Quantitative ultrasonography of skeletal muscles in children: normal values

The purpose of this study was to establish normal values of muscle thickness, ratio of muscle thickness to subcutaneous fat thickness, and muscle echo intensity in children between 11 weeks and 16 years of age. Transverse scans of four muscles were made by standardized real-time ultrasound examination. The scans were digitized, and mean echo intensity was measured using gray-scale analysis. A multiple regression equation was used to study which independent parameter (age, height, weight, or sex) influenced the variables for each muscle. Muscle thickness depended on the child's weight. The other parameters did not significantly influence muscle thickness after correction for weight. The ratio of muscle thickness to subcutaneous fat thickness depended on age. Echo intensity showed no correlation with either of the variables. As a result, all normal values, including the equation to calculate them, are described. These normal data may help to determine the diagnostic value of muscle ultrasound in children with suspected neuromuscular disease.     

Malignant transformation of craniopharyngioma with detailed follow‐up

A 29‐year‐old male patient was admitted into hospital with the main complaint of progressive visual disturbance. Both CT SCAN and MRI demonstrated a cystic‐solid contrast‐enhancing sellar‐suprasellar mass with obvious calcification. Histopathological examination of the first resected specimen showed a typical appearance of adamantinomatous craniopharyngioma. The patient received gamma knife therapy after his first operation because of partial tumor removal. He experienced two relapses in the subsequent 2 years, for which only surgical resection was performed. The later histopathology presented malignant appearance with tumor cells moderate to severe pleomorphism, hyperchromasia, increased nuclear cytoplastic ratio, high mitotic activity (30/10 high power fields) and focal coagulative necrosis. The patient died 9 months after identification of histologic malignancy. Clinical and histopathological features, biological behavior of one case of malignant craniopharyngioma were discussed, with a brief review of the relevant literature.     

Efficacy and safety of creatine supplementation in juvenile dermatomyositis: A randomized,double‐blind,placebo‐controlled crossover trial

Introduction: It has been suggested that creatine supplementation is safe and effective for treating idiopathic inflammatory myopathies, but no pediatric study has been conducted to date. The objective of this study was to examine the efficacy and safety of creatine supplementation in juvenile dermatomyositis (JDM) patients. Methods: In this study, JDM patients received placebo or creatine supplementation (0.1 g/kg/day) in a randomized, crossover, double‐blind design. Subjects were assessed at baseline and after 12 weeks. The primary outcome was muscle function. Secondary outcomes included body composition, aerobic conditioning, health‐related quality of life, and muscle phosphocreatine (PCr) content. Safety was assessed by laboratory parameters and kidney function measurements. Results: Creatine supplementation did not affect muscle function, intramuscular PCr content, or any other secondary outcome. Kidney function was not affected, and no side effects were reported. Conclusions: Twelve weeks of creatine supplementation in JDM patients were well‐tolerated and free of adverse effects, but treatment did not affect muscle function, intramuscular PCr, or any other parameter. Muscle Nerve 53 : 58–66, 2016     

Pharmacological and non‐drug treatment of child bipolar I disorder during prospective eight‐year follow‐up

Geller B, Tillman R, Bolhofner K, Zimerman B. Pharmacological and non‐drug treatment of child bipolar I disorder during prospective eight‐year follow‐up.
Bipolar Disord 2010: 12: 164–171. © 2010 The Authors.
Journal compilation © 2010 John Wiley & Sons A/S. Objectives: The Phenomenology and Course of Pediatric Bipolar Disorders study, a National Institute of Mental Health‐funded study of child bipolar I disorder (BP‐I) begun in 1995, is a prospective follow‐up study that included collecting pharmacological and non‐drug treatment data. Methods: There were 115 first‐episode subjects who fit full DSM‐IV criteria for BP‐I, mixed or manic phase, with severity scores in the clinically impaired range, ascertained by consecutive new case ascertainment. Subjects were assessed with the Washington University in St. Louis Kiddie Schedule for Affective Disorders and Schizophrenia (WASH‐U‐KSADS), given separately to parents about their children and to children about themselves. All treatment was provided by the subjects’ own community practitioners, exactly as if they had not been in the research study. Thus, families were only seen for research assessments, and research staff were not at all involved in their treatment. Data on type, dose, and duration of pharmacological and non‐drug treatment were collected. During follow‐up, 93.9% (n = 108) were assessed at each of the nine assessment times. Results: During the eight years, only 62.6% received any antimanic medication (antipsychotic, anticonvulsant, lithium) at any time. Percents who received non‐antimanic medication included 77.4% medication for attention‐deficit hyperactivity disorder and 64.3% antidepressants. A total of 67.8% of subjects were taking two or more concurrent medication classes. Subjects ascertained from psychiatric versus pediatric sites received antimanics significantly more frequently (p = 0.006). Earlier recovery during eight‐year follow‐up was predicted by greater percent of weeks on lithium (p = 0.017). Conclusions: Given these findings, and the poor prognosis from prospective follow‐up of this sample reported elsewhere, there is a need for further research that informs the development of effective treatment strategies.     

Near‐infrared spectroscopy during exercise and recovery in children with juvenile dermatomyositis

Introduction: We hypothesized that microvascular disturbances in muscle tissue play a role in the reduced exercise capacity in juvenile dermatomyositis (JDM). Methods: Children with JDM, children with juvenile idiopathic arthritis (clinical controls), and healthy children performed a maximal incremental cycloergometric test from which normalized concentration changes in oxygenated hemoglobin (Δ[O₂Hb]) and total hemoglobin (Δ[tHb]) as well as the half‐recovery times of both signals were determined from the vastus medialis and vastus lateralis muscles using near‐infrared spectroscopy. Results: Children with JDM had lower Δ[tHb] values in the vastus medialis at work rates of 25%, 50%, 75%, and 100% of maximal compared with healthy children; the increase in Δ[tHb] with increasing intensity seen in healthy children was absent in children with JDM. Other outcome measures did not differ by group. Conclusions: The results suggest that children with JDM may experience difficulties in increasing muscle blood volume with more strenuous exercise. Muscle Nerve, 2013     

One‐year follow‐up study of neuropathic pain in chronic inflammatory demyelinating polyradiculoneuropathy

We sought to gather information about frequency and features of neuropathic pain (NeP) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients and to investigate course of NeP during 1‐year follow‐up. Study included 105 patients diagnosed with CIDP. Patients with diabetes (N = 26) were excluded. NeP was diagnosed by the official guidelines and painDETECT questionnaire (PD‐Q). Medical Research Council Sum Score (MRC‐SS), INCAT disability and sensory scores, and Beck Depression Inventory were also measured. PD‐Q showed presence of NeP in 16 (20%) of 79 CIDP patients and their mean pain was moderate (5.1 ± 3.0 of 10). Diagnostic delay in CIDP patients with NeP was prolonged compared to CIDP patients without NeP (21 ± 28 vs 9 ± 12 months, P < .05). Slowly progressive course of the disease was more frequent in patients with NeP (81% vs 52%, P < .05). Patients with NeP had worse INCAT sensory score (P < .01), INCAT disability score (P < .05), MRC‐SS, as well as worse disease outcome at time of testing (P < .05). Depression was more common in patients with NeP (69% vs 17%, P < .01). During 1‐year follow‐up, majority of our CIDP patients had good control of NeP with gabapentinoids or amitriptyline. NeP was common in our cohort of non‐diabetic CIDP patients. It was associated with worse functional disability, worse sensory deficit, and depression. Special attention should be paid to CIDP patients with NeP because they request additional symptomatic therapy that appeared efficacious in our cohort.     

Quantitative skeletal muscle ultrasonography in children with suspected neuromuscular disease

We determined prospectively the diagnostic value of quantitative ultrasonography in detecting neuromuscular disorders in children. Ultrasonographic scans of four muscles were made in 36 children with symptoms or signs suggestive of neuromuscular disease, such as muscle weakness and hypotonia. The muscle thickness, ratio of muscle thickness to subcutaneous fat thickness, and echo intensity were determined in each muscle. The echo intensity was measured using computer-assisted gray-scale analysis. Thirteen of the 36 patients had a neuromuscular disorder (6 a myopathy and 7 a neuropathy). Differentiation between neuromuscular diseases and nonneuromuscular diseases could be made on the basis of echo intensities with a sensitivity of 92%, a specificity of 90%, a positive predictive value of 86%, and a negative predictive value of 95%. We conclude that computer-assisted quantitative analysis of muscle echo intensity is a reliable method to discriminate between neuromuscular and nonneuromuscular diseases in children.     

Callosal tissue loss in multiple system atrophy—A one‐year follow‐up study

Multiple system atrophy (MSA) is a neurodegenerative disease not only affecting the basal ganglia, brainstem, cerebellum, and intermediolateral cell columns of the spinal cord but also the cerebral cortex. Clinically, cerebellar (MSA‐C) and parkinsonian variants of MSA (MSA‐P) are distinguished. We investigated 14 MSA patients (10 MSA‐C, 4 MSA‐P, men: 7, women: 7; age: 61.1 ± 3.3 years) and 14 matched controls (men: 7, women: 7; age: 58.6 ± 5.1 years) with voxel‐based morphometry (VBM) to analyze gray and white matter differences both at baseline and at follow‐up, 1 year later. Baseline comparisons between patients and controls confirmed significantly less gray matter in MSA in the cerebellum and cerebral cortex, and significantly less white matter in the cerebellar peduncles and brainstem. Comparisons of tissue‐loss profiles (i.e., baseline versus follow‐up) between patients and controls, revealed white matter reduction in MSA along the middle cerebellar peduncles, reflecting degeneration of the ponto‐cerebellar tract as a particularly prominent and progressive morphological alteration in MSA. Comparisons between baseline and follow‐up, separately performed in patients and controls, revealed additional white matter reduction in MSA along the corpus callosum at follow‐up. This was replicated through additional shape‐based analyses indicating a reduced callosal thickness in the anterior and posterior midbody, extending posteriorly into the isthmus. Callosal atrophy may possibly reflect a disease‐specific pattern of neurodegeneration and cortical atrophy, fitting well with the predominant impairment of motor functions in the MSA patients. © 2010 Movement Disorder Society     

A practical approach to remote longitudinal follow‐up of Parkinson's disease: The FOUND study

The objective of this study was to examine a remote method for maintaining long‐term contact with Parkinson's disease (PD) patients participating in clinical studies. Long‐term follow‐up of PD patients is needed to fill critical information gaps on progression, biomarkers, and treatment. Prospective in‐person assessment can be costly and may be impossible for some patients. Remote assessment using mail and telephone contact may be a practical follow‐up method. Patients enrolled in the multi‐center Longitudinal and Biomarker Study in Parkinson's Disease (LABS‐PD) in‐person follow‐up study in 2006 were invited to enroll in Follow‐up of Persons With Neurologic Diseases (FOUND), which is overseen by a single center under a separate, central institutional review board protocol. FOUND uses mailed questionnaires and telephone interviews to assess PD status. FOUND follow‐up continued when LABS‐PD in‐person visits ended in 2011. Retention and agreement between remote and in‐person assessments were determined. In total, 422 of 499 (84.5%) of eligible patients volunteered, AND 96% of participants were retained. Of 60 patients who withdrew consent from LABS‐PD, 51 were retained in FOUND. Of 341 patients who were active in LABS‐PD, 340 were retained in FOUND (99.7%) when the in‐person visits ceased. Exact agreement between remote and in‐person assessments was ≥ 80% for diagnosis, disease features (eg, dyskinesias), and PD medication. Correlation between expert‐rated and self‐reported Unified Parkinson's Disease Rating Scale and Movement Disorder Society Unified Parkinson's Disease Rating Scale, which were examined at times separated by several months, was moderate or substantial for most items. Retention was excellent using remote follow‐up of research participants with PD, providing a safety net when combined with in‐person visits, and also is effective as a stand‐alone assessment method, providing a useful alternative when in‐person evaluation is not feasible. © 2014 International Parkinson and Movement Disorder Society     

A 15‐year follow‐up of first unprovoked seizures: a prospective study of 200 children

Epilepsy is associated with an extended spectrum of behaviour, psychiatric problems, and learning difficulties. The aim of this study was to establish the natural history of children with first unprovoked seizures. We studied prospectively 200 children under the age of 11 years who attended hospital emergency with a first unprovoked seizure. Demographic variables, personal and family history, neurological examination, EEG, psychiatric, and cognitive and educational profiles were analysed. Patients who developed epilepsy were characterised with respect to: time to relapse, remission rate, duration of epilepsy, neuroimaging, aetiology, epileptic syndrome, and therapeutic regimen. These results were compared to data of patients who had a single seizure over a follow‐up period of 15 years. Thirty percent of children who had a first unprovoked seizure developed epilepsy. Partial seizure type was a statistically significant variable for the development of epilepsy. An EEG with epileptic abnormalities proved to be the main risk factor for recurrence. Fifteen years later, the group with epilepsy exhibited a 2.6 greater risk of psychiatric and academic comorbidities, compared to the group without epilepsy.