Gabriel Rodriguez

Endogenous progesterone is associated to amyotrophic lateral sclerosis prognostic factors

Gargiulo Monachelli G, Meyer M, Rodríguez GE, Garay LI, Sica REP, De Nicola AF, González Deniselle MC. Endogenous progesterone is associated to amyotrophic lateral sclerosis prognostic factors.
Acta Neurol Scand: 2011: 123: 60–67.
© 2010 John Wiley & Sons A/S.

Sporadic amyotrophic lateral sclerosis: new hypothesis regarding its etiology and pathogenesis suggests that astrocytes might be the primary target hosting a still unknown external agent

This article briefly describes the already known clinical features and pathogenic mechanisms underlying sporadic amyotrophic lateral sclerosis, namely excitoxicity, oxidative stress, protein damage, inflammation, genetic abnormalities and neuronal death. Thereafter, it puts forward the hypothesis that astrocytes may be the cells which serve as targets for the harmful action of a still unknown environmental agent, while neuronal death may be a secondary event following the initial insult to glial cells. The article also suggests that an emergent virus or a misfolded infectious protein might be potential candidates to accomplish this task.

Morphological abnormalities in mitochondria of the skin of patients with sporadic amyotrophic lateral sclerosis

OBJECTIVES Mitochondrial dysfunction has been reported in the central nervous system, hepatocytes and peripheral blood lymphocytes from patients with sporadic amyotrophic lateral sclerosis (SALS). However, the status of skin mitochondria has not been reported, in spite of the fact that SALS patients present skin abnormalities. The objective of the present study was to compare mitochondrial ultrastructural parameters in keratinocytes from patients with SALS and healthy controls. METHODS Our study was based on the analysis of 112 skin mitochondria from 5 SALS patients and 99 organelles from 4 control subjects by electron microscopy. RESULTS Computerized image analysis showed that mitochondrial major axis length, area and perimeter of the organelle were significantly smaller in SALS respect of healthy control subjects. Morphologically, SALS mitochondria presented cristolysis and breakage of the outer membrane. CONCLUSIONS Mitochondrial dysfunction in the skin may possibly reflect changes occurring in mitochondria of the central nervous system. The analysis of mitochondrial morphology in this tissue may be of value to follow disease progression and, eventually, the effectiveness of current therapies for SALS.

Epidemiology of amyotrophic lateral sclerosis patients in a centre in Buenos Aires

UNLABELLED Sporadic amyotrophic lateral sclerosis (sALS) is considered a multifactorial disease with genetic and environmental factors causing motor neuron degeneration. OBJECTIVE To describe the epidemiological and occupational characteristics of patients with sALS who attended the Ramos Mejía Hospital at Buenos Aires, Argentina. METHOD We analyzed the medical records of sALS patients diagnosed between 2001 and 2008. All occupations were coded according to the International Standard Classification of Occupation (ISCO). RESULTS 187 patients were assessed, 38.5% were women and 61.5% men. Mean age at diagnosis was 55 years. 16% of them came from rural areas; 68% of the studied population had no health insurance. 40% were employed in elementary occupations, 19 were technicians and 8 handicraftsmen. CONCLUSION The most represented profession was elementary occupation. A large proportion of patients came from rural areas, which might suggest an increased risk of environmental exposure to an unknown agent in those regions.

Progesterone and cortisol levels in sporadic amyotrophic lateral sclerosis (sALS): correlation with prognostic factors

Abstract Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder. Worse prognostic factors in ALS are: (a) advanced age, (b) bulbar onset, and (c) short time between onset and diagnosis. Progesterone (PROG) has been associated with neuroprotective and promyelinating activities in injury, ischemia and degeneration of the central and peripheral nervous system. Cortisol is connected to the response to stress situations and could contribute to neuronal damage. The goals of this study were: (i) to investigate whether PROG levels are modified by ALS prognostic factors and (ii) to determine whether cortisol follows the same pattern. We determined serum steroid levels in 27 patients with sporadic ALS (sALS) and 21 controls. Both steroid hormones showed significantly increased levels in ALS patients versus controls (mean±SEM: PROG ALS vs. control: 0.54±0.05 vs. 0.39±0.04 ng/mL, p<0.05; cortisol ALS vs. control: 17.02±1.60 vs. 11.83±1.38 μg/dL, p<0.05).1 A trend towards higher levels of PROG were demonstrated in spinal onset patients compared with bulbar onset (p=0.07), positive correlation with survival time (RRho=0.43, p=0.04) and a trend towards significance with time to diagnosis (RRho=0.36, p=0.06). These correlations have not been demonstrated for cortisol. Elevated serum steroid levels in sALS were probably due to hyperfunction of the hypothalamic-pituitary-adrenal axis. However, only PROG correlated with better prognostic factors. Future studies will determine if the different behavior of PROG and cortisol relate to any particular role they might play during the course of this motor neuron degenerative disease. 1Conversion factors from conventional units to SI units: Progesterone ng/mL to nmol/L=3.18 Cortisol μg/dL to nmol/L=27.59

Atención del stroke agudo en una unidad de stroke y una sala general

Resumen Introduccion el stroke es una de las principales causas de discapacidad y muerte. Las Unidades de Stroke (US) mejoraron el tratamiento de la enfermedad cerebrovascular. Objetivo comparar la atencion del stroke agudo en una sala de Neurologia general (SNe) y en una US. Pacientes y metodo retrospectivamente se reviso la atencion de pacientes con stroke agudo en la SNe en el periodo comprendido entre agosto de 1997 a agosto de 1998 (previo a la apertura de US) y la atencion en la US en el lapso de tiempo desde agosto de 2004 a agosto de 2005. Se evaluaron los tiempos en llegar al hospital, en pasar a la sala apropiada y el tiempo de internamiento en el hospital. Resultados hubo 164 pacientes; 69 (42,09%) en la SNe y 95 (57,93%) en US. El 86,32% de los casos en US fueron stroke isquemico, en SNe fueron el 72,46%. El tiempo en llegar al hospital fue similar para ambas salas. El lapso en ingresar en la sala especializada fue de 4 horas 19 minutos en US y de 3 dias 43 minutos en Neurologia (p= 0,003). El tiempo medio de estancia hospitalaria fue 7 dias 16 horas en US y 23 dias 13 horas en la sala de Neurologia general (p= 0,001). Conclusiones en la US con un equipo multidisciplinario hubo un menor tiempo de internamiento que en la SNe. La presencia de un medico neurologo entrenado en patologia cerebrovascular, junto con una US, acelero el ingreso en una sala especializada. No hubo diferencias en las complicaciones ni en la mortalidad.