Bower, James H.

Abstract The syndrome of episodic angioedema and eosinophilia is characterized by cyclic edema, marked peripheral blood eosinophilia, and eosinophil degranulation in the dermis. Using a sensitive immunoenzymetric method, we measured serum interleukin (IL)-5 levels in four patients with this syndrome. We also determined the percentage of activated T cells in the peripheral blood of a new patient before and during an attack. In the patient presented, IL-5 levels peaked several days before maximal eosinophilia and then declined. This patient's lymphocytes showed an increased percentage, 28% (normal 2% to 3%), of activated T cells staining for both CD3 and HLA-DR 10 days before maximal eosinophilia, but no increase at the time of peak eosinophilia. In serum from three previously reported cases, elevated serum IL-5 levels were found during attacks. After glucocorticoid administration, IL-5 levels became undetectable in three of the four patients. Production of IL-5 is likely an important determinant of the pathophysiology of this syndrome.

The syndrome of episodic angioedema and eosinophilia is characterized by cyclic edema, marked peripheral blood eosinophilia, and eosinophil degranulation in the dermis. Using a sensitive immunoenzymetric method, we measured serum interleukin (IL)-5 levels in four patients with this syndrome. We also determined the percentage of activated T cells in the peripheral blood of a new patient before and during an attack. In the patient presented, IL-5 levels peaked several days before maximal eosinophilia and then declined. This patient's lymphocytes showed an increased percentage, 28% (normal 2% to 3%), of activated T cells staining for both CD3 and HLA-DR 10 days before maximal eosinophilia, but no increase at the time of peak eosinophilia. In serum from three previously reported cases, elevated serum IL-5 levels were found during attacks. After glucocorticoid administration, IL-5 levels became undetectable in three of the four patients. Production of IL-5 is likely an important determinant of the pathophysiology of this syndrome.

Background: Few studies have investigated the incidence of PSP and CBS in the population. Objective: To examine the incidence of and trends in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) in a population-based cohort of residents of Olmsted County, MN. Methods: We used the 1991-2005 population-based, Olmsted County Parkinsonism-cohort study, defined via the Rochester Epidemiology Project. A movement-disorder specialist reviewed medical records, to confirm PSP and CBS diagnoses. Results: We identified 21 patients with these diagnoses 1991-2005 : 18 (85.7%), PSP; 3 (14.3%), CBS. The median diagnosis age was 78 (range: 66-88). 13/21 (62.0%) were male. MRI was performed pre-diagnosis in 11 patients (8 PSP and 3 CBD); 10 showed atrophy consistent with clinical diagnoses. We observed concordance between clinical and pathological diagnoses in two PSP patients who underwent autopsy. Combined incidence for PSP and CBS in Olmsted County was 3.1 per 100,000 person-years (2.6 per 100,000 person-years, PSP; 0.4 per 100,000 person-years, CBS). Incidence was higher in men (4.5, 95% CI, 2.0-7.0) than women (1.8, 95% CI, 0.5-2.9). A combined, significant trend of increasing incidence was observed between 1991 and 2005 (B=0.69, 95% CI 0.42, 0.96, p<0.001). Median time from symptom onset to death among both groups was 6 years (range PSP, 1-10 years; range CBS, 3-8 years). Conclusions: The combined incidence for PSP and CBS was 3.1 per 100,000 person-years, higher in men than women. We observed a significant increase in both PSP and CBS, likely due to advancing imaging technology and improved diagnostic ability among physicians.

Abstract Background: Non motor symptoms (NMS) of Parkinson’s disease (PD) are common and can be more disabling than motor symptoms.Sleep disorders can be seen in up to 98% of patients with Parkinson disease. Poor sleep quality has been associated with poverty and race, and yet there has been no prior report on sleep disorders in those with PD living in sub Saharan Africa. We wished to document the prevalence of sleep disorders in PD patients in Ethiopia. Methods: We conducted a cross-sectional point prevalence study from July 1 to October 30, 2015 of all patients attending the neurology outpatient department in Tikur Anbessa and Zewuditu Memorial Hospitals, Addis Ababa, Ethiopia. Demographic data, clinical history and physical examination findings were collected from participants using a structured questionnaire. We used the Parkinson’s disease sleep scale version two (PDSS-2) and Epworth Sleepiness Scale (ESS) to assess the sleep symptoms. Results: Of the 155 patients surveyed, all patients reported some sleep problem. Over 43.9% of patients had a PDSS score > 18. The median score of ESS was 9 (IQR = 5-12), with 77/155 (49.7%) of the patients having possible or definite excessive daytime somnolence. A high EDSS score significantly associated with a Hoehn & Yahr score > 4 (p= 0.02). Conclusions: In Ethiopian PD patients, the prevalence of those with severe sleep disorders is the highest reported to date. The prevalence of possible/definite EDS is amongst the highest in the world. Further investigation into whether poverty or race explains this finding is needed.

Objective. Autonomic symptoms are common in α-synuclein disorders: multiple system atrophy (MSA), Parkinson’s disease (PD), dementia with Lewy bodies (DLB), and Parkinson’s disease with dementia (PDD). These symptoms may precede the motor findings/clinical diagnosis by years. Erectile dysfunction (ED) is an autonomic symptom that has rarely been studied in these α-synuclein disorders. In this population-based, case-control study, we investigated the association between premonitory erectile dysfunction surfacing prior to the clinical-motor manifestations of these α-synucleinopathies. Methods. We used the medical record-linkage system of the Rochester Epidemiology Project to identify cases of α-synucleinopathies in Olmsted County from 1991 to 2010. Each male case was matched by age (±1 year) of symptom onset and sex to a control. We reviewed complete medical records of cases and controls to detect erectile dysfunction prior to the clinical-motor onset of α-synucleinopathies of any type. We used conditional logistic regression to calculate the odds ratio of all α-synucleinopathies, as well as by type, adjusting for diabetes, coffee, and smoking. Results. A history of male erectile dysfunction was associated with 1.5-fold increased odds of an α-synucleinopathy diagnosis of any type in univariate analyses (p=0.06). When stratifying α-synucleinopathies by type, early erectile dysfunction was most frequent in MSA cases than matched controls (45% vs. 9%). Premotor phase ED was next most frequent among the DLB cases (46% vs. 27% among the controls; OR = 2.83, p=0.03; when adjusted for diabetes, smoking, and coffee, OR = 2.98, p=0.04). Premotor phase ED was not significantly associated with PD or PDD. Conclusions. Early erectile dysfunction may be a premotor symptom of MSA and DLB, reflecting premonitory dysautonomia. It was not associated with premotor PD or PDD.

Abstract Background: Non motor symptoms (NMS) of Parkinson’s disease (PD) are common and can be more disabling than motor symptoms.Sleep disorders can be seen in up to 98% of patients with Parkinson disease. Poor sleep quality has been associated with poverty and race, and yet there has been no prior report on sleep disorders in those with PD living in sub Saharan Africa. We wished to document the prevalence of sleep disorders in PD patients in Ethiopia. Methods: We conducted a cross-sectional point prevalence study from July 1 to October 30, 2015 of all patients attending the neurology outpatient department in Tikur Anbessa and Zewuditu Memorial Hospitals, Addis Ababa, Ethiopia. Demographic data, clinical history and physical examination findings were collected from participants using a structured questionnaire. We used the Parkinson’s disease sleep scale version two (PDSS-2) and Epworth Sleepiness Scale (ESS) to assess the sleep symptoms. Results: Of the 155 patients surveyed, all patients reported some sleep problem. Over 43.9% of patients had a PDSS score > 18. Presence of previous history of sleep disturbance before PD motor symptoms (OR 3.54; 95% CI 1.61-7.76, p=0.001) and unemployment (OR 2.27; 95% CI 1.07-4.79, p=0.023) associated with a high PDSS-2 score. The median score of ESS was 9 (IQR = 5-12), with 77/155 (49.7%) of the patients having possible or definite excessive daytime somnolence. Conclusions: In Ethiopian PD patients, the prevalence of those with severe sleep disorders is the highest reported to date. The prevalence of possible/definite EDS is amongst the highest in the world. Further investigation into whether poverty or race explains this finding is needed.

Abstract ABSTRACT Background: Non motor symptoms (NMS) of Parkinson’s disease (PD) are common and can be more disabling than motor symptoms. Sleep disorders can be seen in up to 98% of patients with Parkinson disease. Poor sleep quality has been associated with poverty and race, and yet there has been no prior report on sleep disorders in those with PD living in sub Saharan Africa. We wished to document the prevalence of sleep disorders in PD patients in Ethiopia. Methods: We conducted a cross-sectional point prevalence study from July 1 to October 30, 2015 of all patients attending the neurology outpatient department in Tikur Anbessa and Zewuditu Memorial Hospitals, Addis Ababa, Ethiopia. Demographic data, clinical history and physical examination findings were collected from participants using a structured questionnaire. We used the Parkinson’s disease sleep scale version two (PDSS-2) and Epworth Sleepiness Scale (ESS) to assess the sleep symptoms. Results: Of the 155 patients surveyed, all patients reported some sleep problem. Over 23% of patients had a PDSS score > 30. Hoehn and Yahr score > 3 (OR 6.16; 95% CI 1.59-23.9, p=0.009) and PD symptom duration > 5 yrs (OR 5.11; 95% CI 1.41-18.5, p=.013) associated with a high PDSS-2 score. The median score of ESS was 9 (IQR = 5-12), with 77/155 (49.7%) of the patients having possible or definite excessive daytime somnolence. Conclusions: In Ethiopian PD patients, the prevalence of those with severe sleep disorders is the highest reported to date. The prevalence of possible/definite EDS is amongst the highest in the world. Further investigation into whether poverty or race explains this finding is needed. Keywords: Parkinson’s disease, Sleep disorders, Africa, poverty

Abstract Background In 2017, the International Parkinson and Movement Disorder Society put forward new clinical criteria for the diagnosis of PSP, recognizing diverse PSP phenotypes. In this study, we compared the sensitivity and specificity of the new criteria with the National Institutes of Neurological Disease and Society for Progressive Supranuclear Palsy criteria at different times. Methods Patients with clinical parkinsonism, clinical and/or neuropathological diagnosis of PSP, were identified from the Society for Progressive Supranuclear Palsy brain bank. All patients had neuropathologic diagnoses and detailed clinical examination performed by a neurologist at 1 of the 3 Mayo Clinic sites, in Florida, Arizona, and Minnesota. Clinical symptoms and signs were abstracted retrospectively in a blinded fashion and used to determine whether patients met either diagnostic criterion. Patients were divided into early and late disease stage groups using a 3‐year cutoff. Results A total of 129 patients were included, of whom 66 had PSP pathology (51%). The remainder had other neurodegenerative diseases. The overall sensitivity of the International Parkinson and Movement Disorder Society criteria was 87.9%, compared with 45.5% for the National Institutes of Neurological Disease and Society for Progressive Supranuclear Palsy criteria, whereas the specificity of the International Parkinson and Movement Disorder Society probable PSP criteria was 85.7%, compared with 90.5% for the National Institutes of Neurological Disease and Society for Progressive Supranuclear Palsy. Individual patients were noted to have features of multiple PSP phenotypes. Conclusion The International Parkinson and Movement Disorder Society criteria recognize several phenotypes of progressive supranuclear palsy and hence have higher sensitivity than the previous criteria. © 2019 International Parkinson and Movement Disorder Society