Persisting aphasia, cerebral dominance, and painting in the famous artist Carl Fredrik Reuterswärd.

What about artistic creativity following a cerebral lesion? We studied the case of a prominent right-handed Swedish painter and sculptor who suffered a cerebral hemorrhage at the age of 55 years. The patient displayed a lesion of the left capsular lenticular region, which resulted in a right hemiplegia and sensory loss, with aphasia of the subcortical type. The linguistic impairments recovered well but at 1 year postonset, the right hand was still completely paralyzed. After a period of a few weeks, during which the patient refused to use his nondominant hand, he produced his first left-handed drawing, and by 1 year postonset, he had once again resumed an intensive artistic activity using his nondominant hand. The pictorial works were reviewed by several renowned art specialists: changes of style and even of contents were judged without loss of artistic quality. The result was described as a gain in emotional and artistic intensity. We discuss our observation in the context of the literature and focus on the crucial role of cerebral dominance and hand preference. We conclude that pictorial creativity and language are distinct forms of expressions.

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.

Diseases of the nervous system: patients' aetiological beliefs.

BACKGROUND: Patients' opinions about the aetiology of their disease and the implications for compliance have not been well documented at this time. OBJECTIVE: To investigate prospectively aetiological beliefs of a cohort of neurological inpatients. METHODS: Within two days of admission, patients orally answered a short questionnaire regarding their beliefs about the aetiology of their disease and the possible influence of psychological factors, stress, fatigue, excessive work or other activities, poor lifestyle, conflict with another person, a tragic event, chance, and destiny. RESULTS: Of the 342 patients who participated in the study, 49% spontaneously said that they had no idea of what could have caused their disease, 15% gave a congruent medical explanation, 11% mentioned stress and fatigue as a precipitating factor, and 6% evoked a non-congruent medical explanation. Thirty six per cent thought that psychological factors had triggered their disease; such factors being blamed by a higher proportion of young patients and patients with chronic central nervous system diseases. The triggering factors most often blamed were stress (48%, especially by patients with headache), fatigue (51%), chance (54%), and destiny (43%). CONCLUSIONS: Patients' aetiological beliefs only partially concur with medical opinion and this may influence compliance with treatment. This statement should be explored and confirmed by further studies-for example, in cerebrovascular risk factor follow up.

[Prosopagnosia]. / Prosopagnosie.

True prosopagnosy preserves the perception of human faces. However, the latter faces cannot be identified even by patients who have kept an intact implicit recognition. After a thorough examination of the visual recognition disorder, other kinds of information may be involved like, on one hand, those of places, landscapes and building constructions or, on the other hand, those of animals. We observed that a transitorily prosopagnosic farmer was definitively unable to recognize his cows. Arguments for a right hemisphere dominance are discussed.

Interhemispheric transfer of visual motion information after a posterior callosal lesion: a neuropsychological and fMRI study.

Interhemispheric transfer of visual information was investigated behaviourally and with functional magnetic resonance imaging (fMRI) 6 months after a lesion of the posterior two-thirds of the corpus callosum. On tachistoscopical left hemifield presentation, the patient was severely impaired in reading letters, words and geographical names and moderately impaired in naming pictures and colours. In contrast, interhemispheric transfer of visual motion information, tested by verbal report of the direction of short sequences of coherent dot motion presented within the left hemifield, was preserved. The pattern of cerebral activation elicited by apparent motion stimuli was studied with fMRI and compared to that of normal subjects. In normal subjects, apparent motion stimuli, as compared to darkness, activated strongly striate and extrastriate cortex. When presented to one hemifield only, the contralateral calcarine region was activated while regions on the occipital convexity, including putative area V5, were activated bilaterally. A similar activation pattern was found in the patient with a posterior callosal lesion; unilateral left or right hemifield stimulation was accompanied by activation in the contralateral and ipsilateral occipital convexity. Ipsilateral hemifield representation in the extrastriate visual cortex is believed to depend on callosal input. Our observation suggests that this is not the case for visual motion representation and that other, probably parallel, pathways may mediate visual motion transfer after posterior callosotomy.

Auditory agnosia and auditory spatial deficits following left hemispheric lesions: evidence for distinct processing pathways.

Auditory recognition and auditory spatial functions were studied in four patients with circumscribed left hemispheric lesions. Patient FD was severely deficient in recognition of environmental sounds but normal in auditory localisation and auditory motion perception. The lesion included the left superior, middle and inferior temporal gyri and lateral auditory areas (as identified in previous anatomical studies), but spared Heschl's gyrus, the acoustic radiation and the thalamus. Patient SD had the same profile as FD, with deficient recognition of environmental sounds but normal auditory localisation and motion perception. The lesion comprised the postero-inferior part of the frontal convexity and the anterior third of the temporal lobe; data from non-human primates indicate that the latter are interconnected with lateral auditory areas. Patient MA was deficient in recognition of environmental sounds, auditory localisation and auditory motion perception, confirming that auditory spatial functions can be disturbed by left unilateral damage; the lesion involved the supratemporal region as well as the temporal, postero-inferior frontal and antero-inferior parietal convexities. Patient CZ was severely deficient in auditory motion perception and partially deficient in auditory localisation, but normal in recognition of environmental sounds; the lesion involved large parts of the parieto-frontal convexity and the supratemporal region. We propose that auditory information is processed in the human auditory cortex along two distinct pathways, one lateral devoted to auditory recognition and one medial and posterior devoted to auditory spatial functions.

Bilateral contemporaneous posteroventral pallidotomy for the treatment of Parkinson's disease: neuropsychological and neurological side effects. Report of four cases and review of the literature.

The authors report the underestimated cognitive, mood, and behavioral complications in patients who have undergone bilateral contemporaneous pallidotomy, as seen in their early experience with functional neurosurgery for Parkinson's disease (PD) that is accompanied by severe motor fluctuations before pallidal stimulation. Four patients, not suffering from dementia, with advanced (Hoehn and Yahr Stages III-IV), medically untreatable PD featuring severe "on-off" fluctuations underwent bilateral contemporaneous posteroventral pallidotomy (PVP). All patients were evaluated according to the Core Assessment Program for Intracerebral Transplantations (CAPIT) protocol without positron emission tomography scans but with additional neuropsychological cognitive, mood, and behavior testing. For the first 3 to 6 months postoperatively, all patients showed a mean improvement of motor scores on the Unified Parkinson's Disease Rating Scale (UPDRS), in the best "on" (21%) and worst "off" (40%) UPDRS III motor subscale, a mean 30% improvement in the UPDRS II activities of daily living (ADL) subscore, and 60% on the UPDRS IV complications of treatment subscale. Dyskinesia disappeared almost completely, and the mean daily duration of the off time was reduced by an average of 60%. Despite these good results in the CAPIT scores, one patient experienced a partially regressive corticobulbar syndrome with dysphagia, dysarthria, and increased drooling. No emotional lability was found in this patient, but he did demonstrate severe bilateral postoperative pretarsal blepharospasm (apraxia of eyelid opening), which interfered with walking and which required treatment with high-dose subcutaneous injections of botulinum toxin. No patient showed visual field defects or hemiparesis, but postoperative depression, changes in personality, behavior, and executive functions were seen in two individuals. Postoperative abulia was reported by the family of one patient, who lost his preoperative aggressiveness and drive in terms of ADL, speech, business, family life, and hobbies, and became more sleepy and fatigued. One patient reported postoperative mental automatisms, such as compulsive mental counting, and circular thoughts and reasoning during off phases; postoperative depression was found in two patients. However, none of the patients demonstrated these symptoms during intraoperative microelectrode stimulation. These findings are compatible with previous reports on bilateral pallidal lesions. A progressive lowering of UPDRS subscores was seen after 12 months, consistent with the progression of the disease. Bilateral simultaneous pallidotomy may be followed by emotional, behavioral, and cognitive deficits such as depression, obsessive-compulsive disorders, and loss of psychic autoactivation-abulia, as well as disabling corticobulbar dysfunction and apraxia of eyelid opening, in addition to previously described motor and visual field deficits, which make this surgery undesirable even though significant improvement in motor deficits can be achieved.

[Callosal dementia: behavioral disorders related to central and extrapontine myelinolysis]. / Démence callosale. Troubles du comportement lors de myélinolyse centro- et extra-pontique.

We report the behavioral symptoms presented by a 57-year-old man as the first sign of a Marchiafava-Bignami syndrome and by a 44-year-old woman with centro and extrapontine myelinolysis. These observations define a clinical entity, that we named callosal dementia characterized by: 5) fronto-limbic signs with coarse interjections, repetitive and antisocial behavior, alternation of lack of incitation and agitation; 6) elements of a Balint syndrome (suggestive of a posterior callosal involvement), with a pseudo-hallucinated look and a gaze apraxia; 7) signs of callosal dysconnection and; signs of adjacent white matter involvement, with paucity of vocal and facial expression modulation. Early recognition of these features of callosal dementia may be very helpful for diagnosis of suspected myelinolysis, leading to a more careful research of clinical signs of callosal dysconnection and prompting neuroimaging with MRI. A rapid confirmation of the diagnosis may prevent progression to centro or extrapontine myelinolysis, that may sometimes still be lethal, by adequate supportive measures (slow correction of electrolytes imbalance, correction of deficiencies, total alcohol withdrawal).

Short-term memory for colour following posterior hemispheric lesions in man.

Short-term memory for colour was studied in five patients with circumscribed posterior hemispheric lesions. It was impaired independently of colour discrimination in one and more than colour discrimination in two patients. Two patients were normal in colour short-term memory, one with normal and one with deficient colour discrimination performance. Deficient performance in colour short-term memory was associated with bilateral lesions of the inferior occipitotemporal junction including the lateral part of the fusiform gyrus or with a unilateral lesion of the left parieto-occipital convexity. An additional colour constancy deficit was found in the former but not the latter condition. Thus, colour short-term memory can be affected independently of colour discrimination or colour constancy, and may depend on at least two distinct neural circuits.

Colour constancy impairments in patients with lesions of the prestriate cortex.

Colour matching and colour constancy were studied in seven patients and 46 control subjects. Subjects were required to match Munsell Colour Chips presented under either identical or different illumination. Three of the patients had deficits in colour constancy, i.e. failure to compensate for the change in the wavelength composition of the illumination. Two of the patients with defective constancy had suffered bilateral cortical damage to the posterior lingual and fusiform gyri, and one patient had a lesion restricted to the same regions of the right hemisphere. Our observations indicate that these cortical areas, which include part of putative human area V4, play an important role in colour constancy.

Efficiency and safety of bilateral contemporaneous pallidal stimulation (deep brain stimulation) in levodopa-responsive patients with Parkinson's disease with severe motor fluctuations: a 2-year follow-up review.

OBJECT: The aim of this study was to evaluate the long-term safety and efficacy of bilateral contemporaneous deep brain stimulation (DBS) in patients who have levodopa-responsive parkinsonism with untreatable motor fluctuations. Bilateral pallidotomy carries a high risk of corticobulbar and cognitive dysfunction. Deep brain stimulation offers new alternatives with major advantages such as reversibility of effects, minimal permanent lesions, and adaptability to individual needs, changes in medication, side effects, and evolution of the disease. METHODS: Patients in whom levodopa-responsive parkinsonism with untreatable severe motor fluctuations has been clinically diagnosed underwent bilateral pallidal magnetic resonance image-guided electrode implantation while receiving a local anesthetic. Pre- and postoperative evaluations at 3-month intervals included Unified Parkinson's Disease Rating Scale (UPDRS) scoring, Hoehn and Yahr staging, 24-hour self-assessments, and neuropsychological examinations. Six patients with a mean age of 55 years (mean 42-67 years), a mean duration of disease of 15.5 years (range 12-21 years), a mean "on/off' Hoehn and Yahr stage score of 3/4.2 (range 3-5), and a mean "off' time of 40% (range 20-50%) underwent bilateral contemporaneous pallidal DBS, with a minimum follow-up period lasting 24 months (range 24-30 months). The mean dose of levodopa in these patients could not be changed significantly after the procedure and pergolide was added after 12 months in five patients because of recurring fluctuations despite adjustments in stimulation parameters. All but two patients had no fluctuations until 9 months. Two of the patients reported barely perceptible fluctuations at 12 months and two at 15 months; however, two patients remain without fluctuations at 2 years. The mean improvements in the UPDRS motor score in the off time and the activities of daily living (ADL) score were more than 50%; the mean off time decreased from 40 to 10%, and the mean dyskinesia and complication of treatment scores were reduced to one-third until pergolide was introduced at 12 months. No significant improvement in "on" scores was observed. A slight worsening after 1 year was observed and three patients developed levodopa- and stimulation-resistant gait ignition failure and minimal fluctuations at 1 year. Side effects, which were controlled by modulation of stimulation, included dysarthria, dystonia, and confusion. CONCLUSIONS: Bilateral pallidal DBS is safe and efficient in patients who have levodopa-responsive parkinsonism with severe fluctuations. Major improvements in motor score, ADL score, and off time persisted beyond 2 years after the operation, but signs of decreased efficacy started to be seen after 12 months.

Persistent recurrence of hypomania and prosopoaffective agnosia in a patient with right thalamic infarct.

The authors report a 63-year-old man with a history of brief isolated manic episodes who became persistently hypomanic after a small right thalamic infarct. Detailed behavioral and neuropsychologic assessment were performed 18 months after the stroke and revealed a prosopoaffective agnosia as the foremost cognitive disorder, i.e., an impairment in the identification of emotional facial expressions with preserved discrimination of facial identity. Difficulties in reasoning on humorous material and other signs of mild right hemisphere dysfunction were present, but other perceptual, frontal and abstract-reasoning cognitive functions were unimpaired. Prosopoaffective agnosia has not been reported previously in thalamic lesions or in primary or secondary mania. The authors discuss the hypothetical relationships between a right hemisphere deficit in processing emotions and relapsing of the patient's hypomanic behavior.

Sniffing behaviour, or recognizing a lily by smell, but not recognizing a sock on sight.

We report a 65-year-old man with a post-anoxic encephalopathy who showed compulsive sniffing at available objects. This stereotyped environment-driven behaviour has not been previously described. Other compulsive environment-driven responses, such as manipulation and utilization of tools and hyperlexia, were also present. The disorder shared several features with the Klüver-Bucy syndrome where mouthing of objects, rather than smelling them, is common. The patient had a severe dementia, with amnesia, anomia, apraxia, and visual agnosia. Whereas he could not recognize very familiar objects on sight, he could in contrast correctly identify several familiar odours. Although sniffing was a compulsive and purposeless environment-driven behaviour, the question may be asked whether a relatively preserved olfactory recognition, in the presence of a severe disorder of visual recognition and knowledge, could have favoured a stereotyped exploration of objects by smelling.

[Astral and out-of-body voyages. Heautoscopy, ecstasis and experimental hallucinations of epileptic origin]. / Voyages astraux et hors du corps. Héautoscopie, extase et hallucinations expérientielles d'origine épileptique.

We report a 38 year-old patient who had temporoparietal epilepsy and unusual ictal "out of body" experiences that remained undiagnosed for more than ten years, until her admission for a motor seizure of the left hemibody. Out of body episodes were experienced as intense and ecstatic astral journeys. EEG showed a bilateral extension of epileptiform abnormalities to the parietal regions, predominantly on the right side. We discuss the various forms of heautoscopy and their putative mechanisms. We suggest that a disturbance in representing space in independent extrapersonal and personal coordinates might be as crucial as the elusive hypothesis of a body schema disorder. Combined involvement of the parietal neocortex and temporolimbic structures might allow those experiences to gain a subjective vividness which appears to be indissociable from normal conscious experiences.

Bihippocampal damage with emotional dysfunction: impaired auditory recognition of fear.

A right-handed man developed a sudden transient, amnestic syndrome associated with bilateral hemorrhage of the hippocampi, probably due to Urbach-Wiethe disease. In the 3rd month, despite significant hippocampal structural damage on imaging, only a milder degree of retrograde and anterograde amnesia persisted on detailed neuropsychological examination. On systematic testing of recognition of facial and vocal expression of emotion, we found an impairment of the vocal perception of fear, but not that of other emotions, such as joy, sadness and anger. Such selective impairment of fear perception was not present in the recognition of facial expression of emotion. Thus emotional perception varies according to the different aspects of emotions and the different modality of presentation (faces versus voices). This is consistent with the idea that there may be multiple emotion systems. The study of emotional perception in this unique case of bilateral involvement of hippocampus suggests that this structure may play a critical role in the recognition of fear in vocal expression, possibly dissociated from that of other emotions and from that of fear in facial expression. In regard of recent data suggesting that the amygdala is playing a role in the recognition of fear in the auditory as well as in the visual modality this could suggest that the hippocampus may be part of the auditory pathway of fear recognition.

[Cognitive sequelae following rupture of aneurysms of the anterior communicating artery and the anterior cerebral artery. Retrospective study of 56 cases]. / Troubles cognitifs séquellaires de la rupture d'anévrysmes de l'artère communicante antérieure et de l'artère cérébrale antérieure. Etude rétrospective de 56 cas.

The neuropsychological records of 56 patients operated for clipping were studied. Almost every patient remained autonomous and without invalidating motor defect. The present study was aimed at specifying the type and frequency of neuropsychological sequelae and, to a lesser extent, the role of various pathophysiological factors. A main concern was to examine to what extent and at what post-operative interval the neuropsychological assessment can predict the intellectual and socioprofessional outcome of each individual patient. The neuropsychological assessment performed beyond the acute phase showed evidence of intellectual sequelae in about two thirds of the patients. Only one case of permanent anterograde amnesia was observed, probably due to unavoidable inclusion of a hypothalamic artery in the clip during surgery. Transient anterograde amnesia and confabulations were occasionally observed, generally for less than three weeks. A common finding was impaired performance on memory and/or executive tests. In a minority of patients, language disorders, visuoperceptive and visuoconstructive disabilities were found, probably in relation with hemodynamic changes at distance from the aneurysm. Global impairment of intellectual function was not uncommon in the acute post-operative phase but it evolved in most cases towards a more selective impairment, for instance restricted to executive and memory functions, in the chronic phase. The neuropsychological investigation carried out 4 to 15 weeks post-operatively provided satisfactory information about possible long-lasting intellectual disturbances and professional resumption. In particular, persistent global intellectual impairment, persistent amnesia and confabulations 4-15 weeks post-operative were associated with cessation of professional activity; executive and memory impairment, behavioral disturbances such as those encountered in patients with frontal lobe damage were associated with a decreased probability of full-time employment. Pre- and post-operative angiography were not good predictors of long-term cognitive outcome: normal angiography was not necessarily followed by normal neuropsychological outcome, conversely abnormal angiography could be found together with normal neuropsychological outcome. By contrast, there was a relationship between left-lateralised abnormalities on post-operative angiography and occurrence of language disorders; similarly, there was a relationship between side of craniotomy and type of deficits, that is language disorders versus visuoperceptive-visuoconstructive impairments.

Face recognition and postero-inferior hemispheric lesions.

Two patients with severe and lasting prosopagnosia were studied for visuo-cognitive functions and anatomo-clinical correlations. Both patients were deficient in recognizing familiar faces and items of previously well known categories (plants and fish for patient 1, mountains for patient 2). Patient 2, but not patient 1, was also deficient for matching of unknown faces. Patient 1, but not patient 2, was achromatopsic. Both patients had bilateral symmetrical lesions. Patient 1 had a lesion of the inferior occipito-temporal cortex, including inferior parts of early stage visual areas. Patient 2 had a lesion of the inferior temporal and fusiform gyri anterior to the early stage visual areas. When compared in Talairach space, the lesions of both patients had minimal overlap. Thus, severe and lasting prosopagnosia was associated with two almost exclusive lesion sites in the postero-inferior part of the hemispheres. Comparison between activation studies of face processing (by others) and our lesion study uncovered several paradoxes. Lesions of regions involved in a given task in normal subjects do not produce a deficit in this task, as shown here for gender discrimination and partially for face matching. Conversely, lesions of a region not specifically involved in a given task in normal subjects can produce a deficit in the task, as shown here for face identification.

Phantom face: conscious correlate of neural reorganization after removal of primary sensory neurones.

A patient in whom mandibular and maxillary parts of right trigeminal ganglion were removed experienced referred sensations after stimulation of the right hand and right forehead. She described them either as parallel to the perception at the actual stimulation site or as coming uniquely from a (non-existent) stimulation of denervated territory. The latter occurred in 6-19% of stimulations performed on the right forehead and on digits 1, 3 and 4 of the right hand. Thumb stimulations were localized on the right side of the face, stimulations of right forehead, middle and ring fingers more precisely on right cheek. Referred sensations were present on postoperative day 7 and had a more real-like quality than 5 days later.