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Autonomous ship technology is developing at a rapid pace, with the aim of facilitating safe ship operations. Collision avoidance is one of the most critical tasks that autonomous ships must handle. To support the level of safety associated with collision avoidance, this study suggests to provide autonomous ships with the ability to conduct proactive collision avoidance maneuvers. Proactive collision avoidance entails predicting future encounter situations, such that they can preemptively be avoided. However, any such actions must adhere to relevant navigation rules and regulations. As such, it is suggested to predict encounter situations far in advance, i.e. prior to risk of collision existing. Any actions can, therefore, be conducted prior to the applicability of the COLREGs. As such, simple corrective measures, e.g. minor speed and/or heading alterations, can prevent close encounter situations from arising, reducing the overall risk associated with autonomous ship operations, as well as improving traffic flow. This study suggests to facilitate this ability by emulating the development of situation awareness in ship navigators through machine learning. By leveraging historical AIS data to serve as artificial navigational experience, long-range trajectory predictions can be facilitated in a similar manner those conducted by human navigators, where such predictions provide the basis for proactive collision avoidance actions. The development of human situation awareness is, therefore, presented, and relevant machine learning techniques are discussed to emulate the same mechanisms.

Pulmonary embolism (PE) is a significant contributor to morbidity and mortality in the United States. Catheter-directed, ultrasound-assisted thrombolysis (USAT) uses high-frequency, low-energy ultrasound waves to disaggregate uncrosslinked fibrin fibers and increase thrombus penetration of a locally delivered thrombolytic to treat an acute PE. The purpose of this study is to compare the efficacy and safety of catheter-directed USAT versus systemic anticoagulation alone in submassive PE. This was a single-center, retrospective study of patients with a diagnosis of acute submassive PE from April 4, 2014 to May 1, 2019 at a large, academic medical center. Subjects were split into two different groups based on treatment with either USAT with systemic anticoagulation or systemic anticoagulation alone. The primary outcome was the incidence of severe or life-threatening GUSTO bleeding within 72 h or until hospital discharge if sooner. A total of 130 subjects were included (n = 40 in the USAT group and n = 90 in systemic anticoagulation alone group). Significantly fewer subjects in the USAT group had an active diagnosis of cancer at the time of presentation (7.5% vs 28.9%, p = 0.006). There was no difference in severe or life-threatening GUSTO bleeding or any component of the GUSTO bleeding definitions. Administration of USAT with systemic anticoagulation was well-tolerated when compared to systemic anticoagulation alone, but bias may have led to selection of patients for USAT with a lower bleeding risk and higher functional status at baseline.

Ruptured aortic aneurysms carry a high risk of morbidity and mortality, particularly if not rapidly identified. We present an 87-year-old male, with a history of hypertension and prior endovascular aortic repair, who presented to the Emergency Department (ED) with several days of epigastric abdominal pain radiating to his back and flanks. The patient acutely deteriorated in the ED, and point-of-care ultrasound rapidly identified active extravasation from an abdominal aortic aneurysm with visualization of prior endograft. Point-of-care ultrasound in this patient expedited the diagnosis, resuscitation, and transfer to the operating room with definitive repair by vascular surgery. The patient recovered and was discharged in stable condition.

In an effort to expedite the publication of articles, AJHP is posting manuscripts online as soon as possible after acceptance. Accepted manuscripts have been peer-reviewed and copyedited, but are posted online before technical formatting and author proofing. These manuscripts are not the final version of record and will be replaced with the final article (formatted per AJHP style and proofed by the authors) at a later time.

Background We describe the clinical and neuropathological features of a patient with T-cell-mediated paraneoplastic limbic encephalitis, parkinsonism, hypothermia, and narcolepsy-like presentation associated with endometrial carcinoma. Objectives This patient with prominent parkinsonism and narcolepsy broadens the phenotype of known paraneoplastic syndromes and demonstrates the importance of investigation for occult malignancy even in the absence of paraneoplastic antibodies. Methods This is a case report with diagnosis confirmed at postmortem. Results Paraneoplastic antibodies were not detected. The initial improvement with immunosuppression was short lived, and postmortem neuropathological examination demonstrated encephalitis with predominant T-cell infiltration affecting the hypothalamus and extending to the brainstem, suggestive of a paraneoplastic syndrome. Conclusions Although the possibility of a novel antibody cannot be ruled out, consideration must also be given to recent demonstration of purely T-cell-mediated neuronal destruction in the context of paraneoplastic syndromes.

Disclaimer In an effort to expedite the publication of articles related to the COVID-19 pandemic, AJHP is posting these manuscripts online as soon as possible after acceptance. Accepted manuscripts have been peer-reviewed and copyedited, but are posted online before technical formatting and author proofing. These manuscripts are not the final version of record and will be replaced with the final article (formatted per AJHP style and proofed by the authors) at a later time.

This study presents a method in which historical AIS data are used to predict the future trajectory of a selected vessel. This is facilitated via a system intelligence-based approach that can be subsequently utilized to provide enhanced situation awareness to navigators and future autonomous ships, aiding proactive collision avoidance. By evaluating the historical ship behavior in a given geographical region, the method applies machine learning techniques to extrapolate commonalities in relevant trajectory segments. These commonalities represent historical behavior modes that correspond to the possible future behavior of the selected vessel. Subsequently, the selected vessel is classified to a behavior mode, and a trajectory with respect to this mode is predicted. This is achieved via an initial clustering technique and subsequent trajectory extraction. The extracted trajectories are then compressed using the Karhunen-Loeve transform, and clustered using a Gaussian Mixture Model. The approach in this study differs from others in that trajectories are not clustered for an entire region, but rather for relevant trajectory segments. As such, the extracted trajectories provide a much better basis for clustering relevant historical ship behavior modes. A selected vessel is then classified to one of these modes using its observed behavior. Trajectory predictions are facilitated using an enhanced subset of data that likely correspond to the future behavior of the selected vessel. The method yields promising results, with high classification accuracy and low prediction error. However, vessels with abnormal behavior degrade the results in some situations, and have also been discussed in this study.

This paper presents a method for energy efficient weather routing of a ferry in Norway. Historical operational data from the ferry and environmental data are used to develop two models that predict the energy consumption. The first is a purely data-driven linear regression energy model, while the second is as a hybrid model, combining physical models with data-driven models using machine learning techniques. With an established energy model, it is possible to develop a route optimization that proposes efficient routes with less energy usage compared to fixed speed and heading control.

Purpose Obtaining an accurate medication history from patients on hospital admission is a priority in pharmacy practice. Timely and accurate histories are imperative as they may help determine the etiology of illness and prevent medication errors. We conducted a quality improvement project to assess the accuracy of alternate-source medication histories obtained for critically ill patients who were delirious or mechanically ventilated at the time of intensive care unit admission. Methods Included patients were 18 years of age or older, admitted to the medical intensive care unit from August 2017 through January 2018, and had a medication history obtained from a family member or outpatient pharmacy due to active delirium or mechanical ventilation. Patients were directly interviewed after resolution of delirium or extubation. Discrepancies between the initial and follow-up histories were documented and categorized using the National Coordinating Council for Medication Error Reporting and Prevention (NCC MERP) Index for Categorizing Medication Errors. Results Forty patients were included. One hundred four discrepancies were documented, with a median of 2 discrepancies per patient. The most common types of discrepancies were addition (51.9%), followed by omission (24.0%). NCC MERP index category A (51%) was the most common error classification identified. Conclusion Discrepancies between initial and follow-up medication histories occurred at a frequent rate in delirious or mechanically ventilated patients; however, these discrepancies tended to be of low risk severity.

An operationally implementable predictive model has been developed to forecast the number of COVID-19 infections in the patient population, hospital floor and ICU censuses, ventilator and related supply chain demand. The model is intended for clinical, operational, financial and supply chain leaders and executives of a comprehensive healthcare system responsible for making decisions that depend on epidemiological contingencies. This paper describes the model that was implemented at NorthShore University HealthSystem and is applicable to any communicable disease whose risk of reinfection for the duration of the pandemic is negligible.

Pulmonary embolism (PE) is a significant contributor to morbidity and mortality in the United States. Catheter-directed, ultrasound-assisted thrombolysis (USAT) uses high-frequency, low-energy ultrasound waves to disaggregate uncrosslinked fibrin fibers and increase thrombus penetration of a locally delivered thrombolytic to treat an acute PE. The purpose of this study is to compare the efficacy and safety of catheter-directed USAT versus systemic anticoagulation alone in submassive PE. This was a single-center, retrospective study of patients with a diagnosis of acute submassive PE from April 4, 2014 to May 1, 2019 at a large, academic medical center. Subjects were split into two different groups based on treatment with either USAT with systemic anticoagulation or systemic anticoagulation alone. The primary outcome was the incidence of severe or life-threatening GUSTO bleeding within 72 h or until hospital discharge if sooner. A total of 130 subjects were included (n = 40 in the USAT group and n = 90 in systemic anticoagulation alone group). Significantly fewer subjects in the USAT group had an active diagnosis of cancer at the time of presentation (7.5% vs 28.9%, p = 0.006). There was no difference in severe or life-threatening GUSTO bleeding or any component of the GUSTO bleeding definitions. Administration of USAT with systemic anticoagulation was well-tolerated when compared to systemic anticoagulation alone, but bias may have led to selection of patients for USAT with a lower bleeding risk and higher functional status at baseline.

Aim: To assess the cost–effectiveness of onabotulinumtoxinA (onabotA), implantable sacral nerve stimulation devices, percutaneous tibial nerve stimulation, anticholinergic medications and mirabegron compared with best supportive care (BSC) for management of refractory overactive bladder (OAB). Methods: A Markov model was developed to compare the cost–effectiveness of treatment options with BSC over a 10-year time horizon. Resource utilization, discontinuation rates and costs were derived from unpublished and published sources. Quality-adjusted life-years (QALYs) and incremental cost–effectiveness ratios were reported. Results: Treatment with onabotA 100U produced the largest gain in QALYs (7.179) and lowest estimated incremental cost–effectiveness ratio ($32,680/QALY) of all assessed treatments compared with BSC. Conclusion: Compared with BSC, onabotA 100U was the most cost-effective treatment option for patients with refractory OAB.

Purpose Hepatorenal syndrome (HRS) is renal dysfunction associated with the hemodynamic consequences of advanced liver disease and cirrhosis. HRS is associated with a high mortality, and there remain high failure rates with first-line therapy aimed at improving perfusion. We report the use of droxidopa, an oral norepinephrine precursor, to aid in the management of HRS-AKI refractory to first-line therapy. Summary A 51-year-old Caucasian male with alcohol-related cirrhosis presented with 1-week history of pre-syncope and falls. He was found to have acute kidney injury meeting diagnostic criteria of HRS based on absence of identifiable contributing factors. After no response to volume expansion, medical management was initiated with midodrine and octreotide and eventually escalated to norepinephrine intravenous infusion. The patient’s renal function and urine output improved initially on norepinephrine, but worsened when attempting to wean to a suitable outpatient regimen, becoming dependent upon norepinephrine. On day 13 of hospitalization, droxidopa was initiated at a dose of 100 mg three times daily and titrated to a dose of 400 mg three times daily. Norepinephrine infusion was weaned and discontinued on day 16 of hospitalization. The patient remained hemodynamically stable and was able to be discharged on droxidopa 400 mg three times daily, midodrine 20 mg three times day, and octreotide 200 mcg three times daily. Conclusion Droxidopa, an oral norepinephrine precursor, presents a novel adjunctive agent for management of HRS refractory to first-line medical management.

SummaryModern legislation in the UK addressing the issue of decision-making ability uses tests of mental capacity based on the individual's ability to understand relevant information given to them. Alternative models of mental capacity do exist, but are largely considered defunct. This article reviews these alternative models and considers their importance. Far from being irrelevant to modern views on mental capacity, these models have already been incorporated into legislation such as the Mental Capacity Act 2005. A better understanding of these models can improve clinicians’ understanding of mental capacity in general. Modern controversies such as the impact of the United Nations Convention on the Rights of Persons with Disabilities (CRPD) are discussed and ways in which our understanding of mental capacity may have to change in the future are addressed.LEARNING OBJECTIVES•Appreciate the different ways of considering an individual's mental capacity•Understand the primacy and limitations of the functional test of mental capacity•Use this understanding to manage potential conflicts or disagreements when considering an individual's mental capacity

Objective: Inhalation injury is an independent risk factor for mortality in burn patients. The purpose of this study was to observe the effect of inhaled iloprost on gas exchange in patients with inhalation injury and acute respiratory failure as measured by an improved PaO2/FiO2 ratio. Methods: Patients admitted to the burn intensive care unit from 2013 to 2014 meeting Berlin criteria for acute respiratory distress syndrome (ARDS) with a diagnosis of inhalation injury and who received inhaled iloprost were included. Medical records were reviewed to collect patient demographics, characterize iloprost prescribing practices, and observe changes in oxygenation and hemodynamic status after iloprost administration. Differences were evaluated using a t-test with cluster corrected standard errors. Results: A total of eight patients were included with 157 different PaO2/FiO2 ratios calculated. All patients had moderate or severe ARDS with a baseline PaO2/FiO2 ratio of 131.9 mmHg (IQR 119.3–197.3). Median duration of iloprost therapy was 5 days (IQR ± 7). A statistically significant increase in PaO2/FiO2 ratio was observed after iloprost administration with a mean increase of 9.7 mmHg (95% CI 1.8–17.7, p = 0.023). Inhaled iloprost had no effect on hemodynamic parameters. Conclusions: Inhaled iloprost for inhalation injury and ARDS after burn injury was associated with a small but statistically significant improvement in oxygenation. Keywords: Iloprost, Inhaled prostacyclin, Inhalation injury

We show that spironolactone use was associated with an increased rate of all-cause hospitalizations, but no difference in hospitalizations for heart failure or pulmonary arterial hypertension, in patients with World Health Organization Group 1 pulmonary arterial hypertension. A possible reason for this finding is confounding from retrospective study design.

This study presents a method to predict the future trajectory of a target vessel using historical AIS data. The purpose of such a prediction is to aid in collision avoidance in future vessels. The method presented in this study extracts all trajectories present in an initial cluster centered about a vessel position. Features for each trajectory are then generated using Principle Component Analysis and used in clustering via unsupervised Gaussian mixture modeling. Each resultant cluster represents a possible future route the vessel may follow. A trajectory prediction is then conducted with respect to each cluster of trajectories discovered. This results in a prediction of multiple possible trajectories. The results indicate that the algorithm is effective in clustering the trajectories, where at least one cluster corresponds to the true trajectory of the vessel. The resultant predicted trajectories are also found to be reasonably accurate.

Autonomous ship navigation in a mixed environment, where remote-controlled, autonomous and manned vessels are interacting, is considered. Since these vessels can have various encounter situations, adequate knowledge on such situations should be acquired to take appropriate navigation actions. That has often been categorized as situation awareness in a mixed environment, where appropriate tools and techniques to extract the respective knowledge on ship encounter situations should be developed. The collision risk assessment procedure has an important role in the same knowledge and that can eventually be used towards the respective collision avoidance actions. Hence, possible ship collision and near-miss situations can be avoided by both humans as well as systems due to their actions. Ship collision avoidance actions are regulated by the International Regulations for Preventing Collisions at Sea 1972 (COLREGs) in open sea areas and additional local navigation rules and regulations can also enforce especially in confined waters and maritime traffic lanes. It is expected that the COLREGs and other navigation rules and regulations will be interpreted by both humans as well as systems in future vessels and those interpretations will be executed as collision avoidance actions by the respective vessels in a mixed environment. Adequate understanding on situation awareness should be achieved to overcome possible regulatory failure due to human and system decisions in these situations. Hence, this study focuses on identifying such challenges in future ship encounters with possible solutions to improve situation awareness in a mixed environment as the main contribution.

Provision of specialist mental health services for older adults (previously referred to as ‘psychogeriatrics’) is recommended by the World Health Organization. In the UK, this extends to specialist wards for older inpatients with mental health problems. This chapter explores the rationale and provision of specialist older adult mental health beds in the UK and the different types of service offered. Beyond the macro level, it aims to provide a handbook for anyone wishing to set up such a ward from scratch: it explores what a ward designed with older adults in mind would look like (design) and what resources and staffing are necessary. The needs specific to this demographic of the population are explored, including issues such as physical health, frailty, and the most common mental health conditions one might encounter on such as ward (dementia, late-life depression, and late-life psychosis).

During pandemics and global crises, drug shortages become critical as a result of increased demand, shortages in personnel and lockdown restrictions that disrupt the supply chain. The pharmaceutical industry is therefore moving towards continuous manufacturing instead of conventional batch manufacturing involving numerous steps, that normally occur at different sites. In order to validate the use of large-scale industrial processes, feasibility studies need to be performed using small-scale laboratory equipment. To that end, the scale-up of a continuous process and its effect on the critical quality attributes (CQAs) of the end product were investigated in this work. Hydroxychloroquine Sulphate (HCQS) was used as the model drug, Soluplus® as a model polymeric carrier and both horizontal and vertical twin screw extruders used to undertake this hot melt extrusion (HME) study. Seven formulations were processed using a small-scale horizontal extruder and a pilot-scale vertical extruder at various drug loadings, temperature profiles and screw speeds. When utilising a horizontal extruder, formulations with the highest drug load and processed at the lowest screw speed and temperature had the highest crystallinity with higher drug release rates. Upon scale-up to a vertical extruder, the crystallinity of the HCQS was significantly reduced, with less variation in both crystallinity and release profile across the different extrudates. This study demonstrates improved robustness with the pilot-scale vertical extruder compared to lab-scale horizontal extruder. The reduced variation with the vertical extruder will allow for short increases in production rate, with minimum impact on the CQAs of the final product enabling high-performance continuous manufacturing with minimum waste of raw materials. Finally, this research provides valuable information for the pharmaceutical industry in accessing continuous technologies for the manufacture of pharmaceutical products, allowing for efficient utilisation of resources upon scale-up and mass production during global pandemics and drug shortages.

Autosomal recessive hereditary spastic paraparesis is rare.We present 4 patients with slowly progressive predominantly lower limb spasticity and ataxia. Only one patient had family history of ataxia but without any underlying diagnosis. All of them proved negative for the mutation of Spinocerebelalr ataxia genes SCA 1,2,3 and 6. All had mutation in the SPG 7 gene suggestive of autosomal recessive hereditary spastic paraparesis. One of the heterozygous mutatnts showed a novel c1617delC ,p(Val540fs) frameshift mutation in exon 12 of the SPG 7 gene. SPG7 mutation accounts for 1.5-7% of all the HSP but it is the cause of undiagnosed ataxia in 18.6% in a recent case series. SPG7 mutation should be remembered as an important cause of undiagnosed ataxia especially where next generation sequencing is not widely avaialbale or affordable.

Early diagnosis of dementia allows people to access effective treatment and make advance decisions while they still have capacity. We aimed to encourage people to attend memory clinic, in order to boost rates of diagnosis. We created a patient information video about Oxford Health NHS Foundation Trust Memory Clinics, to inform and empower those awaiting assessment and to promote early diagnosis.Fourteen people (patients, carers, and staff) were approached prior to developing the video to ascertain their views on the themes the video should cover. The video consisted of unscripted interviews with patients, carers, and staff. We surveyed participants and new patients attending memory clinic to get feedback on the video and to assess patients’ level of understanding and confidence about a memory assessment before and after watching the video. The video content was refined based on this feedback and a final version was produced.Patient feedback demonstrated that confidence and understanding increased after watching the video.Although this study is limited by its small sample size and lack of access to those with undiagnosed dementia, feedback suggested that the video empowered and reassured those awaiting assessment and could be used as a tool to reduce barriers to early diagnosis. Patients and carers involved in making the video found it a therapeutic activity in itself.

INTRODUCTION: Early commentary on SARS-CoV-2 infection proposed a mechanism of cytokine release syndrome (CRS) to explain severe acute respiratory failure associated with COVID-19 Management strategies have included targeted immunomodulation with biologic agents The role of IL-6 and other cytokines in the pathogenesis of COVID-19 is not well defined Evidence for use of immunomodulators has been mixed and these agents may expose patients to harm This study aims to characterize the expression of cytokines and their association with inflammatory biomarkers and outcomes in critically ill patients with COVID-19 METHODS: This was a single-center, retrospective analysis of critically ill patients with COVID-19 confirmed by PCR Patients were included if they had a partial or full cytokine panel drawn while admitted Descriptive statistics were used to assess demographics, outcomes, and relationships between cytokine levels and inflammatory markers The Mann- Whitney U Test was used to compare IL-6 levels between survivors and nonsurvivors RESULTS: Eighty-nine patients were included with 68 full cytokine panels and 108 IL-6 levels Patients had a mean (range) of 10 ventilator-days (0-47), 15 ICU-days (1-60), 20 hospital-days (3-69) and a mortality rate of 31% Cytokine levels were assessed a median of 10 days from symptom onset and 1 day from ICU admission Levels of IL-1B, IL-2, IL-4, IL-5, IL-8, IL-12, IL-13, IL-17, IFN-G, and TNF-A were undetectable in at least 80% of patients, and expression did not correlate with other inflammatory biomarkers (CRP, ferritin), severity of illness (SOFA), or outcome IL-2R levels were numerically elevated in most patients (n=68;median 1227, range: 76-30670) IL-6 levels were mildly elevated (n=108;median: 31, range: 2-882, SD: 150), and levels were statistically significantly higher in nonsurvivors (p = 0 002) CONCLUSIONS: Assessment of cytokine levels in critically ill patients with COVID-19 does not support a hypothesis of CRS While IL-6 levels were numerically higher in nonsurvivors, the clinical significance of this finding is unknown The role of IL-2R in the pathogenesis of COVID-19 remains unclear Larger studies exploring the role of inflammatory mediators in pathogenesis and targeted immunomudulators in management of COVID-19 are warranted

INTRODUCTION: Severe acute respiratory failure is a common complication of COVID-19, with refractory hypoxemia being a hallmark finding in severe illness and a common cause of mortality With limited therapeutic strategies, management centers on good supportive care Prone positioning has been shown to improve oxygenation and survival in patients with moderate-to-severe acute respiratory distress syndrome (ARDS) but the impact of prone positioning in COVID-19 with severe hypoxemia is unknown This study aims to examine the response to proning as a predictor of COVID-19 related mortality METHODS: This is a single-center, retrospective analysis of critically ill patients with COVID-19 confirmed by PCR Patients were included if they were invasively ventilated, and if supportive care included prone positioning for management of refractory hypoxemia Data points collected include demographics, ventilator settings, rates of mortality and progression to ECMO, ventilator-days, and time between symptom onset and intubation, hospital and ICU admission Endpoints included response in oxygenation (PaO2:FiO2) and mortality RESULTS: Forty-nine patients were included in the analysis The average age was 56 9, and 61% of the patients were male Patients had an average of 19 ventilator-days (2-52), 21 ICU-days (4-54), 26 hospital-days (8-65), an ECMO rate of 27%, and a mortality rate of 55% Of the 22 survivors, there was an average increase in PaO2:FiO2 by 108, 93 1, and 93 for each of the first three pronations respectively For the 27 nonsurvivors, there was an average increase in PaO2:FiO2 by 76 1, 84 3, and 50 9 for the first three pronations The difference in improvement in PaO2:FiO2 was not statistically significant between survivors and non-survivors There was no inflection point that could be determined that provided a high sensitivity and specificity to predict mortality or need for ECMO based on response to pronation at any of the time points CONCLUSIONS: Proning improves PaO2:FiO2 in patients with severe hypoxemia related to COVID-19 Survivors in our study had a numerically greater response to proning, but this finding was not statistically significant The clinical significance remains unclear Larger studies assessing the efficacy of proning in critically ill patients with COVID-19 are needed

Advances in artificial intelligence are driving the development of intelligent transportation systems, with the purpose of enhancing the safety and efficiency of such systems. One of the most important aspects of maritime safety is effective collision avoidance. In this study, a novel dual linear autoencoder approach is suggested to predict the future trajectory of a selected vessel. Such predictions can serve as a decision support tool to evaluate the future risk of ship collisions. Inspired by generative models, the method suggests to predict the future trajectory of a vessel based on historical AIS data. Using unsupervised learning to facilitate trajectory clustering and classification, the method utilizes a cluster of historical AIS trajectories to predict the trajectory of a selected vessel. Similar methods predict future states iteratively, where states are dependent upon the prior predictions. The method in this study, however, suggests predicting an entire trajectory, where all states are predicted jointly. Further, the method estimates a latent distribution of the possible future trajectories of the selected vessel. By sampling from this distribution, multiple trajectories are predicted. The uncertainties of the predicted vessel positions are also quantified in this study.

A 31 year-old man with a history of a depressive episode presented with acute severe ‘thunderclap’ headache. Magnetic resonance imaging (MRI) revealed abnormalities typical of cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL), which was subsequently confirmed by genetic analysis. The psychiatric features of this genetic cause of depression and headache are discussed.