Late-onset Pompe disease (LOPD) in Belgium: clinical characteristics and outcome measures.

Background: Late-onset Pompe disease (LOPD) is a rare, hereditary, progressive disorder that is usually characterized by limb-girdle muscle weakness and/or respiratory insufficiency. LOPD is caused by mutations in the acid alpha-glucosidase (GAA) gene and treated with enzyme replacement therapy (ERT).

Methods: We studied the clinical, brain imaging, and genetic features of the Belgian cohort of late-onset Pompe disease patients (N = 52), and explored the sensitivity of different outcome measures, during a longitudinal period of 7 years (2010-2017), including the activity limitations ActivLim score, 6 min walking distance (6MWD), 10 m walk test (10MWT), MRC sum score, and forced vital capacity (FVC) sitting/supine.

Correction to: Eight years after an international workshop on myotonic dystrophy patient registries: case study of a global collaboration for a rare disease.

The original version of this article [1] unfortunately included an error to an author's name. Author Jordi Díaz-Manera was erroneously presented as Jorge Alberto Diaz Manera. The correct author name has been included in the author list of this Correction article.

Eight years after an international workshop on myotonic dystrophy patient registries: case study of a global collaboration for a rare disease.

Background: Myotonic Dystrophy is the most common form of muscular dystrophy in adults, affecting an estimated 10 per 100,000 people. It is a multisystemic disorder affecting multiple generations with increasing severity. There are currently no licenced therapies to reverse, slow down or cure its symptoms.

Capturing neuroplastic changes after bimanual intensive rehabilitation in children with unilateral spastic cerebral palsy: A combined DTI, TMS and fMRI pilot study.

Intensive rehabilitation interventions have been shown to be efficacious in improving upper extremity function in children with unilateral spastic cerebral palsy (USCP). These interventions are based on motor learning principles and engage children in skillful movements. Improvements in upper extremity function are believed to be associated with neuroplastic changes.

Epidemiological situation of Lyme borreliosis in Belgium, 2003 to 2012.

Background: Some studies show that the incidence of Lyme borreliosis is increasing in different European countries. In order to evaluate if this is also the case in Belgium, different data sources were consulted to describe the epidemiology of Lyme borreliosis in the country during the last decade.

Methods: Data from two databases were analyzed for the time period 2003-2010 and 2003-2012 for respectively: the registration of minimal clinical data from Belgian hospitals (principal and secondary diagnosis), and a sentinel laboratory network reporting positive laboratory results.

Comparison between tibial nerve block with anaesthetics and neurotomy in hemiplegic adults with spastic equinovarus foot.

Objective: The aim of the study was to compare the effect of diagnostic motor nerve block with anaesthetics and of selective tibial neurotomy in the treatment of spastic equinovarus foot in hemiplegic adults.

Methods: In this prospective observational study, 30 hemiplegic adults with spastic equinovarus foot benefited from a diagnostic nerve block with anaesthetics followed by a selective tibial neurotomy performed at the level of the same motor nerve branches of the tibial nerve. Spasticity (Ashworth scale), muscle strength (Medical Research Council scale), passive ankle dorsiflexion (ROM), gait parameters (10 meters walking test) and gait kinematics (video assessment) were assessed before and after the nerve block and two months and two years after selective tibial neurotomy.

Hand and Arm Bimanual Intensive Therapy Including Lower Extremity (HABIT-ILE) in Children With Unilateral Spastic Cerebral Palsy: A Randomized Trial.

Background: Intensive bimanual training results in more improvement in hand function in children with unilateral spastic cerebral palsy (USCP) than lower intensity conventional interventions. However, it is not known whether combined upper and lower extremity training in an intensive protocol is more efficacious for upper and lower functional abilities than conventional therapies provided in usual customary care.

Objective: To determine the efficacy of Hand and Arm Bimanual Intensive Therapy Including Lower Extremity (HABIT-ILE) for children with USCP.

Influence of ankle-foot orthoses on kinematic segmental covariation among stroke patients.

Objectives: To evaluate the effect of ankle-foot orthosis on lower limbs kinematic segmental covariation (KSC) among stroke patients.

Methods: Ten chronic hemiparetic spastic stroke patients presenting with a lack of ankle dorsiflexion were assessed with instrumented gait analysis under three conditions: wearing a shoe, with a prefabricated ankle-foot orthosis (AFO), and with a dynamic AFO. Kinematic parameters were recorded and computed KSC was calculated according to Borghese's methodology.

Kinematic covariation in pediatric, adult and elderly subjects: is gait control influenced by age?

Background: In human walking, kinematics of lower limb segments covary. Our objective was to assess the impact of age at different speeds (slow, medium and fast) on kinematic segmental covariation in normal subjects.

Methods: Thirty subjects ranged into 6 age groups ("5 years", "10 years", "15 years", "20 years" and "70 years") were included.

Treatment of genu recurvatum in hemiparetic adult patients: a systematic literature review.

Introduction And Methods: We carried out a systematic review of the literature on treatment of genu recurvatum in hemiparetic adult patients by searching the PubMed, Pedro, Trip Database and Science Direct databases. The following keywords were used: (recurvatum or hyperextension or knee) and (hemiplegia or hemiparesis).

Results: Nine articles met our selection criteria.

Reliability of lower limb kinematics, mechanics and energetics during gait in patients after stroke.

Objective: To assess the reliability of kinematic, mechanical and energetic gait variables at short (1 day) and medium (1 month) intervals in adult patients after stroke.

Design: Prospective study.

Subjects: Ten patients with chronic post-stroke (mean age 53.

The effect of botulinum toxin injections on gait control in spastic stroke patients presenting with a stiff-knee gait.

Introduction: Botulinum toxin type A (BoNT-A) injections are known to improve walking impairments and activity in spastic hemiparetic stroke patients presenting with a stiff-knee gait [Caty GD, Detrembleur C, Bleyenheuft C, Deltombe T, Lejeune TM. Effect of simultaneous botulinum toxin injections into several muscles on impairment, activity, participation, and quality of life among stroke patients presenting with a stiff knee gait. Stroke 2008;39(October (10)):2803-8].

Effect of upper limb botulinum toxin injections on impairment, activity, participation, and quality of life among stroke patients.

Background And Purpose: The purpose of this study was to study the effect of Botulinum toxin type A (BoNT-A) injections in spastic upper limb muscles on impairment, activity, participation and quality of life in chronic stroke patients.

Methods: BoNT-A (Dysport) was injected into several upper limb spastic muscles in a group of 20 patients. Neurological impairment (muscle tone and strength, dexterity, SIAS), activity (ABILHAND), participation (SATIS-Stroke), and quality of life (SF36) were assessed before and 2 months after the injections.

Quantitative assessment of anaesthetic nerve block and neurotomy in spastic equinus foot: a review of two cases.

Objective: To quantitatively evaluate the effect of motor nerve branch block and neurotomy of the soleus nerve on triceps surae spasticity, reviewing 2 cases.

Methods: Beside clinical assessment, we carried out a quantitative measurement of the stiffness of the ankle flexor muscles. The path length of the phase diagram between elastic and viscous stiffness quantifies the reflex response to movement and reflects the importance of the spasticity.

Effect of simultaneous botulinum toxin injections into several muscles on impairment, activity, participation, and quality of life among stroke patients presenting with a stiff knee gait.

Background And Purpose: Walking is an essential activity for daily life and social participation, and it is frequently limited after stroke. A lack of knee flexion during the swing phase (stiff knee) is one of the impairments that restrict walking ability among patients with hemiparetic spasticity. Our purpose was to study the effect of Botulinum toxin type A (BoNT A) injections in several spastic muscles on the impairment, activity, participation, and quality of life of patients with chronic stroke presenting with a stiff knee gait.

Assessment of the Chignon dynamic ankle-foot orthosis using instrumented gait analysis in hemiparetic adults.

Objective: In the hemiplegic adult, gait is frequently perturbed by lack of ankle dorsiflexion at toe-off and may prompt prescription of an ankle-foot orthosis (AFO). Our objective was to evaluate the effect on gait of a dynamic AFO (the Chignon orthosis) in comparison with a prefabricated AFO (PAFO).

Method: Ten chronic hemiplegic patients performed a 10 m gait test and then underwent an instrumented treadmill gait test under three different sets of conditions (without an orthosis, with a PAFO and with a Chignon orthosis).

Experience with external pump trial prior to implantation for intrathecal baclofen in ambulatory patients with spastic cerebral palsy.

Objectives: To evaluate effectiveness and safety of intrathecal baclofen administration (ITB) testing with continuous infusion via an external pump before the implantation of an internal one in ambulatory spastic patients with cerebral palsy (CP).

Patients And Methods: Seven CP patients (3 diplegic, 4 quadriplegic - 18.4+/-7.