Vassilios Zis

Common carotid arterial stiffness and the risk of ischaemic stroke

In the present case–control study we aimed to investigate the association of common carotid arterial (CCA) stiffness with ischaemic stroke (IS) and to determine whether this relationship was independent of conventional risk factors including CCA intima‐media thickness (CCA‐IMT). CCA distensibility, defined as the change of CCA‐diameter during the cardiac cycle, and CCA‐IMT were evaluated by means of high‐resolution B‐mode carotid ultrasound examination in consecutive, first‐ever IS patients (n = 193) and in age‐ and sex‐matched control subjects (n = 106). The CCA distensibility (inverse of CCA stiffness) was significantly (P = 0.007) lower in IS (0.353 mm, 95% CI: 0.326–0.379) than in control subjects (0.415 mm, 95% CI: 0.378–0.451) even after adjusting for blood pressure values, diastolic CCA‐diameter and height. The multivariate logistic regression procedure selected CCA‐IMT and CCA distensibility as the only independent predictor variables of IS. Each 1 SD increase in the CCA‐IMT and each 1 SD decrease in the CCA distensibility independently increased the likelihood of IS by 167.0% (OR: 2.67, 95% CI: 1.80–3.96, P < 0.001) and 59.0% (OR: 1.59, 95% CI: 1.22–2.07, P = 0.001) respectively. Increased CCA stiffness is associated with IS independent of conventional risk factors and CCA‐IMT. The causal interrelationship between the elastic properties of the CCA and the risk of stroke deserves further investigation by longitudinal studies.

Aetiopathogenesis and long-term outcome of isolated pontine infarcts

Background and purposeIsolated pontine strokes cause characteristic neurological syndromes and have a good short-term prognosis. The aim of this study was to examine the long-term survival, cumulative recurrence rate and clinical handicap of patients with isolated pontine infarcts of different aetiology.MethodsOne hundred consecutive patients with an isolated pontine infarction were identified by imaging studies and evaluated prospectively. After extensive study, cases were classified according to the aetiopathogenetic mechanisms: stroke due to basilar artery branch disease (BABD), small-artery disease (SAD) and large-artery-occlusive disease (LAOD). During a mean follow-up period of 46 months, stroke presentation and initial course, early and long-term mortality, disability and recurrence were evaluated.ResultsBABD was the most frequent cause of isolated pontine ischaemia (43 %), followed by SAD (34%) and LAOD (21%). Hypertension was the most prominent risk factor, especially among patients with SAD (94.1 %). Neurological impairment on admission was more severe in the LAOD group, followed by BABD. After 1 month patients with LAOD had the highest cumulative mortality (14.3%, p=0.026) and more severe disability (61.1%, p=0.001). Five-year mortality rate was 20.6%, 14% and 23.8% in the SAD-, BABD- and in LAOD-group respectively (p=0.776). Cumulative 5-year recurrence rate was 2.3 % for BABD, 14.3 % for LAOD, and 29.4 % for SAD (p=0.011).ConclusionsOverall long-term survival of patients with isolated pontine infarcts is good. Initial differences regarding short-term outcome in infarctions of different aetiology resolve with time. Effective secondary prevention among SAD patients may limit stroke recurrence and positively influence long-term prognosis.

Two-Peak Temporal Distribution of Stroke Onset in Greek Patients

A circadian distribution with a single peak of stroke onset in the morning hours has been described in various countries. Analysis of the temporal pattern of symptom onset in a series of 1,253 Greek first-ever stroke patients revealed a circadian distribution with two incidence peaks between 08.00 and 10.00 h, and between 16.00 and 18.00 h. Considering the different etiopathogenic types of stroke, the same two-peak time pattern was evaluated in all stroke subgroups with the only exception of lacunar stroke, which seems to predominantly occur during night and sleep. A secondary stroke onset peak in the late afternoon has not been previously described in such detail. A relation between the second, however lower, frequency peak and the traditional Greek habit of afternoon sleep (siesta) could be assumed and deserves further investigation.

Incidence of rotational vertigo in supratentorial stroke: a prospective analysis of 112 consecutive patients.

BACKGROUND Single cases with hemispheric, cortical or subcortical, ischemic lesions presenting with rotational vertigo (RV), that challenge the notion of infratentorial or peripheral generation of RV have been published, but the incidence of this symptom in a larger series is unknown. The aim of this study was to investigate whether acute hemispheric cerebrovascular lesions cause vertiginous sensations with particular emphasis on RV. METHODS A total of 112 consecutive stroke patients were assessed in a prospective single-center study over a 22-month inclusion period. Rotational or other vertiginous sensations were assessed using a structured 5-item questionnaire and patients with vertigo were further evaluated with Yardleys Vertigo Symptom Scale. All subjects underwent standard clinical neuro-ophthalmological and neuro-otological testing and data were correlated to imaging findings. RESULTS RV was absent among our patients. Few subjects reported non-rotational vertiginous sensations with stroke onset. These were mainly right-hemispheric strokes with concomitant subcortical leukoaraiosis. CONCLUSION In this case series we did not find any patients with spinning sensations which is supportive of the dogma that supratenotrial lesions do not cause RV. Certain hemispheric stroke patterns, however, may be related to non-rotational dizziness.

Moyamoya syndrome in a Caucasian woman with Turner's syndrome

Moyamoya disease is a rare cerebral arteriopathy of unknown etiology characterized by bilateral obliteration of the internal carotid artery and its branches, with the concomitant development of an abnormal basal meshwork of collateral vessels. Moyamoya syndrome shows a similar angiographic pattern but is usually associated with different diseases and risk factors [1]. Turner’s syndrome (TS) is one of the most common chromosomal anomalies and has been related with different arteriopathies [2,3]. A 47-year-old Greek woman of small stature with a history of hypertension, cataract, and primary amenorrhea presented because of an over 6 days progressing left side hemiparesis with severe hemineglect. Magnetic resonance imaging revealed multiple ischemic lesions of the right hemisphere affecting both the cortical and subcortical structures (Fig. 1a). Magnetic resonance angiography (MRA) demonstrated hypoplastic common carotid arteries and intracranial obstruction of both internal carotid arteries. Both hemispheres were supplied from the vertebrobasilar system through leptomeningeal collaterals (Fig. 1b). These imaging findings are typical for Moyamoya. Because of the adult onset of the disease and the coexistence of risk factors, this case should be rather classified as Moyamoya syndrome. Because of the short stature and the dysgonadal features, further chromosomal examination was conducted. The 46,X,i(Xq) karyotype established the diagnosis of TS. A literature review revealed only a single case of a Japanese girl with TS [karyotype: 45,X/46,X,i(Xq) associated with Moyamoya disease [4]. Congenital malformations of the heart and the major vessels are frequently seen among the patients with TS [5]. Fibromuscular dysplasia affecting the cerebral and renal arteries has also been associated with TS [2]. It is additionally regarded as possible cause of Moyamoya syndrome [1]. Moreover, several cases of Japanese patients with TS suffering stroke because of unilateral or bilateral occlusion, stenosis or dissection of the internal carotid artery have been described [6,7]. According to the angiographic findings, in some of these cases, a Stage 1 Moyamoya cannot be definitely excluded [1]. Considering the pathogenesis of Moyamoya, both the familial occurrence and the higher rates among Japanese patients indicate a genetic predisposition.

Common carotid artery intima–media thickness for the risk assessment of lacunar infarction versus intracerebral haemorrhage

AbstractBackground and purposeArterial hypertension is the major risk factor for intracerebral haemorrhage (ICH) and lacunar infarction (LI) and both types of cerebral lesions originate from pathology of the same deep perforating small arteries. We aimed to evaluate the relationship between vascular risk factors including common carotid artery intima–media thickness (CCA–IMT) with LI versus ICH.MethodsWe prospectively collected data from 159 firstever stroke patients (67 cases with ICH and 92 cases with LI) with documented history of hypertension. All subjects underwent B–mode ultrasonographic measurements of the CCA–IMT. Logistic regression modelling was used to determine the factors (established vascular risk factors, severity and duration of hypertension, concomitant medications and CCA–IMT) that may significantly differentiate LI from ICH.ResultsPatients with LI had significantly (p = 0.002) larger CCA–IMT values (0.926 mm, 95% CI: 0.881–0.971) than subjects with ICH (0.815 mm, 95% CI: 0.762–0.868) even after adjusting for baseline characteristics and cardiovascular medications. The multivariate logistic regression procedure selected CCA–IMT, diabetes mellitus and hypercholesterolaemia as the only independent factors able to discriminate between LI and ICH. The risk for LI versus ICH increased continuously with increasing CCA–IMT. For each increment of 0.1 mm in CCA–IMT the probability of suffering from LI versus ICH increased by 36.6% (95 % CI: 13%–65.2%, p = 0.001) even after adjustment for cardiovascular risk factors.ConclusionsIncreased CCA–IMT values are a factor favouring LI over ICH in hypertensive patients. The measurement of CCA–IMT may be a useful non–invasive diagnostic tool for the risk assessment of LI with respect to ICH in such patients.

Lack of visual evoked potential habituation in the syndrome of HaNDL

Sirs, Diagnosis of migraine implicates the exclusion of structural brain abnormalities, as well as infectious CNS diseases like meningoencephalitis. The latter can be assured by the absence of increased cell counts in cerebrospinal fluid (CSF) samples. A rare entity, however, was first described in 1981 characterized by episodes of migrainous headache in subjects without prior migraine, accompanied by transient neurologic deficits and CSF pleocytosis. Importantly, the condition was self limited in all cases [3]. The benign character of the disorder was established in two subsequent case series [5, 10], where the terms ‘‘headache with neurological deficits and CSF lymphocytosis’’ (HaNDL) and ‘‘pseudomigraine with lymphocytic pleocytosis’’, respectively, were used. The episodes of headache and the clinical deficits recur over less than 3 months. During the acute period of the disease differentiation from a viral or other form of aseptic meningitis is difficult, and in most cases, HaNDL remains a diagnosis of exclusion that is often established retrospectively. Due to the phenotypical similarities with the classical migraine with aura, some authors postulate similar pathophysiological abnormalities during the headache episodes [6, 8, 12]. Consequently, one might take advantage of certain migraine biomarkers, such as the known interictal electrophysiological changes, in order to support an earlier diagnosis of HaNDL. Indeed, there is one recent report of a female teenager suffering from HaNDL that demonstrated a lack of habituation of the P100-amplitude during sequential averaging of pattern-reversal stimuli [9]. Nevertheless, this patient, had a clear familial predisposition to migraine, since her father suffered from migraine with simple visual aura and her mother from migraine without aura. In fact, the lack of VEP-habituation persisted one year after the acute HaNDL period indicating a permanent electrophysiological trait. Unfortunately, this second, follow-up recording was not accompanied by CSF analysis. In this report we describe an adult patient with HaNDL, without prior migraine and without familial predisposition for migraine. We focus on the phenomenon of VEPpotentiation and its correlation with CSF parameters during the course of the syndrome. A 33-year-old man without personal or familial history of migraine noticed a severe left hemiparesis which involved the face, the upper and the lower limb, as well as difficulty in finding the appropriate words. These symptoms lasted approximately 25 min followed by an intense, bitemporal, throbbing headache, accompanied by vomiting and photophobia that persisted over 10 h. Physical examination on admission was unremarkable. The patient was afebrile and routine blood tests were normal. The acute brain-CT, as well as the MRI and MR-angiography on the following day showed no abnormalities. An EEG conducted 3 days after the episode exhibited a high percentage of generalized theta waves. Three similar episodes occurred in the following 15 days. CSF analysis conducted after the second episode revealed 296 cells/mm (95% lymphocytes), 113 mg/dl protein and a markedly elevated opening pressure of 350 mmH2O. Extensive testing for a variety of neurotropic viruses, as well as for borrelia burgdoriferi, syphilis and tuberculosis was negative. Routine blood tests, immunological markers and screening for thrombophilia were also normal. Furthermore, carotidE. Anagnostou (&) K. Spengos D. Naoumis G. P. Paraskevas S. Vassilopoulou V. Zis D. Vassilopoulos Department of Neurology, Eginition Hospital, University of Athens, Vas. Sophias Avenue 74, 11528 Athens, Greece e-mail: granavan@yahoo.com

Extensive corpus callosum infarction : An uncommon pattern of watershed ischaemia?

Sirs: A 60-year old man, current heavy smoker with a history of poorly controlled diabetes mellitus, hypertension and hypercholesterolaemia, but no alcohol consumption, developed hemihypaesthesia and confusion shortly after receiving 20 mg nifedipine sublingually, due to a hypertensive episode (195/ 110 mmHg before; 150/85 mmHg after medication). Detailed neurological examination on hospital admission six hours later revealed additionally an impressive disconnection syndrome with left visual and tactile extinction, anomia, left hemianopic alexia, pronounced apraxia with the left or with both hands and intermanual conflict. Computed tomography excluded haemorrhage, while two days later magnetic resonance imaging (MRI) visualized on FLAIRand T2weighted sequences signal hyperintensities affecting the entire corpus callosum, the left temporal-occipital cortex and the underlying subcortical white matter, adjacent to the posterior watershed area. Diffusion weighted imaging (DWI) and corresponding restricted apparent diffusion coefficient (ADC) maps established the subacute ischaemic nature of the lesion (Fig. 1) [8]. Digital subtraction angiography (DSA) excluded extracranial lesions and demonstrated subtotal and moderate stenosis of the proximal intracranial cavernous portion of the left and right internal carotid artery (ICA) respectively (Fig. 2), with anterograde flow in both ICAs. The left ICA territory was supplied mainly with anterograde flow through the stenotic lesion and to a lesser degree through ophthalmic collaterals from the ispilateral external carotid artery. Both anterior cerebral arteries (ACA) were supplied by the left ICA. The right A1 was not seen and was probably hypoplastic. The combination of hypoplastic right A1 with near occlusion of the ICA cavernous portion seemed to be the underlying cause of infarction. Detailed cardiological studies including transoesophageal echocardiogram and repeated Holter-electrocardiogram recordings revealed no pathological findings suggestive of cardioembolism. Infarction of the corpus callosum is uncommon due to its rich blood supply and resistance to small vessel ischaemic changes [1, 6, 10]. Extensive lesions involving the entire corpus callosum are extremely rare and have been related to bilateral ICA occlusion [5, 7], underlining the importance of haemodynamic insufficiency in such cases [3, 10]. The pericallosal branch of the ACA is the dominant vascular supply of the body of the corpus callosum, whereas other perforating ACA-branches supply rostrum and genu. The splenium is covered by the posterior pericallosal artery; a posterior cerebral artery branch [1]. The watershed area between anterior and posterior pericallosal artery is quite variable explaining the unpredictable size and location of corpus callosum infarctions. There have been few cases of extensive corpus callosum infarction reported [1, 2, 3, 5, 7, 8], two of which were in the presence of bilateral proximal ICA occlusion [5, 7]. Some authors have noted that the majority of ACA and partial corpus callosum strokes are thromboembolic in origin [1], while others attribute them mostly to local atherothrombosis [4]. We present the uncommon case of an entire corpus callosum infarction in a patient where the mainly supplying left ICA was subtotally occluded. In combination with contralateral ICA stenosis and ipsilateral A1 hypoplasia, we assume that there was hypoperfusion due to distal haemodynamic insufficiency after a drug induced abrupt blood pressure decrease as the cause of stroke and suggest that the present case of extensive corpus callosum infarction might be considered as a K. Spengos, MD, FESC (&) G. Tsivgoulis, MD Æ C. Potagas, MD V. Zis, MD Dept. of Neurology University of Athens Eginition Hospital Vas. Sofias 82 11528 Athens, Greece Tel.: +30-694/6466216 Fax: +30-210/6742604 E-Mail: spengos@hol.gr

Isolated dorsal midbrain infarct: an uncommon cause of pure sensory stroke.

a Department of Neurology, University of Athens Medical School, Eginition Hospital, and b Department of Clinical Therapeutics, University of Athens Medical School, Alexandra Hospital, Athens , Greece Case Description A 60-year-old man, with a history of hypercholesterolaemia and smoking, awakened with numbness on the right part of the inner mouth and the right arm and leg. Symptoms persisted over 3 days, when he consulted the family physician, who suspected stroke and referred him to our hospital for further evaluation. On examination the patient was alert, with normal and symmetrical tendon refl exes, while both plantar responses were fl exor. A right hemihypaesthesia involving the face, trunk, arm and leg was manifest. No ataxia, dysarthria, limb weakness, or ocular motor manifestations were present. MRI of the brain was than performed on the fourth day after ictus. A hyperintense lesion of ischaemic origin located in the left dorsal portion of the midbrain was demonstrated on fl uid attenuated inversion recovery (FLAIR, fi g. 1 a), T 2 -, as well as diffusionweighted images. The infarct was obviously affecting structures localised in the dorsal (superior colliculus, periaqueductal grey matter) and the dorsolateral (medial lemsiscus and lateral spinothalamic tract) midbrain territory, supplied by the superior cerebellar artery. MR angiographic images in the time-of-fl ight technique and ultrasound of the carotid and vertebral arteries were normal. Transthoracic echocardiography, 24-hour blood pressure monitoring and Holter electrocardiographic monitoring revealed no abnormalities. Visualand auditory-evoked potentials as well as extensive blood coagulation studies were unremarkable. The patient was diagnosed as having a brainstem lacunar stroke and was discharged on aspirin and lipid-lowering medication. A slight improvement of his sensory symptoms was reported at the follow-up evaluation 6 months later, while a new MRI revealed a substantial decrease in the extent of the hyperintense lesion on the FLAIR images ( fi g. 1 b). Introduction Occlusion of branches of the thalamogeniculate arteries supplying the thalamic somatosensory nuclei is responsible for the vast majority of strokes presenting with the clinical syndrome termed by Fisher as ‘pure sensory stroke’ (isolated hemihypaesthesia or paraesthesia involving the face, trunk, arm and leg) [1] . Occasionally, pure sensory stroke can be caused by a lateral tegmental pontine or medullary infarct [2] . Brain ischaemia limited to the mesencephalon is uncommon, accounting for 0.2–2.3% of total admitted ischaemic strokes [2–5] . The anteromedial territory, supplied by the direct perforators of the basilar artery, is most frequently affected in patients with isolated midbrain infarct, whose neurological picture is dominated by oculomotor disturbances [2–5] . We report the uncommon case of a patient presenting with the clinical syndrome of pure sensory stroke due to an infarction limited to the dorsal midbrain territory.

Hypoglycemia-induced hemichorea in a patient with Fahr’s syndrome

Non-ketotic hyperglycemia may be a cause of hemiballism-hemichorea. We present an elderly female type II diabetic patient with right-sided hemiballism-hemichorea of acute onset during hypoglycemia following insulin overtreatment of non-ketotic hyperglycemia. Brain computerized tomography and magnetic resonance imaging scans revealed characteristic hyperdensity and T1 hyperintensity, respectively, in the left basal ganglia, in addition to pallido-dentate calcifications, suggestive of Fahr’s syndrome. Although extremely rare, hypoglycemia may be a cause of hemiballism-hemichorea especially in the presence of predisposing factors such as previous hyperglycemic episodes and Fahr’s syndrome.