Rational referrals: Neurology

Consultant neurologist Dr Rhys C Roberts advises on how to navigate referral pathways for neurological conditions

One in six people in the UK are living with a neurological condition¹ and that is expected to increase with an ageing population. Many neurological conditions are chronic and cause a significant and enduring impact on wellbeing. They typically require ongoing multidisciplinary and holistic approaches to management and need coordination of a variety of different professionals from health and social care.

People with undiagnosed neurological disorders commonly present first to their GP, but recognising an underlying neurological problem can be a challenge at this early stage, when diagnostic clinical features have yet to fully manifest. Nevertheless, with knowledge of the patient’s medical and social background built up over many years, a GP is likely to be better placed than a neurologist to assess a particular symptom or clinical change before determining an initial plan for investigation, which can range from adopting a watch and wait approach to an urgent referral for specialist input.

Optimising use of Advice and Guidance

Advice and Guidance (A&G) services offer an opportunity for direct and timely interaction between a GP and a neurologist.^2-4 This can involve seeking advice on the management and investigation of new symptoms or on management of people already living with chronic neurological disorders.

A GP might seek advice on the appropriateness of a potential referral to secondary care. In this way, the GP and patient can receive rapid specialist input, potentially avoiding investigative and therapeutic delays along with the need to travel to an outpatient appointment. Travel is often a logistical challenge for people living with neurological disorders.

Queries relating to neurology that are particularly suitable for A&G include:

Headaches. Most headache disorders that have already been diagnosed can be managed effectively in primary care in the absence of red flag features (see below) with appropriate A&G requested from secondary care when required. Patients and clinicians can be pointed towards online resources such as the British Association for the Study of Headache, the Migraine Trust and the Organisation for the Understanding of Cluster Headache for useful information regarding diagnosis and management.
Sensory symptoms. The distribution of isolated sensory symptoms often indicates common syndromes such as carpal tunnel syndrome or meralgia paraesthetica. Reassurance can usually be provided for fleeting or unusual sensory symptoms involving the scalp or related to olfaction in the context of a normal neurological examination.
Chronic neurological disorders. Queries relating to the management of patients already diagnosed with a neurological disorder can generally be addressed by A&G. This can include new symptoms, questions regarding medications, or the potential need for specialist input. If the patient is already known to a specific team or consultant, then queries should usually be directed first to the individual consultant rather than via the generic A&G pathway.

Neurological emergencies requiring same-day referral

Some neurological presentations should be considered as emergencies and trigger a same-day referral to the appropriate acute services in secondary care. These include:

Acute sudden focal neurological symptoms and signs suggestive of a stroke or TIA. With the advent of time-dependent medical and interventional radiological therapies aimed at cerebral reperfusion, all patients with suspected acute stroke should be referred immediately to the nearest stroke service without delay. If the symptoms have resolved consistent with a suspected TIA, then 300mg aspirin should be started immediately and the patient should be referred for specialist assessment and investigations (ideally within 24 hours of symptoms-onset) according to local services and pathways.
New sudden onset headache suggestive of a subarachnoid haemorrhage. Sometimes referred to as a ‘thunderclap headache,’ particularly if this is the first occurrence, the patient should be referred immediately to the nearest appropriate Emergency Department (ED) for investigation and management, especially given that the sensitivity of the required investigations is time-dependent.
Headache suggestive of Giant Cell Arteritis (GCA). Additional symptoms, such as scalp tenderness, jaw claudication, visual loss or diplopia, evolving over days should alert to the possibility of GCA and a same-day referral to the appropriate local service should be made for urgent investigation and the commencement of steroids.
Prolonged seizures suggestive of status epilepticus or when the recovery following a seizure is incomplete.
Rapid change in behaviour or level of consciousness in the presence of a fever or other systemic involvement.
Rapid limb weakness occurring over hours or days, with or without sensory loss, bladder involvement or bulbar symptoms. Such a presentation could be due to spinal pathology (eg, cord compression or intrinsic spinal lesion) or an acute neuromuscular emergency (eg, Guillain-Barre Syndrome or myasthenia gravis). Additionally, the presence of respiratory symptoms or signs should alert the GP to the possibility of impending acute ventilatory failure that requires immediate specialist assessment and support. Refer to ED or the appropriate acute service, depending on local arrangements.
Rapidly evolving symptoms or signs compatible with a new diagnosis of MS also warrant same-day referral (or should at least trigger a discussion with a neurologist on the same day).

Clinical features supporting an urgent two-week wait referral

Urgent two-week wait pathways in neurology are principally aimed at identifying brain malignancies. They have the lowest diagnostic yield for adults compared to other specialties, detecting brain tumours in 0.9% of patients referred compared to an average of 6.6% in other cancer pathways.⁴ This reflects the heterogeneity in which brain malignancies present and the relatively low prevalence of brain tumours in the population at large.

However, the presence of the following clinical features would support a referral for an urgent neurological assessment via the two-week cancer pathway:

Signs suggestive of raised intracranial pressure. These include a worsening new-onset headache in the presence of papilloedema, with or without focal neurological signs or impaired consciousness.
New onset seizure or seizures.
Rapid onset cognitive decline, behavioural change or increasing drowsiness.
Rapidly progressive unexplained focal neurological symptoms or weakness in the presence of abnormal clinical signs on examination.

Separately from cancer, patients displaying symptoms and signs suggestive of rapidly-progressive neurodegenerative conditions such as motor neurone disease (MND) should be referred for an urgent assessment by a neurologist. Useful information regarding MND, including a ‘Red Flag tool,’ developed in conjunction with the RCGP that highlights the relevant clinical features, can be found at the Motor Neurone Disease Association’s Information for GPs.

When to make routine referrals

Routine referrals for outpatient neurology assessments are appropriate for situations where A&G is unsuitable and urgent or two-week-wait referrals are unnecessary.

These situations include:

Undiagnosed movement disorders. This includes suspected Parkinson’s disease or an undiagnosed tremor which require an assessment in the neurology outpatient clinic to make a diagnosis.
Altered gait.
Suspected neuromuscular weakness (focal or generalised).

Routine referrals are also appropriate where there is uncertainty regarding the underlying diagnosis, or when the options for the management of a chronic neurological condition in primary care have been exhausted.

That said, if the clinical features are changing quickly or unexpectedly while waiting for an outpatient appointment, the neurologist should be contacted again via the A&G pathway or any other locally agreed arrangements.

Dr Rhys C Roberts is Consultant Neurologist at Cambridge University Hospitals NHS Foundation Trust