Elsevier

The Lancet Neurology

Volume 10, Issue 6, June 2011, Pages 550-560
The Lancet Neurology

Review
Atypical presentations of acute cerebrovascular syndromes

https://doi.org/10.1016/S1474-4422(11)70069-2Get rights and content

Summary

Correct diagnosis of acute stroke is of paramount importance to clinicians to enable selection of correct treatments and to ensure prevention of acute complications, including recurrent stroke. Timely diagnosis can be difficult in some cases because patients with acute stroke can present with atypical or uncommon symptoms that suggest another cause altogether. Publications on these patients suggest that the following strategies could help to reduce misdiagnosis. First, clinicians should suspect stroke in any patient with abrupt onset of neurological symptoms. Second, clinicians should be aware that some patients will initially present with various uncommon and atypical stroke symptoms. Third, a complete and systematic neurological examination should be routinely done in patients presenting with acute neurological symptoms because this might shed light on the true nature of the problem. Finally, clinicians should be aware that even with the most sophisticated neuroimaging tests, stroke might be missed in the early hours after the event.

Introduction

Worldwide, about 15 million people have a stroke every year, of whom 5 million die and 5 million have a permanent deficit.1 In high-income countries, stroke is the most common cause of disability and is the third most common cause of death. When patients present with acute neurological symptoms, physicians must distinguish between stroke and other causes that mimic it. Accurate and prompt diagnosis is crucial because implementation of time-dependent therapies, identification and treatment of the underlying vascular mechanism, and attention to the underlying risk factors can improve outcomes and prevent subsequent events.

Patients who present with acute neurological symptoms can be divided into four groups (table). The first group includes patients with obvious stroke—eg, an elderly individual with untreated atrial fibrillation who abruptly develops aphasia and hemiparesis. The second group includes patients whose cause of neurological dysfunction is clearly not stroke, such as a patient without vascular risk factors who develops unilateral weakness of the facial muscles in association with hyperacusis and altered taste consistent with a lower motor neuron facial nerve palsy. In the third group, patients seem to have a stroke, but in fact have a non-vascular cause such as conversion reactions, Todd's paralysis (a disorder characterised by a brief period of paralysis with or without aphasia after a seizure), or migraine. Hypoglycaemia is a particularly important stroke mimic because its treatment is simple and effective. In the era of thrombolysis for acute ischaemic stroke, these stroke mimics have received much attention.2, 3, 4, 5 The final group of patients includes those with actual strokes, but whose presentations are unusual or atypical, suggesting a non-vascular cause. Huff has referred to these patients as “stroke chameleons”.2 Compared with stroke mimics, this group of patients with missed or delayed diagnosis of stroke has received much less attention and is the focus of this Review.

Patients with stroke can present with atypical symptoms for various reasons. First, in the first minutes to hours after the event, all the diagnostic information might not be available to the initial health-care providers. Additionally, patients' symptoms can evolve with time. Second, there is substantial variability in the classic cerebrovascular territories that can also result in non-classic presentations. Patients with small strokes, early presentations, young age, posterior circulation location, and deficits that do not result in lateralising motor or speech findings might be more difficult to diagnose clinically.6, 7, 8, 9

In this Review, we aim to help clinicians improve the accuracy and timeliness of their diagnosis of patients with acute stroke by reviewing causes of misdiagnosis and non-classic symptoms of acute stroke (panel 1). Although much of the focus is on acute ischaemic stroke, we also discuss some aspects of misdiagnoses of haemorrhagic strokes, including intracerebral haemorrhage (ICH), subarachnoid haemorrhage (SAH), and haemorrhage resulting from cerebral venous sinus thrombosis (CVST). Panel 2 provides a glossary of terms of atypical symptoms of stroke.

Section snippets

Non-localising symptoms

Stroke is usually characterised by the sudden onset of focal neurological deficits, such as hemiparesis, aphasia, or hemianopia, depending on the affected brain structures and vascular territory. However, some strokes can present in a non-localising manner without clear-cut focal deficits.

Abnormal movements or seizures

Stroke is usually characterised by loss of movement. However, in a small percentage of cases, patients can have various abnormal movements (hyperkinetic, hypokinetic, or seizure-like) at stroke onset.

Peripheral nervous system symptoms

Although stroke is a CNS event, some cases present in ways that suggest a peripheral nervous system (PNS) cause.

Atypical symptoms

Rarely, patients with stroke present with atypical and unusual symptoms and signs. Increased awareness of these unusual presentations facilitates early recognition, minimises unnecessary tests, and facilitates prompt treatment.

Isolated headache

Another stroke presentation, in both ischaemic and haemorrhagic disorders, is the presence of a prominent headache that is either isolated or associated with non-specific symptoms that are not obviously attributable to a cerebrovascular cause.

Isolated headache can occur with arterial dissections, CVST, and SAH.91, 92, 93 Although headache suggests ICH, patients with acute ischaemic stroke can also present with prominent headache.94 Headache at onset (with or without concomitant dizziness,

Limitations of brain imaging

Although stroke is a clinical diagnosis, nearly all patients undergo brain imaging. The purpose of brain imaging in patients with acute neurological symptoms includes establishing a stroke diagnosis and excluding stroke mimics. CT scanning can establish the diagnosis of stroke (ICH, SAH, and occasionally acute ischaemic stroke) and exclude stroke mimics (in patients with acute ischaemic stroke who are candidates for thrombolysis). MRI is being increasingly used to definitively confirm a

Conclusions

The topics covered in this Review might suggest that almost any neurological symptom could be indicative of a stroke; what practical conclusions can the clinician derive from this? By design, we assessed many case reports and case series, which tend to overestimate the atypical manifestations of stroke, but understanding this variability of presentation is key.

In various systematic studies, acute speech and lateralising motor findings consistently correlate with stroke diagnosis.6, 106 In these

Search strategy and selection criteria

We identified articles for this Review through searches of PubMed using the search terms “stroke”, “misdiagnosis”, and “atypical presentations” from 1980 to March, 2011. Additional articles were identified from the bibliographies of the initial articles and from the authors' own files. With one exception, we reviewed articles only published in English. The final reference list was generated on the basis of originality and relevance to the broad scope of this Review; therefore, we included

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