Development of fine motor functions, especially drawing and handwriting, are crucial for performance in school, autonomy in everyday life and the general human development. A variety of neurological and psychiatric conditions in childhood and adolescence stunt the normal development of fine motor skills. We sought to define the normal development of the kinematic parameters of fine motor movement and determine the influence of gender, laterality of handedness and extracurricular training on fine motor skills. One hundred and eighty-seven children and adolescents (mean age: 11.6 years (+/-S.D.: 3.58), range: 6-18 years) were included in the study. Participants performed drawing and handwriting tasks on a digitizing graphic tablet. Movement and pressure data were transferred to a computer using a sensor-equipped pen and post-processed using CSWIN. Movements were segmented into strokes and several kinematic parameters were calculated. The kinematic parameters that were analyzed represented speed (frequency and stroke peak velocity), automation (number of direction changes of velocity per stroke), variability (variation coefficient of stroke peak velocity) and pressure. Progression of kinematic parameters for each movement domain of the handwriting and circle drawing tasks correlated significantly with age (Pearson's correlation, p<0.003). Speed, automation and pressure increased with age, whereas variability decreased. Nonlinear regressions revealed maturation of hand movements at a certain age. Age of completed maturation depended on the task complexity (drawing circles vs. handwriting) and kinematic parameters. In the speed and automation domains, handwriting movements finish maturing later than circle drawing. Male subjects drew circles at significantly higher speeds than female subjects. Fine motor practice and laterality of handedness did not influence kinematic parameters. A repeated measure ANOVA confirmed the significant interdependency between age and complexity level for speed and automation (p<0.001). The digitizing graphic tablet is an extremely valuable tool in determining the normal development of hand movement skills of children and adolescents by measuring relevant daily tasks like handwriting and drawing. In our study, we showed that future analyses of impaired movement in children and adolescents need to take age and gender into consideration. Furthermore, differences were observed in the maturation of different task complexities, the complex fine motor function reaching maturity later than basic and repetitive movement patterns.

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Background Deficiency of interleukin-36 receptor antagonist (DITRA) is a life threatening monogenic autoinflammatory disease caused by loss of function mutations in the IL36RN gene. Affected patients develop recurrent episodes of generalized pustular psoriasis (GPP) with systemic inflammation and fever. We here review and analyze the literature on pediatric DITRA patients who have been treated by biologicals targeting inflammatory cytokines. Method A database research was performed to identify all relevant articles on pediatric DITRA patients treated with biologicals. According to defined response criteria therapeutic efficacy was analyzed. Results Our literature research revealed 12 pediatric patients with DITRA who have received treatment with biologicals and we add a further not yet reported patient. Out of these 13 patients 10 were homozygous including 6 with the p.Leu27Pro, 3 with the p.Arg10 Argfs* and 1 with the p.Thr123Met mutation. 3 patients were compound heterozygous. In total 28 flares were treated with biological agents- targeting IL-1, IL-17, IL-12/23 and TNF-α. Complete response was achieved in 16 flares (57%), a partial reponse was seen in 2 flares (7%), and no response was observed in 10 flares (36%). Response rates were heterogeneous among the different agents. While complete/partial/no response with inhibition of TNF-alpha could be achieved in 7 (58%)/1 (8%)/4 (33%), the inhibition of IL-17 and of IL-12/23 led in each 4 flares to a 100% complete response. IL-1 inhibition led to complete/partial response in each 1 (13%) and was not effective in 6 (76%) flares. Of note, the novel patient was successfully treated with weekly dosed adalimumab. Conclusions DITRA is a rare disease that has to be considered in GPP with systemic inflammation and fever. It can be effectively treated with specific biological inhibition of TNF-alpha, IL-12/23 and IL- 17, while anti-IL-1 treatment seems less effective. Weekly dosed adalimumab appears to be a treatment option for pediatric patients. Further reports and studies of biological treated pediatric DITRA patients are warranted for evaluation of optimal treatment.

The digitizing tablet detected extent of fine motor function loss at varying levels of complexity of pediatric cerebellar tumor survivors. This tool promises to be a potentially effective method for measuring fine motor function in clinical trials and may be helpful in studying mechanisms of neurotoxicity in posterior fossa tumor patients as well as success of rehabilitation.

These findings support the notion that long-term neurocognitive deficits of pediatric posterior fossa tumor survivors significantly correlate with brain tissue damage. The cerebellum plays a role in regulating higher-order functions. On the contrary, the extent brain injury is not detected by HrQoL assessment.

Extracerebral masses involving the skull base in children and adolescents are rare. We report on a 4-month-old infant who was diagnosed with a large extracerebral mass in the anterior fossa. Subtotal resection to prevent impending blindness was complicated by almost fatal bleeding. Histopathologically, vessel malformation, epitheloid hemangioendothelioma, and angiosarcoma were possible diagnoses. The residual tumor regressed spontaneously and completely within 6 months after surgery. Retrospectively, histologic and radiologic findings and clinical course prompted the diagnosis of an infantile hemangioendothelioma. To our knowledge, this is the first report on an infantile hemangioendothelioma of the skull base.

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A 15-year-old girl was diagnosed with a spinal anaplastic pilocytic astrocytoma. The histologic features were similar to pilocytic astrocytoma WHO grade I, but with an increased mitotic rate, high nuclear pleomorphism, microvascular proliferation, and necrosis. The tumor was subtotally resected and treated with chemotherapy and irradiation. Four years after diagnosis no local progression was seen. At the age of 17, the patient developed a supratentorial primitive neuroectodermal tumor. We report the first case of a primary anaplastic pilocytic astrocytoma and primary primitive neuroectodermal tumor occurring in the same patient.