Comparison of 3 instruments to measure muscle strength in children: A prospective study.

Aim: To establish which instrument is the most valid and reliable measure of muscle strength in children aged 4-11 years and can improve the diagnostic procedure in children with suspected myopathy to spare more of them from muscle biopsy.

Methods: In a prospective study over a 2 year period, 22 patients aged 4-11 years were recruited. They had all been referred to our specialist centre on the suspicion of myopathy.

Muscle reflexes and synergies triggered by an unexpected support surface height during walking.

An important phase in the step cycle is foot contact. When the moment of foot contact differs from the one expected, a fast response is needed. Such a mismatch can be caused by hitting a support surface earlier or later than expected.

Validity and reproducibility of the Jamar dynamometer in children aged 4-11 years.

Purpose: Validity and reproducibility of the Jamar dynamometer were evaluated in children aged 4-11 years.

Method: Hand grip strength was measured on the dominant side and non-dominant side in 67 patients who had been referred to our specialist centre in the past 3 years because of suspected myopathy. All the patients had had muscle biopsy.

A new motor performance test in a prospective study on children with suspected myopathy.

In the development of a new diagnostic motor performance test to spare more children from painful muscle biopsy, seven functional items were used to measure muscle strength and muscle endurance in a prospective study on new patients. Over a 2-year period, 22 patients (12 males, 10 females; mean age 8y 1mo [SD 2y 6mo], range 4-11y) were recruited for the study. They had all been referred with suspected myopathy.

Fragmented visuospatial processing in children with pervasive developmental disorder.

Children diagnosed with Pervasive Developmental Disorder Not Otherwise Specified (PDD-NOS) and Asperger Syndrome (AS) may be characterised by a similar perceptual focus on details as children with autistic disorder (AD). This was tested by analysing their performance in a visuoperceptual task [the Children's Embedded Figure Test (CEFT)] and a graphic reproduction task [the Rey Complex Figure Task (Rey CFT)]. Control groups were children with Tourette Syndrome (TS) and typically developing children.

Validity and reproducibility of hand-held dynamometry in children aged 4-11 years.

Objective: To evaluate validity and reproducibility of hand-held dynamometry in 11 different muscle groups in children.

Design And Patients: Maximum isometric muscle strength was measured with a calibrated hand-held dynamometer in 61 patients aged 4-11 years who had been referred to our specialist centre in the past 3 years because of suspected myopathy. All the patients had had muscle biopsy.

Validity and reproducibility of a new diagnostic motor performance test in children with suspected myopathy.

To spare more children from painful muscle biopsy, a new non-invasive diagnostic motor performance test is undergoing development. Fifteen functional items were used to measure muscle strength and muscle endurance in 68 patients (47 males, 21 females; mean age 7y 8mo, SD 2y 2mo; range 4 to 11y), who had been referred to our specialist centre in the past 3 years on suspicion of myopathy. All the patients had undergone muscle biopsy.

Effect of botulinum toxin in the treatment of drooling: a controlled clinical trial.

Objective: To investigate the clinical effectiveness of botulinum neurotoxin type A (BoNT) to reduce drooling in children with cerebral palsy (CP).

Methods: A controlled clinical trial was performed in which the results of single-dose BoNT injections in the submandibular glands were compared with treatment with scopolamine. Forty-five children who had CP and experienced severe drooling were enrolled.

MR imaging and proton MR spectroscopic studies in Sjögren-Larsson syndrome: characterization of the leukoencephalopathy.

Background And Purpose: Sjögren-Larsson syndrome (SLS) is a neurocutaneous syndrome caused by a genetic enzyme deficiency in lipid metabolism. Our purpose was to characterize the nature of the cerebral involvement in SLS.

Methods: MR imaging was performed in 18 patients (aged 5 months to 45 years) and repeated in 14.

Planning of syllables in children with developmental apraxia of speech.

The aim of the present study was to investigate whether children with developmental apraxia of speech (DAS) show a deficit in planning syllables in speech production. Six children with DAS and six normally speaking (NS) children produced high- and low-frequency of occurrence syllable utterances, in which the syllable structure was systematically manipulated in an otherwise unchanging phoneme sequence. Anticipatory coarticulation, using second formant trajectories, and durational structure were analysed.

The treatment of drooling by ultrasound-guided intraglandular injections of botulinum toxin type A into the salivary glands.

Objective: The aim of the study was to present the background, procedure, and technique of bilateral ultrasound-guided, single-dose injections of botulinum toxin type A (BTX) into the salivary glands in patients with severe drooling.

Study Design: Clinical trial.

Methods: Initially, an in vitro study was performed to determine the volume of the dilution of BTX required for optimal spreading and to gain insight in the spreading pattern of the fluid in the submandibular gland.

Coarticulation patterns in children with developmental apraxia of speech.

The aim of this study was to enhance our insight into the underlying deficit in developmental apraxia of speech (DAS). In particular, the involvement of planning and/or programming of speech movements in context was tested by analysing coarticulatory cohesion. For this purpose, second formant frequency measurements were conducted in repetitions of nonsense utterances ([[symbol: see text]] C = /s,x,b,d/; V = /i.

Diagnosis and differential diagnosis of muscle cramps: a clinical approach.

Muscle cramps are one of the most frequently occurring neuromuscular features. They occur normally in healthy persons, can be the manifestation of one of several cramp syndromes, but can also be the expression of various underlying neuromuscular disorders. We review the various types and boundaries of cramps, ranging from true muscle cramps to electromyographic silent muscle contractures (for example, Brody's disease) and cramps associated with dystrophinopathies.

Nonmuscular involvement in merosin-negative congenital muscular dystrophy.

The spectrum of nonmuscular involvement in six children with merosin-negative congenital muscular dystrophy is described. In all children, biochemical, neuroradiologic, cardiac, and neurophysiologic studies were performed. Cerebral structures that were myelinated at gestation, including internal capsule, corpus callosum, brainstem, and cerebellar white matter, demonstrated no abnormalities, whereas the periventricular and subcortical white matter, which were myelinated in the first postnatal year, demonstrated signs of leukoencephalopathy.