Parkinson disease (PD) follows a defined clinical pattern, and a range of nonmotor symptoms precede the motor phase. The predominant early nonmotor manifestations are olfactory impairment and constipation. The pathology that accompanies these symptoms is consistent with the Braak staging system: α-synuclein in the dorsal motor nucleus of the vagus nerve, the olfactory bulb, the enteric nervous system (ENS) and the submandibular gland, each of which is a gateway to the environment. The neuropathological process that leads to PD seems to start in the ENS or the olfactory bulb and spreads via rostrocranial transmission to the substantia nigra and further into the CNS, raising the intriguing possibility that environmental substances can trigger pathogenesis. Evidence from epidemiological studies and animal models supports this hypothesis. For example, in mice, intragastric administration of the pesticide rotenone can almost completely reproduce the typical pathological and clinical features of PD. In this Review, we present clinical and pathological evidence to support the hypothesis that PD starts in the gut and spreads via trans-synaptic cell-to-cell transfer of pathology through the sympathetic and parasympathetic nervous systems to the substantia nigra and the CNS. We also consider how environmental factors might trigger pathogenesis, and the potential for therapeutically targeting the mechanisms of these initial stages.

ObjectiveDecrease of olfactory function in Parkinson's disease (PD) is a well-investigated fact. Studies indicate that pharmacological treatment of PD fails to restore olfactory function in PD patients. The aim of this investigation was whether patients with PD would benefit from “training” with odors in terms of an improvement of their general olfactory function. It has been hypothesized that olfactory training should produce both an improved sensitivity towards the odors used in the training process and an overall increase of olfactory function.MethodsWe recruited 70 subjects with PD and olfactory loss into this single-center, prospective, controlled non-blinded study. Thirty-five patients were assigned to the olfactory training group and 35 subjects to the control group (no training). Olfactory training was performed over a period of 12 weeks while patients exposed themselves twice daily to four odors (phenyl ethyl alcohol: rose, eucalyptol: eucalyptus, citronellal: lemon, and eugenol: cloves). Olfactory testing was performed before and after training using the “Sniffin' Sticks” (thresholds for phenyl ethyl alcohol, tests for odor discrimination, and odor identification) in addition to threshold tests for the odors used in the training process.ResultsCompared to baseline, trained PD patients experienced a significant increase in their olfactory function, which was observed for the Sniffin' Sticks test score and for thresholds for the odors used in the training process. Olfactory function was unchanged in PD patients who did not perform olfactory training.ConclusionThe present results indicate that olfactory training may increase olfactory sensitivity in PD patients.

Restless legs syndrome (RLS), also known as Willis-Ekbom disease (WED), is a common movement disorder characterised by an uncontrollable urge to move because of uncomfortable, sometimes painful sensations in the legs with a diurnal variation and a release with movement. The pathophysiology is only partially known and a genetic component together with dopaminergic and brain iron dysregulation plays an important role. Secondary causes for RLS need to be excluded. Treatment depends on the severity and frequency of RLS symptoms, comprises non-pharmacological (eg lifestyle changes) and pharmacological interventions (eg dopaminergic medication, alpha-2-delta calcium channel ligands, opioids) and relieves symptoms only. Augmentation is the main complication of long-term dopaminergic treatment of RLS. This article will provide a clinically useful overview of RLS with provision of diagnostic criteria, differential diagnoses, possible investigations and different treatment strategies with their associated complications. Definition and diagnostic criteriaRestless legs syndrome (RLS), also known as Willis-Ekbom disease (WED), is a common neurological movement disorder characterised by an uncontrollable urge to move (mainly the legs).1 Diagnosis of RLS is based primarily on the patient's history and on a neurological examination to exclude differential diagnoses. The diagnosis can be made if all of the following five criteria are met (International RLS Study Group (IRLSSG) diagnostic criteria): 2 1 A need to move the legs usually accompanied or caused by uncomfortable, unpleasant sensations in the legs. 2 Symptoms are exclusively present or worsen during times of inactivity/rest. 3 Partial or total relief of symptoms by movement, such as walking or stretching, at least as long as the activity continues. 4 Symptoms are generally worse or exclusively occur in the evening or during the night. ABSTRACT Restless legs syndrome5 The occurrence of the fi rst four essential criteria must not be solely accounted for as symptoms primary to another medical or a behavioural condition.A single standard question for rapid screening of RLS has been validated by the IRLSSG, 3 which is: 'when you try to relax in the evening or sleep at night, do you ever have unpleasant, restless feelings in your legs that can be relieved by walking or movement?' This question can be used to effectively screen large patient groups as it has 100% sensitivity and 96.8% specificity for the diagnosis of RLS. However, the final diagnosis should always be confirmed by matching the patient's history and symptoms with the IRLSSG diagnostic criteria, accompanied by an exclusion of secondary conditions. Aetiology and differential diagnosesThe aetiology of RLS can be categorised as primary (idiopathic) or secondary. The majority of cases are primary Key pointsRestless legs syndrome (RLS) is a common neurological movement disorder, characterised by an uncontrollable urge to move the legs combined with an uncomfortable sensation in the legs.Diagnosis of RLS is mainly base...

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ObjectiveTo develop and validate a novel 14‐item self‐completed questionnaire (in English and German) enquiring about the presence of non‐motor symptoms (NMS) during the past month in patients with craniocervical dystonia in an international multicenter study.MethodsThe Dystonia Non‐Motor Symptoms Questionnaire (DNMSQuest) covers seven domains including sleep, autonomic symptoms, fatigue, emotional well‐being, stigma, activities of daily living, sensory symptoms. The feasibility and clinimetric attributes were analyzed.ResultsData from 194 patients with CD (65.6% female, mean age 58.96 ± 12.17 years, duration of disease 11.95 ± 9.40 years) and 102 age‐ and sex‐matched healthy controls (66.7% female, mean age 55.67 ± 17.62 years) were collected from centres in Germany and the UK. The median total NMS score in CD patients was 5 (interquartile range 3–7), significantly higher than in healthy controls with 1 (interquartile range 0.75–2.25) (P < 0.001, Mann–Whitney U‐test). Evidence for intercorrelation and convergent validity is shown by moderate to high correlations of total DNMSQuest score with motor symptom severity (TWSTRS: r s = 0.61), clinical global impression (r s = 0.40), and health‐related quality of life measures: CDQ‐24 (r s = 0.74), EQ‐5D index (r s = −0.59), and scale (r s = −0.49) (all P < 0.001). Data quality and acceptability was very satisfactory.InterpretationThe DNMSQuest, a patient self‐completed questionnaire for NMS assessment in CD patients, appears robust, reproducible, and valid in clinical practice showing a tangible impact of NMS on quality of life in CD. As there is no specific, comprehensive, validated tool to assess the burden of NMS in dystonia, the DNMSQuest can bridge this gap and could easily be integrated into clinical practice.

The susceptibility-weighted 3-T MRI-based subthalamic nucleus localization shows the best accuracy compared with T2-weighted and fluid-attenuated inversion recovery 3-T MRI. Therefore, the susceptibility-weighted 3-T MRI should be preferred for surgical planning when the operation procedure is performed under general anesthesia without microelectrode recordings.

Background and purpose Neurology is rapidly evolving as a result of continuous diagnostic and therapeutic progress, which influences the daily work of neurologists. Therefore, updating residency training programmes is crucial for the future of neurology. Several countries are currently discussing and/or modifying the structure of their neurology residency training programme. A detailed and up‐to‐date overview of the available European residency training programmes will aid this process. Methods A questionnaire addressing numerous aspects of residency training programmes in neurology was distributed among 38 national representatives of the Resident and Research Fellow Section of the European Academy of Neurology. Results We obtained data from 32 European countries (response rate 84%). The median (range) duration of the residency training programmes was 60 (12–72) months. In the majority of countries, rotations to other medical disciplines were mandatory, mostly psychiatry (69%), internal medicine (66%) and neurosurgery (59%). However, the choice of medical fields and the duration of rotations varied substantially between countries. In 50% of countries, there were formal regulations regarding training in evidence‐based medicine, teaching skills and/or leadership qualities. In many countries (75%), residents had to take an examination. Conclusions We found substantial variation among European countries in the duration of residency training programmes, and especially in the choice of obligatory rotations to external medical disciplines. Despite a presumably similar spectrum of patients, neurology residency training programmes across Europe are not harmonized. The structure of the programme should be determined by its relevance for neurologists today and in the future.