Tabes dorsalis in the 19th century. The golden age of progressive locomotor ataxia.

Tabes dorsalis, a late neurological complication of syphilis, is nowadays almost extinct. The path to understanding this disease and its pathophysiology was long and winding, spanning multiple centuries. The 19th century was a crucial period for understanding it. In the first third of the century, German and French physicians defined the semiology of tabes dorsalis, renamed in France "ataxie locomotrice progressive [progressive locomotor ataxia]." Nevertheless, the multiplicity of ancient and recent terms and the description of sometimes unclear nosological concepts (tabes nervosa, tabes spasmodic, nervo-tabes, etc.) were a hindrance to understanding it. Tabes dorsalis was a fertile ground for the description of many clinical signs that have become classics in medicine. No real treatment was available and various unusual therapies were performed. For a long time, the etiology of this disease remained unknown. The link between syphilis and tabes dorsalis was slowly established in the second part of the 19th century from epidemiologic observations. We present an overview of the concept of tabes dorsalis in the medical context of the 19th century and discuss the medical observations of some famous patients suffering from the disease such as Édouard Manet (1832-1883) and Alphonse Daudet (1840-1897).

The medical itineraries of Blaise Cendrars. Neuropsychiatry marks life and literature.

Neuropsychiatry had a profound impact on the life and work of one of the most influential French writers of the 20th century, Frédéric Sauser, better known by his pen name Blaise Cendrars (1887-1961). Cendrars, whose right writing hand was amputated after a battlefield wound in 1915, described with acuity his stump pain and phantom limb syndrome. He became a left-handed writer. Between 1956 and his death in 1961, he also suffered two strokes that progressively paralyzed his left side and greatly diminished his ability to speak. Cendrars had started medical school in his youth and found that his ideas about the genesis of mental disorders conflicted with the generally accepted psychiatric conceptions of hysteria or psychoanalysis. His theories were greatly enriched by his observations of fellow World War I soldiers, victims of neuropsychiatric disorders. In his novels, many of his characters had borderline conditions, including two spectacularly mad serial killers, Moravagine and Fébronio. The case of Moravagine, fashioned after a patient with a brain tumor, allowed Cendrars to examine the nebulous frontier between neurological and psychiatric diseases.

The ASCOD phenotyping of ischemic stroke (Updated ASCO Phenotyping).

ASCO phenotyping (A: atherosclerosis; S: small-vessel disease; C: cardiac pathology; O: other causes) assigns a degree of likelihood of causal relationship to every potential disease (1 for potentially causal, 2 for causality is uncertain, 3 for unlikely causal but the disease is present, 0 for absence of disease, and 9 for insufficient workup to rule out the disease) commonly encountered in ischemic stroke describing all underlying diseases in every patient. In this new evolution of ASCO called ASCOD, we have added a 'D' for dissection, recognizing that dissection is a very frequent disease in young stroke patients. We have also simplified the system by leaving out the 'levels of diagnostic evidence', which has been integrated into grades 9 and 0. Moreover, we have also changed the cutoff for significant carotid or intracranial stenosis from 70% to more commonly used 50% luminal stenosis, and added a cardiogenic stroke pattern using neuroimaging. ASCOD captures and weights the overlap between all underlying diseases present in ischemic stroke patients.

Creatividad pictórica en pacientes de ictus / Pictorial creativity in patients who have suffered a stroke

La biología del arte visual proporciona a los neurocientíficos, a los neurólogos y también a los artistas una herramienta para entender la creación y la apreciación de las artes visuales. Tanto los productores de arte como los consumidores del mismo necesitan entender la complejidad de esta actividad biológica y sus connotaciones emocionales, culturales y conductuales. Como son frecuencia la enfermedad se convierte en un impulso para explorar lo desconocido, la existencia de cambios en la producción artística de artistas plásticos como pintores o escultores se ha convertido en un estímulo para el desarrollo de la neuroestética. En este artículo presentamos una visión general del impacto que tiene el ictus en personas sin experiencia artística y en artistas productores de arte visual (AU)

Biology of visual art provides neuroscientists, neurologists as well as artists with a tool to understand artistic creation and appreciation of visual art. Both parties the art producers and art consumers need to understand the complexity if this biological activity and its emtional, cultural and behavioural connotations. As disease is frequently a drive for exploration of the unknown the occurrence of change in artistic output in visual artists such as painters, sculptors became a stimulus for development of neuroesthetics. In this paper we present an overview of impact of stroke in art naïve people and artists on the visual art production (AU)

Antoine Ritti (1844-1920), forgotten alienist and innovative theorist on the pathophysiology of hallucinations.

Antoine Ritti (1844-1920), now forgotten, was an "alienist" or psychiatrist who formulated a theory of hallucination based on thalamic dysfunction, as described in his thesis defended in 1874. Ritti was a student of Jules Luys and used the anatomical-functional discoveries of his teacher to explain that an automatic activity in the thalamus, by stimulating the cortex without reception of sensory information, created autonomous representations, perceived by the patient but not by his entourage, a process occurring spontaneously to some degree. Hence, Ritti seems the first author to introduce the concept of sensory deprivation and release of subcortical function into the pathophysiology of hallucinations. This innovative theory, which gave subcortical structures a role in high-level cognitive function, is very resonant today but was ignored for several decades after Ritti published his work.

'The adventure': Charles-Ferdinand Ramuz's extraordinary stroke diary.

The famous Swiss writer Charles-Ferdinand Ramuz suffered a stroke at 65 years, which he called 'the adventure' or 'the accident'. He developed language disturbances suggesting crossed aphasia in a right hander with left hemiparesis. This uncommon pattern allowed him to continue to write his diary and to report his disturbances, with a unique depth and precision, especially for cognitive-emotional changes. Language and motor dysfunction recovered within a few weeks, but Ramuz complained of persisting emotional flattening alternating with irritability, fatigue, depression, anxiety, and concentration difficulty, which gave him the feeling to have become another person and to be inhabited by a stranger, whom he compared with devils. Ramuz fought several months to resume his literary activity, having the impression to have lost inspiration and creativity. However, the novels he wrote less than 6 months after stroke show no stylistic changes and have been found to be of the same quality as his previous production. Ramuz even 'used' his stroke experience in his work, in particular in a novel depicting an old man who has a stroke and dies of it. Ramuz's diary, with his own daily description of stroke features and consequences during acute and recovery phases, is a unique document in a writer of his importance, and provides invaluable information on subjective emotional and cognitive experience of stroke.

Hand up! Yawn and raise your arm.

BACKGROUND: In some cases of hemiplegia, the initiation of yawning is associated with involuntary raising of the paralysed arm. Reports are scarce in the literature, probably because the phenomenon has largely been overlooked. METHODS: We studied six patients from two neurologic units, and compared them with published cases from the last 200 years. Brain imaging typically shows a small vascular lesion most often located in the internal capsule. RESULTS: After comparison with experimental models in cats, we suggest that damage to the cortico-neocerebellar tract of the extrapyramidal system disinhibits the spino-archeocerebellar tract, enabling a motor stimulation of the arm by the lateral reticular nucleus, which harmonises both central respiratory and locomotor rhythms. CONCLUSIONS: When phylogenetically primitive structures are disinhibited, they regain autonomy in the homeostatic process associating the massive inspiration of yawning--a form of behaviour that stimulates vigilance--with a motor control mechanism that is active during locomotion. For this phenomenon, we coined the term 'parakinesia brachialis oscitans'.

Early profiles of clinical evolution after intravenous thrombolysis in an unselected stroke population.

BACKGROUND: Intravenous recombinant tissular plasminogen activator (rt-PA) is the only approved pharmacological treatment for acute ischaemic stroke. The authors aimed to analyse potential causes of the variable effect on early course and late outcome. METHODS AND RESULTS: 136 patients (42% women, 58% men) treated with intravenous rt-PA within 3 h of stroke onset in an acute stroke unit over a 3-year period, were included. Early clinical profiles of evolution at 48 h were divided into clinical improvement (CI) (decrease >4 points in the National Institute of Health Stroke Scale (NIHSS)); clinical worsening (CW) (increase >4 points NIHSS); clinical worsening after initial improvement (CWFI) (variations of >4 points in the NIHSS). Patients with clinical stability (no NIHSS modification or <4 points) were excluded. The patients showed in 66.9% CI, 13.2% CW 8.1 % CWFI and 11.8% remained stable. Female sex, no hyperlipaemia and peripheral arterial disease were associated with CW. Male sex and smoking were associated with CI. Absence of arterial occlusion on admission (28.4%) and arterial recanalisation at 24 h were associated with CI. Main causes of clinical deterioration included symptomatic intracranial haemorrhage (sICH), persistent occlusion and cerebral oedema. 23.5% developed ICH, 6.6% of which had sICH. At 3 months, 15.5% had died. Mortality was increased in CW, mainly related to sICH and cerebral oedema. The outcome of CWFI was intermediate between CW and CI. CONCLUSIONS: Early clinical profiles of evolution in thrombolysed patients vary considerably. Even with CI, it is critical to maintain vessel permeability to avoid subsequent CW.

Classification of stroke subtypes.

This article reviews published stroke subtype classification systems and offers rules and a basis for a new way to subtype stroke patients. Stroke subtyping can have different purposes, e.g. describing patients' characteristics in a clinical trial, grouping patients in an epidemiological study, careful phenotyping of patients in a genetic study, and classifying patients for therapeutic decision-making in daily practice. The classification should distinguish between ischemic and hemorrhagic stroke, subarachnoid hemorrhage, cerebral venous thrombosis, and spinal cord stroke. Regarding the 4 main categories of etiologies of ischemic stroke (i.e. atherothrombotic, small vessel disease, cardioembolic, and other causes), the classification should reflect the most likely etiology without neglecting the vascular conditions that are also found (e.g. evidence of small vessel disease in the presence of severe large vessel obstructions). Phenotypes of large cohorts can also be characterized by surrogate markers or intermediate phenotypes (e.g. presence of internal carotid artery plaque, intima-media thickness of the common carotid artery, leukoaraiosis, microbleeds, or multiple lacunae). Parallel classifications (i.e. surrogate markers) may serve as within-study abnormalities to support research findings.

New approach to stroke subtyping: the A-S-C-O (phenotypic) classification of stroke.

We now propose a new approach to stroke subtyping. The concept is to introduce a complete 'stroke phenotyping' classification (i.e. stroke etiology and the presence of all underlying diseases, divided by grade of severity) as distinguished from past classifications that subtype strokes by characterizing only the most likely cause(s) of stroke. In this phenotype-based classification, every patient is characterized by A-S-C-O: A for atherosclerosis, S for small vessel disease, C for cardiac source, O for other cause. Each of the 4 phenotypes is graded 1, 2, or 3. One for 'definitely a potential cause of the index stroke', 2 for 'causality uncertain', 3 for 'unlikely a direct cause of the index stroke (but disease is present)'. When the disease is completely absent, the grade is 0; when grading is not possible due to insufficient work-up, the grade is 9. For example, a patient with a 70% ipsilateral symptomatic stenosis, leukoaraiosis, atrial fibrillation, and platelet count of 700,000/mm(3) would be classified as A1-S3-C1-O3. The same patient with a 70% ipsilateral stenosis, no brain imaging, normal ECG, and normal cardiac imaging would be identified as A1-S9-C0-O3. By introducing the 'level of diagnostic evidence', this classification recognizes the completeness, the quality, and the timing of the evaluation to grade the underlying diseases. Diagnostic evidence is graded in levels A, B, or C: A for direct demonstration by gold-standard diagnostic tests or criteria, B for indirect evidence or less sensitive or specific tests or criteria, and C for weak evidence in the absence of specific tests or criteria. With this new way of classifying patients, no information is neglected when the diagnosis is made, treatment can be adapted to the observed phenotypes and the most likely etiology (e.g. grade 1 in 1 of the 4 A-S-C-O phenotypes), and analyses in clinical research can be based on 1 of the 4 phenotypes (e.g. for genetic analysis purpose), while clinical trials can focus on 1 or several of these 4 phenotypes (e.g. focus on patients A1-A2-A3).

Effects of different types of exercise training followed by detraining on endothelium-dependent dilation in patients with recent myocardial infarction.

BACKGROUND: In coronary artery disease, exercise training (ET) is associated with an improvement in endothelial function, but little is known about the relative effect of different types of training. The purpose of this study was to prospectively evaluate the effect of different types of ET on endothelial function in 209 patients after a first recent acute myocardial infarction. METHODS AND RESULTS: Endothelial function was evaluated before and after 4 weeks of different types of ET and after 1 month of detraining by measuring flow-mediated dilation and von Willebrand factor levels at baseline and after ET. Patients were randomized into 4 groups: group 1, aerobic ET (n=52); group 2, resistance training (n=54); group 3, resistance plus aerobic training (n=53); and group 4, no training (n=50). At baseline, flow-mediated dilation was 4.5+/-2.6% in group 1, 4.01+/-1.6% in group 2, 4.4+/-4% in group 3, and 4.3+/-2.3% in group 4 (P=NS). After ET, flow-mediated dilation increased to 9.9+/-2.5% in group 1, 10.1+/-2.6% in group 2, and 10.8+/-3% in group 3 (P<0.01 versus baseline for all groups); it also increased in group 4 but to a much lesser extent (to 5.1+/-2.5%; P<0.01 versus trained groups). The von Willebrand factor level after ET decreased by 16% (P<0.01) similarly in groups 1, 2, and 3 but remained unchanged in group 4. Detraining returned flow-mediated dilation to baseline levels (P<0.01 versus posttraining). CONCLUSIONS: In patients with recent acute myocardial infarction, ET was associated with improved endothelial function independently of the type of training, but this effect disappeared after 1 month of detraining.

'The Adventure': Charles-Ferdinand Ramuz's extraordinary stroke diary.

The famous Swiss writer Charles-Ferdinand Ramuz suffered a stroke at 65 years, which he called 'the adventure' or 'the accident'. He developed language disturbances suggesting crossed aphasia in a right hander with left hemiparesis. This uncommon pattern allowed him to continue to write his diary and to report his disturbances, with a unique depth and precision, especially for cognitive-emotional changes. Language and motor dysfunction recovered within a few weeks, but Ramuz complained of persisting emotional flattening alternating with irritability, fatigue, depression, anxiety, and concentration difficulty, which gave him the feeling to have become another person and to be inhabited by a stranger, whom he compared with devils. Ramuz fought several months to resume his literary activity, having the impression to have lost inspiration and creativity. However, the novels he wrote less than 6 months after stroke show no stylistic changes and have been found to be of the same quality as his previous production. Ramuz even 'used' his stroke experience in his work, in particular in a novel depicting an old man who has a stroke and dies of it. Ramuz's diary, with his own daily description of stroke features and consequences during acute and recovery phases, is a unique document in a writer of his importance, and provides invaluable information on subjective emotional and cognitive experience of stroke.

[Fatigue in neurological disease: different patterns in stroke and multiple sclerosis]. / Dimensions multiples de la fatigue d'origine neurologique: différences entre l'accident vasculaire cérébral et la sclérose en plaques.

INTRODUCTION: Fatigue is a complex, subjective experience, frequent in multiple sclerosis (MS) and stroke patients. The tiredness these patients experience can take on many features depending not only on the cerebral location of the lesions and mood aspects, but also on the pathophysiology of the disease. Thus, it is reasonable to expect that fatigue may have different implications in MS and stroke. The aim of the present work was to compare fatigue syndrome in these two populations. Patients were matched for handicap. MATERIALS AND METHODS: Seventy-nine stroke and 39 MS outpatients were included with the following inclusion criteria: i) patients with possible or relapsing-remitting MS with an Expanded Disability Status Scale (EDSS) score<2.5, disease duration<6 years, and stable medical condition for at least 6 weeks; ii) stroke patients with mild neurological impairment, i.e. scoring<3 at the National Institute of Health Stroke Scale (NIHSS) one year after stroke; iii) absence of functional impairment (Barthel index=100) and similar negligible handicap (Rankin scale<2 for both groups); no or mild cognitive deficit; iv) neither DSMIV criteria of depression, nor significant anxious/depressive symptoms (Hospital Anxiety and Depression scale; HAD; score<8) in both groups. The Fatigue Assessing Instrument (FAI) was used to assess fatigue. RESULTS: Twenty-nine percent of stroke and 46 p. cent of MS patients had a significant score on the FAI (p<0.05). Multiple regression analysis using groups, gender and age as factors showed a group effect in 3 out of 4 subscales: MS patients scored higher than stroke patients mainly for psychic impact (4.86 vs. 3.28), but also for severity (mean 3.86 vs. 2.97) and specificity (4.36 vs. 3.32). Response to rest (5.36 vs. 6.06) only tended to be better in the stroke group. In the subpopulation with significant fatigue scores, psychic impact was more elevated in the MS group. The functional consequence of fatigue in physical, professional and social activities were similar. DISCUSSION: Fatigue was more severe in MS than stroke patients, independently of disability. The most significant factor in the MS group was the psychic impact, reflecting impaired motivation, concentration and irritability, despite the absence of depression. However, subjective consequences of fatigue on work, family and leisure activities were comparable in both groups.

Comparison of CT perfusion and angiography and MRI in selecting stroke patients for acute treatment.

Forty-two stroke patients successively underwent perfusion CT (PCT)/CT angiography (CTA) and MRI examinations within 3 to 9 hours following symptom onset; 14 would have been suitable candidates for reperfusion treatment based on MRI findings. Correlation between PCT/CTA and MRI was excellent for infarct size, cortical involvement, and internal cerebral artery occlusion and substantial for penumbra/infarct ratio. Relying on MRI or PCT/CTA would have led to the same treatment decisions in all cases but one.

Intentional motor phantom limb syndrome.

OBJECTIVE: To investigate the clinical and anatomic correlates of a previously unreported form of chronic supernumerary phantom limb, which developed only in association with motor intent directed at a hemiplegic-anesthetic upper limb. METHODS: We explored the phenomenology of the phantom illusion in the light of motor control models. Hemodynamic correlates of supernumerary phantom limb were studied with an fMRI sensorimotor paradigm consisting of finger-thumb opposition movements. RESULTS: The kinesthetic-proprioceptive illusion of a third arm was triggered by any attempt to move the paretic limb, by bimanual actions, and by motor imagery involving the nonfunctional limb. The responsible lesion destroyed the posterior part of the posterior limb of the internal capsule on the opposite side, damaging corticospinal and thalamocortical tracts. Comparison between fMRI signals performed during virtual movement of the phantom hand vs imaginary movement of the paretic hand showed increased activation in thalamus and caudate nucleus in the first condition. CONCLUSIONS: A preserved sense of agency provided by intact premotor processes translating intention into action may lead to the vivid feeling of movement in a paralyzed limb, similar to kinesthetic illusions in amputees. The interruption of thalamic afferences may explain the persistence and stability of the phantom by preventing any correction of the mismatch between expected and effective movement. The increased blood oxygen level-dependent (BOLD) signal in the basal ganglia-thalamus-cortex pathway during movement of the supernumerary hand may reflect an abnormal closed-loop functioning of the thalamocortical system underlying the phantom phenomenon.

EFNS guideline on neuroimaging in acute stroke. Report of an EFNS task force.

Neuroimaging techniques are necessary for the evaluation of stroke, one of the leading causes of death and neurological impairment in developed countries. The multiplicity of techniques available has increased the complexity of decision making for physicians. We performed a comprehensive review of the literature in English for the period 1965-2005 and critically assessed the relevant publications. The members of the panel reviewed and corrected an initial draft, until a consensus was reached on recommendations stratified according to the European Federation of Neurological Societies (EFNS) criteria. Non-contrast computed tomography (CT) scan is the established imaging procedure for the initial evaluation of stroke patients. However, magnetic resonance imaging (MRI) has a higher sensitivity than CT for the demonstration of infarcted or ischemic areas and depicts well acute and chronic intracerebral hemorrhage. Perfusion and diffusion MRI together with MR angiography (MRA) are very helpful for the acute evaluation of patients with ischemic stroke. MRI and MRA are the recommended techniques for screening cerebral aneurysms and for the diagnosis of cerebral venous thrombosis and arterial dissection. For the non-invasive study of extracranial vessels, MRA is less portable and more expensive than ultrasonography but it has higher sensitivity and specificity for carotid stenosis. Transcranial Doppler is very useful for monitoring arterial reperfusion after thrombolysis, for the diagnosis of intracranial stenosis and of right-to-left shunts, and for monitoring vasospasm after subarachnoid hemorrhage. Currently, single photon emission computed tomography and positron emission tomography have a more limited role in the evaluation of the acute stroke patient.

Acute autonomic dysfunction contralateral to acute strokes: a prospective study of 100 consecutive cases.

Complex painful reflex syndrome is sometimes described in the chronic phase of stroke. Acute autonomic dysfunction (AAD), which is occasionally present in cases of acute stroke, has not been studied prospectively. The aim of the study was to investigate AAD on the hemibody contralateral to the lesion in the acute phase of stroke. One hundred consecutive patients (median age +/- interquartile range, 74 +/- 21; range 19-93; 51 women: 80 +/- 17 and 49 men: 70 +/- 17 years) in the acute phase of stroke were studied prospectively. Changes in skin temperature or coloration, diaphoresis, pain, or edema were noted in the first 3 days post-stroke. Associations between AAD and topography (cortical pre- and/or post-central, insular, corona radiata, basal ganglia, internal capsule, thalamus, and brainstem), age, gender, ischemic or hemorrhagic etiology, or the presence of sensorimotor deficits or ataxia were examined using the chi-squared or Fisher's exact test and logistic regression analysis. AAD was found in 71% of the patients and showed a significant positive association with the presence of a lesion in the post-central cortex (P = 0.037), internal capsule (P = 0.005), basal ganglia (P = 0.002), or insula (P = 0.011) and a negative association with the presence of a lesion in the brainstem (P = 0.004). Multivariate logistic regression analysis including all studied topographic variables showed that only brainstem lesions were significantly associated with a decreased risk of developing AAD (odds ratio = 0.08, 95% confidence interval: 0.01-0.69, P = 0.022). AAD was not associated with age, gender, the ischemic or hemorrhagic nature of the lesion, the side of lesion, hypertonic or hypotonic paresis, or hyperreflexia or hyporeflexia. AAD was found in association with sensory deficits (P = 0.001) and contralateral hyperkinesia (P = 0.004). Acute AAD is significantly more likely to occur in the presence of hemispheric lesions involving sensory pathways from the cortex to the internal capsule and insula and is significantly less prevalent in the presence of brainstem lesions.