Impairment in oral medical comprehension in multiple sclerosis.

In multiple sclerosis (MS), medical comprehension of website information and informed consent is reported to be impaired. The aim of the present study was to investigate oral medical comprehension of literal, figurative and humorous language in MS through videos of physician-patient exchanges. A group of 35 MS patients was compared to a control group (38 healthy participants). Participants were shown twelve filmed sketches consisting of a patient's question followed by the doctor's response and had to choose the meaning of the physician's response among three possibilities. Group analyses (non-parametric tests, Mann-Whitney) revealed significantly lower scores for MS patients compared to controls for figurative and humorous items. The opposite was observed for literal items. At the individual level, 17% of MS patients were impaired in their comprehension of figurative items and 32% for humorous items. Among them, 20% were impaired in both categories, 20% selectively in figurative comprehension and 60% selectively in humorous comprehension. These preliminary results suggest that a high proportion of MS patients (40%) may be impaired in the non-literal comprehension of simple medical responses. The better performances obtained for literal items suggest a more concrete linguistic decoding in MS. On a theoretical level, the double dissociations shown between figurative and humorous items in patients are not compatible with a serial model of linguistic processing.

Is there a benefit of mechanical thrombectomy in patients with large stroke (DWI-ASPECTS ≤ 5)?

BACKGROUND AND PURPOSE: Whether to withhold mechanical thrombectomy when the diffusion-weighted imaging (DWI) lesion exceeds a given volume is undetermined. Our aim was to identify markers that will help to select patients with large DWI lesions [DWI-Alberta Stroke Program Early Computed Tomography Score (DWI-ASPECTS) ≤ 5] that may benefit from thrombectomy. METHODS: From May 2010 to November 2016, 82 acute ischaemic stroke patients with DWI-ASPECTS ≤5 (43 men, 64.6 ± 14.4 years, National Institutes of Health Stroke Scale 18.4 ± 5.4) treated with state-of-the-art mechanical thrombectomy were studied. Thrombectomy alone was performed in 28 (34%) and bridging therapy in 54 (66%) patients. Recanalization was defined as a thrombolysis in cerebral infarction score 2B-3 and significant hemorrhagic transformation as parenchymal haematoma type 2 (European Cooperative Acute Stroke Study 3 classification). Pretreatment variables were compared between patients with a good (modified Rankin Scale 0-2) and a poor (modified Rankin Scale 3-6) neurological outcome at 3 months. RESULTS: Overall, 28 patients (34%) achieved good neurological outcome at 3 months. Recanalizers were significantly more likely to achieve good outcome (61% vs. 7.3%, P < 0.0001), had lower mortality (24% vs. 49%, P = 0.03) and similar rates of parenchymal haematoma type 2 (9.8% vs. 7.3%, P = 1) compared to non-recanalizers. Regression modelling identified DWI-ASPECTS >2 [odds ratio (OR) 6.93; 95% confidence interval (CI) 1.05-45.76, P = 0.04), glycaemia ≤6.8 mmol/l (OR 4.05; 95% CI 1.09-15.0, P = 0.03) and thrombolysis (OR 3.67; 95% CI 1.04-12.9, P = 0.04) as independent predictors of good neurological outcome. CONCLUSIONS: In patients with DWI-ASPECTS ≤5, two-thirds of patients experienced good neurological outcome when recanalized by state-of-the-art thrombectomy, whilst only one in 14 non-recanalizers achieved similar outcomes. Pretreatment markers of good neurological outcomes were DWI-ASPECTS >2, intravenous thrombolysis and glycaemia ≤6.8 mmol/l.

[Multiple sclerosis and verbal episodic memory: Critical review of cognitive processes and their assessment]. / Atteinte de la mémoire épisodique verbale dans la sclérose en plaques: revue critique des processus cognitifs concernés et de leur exploration.

Memory impairment, especially verbal episodic memory (VEM), represents a common ground for cognitive complaint in patients with multiple sclerosis (MS). Beyond the difficulty caused in daily life, these deficits may impact on occupational activities. Neuropsychological assessment of these patients has to include VEM tests, to describe the level of dysfunction of the different processes contributing to VEM and, if required, to guide adapted cognitive rehabilitation. The objective of the present paper is to propose a critique review of the literature on VEM abilities in MS. This review will present the conceptual references and the psychometric characteristics of the main VEM tests applied in MS (isolated tests or included within more general batteries developed specifically for MS). In a second phase, we propose an inventory of work on MS presented as a function of the cognitive processes involved. This approach provides an approach to the limitations of each conception and possible terminological ambiguities. Contributions to knowledge of MS memory impairments will be clarified, as well as the impact of the disease characteristics (MS forms, disease duration, EDSS).

[Hashimoto's encephalopathy: a rare cause of status epilepticus]. / Encéphalopathie de Hashimoto : une cause rare d'état de mal épileptique.

Hashimoto's Encephalopathy (HE) is a rare condition defined by the association of encephalopathy and autoimmune thyroiditis with increased levels of antithyroid antibodies. Presenting symptoms of HE may be quite variable. Although seizures are rather frequent, status epilepticus seems very rare (10 reported cases to date) and exceptionally revealing. We report the case of a 48-year-old female, who presented with a series of status epilepticus. The only positive result of the initial exploration was an increased level of antithyroid antibodies. The patient's condition improved only after initiation of corticosteroid treatment, which provided a stable remission. HE pathophysiology still remains poorly understood and controversial. Pathological data are sparse and provide variable pictures. Although an autoimmune mechanism looks very likely, the precise role of antithyroid antibodies is still discussed. A direct toxicity has not been demonstrated and antibodies could simply be a marker of impaired immunity. Occurrence of status epilepticus in HE could be related to the presence of active inflammation of cortical or para-cortical tissue, although such a hypothesis remains to be demonstrated. The diagnosis of HE should be systematically considered in patients with unexplained episodes of status epilepticus. Search for abnormal thyroid function and increased levels of antithyroid antibodies allow both early diagnosis and treatment, with a clear benefit for the patients.

A young adult with sarcosinemia. No benefit from long duration treatment with memantine.

Sarcosinemia is a rare inborn error of metabolism that is characterised by an increased level of sarcosine (N-methylglycine) in the plasma and urine. The enzymatic block results from a deficiency of sarcosine dehydrogenase (SarDH), a liver mitochondrial matrix enzyme that converts sarcosine into glycine. Although this condition may remain inapparent until later life, it has been reported in rare cases to lead to neurodevelopmental disability. A 19-year-old male with sarcosinemia presented with dystonia, developmental delay and cognitive impairment. Magnetic resonance imaging revealed vermian hypotrophy. A 2-year pharmacological treatment with memantine was negative on the clinical signs. In this case, it was concluded that the metabolic block leading to sarcosinemia was responsible of a pathologic condition with mental deficiency and complex neurological signs. A maternal isodisomy discovered in the vicinity of SarDH gene could contribute to this pathology. Deficit of SarDH may be considered as a differential diagnosis of growth failure during prenatal stages and respiratory failure at birth following a slowly progressive developmental delay.

Recognition of facial and musical emotions in Parkinson's disease.

BACKGROUND AND PURPOSE: Patients with amygdala lesions were found to be impaired in recognizing the fear emotion both from face and from music. In patients with Parkinson's disease (PD), impairment in recognition of emotions from facial expressions was reported for disgust, fear, sadness and anger, but no studies had yet investigated this population for the recognition of emotions from both face and music. METHODS: The ability to recognize basic universal emotions (fear, happiness and sadness) from both face and music was investigated in 24 medicated patients with PD and 24 healthy controls. The patient group was tested for language (verbal fluency tasks), memory (digit and spatial span), executive functions (Similarities and Picture Completion subtests of the WAIS III, Brixton and Stroop tests), visual attention (Bells test), and fulfilled self-assessment tests for anxiety and depression. RESULTS: Results showed that the PD group was significantly impaired for recognition of both fear and sadness emotions from facial expressions, whereas their performance in recognition of emotions from musical excerpts was not different from that of the control group. The scores of fear and sadness recognition from faces were neither correlated to scores in tests for executive and cognitive functions, nor to scores in self-assessment scales. CONCLUSION: We attributed the observed dissociation to the modality (visual vs. auditory) of presentation and to the ecological value of the musical stimuli that we used. We discuss the relevance of our findings for the care of patients with PD.

Mechanical thrombectomy in acute stroke: prospective pilot trial of the solitaire FR device while under conscious sedation.

BACKGROUND AND PURPOSE: The best approach between general anesthesia and conscious sedation to perform mechanical thrombectomy remains unknown. The goal of our study was to evaluate the feasibility, safety, and efficacy of mechanical thrombectomy under conscious sedation in patients with acute ischemic stroke, using the Solitaire FR device, in a prospective, single-center, single-arm study. MATERIALS AND METHODS: The study included consecutive patients with acute ischemic stroke due to a large artery occlusion within 6 hours of symptom onset for the anterior circulation, and within 24 hours for the posterior circulation. After intravenous thrombolysis (when no contraindications), thrombectomy was performed with the Solitaire device in patients under conscious sedation. Primary efficacy and safety end points were good functional outcome (mRS ≤2) at 3 months and mortality at 3 months. Secondary end points were recanalization (TICI ≥2) and failure rate. RESULTS: From May 2010 to July 2011, 36 patients were treated. Median baseline NIHSS score was 17.5. The occlusion site was MCA in 21 patients (58.4%), ICA-MCA tandem occlusion in 9 patients (25.0%), terminal ICA in 2 patients (5.5%), and basilar artery in 4 patients (11.1%). Twenty-three patients (63.9%) received intravenous thrombolysis. Superselective catheterization of the occluded vessel was not feasible in 5/36 cases (13.9%). Successful revascularization was achieved in 28/36 patients (77.8%). After 3 months, 22 patients (61.1%) showed good functional outcome (mRS ≤2) and the median NIHSS score was 8.5. Overall mortality rate at 3 months was 22.2% (8/36). CONCLUSIONS: In acute ischemic stroke, mechanical thrombectomy while under conscious sedation is feasible in a large percentage of cases (86.1%) and is associated with a short procedure delay and a high percentage of good functional outcomes at 3 months (61.1%).

[Acquired and developmental Gerstmann syndrome. Illustration from a patient with multiple sclerosis]. / Syndrome de Gerstmann acquis ou développemental. Illustration chez une patiente atteinte de sclérose en plaques.

Gerstmann's syndrome (GS) is defined by a clinical tetrad including acalculia, finger anomia, left-right disorientation and agraphia. In this article, we describe the case of a 42-year-old woman suffering from an aggressive relapsing-remitting multiple sclerosis in which a systematic neuropsychological assessment revealed Gertsmann's syndrome amongst other cognitive disturbances. Brain MRI showed a high concentration of plaques within a left subcortical parietal region that has recently been considered as a crucial node for GS appearance. However, history, taking provided information suggesting that an important part of the GS, may have been present since childhood, evoking a possible neurodevelopmental origin in this patient. This article reviews the role of the GS concept in contemporary literature, with a special attention to pathophysiological hypotheses and to precautions necessary to study such cases.

[NeuroBehcet disease with corpus callosum involvement]. / Neuro-Behçet avec atteinte du corps calleux.

INTRODUCTION: Behçet's disease is a multi-system vascular-inflammatory disease with possible involvement of the central nervous system. Lesions of the corpus callosum on MRI have been rarely reported in this disease. CASE REPORT: A 47-year-old woman was admitted for a sudden right hemiplegia and confusion revealing a Behcet's disease. MRI showed a pedonculo-thalamic lesion and a white matter hypersignals, which was suggestive of the disease. Besides, involvement of the corpus callosum was observed. CONCLUSION: This case demonstrates that Behcet's disease should be considered among diseases with corpus callosum involvement.

Memantine treatment in patients with mild to moderate Alzheimer's disease: results of a randomised, double-blind, placebo-controlled 6-month study.

Memantine is a moderate affinity, uncompetitive NMDA receptor antagonist currently approved for the treatment of moderate to severe Alzheimer's disease (AD). A 24-week, double-blind, placebo-controlled, study (Study 99679) conducted in Europe evaluated the efficacy and tolerability of 20 mg/day memantine in patients with mild to moderate AD. Patients were randomised to either memantine or placebo in a 2:1 ratio. Efficacy was primarily assessed as change from baseline in ADAS-cog and CIBIC-plus score. Of 470 patients randomised and treated (memantine, n=318; placebo, n=152), 85% and 91% completed the study. Memantine-treated patients showed statistically significant improvement relative to placebo at weeks 12 and 18, and numerical superiority at week 24 on both efficacy scales. The lack of significance at week 24 was attributed to an unexpectedly high placebo response. Memantine was well tolerated with an adverse event profile similar to placebo. The data presented support the efficacy of memantine in mild to moderate AD.

[Effects of the association of sulbutiamine with an acetylcholinesterase inhibitor in early stage and moderate Alzheimer disease]. / Effets de l'association de la Sulbutiamine à un inhibiteur de l'acétylcholinestérase dans les formes légères à modérées de la maladie d'Alzheimer.

The efficacy of the inhibitors of acetylcholinesterase in Alzheimer's Disease (AD) is moderated and some patients do not respond to these treatments. Sulbutiamine potentializes cholinergic and glutamatergic transmissions, mainly in hippocampus and prefrontal cortex. This multicentric, randomized and double-blind trial evaluates the effects of the association of sulbutiamine to an anticholinesterasic drug in cognitive functions in patients with AD at an early stage (episodic memory, working memory, executive functions, attention). Patients had first donepezil (D) or sulbutiamine (S) during three months. During this period, only attention improved in both groups. During the three following months, a placebo (P) in patients D and donepezil in patients S were added. Compared to entry results, episodic memory decreased in group D + P but improved in group S + D. At the same time the improvement of attention persisted in both groups. Daylife activities only improved in group S + D. In conclusion sulbutiamine can be an adjuvant to treatment in early stage and moderate AD by anticholinesterasic drugs.

Memantine treatment in patients with mild to moderate Alzheimer's disease: results of a randomised, double-blind, placebo-controlled 6-month study.

Memantine is a moderate affinity, uncompetitive NMDA receptor antagonist currently approved for the treatment of moderate to severe Alzheimer's disease (AD). A 24-week, double-blind, placebo-controlled, study (Study 99679) conducted in Europe evaluated the efficacy and tolerability of 20mg/day memantine in patients with mild to moderate AD. Patients were randomised to either memantine or placebo in a 2:1 ratio. Efficacy was primarily assessed as change from baseline in ADAS-cog and CIBIC-plus score. Of 470 patients randomised and treated (memantine, n=318; placebo, n=152), 85% and 91% completed the study. Memantine-treated patients showed statistically significant improvement relative to placebo at weeks 12 and 18, and numerical superiority at week 24 on both efficacy scales. The lack of significance at week 24 was attributed to an unexpectedly high placebo response. Memantine was well tolerated with an adverse event profile similar to placebo. The data presented support the efficacy of memantine in mild to moderate AD.

Health status in patients with Alzheimer's disease: an investigation of inter-rater agreement.

OBJECTIVE: The purpose of the present study was to examine the level of agreement between health status ratings provided by patients with Alzheimer's disease and by their proxies. BACKGROUND: Because proxy-completed responses are often necessary in assessing health outcomes for the elderly, it is necessary to determine the feasibility and potential limitations of using proxies as a patient substitutes. METHODS: To assess the potential utility of proxy responses on health status when subjects present a cognitive impairment, this study compared the responses of 70 subjects with Alzheimer's disease and those of their family and/or care provider proxy using the SF-36. Agreement between proxies and patients was measured by intraclass correlation coefficients (ICCs). RESULTS: The proportion of exact agreement between patients and proxies on the 36 items ranged from 3.3 to 41.7%. Results reveal poor to moderate agreement between patient and proxy reports. Proxy reliability varied according to the relationship of the proxy to the index subject. Agreement decreased significantly with increasing severity of dementia and with increasing severity of Physical status (Katz ADL). Agreement was better for measures of functions that are directly observable and relatively poor for more subjective measures. CONCLUSIONS: Our results confirm the importance of the information source used for patient health status.

Implicit memory for words presented in short texts is preserved in Alzheimer's disease.

BACKGROUND: The level of efficiency of implicit memory in Alzheimer's disease remains unclear as previous studies using stem completion tasks have led to contradictory results. METHOD: The present study used target words embedded in significant short texts that subjects were required to read aloud (i.e. to enhance semantic processing). Texts were presented in two perceptual situations: 'simple' (blank spaces delimitating words) and complex' (spaces were filled by '8's). In the completion phase, patients had to write the first word that came to mind in order to complete a three-letter stem. The recognition phase explored explicit memory performance. The performance of 24 Alzheimer patients was compared to a matched sample of healthy controls. RESULTS: Reading times differed between groups and were shorter for healthy controls. Recognition was dramatically lower in patients, thus confirming the alteration of explicit memory in this pathology. However, a significant priming effect (e.g. the tendency to complete the stem with the aid of a previously explored word) was present in both groups and did not differ between patients and healthy controls. CONCLUSIONS: The absence of a correlation between priming and recognition scores suggests that this result cannot be explained by an explicit memory bias. Moreover, as the priming level was identical whatever the perceptual aspect of the text, we suggest that the priming effect is not only mediated by perceptual processes but also by lexical and conceptual processes, which to some extent are preserved during the light and moderate stages of this disease.

Dissociation of reading strategies: letter-by-letter reading in the native language and normal reading in the learned language. A case study.

In letter-by-letter reading, which is typically observed in Dejerine's (1892) "pure alexia," oral reading seems to be mediated by the naming of the constituent letters of the printed sequence: reading time rises abnormally as a function of the number of letters of the target item. We describe a patient with fluent aphasia who showed the unusual pattern of letter-by-letter reading together with surface dyslexia in her native language (French) and apparently normal reading in the second, learned language (English). Thus, letter-by-letter reading should be considered as a description of a symptom rather than the consequence of a unique type of functional impairment.

[Four "alien" hands for two hands after a lesion in corpus callosum]. / Quatre mains "étranges" pour deux mains après une lésion calleuse.

Callosal lesions, associated or not to internal frontal lesions, may produce different types of complex gestural behaviors. Four signs can be identified, each of which has been generally reported separately: the "alien hand" sign, the "diagnostic apraxia", the "wayward hand" and the "callosal apraxia". Some authors justify considering these signs as different entities, while others propose regrouping them either in an unique syndrome--the "alien hand"--or as two syndromes--the "frontal alien hand" and the "callosal alien hand". We present the observation of a patient who presented with the four mentioned syndromes in association. In this context, we review the clinical features of each of the four signs and the arguments supporting their individualization.