End-of-life care.

My 96-year-old aunt taught me so much about end-of-life care. As doctors dealing with long-term neurological conditions, our relationships with patients over many years should enable us to be much more involved in helping them make decisions about the ceilings of treatment.

Outpatient neurology diagnostic coding: a proposed scheme for standardised implementation.

Clinical coding uses a classification system to assign standard codes to clinical terms and so facilitates good clinical practice through audit, service design and research. However, despite clinical coding being mandatory for inpatient activity, this is often not so for outpatient services, where most neurological care is delivered. Recent reports by the UK National Neurosciences Advisory Group and NHS England's 'Getting It Right First Time' initiative recommend implementing outpatient coding. The UK currently has no standardised system for outpatient neurology diagnostic coding. However, most new attendances at general neurology clinics appear to be classifiable with a limited number of diagnostic terms. We present the rationale for diagnostic coding and its benefits, and the need for clinical engagement to develop a system that is pragmatic, quick and easy to use. We outline a scheme developed in the UK that could be used elsewhere.

Orthostatic hearing loss: audiovestibular manifestations of spontaneous intracranial hypotension.

A 36-year-old woman with severe postural headaches caused by spontaneous intracranial hypotension developed bilateral hearing loss. Her hearing loss varied in severity and also at times affected one ear more than the other. She noticed her hearing returned to normal on lying flat, and this was confirmed on audiometry. Her hearing fully recovered after treatment with blood patches. Audiovestibular symptoms affect up to 70% of people with spontaneous intracranial hypotension but are probably under-reported. Cerebrospinal fluid and inner ear fluids are related in two separate channels: the vestibular and the cochlear aqueducts. We discuss their role in the postural hearing loss of spontaneous intracranial hypotension.

Encephalomyelitis with Retinopathy in Common Variable Immunodeficiency (CVID).

Common variable immunodeficiency is the most common primary immunodeficiency and rarely causes neurological manifestations since the introduction of IVIg, but here, the authors present a case of a 31-year-old Afro-Caribbean man who after short non-adherence to his immunoglobulins, develops encephalomyelitis with retinopathy. To the authors' knowledge, this is the first case presented with retinal photographs, OCT, CT, MRI and brain biopsies.

Microvascular Non-Arteritic Ocular Motor Nerve Palsies-What We Know and How Should We Treat?

Patients with isolated unilateral pupil-sparing third or isolated fourth or sixth nerve palsies over 50 years are often diagnosed with "microvascular extraocular palsies". This condition and its management provoke divergent opinions. We review the literature and describe the incidence, pathology, clinical presentation, yield of imaging, and management. A retrospective diagnosis of exclusion has little practical use. We suggest a pragmatic approach to diagnosis, investigation, and management from initial presentation.

Systemic Lupus Erythematosus Presenting as Orbital Myositis.

The authors present a case of diplopia and eye pain due to orbital myositis in a patient with a de novo diagnosis of systemic lupus erythematosus. Systemic lupus erythematosus is a rare cause of orbital myositis and should be considered when other, more common, conditions have been excluded.

Brain atrophy and cerebral small vessel disease: a prospective follow-up study.

BACKGROUND AND PURPOSE: cerebral small vessel disease (SVD) is the most common cause of vascular dementia. Interest in the use of surrogate markers is increasing. The aims of this study were to determine if brain volume was different between patients with SVD and control subjects, whether it correlated with cognition in SVD, and whether changes in brain volume could be detected during prospective follow-up. METHODS: thirty-five patients (mean age, 68.8 years) who had a lacunar stroke and radiological evidence of confluent leukoaraiosis and 70 age- and gender-matched control subjects were recruited. Whole-brain T1-weighted imaging and neuropsychological testing were performed after 1 year on all patients and after 2 years for the control subjects. Fully automated software was used to determine brain volume and percentage brain volume change. An executive function score was derived. RESULTS: there was a significant difference in brain volume between the patients with SVD and control subjects (mean ± SD [mL] 1529 ± 84 versus 1573 ± 69, P=0.019). In the patients with SVD, there was a significant association between brain volume and executive function (r=0.501, P<0.05). The mean ± SD yearly brain atrophy rate for patients with SVD and control subjects was significantly different (-0.914% ± 0.8% versus -0.498% ± 0.4%, respectively, P=0.017). No change in executive function score was detected over this period. CONCLUSIONS: brain volume is reduced in SVD and a decline is detectable prospectively. The correlation with executive function at a cross-sectional level and the change in brain volume with time are both promising for the use of brain atrophy as a surrogate marker of SVD progression.

Prevalence of acute neurology: a 2-week snapshot in a district general hospital.

Neurological illnesses are a common cause of hospital admissions, yet most patients are not primarily managed by neurologists. Acute neurology service provision varies across the UK. This study aimed to establish the number of neurological admissions during a 2-week period in a district general hospital, as well as the proportion of those patients who were seen by the hyperacute neurology team. This was done by analysing the diagnostic codes. It was established that there was a total of 2,242 admissions during the study period and, of those, 491 (22%) had neurological codes. Analysis of the 491 hospital electronic records revealed that 229 (10%) were truly neurologically relevant and, of those, 14% of patients received an opinion from the neurology team. Therefore, it can be concluded that only a small proportion of acute neurology is currently being seen by neurologists.

Multimodal MRI in cerebral small vessel disease: its relationship with cognition and sensitivity to change over time.

BACKGROUND AND PURPOSE: Cerebral small vessel disease is the most common cause of vascular dementia. Interest in using MRI parameters as surrogate markers of disease to assess therapies is increasing. In patients with symptomatic sporadic small vessel disease, we determined which MRI parameters best correlated with cognitive function on cross-sectional analysis and which changed over a period of 1 year. METHODS: Thirty-five patients with lacunar stroke and leukoaraiosis were recruited. They underwent multimodal MRI (brain volume, fluid-attenuated inversion recovery lesion load, lacunar infarct number, fractional anisotropy, and mean diffusivity from diffusion tensor imaging) and neuropsychological testing. Twenty-seven agreed to reattend for repeat MRI and neuropsychology at 1 year. RESULTS: An executive function score correlated most strongly with diffusion tensor imaging (fractional anisotropy histogram, r=-0.640, P=0.004) and brain volume (r=0.501, P=0.034). Associations with diffusion tensor imaging were stronger than with all other MRI parameters. On multiple regression of all imaging parameters, a model that contained brain volume and fractional anisotropy, together with age, gender, and premorbid IQ, explained 74% of the variance of the executive function score (P=0.0001). Changes in mean diffusivity and fractional anisotropy were detectable over the 1-year follow-up; in contrast, no change in other MRI parameters was detectable over this time period. CONCLUSIONS: A multimodal MRI model explains a large proportion of the variation in executive function in cerebral small vessel disease. In particular, diffusion tensor imaging correlates best with executive function and is the most sensitive to change. This supports the use of MRI, in particular diffusion tensor imaging, as a surrogate marker in treatment trials.

Acute neurology: a suggested approach.

Acute neurological problems are common, accounting for 10-20% of medical admissions. In the coming years, there will be increased neurology involvement in the acute care of these patients complementing traditional outpatient-based services. Models of acute neurology are reliant on close collaboration between the emergency department, acute medicine and neurology and should integrate with existing hyperacute stroke pathways. In this article the authors briefly describe the two models of acute neurology set up recently in our neuroscience group and suggest a clinical approach that may help non-neurologists involved in acute care settings. The authors emphasise some of the lessons learnt in delivering the service, particularly the importance of focusing on the acute problem and tailoring the examination and investigations to tackling it in the context of the patient's functional level and personal circumstances. Early neurology intervention can reduce admission and hospital length of stay.

A hyperacute neurology team - transforming emergency neurological care.

We present the results of an 18-month study of a new model of how to care for emergency neurological admissions. We have established a hyperacute neurology team at a single district general hospital. Key features are a senior acute neurology nurse coordinator, an exclusively consultant-delivered service, acute epilepsy nurses, an acute neurophysiology service supported by neuroradiology and acute physicians and based within the acute medical admissions unit. Key improvements are a major increase in the number of patients seen, the speed with which they are seen and the percentage seen on acute medical unit before going to the general wards. We have shown a reduced length of stay and readmission rates for patients with epilepsy. Epilepsy accounted for 30% of all referrals. The cost implications of running this service are modest. We feel that this model is worthy of widespread consideration.