Hyperacute neurology at a regional neurosciences centre: a 1-year experience of an innovative service model.

St George's Hospital hyperacute neurology service (HANS) is a comprehensive, consultant-delivered service set in a teaching hospital regional neuroscience centre. The service addresses deficiencies in acute neurological care previously highlighted by the Royal College of Physicians and the Association of British Neurologists. HANS adopts a disease-agnostic approach to acute neurology, prioritising the emergency department (ED) management of both stroke and stroke mimics alike alongside proactive daily support to the acute medical unit and acute medical take. Rapid access clinics provide a means to assess ambulatory patients, providing an outlet to reduce the burden of referral from primary care to acute medicine. This paper reports the results from the first year of the service. Admission was avoided in 25% of the cases reviewed in the ED. Compared to historic data, there was a significant improvement in the length of stay for non-stroke disorders while the occupancy of stroke beds by non-stroke cases reduced by 50%. The configuration of this service is replicable in other neuroscience centres and provides a framework to reduce the barriers facing patients who present with acute neurological symptoms.

Is REM sleep behaviour disorder (RBD) a risk factor of dementia in idiopathic Parkinson's disease?

OBJECTIVE: To study the sequence of occurrence of REM-sleep behaviour disorder (RBD) and dementia and their frequency among a population of patients with idiopathic Parkinson's disease (PD). METHODS: We performed a cross-sectional study on 65 PD patients seen in a movement disorder clinic and their bed partner, and asked them to complete the validated Mayo Sleep Questionnaire for RBD and sleep disorders. The diagnosis of PD with dementia (PD-D) was based on a clinical diagnosis of dementia; following DSM-IV criteria and MMSE score less than 25 and a battery of cognitive tests. RESULTS: From the 65 patients that completed the study, twenty-four met the clinical diagnosis of RBD. Ten of the 24 (42%) RBD patients met the clinical criteria of PD-D, whereas the remaining 14 patients were non-demented at the time of the study. The frequency of RBD was significantly higher in the PD-D group (n = 10, 77%) compared to the PD-ND group (n = 14, 27%, chi squared test: p = 0.0008). PD non-RBD had a lower occurrence of dementia (7.3%, 3 of 41) compared to those suffering from RBD (42%, 10 of 24). Of the 65 PD patients, 13 were diagnosed with PD-D and the remaining 52 were non-demented PD (PD-ND) patients. PD with RBD showed a faster decline in the number of dementia-free patients compared to the non-RBD patients (Log Rank test: p < 0.001). RBD preceded, coincided or followed the onset of the motor symptoms. CONCLUSION: This study shows that RBD and dementia have a significant coincidence in the course of PD, and RBD not only precedes or coincides with the motor signs, but can occur during the course of the progression of the PD, suggesting a degenerative process of the dopaminergic and cholinergic neurons of the brainstem nuclei, progressing at a different pace in each patient.

Alteration in alpha-synuclein mRNA expression in Parkinson's disease.

The presynaptic protein alpha-synuclein is considered to play an important role in the pathophysiology of Parkinson's disease (PD). Point mutations in the alpha-synuclein gene have been demonstrated in familial PD and alpha-synuclein is a major component of Lewy bodies, the pathological hallmark of the sporadic disease. It is not clear whether abnormal accumulation of alpha-synuclein is the result of abnormal levels of expression of the gene in neurodegenerative conditions. Expression of alpha-synuclein mRNA was therefore studied in control and PD brain using semiquantitative in situ hybridization. alpha-synuclein was expressed widely and hybridization signal was seen in most cortical regions, hippocampus, cerebellum, and brain stem. There was little mRNA in the striatum and no hybridization signal was detected in glia. High levels of alpha-synuclein mRNA expression in neurons did not seem to be a marker for Lewy body formation. Abundant signal was seen both in regions in which Lewy body deposition occurs commonly in idiopathic PD (PD), such as substantia nigra and frontal and temporal cortex, as well as in less susceptible regions, e.g. visual cortex. Quantitative comparison of mRNA expression in regions of predilection for Lewy body formation showed that mRNA expression was reduced significantly in melanized substantia nigra neurons and frontal cortex neurons in Parkinson's disease. In substantia nigra neurons there seemed to be a negative correlation between cellular mRNA expression and disease duration. These findings are in broad agreement with other studies of the expression of alpha-synuclein mRNA in human brain and suggest that Lewy body formation is unlikely to be the result of overexpression of alpha-synuclein.