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Cerebral amyloid angiopathy: amyloid spells and cortical superficial siderosis Rebecca Coates,1 Simon M Bell,1 Stuart Coley,2 Daniel J Blackburn1,3 ▸ Additional material is published online only. To view please visit the journal online (http://dx.doi.org/10.1136/ practneurol-2014-000952). 1

Department of Neurology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK 2 Department of NeuroRadiology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK 3 Department of Neurology, Sheffield Institute of Translation Neuroscience (SITraN), University of Sheffield, Sheffield, UK Correspondence to Dr Simon M Bell, Department of Neurology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Glossop Road, Sheffield S10 2JF, UK; [email protected] Accepted 13 October 2014

To cite: Coates R, Bell SM, Coley S, et al. Pract Neurol Published Online First: [ please include Day Month Year] doi:10.1136/practneurol2014-000952

CASE REPORT A 66-year-old man presented to the emergency department with paraesthesia starting in the first two fingers of his right hand, spreading up his right arm into the right side of his face and mouth over 10 min. During this, his mouth felt numb “like after a dental injection”. He had three further similar episodes in the emergency department. He had a past history of hypothyroidism and irritable bowel syndrome. After a CT scan of head (figure 1), he started treatment with an antiplatelet medication for a presumed transient ischaemic attack (TIA). An MR scan of brain the next day showed a small amount of subarachnoid blood in the left central (rolandic) sulcus (figure 2). We therefore stopped the antiplatelet treatment. Over the next month, he continued having almost daily episodes of tingling and numbness affecting his hand and face, each lasting 20 min. One episode affected his throat, leaving him unable to speak for several minutes (figure 3 and supplementary video on the website). He also had a frequent ‘muzzy’ headache. A second MR scan of brain with gradient echo sequences showed several areas of superficial haemosiderin staining in both central sulci but with no cortical microbleeds or haemorrhages (figure 4). These findings are consistent with the Boston criteria for possible cerebral amyloid angiopathy,1 which was our unifying diagnosis. DISCUSSION This patient’s MR scan of brain shows cortical superficial siderosis caused by haemosiderin deposition in the subarachnoid space around the cortical cerebral convexities. This imaging finding is increasingly recognised in patients with cerebral amyloid angiopathy. In this condition, there is deposition of β-amyloid protein in the leptomeningeal and cortical vessels, causing vessel wall damage,

and leading to multifocal subarachnoid or intracerebral bleeding. Cortical superficial siderosis suggests a higher risk of intracerebral haemorrhage.1 2 About 20% of patients with cerebral amyloid angiopathy develop transient focal neurological events or ‘amyloid spells’; these are especially likely if cerebral imaging shows cortical superficial siderosis.2 3 Amyloid spells typically include both positive and negative symptoms and last up to 30 min. The positive symptoms probably result from cortical spreading depression because of the irritant effect of haemosiderin.4 The negative symptoms probably result from cerebral vessel occlusion and spasm following amyloid deposition.5 Negative symptoms are as common as positive ones2: thus, all patients describing atypical recurrent focal episodes— regardless of their exact nature—should have both blood-sensitive imaging and diffusion-weighted imaging. In younger patients with isolated superficial siderosis, doctors should consider reversible vasoconstriction syndrome. In the accompanying video (figure 3 and supplementary video on the website), the patient describes transient focal neurological symptoms that are consistent with previous descriptions of amyloid spells. The slow spread from hand to face probably reflects cortical representation because cortical superficial siderosis typically develops in the rolandic fissure and central sulcus, which overlie the hand and face cortical sensory areas. Amyloid spells are frequently mistaken for seizures, migraines or TIAs,1 often delaying or missing the diagnosis of cerebral amyloid angiopathy. The role of EEG in the work-up for amyloid spells is not clear; we made a confident diagnosis based on clinical history and neuroimaging and did not perform an EEG. Some reports suggest that antiepileptic medication and migraine preventatives

Coates R, et al. Pract Neurol 2014;0:1–3. doi:10.1136/practneurol-2014-000952

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Figure 3 Still image from a video showing the patient’s description of an ‘amyloid spell’. The video can be viewed in the supplementary material section of the Practical Neurology website.

Figure 1 Non-enhanced CT brain shows high attenuation acute subarachnoid blood within the left central sulcus.

may stop amyloid spells. Roch et al,3 in a small study, showed that four out of six patients with amyloid spells responded to antiepileptics. The other two patients improved after stopping antiplatelet therapy alone. Our patient’s symptoms resolved spontaneously, without antiepileptics or migraine

Figure 2 Fluid-attenuated inversion recovery (FLAIR) MRI sequences performed the following day confirms the presence of high signal acute blood at the same site.

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preventatives, coinciding with blood pressure control. Prompt and appropriate management of hypertension is important in this patient population to reduce the later risk of intracerebral haemorrhage4 The headaches, muzziness and ‘flashing lights’ raised the possibility of a ‘late life migraine accompaniment’—as described by Miller Fisher5—as the cause of a TIA. Patients presenting with late life onset of migraine, with or without aura, should also undergo MR imaging with blood-sensitive sequences.

Figure 4 Gradient echo MR imaging shows the acute subarachnoid blood plus mature haemosiderin staining of the left parietal lobe and contralateral hemisphere, suggesting previous subarachnoid bleeding.

Coates R, et al. Pract Neurol 2014;0:1–3. doi:10.1136/practneurol-2014-000952

IMAGE OF THE MOMENT Contributors RC helped in writing the article and interviewing the patient; SMB is the interviewer on the attached video and also was involved in writing the article and interviewing the patient; SC developed the radiological images in the publications and wrote image leger lines; DJB was the supervisor of the project. He was involved in writing the article and interviewing the patients. Funding The video for this article was recorded as part of independent research funded by the National Institute for Health Research (NIHR) under its Research for Patient Benefit Programme (Grant Reference Number PB-PG-0211-24079). The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by David Werring, London, UK.

Coates R, et al. Pract Neurol 2014;0:1–3. doi:10.1136/practneurol-2014-000952

REFERENCES 1 Charidimou A, Baron JC, Werring DJ. Transient focal neurological episodes, cerebral amyloid angiopathy, and intracerebral hemorrhage risk: looking beyond TIAs. Int J Stroke 2013;8:105–8. 2 Charidimou A, Peeters A, Fox Z, et al. Spectrum of transient focal neurological episodes in cerebral amyloid angiopathy: multicentre magnetic resonance imaging cohort study and meta-analysis. Stroke 2012;43:2324–30. 3 Roch JA, Nighoghossian N, Hermier M, et al. Transient neurologic symptoms related to cerebral amyloid angiopathy: usefulness of T2*-weighted imaging. Cerebrovasc Dis 2005;20:412–14. 4 Arima H, Chalmers J. PROGRESS: prevention of recurrent stroke. J Clin Hypertens (Greenwich) 2011;13:693–702. 5 Fisher CM. Late-life migraine accompaniments as a cause of unexplained transient ischemic attacks. Can J Neurol Sci 1980;7:9–17.

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Cerebral amyloid angiopathy: amyloid spells and cortical superficial siderosis.

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