Elena Carapelle

Potential links between neurological disease and Tako-Tsubo cardiomyopathy: A literature review

Tako-Tsubo cardiomyopathy (TTC), is defined as a fully reversible acute deterioration of left-ventricular (LV) function, which is mainly found in women after an episode of emotional or physical stress (e.g. psychosocial stress, sepsis, surgery). The underlying mechanisms remain unclear. There is evidence suggesting a possible link between neurological disease and TTC. The pathophysiology of the several neurologic diseases has been reviewed searching for possible mechanisms that could lead to TTC in these patients.

Intravenous lacosamide in seizure emergencies: Observations from a hospitalized in-patient adult population

PURPOSE to evaluate the efficacy and safety of intravenous (IV) lacosamide (LCM) in the treatment of seizure clusters (SC) and status epilepticus (SE) in hospitalized adult patients. METHODS we prospectively analyzed treatment response, seizure outcome, and adverse effects of IV LCM in 38 patients with seizure emergencies (15 with SC, 23 with SE) during a hospital stay. The loading dose of IV LCM was 200-400mg and the maintenance dose was 200-400mg daily. Response to IV LCM was evaluated within 20min, 4h and 24h of LCM infusion. RESULTS an acute anti-seizure effect after IV LCM was especially evident when it was first used - (SC) or second line (established SE) treatment. In particular, 87% of SC patients (13/15) and 80% of established SE (8/10) demonstrated response to LCM treatment, while no patients with super-refractory SE (0/8) responded to IV LCM according to our criteria. The loading of IV LCM was well tolerated, with mild adverse effects (2/38 temporary dizziness). In most patients, during and after administration of the loading dose of IV LCM a temporary (30min-1h) sedation was observed. No ECG and laboratory values-changes were documented in any of the patients. CONCLUSIONS LCM is an effective and well-tolerated treatment when used to treat SC in hospitalized adult patients. As add-on therapy, it may be useful to stop seizure activity in patients with focal SE not responding to first/second-line intravenous AEDs.

How the cognitive reserve interacts with β-amyloid deposition in mitigating FDG metabolism: An observational study.

Abstract This observational study had the aim to assess the interaction between cognitive reserve (CR) and cerebrospinal fluid &bgr;-amyloid1-42 (A&bgr;1-42) in modulating brain [18F]fluorodeoxyglucose positron emission tomography (FDG-PET) metabolism in patients with moderate Alzheimer disease (AD). Twenty-seven patients with probable AD and 25 neurological normal subjects (NNS) entered the study. All participants had an FDG-PET scan, and AD patients also received a lumbar puncture to measure A&bgr;1-42, 181p-tau, and Tau concentrations. Based on years of formal education, AD patients were classified as highly educated-AD (years of formal education >5) or less educated-AD (years of formal education <5). By using a voxel-wise approach, we first investigated differences in the cerebral glucose uptake between AD and NNS, then we assessed the interaction between level of education (a proxy of CR) and cerebrospinal fluid biomarkers on FDG-PET metabolism in the patient groups. Significantly lower glucose uptake was observed in the posterior cingulate gyrus, in the precuneus, in the inferior and medial temporal gyrus, and in the inferior parietal lobule of AD patients compared with NNS. A significant interaction was found between CR and A&bgr;1-42 values on brain metabolism in the inferior and medial temporal gyrus bilaterally. The AD patients with higher CR level and marked signs of neuropathology showed glucose hypometabolism in regions typically targeted by AD pathology. This finding supports the hypothesis that CR partially compensates for the effect of A&bgr; plaques on cognitive impairment, helps in patients’ clinical staging, and opens new possibilities for the development of nonpharmacological interventions.

Partial Trisomy 18q and Epileptic Spasms Induced by Eating Associated With Bilateral Opercular Dysplasia

In2007,wedescribedtheclinicalandvideo-polygraphicfeaturesofa patient with reflex periodic spasms triggered by eating [d’Orsietal.,2007].Thepatientwasa30-year-old,right-handedmanwithsevereasphyxiaatbirthandsignificantintellectualdisability.Fromthe age of 25, he began to experience generalized tonic–clonicseizures,atonicdropattacksandeating-inducedrepetitiveepilepticspasmscharacterizedbysuddenheadandupperlimbsdropping.Inparticular, during the longer spasms (at least 1sec) the ictalpolygraphy showed a diffuse slow wave complex prevalent onanterior regions with an activation in crescendo-decrescendo onthe neck and the right sternocleidomastoideus muscles. Subse-quently, chromosome analysis, fluorescence in situ hybridization(FISH), and array comparative genomic hybridization (array-CGH) were performed. The array-CGH was carried out usingthe GenomeARRAY slide (TechnoGenetics Srl-Bouty Spa, Italy),containing 5,380 BAC clones, with a genomic resolution ofabout 0.5Mb, and increasing to 0.25Mb in the subtelomericregions. These studies revealed the presence of a partialtrisomy of chromosome 18 in the 18 q12.2q22.3 region (partialtrisomy 18q; see Fig. 1). The parents had normal karyotypes andarray-CGH.The relationship between chromosomal 18 anomalies and epi-lepsy was evaluated in a previous review [Grosso et al., 2005], andpartial seizures and focal EEG abnormalities were observed inpatients with 18 qDS, trisomy 18p and translocation betweenchromosome17and18.Epilepsyonsetinpatientscarryingtrisomy18p is usually during infancy, and focal seizures remitted in themajority. EEG pattern disclosed symmetric or asymmetric parox-ysmal activity in posterior regions associated with generalizedspikes and spike and waves, while brain MRI showed agenesis ofthe splenium and thin of corpus callosum.Toourknowledge,nopreviouscaseshavebeenreportedregardingthe relationship between partial trisomy 18q and epilepsy, andour patient expands the clinical spectrum and genomic character-izationoftrisomy18.Infact,fromaclinicalpointofviewageseizureonset (25 years) and seizure semiology (epileptic spasms inducedbyeating)differentiateourcasefromothersreportedintheliterature-[Grosso et al., 2005]. In addition, genetic testing based on array-CGH and FISH have revealed additional material on chromosome18, specificallya partialduplication of the longarm of chromosome18 (trisomy of 18q12.2q22.3 region), not previously associatedwith epileptic phenotype. Deletion of genes located in the distalportion of 18q seems to be important in conferring susceptibilityfor the clinical features, including epilepsy [Strathdee et al., 1995].Deletion of the long arm of the chromosome 18 is associated withepilepsy witha frequency ranging from 10% [Wilson et al., 1979] to31%[Strathdeeetal.,1995].Notablythepatientshavebeenreportedin the literature with partial duplication of the short arm of chro-mosome 18 and epilepsy, but no previous cases have been reported

Left Ventricular Thrombi in Takotsubo Syndrome: Incidence, Predictors, and Management: Results From the GEIST (German Italian Stress Cardiomyopathy) Registry

Background Left ventricular (LV) thrombi during Takotsubo syndrome represent a potential complication and can be associated with cerebrovascular embolic events. The aim of this study was to evaluate the exact incidence, predictors, and management strategies of LV thrombi in patients with Takotsubo syndrome. Methods and Results We enrolled 541 consecutive patients in a multicenter international registry. Clinical features and echocardiographic data at admission, during hospitalization, and after 3 months were evaluated. Survival rates for long‐term follow‐up (mean 984±908 days) were recorded. Twelve Takotsubo syndrome patients (2.2%) developed LV thrombi (all female presenting with apical ballooning pattern). All patients with LV thrombi were treated with oral anticoagulation therapy; however, 2 (17%) had a stroke before treatment initiation. These patients were characterized by a higher prevalence of ST‐elevation (56% versus 16%; P<0.001) and higher troponin I levels (10.8±18.3 ng/mL versus 3.5±4.3 ng/mL; P=0.001) as compared with those without LV thrombi. At multivariate analysis including age, sex, LV ejection fraction, ST‐elevation at admission, and apical ballooning pattern, troponin I level >10 ng/mL was the only predictor for LV thrombosis (hazard ratio 6.6, confidence interval, 1.01–40.0; P=0.04). After 3 months all LV thrombi disappeared. Oral anticoagulation therapy was interrupted in all patients except 1. At long‐term follow‐up, the survival rate was not different between patients with and without LV thrombi (84% versus 85%; P=0.99). Conclusions LV thrombi have a relatively low incidence among patients with Takotsubo syndrome and were detected in female patients with apical ballooning pattern and increased troponin levels. Oral anticoagulation therapy for 3 months seems reasonable in these high‐risk patients.

Early recurrence of Tako-Tsubo cardiomyopathy in an elderly woman with amyotrophic lateral sclerosis: different triggers inducing different apical ballooning patterns

&NA; We report the case of early recurrence of Tako-Tsubo cardiomyopathy in an elderly woman with amyotrophic lateral sclerosis triggered by different stressors. A first episode with typical apical ballooning was anticipated by an emotional stress; a second, characterized by systolic anterior motion of the mitral valve associated with mitral regurgitation and severe intra-ventricular gradient, was precipitated by surgical stress and hypovolemia. We therefore hypothesize both a possible link between amyotrophic lateral sclerosis and Tako-Tsubo cardiomyopathy, and between different stressors and different Tako-Tsubo patterns.

Wernicke's encephalopathy following reduced food intake due to depressive disorders

Wernickes encephalopathy (WE) is an unexpected common neurological disorder caused by thiamine deficiency often due to alcohol abuse, but WE-not alcohol related is also frequent. A prolonged reduction of food intake can cause WE. This condition can arise in depression disorders, especially in the early stages of these psychiatric syndromes. WE is characterized by the triad of signs: ataxia, ocular dysfunctions and confusional state. However, they rarely appear together and this makes the diagnosis particularly difficult, especially when there is not a history of alcohol abuse. Electroencephalography, since in the early stage of the disease, can be helpful in detecting pattern of metabolic encephalopathy. We describe three cases of thiamine deficiency responsible of WE, caused by a decrease in appetite and food intake due to the onset of a depressive disorder. In our series, the most frequent symptom observed at the onset of the disease was the motor incoordination. We recommend to perform quickly thiamine infusion in all depressed patients with a history of reduced food intake, presenting to Emergency Department with recent onset of motor incoordination, with or without alterations in eyes’ movements and confusional state, after exclusion of other neurological conditions.

Bilateral putaminal necrosis and bronopol toxicity

Among alcohols, methanol intoxication is the most frequently associated with cerebral toxicity, causing retinal damage and putaminal necrosis. This consequence is believed to be due to the transformation of methanol into formic acid. We describe the case of a patient who presented with acute impairment of consciousness and tetraparesis after she had been drinking several bottles of a topical antiseptic solution (Lysoform Medical) containing 2-bromo-2-nitro-1,3-propandiol (bronopol) among excipients, in order to lose weight during previous months. Moreover, she had been on a strict slimming diet. Soon after admission, a severe respiratory and metabolic impairment became rapidly evident, requiring an intensive care unit admission. Cerebral MRI showed the presence of bilateral putaminal necrosis. She recovered in 10 days, surprisingly, without any evident clinical neurological signs. Methanol, also bronopol, when diluted in aqueous solution, at warm temperature and/or higher pH, may release formaldehyde, which is converted into formic acid, a basal ganglia toxic compound.