Patient and treatment characteristics and safety outcomes of patients with relapsing-remitting multiple sclerosis treated with natalizumab in Greece: Results from the multicenter, 5-year prospective observational study 'TOPICS greece'.

BACKGROUND: Natalizumab is a highly efficacious treatment for relapsing-remitting multiple sclerosis (RRMS). OBJECTIVE: To assess the real-world long-term safety of natalizumab in RRMS. METHODS: This multicenter, 5-year prospective observational study, included adults with RRMS newly initiated on natalizumab as per the approved product label in the routine care in Greece. Safety was evaluated by collecting serious adverse events (SAEs) following study enrollment. RESULTS: Between 19-Apr-2012 and 18-Dec-2014, 304 eligible patients (median age at natalizumab initiation: 38.0 years; median disease duration: 6.2 years) were enrolled by 20 hospital-based neurologists. Over a median treatment duration period of 58.7 months, 50.7% of the patients discontinued natalizumab, mainly due to anti-JCV antibody detection (59.1%). The adverse event treatment discontinuation rate was 5.2%. The SAE incidence rate during the safety data collection period (median: 48.7 months) was 4.6%. The most common SAEs were infections (1.0%), including 2 cases (0.7%) of progressive multifocal leukoencephalopathy (PML), and no other opportunistic infections. PML diagnoses occurred 6.2-6.7 years after natalizumab initiation, and approximately 2 years after first detection of anti-JCV antibody for both patients. The incidence rate of malignancies was 0.7%. CONCLUSION: In real-world settings in Greece, natalizumab displayed an acceptable safety profile, with no new safety signals emerging.

Exploring hybrid consensus models to assess roadkill.

Accurate information provided by reliable models is essential for identifying hotspots and mitigating roadkill. However, existing methods, such as kernel density estimation (KDE) and maximum entropy modeling (ME) may individually identify only a subset of the suitable locations for mitigation, because KDE cannot detect hotspots once local abundances are depressed, and ME may only partially identify current hotspots due to imperfect discrimination skill. Here, we propose a hybrid consensus modeling (HCM) approach that leverages the strengths of both KDE and ME by using their consensus to identify the core subset of hotspots. We collected herpetofauna (amphibians and reptiles) roadkill data (N = 839) along four roads in Taiwan (R.O.C.) to evaluate the statistical performance and theoretical mitigation efficiency of HCM, KDE and ME, and to compare the allocation among roads, spatial clustering, and environmental conditions in the identified hotspots. HCM was applied on the herpetofauna dataset as well as separately on amphibians and reptiles. Although the discrimination skill of KDE and ME models for both target clades together was good to excellent (AUCKDE = 0.944, AUCME = 0.822), the highest theoretical mitigation efficiency, was displayed by HCM Consensus (2.89), followed by KDE (2.58), and ME (1.91). Furthermore, we show that theoretical mitigation efficiency increases with decreasing spatial clustering (Moran's I). Given pervasive budget constraints, we recommend to limit permanent mitigation measures such as fenced culverts to HCM Consensus hotspots, temporary measures to KDE hotspots, and to target additional monitoring at ME hotspots.

Fatal, paraparetic acute motor axonal neuropathy, early complicating chemotherapy with nab-paclitaxel.

So far, very few cases describing an interrelation between chemotherapy and Guillain-Barré syndrome have been published. We describe the first case of a paraparetic, pure acute motor axonal neuropathy variant of Guillain-Barré syndrome, early complicating protein-bound paclitaxel (nab-paclitaxel/abraxane) chemotherapy (first and sole session at a 350 mg dose) in a female patient with metastatic breast cancer. Although our patient was treated with the standard regimen of intravenous immunoglobulin for 5 days, she showed no evidence of motor improvement and died 1 month after the onset of the neurological deficit.

Neurobrucellosis presenting as clinically definite amyotrophic lateral sclerosis.

Purpose/Aim: We describe the first case of a patient with neurobrucellosis presenting with clinically-definite ALS. MATERIAL AND METHODS: A 48-year old male patient, in whom the diagnoses of systemic brucellosis and clinically definite ALS were undoubtedly confirmed and were eventually causally interrelated. The disease-specific antibiotic therapy was unsuccessful to slow the evolution of the motor neuron disease and the patient became non ambulatory over time. RESULTS: Considering the close temporal association of ALS onset with the systemic Brucella infection and consequent antigenic stimuli, we might suggest that human brucellosis might have triggered a process of motor neuron degeneration in keeping with neurobrucellosis, primarily due to parainfectious mechanism. CONCLUSION: Our case helps to shed light on the factors that may trigger or only fasten motor neuron disease manifestations.

Dorsal scapular neuropathy causing rhomboids palsy and scapular winging.

Most cases of scapular winging (SW) are attributed to either long thoracic or spinal accessory nerve lesions. Dorsal scapular nerve lesions are quite rare and the literature contains very few case reports of SW secondary to rhomboid paralysis. We are reporting the unusual case of a young patient who developed right-side scapular winging due to dorsal scapular neuropathy and rhomboids palsy, and we highlight the role of conservative treatment and rehabilitation for cases of mild/medium injury to the dorsal scapular nerve or to the rhomboid muscles. For those cases, physiotherapy is recommended, and this is mainly aimed at strengthening the trapezius in order to compensate for rhomboids weakness.

Cobalamin deficiency triggering de novo status epilepticus.

Cobalamin deficiency is included in the spectrum of very uncommon underlying causes of status epilepticus (SE) and the literature contains very few such cases. We herein report a case of unusual presentation of cobalamin (vitamin B12) deficiency with de novo SE with the intention to bolster the argument that a de novo manifestation of SE due to cobalamin deficiency might not be that uncommon. We also support the importance of prompt identification and treatment of the underlying causes of SE, particularly those which are uncommon.

Quality of life and emotional burden of primary caregivers: a case-control study of multiple sclerosis patients in Greece.

PURPOSE: Our aim was to assess the perceived emotional burden and quality of life (QOL) in a sample of Greek primary caregivers of patients with multiple sclerosis (MS). METHODS: Twenty-two male and 13 female primary caregivers (mean age 47.3 ± 12.4 years), and an equal number of patients with MS, completed the Greek validated version of the hospital anxiety and depression scale (HADS) and the Greek validated version of EuroQOL (EQ-5D). Thirty-five age-, gender-, and education-matched healthy controls were used for comparison. RESULTS: Caregivers experienced higher degree of anxiety than depression. The mean score of the HADS-A subscale was 9.5 ± 4 (range 3-15), and the mean score of the HADS-D subscale was 7.1 ± 3.1 (range 2-14). The mean scores of caregivers on both HADS-A and HADS-D were significantly higher than the controls' (P < 0.0001). Twenty-two caregivers were diagnosed with manifesting anxiety, whereas 12 of them also presented depression. Highly educated caregivers were more prone to manifest increased levels of anxiety and depression. The increased psychological distress was further supported by the responses in the anxiety/depression dimension on the EQ-5D: 27 reported a moderate level of anxiety/depression and three indicated an extreme degree. The caregivers reported a mean EQ-VAS value of 61.9 ± 13.8 (range 40-100), with 10 caregivers rating their health status a score of 50 or lower; the controls scored significantly higher (90.3 ± 7.1; P < 0.0001). CONCLUSION: The sample of caregivers we studied was psychologically burdened to a significant degree, a fact that obviously deteriorates their QOL. Appropriate psychopharmacological interventions are warranted to reduce caregivers' burden.

Assessing the quality of sleep in Greek primary caregivers of patients with secondary progressive multiple sclerosis: a cross-sectional study.

CONTEXT: Several studies have investigated the prevalence of sleep disorders in patients suffering from multiple sclerosis (MS) and have shown that up to 54% of patients may have significantly more sleep problems than the general population. To our knowledge, however, no data are available about the quality of sleep of the primary caregivers of patients with MS. OBJECTIVES: The objectives of the current cross-sectional study were to assess the quality of sleep in Greek primary caregivers of patients with MS and to investigate its relationship with the degree of caregivers' emotional distress. METHODS: Twenty-two male and 13 female primary caregivers (mean age 47.3±12.4 years) of an equal number of patients with MS, who consented to participate, completed the validated Greek version of the Pittsburgh Sleep Quality Index (PSQI) and the validated Greek version of the Hospital Anxiety and Depression Scale (HADS). Thirty-five age-, gender-, and education-matched healthy controls were used for comparisons. RESULTS: Caregivers experienced a higher degree of anxiety than depression. The mean score in the seven-item HADS-A subscale was 9.5±4 (range 3-15) and the mean score in the seven-item HADS-D subscale was 7.1±3.1 (range 2-14). The mean scores of caregivers on both HADS-A and HADS-D were significantly higher than those of controls (P<0.001). The PSQI scoring demonstrated that 19 (54.3%) caregivers had poor sleep quality (cut-off value of >5). The mean values of caregivers for the PSQI were 6.0±2.8 (range 2-12) compared with controls, who scored at a significantly lower level (1.5±0.8; P<0.001). Poor quality of sleep was significantly correlated with increased levels of anxiety (r=0.392; P=0.02) and depression (r=0.424; P=0.01). Among the PSQI components, the sleep duration and sleep latency were mostly influenced by the degree of emotional distress. CONCLUSION: A significant proportion of primary caregivers of MS patients experience poor sleep quality. The degree of their emotional distress appears to significantly influence their quality of sleep. Appropriate psychopharmacological interventions may be required for those individuals.

Religiosity and its relation to quality of life in primary caregivers of patients with multiple sclerosis: a case study in Greece.

The first objective of the current observational study was to assess the degree of religiosity in Greek Christian Orthodox primary caregivers of patients with multiple sclerosis (MS). The second objective was to evaluate the interrelations between religiosity and quality of life (QOL) and to identify the determinants of QOL, an endpoint of considerable importance in clinical research and practice. Twenty-two male and 13 female primary caregivers (mean age 47.3 ± 12.4 years) of an equal number of patients with MS, who consented to participate, completed the Systems of the Greek version of the Belief Inventory (SBI-15R) and the Greek validated version of EuroQOL (EQ-5D). The analysis revealed high scores on religiosity, especially among females. Caregivers scored in the religious beliefs and practices subscale of SBI-15R with a mean score of 22.8 ± 7.8 (range 0-30) and with 7.1 ± 4.8 (range 0-14) in the social support subscale. However, both of the SBI-15R domains were almost unrelated to the degree of overall QOL. There was only a reliable (but with little clinical value) association between the pain/discomfort domain of the EQ-5D with the SBI-15R beliefs and practices subscale (r = -0.38, p = 0.03). Although high levels of religiosity among Greek Christian Orthodox primary caregivers of MS patients were evident, this study did not demonstrate any beneficial effect of religious beliefs and practices on their QOL. Further prospective studies with a population with the same and/or diverse religious and cultural backgrounds are needed to better elucidate the complex association between religiosity and QOL in primary caregivers of MS patients.

Foreign accent syndrome caused by a left temporal-parietal ischaemic stroke.

UNLABELLED: Karanasios P, Loukopoulou P, Zampakis P, Tiligadas T, Makridou A, Doukas V, Argyriou AA. Foreign accent syndrome caused by a left temporal-parietal ischaemic stroke. AIM: We present the first reported case of a Greek patient with foreign accent syndrome (FAS) secondary to a left temporal-parietal ischemic stroke. CASE REPORT: A 76 year-old right-handed, Greek in origin, male was referred because he had suddenly manifested changes in speech expression. The neurological examination revealed that his prior typical English-Australian accent resembled a mixture of Greek and English-Britain accent consistent with FAS, though he had visited only once Greece the last 15 years and never had been to United Kingdom. RESULTS: A brain Magnetic Resonance Imaging scan depicted an ischemic temporal lesion in the language-dominant left hemisphere, affecting the left posterior superior and middle temporal gyri, as well as the ipsilateral inferior supramarginal angular gyrus and posterior insula. CONCLUSION: We might suggest that FAS in our patient was induced because of interrupted cortical-subcortical feedback pathways. The phenomenon of subcortical-cortical diaschisis might also have contributed to its clinical manifestation.

Cocaine use and abuse triggering sporadic young-onset amyotrophic lateral sclerosis.

To our knowledge, we describe for the first time the case of a male patient with sporadic young-onset amyotrophic lateral sclerosis, most likely attributed to chronic regular cocaine use and abuse. Our case supports the view that cocaine use and abuse may trigger a process of motor neuron degeneration by mechanisms implicating alterations in the neurobiology of the excitatory neurotransmitter glutamate and its receptors.

F-wave characteristics as surrogate markers of spasticity in patients with secondary progressive multiple sclerosis.

We sought to identify significant ulnar nerve conduction abnormalities and also to detect ulnar F-wave variable changes in patients with secondary progressive multiple sclerosis (MS). Conventional conduction study was performed unilaterally to ulnar nerves of eight men and 12 women with secondary progressive MS (mean age, 47.5 +/- 6.6 years), having spastic hemiparesis and hand spasticity. A series of 40 electrical stimuli were also delivered to their ulnar nerves unilaterally so as to obtain F waves. The side of examination was ipsilateral to the side of spastic paresis. The following F-wave variables were estimated: F-wave persistence, latency, amplitude, duration, and F chronodispersion. Ten patients with remitting-relapsing MS without any evidence of hand spasticity and 20 age- and gender-matched healthy volunteers served as controls. Motor and sensory conduction study was normal in all participants. The F-wave persistence, latency, and duration parameters and also the F chronodispersion were comparable between groups. The mean and maximum F-wave amplitude values (P = 0.005) and the F mean/M (P = 0.001) and F maximal/M (P = 0.001) ratios were significantly higher than those of controls. Finally, the F-wave amplitude parameters in patients with secondary progressive MS significantly correlated with the degree of spasticity and the duration of disease. Significant amplitude F-wave abnormalities occurred in patients with secondary progressive MS and hand spasticity, emphasizing the contribution of upper motor neurons damage in the genesis of F waves.

Myasthenia gravis initially presenting with pseudo-internuclear ophthalmoplegia.

Myasthenia gravis (MG) can rarely be manifested with ocular motility disturbances, simulating internuclear ophthalmoplegia. Pseudo-internuclear ophthalmoplegia (PINO) may occur during the course of MG, however, the initial presentation of MG with PINO in rather unlikely. We herein describe the case of a male patient who developed PINO, as an initial manifestation of MG.

Bilateral drop foot secondary to a primary tumor in the conus medullaris.

Intramedullary tumors of the spinal cord are rare neoplasms that can be associated with severe neurological and functional handicaps. To our knowledge, we describe for the first time the case of a male patient who developed bilateral drop foot as an initial manifestation of a primary tumor in the conus medullaris of the spinal cord, probably an astrocytoma.