Movement Disorders in Prionopathies: A Systematic Review

Background: Movement disorders are frequent features of prionopathies. However, their prevalence and onset remain poorly described. Methods: We performed a systematic review of case reports and case series of pathologically- and genetically confirmed prionopathies. Timing of symptom and movement disorder onset were documented. Continuous variables were compared between two groups using the Wilcoxon rank sum test and between multiple groups using Kruskal–Wallis test. Categorical variables were compared using Fisher’s exact test. Results: A total of 324 cases were included in this analysis. Movement disorders were a common feature at the onset of symptoms in most prionopathies. Gait ataxia was present in more than half of cases in all types of prionopathies. The prevalence of limb ataxia (20%) and myoclonus (24%) was lower in Gerstmann–Sträussler–Scheinker disease compared to other prionopathies (p ≤ 0.004). Myoclonus was common but often a later feature in sporadic Creutzfeldt–Jakob disease (2 months before death). Chorea was uncommon but disproportionately prevalent in variant Creutzfeldt–Jakob disease (30% of cases; p < 0.001). In genetic Creutzfeldt–Jakob disease, E200K PRNP carriers exhibited gait and limb ataxia more often when compared to other mutation carriers. Discussion: Movement disorders are differentially present in the course of the various prionopathies. The movement phenomenology and appearance are associated with the type of prion disease and the PRNP genotype and likely reflect the underlying pattern of neurodegeneration. Reliance on myoclonus as a diagnostic feature of sporadic Creutzfeldt–Jakob disease may delay its recognition given its relatively late appearance in the disease course

Coining the Pablo Picasso Syndrome.

BACKGROUND: The Alice in Wonderland Syndrome has been well described in the literature. Along with sensory disturbances, patients notably experience metamorphopsias, or distortions in size (micropsia/macropsia) and distance (teleopsia), particularly of body parts. Some migraineurs, however, report dislocation and disorientation of body parts, which are common features found in paintings of Pablo Picasso. METHODS: A 60-year-old female with a 20-year history of chronic migraine presented for evaluation of frequent headaches and visual disturbances. In between her attacks of migraine, she would occasionally notice alterations which she describes as a Picasso painting. When looking at people, she would note their ear on top of their head, or their right arm would be short and attached to the face. This would occur 1-2 times per week and would last 5 to 10 minutes. Inanimate objects, however, would not appear distorted. RESULTS: The available literature, including published works and biographies, suggests Picasso did not suffer from migraine. Nonetheless, careful descriptions taken from migraineurs with visual disturbances may uncover features that resemble his paintings, be it dislocation of limbs with hints of cubism, as reported above, or illusory splitting as has been previously reported. CONCLUSION: Dislocation or disorientation of body parts as a migraine-related visual phenomenon is rare and only sparsely reported in the medical literature. Coining of a Pablo Picasso Syndrome may better describe this occurrence

Utility of Continuous Video EEG in patients with Cardiac Arrest

Objective: To assess the utility of continuous video EEG in prognosis of patients after cardiac arrest Background: Continuous video electroencephalography recording (CEEG) is often used as one of the non-invasive methods of prognostication in comatose patients with cardiac arrest; however, it’s utility and outcomes in this patient population is uncertain. The aim of this study was to assess the utility of CEEG in prognosis of patients with cardiac arrest. Design/Methods: Retrospective chart review was performed on patients who had cardiac arrest between January 1, 2018 to June 30, 2018, who underwent CEEG. Variables analyzed included presence of clinical seizures and electrographic characteristics on EEG, comorbidities, duration of downtime after arrest, use of sedation, anti-seizure drugs (ASD) utilized, and pre and post modified Rankin scores. Fisher’s exact tests and two sample t-tests were used to compare those who lived and died. Results: CEEG on 19 patients who met the inclusion criteria, showed background slowing or generalized periodic discharges with triphasic morphology in 53%, lateralized periodic discharges (LPDS), quasi-LPDs/SIRPIDs, sharps, spike and wave discharges in 26%, clinical status epilepticus in 11%, and non-epileptic spells in 11%. Sixty eight percent of patients received sedation and/or ASDs. Hypoxic/anoxic brain injury was diagnosed in 47% of patients and sepsis or metabolic abnormalities in 79%. A total of 74% patients died in the hospital, majority from withdrawal of care. There were no differences in the CEEG findings and duration of downtime after cardiac arrest between patients who survived and died. Conclusions: This study indicates that CEEG appears to have limited utility in prognostication after cardiac arrest. Limitations of the study include small study sample and its retrospective nature. Prospective studies with larger populations are warranted to further address the utility and cost effectiveness of CEEG in the overall prognosis of patients with cardiac arrest

Post-stroke Movement Disorders: The Clinical, Neuroanatomic, and Demographic Portrait of 284 Published Cases

PURPOSE: Abnormal movements are a relatively uncommon complication of strokes. Besides the known correlation between stroke location and certain movement disorders, there remain uncertainties about the collective effects of age and stroke mechanism on phenomenology, onset latency, and outcome of abnormal movements. MATERIALS AND METHODS: We systematically reviewed all published cases and case series with adequate clinical-imaging correlations. A total of 284 cases were analyzed to evaluate the distribution of different movement disorders and their association with important cofactors. RESULTS: Posterolateral thalamus was the most common region affected (22.5%) and dystonia the most commonly reported movement disorder (23.2%). The most common disorders were parkinsonism (17.4%) and chorea (17.4%) after ischemic strokes and dystonia (45.5%) and tremor (19.7%) after hemorrhagic strokes. Strokes in the caudate and putamen were complicated by dystonia in one third of the cases; strokes in the globus pallidus were followed by parkinsonism in nearly 40%. Chorea was the earliest poststroke movement disorder, appearing within hours, whereas dystonia and tremor manifested several months after stroke. Hemorrhagic strokes were responsible for most delayed-onset movement disorders (\u3e6 months) and were particularly overrepresented among younger individuals affected by dystonia. CONCLUSIONS: This evidence-mapping portrait of poststroke movement disorders will require validation or correction based on a prospective epidemiologic study. We hypothesize that selective network vulnerability and resilience may explain the differences observed in movement phenomenology and outcomes after stroke

Orthostatic hypotension in Parkinson disease: Impact on health care utilization

INTRODUCTION: Orthostatic hypotension (OH) represents a frequent yet overlooked source of disability in Parkinson disease (PD). In particular, its impact on health care utilization has been insufficiently examined. We sought to determine the differential health care utilization in PD patients with (PDOH+) and without OH (PDOH-). METHODS: We quantified the emergency room (ER) visits, hospitalizations, outpatient clinic evaluations, phone calls, and e-mails from PD patients on whom supine and orthostatic blood pressure (BP) measurements were obtained during routine clinical practice between June 2013 and July 2016. Comparative costs between PDOH+ and PDOH- were adjusted for age, disease duration, motor severity, levodopa equivalent daily dose, and Montreal Cognitive Assessment. RESULTS: From a total of 317 PD patients, 29.3% were classified as PDOH+ (n = 93) and 70.6% as PDOH- (n = 224) over 30.2 ± 11.0 months, in which there were 247 hospitalizations, 170 ER visits, 2386 outpatient evaluations, and 4747 telephone calls/e-mails. After-adjusting for relevant covariates, PDOH+ was associated with more hospitalization days (+285%; p = 0.041), ER visits (+152%; p = 0.045), and telephone calls/e-mails than PDOH- (+142%; p = 0.009). The overall health care-related cost in PDOH+ was 2.5-fold higher than for PDOH- ($25,205 ±$6546 vs. $9831 ±$4167/person/year; p = 0.037). CONCLUSION: OH increases health care utilization in PD independently from age, disease duration, motor severity, dopaminergic treatment, and cognitive function

Students' participation in collaborative research should be recognised

Letter to the editor