Multi-Residency Implementation of an Online Movement Disorders Curriculum Based on Real Patient Videos.

Background: In-person didactic education in residency has numerous challenges including inconsistent availability of faculty and residents, limited engagement potential, and non-congruity with clinical exposure. Methods: An online curriculum in movement disorders was implemented across nine neurology residency programs (six intervention, three control), with the objective to determine feasibility, acceptability, and knowledge growth from the curriculum. Residents in the intervention group completed ten modules and a survey. All groups completed pre-, immediate post-, and delayed post-tests. Results: Eighty-six of 138 eligible housestaff (62.3%) in the intervention group completed some modules and 74 completed at least half of modules. Seventy-four, 49, and 30 residents completed the pre-, immediate post-, and delayed post-tests respectively. Twenty-five of 42 eligible control residents (59.5%) completed at least one test. Mean pre-test scores were not significantly different between groups (6.33 vs. 6.92, p = 0.18); the intervention group had significantly higher scores on immediate post- (8.00 vs. 6.79, p = 0.001) and delayed post-tests (7.92 vs. 6.92, p = 0.01). Residents liked having a framework for movement disorders, appreciated the interactivity, and wanted more modules. Residents completed the curriculum over variable periods of time (1-174 days), and at different times of day. Discussion: This curriculum was feasible to implement across multiple residency programs. Intervention group residents showed sustained knowledge benefit after participating, and residents took advantage of its flexibility in their patterns of module completion. Similar curricula may help to standardize certain types of clinical learning and exposure across residency programs. Highlights: Interactive online tools for resident didactic learning are valuable to residents. Residents learn from interactive online curricula, find the format engaging, and take advantage of the flexibility of online educational tools. Beginner learners appreciate algorithms that help them to approach a new topic.

Antipsychotic Prescribing Practices in Those with Parkinsonism: Adherence to Guidelines.

Several evidence-informed treatment guidelines recommend against the use of typical antipsychotics in patients with Parkinson's disease; of the atypical antipsychotics, clozapine and quetiapine are preferred. The purpose of this study is to determine the frequency with which potentially inappropriate antipsychotics are dispensed to older adults in Nova Scotia who are on levodopa-containing medications. In this cohort, 59.9% were dispensed a preferred atypical antipsychotic and 12.6% a potentially harmful typical antipsychotic. Our results suggest that potentially inappropriate prescribing practices are common in the neuropsychiatric management of patients with parkinsonism and that there is an opportunity for education and improvement in prescribing practices.

Orthostatic Tremor: A Spectrum of Fast and Slow Frequencies or Distinct Entities?

BACKGROUND: Orthostatic tremor (OT) is defined by the presence of a high-frequency (13-18 Hz) tremor of the legs upon standing associated with a feeling of unsteadiness. However, some patients have discharge frequencies of <13 Hz, so-called "slow OT". The aim of this study was to characterize patients with unsteadiness upon standing found to have <13 Hz tremor discharges on neurophysiologic testing. METHODS: A retrospective review was performed on all subjects with a diagnosis of OT who were referred to the Mayo Clinic, Scottsdale, AZ, between 1999 and 2013 for confirmation using neurophysiology. RESULTS: Fourteen of 28 subjects (50%) had OT discharges of <13 Hz, of whom eight had frequencies of <10 Hz and six had frequencies of 10-13 Hz. Lower frequency discharges tended to have a broader spectral peak, greater variability in discharge duration, and lower inter-muscular coherence. Subjects with <13 Hz OT had shorter mean disease duration at time of neurophysiology testing (2.00 years in <10 Hz group, 7.96 years 10-13 Hz group, and 11.43 years >13 Hz; p = 0.002). The proportion of subjects who experienced gait unsteadiness (85.7% vs. 66.6% vs. 21.4%; p = 0.016), falls (37.5% vs. 50% vs. 0%; p = 0.010), and had abnormal gait on examination (71.4% vs. 66.0% vs. 14.3%; p = 0.017) was greater in those with low and intermediate frequencies. DISCUSSION: Slow tremor electromyography frequencies (<13 Hz) may characterize a substantial proportion of patients labeled as OT. These subjects may have greater gait involvement and higher likelihood of falls leading to earlier presentation to subspecialty care.

Abnormal visual contrast acuity in Parkinson's disease.

BACKGROUND: Low-contrast vision is thought to be reduced in Parkinson's disease (PD). This may have a direct impact on quality of life such as driving, using tools, finding objects, and mobility in low-light condition. Low-contrast letter acuity testing has been successful in assessing low-contrast vision in multiple sclerosis. We report the use of a new iPad application to measure low-contrast acuity in patients with PD. OBJECTIVE: To evaluate low- and high-contrast letter acuity in PD patients and controls using a variable contrast acuity eye chart developed for the Apple iPad. METHODS: Thirty-two PD and 71 control subjects were studied. Subjects viewed the Variable Contrast Acuity Chart on an iPad with both eyes open at two distances (40 cm and 2 m) and at high contrast (black and white visual acuity) and 2.5% low contrast. Acuity scores for the two groups were compared. RESULTS: PD patients had significantly lower scores (indicating worse vision) for 2.5% low contrast at both distances and for high contrast at 2 m (p < 0.003) compared to controls. No significant difference was found between the two groups for high contrast at 40 cm (p = 0.12). CONCLUSIONS: Parkinson's disease patients have reduced low and high contrast acuity compared to controls. An iPad app, as used in this study, could serve as a quick screening tool to complement more formal testing of patients with PD and other neurologic disorders.

Clinical Features of Patients with Concomitant Parkinson's Disease and Progressive Supranuclear Palsy Pathology.

The pathologic changes of Parkinson's disease (PD) and Progressive Supranuclear Palsy (PSP) have been reported to coexist, but whether PSP pathology modifies the clinical course of those individuals is unknown. The aim of this study was to determine whether clinical features of pathologically confirmed PD subjects with concomitant PSP pathology differ from those with PD alone. Subjects enrolled in the Arizona Study of Aging and Neurodegenerative Disorders had annual movement and cognitive evaluations from enrollment until death/autopsy. All cases between 1997 and 2014 with a final clinicopathological diagnosis of PD with or without PSP at autopsy were analyzed. Overall, 12 of the 125 cases with pathologically confirmed PD had coexisting PSP pathology (9.6%). Those with PD-PSP had more-prominent postural instability, body bradykinesia, difficulty arising from a chair, and falls; asymmetric onset was less common in this group. Downgaze palsy and square wave jerks were infrequent in both groups. Gender, age at death, disease duration, rate of dementia, and presence of rest tremor did not differ between groups. Only 58% of subjects in the PD-PSP group were correctly given a final diagnosis in life of PD, compared to 91% of those with PD alone. The combination of PD and PSP pathology yields a heterogeneous clinical syndrome that often resembles PD, but may be more symmetric at onset and have more-prominent postural instability and falls. Our observations suggest that coexisting PSP pathology may be an important factor contributing to the clinical heterogeneity in PD and a potential confounder in diagnosis.

Dysmetropsia and Cotard's syndrome due to migrainous infarction - or not?

INTRODUCTION: Migrainous infarction accounts for 12.8% of ischemic strokes of unusual etiology. CASE REPORT: A 59-year-old woman with longstanding migraine with aura experienced what appeared to be migrainous infarction characterized by dysmetropsia and transient Cotard's syndrome. Imaging demonstrated right temporal-parietal-occipital changes with apparent cortical laminar necrosis. CONCLUSION: The spectrum of the pathophysiology of migrainous infarction has not been established; however, cortical spreading depression may explain the appearance of imaging findings that do not obey a vascular territory.

Longitudinal neuropsychological findings in amyloid beta-related angiitis (AßRA): a case report.

Amyloid ß-related angiitis (AßRA) is a clinicopathological diagnosis of primary central nervous system angiitis theoretically triggered by vascular deposition of amyloid ß peptide. Deposits of Aß are associated with degeneration of the vasculature, thereby increasing risks of a stroke and/or cognitive impairment. Despite this, no prior studies have presented a detailed neuropsychological profile associated with AßRA. We present longitudinal neuropsychological findings for the case of a 58-year-old man with biopsy-diagnosed AßRA. Neuropsychological test results and clinical presentation demonstrated a mild to moderate dysexecutive syndrome implicating dorsolateral frontal and orbitofrontal-subcortical systems involvement. Despite prior reports of cognitive decline following a diagnosis of AßRA, cognitive functioning remained relatively stable over a 15-month period after immunosuppressive treatment. For the most part, objective measures did not demonstrate a measurable change in cognitive functioning, except for a mild decline in memory. There were subjective reports of improvement in cognitive and adaptive functioning from the patient and his spouse over this time period. The clinical significance of these results is discussed in the context of theories of executive dysfunction, and with reference to previously-published cases of AßRA.

Diagnostic challenges revealed from a neuropsychiatry movement disorders clinic.

BACKGROUND: Abnormal movements are frequently associated with psychiatric disorders. Optimized management and diagnosis of these movements depends on correct labeling. However, there is evidence of reduced accuracy in the labeling of these movements, which could result in sub-optimal care. OBJECTIVE: To determine the consensus inter-rater reliability between a movement disorders neurologist and physicians referring from the community for phenomenology and diagnoses of individuals with co-existing psychiatric conditions and movement disorders. METHOD: Charts of all consecutive patients seen in a combined Movement Disorders and Neuropsychiatry Clinic between 2001-2009 were reviewed retrospectively. Consensus estimates and kappa values for inter-rater reliability were determined for phenomenology and diagnostic terms for the respective referring source and movement disorders neurologist for each patient. RESULTS: A total of 106 charts were reviewed (62 men and 44 women). Agreement for phenomenology terms ranged from 0% (psychogenic) to 73% (tremor). Only 3 terms had kappa values that met or exceeded criteria for moderate inter-rater reliability. Agreement for diagnosis terms was highest for tardive dyskinesia (83%), drug induced tremor (33%), and drug induced parkinsonism (20%). In 18 of the 22 charts (82%), a diagnosis was made of drug induced movement disorder (DIMD) by the referring physician. In contrast, a diagnosis of DIMD was made in only 54 of 106 charts (51%) after the patients were assessed in the clinic. CONCLUSIONS: A movement disorders specialist frequently disagreed with referring physicians' identification of patient phenomenology and diagnosis. This suggests that clinicians would benefit from educational resources to assist in characterizing abnormal movements.

Education Research: An exploration of case-based learning in neuroscience grand rounds using the Delphi technique.

BACKGROUND: Neuroscience grand rounds (NSGR) is a key educational exercise in most academic medical centers. Despite its importance, there are few published studies evaluating the manner in which it is conducted. Our objective was to obtain consensus opinion from staff neurologists, neurosurgeons, neuroradiologists, and neuropathologists on the features that best characterize a highly educational NSGR. METHODS: Using the Delphi technique, multiple rounds of questionnaires were presented to a panel of neurologists, neurosurgeons, neuropathologists, and neuroradiologists. The anonymous responses were analyzed and fed back to participants. Each round, the participants were given the opportunity to react to collective opinion by changing their response or by presenting arguments in favor or against the item in question. RESULTS: We found that support for NSGR in its present form is high and that particularly strong support exists for 1) case-based rounds, 2) high level of audience interaction, 3) resident participation in case presentation and analysis, 4) formal training for residents in leading case-based presentations, and 5) resident feedback and evaluation. CONCLUSIONS: Our results offer centers that use a case-based format for NSGR with guidance to maximize the important learning opportunity that it provides. We provide an organized evaluation of expert opinion on how this important educational exercise should be conducted. The results expose some fresh insights into traditional values in medical education.

Amyloid ß-related angiitis of the central nervous system: report of 3 cases.

OBJECTIVE: Amyloid-ß (Aß) related angiitis (ABRA) is a recently described clinicopathological entity characterized by cerebrovascular Aß deposition and arteritis. Cerebral Aß deposition is commonly present in cerebal amyloid angiopathy (CAA) and Alzheimer's disease (AD) but is rarely associated with inflammatory infiltration of vessel walls. Our objective is to help clarify the clinical spectrum, radiographic findings, response to treatment, and outcomes of ABRA. The neuropathological relationship between ABRA, cerebral amyloid angiopathy, and Alzheimer's disease is discussed. METHODS: We present three cases of ABRA managed at a tertiary care centre. RESULTS: All three patients presented with seizures and cognitive dysfunction; one had multifocal neurologic findings. Brain biopsies revealed inflammatory arteritis with Aß deposits in the vessel walls. All were treated with steroids and cyclophosphamide. Two had favorable outcomes and one stabilized but with severe residual neurologic disability. CONCLUSIONS: ABRA is an unusual but likely under-recognized and potentially treatable disorder. As in other reported cases, our findings suggest that many patients respond favorably to immunosuppressive therapy. We believe that all biopsy specimens consistent with primary angiitis of the central nervous system (CNS) should be further examined for vascular Aß deposition.

Routine surveillance for bloodstream infections in a pediatric hematopoietic stem cell transplant cohort: Do patients benefit?

BACKGROUND: Hematopoietic stem cell transplant (HSCT) recipients are at a high risk for late bloodstream infection (BSI). Controversy exists regarding the benefit of surveillance blood cultures in this immunosuppressed population. Despite the common use of this practice, the practical value is not well established in non-neutropenic children following HSCT. METHODS: At the IWK Health Centre (Halifax, Nova Scotia), weekly surveillance blood cultures from central lines are drawn from children following HSCT until the line is removed. A retrospective chart review was performed to determine the utility and cost of this practice. Eligible participants were non-neutropenic HSCT recipients with central venous access lines. The cost of laboratory investigations, nursing time, hospital stay and interventions for positive surveillance cultures was calculated. RESULTS: Forty-three HSCTs were performed in 41 children. Donors were allogenic in 33 cases (77%) and autologous in 10 cases (23%). There were 316 patient contacts for surveillance cultures (mean seven per patient) and 577 central line lumens sampled. Three of 43 patients (7%) had clinically significant positive surveillance blood cultures. Bacteria isolated were Klebsiella pneumoniae (n=2) and Corynebacterium jeikeium (n=1). All follow-up cultures before initiation of antimicrobial therapy were sterile. All three patients were admitted for antimicrobial therapy if they were not already hospitalized and/or had an uncomplicated course. The estimated total cost of BSI surveillance and management of asymptomatic infection over six years was $27,989. CONCLUSION: The present study suggests that BSI surveillance in children following HSCT engraftment has a very low yield and significant cost. It is unclear whether it contributes to improved patient outcomes.

Providing research results to study participants: support versus practice of researchers presenting at the American Society of Hematology annual meeting.

Offering to provide research results to study participants is gaining increasing support based, in part, on the principle of respect for persons. The frequency and means of this practice is unknown in national and international research communities. All investigators who presented oral abstracts involving human research at the American Society of Hematology Annual Meeting (December 2003) were surveyed. Responses were received from 197 (42%) of 472 eligible investigators. Nonrespondents did not differ in study type or country of origin. Only 30% (n = 48) of those who completed the survey had a formal plan for the return of research results; 40% of these would return both a summary plus individual level results. Of the respondents, 69% (n = 109) supported or strongly supported the practice; only 3% opposed the practice. The most commonly cited reasons for not returning results were: did not consider it (38%), anticipated contact difficulties (32%), and participant difficulty understanding results (26%). Only 11 (7%) indicated that their institutional review board (IRB) mandates the offer to provide results to all participants; this did not vary significantly by country. Given the high level of support in the international research community, evaluation of well-planned interventions for offering to provide research results to participants should be a priority.

Lack of response of a metastatic renal perivascular epithelial cell tumor (PEComa) to successive courses of DTIC based-therapy and imatinib mesylate.

An 11 year-old girl presented with two large abdominal masses in the left flank and epigastrium and left supraclavicular lymphadenopathy. Subsequent investigations led to the diagnosis of metastatic perivascular epithelioid cell tumor (PEComa) arising from the left kidney. Effective treatment for this rare tumor is not yet known. The tumor did not respond to an initial treatment of two cycles of a dacarbazine (DTIC) based regimen. She was placed on a trial of imatinib mesylate based on tumor expression of c-KIT, a tyrosine kinase targeted by this drug. This report highlights the first documented case of the use of imatinib for PEComa. Lack of response and adverse effects of the drug required discontinuation of therapy.