S. Vassilopoulou

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.

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

Apolipoprotein E polymorphisms and ischaemic stroke: a two-center Greek study.

Apolipropotein E(apoE) is a plasma protein exhibiting three common isoforms (E2, E3, E4). Its involvement in lipoprotein metabolism may have an impact on stroke occurrence. As results in the literature are inconclusive further studies are needed to elucidate its role. Our objective was to study the role of apoE isoforms and the interplay with environmental risk factors in patients with first ischaemic stroke occurrence in the Greek population.

CADASIL and autoimmunity: coexistence in a family with the R169C mutation at exon 4 of the NOTCH3 gene.

Background: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited small vessel disease caused by mutations of the NOTCH3 gene, which result in degeneration of vascular smooth muscle cells, arteriolar stenosis, and impaired cerebral blood flow. For clinicians this is the commonest hereditary adult-onset condition causing stroke and vascular dementia at middle age. Atypical phenotypes have been recognized, and the disease is probably underdiagnosed in the wider stroke population. Coexistence of autoimmunity is atypical and has been described only in occasional patients. Methods: Three members of a Greek family from the island of Lesvos of North East Greece were evaluated. The patients come from a four-generation family in which there were at least seven members with clinical data suggestive of CADASIL. We describe here the clinical, imaging and biochemical findings in this family with R169C mutation at exon 4 and presenting additional clinical and biochemical findings suggestive of autoimmune disorder. DNA was extracted from whole blood using standard procedures for sequencing. Results: Three affected members of this family carried the R169C. In a phenotypic analysis of affected individuals from four generations with CADASIL, the disease was characterized by migraine attacks, recurrent subcortical infarcts, and cognitive decline with typical anterior temporal lobe white matter lesions. At least 3 mutation carriers from two generations had increased antinuclear antibody (ANA) titers and various combinations of rash, joint pains, photosensitivity, and renal involvement. Conclusion: This is a rare description of the coexistence of autoimmunity in CADASIL patients with possible worsening clinical effects. The study extends the spectrum of atypical presentation of CADASIL. The coexistence of autoimmunity does not necessarily exclude CADASIL, but may cause an additional diagnostic and therapeutic challenge. This autoimmune disorder may have increased the severity of the disease and, additionally, may be related to the pathogenetic mechanisms of CADASIL. It is possible that the NOTCH3 mutation alone is not enough to trigger autoimmunity since, in the case of our family, the R169C mutation has already been described in other families with no evidence of coexistent autoimmunity. Other genetic or environmental factors or interactions and/or common pathways between the vascular and immune systems are probably co-operating. Further, prospective studies are needed to clarify the prevalence and types of autoimmune disorders present in CADASIL families.

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.

Convexity Subarachnoid Hemorrhage Due to Cardioembolic Stroke in a Woman with Thyrotoxicosis: Α Case Report

BACKGROUND Non-traumatic convexity subarachnoid hemorrhage (cSAH) is a rarely reported condition with a wide spectrum of etiologies. Cerebral ischemia secondary to extracranial or intracranial atherosclerotic disease has been identified as a relatively uncommon cause of cSAH. CASE REPORT We report a case of cSAH caused by cardioembolic stroke. A 69-year old female patient developed suddenly left-sided face and body weakness and numbness and visual neglect on the left. She was newly detected with paroxysmal atrial fibrillation on the ground of thyrotoxicosis. Brain magnetic resonance imaging revealed ischemia of embolic pattern with cSAH. Further evaluation excluded other cause of hemorrhage. Dilation of leptomeningeal collateral vessels and rupture of pial vessels in distal cortical arteries may caused cSAH. Full anticoagulation was initiated. After one month, her condition improved significantly (NIHSS from 6 to 2). CONCLUSIONS cSAH may be a rare complication of cardioembolic stroke.