Motor inhibition and its contribution to recovery of dexterous hand use after stroke.

Recovery of dexterous hand use is critical for functional outcome after stroke. Grip force recordings can inform on maximal motor output and modulatory and inhibitory cerebral functions, but how these actually contribute to recovery of dexterous hand use is unclear. This cohort study used serially assessed measures of hand kinetics to test the hypothesis that behavioural measures of motor modulation and inhibition explain dexterity recovery beyond that explained by measures of motor output alone.

Recovery and Prediction of Bimanual Hand Use After Stroke.

Objective: To determine similarities and differences in key predictors of recovery of bimanual hand use and unimanual motor impairment after stroke.

Method: In this prospective longitudinal study, 89 patients with first-ever stroke with arm paresis were assessed at 3 weeks and 3 and 6 months after stroke onset. Bimanual activity performance was assessed with the Adult Assisting Hand Assessment Stroke (Ad-AHA), and unimanual motor impairment was assessed with the Fugl-Meyer Assessment (FMA).

Quantitative Assessment of Hand Spasticity After Stroke: Imaging Correlates and Impact on Motor Recovery.

This longitudinal observational study investigated how neural stretch-resistance in wrist and finger flexors develops after stroke and relates to motor recovery, secondary complications, and lesion location. Sixty-one patients were assessed at 3 weeks (T1), three (T2), and 6 months (T3) after stroke using the NeuroFlexor method and clinical tests. Magnetic Resonance Imaging was used to calculate weighted corticospinal tract lesion load (wCST-LL) and to perform voxel-based lesion symptom mapping.

More complications with uncemented than cemented femoral stems in total hip replacement for displaced femoral neck fractures in the elderly.

Background and purpose - Total hip replacement (THR) is the preferred method for the active and lucid elderly patient with a displaced femoral neck fracture (FNF). Controversy still exists regarding the use of cemented or uncemented stems in these patients. We compared the effectiveness and safety between a modern cemented, and a modern uncemented hydroxyapatite-coated femoral stem in patients 65-79 years of age who were treated with THR for displaced FNF.

Clopidogrel Resistance after Minor Ischemic Stroke or Transient Ischemic Attack is Associated with Radiological Cerebral Small-Vessel Disease.

Background: The objective of this study was to compare nonresponders (NR) and responders (R) to clopidogrel with respect to presence of microvascular and macrovascular pathology in a cohort of patients with recent minor ischemic stroke (IS) or transient ischemic attack (TIA).

Methods: Seventy-two patients treated with clopidogrel after IS or TIA were evaluated 1 month after onset. Platelet aggregation was measured by multiple electrode aggregometry (Multiplate).

Intravenous pamidronate treatment improves growth in prepubertal osteogenesis imperfecta patients.

Background/aims: Pamidronate is widely used to treat pediatric patients with osteogenesis imperfecta (OI). We aimed at delineating the effects of monthly pamidronate therapy on the growth of different body segments in prepubertal OI patients.

Methods: The study included 14 prepubertal patients (12 boys, 2 girls) with mild forms of OI (type I and IV).

Fifteen years of treatment with intravenous immunoglobulin in central nervous system Langerhans cell histiocytosis.

Aim: There is currently no well-accepted therapy for central nervous system Langerhans cell histiocytosis (CNS-LCH), a neuroinflammatory disease clinically characterized by often progressive, neurological symptoms including ataxia, dysarthria, dysphagia, hypertonicity, intellectual impairment and behavioural abnormalities. We applied immunomodulative/anti-inflammatory treatment on a patient with progressive CNS-LCH disease.

Method: Intravenous immunoglobulin (IVIG) was administered monthly for 15 years to a patient with severe, image-verified neurodegenerative CNS-LCH.

Incidence and pattern of radiological central nervous system Langerhans cell histiocytosis in children: a population based study.

Background: Patients with Langerhans cell histiocytosis (LCH) may develop neurodegeneration and other central nervous system (CNS) dysfunctions revealed by brain magnetic resonance imaging (MRI). We estimated the incidence and pattern of pathological brain MRI findings in a well-defined, population-based cohort of children with LCH.

Methods: Among children under 15 years of age diagnosed with LCH in the Stockholm County during 1992-2001, brain MRI was performed at a single center in children with clinical and/or laboratory signs of CNS involvement, including endocrine dysfunction.

Biomarkers in the cerebrospinal fluid and neurodegeneration in Langerhans cell histiocytosis.

Background: Progressive neurodegeneration may result in potentially severe cognitive and motor dysfunctions as a complication of Langerhans cell histiocytosis (LCH), a suggested IL-17A-associated inflammatory condition. To detect this complication (CNS-LCH) early and to evaluate the potential efficacy of therapeutic interventions, biomarkers detecting and measuring ongoing neurodegeneration would be valuable. We evaluated cerebrospinal fluid (CSF) biomarkers of ongoing neurodegeneration in CNS-LCH patients.

Neuropsychological sequelae in patients with neurodegenerative Langerhans cell histiocytosis.

Background: Patients with Langerhans cell histiocytosis (LCH) may develop neurodegeneration and significant CNS sequelae, affecting a significant proportion of the patients. We here aimed to investigate the neuropsychological consequences in more detail.

Methods: Using an extensive neuropsychological test battery, we evaluated nine LCH patients, 6-20 years old, with radiological signs indicative of neurodegeneration.

Central nervous system involvement in severe congenital neutropenia: neurological and neuropsychological abnormalities associated with specific HAX1 mutations.

Objectives: Homozygous mutations in the HAX1 gene were recently identified in severe congenital neutropenia patients belonging to the original Kostmann family in northern Sweden. Our observations suggested that these patients also develop neurological and neuropsychological symptoms.

Methods: Detailed clinical studies and mutation analyses were performed in the surviving patients belonging to the Kostmann kindred and in two patients not related to this family, along with studies of HAX1 splice variant expression in normal human tissues.

Incidence of Langerhans cell histiocytosis in children: a population-based study.

Background: Langerhans cell histiocytosis is a rare disease of unknown etiology. We wanted to assess the population-based incidence of LCH in a well-defined cohort of children.

Methods: We identified all children <15-years old treated with LCH during the 10 years period 1992-2001 at the Department of Pediatrics, Karolinska University Hospital in Stockholm, the referral center for children with LCH in Stockholm County.

Intrathecal aspergillosis and fusiform arterial aneurysms in an immunocompromised child: a clinico-pathological case report.

Case Report: We describe a 12-year-old boy with subarachnoid hemorrhage. Cerebral angiography revealed multiple fusiform intracranial arterial aneurysms. Surgical therapy other than ventricular drainage was not feasible due to the extent and pattern of pathological changes.

Metacarpophalangeal pattern profile analysis: useful diagnostic tool for differentiating between dyschondrosteosis, Turner syndrome, and hypochondroplasia.

Purpose: To assess the value of the metacarpophalangeal pattern profile (MCPP) analysis as a diagnostic tool for differentiating between patients with dyschondrosteosis, Turner syndrome, and hypochondroplasia.

Material And Methods: Radiographic and clinical data from 135 patients between 1 and 51 years of age were collected and analyzed. The study included 25 patients with hypochondroplasia (HCP), 39 with dyschondrosteosis (LWD), and 71 with Turner syndrome (TS).

Metacarpophalangeal pattern profile analysis as a tool for early diagnosis of Turner syndrome.

Purpose: To analyze the metacarpophalangeal pattern profile (MCPP) in a cohort of individuals with Turner syndrome (TS), and to assess its value as a tool for early diagnosis of TS.

Material And Methods: Medical records and radiological material were collected of 71 patients with TS aged between 3 and 21 years. Forty-six patients received growth hormone therapy (33-66 microg kg(-1) day(-1)) and 14 of these were also treated with the anabolic steroid oxandrolone (1.

Metacarpophalangeal pattern profile analysis in Leri-Weill dyschondrosteosis.

Purpose: To analyze the metacarpophalangeal profile (MCPP) in individuals with Leri-Weill dyschondrosteosis (LWD) and to assess its value as a possible contributor to early diagnosis.

Material And Methods: Hand profiles of 39 individuals with a diagnosis of LWD were calculated and analyzed. Discriminant analysis was applied to differentiate between LWD and normal individuals.

Asn540Lys mutation in fibroblast growth factor receptor 3 and phenotype in hypochondroplasia.

Unlabelled: Hypochondroplasia is characterized by a disproportionate short stature with rhizomelic shortening of the limbs. Amino acid substitutions Asn540Lys, Asn540Thr and Ile538Val in the fibroblast growth factor receptor 3 (FGFR3) are considered to cause hypochondroplasia. In this study we examined the FGFR3 gene for the previously described hypochondroplasia mutations and the phenotype of 23 probands with clinically and radiologically diagnosed hypochondroplasia.

Swedish metacarpophalangeal standards compared with previously published norms.

Purpose: Metacarpophalangeal lengths are used to create pattern profiles that are useful for assessment of skeletal dysplasias and malformation syndromes. The purpose of this study was to establish Swedish standards and compare them with previously published norms.

Material And Methods: A group of healthy Swedish children was followed longitudinally from the age of 1 month to 18 years.