by Anastasia Zekeridou, M. Tschirren, A. Fumeaux, Patrik Michel
A 71-year-old man presented with a sudden onset paresis of his left side. On clinical examination a pure left hemiparesis with mild dysarthria and with Babinski sign was found. The signs were fluctuating with an NIHSS score (National Institute of Health Stroke Score) varying between 3 and 8 points. Non-contrast CT was normal, and the patient received thrombolysis with rtPA 95 minutes after symptom onset. The patient had no history of conventional risk factors. He reported no headache, but an involuntary weight loss of 4kg in the last 3 months. On admission, moderate hypertension and a new hyperlipidemia were found. The clinical hypothesis was that of a lacunar warning syndrome.
On the admission CT-angiography, cervical arteries were found to be irregular (figure 1: reconstruction of the right vertebral artery) with discrete vessel wall enhancement after iodine injection, mainly on the vertebral arteries (figures 2a and 2b) and common carotid arteries. The MRI confirmed right lenticular lacunar stroke (figure 3: DWI MRI), but also confirmed the presence of wall thickening of extracranial arteries as seen in figures 4 and 5 (ophthalmic and temporal artery, respectively). The ESR was 20mm/h and the CRP 30mg/l (normal: <2) and the rest of the work-up for systemic vasculitis, eye fundoscopy and lumbar puncture were normal.
As a giant cell arteritis was suspected on radiological basis, a right temporal artery biopsy was performed that showed inflammation signs with the presence of giant cells and the diagnosis was confirmed (figure 6). The patient was given Prednisone treatment (60mg/day) with a normalisation of the CRP and ESR values.
Comment by the authors
This patient illustrates the rare situation of cerebral stroke related to Horton’s arteritis. In our patient, the small lacunar-like stroke could be due to participation of a small vessel to the vasculitis, or from an embolus from the inflamed carotid artery. This patient was lucky that the diagnosis of Horton’s arteritis was made despite near complete absence of typical symptoms, signs, and elevated ESR. This was due to the regular use and careful analysis of cervico-cerebral CT-angiography in our stroke patients already in the emergency room. Unfortunately diagnostic accuracy of CTA and MRA for Horton’s arteritis is not yet sufficiently known (1). Although the main burden of radiological vasculitis in our patient was in the vertebral arteries, as frequently described (2), his stroke occurred in the deep anterior circulation, suggesting a classical lacunar (or “capsular” warning syndrome).
1. Prieto-González S et al Large vessel involvement in biopsy-proven giant cell arteritis: prospective study in 40 newly diagnosed patients using CT angiography. Ann Rheum Dis. 2012
2. Cid et al, The spectrum of vascular involvement in giant-cell arteritis: clinical consequences of detrimental vascular remodelling at different sites. APMIS Suppl. 2009 Jun;(127):10-20
Anastasia Zekederiou, M. Tschirren, A. Fumeaux and Patrik Michel work at the Neurology Service, Department of Clinical Neurosciences in Lausanne, Switzerland.
Comment by Gian Luigi Lenzi
The neurological case “Lacunar warning syndrome…” described by Zekeridou et al is an interesting hypothesis coupling an ischemic stroke as the final effect and Horton’s arteritis as its (possible) pathogenetic cause. Two facts are crystal clear: the patient had an ischemic stroke with a clinical presentation that is not easy to reconcile with a single small lesion in the right lenticular nucleus; the patient had a paucisymptomatic Horton’s arteritis.
I would like to make a few general comments as well as posing a few questions to the AA:
The definition of this stroke moves from the “lacunar warning syndrome” in the title, to a “right lenticular lacunar stroke” or a “capsular warning syndrome” in the text.
So, we have: a) a lacunar syndrome; b) a capsular warning syndrome.
Is it lacunar?
Is it a warning syndrome?
About the lacunar syndrome, we have to remind the definition of lacunar stroke: an infarct 10-15mm in size, caused by the occlusion of a single perforating artery [if we use pathology as the main reference point] ; or one of the 23 different clinical syndromes described by M. Fisher [if we remain on clinical backgrounds]. The MR findings in this patient indicate a lacunar stroke and this lacunar stroke may well explain the clinical presentation of a left hemiparesis. However the mild dysarthria is left without an MR corrispective. The coupling of a left hemiparesis with dysarthria is not a “classical” lacunar syndrome. However M. Fisher in 1991 wrote “…MR has made dependence on clinical detail more or less obsolete”. And in 2002 J. Bamford summarised that “… up to 80 per cent of lacunes (or radiological small, deep infarcts) are clinically < silent >…” However, P. Rothwell in: Brain’s Diseases of the Nervous System, XII Ed, 2008, pg 1047, points out that “Lacunar syndromes are defined clinically “. Therefore it looks like we have in general a paradoxical situation: a lacunar syndrome is a clinical definition of a particular clinical presentation of a lacunar stroke that is due to a lacuna that in 80% of the cases is without a clinical presentation. The particularity of this case is the lacunar-plus-clinical presentation. The origin of this “plus” is not explained.
The second point refers to the original definition of the capsular warning syndrome by GA Donnan and coll., who described the occurrence of lacunar TIAs as prodromic to the final infarct in a crescendo flurry pattern. All this is not present in the patient presented by Zekeridou et al, who had no warning at all. Furthermore, assuming that the clinical presentation was a “warning” in the meaning of Donnan et al., it could be argued that it was a “warning TIA”, casting some concerns on the appropriateness of the thrombolytic therapy. That is, it could be more appropriate to delete the “warning” from the label given to the stroke of this patient.
a) Could the mild dysarthria have been due to a TIA in a different region, without MR counterpart?
b) How was the evolution of the clinical presentation and of the MR findings?
c) Since Horton arteritis is a “large arteries” disease, are there reports on giant cells presence in the walls of cerebral small arteries?
d) Which kind of therapeutical prescriptions were made? It appears that the AA prescribed prednisone; that is they treated the temporal arteritis only. No antiaggregants?
Gian Luigi Lenzi is Professor of Neurology at University “La Sapienza” in Rome, Italy; EFNS Vice President and Editor-in-chief of Neuropenews.
This case isn’t a typical case of temporal arteritis at all. I think the treatment with prednisolone in this time wasn’t necessary because neither we have visual loss or satroke in progress. There are many reports that prove CRP could increas during acute stroke. No headach, no high ESR and no visual loss and no large arteries related cerebral infarctions. The only finding in favour of temporal arteritis are pathological findings. Is that enouph for definite diagnosis?
Three of the following five criteria were required to meet American College of Rheumatology (ACR) classification criteria for giant-cell arteritis:
1) Age 50 years or older,
2) New-onset localized headache,
3) Temporal artery tenderness or decreased temporal artery pulse,
4) Erythrocyte sedimentation rate of at least 50 mm/h
5) Abnormal artery biopsy specimen characterized by mononuclear infiltration or granulomatous inflammation.
Do we need a new criteria for “probable giant cell arteritis” or “pathologically defined giant cell arteritis”?
Finally I prefer closed follow up of the patient and begining of steroid if other clinical or laboratory (including ESR) findings support the giant cell arteritis diagnosis.