The Experts below are selected from a list of 360 Experts worldwide ranked by ideXlab platform
Marco Sarà - One of the best experts on this subject based on the ideXlab platform.
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intrathecal Baclofen: Effects on Spasticity, Pain, and Consciousness in Disorders of Consciousness and Locked-in Syndrome
Current Pain and Headache Reports, 2014Co-Authors: Francesca Pistoia, Marco Sarà, Simona Sacco, Marco Franceschini, Antonio CaroleiAbstract:Disorders of consciousness (DOCs) include coma, vegetative state (VS), and minimally conscious state (MCS). Coma is characterized by impaired wakefulness and consciousness, while VS and MCS are defined by lacking or discontinuous consciousness despite recovered wakefulness. Conversely, Locked-in Syndrome (LIS) is characterized by quadriplegia and lower cranial nerve paralysis with preserved consciousness. intrathecal baclofen (ITB) is a useful treatment to improve spasticity both in patients with DOCs and LIS. Moreover, it supports the recovery of consciousness in some patients with VS or MCS. The precise mechanism underlying this recovery has not yet been elucidated. It has been hypothesized that ITB may act by reducing the overload of dysfunctional sensory stimuli reaching the injured brain or by stabilizing the imbalanced circadian rhythms. Although the current indication of ITB is the management of severe spasticity, its potential use in speeding the recovery of consciousness merits further investigation.
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recognition and mental manipulation of body parts dissociate in Locked in Syndrome
Brain and Cognition, 2010Co-Authors: Massimiliano Conson, Marco Sarà, Francesca Pistoia, Dario Grossi, Luigi TrojanoAbstract:Several lines of evidence demonstrate that the motor system is involved in motor simulation of actions, but some uncertainty exists about the consequences of lesions of descending motor pathways on mental imagery tasks. Moreover, recent findings suggest that the motor system could also have a role in recognition of body parts. To address these issues in the present study we assessed patients with a complete damage of descending motor pathways (Locked-in Syndrome, LIS) on the hand laterality task, requiring subjects to decide whether a hand stimulus in a given spatial orientation represents a left or a right hand. LIS patients were less accurate than healthy controls in judging hand laterality; more importantly, LIS patients' performance was modulated by spatial orientation of hand stimuli whereas it was not affected by biomechanical constraints. These findings demonstrate a dissociation between spared hand recognition and impaired access to action simulation processes in LIS patients.
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Impaired Conscious Recognition of Negative Facial Expressions in Patients with Locked-in Syndrome
The Journal of Neuroscience, 2010Co-Authors: Francesca Pistoia, Marta Ponari, Maria Letizia Pistoia, Filippo Carducci, Claudio Colonnese, Massimiliano Conson, Dario Grossi, Luigi Trojano, Marco SaràAbstract:The involvement of facial mimicry in different aspects of human emotional processing is widely debated. However, little is known about relationships between voluntary activation of facial musculature and conscious recognition of facial expressions. To address this issue, we assessed severely motor-disabled patients with complete paralysis of voluntary facial movements due to lesions of the ventral pons [Locked-in Syndrome (LIS)]. Patients were required to recognize others' facial expressions and to rate their own emotional responses to presentation of affective scenes. LIS patients were selectively impaired in recognition of negative facial expressions, thus demonstrating that the voluntary activation of mimicry represents a high-level simulation mechanism crucially involved in explicit attribution of emotions.
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Opsoclonus-Myoclonus Syndrome in Patients With Locked-in Syndrome: A Therapeutic Porthole With Gabapentin
Mayo Clinic proceedings, 2010Co-Authors: Francesca Pistoia, Massimiliano Conson, Marco SaràAbstract:Patients with Locked-in Syndrome, although fully conscious, have quadriplegia, mutism, and lower cranial nerve paralysis. The preservation of vertical gaze and upper eyelid movements usually enables them to interact with the environment through an eye-coded communication. However, Locked-in Syndrome may be complicated by the development of an opsoclonus-myoclonus Syndrome that may represent an additional impediment to communication. We evaluated whether off-label treatment with gabapentin could help patients with Locked-in Syndrome and opsoclonus-myoclonus symptoms regain voluntary control of full eye movements. A mechanism responsible for gabapentin-induced improvement has been also hypothesized. in this study, 4 patients presenting with Locked-in Syndrome complicated by opsoclonus-myoclonus Syndrome were continuously treated with gabapentin up to 1200 mg/d. The treatment resulted in a rapid and long-lasting resolution of opsoclonus-myoclonus symptoms without adverse effects. After 2 weeks, patients showed voluntary attempts to communicate through eye blinking and thereafter regained voluntary control of full eye movements. This event enabled them to regain a communication channel with relatives and physicians and to start using eye-controlled brain-computer interfaces. Because of its effectiveness in restoring eye movement control, gabapentin opened a communicative porthole in the patients' lives. Since opsoclonus may be related to disorders of the inhibitory control of saccadic burst neurons by pontine pause cells, we hypothesize that gabapentin acts as a regulator of saccadic circuits.
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gabapentin therapy for ocular opsoclonus myoclonus restores eye movement communication in a patient with a Locked in Syndrome
Neurorehabilitation and Neural Repair, 2010Co-Authors: Francesca Pistoia, Marco SaràAbstract:The experience with a patient with Locked-in Syndrome suffering from opsoclonus-myoclonus symptoms is described: gabapentin successfully reversed the symptoms, just favoring the regaining of eye-dependent communication strategies.
Francesca Pistoia - One of the best experts on this subject based on the ideXlab platform.
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Disembodied Mind: Cortical Changes Following Brainstem injury in Patients with Locked-in Syndrome
The Open Neuroimaging Journal, 2016Co-Authors: Francesca Pistoia, Riccardo Cornia, Olivia Gosseries, Antonio Carolei, Simona Sacco, Massimiliano Conson, Cristina Iani, Carlo Augusto Mallio, Carlo Cosimo Quattrocchi, Debora Di MambroAbstract:Locked-in Syndrome (LIS) following ventral brainstem damage is the most severe form of motor disability. Patients are completely entrapped in an unresponsive body despite consciousness is preserved. Although the main feature of LIS is this extreme motor impairment, minor non-motor dysfunctions such as motor imagery defects and impaired emotional recognition have been reported suggesting an alteration of embodied cognition, defined as the effects that the body and its performances may have on cognitive domains. We investigated the presence of structural cortical changes in LIS, which may account for the reported cognitive dysfunctions. For this aim, magnetic resonance imaging scans were acquired in 11 patients with LIS (6 males and 5 females; mean age: 52.3±5.2SD years; mean time interval from injury to evaluation: 9±1.2SD months) and 44 healthy control subjects matching patients for age, sex and education. Freesurfer software was used to process data and to estimate cortical volumes in LIS patients as compared to healthy subjects. Results showed a selective cortical volume loss in patients involving the superior frontal gyrus, the pars opercularis and the insular cortex in the left hemisphere, and the superior and medium frontal gyrus, the pars opercularis, the insular cortex, and the superior parietal lobule in the right hemisphere. As these structures are typically associated with the mirror neuron system, which represents the neural substrate for embodied simulation processes, our results provide neuroanatomical support for potential disembodiment in LIS.
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intrathecal Baclofen: Effects on Spasticity, Pain, and Consciousness in Disorders of Consciousness and Locked-in Syndrome
Current Pain and Headache Reports, 2014Co-Authors: Francesca Pistoia, Marco Sarà, Simona Sacco, Marco Franceschini, Antonio CaroleiAbstract:Disorders of consciousness (DOCs) include coma, vegetative state (VS), and minimally conscious state (MCS). Coma is characterized by impaired wakefulness and consciousness, while VS and MCS are defined by lacking or discontinuous consciousness despite recovered wakefulness. Conversely, Locked-in Syndrome (LIS) is characterized by quadriplegia and lower cranial nerve paralysis with preserved consciousness. intrathecal baclofen (ITB) is a useful treatment to improve spasticity both in patients with DOCs and LIS. Moreover, it supports the recovery of consciousness in some patients with VS or MCS. The precise mechanism underlying this recovery has not yet been elucidated. It has been hypothesized that ITB may act by reducing the overload of dysfunctional sensory stimuli reaching the injured brain or by stabilizing the imbalanced circadian rhythms. Although the current indication of ITB is the management of severe spasticity, its potential use in speeding the recovery of consciousness merits further investigation.
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recognition and mental manipulation of body parts dissociate in Locked in Syndrome
Brain and Cognition, 2010Co-Authors: Massimiliano Conson, Marco Sarà, Francesca Pistoia, Dario Grossi, Luigi TrojanoAbstract:Several lines of evidence demonstrate that the motor system is involved in motor simulation of actions, but some uncertainty exists about the consequences of lesions of descending motor pathways on mental imagery tasks. Moreover, recent findings suggest that the motor system could also have a role in recognition of body parts. To address these issues in the present study we assessed patients with a complete damage of descending motor pathways (Locked-in Syndrome, LIS) on the hand laterality task, requiring subjects to decide whether a hand stimulus in a given spatial orientation represents a left or a right hand. LIS patients were less accurate than healthy controls in judging hand laterality; more importantly, LIS patients' performance was modulated by spatial orientation of hand stimuli whereas it was not affected by biomechanical constraints. These findings demonstrate a dissociation between spared hand recognition and impaired access to action simulation processes in LIS patients.
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Impaired Conscious Recognition of Negative Facial Expressions in Patients with Locked-in Syndrome
The Journal of Neuroscience, 2010Co-Authors: Francesca Pistoia, Marta Ponari, Maria Letizia Pistoia, Filippo Carducci, Claudio Colonnese, Massimiliano Conson, Dario Grossi, Luigi Trojano, Marco SaràAbstract:The involvement of facial mimicry in different aspects of human emotional processing is widely debated. However, little is known about relationships between voluntary activation of facial musculature and conscious recognition of facial expressions. To address this issue, we assessed severely motor-disabled patients with complete paralysis of voluntary facial movements due to lesions of the ventral pons [Locked-in Syndrome (LIS)]. Patients were required to recognize others' facial expressions and to rate their own emotional responses to presentation of affective scenes. LIS patients were selectively impaired in recognition of negative facial expressions, thus demonstrating that the voluntary activation of mimicry represents a high-level simulation mechanism crucially involved in explicit attribution of emotions.
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Opsoclonus-Myoclonus Syndrome in Patients With Locked-in Syndrome: A Therapeutic Porthole With Gabapentin
Mayo Clinic proceedings, 2010Co-Authors: Francesca Pistoia, Massimiliano Conson, Marco SaràAbstract:Patients with Locked-in Syndrome, although fully conscious, have quadriplegia, mutism, and lower cranial nerve paralysis. The preservation of vertical gaze and upper eyelid movements usually enables them to interact with the environment through an eye-coded communication. However, Locked-in Syndrome may be complicated by the development of an opsoclonus-myoclonus Syndrome that may represent an additional impediment to communication. We evaluated whether off-label treatment with gabapentin could help patients with Locked-in Syndrome and opsoclonus-myoclonus symptoms regain voluntary control of full eye movements. A mechanism responsible for gabapentin-induced improvement has been also hypothesized. in this study, 4 patients presenting with Locked-in Syndrome complicated by opsoclonus-myoclonus Syndrome were continuously treated with gabapentin up to 1200 mg/d. The treatment resulted in a rapid and long-lasting resolution of opsoclonus-myoclonus symptoms without adverse effects. After 2 weeks, patients showed voluntary attempts to communicate through eye blinking and thereafter regained voluntary control of full eye movements. This event enabled them to regain a communication channel with relatives and physicians and to start using eye-controlled brain-computer interfaces. Because of its effectiveness in restoring eye movement control, gabapentin opened a communicative porthole in the patients' lives. Since opsoclonus may be related to disorders of the inhibitory control of saccadic burst neurons by pontine pause cells, we hypothesize that gabapentin acts as a regulator of saccadic circuits.
Steven Laureys - One of the best experts on this subject based on the ideXlab platform.
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toward an attention based diagnostic tool for patients with Locked in Syndrome
Clinical Eeg and Neuroscience, 2018Co-Authors: Damien Lesenfants, Steven Laureys, Camille Chatelle, Andrea Soddu, Dina Habbal, Quentin NoirhommeAbstract:Electroencephalography (EEG) has been proposed as a supplemental tool for reducing clinical misdiagnosis in severely brain-injured populations helping to distinguish conscious from unconscious patients. We studied the use of spectral entropy as a measure of focal attention in order to develop a motor-independent, portable, and objective diagnostic tool for patients with Locked-in Syndrome (LIS), answering the issues of accuracy and training requirement. Data from 20 healthy volunteers, 6 LIS patients, and 10 patients with a vegetative state/unresponsive wakefulness Syndrome (VS/UWS) were included. Spectral entropy was computed during a gaze-independent 2-class (attention vs rest) paradigm, and compared with EEG rhythms (delta, theta, alpha, and beta) classification. Spectral entropy classification during the attention-rest paradigm showed 93% and 91% accuracy in healthy volunteers and LIS patients respectively. VS/UWS patients were at chance level. EEG rhythms classification reached a lower accuracy than spectral entropy. Resting-state EEG spectral entropy could not distinguish individual VS/UWS patients from LIS patients. The present study provides evidence that an EEG-based measure of attention could detect command-following in patients with severe motor disabilities. The entropy system could detect a response to command in all healthy subjects and LIS patients, while none of the VS/UWS patients showed a response to command using this system.
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consciousness in the Locked in Syndrome
The Neurology of Conciousness (Second Edition)#R##N#Cognitive Neuroscience and Neuropathology, 2016Co-Authors: Marie-aurélie Bruno, Olivia Gosseries, Steven Laureys, Mariechristine NizziAbstract:Patients in a Locked-in Syndrome (LIS) are selectively de-efferented (i.e., they have no means of producing speech, limb, or face movements). Usually the anatomy of the responsible lesion in the brainstem is such that Locked-in patients are left with the capacity to use vertical eye movements and blinking to communicate with the external world. The Syndrome is subdivided as: (i) classical LIS, which is characterized by total immobility except for vertical eye movements or blinking; (ii) incomplete LIS, which allows remnants of voluntary motion such as head or finger movements; and (iii) total LIS, which is a complete immobility including all eye movements. in all three cases, consciousness is usually fully preserved. Eye-controlled computer-based communication and brain-computer interface technology currently allows these patients to control their environment, use a word processor coupled to a speech synthesizer and access the world wide net. Available literature raises important ethical considerations in terms of quality of life and end-of-life decisions in such challenging patients.
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brain computer interfaces Locked in Syndrome and disorders of consciousness
M S-medecine Sciences, 2015Co-Authors: Damien Lesenfants, Steven Laureys, Camille Chatelle, Quentin NoirhommeAbstract:Detecting signs of consciousness in patients with severe brain injury constitutes a real challenge for clinicians. The current gold standard in clinical diagnosis is the behavioral scale relying on motor abilities, which are often impaired or nonexistent in these patients. in this context, brain-computer interfaces (BCIs) could offer a potential complementary tool to detect signs of consciousness whilst bypassing the usual motor pathway. in addition to complementing behavioral assessments and potentially reducing error rate, BCIs could also serve as a communication tool for paralyzed but conscious patients, e.g., suffering from Locked-in Syndrome. in this paper, we report on recent work conducted by the Coma Science Group on BCI technology, aiming to optimize diagnosis and communication in patients with disorders of consciousness and Locked-in Syndrome.
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interfaces cerveau ordinateur Locked in Syndrome et troubles de la conscience
M S-medecine Sciences, 2015Co-Authors: Steven Laureys, Damien Lesenfants, Camille Chatelle, Quentin NoirhommeAbstract:La detection de signes de conscience chez des patients souffrant de graves lesions cerebrales constitue un veritable defi pour les cliniciens. Actuellement, la norme de reference pour le diagnostic clinique est l’evaluation comportementale. Cependant, celle-ci depend des capacites motrices qui sont souvent diminuees chez ces patients. Dans ce contexte, les interfaces cerveau-ordinateur pourraient offrir un outil diagnostique complementaire, independant des voies motrices. Ces interfaces pourraient egalement fournir un moyen de communication pour les patients victimes de tetraplegie, comme les patients atteints de Locked-in Syndrome. Dans cet article, nous presentons les travaux de recherche recents menes au sein du Coma Science Group dans le domaine des interfaces cerveau-ordinateur, visant a ameliorer le diagnostic et la communication de ces patients.
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Beyond the gaze: Communicating in chronic Locked-in Syndrome
Brain Injury, 2015Co-Authors: Zulay R. Lugo, Frédéric Pellas, Blandin, Marie-aurélie Bruno, Lizette Heine, Marie Thonnard, Olivia Gosseries, Athena Demertzi, Steven LaureysAbstract:AbstractObjective: Locked-in Syndrome (LIS) usually follows a brainstem stroke and is characterized by paralysis of all voluntary muscles (except eyes’ movements or blinking) and lack of speech with preserved consciousness. Several tools have been developed to promote communication with these patients. The aim of the study was to evaluate the current status regarding communication in a cohort of LIS patients.Design: A survey was conducted in collaboration with the French Association of Locked-in Syndrome (ALIS).Subjects and methods: Two hundred and four patients, members of ALIS, were invited to fill in a questionnaire on communication issues and clinical evolution (recovery of verbal language and movements, presence of visual and/or auditory deficits).Results: Eighty-eight responses were processed. All respondents (35% female, mean age = 52 ± 12 years, mean time in LIS = 10 ± 6 years) reported using a yes/no communication code using mainly eyes’ movements and 62% used assisting technology; 49% could comm...
Richard T Katz - One of the best experts on this subject based on the ideXlab platform.
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survival of persons with Locked in Syndrome
Archives of Physical Medicine and Rehabilitation, 2008Co-Authors: Robert M Shavelle, David J Strauss, Richard T KatzAbstract:in a 1987 study, Haig et al reported on 27 patients with Locked-in Syndrome (LIS). A slightly expanded group of 29 patients was studied by Katz and Doble and colleagues. To our knowledge, these are the largest follow-up studies of persons with LIS, and the only ones to report survival probabilities. Their findings on longevity were summarized by Doble as follows: “Five-, 10-, and 20-year survival were 83%, 83%, and 40% respectively.” Unfortunately, these probabilities reflect a methodologic problem. Survival time was counted from the first anniversary of the onset of LIS. However, in some instances, follow-up began many years later, and thus subjects were “guaranteed” to survive until the beginning of follow-up: those who died in the interim never entered the study. As an illustration of the problem, figure 1 of Doble shows survival over a 25-year period even though the subjects were followed for only 11 years. To correct the survival probabilities, we counted each person’s survival time only from the time at which they were “exposed” to death (ie, if they died they would still have been included in the study). Then a Kaplan-Meier analysis gave survival probabilities of 84% at 5 years and 56% at 10 years. The 20-year survival probability cannot be computed directly from the observed data, but the use of some standard actuarial assumptions leads to an estimate of 31%. in addition to the above, 2 additional facts should be noted: (1) the subject population included primarily elective admissions to a world-class facility, and this may have led to an overestimate of survival; and (2) any advances in medical care since the study period would suggest that these figures underestimate survival. Nevertheless, the above figures are compatible with those derived for other types of neurologic injury and comparable physical disabilities. Examples are the permanent vegetative state, traumatic brain injury, and cerebral palsy.
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impairment activity participation life satisfaction and survival in persons with Locked in Syndrome for over a decade follow up on a previously reported cohort
Journal of Head Trauma Rehabilitation, 2003Co-Authors: Jennifer E Doble, Andrew J Haig, Christopher Anderson, Richard T KatzAbstract:Objective: To determine the long-term outcome of patients with stable Locked-in Syndrome. Setting: The community. Design: Retrospective phone survey. This study was further follow-up on a previously reported cohort. Participants: Twenty-nine persons with Locked-in Syndrome were included in an initial cohort 11 years prior to the current study. Records or contact with family showed that 16 were deceased. Telephone interviews were made with 1 living patient and the caregivers of 11 others. Public records documented survival of 1 nonrespondent. Outcome measures: Survival, codified responses regarding functional activities, social activities, and satisfaction with life. Results: Five-, 10-, and 20-year survival were 83%, 83%, and 40%, respectively. Ten subjects had not been hospitalized in the previous year. Eight lived with family. Little change in impairment occurred, but care was simplified. Improvements in communication related to technology, including computer and internet access. Eleven left home at least monthly. Caregivers reported seven expressed satisfaction with life; five were occasionally depressed. No deaths could be attributed to euthanasia and no survivor had a “no code” status. One patient wished to die, seven had never considered euthanasia, six had considered and rejected it. Conclusions: Persons with initially stable Locked-in Syndrome can have prolonged survival, can live in the community if there is enough support, and have some measure of quality of life.
Massimiliano Conson - One of the best experts on this subject based on the ideXlab platform.
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Disembodied Mind: Cortical Changes Following Brainstem injury in Patients with Locked-in Syndrome
The Open Neuroimaging Journal, 2016Co-Authors: Francesca Pistoia, Riccardo Cornia, Olivia Gosseries, Antonio Carolei, Simona Sacco, Massimiliano Conson, Cristina Iani, Carlo Augusto Mallio, Carlo Cosimo Quattrocchi, Debora Di MambroAbstract:Locked-in Syndrome (LIS) following ventral brainstem damage is the most severe form of motor disability. Patients are completely entrapped in an unresponsive body despite consciousness is preserved. Although the main feature of LIS is this extreme motor impairment, minor non-motor dysfunctions such as motor imagery defects and impaired emotional recognition have been reported suggesting an alteration of embodied cognition, defined as the effects that the body and its performances may have on cognitive domains. We investigated the presence of structural cortical changes in LIS, which may account for the reported cognitive dysfunctions. For this aim, magnetic resonance imaging scans were acquired in 11 patients with LIS (6 males and 5 females; mean age: 52.3±5.2SD years; mean time interval from injury to evaluation: 9±1.2SD months) and 44 healthy control subjects matching patients for age, sex and education. Freesurfer software was used to process data and to estimate cortical volumes in LIS patients as compared to healthy subjects. Results showed a selective cortical volume loss in patients involving the superior frontal gyrus, the pars opercularis and the insular cortex in the left hemisphere, and the superior and medium frontal gyrus, the pars opercularis, the insular cortex, and the superior parietal lobule in the right hemisphere. As these structures are typically associated with the mirror neuron system, which represents the neural substrate for embodied simulation processes, our results provide neuroanatomical support for potential disembodiment in LIS.
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recognition and mental manipulation of body parts dissociate in Locked in Syndrome
Brain and Cognition, 2010Co-Authors: Massimiliano Conson, Marco Sarà, Francesca Pistoia, Dario Grossi, Luigi TrojanoAbstract:Several lines of evidence demonstrate that the motor system is involved in motor simulation of actions, but some uncertainty exists about the consequences of lesions of descending motor pathways on mental imagery tasks. Moreover, recent findings suggest that the motor system could also have a role in recognition of body parts. To address these issues in the present study we assessed patients with a complete damage of descending motor pathways (Locked-in Syndrome, LIS) on the hand laterality task, requiring subjects to decide whether a hand stimulus in a given spatial orientation represents a left or a right hand. LIS patients were less accurate than healthy controls in judging hand laterality; more importantly, LIS patients' performance was modulated by spatial orientation of hand stimuli whereas it was not affected by biomechanical constraints. These findings demonstrate a dissociation between spared hand recognition and impaired access to action simulation processes in LIS patients.
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Impaired Conscious Recognition of Negative Facial Expressions in Patients with Locked-in Syndrome
The Journal of Neuroscience, 2010Co-Authors: Francesca Pistoia, Marta Ponari, Maria Letizia Pistoia, Filippo Carducci, Claudio Colonnese, Massimiliano Conson, Dario Grossi, Luigi Trojano, Marco SaràAbstract:The involvement of facial mimicry in different aspects of human emotional processing is widely debated. However, little is known about relationships between voluntary activation of facial musculature and conscious recognition of facial expressions. To address this issue, we assessed severely motor-disabled patients with complete paralysis of voluntary facial movements due to lesions of the ventral pons [Locked-in Syndrome (LIS)]. Patients were required to recognize others' facial expressions and to rate their own emotional responses to presentation of affective scenes. LIS patients were selectively impaired in recognition of negative facial expressions, thus demonstrating that the voluntary activation of mimicry represents a high-level simulation mechanism crucially involved in explicit attribution of emotions.
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Opsoclonus-Myoclonus Syndrome in Patients With Locked-in Syndrome: A Therapeutic Porthole With Gabapentin
Mayo Clinic proceedings, 2010Co-Authors: Francesca Pistoia, Massimiliano Conson, Marco SaràAbstract:Patients with Locked-in Syndrome, although fully conscious, have quadriplegia, mutism, and lower cranial nerve paralysis. The preservation of vertical gaze and upper eyelid movements usually enables them to interact with the environment through an eye-coded communication. However, Locked-in Syndrome may be complicated by the development of an opsoclonus-myoclonus Syndrome that may represent an additional impediment to communication. We evaluated whether off-label treatment with gabapentin could help patients with Locked-in Syndrome and opsoclonus-myoclonus symptoms regain voluntary control of full eye movements. A mechanism responsible for gabapentin-induced improvement has been also hypothesized. in this study, 4 patients presenting with Locked-in Syndrome complicated by opsoclonus-myoclonus Syndrome were continuously treated with gabapentin up to 1200 mg/d. The treatment resulted in a rapid and long-lasting resolution of opsoclonus-myoclonus symptoms without adverse effects. After 2 weeks, patients showed voluntary attempts to communicate through eye blinking and thereafter regained voluntary control of full eye movements. This event enabled them to regain a communication channel with relatives and physicians and to start using eye-controlled brain-computer interfaces. Because of its effectiveness in restoring eye movement control, gabapentin opened a communicative porthole in the patients' lives. Since opsoclonus may be related to disorders of the inhibitory control of saccadic burst neurons by pontine pause cells, we hypothesize that gabapentin acts as a regulator of saccadic circuits.
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selective motor imagery defect in patients with Locked in Syndrome
Neuropsychologia, 2008Co-Authors: Massimiliano Conson, Marco Sarà, Francesca Pistoia, Simona Sacco, Dario Grossi, Luigi TrojanoAbstract:Recent studies indicate that motor imagery is subserved by activation of motor information. However, at present it is not clear whether the sparing of motor efferent pathways is necessary to perform a motor imagery task. To clarify this issue, we required patients with a selective, severe de-efferentation (Locked-in Syndrome, LIS) to mentally manipulate hands and three-dimensional objects. Compared with normal controls, LIS patients showed a profound impairment on a modified version of the hand-laterality task and a normal performance on mental rotation of abstract items. Moreover, LIS patients did not present visuomotor compatibility effects between anatomical side of hands and spatial location of stimuli on the computer screen. Such findings confirmed that the motor system is involved in mental simulation of action but not in mental manipulation of visual images. To explain LIS patients' inability in manipulating hand representations, we suggested that the pontine lesion, both determined a complete de-efferentation, and affected a component of the motor system, which is crucial for mental representation of body parts, probably the neural connections between parietal lobes and cerebellum.
