The role of emergency neurology in Italy: outcome of a consensus meeting for a intersociety position

Most neurological conditions have an acute onset or present as emergency situations requiring urgent intervention. Consequently, neurological diseases account for a considerable share of the medical emergencies dealt with in any hospital setting [1]. Furthermore, neurological emergencies are often severe conditions associated with high mortality and high costs, the latter not only generated by the emergency care provided in the acute phase, but also resulting from their frequent disabling outcomes. Thirty-day mortality is as high as 50% in cases of intraparenchymal haemorrhage, 50% in subarachnoid haemorrhage, 29% in cranial trauma and 20% in status epilepticus [2]. These conditions are frequent in the general population and the treatment provided within the first hours of the acute event may be crucial for patient survival and functional outcome. Other diseases (e.g. acute spinal cord injuries), though less frequent, are associated with similar high mortality, morbidity and costs.

Whereas there is a large amount of epidemiological and clinical data on the specific problems associated with single neurological disorders, there is a lack of updated, large-scale data sets describing the hospital management of neurological emergencies. In 2004, the National Institute of Neurological Disorders and Stroke (NINDS) in the US proposed the creation of a multicentre network focusing on neurological emergencies as a whole, rather than on specific neurological disorders, aimed at creating opportunities for clinical research in this area, and thus to improve acute neurological care and patient outcomes [3].

There is no doubt that the frequency and impact of neurologist assessments in the emergency room (ER) are still greatly underestimated, and not only in Italy [4, 5]. In the UK, around one patient in 10 attending an A&E department has a neurological problem [6], neurological disorders account for 10–20% of acute hospital admissions, and 10% of adults consult their GP at least once a year in connection with neurological symptoms, even though less than 10% of these patients are then referred to a hospital [7, 8]. Furthermore, again in the UK, patients admitted to hospital with acute neurological problems are rarely seen by a consultant neurologist [9]. In USA the figure of “neurohospitalist” (neurology hospitalist specialized in the care of patients admitted to the hospital) is emerging since the increased complexity of inpatient neurology care [10‐12].

According to the Italian Health Ministry data, neurological disorders, i.e., those falling into the Major Diagnostic Category 1 (MDC 1), accounted for 7.6% of all hospital admissions in Italy in 2003 (639,000 out of a total of 8,433,471), a figure basically in line with the 7.4% (731,008 out of 9,875,106) recorded in 2000. The Disease-Related Groups (DRGs) covering non-surgical neurological diseases, stroke (DRG 14), transient ischaemic attack (DRG 15) and “seizures and headache” (DRGs 24–26) by themselves account for 42.3% of patients discharged from hospital with a neurological diagnosis, a proportion that rises to 49.8% if non-surgical head traumas are added (DRGs 27–30).

Moreover, many patients with acute conditions that fall into non-neurological MDCs—such as syncope/collapse, balance disorders (44,217 discharges in 2003), and several psycho-organic syndromes—receive neurological treatment in the ER, or are admitted to specialized neurology units. It is also worth pointing out, again on the basis of Italian Ministry of Health data for 2003 [13], that even though acute cerebrovascular diseases ranks eighth in the top 50 DRGs classified by number of discharges (135,012 discharges) and acute myocardial infarction ranks fourteenth (114,632 discharges), the latter receives far more attention (in terms of dedicated beds, technologies and funding) than does stroke.

If the annual number of hospital admissions for acute neurological conditions is high, the number of patients seen in ER settings for neurological or presumed neurological problems will obviously be even higher, given that only a limited proportion of these cases are actually admitted to hospital. The demand for emergency neurological assessments is actually very high. As well as for life-threatening conditions, such as stroke, status epilepticus and encephalitis, neurological assessment is also often sought for clinical pictures that, while requiring careful evaluation, do not usually have severe prognostic implications (e.g., vertiginous symptoms or syncope). Neurological assessments are thus fraught with responsibility and carry considerable litigation risks [14, 15] as, for instance, it is indicated by the high incidence of ER visits for headache, a notoriously “insidious” symptom [16, 17], for minor head injury [18] especially in hospitals without a neurosurgery unit, and for “transient loss of consciousness” [19], etc. Stroke is undoubtedly the most important neurological emergency, in terms both of frequency [4, 5, 20] and of the need, shown by evidence-based medical data [21, 22], for prompt intervention with diagnostic-treatment protocols designed to reduce mortality and improve outcome.

Whereas, in the past, neurology was not generally considered a discipline connected with emergency situations, the situation is now very different: with greater understanding of the pathophysiology of acute neurological conditions, advances in the field of diagnostic imaging and the availability of new treatment options [23] the diagnostic-therapeutic approach to neurological emergencies has now changed completely and the neurologist is emerging as an increasingly important figure in emergency departments [16]. Yet, even though neurology units frequently admit patients with acute problems requiring high-quality care, neurology continues to be classed as a medium care specialisation. Furthermore, these units often do not have sufficient medical staff to provide round-the-clock care. Evidence shows that the neurologist can make a fundamental contribution within the emergency department, changing the initial diagnosis in a high proportion (up to 52.5%) of cases [4], contributing to the clinical evaluation and therapeutic planning of patients and adequately select hospital admissions and providing pathways of care more efficient and less expensive in terms of instrumental investigations and consultations. According to data from the NEU study [24], which evaluated the work of Italian hospital neurology units in the emergency setting, the main cause of neurological consultations in the ER is acute cerebrovascular disease, followed by headache, vertigo, head trauma, acute impairment of consciousness, and epilepsy (Fig. 1).

Acute cerebrovascular disease is the clinical condition most frequently requiring neurological attention in the ER, accounting for 27% of all emergency neurological consultations. Prompt “recognition” of the clinical symptoms of cerebrovascular diseases, early diagnosis of the site and nature of the injury, and rapid therapeutic decision-making, particularly as regards the decision to perform thrombolysis in ischaemic stroke, will influence the patient’s clinical course and outcome.

Between 1.2 and 4.5% of all adults seeking emergency care do so because of headache [11]. Of these, between 4.3 and 6.4% are affected by secondary headaches [25]. The NEU data show that, in Italy, 23% of neurological consultations in the ER are for headache. Subarachnoid haemorrhage is a rare condition, occurring in less than one in 1,000 patients arriving at the ER with headache [26]; prompt diagnosis of the condition is crucial, given its severe prognosis. In a large, prospective observational study of 455 patients arriving at an emergency department with headache, 107 had a severe acute-onset form and, of these, 19% had a subarachnoid haemorrhage; this was diagnosed by cranial CT scan in 18 patients and by lumbar puncture in the two who had a negative CT scan [27]. Obviously, the expertise of the consultant neurologist working in the ER is fundamental in order to ensure a correct clinical-anamnestic approach and thus a timely differential diagnosis between primary and secondary (e.g. meningoencephalitic, neoplastic or acute vascular) headache forms. In a Spanish study, the percentage of secondary headaches diagnosed thanks to the presence of a consultant neurologist in the ER was very high (13.4% of the evaluations requested); furthermore, the presence of a neurologist was found to halve the number of hospital admissions for headache [28].

The epileptic seizure is another acute neurological condition demanding prompt and expert diagnostic-therapeutic assessment in the ER. Status epilepticus is a non infrequent medical emergency, having an incidence of 18–28/100,000 people/year and a mortality of 5–10%. Tonic–clonic convulsions and convulsive status epilepticus are generally easy to recognise; conversely, the non-convulsive forms can be difficult to identify, as the state of coma with general clinical deterioration and lack of overt motor manifestations can sometimes delay the diagnosis. Functional manifestations, too, characterizing pseudosyncopal episode, can require differential diagnosis.

Neurotraumatological injuries make up quite a large share of the workload of neurology units, particularly those based in hospitals where there are no neurosurgery facilities. On average, head injury is the fourth most frequent clinical problem (12%) that the consultant neurologist in the ER will be called upon to deal with, after acute cerebrovascular disease, headache, and vertigo. The absence of a neurosurgery unit on site is associated with a highly significant increase in the frequency of specialist neurological consultations for head injury in the ER.

Disturbances of consciousness are other clinical situations frequently requiring the intervention of a neurologist, and account for 12% of neurological consultations in Italian hospital ERs. Disturbances of consciousness are a clinical group embracing a wide range of different disorders, from epileptic seizures to syncope, acute intoxication, and metabolic imbalances. For this reason they need particularly skilled neurological assessment. In a Scandinavian study, 1.2% of all ER visits were for transient loss of consciousness [19].

While the impact of neurological disorders in the emergency setting has thus been illustrated, the advantage, in the treatment of acute neurological disorders, of having a neurologist on call in the ER has not yet been adequately demonstrated.

As indicated earlier, in the study conducted by Moulin et al. [4], a high percentage of incorrect diagnoses (37.3% false positives and 36.6% false negatives) are recorded in ERs where no neurologist is on call. Furthermore, in 52% of cases subsequent neurological assessment completely changed the initial diagnosis.

Similar results were reported in an Irish study, in which neurological referral resulted in a significant change in diagnosis in 55% of patients and in management in nearly 70%; in 65% it also facilitated earlier discharge [29].

In a further study, published in 2008, the initial diagnosis made by the ER physician agreed with the final diagnosis in 60.4% of cases (298/493); instead, it disagreed or was uncertain in 35.7% (19.1 and 16.6%, respectively) [30].