Primary angiitis of the central nervous system in a patient with transient episodes of headache and aphasia: A case report.

Primary angiitis of the central nervous system (PACNS) refers to a rare form of vasculitis of unknown cause, with a challenging diagnostic work-up. We report the case of a 57-year-old patient who presented with transient episodes of headache and global aphasia. Cerebrospinal fluid (CSF) examination revealed lymphocytic pleocytosis with moderate elevated protein and normal glucose. CSF and serum tests for infections and autoimmune/paraneoplastic antibodies were negative, except CSF polymerase chain reaction testing that detected Epstein-Barr virus (EBV). Magnetic resonance imaging of the brain with intravenous gadolinium showed meningeal enhancement and pachymeningitis. Due to continuous relapsing episodes of aphasia, a leptomeningeal and brain tissue biopsy was performed and revealed lesions of granulomatous necrotising vasculitis of medium-sized leptomeningeal and intracranial vessels, as well as negative in situ hybridism for EBV. A diagnosis of primary granulomatous necrotising angiitis of the central nervous system was made, and the patient was treated with intravenous methylprednisolone and oral cyclophosphamide, showing excellent response to treatment. Diversity in clinical and laboratory features makes it difficult for PACNS to be distinguished by other systemic vasculitides. Laboratory tests and neuroimaging can provide guidance in evaluation of the patients and exclude other possible causes, but tissue biopsy remains the gold standard for a definite diagnosis.

Quality metrics in the management of acute stroke in Greece during the first 5 years of Registry of Stroke Care Quality (RES-Q) implementation.

Introduction: Establishment of a prospective stroke registry may promote the documentation and improvement of acute stroke care. We present the status of stroke management in Greece using the Registry of Stroke Care Quality (RES-Q) dataset. Methods: Consecutive patients with acute stroke were prospectively registered in RES-Q registry by contributing sites in Greece during the years 2017-2021. Demographic and baseline characteristics, acute management, and clinical outcomes at discharge were recorded. Stroke quality metrics, with a specific interest in the association between acute reperfusion therapies and functional recovery in ischemic stroke patients are presented. Results: A total of 3590 acute stroke patients were treated in 20 Greek sites (61% men, median age 64 years; median baseline NIHSS 4; 74% ischemic stroke). Acute reperfusion therapies were administered in almost 20% of acute ischemic stroke patients, with a door to needle and door to groin puncture times of 40 and 64 min, respectively. After adjustment for contributing sites, the rates of acute reperfusion therapies were higher during the time epoch 2020-2021 compared to 2017-2019 (adjusted OR 1.31; 95% CI 1.04-1.64; p < 0.022; Cochran-Mantel-Haenszel test). After propensity-score-matching, acute reperfusion therapies administration was independently associated with higher odds of reduced disability (one point reduction across all mRS scores) at hospital discharge (common OR 1.93; 95% CI 1.45-2.58; p < 0.001). Conclusions: Implementation and maintenance of a nationwide stroke registry in Greece may guide the stroke management planning, so that prompt patient transportation, acute reperfusion therapies, and stroke unit hospitalization become more widely accessible, improving the functional outcomes of stroke patients.

Seasonal adherence to, and effectiveness of, subcutaneous interferon ß-1a administered by RebiSmart® in patients with relapsing multiple sclerosis: results of the 1-year, observational GEPAT-SMART study.

BACKGROUND: Little is known about whether tolerability and adherence to treatment can be influenced by weather and temperature conditions. The objective of this study was to assess monthly and seasonal adherence to and safety of sc IFN-ß1a (Rebif®, Merck) in relapsing-remitting multiple sclerosis (RRMS) patients using the RebiSmart® electronic autoinjector. METHODS: A multicentre, prospective observational study in Greece in adult RRMS patients with EDSS < 6, under Rebif®/RebiSmart® treatment for ≤6 weeks before enrollment. The primary endpoint was monthly, seasonal and annual adherence over 12 months (defined in text). Secondary endpoints included number of relapses, disability, adverse events. RESULTS: Sixty four patients enrolled and 47 completed all study visits (Per Protocol Set - PPS). Mean annual adherence was 97.93% ± 5.704 with no significant monthly or seasonal variations. Mean relapses in the pre- and post- treatment 12-months were 1.1 ± 0.47 and 0.2 ± 0.54 (p < 0.0001, PPS). 10 patients (22%) showed 3-month disability progression, 19 (40%) stabilization and 18 (38%) improvement. EDSS was not correlated to pre- (r = 0.024, p = 0.87) or post-treatment relapses (r = 0.022, p = 0.88). CONCLUSION: High adherence with no significant seasonal or weather variation was observed over 12 months. While the efficacy on relapses was consistent with published studies, we could not identify a relationship between relapses and disability. TRIAL REGISTRATION: Greek registry of non-interventional clinical trials ID: 200136 , date of registration: February 18th, 2013.

Intravenous thrombolysis for acute ischemic stroke in Greece: the Safe Implementation of Thrombolysis in Stroke registry 15-year experience.

BACKGROUND: Intravenous thrombolysis (IVT) remains the only approved systemic reperfusion treatment for acute ischemic stroke (AIS), however there are scarce data regarding outcomes and complications of IVT in Greece. We evaluated safety and efficacy outcomes of IVT for AIS in Greece using the Safe Implementation of Thrombolysis in Stroke: International Stroke Thrombolysis Register (SITS-ISTR) dataset. METHODS: All AIS patients treated with IVT in Greece between December 2002 and July 2017 and recorded in the SITS-ISTR were evaluated. Demographics, risk factors, baseline stroke severity [defined using National Institutes of Health Stroke Scale (NIHSS)], and onset-to-treatment time (OTT) were recorded. Safety outcomes included symptomatic intracranial hemorrhage (sICH) and 3-month mortality rates. The efficacy outcomes evaluated a reduction in baseline NIHSS score at 2 and 24 h following IVT onset, 3-month favorable functional outcome [FFO; modified Rankin scale (mRS) scores of 0-1] and 3-month functional independence (FI; mRS-scores of 0-2). The safety and efficacy outcomes were assessed comparatively with previously published data from SITS national and international registries. RESULTS: A total of 523 AIS patients were treated with IVT in 12 Greek centers participating in the SITS-ISTR during the study period (mean age 62.4 ± 12.7; 34.6% women; median baseline NIHSS score: 11 points; median OTT: 150 min). The rates of sICH were 1.4%, 2.3%, and 3.8% according to the SIST-MOST, ECASS II, and NINDS criteria respectively. The median reduction in NIHSS score at 2 and 24 h was 3 [interquartile range (IQR): 1-5] and 5 (IQR: 2-8) points respectively. The 3-month FI, FFO and mortality were 66.5%, 55.6% and 7.9%. All safety and efficacy outcomes were comparable with available data from SITS-ISTR in other European countries. CONCLUSIONS: Our study underscores the safety and efficacy of IVT for AIS in Greece. Additional action is necessary in order to increase the availability of IVT in the Greek population and to include more centers in the SITS-ISTR.

Cerebrospinal fluid analysis after unprovoked first seizure.

The aim of this study was to determine cerebrospinal fluid (CSF) characteristics after an unprovoked first seizure (UFS). We reviewed the medical records of 71 patients with UFS who underwent lumbar puncture, and examined the CSF parameters. Each CSF parameter was evaluated separately for potential correlations with the other study variables. We observed an overall frequency of CSF abnormalities of 35.2%. CSF protein was the most common abnormal parameter (31%) and showed significant positive correlations with male gender (p=0.037) and older age (p=0.007). Only seven patients (9.9%) had an abnormal cell count (5-40 cells/µl). Higher CSF cell counts were found to predict a longer hospitalization period (p=0.005). No relationship with abnormal EEG findings could be established (p=0.169). This study is one of the few to evaluate postictal CSF parameters in a clinical setting, and to our knowledge the first to investigate these parameters specifically in the emergency department. The development of a rapid, easy-to-use test that does not require extensive laboratory equipment to differentiate UFS from other conditions could be of great value in everyday clinical practice.

Herpes Simplex Virus Type 2 Encephalitis as a Cause of Ischemic Stroke: Case Report and Systematic Review of the Literature.

BACKGROUND AND AIM: Our objective is to describe a patient who developed an ischemic stroke as a complication of herpes simplex virus type 2 (HSV-2) encephalitis and to review the literature. PATIENTS AND METHODS: A 45-year-old immune-competent Caucasian man presented with a 24-hour history of confusion and fever, and following clinical and laboratory examination was diagnosed with HSV-2 encephalitis. However, the brain magnetic resonance imaging also showed an acute ischemic infarct in the left frontal lobe corresponding to vascular territories of middle cerebral artery branches. Further screening failed to identify any other cause of the stroke. A systematic literature search was conducted in February 2015 using the PubMed database. RESULTS: Six more cases of herpes simplex virus (HSV) central nervous system (CNS) infection that developed a definite ischemic stroke as a complication of the infection were identified. CONCLUSIONS: Ischemic stroke, although infrequent, can complicate the evolution of herpes simplex meningitis or encephalitis. Clinicians should include HSV CNS infection as a possible cause of ischemic stroke, especially in young patients with ischemic stroke of unknown etiology.

Vertebrobasilar Dolichoectasia Induced Hydrocephalus: the Water-Hammer Effect.

Vertebrobasilar dolichoectasia is a clinical entity associated rarely with obstructive hydrocephalus. We present a 48-year old male with a profound dilatation of the ventricular system due to a dolichoectatic basilar artery, as appeared in imaging studies. The patient suffered from longstanding hydrocephalus and presenile dementia. The underlying mechanism for obstructive hydrocephalus due to vertebrobasilar dolichoectasia is considered to be both a water-hammer effect and a direct compression of adjacent structures. We suggest prompt surgical intervention upon diagnosis as a first choice treatment in order to avoid further complications.

From basilar artery dolichoectasia to basilar artery aneurysm: natural history in images.

Dolichoectasia is a medical term used to describe elongated and dilated vessels that follow a tortuous and windy course with frequent loops and curves. We are presenting the natural history in images of a normal basilar artery becoming dolichoectatic, followed by the formation of an aneurysm, over a period of many years, in 60-year-old Caucasian man with a long history of secondary progressive multiple sclerosis and uncontrolled arterial hypertension, who was diagnosed with dolichoectasia of basilar artery in 2008. Although relatively stable at this point, eventually his mobility deteriorated and signs from the cranial nerves, such as trigeminal neuralgia and bilateral palsy of the VI and the VII nerves were added in the clinical picture. In 2014, both computed tomography and magnetic resonance imaging of the brain revealed the formation of an unruptured aneurysm of the basilar artery.

Polymorphisms in prothrombotic genes in young stroke patients in Greece: a case-controlled study.

Mechanisms of ischemic stroke in young adults are poorly understood. The aim of the study was to investigate and compare the frequency of common variations in prothrombotic genes between young patients with ischemic stroke and controls. Fifty-one cases of first-ever ischemic stroke and 70 community-based controls aged below 50 years were studied. In both groups, the insertion/deletion 4G/5G variation (-675 4G/5G PAI-1) as well as the single-nucleotide polymorphism-844 G/A of the PAI-1 (-844 G/A PAI-1) gene promoter, factor V Leiden (FVL) G1691Α, the prothrombin variant (allele 20210A, FIIG20210A), factor XIII-A Val34Leu polymorphism (FXIII-AVal34Leu) and C677T methylenotetrahydrofolate reductase (C677T MTHFR) polymorphism have been assessed. The -675 4G/5G PAI-1 allele distribution differed significantly between patients and controls (P = 0.020), but no difference was found regarding the distribution of -844 G/A PAI-1 (P = 0.493), FVL (P = 0.199), FIIG20210A (P = 0.410), FXIII-AVal34leu (P = 0.160) and C677T MTHFR (P = 0.788). A lower frequency of 5G/5G genotype and a higher frequency of the 4G/5G genotype of the PAI -675 4G/5G polymorphism was found in young ischemic stroke patients compared to healthy controls. Further epidemiological studies are needed to investigate the differences between different geographic areas, and prospective cohort studies are needed to investigate the possible protective role of 5G/5G polymorphism.

Predicting 30-day case fatality of primary inoperable intracerebral hemorrhage based on findings at the emergency department.

BACKGROUND: Early survival of patients with intracerebral hemorrhage (ICH) depends on several factors, including the location and size of the hematoma and the level of consciousness on admission. The aim of our study was to estimate the case fatality of primary inoperable ICH 30 days after the event in our hospital and to identify clinical and laboratory characteristics, recordable at the Emergency Department (ED), which could predict death at 30 days. METHODS: Clinical and laboratory data on all patients with primary ICH admitted to our hospital were retrospectively collected. RESULTS: Between January 2011 and June 2013 191 patients with primary ICH were admitted to our hospital. The 30-day case fatality rate was estimated to be 31.9%, as 61 patients died within 30 days after the ICH. Five variables were independently associated with 30-day case fatality: each decreased point at the Glasgow Coma Scale (GCS) is associated with a 1.3-fold increase in the odds of death at 30 days; infratentorial location and intraventricular extension are associated with a 5.5-fold and a 4.7-fold increase in the odds of death at 30 days, respectively; each centimeter of the maximum diameter of the hematoma and each point increase of the international normalized ratio (INR) are associated with a 1.9-fold and a 3.5-fold increase in the odds of death at 30 days, respectively. CONCLUSIONS: GCS score on admission, infratentorial location of the hematoma, intraventricular extension of the hematoma, INR on admission, and maximum diameter of the hematoma are the 5 variables that are independently associated with 30-day case fatality of primary inoperable ICH. EDICH is introduced as a new grading scale, which includes laboratory and clinical findings at the ED and has predicting value of the 30-day case fatality.

Determinants of depression among patients with epilepsy in Athens, Greece.

OBJECTIVE: Depression is common among patients with epilepsy. The aim of our study was twofold: to estimate the prevalence of a major depressive episode and to identify its determinants among patients with epilepsy treated in the largest Greek hospital in Athens. METHODS: All consecutive patients with epilepsy that visited the epilepsy outpatient clinic of Evangelismos General Hospital were invited to participate in the study. Ninety-four patients met our inclusion criteria. RESULTS: A diagnosis of a current major depressive episode was established in 21 out of 94 eligible to participate (22.3%) patients. Being a female was associated with a 19.68-fold increase in the odds of having a major depressive episode (95% CI 3.39-114.14, p=0.001); being unemployed was associated with a 6.46-fold increase in the odds of having a major depressive episode (95% CI 1.23-34.07, p=0.028), and each extra seizure experienced per month was associated with a 1.38-fold increase in the odds of having a major depressive episode (95% CI 1.03-1.85, p=0.031). CONCLUSION: Unemployment, female gender, and seizure control are important determinants of a major depression episode among patients with epilepsy.

Natalizumab-related progressive multifocal leukoencephalopathy in Greece.

BACKGROUND & OBJECTIVES: Progressive multifocal leukoencephalopathy (PML) may complicate natalizumab treatment in multiple sclerosis patients. We sought to characterize the clinical and laboratory features of natalizumab-related PML (NR-PML) cases from Greece. METHODS: Pharmaceutical industry, national drug authorities and all neurology departments within the Greek territory were asked to provide data for cases of NR-PML until October 2012. Collected cases were classified according to their level of diagnostic certainty using the five-level system introduced by Mentzer et al. (2012). RESULTS: Thirteen NR-PML cases were identified by the neurology departments. Data were provided for only 9 cases. PML manifestations appeared after a median number of 40 (21-52) natalizumab infusions. All but two patients were treated with plasma exchange and some were treated adjunctively with mirtazapine while the others were treated with mefloquine. IRIS developed in 6 cases after a median time of 6 (2-10) weeks from PML presentation and were treated with different regimens of corticosteroids. PML was fatal in 3 cases. The median EDSS after a median follow-up time of 12 (8-23) months in the surviving cases was 4.75 (2-8.5). CONCLUSIONS: Outcomes for collected NR-PML cases varied from death to returning to baseline. Close surveillance is essential for early diagnosis and treatment of NR-PML patients.

Validation of the Greek version of the Neurological Disorders Depression Inventory for Epilepsy (NDDI-E).

The Neurological Disorders Depression Inventory for Epilepsy (NDDI-E) was developed as a screening tool for symptoms of major depressive episodes in people with epilepsy. Our study describes the development, validation, and psychometric properties of the Greek version of the NDDI-E. A consecutive sample of 101 patients with epilepsy, eligible to participate in the study, has been assessed using the Mini International Neuropsychiatric Interview version 5.0.0 and the NDDI-E. All patients had no major difficulties in understanding or answering the questions of the Greek version. Cronbach's alpha coefficient was 0.74. Receiver operating characteristic analysis showed an area under the curve of 91% (95% CI=83%-99%; SE: 0.040, p<0.001). At a cutoff score of greater than 15, the NDDI-E showed a sensitivity of 91%, a specificity of 81%, and a negative predictive value of 97%.