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Biomedical subjects

Jan J Rotteveel

Publications and source records attributed to Jan J Rotteveel.

12 recordsLinked to original sources

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. The TS sample performed similarly to the normal control group in both tasks. The CEFT results did not show the expected preference for local processing in children with PDD-NOS. However, the Rey CFT data revealed that the children with this lesser variant of PDD processed visuospatial information in a fragmented way and were deficient in global processing.

Asperger Syndrome↗

Responses to lumbar magnetic stimulation in newborns with spina bifida.

Searching for a tool to quantify motor impairment in spina bifida, transcranial and lumbar magnetic stimulation were applied in affected newborn infants. Lumbar magnetic stimulation resulted in motor evoked potentials in both the quadriceps muscle and the tibialis anterior muscle in most (11/13) subjects. However, transcranial magnetic stimulation did not lead to any response at all. A strong left-to-right correlation existed for amplitude and for latency. Lumbar magnetic stimulation proved to be applicable in newborn infants with spina bifida. Although current concepts regarding spina bifida suppose lower motor neuron dysfunction, the results of this study suggest that lower motor neuron integrity is at least partly preserved after birth. Transcranial magnetic stimulation does not lead to responses in healthy newborn infants because of insufficient synaptogenesis, myelinogenesis, and axon thickness. Therefore, conclusions on upper motor neuron function in spina bifida cannot be drawn. To what extent the method used here can achieve the aim of quantifying motor impairment is a matter of further study.

Electromyography↗

Chronic herpes simplex virus encephalitis in childhood.

Although herpes simplex virus is a major cause of acute encephalitis in childhood, chronic herpes simplex virus encephalitis has only rarely been reported. This report presents a case of chronic herpes simplex virus encephalitis in a 6-year-old female. Diagnosis was based on the detection of herpes simplex virus deoxyribonucleic acid by polymerase chain reaction in combination with the cerebrospinal fluid/serum ratio of herpes simplex virus-specific immunoglobulin G, the presence of herpes simplex virus-specific oligoclonal immunoglobulin G bands in cerebrospinal fluid, and calcifications in the temporal regions found on cerebral computed tomographic scan. Prolonged antiviral therapy was beneficial to later mental development.

Antiviral Agents↗

Drooling in children with cerebral palsy: effect of salivary flow reduction on daily life and care.

The purpose of this study was to investigate the effect of salivary flow reduction on daily life and provision of care in children with cerebral palsy (CP). Parents of children with CP were asked to fill in a questionnaire on the impact of drooling on the daily life of their children and their families and the data were then analyzed. Forty-five children with severe drooling (28 males, 17 females; mean age 9y 5mo [SD 3y 7mo]; range 3 to 16y) were monitored before and after receiving medication (scopolamine and botulinum toxin) to reduce salivary flow. Type of CP included hypotonia (n = 1), spastic paresis (n = 27), and mixed motor disorders with spastic and dyskinetic paresis (n = 17). Eight children were independently ambulant and 37 children were wheelchair users. Thirty-four children had learning disability with a developmental age of below 6 years. Six participants dropped out of the study; data on 39 children were analyzed. Results showed that anticholinergic agents effectively reduced salivary flow. Drooling diminished substantially and this was accompanied by a significant reduction in care needs, making daily care less demanding. The amount of reported damage to communication devices and computers decreased. In addition to the evaluation of primary variables, such as the salivary flow rate, investigation of impact of drooling on daily life provides useful information about the outcome of treatment for reduction in drooling.

Activities of Daily Living↗

Social interaction and self-esteem of children with cerebral palsy after treatment for severe drooling.

UNLABELLED: The impact of salivary flow reduction following medication (scopolamine and botulinum neurotoxin) on social interaction and emotional development (self-esteem) was evaluated in a group of 45 children with cerebral palsy who suffered from severe drooling. The children ranged in age from 3 to 16 years (median 9.1 years); 28 were male, 17 female. A questionnaire to document the impact of drooling on social interaction and self-esteem for both the children and their parents was developed and administered during the use of scopolamine and up to 24 weeks after intraglandular botulinum neurotoxin in the submandibular glands. The reduction of drooling was related to increased social contacts with peers. In addition, parents perceived that the impact of drooling on the level of the child's satisfaction on physical appearance, relations within the extended family, and life in general increased. Although medication led to (temporary) positive changes, many social and emotional consequences remained unchanged. CONCLUSION: Interventions to treat drooling should not only be evaluated using measurements of drooling, but the consequences on social interaction and self-esteem should also be assessed.

Adolescent↗

Fright is a provoking factor in vanishing white matter disease.

Leukoencephalopathy with vanishing white matter is an inherited disorder with a chronic progressive disease course and additional episodes of rapid neurological deterioration. These episodes typically are provoked by febrile infections or minor head trauma. We report on two patients who experienced an episode of rapid neurological deterioration after a fright.

Brain Diseases↗

Assessment of salivary flow rate: biologic variation and measure error.

OBJECTIVE: To investigate the applicability of the swab method in the measurement of salivary flow rate in multiple-handicap drooling children. To quantify the measurement error of the procedure and the biologic variation in the population. STUDY DESIGN: Cohort study. METHODS: In a repeated measurements design, a baseline series of salivary flow rates were obtained from 45 children. The within-subject SD (SW) was calculated to express the measurements error according to a procedure introduced by Bland and Altman. RESULTS: Two hundred twenty-four samples (mean 0.40 mL/min, SD 0.19 mL/min) were obtained and analyzed. The results of this study indicate that consistent scores were obtained at subsequent measurements, and good parity existed between the two measurements of salivary flow rate at each session. The SW could be estimated (0.11 mL/min), which was applied to quantify the specific variation of the salivary flow rate in our population. CONCLUSION: According to Bland and Altman, the SW, which is a quantification of the measurement error and biologic variation, was found to be a useful tool to evaluate the obtained baseline salivary flow rate measurements. The swab method can be used to evaluate salivary flow rates in drooling children with cerebral palsy during interventional studies that aim to reduce saliva production.

Adolescent↗

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. Drooling severity was measured at baseline, during application of scopolamine, and at different intervals after BoNT injections up to 24 weeks, using the Drooling Quotient (DQ), the Teacher Drooling Scale (TDS), and Visual Analog Scales (VAS). RESULTS: Drooling was reduced during scopolamine application as well as after BoNT injections. Compared with baseline, the mean DQ showed a significant decrease throughout the study. Greatest reductions were achieved 2 to 8 weeks after BoNT injection. No significant differences were found between scopolamine measurements and those up to 24 months after BoNT injection. Using VAS, parents recorded the effect on drooling in which significant differences were found between baseline VAS score and all follow-up assessments. According to our definition of "success to therapy," demanding a 2-point decrease on the TDS, 61.5% of patients responded to BoNT injections. Analysis of the DQ demonstrated a response rate of 53% of the patients to scopolamine and 48.7% to BoNT until 24 weeks after BoNT injections, the actual duration of this study. As a reaction to scopolamine, 71.1% of the patients had moderate to severe side effects. Only nonsevere, incidental side effects were reported after BoNT injections. CONCLUSIONS: During scopolamine application as well as after intraglandular BoNT injections, a clinically relevant reduction in drooling was achieved in children with CP, demonstrating maximum effect 2 to 8 weeks after injections. This is the first controlled clinical trial that confirmed a significant effect of BoNT injections in the treatment of drooling. General anesthesia was needed for all children. BoNT injections show fewer and less serious side effects than transdermal scopolamine treatment.

Administration, Cutaneous↗

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. Single-voxel proton MR spectra were acquired from cerebral white matter and gray matter in 16 patients, with follow-up studies in 11. LCModel fits were used to determine brain metabolite levels. RESULTS: MR imaging showed retardation of myelination and a mild persistent myelin deficit. A zone of increased signal intensity was seen in the periventricular white matter on T2-weighted images. Proton MR spectroscopy of white matter revealed a prominent peak at 1.3 ppm, normal levels of N-acetylaspartate, and elevated levels of creatine (+14%), choline (+18%), and myo-inositol (+54%). MR imaging and proton MR spectroscopy of gray matter were normal. In the two patients examined during the first years of life, abnormalities on MR imaging and proton MR spectroscopy gradually emerged and then stabilized, as in all other patients. CONCLUSION: Abnormalities on MR imaging and proton MR spectroscopy emerge during the first years of life and are similar in all patients with SLS, but the severity varies. The changes are confined to cerebral white matter and suggest an accumulation of lipids, periventricular gliosis, delayed myelination, and a mild permanent myelin deficit.

Adolescent↗

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. Subsequently, patients with severe drooling were included in a clinical study. Salivary flow was measured under standardized conditions, and BTX was injected into the submandibular glands with the patient under general anesthesia and with ultrasound guidance as an outpatient procedure or during a short stay at the daytime care unit. RESULTS: BTX for each gland should be diluted in a volume of 1 to 1.5 mL saline to achieve adequate spreading within the gland and to diminish the risk of diffusion into surrounding structures. With ultrasound guidance, separate structures surrounding the glands and structures within the glandular parenchyma are well recognized and injection errors can be avoided. CONCLUSIONS: With the procedure described, it is possible to accurately inject BTX directly into the glandular parenchyma and to visualize spreading of the fluid in the glandular parenchyma. It is found to be a safe method that guarantees an optimal clinical effect and avoids potentially harmful side effects. We recommend ultrasound guidance if injections of BTX into the salivary glands are considered.

Botulinum Toxins, Type A↗