Methotrexate-induced Acute Myelopathy in a Teenager With High-risk Acute Lymphoblastic Leukemia.

Acute lymphoblastic leukemia (ALL) is one of the most frequent malignancies in childhood whose long-term survival has increased up to 80% thanks to modern therapy enhancements. Nevertheless, methotrexate (MTX) remains a mainstay of ALL therapy, but also represents one of the major causes of neurotoxicity in patients with ALL. MTX-induced toxicity occurs in about 9% of patients treated for ALL. It usually affects deep white matter region leading to leukoencephalopathy, which has varying clinical manifestations ranging from acute neurologic disturbances to seizures or chronic permanent encephalopathy. Here we describe a 13-year-old girl affected with ALL who developed lower limbs hypesthesia and static ataxia due to transverse myelopathy after intrathec administration of MTX therapy. A high-dose corticotherapy combined to vitamin supplementation and rehabilitation was tested. Neurological evolution was characterized by slow and partial recovery.

[Plum-blossom needle tapping at Jiaji(EX-B 2) combined with rehabilitation for ataxia of cerebellar apoplexy: a randomized controlled trial].

OBJECTIVE: To compare the effects between plum-blossom needle tapping at Jiaji (EX-B 2) combined with rehabilitation and simple rehabilitation for ataxia of cerebellar apoplexy. METHODS: Sixty patients with ataxia of cerebellar apoplexy were randomly divided into an observation group,and a control group,30 cases in each one. Conventional rehabilitation training was applied in the control group. While based on the treatment as that of the control group, plum-blossom needle tapping at Jiaji (EX-B 2) was used in the observation group. All the treatment was given for 4 weeks. International cooperative ataxia rating scale (ICARS), Berg balance scale (BBS) and Barthel index (BD were observed before treatment,after 2-week treatment and 4-week treatment. Also, clinical effects After treating for 2 weeks and for 4 weeks, the results of ICARS,BBS and BI were obvi were compared. RESULTS: After treating for 2 weeksand for 4 weeks, the results of ICARS, BBS and BI were obviously better than those before treatment in the two groups (all P<0.05). After 2-week treatment,there was no sta tistical significance between the two groups (all P>0. 05). After 4-week treatment,all the scores of the observation group were apparently superior to those of the control group (all P<0. 05). The total effective rate was 96. 7% (29/30) in the observation group, better than 80. 0% (24/30) in the control group after 4-week treatment (P<0. 05). CCONCLUSION:Plum-blossom needle tapping at Jiaji(EX-B 2) combined with rehabilitation training markedly improve the coordination and balance functions and activities of daily life, which are better than simple rehabilitation.

"Six sessions is a drop in the ocean": an exploratory study of neurological physiotherapy in idiopathic and inherited ataxias.

OBJECTIVE: An exploratory study to examine specialist neurological physiotherapy service provision and utilisation for people with progressive ataxia. DESIGN: Qualitative study involving thematic analysis of accounts in semi-structured interviews with physiotherapists and patients. SETTING: People with ataxia and specialist neuro-rehabilitation physiotherapists in Greater Manchester, UK. PARTICIPANTS: 38 people with ataxia and 8 neurological-physiotherapists working in academic and hospital and community-based services in NHS and private settings. Recruiting physiotherapists experienced in working with the patient group was a challenge. INTERVENTIONS: One hour cross-sectional semi-structured interview at physiotherapists' workplaces or in patients' own homes. RESULTS: Neurological physiotherapy was experienced by 25 (66%) of the 38 people with ataxia. The overarching themes emerging from the analysis were 'making a difference,' engagement and service provision. A majority of both samples felt that services should be organised so as to provide longer term therapy and support that goes beyond short care packages followed by provision of home exercise programme. Engagement with services was linked to patient expectations, adherence and perception of outcomes. The most predominant codes in the data set were encapsulated by the theme 'making a difference,' which further included concerns about how to measure perceived clinical improvement (as experienced by patients) in the context of progressive decline. CONCLUSIONS: The findings suggest a model of idealised service provision involving a holistic, open-access service including research efforts to improve the evidence base. Special attention needs to be paid to measuring improvements following therapy.

AsTeRICS: a new flexible solution for people with motor disabilities in upper limbs and its implication for rehabilitation procedures.

PURPOSE: To present the AsTeRICS construction set, and examine different combinations of sensors installed in the platform and how users interact with them. METHOD: Nearly 50 participants from Austria, Poland and Spain were included in the study. They had a heterogeneous range of diagnoses, but as a common feature all of them experienced motor limitations in their upper limbs. The study included a 1 h session with each participant where the user interacted with a personalized combination of sensors, based on a previous assessment on their motor capabilities performed by healthcare professionals. The sensors worked as substitutes for a standard QWERTY keyboard and a standard mouse. Semi-structured interviews were conducted to obtain participants' opinions. All collected data were analyzed based on the qualitative methodology. RESULTS: The findings illustrated that AsTeRICS is a flexible platform whose sensors can adapt to different degrees of users' motor capabilities, thus facilitating in most cases the interaction of the participants with a common computer. CONCLUSION: AsTeRICS platform can improve the interaction between people with mobility limitations and computers. It can provide access to new technologies and become a promising tool that can be integrated in physical rehabilitation programs for people with motor disabilities in their upper limbs. IMPLICATIONS FOR REHABILITATION: The AsTeRICS platform offers an interesting tool to interface and support the computerized rehabilitation program of the patients. Due to AsTeRICS platform high usability features, family and rehabilitation professionals can learn how to use the AsTeRICS platform quickly fostering the key role of their involvement on patients' rehabilitation. AsTeRICS is a flexible, extendable, adaptable and affordable technology adapted for using computer, environmental control, mobile phone, rehabilitation programs and mechatronic systems. AsTeRICS makes possible an easy reconfiguration and integration of new functionalities, such as biofeedback rehabilitation, without major changes in the system.

Effect of coordination biofeedback on (re)learning preferred postural patterns in post-stroke patients.

After stroke, ankle-hip coordination during stance is characterized by changes in the postural system dynamics, specifically the disappearance of the in-phase pattern and the reduced stability of the anti-phase pattern. This study was conducted to assess the success of a coordination visual biofeedback for the (re)learning of the two preferred patterns, and to explore the effect of this treatment on postural and functional abilities. Twenty four patients were randomly assigned to one of two experimental groups or to a control group. During one month, patients from experimental groups followed a training protocol on the two preferred postural patterns using the biofeedback device. These two groups improved their in-phase coordination after the (re)learning compared with control group, and showed a related improvement of the functional independence measure. Results suggest that (re)learning the in-phase pattern is possible and seems to improve independence in poststroke patients.

Ambulatory rehabilitation in multiple sclerosis.

Multiple sclerosis (MS) is an autoimmunogenic disease involving demyelination within the central nervous system. Many of the typical impairments associated with MS can affect gait patterns. With walking ability being one of the most decisive factors when assessing quality of life and independent living, this review focuses on matters, which are considered of significance for maintaining and supporting ambulation. This article is an attempt to describe current research and available interventions that the caring healthcare professional can avail of and to review the present trends in research to further these available options. Evidence-based rehabilitation techniques are of interest in the care of patients with MS, given the various existing modalities of treatment. In this review, we summarise the primary factors affecting ambulation and highlight available treatment methods. We review studies that have attempted to characterise gait deficits within this patient population. Finally, as ambulatory rehabilitation requires multidisciplinary interventions, we examine approaches, which may serve to support and maintain ambulation within this patient group for as long as possible.

Treatment for ataxia in multiple sclerosis.

BACKGROUND: Disabling tremor or ataxia is common in multiple sclerosis (MS) and up to 80% of patients experience tremor or ataxia at some point during their disease. A variety of treatments are available, ranging from pharmacotherapy or stereotactic neurosurgery to neurorehabilitation. OBJECTIVES: To assess the efficacy and tolerability of both pharmacological and non-pharmacologic treatments of ataxia in patients with MS. SEARCH STRATEGY: The following electronic resources were searched: Cochrane MS Group trials register (June 2006), the Cochrane Central Register of Controlled Trials (CENTRAL) (Issue 2, 2006), National Health Service National Research Register (NRR) including the Medical Research Council Clinical Trials Directory (Issue 2, 2006), MEDLINE (January 1996 to June 2006), and EMBASE (Jan 1988 to June 2006). Manual searches of bibliographies of relevant articles, pertinent medical and neurology journals and abstract books of major neurology and MS conferences (2001-2006) were also performed. Direct communication with experts and drug companies was sought. SELECTION CRITERIA: Blinded, randomised trials which were either placebo-controlled or which compared two or more treatments were included. Trials testing pharmacological agents must have had both participant and assessor blinding. Trials testing surgical interventions or effects of physiotherapy, where participants could not have been blinded to the treatment, must have had independent assessors who were blinded to the treatment. Cross-over trials were included. DATA COLLECTION AND ANALYSIS: Three independent reviewers extracted data and the findings of the trials were summarised. A meta-analysis was not performed due to the inadequacy of outcome measures and methodological problems with the studies reviewed. MAIN RESULTS: Ten randomised controlled trials met the inclusion criteria. Six placebo-controlled studies (pharmacotherapy) and four comparative studies (one stereotactic neurosurgery and three neurorehabilitation) were reviewed. No standardised outcome measures were used across the studies. In general, pharmacotherapies were unrewarding and data on neurosurgery or rehabilitation is insufficient to lead to a change in practice. AUTHORS' CONCLUSIONS: The absolute and comparative efficacy and tolerability of pharmacotherapies to treat ataxia in MS are poorly documented and no recommendations can be made to guide prescribing. Although studies on neurosurgery and neurorehabilitation showed promising results, the absolute indications for treating with those methods cannot be developed. Standardised, well validated measures of ataxia and tremor need to be developed and employed in larger randomised controlled trials with careful blinding.

Síndrome de Rett: actualización del proceso de rehabilitación / Rett syndrome. Updating of rehabilitation process

Los recientes hallazgos genéticos en el síndrome de Rett obligan a una actualización de los recursos terapéuticos actuales y de la metodología empleada en su rehabilitación. Se establecen explícitamente los objetivos generales y específicos en el marco de una rehabilitación integral y los medios para alcanzarlos. Estas niñas presentan un abigarrado cuadro en el que destacan los problemas ortopédicos (escoliosis, luxación adquirida de cadera, pie equino, fracturas óseas), alteraciones del tono muscular, problemas cognitivos y del lenguaje. Además se revisa el afrontamiento terapéutico de los graves problemas neurológicos y del comportamiento, como la ataxia, apraxia, movimientos estereotipados, problemas foniátricos y respiratorios, ataques de llanto, problemas de autonomía personal, problemas senso-perceptivos, alteraciones cognitivas, y problemas del comportamiento. Se establece la utilidad clínica de diferentes terapias: "técnica de energía muscular", "reptación refleja y volteo reflejo de la terapia Vojta", "concepto Bobath", estimulación del equilibrio y transferencia de peso, patrones progresivos y de repetición, férulas, estimulación eléctrica transcutánea, comunicación aumentativa y alternativa, musicoterapia, etc. El objetivo global es establecer un programa individualizado de enseñanza y terapéutico que, manteniendo en permanente contacto y supervisión a los pacientes y su familia con los servicios especializados asistenciales de rehabilitación, mejore la calidad de vida de estas niñas y de sus familias (AU)

Two coupled motor recovery protocols are better than one: electromyogram-triggered neuromuscular stimulation and bilateral movements.

BACKGROUND AND PURPOSE: Overcoming chronic hemiparesis from a cerebrovascular accident (CVA) can be challenging for many patients, especially after the first 12 months after the CVA. With the use of established motor control theories, the present study investigated electromyogram (EMG)-triggered neuromuscular stimulation and bilateral coordination training. METHODS: Twenty-five CVA subjects volunteered to participate in this motor recovery protocol study. Subjects were randomly assigned to 1 of 3 groups: (1) coupled protocol of EMG-triggered stimulation and bilateral movement (n=10); (2) EMG-triggered stimulation and unilateral movement (n=10); or (3) control (n=5). All participants completed 6 hours of rehabilitation during a 2-week period according to group assignments. Motor capabilities of the wrist and fingers were evaluated on the basis of 3 categories of motor tasks in a pretest-posttest control group design. RESULTS: Significant findings for the (1) number of blocks moved in a functional task, (2) chronometric reaction times to initiate movements, and (3) sustained muscle contraction capability all favored the coupled bilateral movement training and EMG-triggered neuromuscular stimulation protocol group. In addition, the unilateral movement/stimulation group exceeded the control group in the number of blocks moved and rapid onset of muscle contractions. CONCLUSIONS: This new evidence is convincing in that subjects in the coupled protocol group were able to demonstrate enhanced voluntary motor control across 3 categories of tasks. Chronic hemiparesis decreased considerably in the wrist and fingers as CVA patients expanded their motor repertoire.

Knowing no fear.

People with brain injuries involving the amygdala are often poor at recognizing facial expressions of fear, but the extent to which this impairment compromises other signals of the emotion of fear has not been clearly established. We investigated N.M., a person with bilateral amygdala damage and a left thalamic lesion, who was impaired at recognizing fear from facial expressions. N.M. showed an equivalent deficit affecting fear recognition from body postures and emotional sounds. His deficit of fear recognition was not linked to evidence of any problem in recognizing anger (a common feature in other reports), but for his everyday experience of emotion N.M. reported reduced anger and fear compared with neurologically normal controls. These findings show a specific deficit compromising the recognition of the emotion of fear from a wide range of social signals, and suggest a possible relationship of this type of impairment with alterations of emotional experience.

The role of therapy in Rett syndrome.

The purpose of Occupational, Physical and Music Therapy in the treatment of girls with Rett syndrome is to maintain and maximize function. Specific approaches to the problems of ataxia, spasticity, spinal deformity, loss of ambulation, loss of hand function, loss of contact with the environment, feeding, irritability, and family coping are discussed.