K. Spengos

Etiology of first‐ever ischaemic stroke in European young adults: the 15 cities young stroke study

Risk factors for IS in young adults differ between genders and evolve with age, but data on the age‐ and gender‐specific differences by stroke etiology are scare. These features were compared based on individual patient data from 15 European stroke centers.

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.

Twenty four hour pulse pressure predicts long term recurrence in acute stroke patients

Objectives: The impact of different blood pressure (BP) components during the acute stage of stroke on the risk of recurrent stroke is controversial. The present study aimed to investigate by 24 hour BP monitoring a possible association between acute BP values and long term recurrence. Methods: A total of 339 consecutive patients with first ever acute stroke underwent 24 hour BP monitoring within 24 hours of ictus. Known stroke risk factors and clinical findings on admission were documented. Patients given antihypertensive medication during BP monitoring were excluded. The outcome of interest during the one year follow up was recurrent stroke. The Cox proportional hazard model was used to analyse association of casual and 24 hour BP recordings with one year recurrence after adjusting for stroke risk factors, baseline clinical characteristics, and secondary prevention therapies. Results: The cumulative one year recurrence rate was 9.2% (95% CI 5.9% to 12.3%). Multivariate Cox regression analyses revealed age, diabetes mellitus, and 24 hour pulse pressure (PP) as the only significant predictors for stroke recurrence. The relative risk for one year recurrence associated with every 10 mm Hg increase in 24 hour PP was 1.323 (95% CI 1.019 to 1.718, p = 0.036). Higher casual PP levels were significantly related to an increased risk of one year recurrence on univariate analysis, but not in the multivariate Cox regression model. Conclusions: Elevated 24 hour PP levels in patients with acute stroke are independently associated with higher risk of long term recurrence. Further research is required to investigate whether the risk of recurrent stroke can be reduced to a greater extent by decreasing the pulsatile component of BP in patients with acute stroke.

Body composition analysis in late-onset Pompe disease.

Pompe disease is an inherited metabolic disorder caused by α-glycosidase deficiency. The adult onset form is mainly characterized by progressive proximal muscle weakness and respiratory dysfunction. The aim of the present study is to evaluate body composition in adult patients before and after enzyme replacement therapy (ERT). Body composition was examined at baseline by means of dual x-ray absorptiometry (DXA) in nine adult patients and after different time periods in six of them who received ERT. Total BMD (bone mineral density) was initially mildly decreased in two patients, while femoral neck BMD was decreased in five patients. On the other hand fat mass was increased in the majority of patients, while body mass index (BMI) was high in four. ERT administration did not seem to induce obvious BMD changes in any patient. Conclusively, the greater femoral neck BMD involvement may be attributed to the lower mechanical load applied by the selectively weakened muscles, whereas the increased fat mass may be the result of metabolic and nutritional derangement.

Adult Pompe disease: clinical manifestations and outcome of the first Greek patients receiving enzyme replacement therapy.

OBJECTIVE Pompe disease is an autosomal recessive lysosomal disorder caused by α-glucosidase deficiency. A specific treatment for the disease with enzyme replacement therapy is currently available. The aim of the present study is to describe the clinical manifestations and the effect of treatment in the first Greek patients with the adult form of the disease. METHODS Five Greek patients with adult onset Pompe disease aged between 40 and 73 years received 20 mg/kg Myozyme intravenously at two weekly intervals over a different period. Clinical and functional parameters were longitudinally registered. RESULTS Proximal muscle weakness and respiratory insufficiency were the most common manifestations of the disease, but their severity was different even among patients with similar genotype. The effect of treatment varied with most patients experiencing some improvement in muscle strength and fatigability, while the most severely affected patient did not benefit and stopped therapy. CONCLUSION No clear genotype-phenotype correlation emerges from our study. The different effect of treatment on our patients seems to be mainly related to their pre-treatment condition and can be reliably assessed only on a long term basis.

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.

Long-term outcome in posterior cerebral artery stroke

Introduction:  Previous studies on posterior cerebral artery (PCA) strokes focused mainly on topography and underlying pathophysiology. However, there are no data on long‐term prognosis and its association with the localization of the infarct.

Etiology and treatment of ischaemic stroke in patients with β‐thalassemia major

Background and purpose:  Although hypercoagulability‐induced thromboembolism is generally accepted as cause of cerebral ischaemia in thalassemic patients, cardiogenic embolism has been recently suggested as another possible stroke etiology.

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