐linked database for individuals aged >65 years who received platinum‐based chemotherapy within 12 months after a first diagnosis of lung cancer from 2001 to 2007; and all patients had continuous Medicare Part A and Part B coverage for the same period. Adherence to recommended regimens for N/V prophylaxis (established by the National Comprehensive Cancer Network) was scored as a binary variable (adherent vs nonadherent) and was calculated as the percentages of treated patients receiving each recommended agent within 1 day of beginning chemotherapy. Logistic regression with stepwise selection was used to examine whether patient characteristics influenced adherence.

RESULTS:

Of 4566 selected patients, adherence rates for the receipt of serotonin antagonists (eg, ondansetron) with dexamethasone were 60% to 90% regardless of whether the chemotherapy agent was considered moderately or highly emetogenic. The receipt of substance‐P antagonists was much less common (<10%) during any period. On multivariate logistic regression modeling, variables that predicted adherence were older age, white race, higher median income, and concurrent radiation therapy.

CONCLUSIONS:

Recommended use of antiemetics for prophylaxis, especially substance‐P antagonists, during chemotherapy for lung cancer is suboptimal. Factors that were correlated with adherence suggest socioeconomic barriers in the community. Cancer 2013. © 2012 American Cancer Society.     

A systematic review of orofacial pain in patients receiving cancer therapy

Purpose

We present the findings of a structured systematic review of the literature assessing orofacial pain induced by malignant disease and/or its therapy (excluding mucositis). This evaluation of the literature published after the 1989 NIH Development Consensus conference on the oral complications of cancer therapies is an effort to assess the prevalence of pain, quality of life and economic impact, and management strategies for cancer therapy-induced orofacial pain.

Methods

A systematic medical literature search was conducted with assistance from a research librarian in MEDLINE/PubMed and EMBASE databases for articles published between January 1, 1990 and December 31, 2008. Each study was independently assessed by two reviewers with expertise in the field of oral oncology.

Results

Thirty-nine studies assessed pain in the head and neck region. The measure was commonly embedded in quality of life studies. Most of these studies described pain in head and neck cancer (HNC) patients, which therefore became the focus of the report. Pain is common in patients with HNC and is reported by approximately half of patients prior to cancer therapy, 81% during therapy, 70% at the end of therapy, and by 36% at 6 months after treatment. Pain is experienced beyond the 6-month period by approximately one third of patients and is typically more severe than pre-treatment cancer-induced pain.

Conclusions

This systematic review identified the presence of pain before cancer therapy, likely attributable to the cancer; an increase in pain during therapy and the common persistence of pain following cancer treatment. Continuing research should use validated tools to prospectively assess orofacial pain, its causes and pathophysiology, and its effect on quality of life and economic impact. Clinical trials of pain management in this setting are also warranted.     

A multilayered approach for the analysis of perinatal mortality using different classification systems

Many classification systems for perinatal mortality are available, all with their own strengths and weaknesses: none of them has been universally accepted. We present a systematic multilayered approach for the analysis of perinatal mortality based on information related to the moment of death, the conditions associated with death and the underlying cause of death, using a combination of representatives of existing classification systems. We compared the existing classification systems regarding their definition of the perinatal period, level of complexity, inclusion of maternal, foetal and/or placental factors and whether they focus at a clinical or pathological viewpoint. Furthermore, we allocated the classification systems to one of three categories: ‘when’, ‘what’ or ‘why’, dependent on whether the allocation of the individual cases of perinatal mortality is based on the moment of death (‘when’), the clinical conditions associated with death (‘what’), or the underlying cause of death (‘why’). A multilayered approach for the analysis and classification of perinatal mortality is possible by using combinations of existing systems; for example the Wigglesworth or Nordic Baltic (‘when’), ReCoDe (‘what’) and Tulip (‘why’) classification systems. This approach is useful not only for in depth analysis of perinatal mortality in the developed world but also for analysis of perinatal mortality in the developing countries, where resources to investigate death are often limited.     

Keratotic skin lesions and other risk factors are associated with skin cancer in organ-transplant recipients: a case-control study in The Netherlands, United Kingdom, Germany, France, and Italy

This study examines the association of keratotic skin lesions with the development of skin cancer in 915 solid organ-transplant recipients in five European countries. In a hospital-based case-control study, cases with squamous- and basal-cell carcinoma were compared with controls without skin cancer. Questionnaires, scrutiny of medical charts, and skin examination were delivered according to a standardized protocol. Keratotic skin lesions and viral warts were counted on different body sites. Keratotic skin lesions were strongly associated with an increased risk of squamous-cell carcinoma, with adjusted odds ratios of 4.1 (2.4;7.0) and 12.1 (6.1;24) for 1-49 and 50 and more keratotic skin lesions compared with no lesions, respectively. Keratotic skin lesions were also associated with basal-cell carcinoma with adjusted odds ratios of 2.9 (1.7;4.9) and 4.0 (1.7;9.2) for 1-49 and 50 and more lesions, respectively. Lighter skin types and painful sunburns were also significantly associated with an increased risk of squamous- and basal-cell carcinoma. Keratotic skin lesions are strongly associated with skin cancer and are, thus, an important clinical criterion for identifying those organ-transplant recipients at an increased risk of skin cancers who should be offered more intensive skin surveillance.