Cross sectional area reference values for sonography of peripheral nerves and brachial plexus

ObjectiveUltrasound measurements of the cross sectional area (CSA) variability have been recently introduced to quantify pathological changes in peripheral nerves (PN).MethodsReference values from 75 healthy subjects and their correlation to age, height, weight and sex are reported.ResultsThe mean values in PN were: (1) intranerve CSA-variability: median 1.05 (SD ± 0.13), ulnar 1.53 (SD ± 0.51), fibular 1.33 (SD ± 0.37), tibial 1.39 (SD ± 0.39), (2) internerve CSA-variability 1.76 (SD ± 0.37), (3) intraplexus CSA-variability 1.52 (SD ± 0.37), (4) side-to-side difference ratio of the CSA-variability: median 1.21 (SD ± 0.04), ulnar 1.2 (SD ± 0.25), fibular 1.19 (SD ± 0.23), tibial 1.28 (SD ± 0.24) and brachial plexus 1.19 (SD ± 0.23). CSA did not correlate with height in PN, but correlated with weight in the ulnar nerve [Guyon’s canal, r = 0.411, p = 0.0237, elbow r = 0.409, p = 0.0248]. Significant changes between sex were found only in the ulnar (Guyon’s canal, p = 0.0265), fibular (popliteal fossa, p = 0.0336) and sural nerve (p = 0.048). CSA decreased with age in the median (axilla, p = 0.0236), and radial nerve (spiral groove, p = 0.0037) and increased in the tibial nerve (ankle, p < 0.0001).ConclusionsThe CSA reference values reported seem to correlate at certain sites with age, weight and sex but not with height.SignificanceThe new CSA variability measures may be helpful in investigating pathologies of the PN.     

Nerve cross-sectional area in extremes of age

Introduction: Nerve cross-sectional area reference values have been reported for many nerves, but there have been few studies in pediatric and geriatric populations. This study was conducted to determine the influence of age on nerve cross-sectional area. Methods: Thirty-two children (3 months to 16 years) and 20 geriatric adults (67–92 years) without known neurologic conditions underwent bilateral ultrasound to measure the area of the following nerves: median at the wrist and forearm; ulnar at the wrist and elbow; radial in the spiral groove; sciatic in the distal thigh; fibular at the knee; tibial at the knee and ankle; and sural at the ankle. Results: In general, nerve cross-sectional area increased with age. Nerve size correlated most closely with age, but a correlation was also seen with body mass index. Conclusions: Nerve cross-sectional area increases with age, which is important to note when using ultrasound to evaluate children and geriatric patients. Muscle Nerve 47: 890–893, 2013     

Head circumference measurement of urban children aged between 6 and 12 in Malatya, Turkey

Head circumference (HC) is one of the most significant findings in physical examination, especially in the evaluation of the development and early diagnosis of neurological disorders in children. In the standard charts for developmental evaluation of Turkish children, there is no HC reference values for children over 6 years of age. Since the HC standards show differences among races and generations, many researchers have obtained normal values for their own populations, and recommend periodic reevaluation of these standards. In this study, the HC of 1826 healthy children (945 male, 881 female) aged between 6 and 12 years was measured in order to establish the Turkish standards. The sample represented various socioeconomic levels in the city of Malatya, Turkey. The study was conducted in ten schools and measurements were done twice by a pediatrician and the mean was recorded. Charts and graphs for boys and girls were prepared separately. Results were compared to the values of other populations. HC values of Turkish children were similar to that of Irish children. The data obtained in this study may replace the Nelhaus criteria to be used in clinics. However, a more widespread study should be carried out by including children from different regions of Turkey.     

A follow-up study of 29 borderline psychotic children 5 to 20 years after discharge

Twenty-nine children who during 1956 through 1970 were hospitalized for borderline psychosis were followed up in 1975. Twelve of them had received inpatient psychiatric treatment for 1–3 years. Seventeen were sent home for local, nonspecific help. At follow-up 67% of the treated group were no longer considered psychotic, compared with 70% of the untreated group. The two groups differ somewhat in a series of parameters, the treated group showing more severe psychopathological symptoms. The differences do not reach significant levels. No useful prognostic criteria were obtained from the study. The prognosis seems to be much better than for other types of psychosis in children.     

French validation of the sleep disturbance scale for children (SDSC) in young children (aged 6 months to 4 years)

The sleep disturbance scale for children (SDSC) has been translated and adapted into several languages and its psychometric properties are good. Notably, a French version has been validated for 4- to 16-year olds. The objective of the current study was therefore to adapt and validate the SDSC for French young children (aged 6 months to 4 years).Method421 French-speaking mothers of children aged between 6 months and 4 years completed the SDSC and the Brief Infant Sleep Questionnaire (BISQ) which is specifically for young children. Of these, 105 children were diagnosed with sleep disorders (clinical group) during a sleep consultation, and 316 completed the SDSC and BISQ in a network of nurseries (control group). Several factor analyses were conducted to identify the most consistent factor structure for this sample.ResultsThree items from the previous version were deleted due to lack of clinical relevance for this age group. The best factor analysis revealed six factors, comparable to the Italian version of the SDSC for children: Disorders of Initiating Sleep, Disorders of Maintaining Sleep, Sleep Hyperhidrosis, Sleep Breathing Disorders, Parasomnias, and Non-Restorative Sleep and Excessive Somnolence. This psychometric structure is reliable and aligned with expert diagnoses. The convergent validity, and divergent and internal reliability were acceptable.ConclusionThis study validates a 22-item SDSC for French young children. Coupled with some questions from the BISQ, the SDSC could therefore be used to facilitate the detection of sleep disturbances in children aged between 6 months and 4 years old.     

Quantitative gray‐scale analysis in skeletal muscle ultrasound: A comparison study of two ultrasound devices

Muscle ultrasound is a useful technique to detect neuromuscular disorders. Quantification of muscle echo intensity (EI) using gray‐scale analysis is more reliable and more sensitive compared with visual evaluation of the images. We devised a method to reliably use EI normal values established with one ultrasound device for use with another device. Based on measurements in a dedicated phantom and in 7 healthy subjects, a conversion equation was calculated to convert the mean EI. The reliability of this equation was next evaluated in a follow‐up study of 22 healthy children. Mean muscle EI could be reliably converted from one ultrasound device to another. This allows for normal values obtained with one device to be used with other devices, which is an important step forward toward the use of quantitative muscle ultrasound in daily clinical care. Muscle Nerve, 2009     

Nerve ultrasound findings differentiate Charcot-Marie-Tooth disease (CMT) 1A from other demyelinating CMTs

Objective

Ulnar/median motor nerve conduction velocity (MNCV) is ≤38?m/s in demyelinating Charcot-Marie-Tooth disease (CMT). Previous nerve high resolution ultrasound (HRUS) studies explored demyelinating CMT assuming it as a homogeneous genetic/pathological entity or focused on CMT1A.

Topiramate pharmacokinetics in children with epilepsy aged from 6 months to 4 years

PURPOSE: To study the pharmacokinetics of topiramate (TPM) at steady state in children younger than 4 years comedicated with other antiepileptic drugs (AEDs). METHODS: Twenty-two children aged 6 months to 4 years with pharmacoresistant partial or generalized epilepsy were enrolled in an open-label prospective study. Children were assigned to different groups according to comedication with enzyme-inducing AEDs (n = 8), valproic acid (VPA) (n = 6), or other AEDs not known to affect drug metabolism (neutral AEDs, n = 7). One child was receiving treatment with both enzyme-inducing AEDs and VPA. After dose titration, blood samples were collected at steady state just before and 0.5, 1, 1.5, 2, 4, 6, 8, and 12 h after the morning dose of TPM. Pharmacokinetic parameters were determined by a noncompartmental method. RESULTS: TPM apparent oral clearance (CL/F) was significantly higher in children taking enzyme-inducing AEDs (85.4 +/- 34.0 ml/h/kg) than in those receiving VPA (49.6 +/- 13.6 ml/h/kg) or neutral AEDs (46.5 +/- 12.8 ml/h/kg). Conversely, dose-normalized areas under the plasma TPM concentration curves (0-12 h) were significantly lower in enzyme-induced patients than in patients receiving VPA or other AEDs. CONCLUSIONS: Compared with children not receiving enzyme inducers, children younger than 4 years who receive concomitant enzyme-inducing AEDs need higher doses (milligrams per kilogram) to achieve comparable plasma TPM concentrations.     

A detailed analysis of symptomatic posterior cortex seizure semiology in children younger than seven years

PURPOSE: To analyze the semiology of seizure onset and evolution in young children with posterior cortex epilepsy (PCE), compare this with adult reports, and assess age-related differences. METHODS: We videotaped and analyzed 110 seizures from 18 patients with PCE, aged 3-81 months. All had a good prognosis after posterior epileptogenic zone removal. Ictal events were categorized by behavioral, consciousness, autonomic, and sensory features, as well as motor patterns, which included myoclonic, tonic, clonic, unclassified motor seizures, and epileptic spasm. A time-scaled data sheet was developed to record each epileptic event as onset, very early, early, or late manifestation. RESULTS: Patients had a high seizure frequency with < or =100 attacks/day; one third of them showed a cluster tendency. The mean duration of seizures was 67 s. The most common seizure components were motor manifestations (with myoclonic and tonic seizures), but psychomotor (automotor), hypomotor attacks, and isolated auras also were frequently observed. Clinical seizure spread was frequent; auras and visual sensory signs were difficult to record in this age. Typical phenomena during seizures included behavioral changes, ictal vocalization, smile, flush, head nod, oculomotor features, and late-appearing oral automatisms, whereas hypermotor and secondarily generalized tonic-clonic seizures were not seen. CONCLUSIONS: Our results suggest that PCE in infants and young children is very heterogeneous but shows important age-related features. Compared with adults, children with PCE have shorter but more frequent seizures; they rarely report aura or visual sensory signs, only sporadically develop hypermotor and secondarily generalized tonic-clonic seizures, whereas ictal smile, flush, head nod, and behavioral change are typical features at this age. Because of frequent subtle ictal phenomena, long-term video-EEG monitoring is a useful diagnostic tool with infants and young children with PCE.     

Nerve ultrasound score in distinguishing chronic from acute inflammatory demyelinating polyneuropathy

ObjectiveAim of this study was to develop and evaluate the applicability of an ultrasound score (Bochum ultrasound score – BUS) in distinguishing chronic (CIDP) from acute inflammatory demyelinating polyneuropathy (AIDP).Methods

• •Step 1: For the development of BUS 75 healthy-controls, 20 CIDP, 20 AIDP patients underwent US 4.55 ± 3.5 and 3.4 ± 2.91 years, respectively after onset. After comparing the distribution pattern and frequency of pathological US changes between the two study groups, we developed BUS, summarizing the cross sectional area (CSA) of: (1) the ulnar nerve in Guyons’ canal, (2) the ulnar nerve in upper-arm, (3) the radial nerve in spiral groove, (4) the sural nerve between the gastrocnemius muscle.
• •Step 2: The BUS underwent blinded evaluation in further 10 CIDP, 21 AIDP patients 3.8 ± 2.7 and 2.3 ± 1.5 years, respectively after onset.
• •Step 3: The BUS underwent blinded, prospective evaluation in 8 patients with acute/subacute polyradiculoneuropathy (5 CIDP, 3 AIDP) 2.6 ± 1.8 weeks after onset.

ResultsThe BUS showed a sensitivity of 90% and specificity of 90.4% (positive predictive value, PPV = 81.8%; negative predictive value, NPV = 95%) in distinguishing CIDP from AIDP, when they showed no differences in disease duration (p = 0.0551).In addition, the BUS distinguished subacute-CIDP from AIDP with a sensitivity of 80%, specificity of 100% (PPV = 100%, NPV = 75%).ConclusionThe BUS seems to allow a reliable distinction of CIDP from AIDP.SignificanceThe BUS may be helpful in distinguishing subacute-CIDP from AIDP.     

Reliability of bedside ultrasound of limb and diaphragm muscle thickness in critically ill children

Introduction: We evaluated the reliability of measuring muscle thickness with ultrasound in limbs and diaphragms of critically ill children and determined the sensitivity of these measures to quantitate muscle atrophy over time. Methods: An expert and trained novice sonographers prospectively measured limb and diaphragm muscle thickness in 33 critically ill children. Results: Expert and novice intrarater and interrater reliability were similar. Intraclass correlations (ICC) and coefficients of variation (CoV) were better in limbs (ICC > 0.9; CoV 3.57%–5.40%) than in diaphragm (ICC > 0.8; CoV novice 11.88%, expert, 12.28%). Mean relative difference in all muscles was small (1%-8%). Limits of agreement of the relative difference were smaller in limb (<13%–18%) than in diaphragm (<38%) muscles. Discussion: Muscle thickness is reliably measured with ultrasound by trained examiners in critically ill children. Our approach detects atrophy >13% in limb and >38% in diaphragm muscles. The smaller detectable change in limb muscles is likely due to their greater thickness. Muscle Nerve 59 :88–94, 2019     

CSF/plasma ratios of amino acids: Reference data and transports in children

Objective: We intended to investigate the effects of age, gender, and medications on amino acid cerebrospinal fluid (CSF)/plasma ratios in children, and to determine whether amino acid transports across the blood–CSF barrier in children differ from those in adults. Patients and methods: Amino acid concentrations measured by ion-exchange high-performance liquid chromatography were used (CSF from 99 children, simultaneously collected plasma from 76 children). Influence of age, gender, and medications on the amino acid CSF concentrations and CSF/plasma ratios were analyzed by linear multiple regression. Interactions of amino acid transports were analyzed by correlation analysis of CSF/plasma ratios. Results: CSF/plasma ratios of serine, valine, histidine, and arginine were higher in younger children. The glutamate CSF/plasma ratio was higher in older children. Serine, alanine, threonine, valine, and histidine CSF/plasma ratios were lower in females. Glutamine, methionine, tyrosine, and phenylalanine CSF/plasma ratios were elevated with valproate therapy. Serine, threonine, valine, leucine, and tyrosine CSF/plasma ratios were lower with clobazam therapy. The asparagine CSF/plasma ratio was elevated with pyridoxal phosphate therapy. Transports of most essential neutral amino acids interacted with each other, as did neutral amino acids with low molecular weights. Cationic amino acids interacted with each other and some essential neutral amino acids. Acidic amino acids had no interactions with other amino acids. Conclusions: Age, gender, and anti-epileptic drugs affect amino acid CSF/plasma ratios in children. Transport interactions between amino acids in children showed no remarkable difference from those of adults and generally followed the substrate specificities of multiple amino acid transport systems.     

Health-related quality of life and self-perceived competence of children assessed before and up to two years after epilepsy surgery

PURPOSE: To measure outcome of epilepsy surgery in terms of health-related quality of life (HrQoL) and self-perceived competence of children and adolescents. METHODS: Prospective longitudinal follow-up study of 21 patients (aged 6.2 to 16.8 years). Frequency and severity of seizures and epilepsy-related restrictions, HrQoL, and self-perceived competence were rated before and 6, 12, and 24 months after epilepsy surgery. Data were analysed nonparametrically and using analysis of variance for repeated measures. RESULTS: Group-wise, seizure parameters had almost normalized 6 months after surgery (p<0.001) and remained so. Two years after surgery, 15 (72%) patients were free of seizures. At the first postsurgical assessment, parents and children evaluated the frequency of activities as improved and that of seizures as diminished (p<0.05). Parents evaluated their children as having positive emotions more frequently (p<0.05). Children started to feel better about seizure variables in the second year after surgery. Two years after surgery, children perceived themselves as being socially more competent and having greater self-worth (p=0.05). In the adolescent group, several aspects of self-perceived competence improved shortly after surgery (p<0.05), whereas 2 years after surgery, athletic competence and romance had improved (p<0.05). CONCLUSIONS: In children and adolescents, epilepsy surgery sets the stage for improvement in HrQoL and in competence to participate in social and societal domains. Most improvement occurs in the first 6 months after surgery.     

大鼠脑干前庭核团向大脑前庭皮层的投射研究

目的观察Wistar大鼠脑干前庭核团是否存在直接向大脑皮质的纤维投射。方法健康Wistar大鼠20只,随机分为实验组(10只)和对照组(10只)。实验组脑干前庭内、外侧核团注射绿色荧光标记的顺行示踪剂刀豆凝集素,对照组脑干前庭内、外侧核团注射生理盐水。5d后处死大鼠,行大脑连续冰冻切片,荧光显微镜下观察荧光细胞在大脑皮质的分布及形态。结果绿色荧光标记的神经元主要位于大脑皮质的前肢感觉区、后肢感觉区和本体感觉区。结论 Wistar大鼠脑干前庭核团存在直接向大脑皮质的纤维投射,部分和本体感觉区相重叠。     

Brain tumors with symptomatic onset in the first two years of life

Eighty children who in the first 2 years of life had signs and symptoms relating to a cerebral neoplasm were studied over an 18-year period (1970–1987), the mean follow-up being 8.2 years. In each case age at onset, clinical presentation, tumor location and pathological diagnosis, extent of surgical resection, postoperative mortality, adjuvant therapy length of survival and quality of life were assessed. Supratentorial tumors (59%) were more common than infratentorial. The most frequent clinical presenting feature (70%) was increased intracranial pressure. Sixty-three patients (79%) were operated on and in all of these cases a histological diagnosis was obtained. Astrocytomas (41%) and medulloblastomas (20%) were the most common oncotypes. Surgical mortality was 17.4% and the 5-year survival rate was 54%. Quality of life was assessed for all long-term surviving patients using a specifically designed protocol. Normal physical and intellectual performances were found in 46% of cases, and all together 75% of the patients had sufficient autonomy in daily life. The prognosis is more closely related to tumor location and type of treatment than to histological diagnosis or age at onset.