Sofia Vassilopoulou

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.

Demographic and geographic vascular risk factor differences in european young adults with ischemic stroke: The 15 cities young stroke study

Background and Purpose— We compared among young patients with ischemic stroke the distribution of vascular risk factors among sex, age groups, and 3 distinct geographic regions in Europe. Methods— We included patients with first-ever ischemic stroke aged 15 to 49 years from existing hospital- or population-based prospective or consecutive young stroke registries involving 15 cities in 12 countries. Geographic regions were defined as northern (Finland, Norway), central (Austria, Belgium, France, Germany, Hungary, The Netherlands, Switzerland), and southern (Greece, Italy, Turkey) Europe. Hierarchical regression models were used for comparisons. Results— In the study cohort (n=3944), the 3 most frequent risk factors were current smoking (48.7%), dyslipidemia (45.8%), and hypertension (35.9%). Compared with central (n=1868; median age, 43 years) and northern (n=1330; median age, 44 years) European patients, southern Europeans (n=746; median age, 41 years) were younger. No sex difference emerged between the regions, male:female ratio being 0.7 in those aged <34 years and reaching 1.7 in those aged 45 to 49 years. After accounting for confounders, no risk-factor differences emerged at the region level. Compared with females, males were older and they more frequently had dyslipidemia or coronary heart disease, or were smokers, irrespective of region. In both sexes, prevalence of family history of stroke, dyslipidemia, smoking, hypertension, diabetes mellitus, coronary heart disease, peripheral arterial disease, and atrial fibrillation positively correlated with age across all regions. Conclusions— Primary preventive strategies for ischemic stroke in young adults—having high rate of modifiable risk factors—should be targeted according to sex and age at continental level.

Duloxetine Versus Citalopram and Sertraline in the Treatment of Poststroke Depression, Anxiety, and Fatigue

The authors sought to determine the relative efficacy and tolerability of duloxetine versus citalopram and sertraline in the treatment of poststroke depression (PSD), anxiety, and fatigue. A group of 60 patients with PSD were assigned to receive duloxetine, citalopram, or sertraline and were assessed over a 3-month period for depression, anxiety, and fatigue. Improvement of depression and anxiety, but not fatigue, was observed in all study groups. Duloxetine was well tolerated and significantly more effective than citalopram and sertraline for the treatment of anxiety symptoms in PSD patients. None of the antidepressants used was effective for reducing symptoms of fatigue.

Spinal cord stroke in a ballet dancer.

Fibrocartilaginous embolism of the intervertebral disc represents an uncommon cause of spinal cord infarction. We present the case of a previously healthy 30-year old ballet dancer who noted acute severe neck pain shortly after an intensive training session and developed weakness and numbness of both arms, as well as difficulties in emptying the bladder and bowel. Her clinical presentation and neuroimaging studies including diffusion weighted imaging were consistent with a spinal cord infarction in the anterior spinal territory at the C3-C6 spinal cord level. Although no histological confirmation was obtained, lack of evidence of other plausible diagnoses in the setting of the patients clinical presentation and neuroimaging findings made fibrocartilaginous embolism the most likely etiopathogenetic mechanism of spinal 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.

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.

Dropped head syndrome as prominent clinical feature in MuSK-positive Myasthenia Gravis with thymus hyperplasia

MuSK-positive Myasthenia Gravis is in most cases clinically characterized by a progressive course with severe oculobulbar involvement or prominent neck, shoulder and respiratory muscle weakness. It is also distinguished from other forms of myastehnia through its lack of germinal centers or lymphocytic infiltrates in the thymic tissue. We present the case of a MuSK-positive female myasthenic patient with over four years slowly progressive weakness of the neck extensor muscles in the presence of thymus hyperplasia and discuss its uncommon and markedly focal clinical and electrophysiological features, as well as the excellent course under medication with pyridostigmine and prednisone, especially after thymectomy.

Painful ophthalmoplegia with simultaneous orbital myositis, optic and oculomotor nerve inflammation and trigeminal nucleus involvement in a patient with herpes zoster ophthalmicus

Viral infection is a rare cause of painful ophthalmoplegia. We report on a 67-year-old patient who developed painful double vision after a vesicular skin rash on the left forehead. MRI disclosed simultaneous inflammatory lesions in all extraocular muscles, the second and third cranial nerve, as well as pathological signal intensity along the spinal trigeminal tract and nucleus within the medulla oblongata and the pons. Cerebrospinal fluid and serum tests for varicella zoster were positive. The patient was treated effectively with intravenous acyclovir and methylprednisolone. Simultaneous lesions in various neighbouring neural structures may be characteristic for the highly neurotropic behaviour of the herpesviridae and should be considered as a cause of painful ophthalmoplegia that can be depicted by appropriate imaging.

Ischemic Stroke as an Uncommon Complication of Cor Triatriatum

Cor triatriatum (CT) is a rare congenital heart disease characterized by a fibromuscular membrane that divides the left atrium into two distinct chambers and mostly manifests during infancy mimicking mitral valve stenosis. We present the uncommon case of cardioembolic ischemic stroke in an adult man on the grounds of an otherwise asymptomatic CT with intra-atrial thrombus formation, which was established by means of transesophageal echocardiography and cardiac magnetic resonance imaging. Because exact diagnosis and surgical treatment is feasible, we suggest CT as a rare, but potentially treatable source of cerebral embolism in adult patients with stroke and dilatated left atrium.