Crowned dens syndrome is a rare presentation of calcium pyrophosphate deposition disease. It is characterised by severe occipital pain and neck stiffness. Acute presentations are typically accompanied by fever and an inflammatory response and hence can be misdiagnosed as polymyalgia rheumatica or meningitis. Chronic relapsing presentations may be misdiagnosed as cervicogenic neck pain or occipital neuralgia. We present a patient who presented with a chronic relapsing form of crowned dens syndrome and discuss the epidemiology, typical presentation and management of this eminently treatable condition.

Diagnosis, treatment, and secondary management of cryptogenic stroke patients pose a formidable challenge. The scenario is further complicated in patients with native and prosthetic valvular heart disease. We present a case study of a 36‐year‐old man who received intravenous thrombolysis (IV‐tPA) and endovascular thrombectomy (EVT) for presumed “cryptogenic” complete middle cerebral artery infarction who made a surprisingly excellent clinical recovery despite poor baseline and postintervention neuroimaging. Retrospective gram stain of his clot confirmed a diagnosis of infective endocarditis. This raises an important issue regarding need for more routine histopathological analysis of clot retrieved after EVT in “cryptogenic” stroke patients particularly those with valvular heart disease.

Heyde's syndrome was first proposed in 1958. It refers to gastrointestinal haemorrhage resulting from a combination of aortic stenosis with angiodysplasia. This report explores the case of a 93-year-old lady who was admitted to hospital following a neck of femur fracture. She suffered from multiple comorbidities including renal failure and congestive heart failure secondary to critical aortic stenosis. As an inpatient she suffered an exacerbation of both her heart and renal failure postoperatively. A week later she suffered from heavy upper gastro-intestinal bleeding, which failed to respond to pharmacological and endoscopic therapies as well as angiographic embolisation. The pathophysiology of Heyde's syndrome: an acquired von Willebrand deficiency syndrome has a much wider impact than was commonly thought, both in terms of how common it is and in how the association may be extrapolated to a wide range of bleeding disorders, rather than simply angiodysplasia associated gastrointestinal haemorrhage.

Twenty percent of patients with Sjögren’s syndrome experience associated neurological disease. Transverse myelitis (TM) frequently forms part of a neuromyelitis optica spectrum disorder associated with the presence of anti-aquaporin 4 (AQP4) antibodies. We report the first described case of a patient who developed TM and the presence of a newly recognized antibody, anti-myelin oligodendrocyte protein (MOG), who went on to develop Sjögren’s syndrome. AQP4 and MOG antibodies should be tested to guide prognostically the chances of further relapse as well as the type and duration of immunotherapy in patients with coexisting Sjögren’s syndrome and TM.

BackgroundAutonomic dysfunction, including gastrointestinal, cardiovascular, and urinary dysfunction, is often present in early Parkinson's Disease (PD). However, the knowledge of the longitudinal progression of these symptoms, and the connection between different autonomic domains, is limited. Furthermore, the relationship between the presence of autonomic symptoms in early-stage PD and olfactory dysfunction, a possible marker of central nervous system involvement, has not been fully investigated.ObjectivesWe aimed to investigate the occurrence and progression of autonomic dysfunction in recently diagnosed (< 2 years) untreated PD patients and determine any coexistence of symptoms in individual patients. We also investigated the relationship between autonomic symptoms, olfactory dysfunction, and motor impairment.MethodsData were obtained from the Parkinson's Progression Markers Initiative (PPMI) database. Autonomic dysfunction was measured using the Scales for Outcomes in Parkinson's Disease (SCOPA-AUT). Symptom frequency and mean scores over 7 years were determined. The simultaneous occurrence of different autonomic symptoms was also examined. Finally, the relationships between SCOPA-AUT scores, olfactory dysfunction, and motor impairment were investigated using the University of Pennsylvania Smell Identification Test (UPSIT) and the Movement Disorder Society—Unified Parkinson's Disease Rating Scale (MDS-UPDRS), respectively.ResultsFollow-up data were available for 7 years for 171 PD patients and for 5 years for 136 HCs. Mean SCOPA-AUT score increased significantly from baseline to the 7-year follow-up for each autonomic domain, except for female sexual dysfunction. Most patients reported three or more autonomic symptoms. Common clusters of symptoms were composed of combinations of gastrointestinal, urinary, thermoregulatory, and sexual dysfunction. At baseline, greater SCOPA-AUT total score was associated with lower UPSIT scores (r = −0.209, p = 0.006) and with greater total MDS-UDPRS III score (r = 0.218, p = 0.004).ConclusionsAutonomic dysfunction, often with coexistence of autonomic manifestations, is common in early PD and progressively worsens over the first 7 years of disease, suggesting that these symptoms should be addressed with appropriate treatments early in the disease. The association between greater autonomic dysfunction and greater olfactory impairment, coupled with the association with more severe motor scores at baseline, indicates that patients who show more severe autonomic dysfunction could also have more severe involvement of the central nervous system at the time of diagnosis.

Patients with psychotic illnesses are predicted to die 15 years younger than the national average. The chief cause is cardiovascular disease (1). Evidence-based guidelines including those produced by the National Institute of Health and clinical Excellence and the Quality Outcomes Framework, recommend regular monitoring of their cardiovascular risk (2,3,4).Primary health care audits were undertaken in an urban and a rural setting. These looked at the proportion of patients who had their physical health regularly monitored in line with NICE guidelines. Following an audit in general practice, it became clear that there was a group of patients that were chronic non-attenders. It was not clear whether these patients were the responsibility of the general practices or psychiatric services. An audit in secondary care then looked at the level of cardiovascular health monitoring in that setting, and the communication of results to primary care. These audits demonstrated that monitoring of cardiovascular health did not meet standards as set by NICE. Further to this, communication of findings between primary and secondary care was also poor.Primary care interventions included setting up Alert reminder boxes on the computer system and sending invitations for clinic attendance to ‘at risk’ patients. In secondary care interventions included redesign of the patient lists to include a way of monitoring cardiovascular health and generation of a new discharge summary to facilitate communication of cardiovascular indicators to primary care.These interventions have resulted in marked improvements in cardiovascular health monitoring in primary care, however, there is still room for considerable improvement. Discussions about further intervention strategies, and further audit cycles, are ongoing.

Cytotoxic agents such as cyclophosphamide are infrequently used in neurological practice. When they are, it is commonly in critically ill patients or in those with refractory inflammatory disorders. Cyclophosphamide in particular has a well-recognised negative impact on both female and male long-term fertility. This article summarises the data with regards the impact of cytotoxics on long-term fertility and describes the current options to preserve fertility in these patients. We hope this will provide neurologists with a useful aid for counselling patients for whom they are considering these treatments.

Iodine-based contrast agents are widely used in angiographic and other radiological procedures. Clinicians are familiar with many of the potential adverse events from contrast agents including allergic reactions and contrast-induced nephropathy. This case describes a lesser known adverse event: ‘contrast-induced thyrotoxicosis’ and its implications on the presentation and management of a patient with severe coronary artery disease. The management of this case was difficult and required a long inpatient admission with use of prednisolone, propylthiouracil and planned treatment with radioiodine to control the thyrotoxicosis, as well as the use of several rate-limiting agents and antianginal medications to control atrial fibrillation and prevent further episodes of angina.