Innovative Therapy Combining Neck Muscle Vibration and Transcranial Direct Current Stimulation in Association with Conventional Rehabilitation in Left Unilateral Spatial Neglect Patients: HEMISTIM Protocol for a Randomized Controlled Trial.

Unilateral spatial neglect (USN) rehabilitation requires the development of new methods that can be easily integrated into conventional practice. The aim of the HEMISTIM protocol is to assess immediate and long-term recovery induced by an innovative association of left-side neck-muscle vibration (NMV) and anodal transcranial Direct Current Stimulation (tDCS) on the ipsilesional posterior parietal cortex during occupational therapy sessions in patients with left USN. Participants will be randomly assigned to four groups: control, Left-NMV, Left-NMV + sham-tDCS or Left-NMV + anodal-tDCS. NMV and tDCS will be applied during the first 15 min of occupational therapy sessions, three days a week for three weeks. USN will be assessed at baseline, just at the end of the first experimental session, after the first and third weeks of the protocol and three weeks after its ending. Our primary outcome will be the evolution of the functional Catherine Bergego Scale score. Secondary outcome measures include five tests that investigate different neuropsychological aspects of USN. Left NMV, by activating multisensory integration neuronal networks, might enhance effects obtained by conventional therapy since post-effects were shown when it was combined with upper limb movements. We expect to reinforce lasting intermodal recalibration through LTP-like plasticity induced by anodal tDCS.

Perceptual post-effects of left neck muscle vibration with visuo-haptic feedback in healthy individuals: A potential approach for treating spatial neglect.

Among the techniques used to reduce spatial neglect's symptoms, left neck muscle vibration (NMV) is alluring because it does not require the patient's attentional co-operation. The aim of this study was to determine the type of NMV-associated feedback that induced the most intense and longest-lasting egocentric post-effects. Eighty-seven healthy individuals were randomly assigned to four intervention groups: "neck muscle vibration, blindfolded" (NMV), "neck muscle vibration with vision" (NMV + V), "neck muscle vibration and visual finger-pointing" (NMV + P), and "visual finger-pointing" (P). An eyes-closed finger-pointing subjective straight-ahead (SSA) test was carried out before the intervention, immediately afterwards, and 30 min afterwards. The results showed that only the NMV + P intervention induced a lasting leftward bias of SSA. In addition, the deviation reported in this intervention group differed significantly from those observed in the other interventions. The combination of visuo-haptic feedback and neck-somatosensory stimulation may enable a full, lasting intermodal recalibration, which could be potentiated by the attention level engaged during voluntary pointing. These outcomes highlighted that the NMV technique could easily integrate into routine occupational therapy sessions for treating various aspects of neglect disorders.

Lasting improvements in left spatial neglect following a protocol combining neck-muscle vibration and voluntary arm movements: a case-study.

PURPOSE: Beyond promising experimental results of sensory passive stimulations in spatial cognition disorders, some questions still remain regarding interests of these stimulations during the daily activities in neglect. The aim of this case-study was to evaluate the effects of a protocol combining left neck-muscle vibration with daily simple movements, like arm pointing movements, on perceptivo-locomotor deficits in a left spatial neglect patient. MATERIALS AND METHODS: Two neuropsychological tests, one subjective straight-ahead pointing (SSA) test and one wheelchair navigation test were carried out before the combination protocol, immediately after, 1 h later, and 24 h later. RESULTS: The results showed a reduction of neglect spatial bias following the protocol lasted at least 24 h in all the tests (except for the SSA test due to the unavailability of the pointing device). CONCLUSIONS: The range of improvements in the symptoms of spatial neglect suggests that this therapeutic intervention based on the combining neck-muscle vibration to voluntary arm movements could be a useful treatment for this condition. One of future investigation axes should be the development of a vibratory tool in order to facilitate the combining this proprioceptive stimulation to daily activities. Implications for rehabilitation Spatial neglect is a perplexing neuropsychological syndrome, affecting different domains of spatial cognition and impacting also the functional domain. The treatments based on neck-muscle vibration are simple to use, non-invasive and requires none active participation of patient. A therapeutic intervention based on the combining left neck-muscle vibration and voluntary arm movements in a left-spatial-neglect show a lasting reduction of symptoms especially in daily activities. The combination of treatments based on the Bottom-Up approach opens innovative perspectives in rehabilitation.

Clinical, Biomarker, and Molecular Delineations and Genotype-Phenotype Correlations of Ataxia With Oculomotor Apraxia Type 1.

Importance: Ataxia with oculomotor apraxia type 1 (AOA1) is an autosomal recessive cerebellar ataxia due to mutations in the aprataxin gene (APTX) that is characterized by early-onset cerebellar ataxia, oculomotor apraxia, axonal motor neuropathy, and eventual decrease of albumin serum levels. Objectives: To improve the clinical, biomarker, and molecular delineation of AOA1 and provide genotype-phenotype correlations. Design, Setting, and Participants: This retrospective analysis included the clinical, biological (especially regarding biomarkers of the disease), electrophysiologic, imaging, and molecular data of all patients consecutively diagnosed with AOA1 in a single genetics laboratory from January 1, 2002, through December 31, 2014. Data were analyzed from January 1, 2015, through January 31, 2016. Main Outcomes and Measures: The clinical, biological, and molecular spectrum of AOA1 and genotype-phenotype correlations. Results: The diagnosis of AOA1 was confirmed in 80 patients (46 men [58%] and 34 women [42%]; mean [SD] age at onset, 7.7 [7.4] years) from 51 families, including 57 new (with 8 new mutations) and 23 previously described patients. Elevated levels of α-fetoprotein (AFP) were found in 33 patients (41%); hypoalbuminemia, in 50 (63%). Median AFP level was higher in patients with AOA1 (6.0 ng/mL; range, 1.1-17.0 ng/mL) than in patients without ataxia (3.4 ng/mL; range, 0.8-17.2 ng/mL; P < .01). Decreased albumin levels (ρ = -0.532) and elevated AFP levels (ρ = 0.637) were correlated with disease duration. The p.Trp279* mutation, initially reported as restricted to the Portuguese founder haplotype, was discovered in 53 patients with AOA1 (66%) with broad white racial origins. Oculomotor apraxia was found in 49 patients (61%); polyneuropathy, in 74 (93%); and cerebellar atrophy, in 78 (98%). Oculomotor apraxia correlated with the severity of ataxia and mutation type, being more frequent with deletion or truncating mutations (83%) than with presence of at least 1 missense variant (17%; P < .01). Mean (SD) age at onset was higher for patients with at least 1 missense mutation (17.7 [11.4] vs 5.2 [2.6] years; P < .001). Conclusions and Relevance: The AFP level, slightly elevated in a substantial fraction of patients, may constitute a new biomarker for AOA1. Oculomotor apraxia may be an optional finding in AOA1 and correlates with more severe disease. The p.Trp279* mutation is the most frequent APTX mutation in the white population. APTX missense mutations may be associated with a milder phenotype.

Plantar pressure displacement after anesthetic motor block and tibial nerve neurotomy in spastic equinovarus foot.

The aim of this study was to analyze the displacements of center of pressure (COP) using an in-shoe recording system (F-Scan) before and after motor nerve block and neurotomy of the tibial nerve in spastic equinovarus foot. Thirty-nine patients (age 45 ± 15 yr) underwent a motor nerve block; 16 (age 38 ± 15.2 yr) had tibial neurotomy, combined with tendinous surgery (n = 9). The displacement of the COP (anteroposterior [AP], lateral deviation [LD], posterior margin [PM]) was compared between paretic and nonparetic limbs before and after block and surgery. At baseline, the nonparetic limb had a higher AP (17.3 vs 12.3 cm, p < 0.001) and LD (4.0 vs 3.3 cm, p = 0.001) and a smaller PM (2.9 vs 4.7 cm, p = 0.001). For the paretic limb, a significant increase of AP was observed after block (13.5 vs 12.3 cm, p = 0.02) and after surgery (13.7 vs 12.3 cm, p = 0.03). A significant decrease of PM was observed after surgery (4.5 vs 3.3 cm, p < 0.001) with no more difference between two limbs (2.8 vs 3.3 cm; p = 0.44). This study shows that the F-Scan system can be used to quantify impairments and be useful to evaluate the effects of treatment for spastic foot. It suggests that changes in AP displacement following block may predict the effects of neurotomy.

Factitious torsion dystonia in rehabilitation: a singular new case and literature review.

We report a case of a 29-year-old woman suffering from chronic factitious disorder (FD) with torsion dystonia. For nearly five years, she traveled widely over the country, going from one hospital to another, taking serious medical risk in order to prolong her illness. After several admissions to Rehabilitation Units and multiple explorations, we find convincing evidence for factitious origin and the diagnosis of Munchausen syndrome was evoked. Such a clinical presentation is infrequent in Munchausen's syndrome. Indeed, most often the clinical picture is characterized by acute abdominal pain, fainting, hemoptysis, precordialgia, hematemesis or dermatological lesions. Physicians should be aware of this rare and potentially critical form of FD. Awareness in identifying these patients may lead to prevent unnecessary medical and/or surgical interventions.

Specular right-left disorientation, finger-agnosia, and asomatognosia in right hemisphere stroke.

OBJECTIVE: To search for specular disorders of body representations in right hemisphere stroke. BACKGROUND: Mirror self-misidentification, asomatognosia, and personal confabulation are similar to body illusions or changes in sensorial or sensorimotor perceptions generated by mirror in right brain damage patients with body image disorders. METHOD: Prospective study. Ten consecutive right-handed patients (1) performed body part naming and localization tasks and (2) were examined for asomatognosia at the acute phase of stroke, then at least 3 months after stroke, under 3 test conditions: without a mirror, with a conventional mirror, and with an inverted mirror. Video recordings of the tests were analyzed to assess performance. RESULTS: Analysis of variance of the data confirmed that the interaction of mirror's conditions (specifically without a mirror vs. an inverted mirror) with subtest type was significant. The errors are symmetrically distributed. Asomatognosia was "reactivated" in 10 patients who experienced asomatognosia during the acute phase. No particular pattern characterized the clinical manifestations of asomatognosia. CONCLUSIONS: A causal conflict of sensorial input is proposed. The specific symptoms observed would suggest the existence of an incomplete specular Gerstmann syndrome and/or Anton Babinski syndrome. These results emphasize the role of specular input in the generation of body representations and self-awareness.

A battery of tests for the quantitative assessment of unilateral neglect.

PURPOSE: The lack of agreement regarding assessment methods is responsible for the variability in the reported rate of occurrence of unilateral neglect (UN) after stroke. In addition, dissociations have been reported between performance on traditional paper-and-pencil tests and UN in everyday life situations. METHODS: In this paper, we present the validation studies of a quantitative test battery for UN, including paper-and-pencil tests, an assessment of personal neglect, extinction, and anosognosia, and a behavioural assessment, the Catherine Bergego Scale (CBS). The battery was given to healthy subjects (n=456-476) and to patients with subacute stroke, either of the right or the left hemisphere. RESULTS: In healthy subjects, a significant effect of age, education duration and acting hand was found in several tasks. In patients with right hemisphere stroke, the most sensitive paper and pencil measure was the starting point in the cancellation task. The whole battery was more sensitive than any single test alone. An important finding was that behavioural assessment was more sensitive than any other single test. Neglect was two to four times less frequent, but also less severe and less consistent after left hemisphere stroke. CONCLUSION: Assessment of UN should rely on a battery of quantitative and standardised tests. Some patients may show clinically significant UN in everyday life while obtaining a normal performance on paper-and-pencil measures. This underlines the necessity to use a behavioural assessment of UN.

Initial experience with successful totally robotic laparoscopic cystoprostatectomy and ileal conduit construction in tetraplegic patients: report of two cases.

PURPOSE: To evaluate the feasibility and safety of totally robotic laparoscopic cystoprostatectomy for neurogenic bladder, with intracorporeal ileal-conduit urinary diversion in tetraplegic patients with severe neurogenic bladder complications. PATIENTS AND METHODS: Two men, 41 and 38 years old, with complete post-traumatic C7-C8 quadriplegia and poor lower urinary-tract condition underwent totally transperitoneally performed cystoprostatectomy and ileal-conduit urinary diversion with robotic assistance (Da Vinci). RESULTS: The procedures were completed without open conversion. The total surgical time was 9.25 and 6.75 hours, respectively. There were no intraoperative complications. In the postoperative period, both patients had mild complications (pulmonary and urinary infections) that were treated successfully medically. The postoperative hospital stay was 13 days in both cases. CONCLUSIONS: Our results demonstrate the safety and feasibility of robot-assisted laparoscopic cystoprostatectomy with ileal-conduit urinary diversion in two tetraplegic patients.

Transcranial magnetic stimulation for diagnosis of residual limb neuromas.

OBJECTIVE: To analyze the mechanism and examine the potential diagnostic contribution of transcranial magnetic stimulation (TMS) in diagnosing painful, clonic, and/or autonomic manifestations in amputees' residual limbs. DESIGN: Prospective study. SETTING: Regional rehabilitation institute at a medical school in France. PARTICIPANTS: Thirty-four amputees (24 with myoclonus, stump pain, or trophic skin disorders; 10 controls with no stump symptoms). INTERVENTIONS: Not applicable. MAIN OUTCOMES MEASURES: TMS performed before and after correcting prosthesis adaptation. RESULTS: TMS induced pain in 12 amputees, clonic manifestations in 4, and autonomic manifestations in 2. Twelve patients underwent magnetic resonance imaging that showed neuromas in all 12. After neuroma resection in 9 amputees, TMS no longer provoked abnormal manifestations. TMS did provoke abnormal manifestations after resection in 3 patients who had postoperative recurrent neuromas. The response to TMS was negative in subjects with a clinically silent neuroma (n=7). The response was also negative in all patients with other stump anomalies whose clinical manifestations fully regressed after conservative treatment. There was a highly significant correlation between the presence of a pathologic neuroma and TMS-induced abnormal manifestations (P<.0001). CONCLUSIONS: TMS can provoke symptoms in patients who experience spontaneous or evoked symptoms related to a neuroma. Induced symptoms are proportional to spontaneous symptoms. Removing the neuroma can stop stump symptoms and reverse the TMS effect. The response to TMS was negative in the control subjects with clinically silent neuromas; conservative treatment was successful in these cases. TMS-induced abnormal manifestations underlying mechanisms are discussed (ephaptic transmission in neuromas).

Laparoscopic robotic-assisted ileal conduit urinary diversion in a quadriplegic woman.

Urinary diversion is a therapeutic option in quadriplegic patients with poor lower urinary tract conditions, but it is an invasive procedure. Laparoscopic techniques are less invasive, but are technically demanding, and only a few surgical teams are able to complete such a complex procedure endoscopically. Robotics bring an unprecedented control of surgical instruments, shorten the learning curve, and allow open surgeons to apply more easily their technical skill to the laparoscopic approach. This complex case of laparoscopic ileal conduit in a quadriplegic woman was completed by our team with the Da Vinci system after 6 months of clinical experience in robotic surgery.

Cerebellar ataxia with oculomotor apraxia type 1: clinical and genetic studies.

Ataxia with ocular motor apraxia type 1 (AOA1) is an autosomal recessive cerebellar ataxia (ARCA) associated with oculomotor apraxia, hypoalbuminaemia and hypercholesterolaemia. The gene APTX, which encodes aprataxin, has been identified recently. We studied a large series of 158 families with non-Friedreich progressive ARCA. We identified 14 patients (nine families) with five different missense or truncating mutations in the aprataxin gene (W279X, A198V, D267G, W279R, IVS5+1), four of which were new. We determined the relative frequency of AOA1 which is 5%. Mutation carriers underwent detailed neurological, neuropsychological, electrophysiological, oculographic and biological examinations, as well as brain imaging. The mean age at onset was 6.8 +/- 4.8 years (range 2-18 years). Cerebellar ataxia with cerebellar atrophy on MRI and severe axonal sensorimotor neuropathy were present in all patients. In contrast, oculomotor apraxia (86%), hypoalbuminaemia (83%) and hypercholesterolaemia (75%) were variable. Choreic movements were frequent at onset (79%), but disappeared in the course of the disease in most cases. However, a remarkably severe and persistent choreic phenotype was associated with one of the mutations (A198V). Cognitive impairment was always present. Ocular saccade initiation was normal, but their duration was increased by the succession of multiple hypometric saccades that could clinically be confused with 'slow saccades'. We emphasize the phenotypic variability over the course of the disease. Cerebellar ataxia and/or chorea predominate at onset, but later on they are often partially masked by severe neuropathy, which is the most typical symptom in young adults. The presence of chorea, sensorimotor neuropathy, oculomotor anomalies, biological abnormalities, cerebellar atrophy on MRI and absence of the Babinski sign can help to distinguish AOA1 from Friedreich's ataxia on a clinical basis. The frequency of chorea at onset suggests that this diagnosis should also be considered in children with chorea who do not carry the IT15 mutation responsible for Huntington's disease.

Eye movements and verbal reports in neglect patients during a letter reading task.

OBJECTIVES: To study different typolologies from visuo-verbal behavior concerning the arrest and the treatment of visual information in patients with spatial hemineglect. METHODS: Ocular movements were monitored by photo-oculography in thirty controls and twenty-three patients with neglect. During a letter reading task, the movements, number of entries into predetermined zones of the visual field, time spent in each zone, and the verbal responses were recorded. Four profiles may be anticipated for such trials: normal scanning and reading (S+R+), normal scanning without reading (S+R-), absence of both scanning and reading (S-R-), abnormal scanning with reading (S-R+). RESULTS: The letter projected in each of the 4 quadrants was scanned and correctly identified by all of the controls with no differences in scanning times. Among patients, the 4 possible behavior patterns were found along with differences in exploration duration per zone (p < 0.05) and number of times specific zones were attained (p < 0.05). CONCLUSIONS: Our results confirmed the heterogeneity of the mechanisms of neglect and suggest that rehabilitation procedures adapted to each profile might be useful.