Long-term outcomes of adult chronic idiopathic hydrocephalus treated with a ventriculo-peritoneal shunt. / Evolución a largo plazo de la hidrocefalia crónica del adulto idiopática tratada con válvula de derivación ventrículo-peritoneal.

INTRODUCTION: Adult chronic idiopathic hydrocephalus (ACIH) is a cause of dementia that can be treated by implanting a ventriculo-peritoneal shunt (VPS). We aim to study clinical and functional outcomes in patients with ACIH corrected with a VPS. SUBJECTS AND METHODS: Observational cohort study of patients diagnosed with probable ACIH (Japan Neurosurgical Society guidelines) and undergoing shunt placement between 2008 and 2013 in a centre of reference for neurosurgery in Spain. Clinical improvement was classified in 4 categories (resolution, partial improvement, equivocal improvement, and no improvement); functional outcome was assessed on the modified Rankin scale (mRS). RESULTS: The study included 29 patients with a mean age of 73.9 years; 62.1% were male and 65.5% had hypertension. Clinical improvement (complete or partial) was observed in 58% after one year and in 48% by the end of the follow-up period (mean follow-up time was 37.8 months). Older age, presence of hypertension, and surgery-related complications were more prevalent in the group responding poorly to treatment. One patient died, 20.7% experienced severe complications, and 69% were dependent (mRS ≥ 3) by the end of the follow-up period. Age at diagnosis was independently associated with poorer clinical response at one year and a higher degree of dependency by the end of follow-up. CONCLUSION: Symptomatic benefits offered by VPS were partial and transient; treatment was associated with a high complication rate and poor functional outcomes in the long term, especially in the oldest patients.

Clinical practice guidelines for the diagnosis and management of Charcot-Marie-Tooth disease.

INTRODUCTION: Charcot-Marie-Tooth disease (CMT) is classified according to neurophysiological and histological findings, the inheritance pattern, and the underlying genetic defect. The objective of these guidelines is to offer recommendations for the diagnosis, prognosis, follow-up, and treatment of this disease in Spain. MATERIAL AND METHODS: These consensus guidelines were developed through collaboration by a multidisciplinary panel encompassing a broad group of experts on the subject, including neurologists, paediatric neurologists, geneticists, physiatrists, and orthopaedic surgeons. RECOMMENDATIONS: The diagnosis of CMT is clinical, with patients usually presenting a common or classical phenotype. Clinical assessment should be followed by an appropriate neurophysiological study; specific recommendations are established for the parameters that should be included. Genetic diagnosis should be approached sequentially; once PMP22 duplication has been ruled out, if appropriate, a next-generation sequencing study should be considered, taking into account the limitations of the available techniques. To date, no pharmacological disease-modifying treatment is available, but symptomatic management, guided by a multidiciplinary team, is important, as is proper rehabilitation and orthopaedic management. The latter should be initiated early to identify and improve the patient's functional deficits, and should include individualised exercise guidelines, orthotic adaptation, and assessment of conservative surgeries such as tendon transfer. The follow-up of patients with CMT is exclusively clinical, and ancillary testing is not necessary in routine clinical practice.

Evolución a largo plazo de la hidrocefalia crónica del adulto idiopática tratada con válvula de derivación ventrículo-peritoneal / Long-term outcomes of adult chronic idiopathic hydrocephalus treated with a ventriculo-peritoneal shunt

Introducción. La hidrocefalia crónica del adulto idiopática (HCAI) es considerada una causa de demencia tratable mediante la implantación de una válvula de derivación ventrículo-peritoneal (VDVP). Nos planteamos estudiar la evolución clínica y funcional de la HCAI tratada con VDVP, así como los factores asociados con una mejor evolución a largo plazo. Sujetos y métodos. Estudio observacional de pacientes con diagnóstico de HCAI probable (según criterios de la Sociedad Japonesa de Neurocirugía) y tratados con VDVP entre 2008 y 2013 en un hospital de tercer nivel español. Se establecieron 4 grupos de respuesta clínica (normalización, mejoría parcial, mejoría dudosa y empeoramiento) y la situación funcional se evaluó mediante la escala de Rankin modificada (ERm). Resultados. Se incluyó a 29 pacientes con una edad media de 73,9 años. El 62,1% eran hombres y el 65,5% presentaban HTA. Se observó una respuesta clínica al menos parcial en el 58 y el 48% al año y al final del seguimiento (seguimiento medio de 37,8 meses), respectivamente. La edad, la frecuencia de HTA y las complicaciones quirúrgicas fueron superiores en el grupo con mala respuesta. Un paciente falleció, el 20,7% presentó complicaciones graves y el 69% era dependiente (ERm ≥ 3) al final del seguimiento. La edad se asoció de manera independiente a peor respuesta clínica al año y una mayor dependencia al final del seguimiento. Conclusión. El beneficio de la VDVP fue parcial y transitorio, con una alta frecuencia de complicaciones y dependencia funcional en el seguimiento a largo plazo, especialmente en los pacientes de mayor edad (AU)

Introduction. Adult chronic idiopathic hydrocephalus (ACIH) is a cause of dementia that can be treated by implanting a ventriculo-peritoneal shunt (VPS). We aim to study clinical and functional outcomes in patients with ACIH corrected with a VPS. Subjects and methods. Observational cohort study of patients diagnosed with probable ACIH (Japan Neurosurgical Society guidelines) and undergoing shunt placement between 2008 and 2013 in a centre of reference for neurosurgery in Spain. Clinical improvement was classified in 4 categories (resolution, partial improvement, equivocal improvement, and no improvement); functional outcome was assessed on the modified Rankin scale (mRS). Results. The study included 29 patients with a mean age of 73.9 years; 62.1% were male and 65.5% had hypertension. Clinical improvement (complete or partial) was observed in 58% after one year and in 48% by the end of the follow-up period (mean follow-up time was 37.8 months). Older age, presence of hypertension, and surgery-related complications were more prevalent in the group responding poorly to treatment. One patient died, 20.7% experienced severe complications, and 69% were dependent (mRS ≥ 3) by the end of the follow-up period. Age at diagnosis was independently associated with poorer clinical response at one year and a higher degree of dependency by the end of follow-up. Conclusion. Symptomatic benefits offered by VPS were partial and transient; treatment was associated with a high complication rate and poor functional outcomes in the long term, especially in the oldest patients (AU)

Long-term effect of glucagon administration on rat liver L-type pyruvate kinase.

After 5 h of treatment with glucagon, liver L-type pyruvate kinase (ATP: pyruvate 2-0-phosphotransferase; EC 2.7.1.40) showed a significant decrease of K0.5 and the Hill coefficient (nH) in the absence of fructose 1,6-diphosphate. However, in the presence of fructose 1,6-diphosphate, liver enzymes from treated rats showed a slight decrease of K0.5 but nH remained unchanged. In both circumstances, no changes of Vmax were observed after treatment. These changes in the kinetic properties of liver L-type pyruvate kinase are consistent with the dephosphorylation of the enzyme caused by insulin release in response to treatment with glucagon.