Wileyklit8457

Specific force, that is the amount of force generated per unit of muscle tissue, is reduced in patients with facioscapulohumeral muscular dystrophy (FSHD). The causes of reduced specific force and its relation with FSHD disease severity are unknown.

Quantitative muscle magnetic resonance imaging (MRI), measurement of voluntary maximum force generation and quadriceps force-frequency relationship, and vastus lateralis muscle biopsies were performed in 12 genetically confirmed patients with FSHD and 12 controls.

Specific force was reduced by ~33% in all FSHD patients independent of disease severity. Quadriceps force-frequency relationship shifted to the right in severe FSHD compared to controls. Fiber type distribution in vastus lateralis muscle biopsies did not differ between groups.

Reduced quadriceps specific force is present in all FSHD patients regardless of disease severity or fatty infiltration. Early myopathic changes, including fibrosis, and non-muscle factors, such as physical fatigue and musculoskeletal pain, may contribute to reduced specific force.

Reduced quadriceps specific force is present in all FSHD patients regardless of disease severity or fatty infiltration. Early myopathic changes, including fibrosis, and non-muscle factors, such as physical fatigue and musculoskeletal pain, may contribute to reduced specific force.

Myelin oligodendrocyte glycoprotein (MOG) antibody disease is a rare and practically new entity in medicine. In developing countries, there are still important limitations for the detection of anti-MOG antibodies by cell-based assay, so knowing the clinical characteristics of the different phenotypes and their differences with other demyelinating pathologies of the central nervous system is essential in order to make a proper diagnostic and therapeutic approach of the patients.

To present an update regarding the clinical characteristics of the disease spectrum, being the first article in Spanish that gathers the most frequent phenotypes and provides a clear description of what should be considered to identify each of these phenotypes.

This disease is characterized by having a monophasic or recurrent course, with optic neuritis being the most frequent presentation phenotype in general population and disseminated acute encephalomyelitis the most frequent in children. Other phenotypes described in this review are transverse myelitis, focal cortical encephalitis and cerebral stem syndromes, as well as the proposed criteria for the diagnosis of the disease associated with MOG antibody disease.

Currently there are no studies that seek to characterize the Spanish-speaking population with this disease, or review articles in Spanish, so it is important to disseminate knowledge and develop research in this area.

Currently there are no studies that seek to characterize the Spanish-speaking population with this disease, or review articles in Spanish, so it is important to disseminate knowledge and develop research in this area.

Superficial siderosis of the central nervous system is an infrequent pathology secondary to chronic bleeding into the cerebrospinal fluid. Spinal tumors are infrequent cause of superficial siderosis being ependymoma the most common etiology.

We report the case of a woman with sensorineural hearing loss and cerebellar ataxia, diagnosed of superficial siderosis on brain MRI. selleck inhibitor She had no previous history of axial back pain or radicular leg pain or bowel or bladder incontinence. On spine MRI an intradural lesion was found at the S1 level. No signs of intratumoral hemorrhage were observed on MRI gradient-echo images. At surgery, an intradural soft mass with signs of chronic bleeding was completely resected. Based on microscopic examination and immunohistochemistry of the specimen, a diagnosis of paraganglioma World Health Organization grade I was made.

Since the only proven treatment able to prevent further deterioration from superficial siderosis is to stop chronic bleeding into subarachnoid space, is of parsidered for resection.

The Gross Motor Function Classification System has allowed us to stratificate cerebral palsy patients, according to their walking abilities. The lack of sensitivity about detecting changes and the absence of a global patient evaluation, justify the search of new pre-operative evaluation tools.

To present the Walking Abilities Levels Classification System (WALCS) and to show the first inter-observer agreement study that has been carried out. This system uses first a different pattern for ordering gait functional skills, and after that, evaluates the reversibility of the contextual factors that may limit the result of a gait disorder treatment.

A new evaluation frame was built by an interdisciplinary team with an average professional experience of more than 15 years, initially focused as part of the pre-surgical patient evaluation. An inter-observer agreement study was held to gain the first insight of it. 14 participants studied the medical reports and gait lab video images of 10 cases.

The kappa index was 0.76 for the walking ability level, 0.79 for the biological type, 0.69 psychological type and 0.64 social type of limiting factors.

The WALCS offers a new evaluation frame gathering patient walking skills and limiting factors treatment. The initial inter-observer agreement rate endorsed more intra- and inter-studies in order to achieve a more robust validation.

The WALCS offers a new evaluation frame gathering patient walking skills and limiting factors treatment. The initial inter-observer agreement rate endorsed more intra- and inter-studies in order to achieve a more robust validation.

Neurocognitive impairment associated to human immunodeficiency virus (HIV) is a current problem despite the effectiveness of antiretroviral treatment. The screening tests which best suit to Spanish population are Brief Neuro-cognitive Scale (BNCS) and Neu Screening. We are unaware of our HIV population' neurocognitive impairment magnitude.

To verify if association between HIV and neurocognitive impairment does exist and to know its prevalence.

An observational, descriptive and transversal study comparing 24 HIV-outpatients and 21 non-HIV-healthy control matched by age, gender and educational level. Hospital Anxiety and Depression Scale, Neu Screening and BNCS were used as neurocognitive impairment screening. Positive screening was considered with one or more abnormal test.

33.3% of VIH+ and 33.3% of healthy controls had positive screening without significant difference between both populations. Positive screening was significantly associated with anxiety-punctuation and depression-punctuation. A lineal correlation between CD4-nadir-levels and Digit Symbol and between CD4-nadir-levels and verbal fluency results were found.