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).

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.

[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.

[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.

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.

[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.

Heterogeneity and selectivity of the degeneration of cholinergic neurons in the basal forebrain of patients with Alzheimer's disease.

Cholinergic neurons were studied by immunohistochemistry, with an antiserum against choline acetyltransferase (ChAT), in the basal forebrain (Ch1 to Ch4) of four patients with Alzheimer's disease (AD) and four control subjects. ChAT-positive cell bodies were mapped and counted in Ch1 (medial septal nucleus), Ch2 (vertical nucleus of the diagonal band), Ch3 (horizontal nucleus of the diagonal band) and Ch4 (nucleus basalis of Meynert). Compared to controls, the number of cholinergic neurons in AD patients was reduced by 50% on average. The interindividual variations in cholinergic cell loss were high, neuronal loss ranging from moderate (27%) to severe (63%). Despite the small number of brains studied, a significant correlation was found between the cholinergic cell loss and the degree of intellectual impairment. To determine the selectivity of cholinergic neuronal loss in the basal forebrain of AD patients, NPY-immunoreactive neurons were also investigated. The number of NPY-positive cell bodies was the same in controls and AD patients. The results (1) confirm cholinergic neuron degeneration in the basal forebrain in AD and the relative sparing of these neurons in some patients, (2) indicate that degeneration of cholinergic neurons in the basal forebrain contributes to intellectual decline, and (3) show that, in AD, such cholinergic cell loss is selective, since NPY-positive neurons are preserved in the basal forebrain.

Alexia without agraphia in a left-handed patient with a right occipital lesion.

A left-handed patient who had suffered an ischemic vascular accident in the territory of the right posterior cerebral artery displayed alexia without agraphia. Clinical examination of this alexia revealed semiological characteristics comparable with those observed in cases of left temporo-occipital lesions in right-handed patients. Analysis of these disturbances indicated that they were agnosic in nature. The associated disturbances included problems of visual representation and topographic memory together with an amnesic syndrome.

Manic-like state after bilateral orbitofrontal and right temporoparietal injury: efficacy of clonidine.

A manic-like state occurred in a 44-year-old right-handed woman with bilateral orbitofrontal and right temporoparietal traumatic contusions. In a brief trial, we assessed the effect of clonidine, carbamazepine, dopa therapy, and placebo on manic symptoms and cognitive functions. Clonidine rapidly reversed the manic syndrome. The patient's behavior did not change with carbamazepine and worsened with levodopa. We suggest that the manic-like syndrome was related to noradrenergic overactivity secondary to the fronto-orbital lesions.

Paraspinal arteriovenous fistula with perimedullary venous drainage.

A paravertebral presacral posttraumatic arteriovenous fistula drained through the ascending lumbar vein to the epidural plexuses and perimedullary veins. The patient did not have symptoms of myelopathy, only low-back pain and radicular hypoesthesia. The lesion was embolized with a large balloon and clinical symptoms disappeared.

PET studies of cortical diaschisis in patients with motor hemi-neglect.

Six patients presenting with multimodal, predominantly motor hemi-neglect, were investigated by 15O2 or 18F-DG and PET to study the local cerebral metabolism in intact regions. All had suffered from an acute right-sided (n = 5) or left-sided (n = 1) focal hemispheric lesion (cortico-subcortical and purely subcortical in three patients each). Frontal and parietal cortices on the side of the lesion were significantly hypometabolic relative to the other side in each case, presumably as a result of diaschisis, whereas thalamic hypometabolism was significant in only 4 patients. Compared to absolute metabolic rates for controls, the uninjured hemisphere showed a trend towards hypometabolism. Hence, true hyperactivity of the contralateral hemisphere with respect to the ipsilateral cerebral cortex was not associated with motor hemineglect in this series. On the contrary, hemineglect was associated with a widespread hypometabolism of the entire ipsilateral cerebral cortex in the context of a moderately reduced metabolism of the ipsilateral thalamus and contralateral cerebral cortex. These findings are consistent with the implication of a cortico-subcortical network serving attention.

Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndromes.

We describe eight patients with slowly progressive speech production deficit combining speech apraxia, dysarthria, dysprosody and orofacial apraxia, and initially no other deficit in other language and non-language neuropsychological domains. Long-term follow-up (6-10 years) in 4 cases showed an evolution to muteness, bilateral suprabulbar paresis with automatic-voluntary dissociation and frontal lobe cognitive slowing without generalised intellectual deterioration. Most disabled patients presented with an anterior opercular syndrome (Foix-Chavany-Marie syndrome), and pyramidal or extrapyramidal signs. CT and MRI findings disclosed asymmetric (left > right) progressive cortical atrophy of the frontal lobes predominating in the posterior inferior frontal region, notably the operculum. SPECT and PET revealed a decreased cerebral blood flow and metabolism, prominent in the left posterior-inferior frontal gyrus and premotor cortex, extending bilaterally in the most advanced cases. Pathological study of two cases showed non-specific neuronal loss, gliosis, and spongiosis of superficial cortical layers, mainly confined to the frontal lobes, with no significant abnormalities in the basal ganglia, thalamus, cerebellum, brain stem (except severe neuronal loss in the substantia nigra in one case), and spinal cord. We propose to call this peculiar syndrome Slowly Progressive Anarthria (SPA), based on its specific clinical presentation, and its metabolic and pathological correlates. SPA represents another clinical expression of focal cortical degeneration syndromes, that may overlap with other similar syndromes, specially primary progressive aphasia and the various frontal lobe dementias.

[Extrapyramidal syndrome during treatment with flunarizine]. / Syndrome extra pyramidal au cours d'un traitement par la flunarizine.

A 68 year-old woman had been taking flunarizine 10 mg daily for 10 weeks when she developed severe bradykinesia and rigidity, resting tremor of both hands, akathisia, buccolinguofacial dyskinesias and depressed mood. Flunarizine was discontinued. After 3 months the patient was asymptomatic. This case and data from the literature suggest that extrapyramidal symptoms and depression may be observed even at the recommended daily dose of 10 mg. Flunarizine should be avoided in patients with Parkinson's disease. Patients on flunarizine should be watched for depressive and extrapyramidal signs, especially those aged over 60.

[Complete human Klüver-Bucy syndrome after encephalitis caused by herpes simplex type 2]. / Syndrome de Klüver-Bucy humain complet après une encéphalite à herpès simplex type 2.

A case of complete Klüver-Bucy syndrome is reported. A 42 year-old man developed Herpes Simplex type II (H.S.V.II) encephalitis. Good sparing of language functions allowed thorough neuropsychological testing. The troubles usually described in Klüver-Bucy syndrome as psychic blindness, colour agnosia, prosopagnosia, auditive and tactile agnosia were present. The authors theorize that these symptoms are mainly correlated with the amnestic syndrome, which is constantly reported in human Klüver-Bucy syndrome. Unfortunately, after 8 weeks, the encephalitis recurred and the patient was left demented and untestable.

[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.