The use of accelerometry as a tool to measure disturbed nocturnal sleep in Parkinson's disease.

Sleep disturbances are common in Parkinson's disease (PD). We used the Parkinson's KinetiGraph (PKG), an objective movement recording system for PD to assess night time sleep in 155 people aged over 60 and without PD (controls), 72 people with PD (PwP) and 46 subjects undergoing a Polysomnogram (PSG: 36 with sleep disorder and 10 with normal sleep). The PKG system uses a wrist worn logger to capture acceleration and derive a bradykinesia score (BKS) every 2 min over 6 days. The BKS ranges from 0-160 with higher scores associated with lesser mobility. Previously we showed that BKS > 80 were associated with day time sleep and used this to produce scores for night time sleep: Efficiency (Percent time with BKS > 80), Fragmentation (Average duration of runs of BKS > 80) and Sleep Quality (BKS > 111 as a representation of atonia). There was a fair association with BKS score and sleep level as judged by PSG. Using these PKG scores, it was possible to distinguish between normal and abnormal PSG studies with good Selectivity (86%) and Sensitivity (80%). The PKG's sleep scores were significantly different in PD and Controls and correlated with a subject's self-assessment (PDSS 2) of the quality, wakefulness and restlessness. Using both the PDSS 2 and the PKG, it was apparent that sleep disturbances were apparent early in disease in many PD subjects and that subjects with poor night time sleep were more likely to have day time sleepiness. This system shows promise as a quantitative score for assessing sleep in Parkinson's disease.

[Central nervous system involvement at diagnosis and at relapse in children with neuroblastoma]. / Zajecie osrodkowego ukladu nerwowego w chwili rozpoznania i we wznowach u dzieci ze zwojakiem zarodkowym wspólczulnym.

Central nervous system (CNS) involvement in the course of neuroblastoma (NBL) in children is relatively rare. However, it seems to become serious clinical problem in the group of patients from high risk group. They presently achieve longer time of survival caused by employment of more intensive treatment modalities. The aim of the study was clinical evaluation of the patients over 1 year of age with stage 4 NBL with CNS involvement, both at diagnosis and at relapse. From 1997 to 2007, 117 patients (age 0.2-13.5 years) started NBL treatment. In 58 children over 1 year, stage 4 of disease was diagnosed. In 4 (6.9%) cases the CNS involvement was found at diagnosis. In 5 patients (8.6%) the isolated relapse in brain was diagnosed. The clinical symptoms caused by increased intracranial pressure were observed in all patients at relapse. In the case of initial involvement no neurological symptoms were observed. All 5 children with CNS involvement as isolated relapse did not present with infiltration of skull bones, whereas at initial diagnosis the brain lesions were continuous with bone metastases. Among 5 patients with isolated relapse 4 died because of NBL progression. Among 4 children with initial CNS involvement 1 died due to haemorrhage to CNS. Probably brain involvement at initial NBL diagnosis is not an additional negative prognostic factor. Because of extremely poor prognosis in patients with CNS relapse it should be advised to consider possible implementation of preventive treatment. It is also necessary to invent new more effective treatment methods.

[Hemophagocytic lymphohistiocytosis: diagnostic problems in pediatrics]. / Limfohistiocytoza hemofagocytarna: problemy diagnostyczne w pediatrii.

Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome (HS), is a life-threatening hyperinflammatory condition caused by uncontrolled proliferation of activated lymphocytes and histiocytes producing excessively proinflammatory cytokines. HLH can occur in all age groups. The most common syndromes are: prolonged fever, hepatosplenomegaly, unspecific neurological symptoms, pancytopenia, hemophagocytosis. Characteristic biochemical markers include high triglycerides, ferritin and decreased fibrinogen. There are two forms of HLH, primary (genetic) and acquired (secondary to infections, malignant and autoimmune diseases). Occurrence of both forms of HLH, acquired and genetic, is induced by infections, usually viral or by other triggering agents. Despite established diagnostic criteria many cases probably remain unrecognized. The main diagnostic difficulty is low specificity of symptoms, and in secondary form contemporary occurrence of symptoms of underlying disease. The aim of HLH treatment is to suppress hyperinflammation, what can be achieved by use of immunosuppressive/immunomodulating agents or cytostatics. Patients with genetic form of HLH require hematopoietic stem cell transplantation. Awareness of the symptoms and diagnostic criteria of HLH is important for early diagnosis and immediate application of life-saving therapy.