Longer Survival Period Research Articles

Interactions Between Yeasts and Grapevines: Filamentous Growth, Endopolygalacturonase and Phytopathogenicity of Colonizing Yeasts

It has been clearly established that phytopathogenic fungi, bacteria, and viruses exert biotic stresses on plants. Much less is known, however, about the interactions between enological species of yeast and their host plants. In a previous study, we described how Saccharomyces cerevisiae, the most common enological yeast, can act as a grapevine (Vitis vinifera L.) pathogen, causing growth retardation or plant death. In the present in vitro study on 11 strains of yeast belonging to different genera, which often occur on the surfaces of vineyard grapes and V. vinifera, a link was found to exist between strain phytopathogenecity and pseudohyphal growth habits and/or endopolygalacturonase activity. The results obtained here are consistent with earlier findings showing that the phytopathogenicity of yeast strains depends on the filamentous growth process, and show that endopolygalacturonase alone is not responsible for the invasion of plants tissues. The mechanisms observed here may be of significant ecological importance and may help to explain the long periods of yeast survival found to occur in vineyards.

Characterization of cortical neuron outgrowth in two‐ and three‐dimensional culture systems

To improve the ability of regeneration by grafting living cells or by adding growth factor to a lesion site, it is important to find good biomaterials for neuron survival and regeneration. This study focused on two- and three-dimensional cultures in a matrix using biomaterials such as agarose, collagen, fibrin, and their mixtures, because these are considered to be suitable biomaterials for neuron outgrowth. Cortical neurons were dissected from E17 rat embryos and cultured in agarose gel, collagen gel, fibrin glue, and mixtures of collagen and fibrin. Results showed that neurons cultured in collagen gel and fibrin glue had longer periods of survival (more than 3 weeks) and better neurite extension than those observed in agarose gels. As to the survival rate according to the MTT and lactate dehydrogenase assays, fibrin glue was the most suitable biomaterial for neuron survival among the biomaterials examined. With two-dimensional fibrin plating, neuron cells exhibited cell aggregation and stress fibers, but the same results were not observed with collagen gel. There were no differences in neurite extension and survival in the mixtures of collagen and fibrin. The results suggest that collagen and fibrin can provide a suitable substrate for a three-dimensional culture matrix for neuronal survival and differentiation.

Adenoid Cystic Carcinoma of the Major Salivary Glands Treated with Surgery and Radiation

To examine patient characteristics, pathologic features, and treatment outcomes of adenoid cystic carcinoma of the major salivary glands. Retrospective review of patients in an academic medical center. Review of medical records regarding demographics, extent of tumor, stage, histologic characteristics, and treatment outcomes of patients treated with surgery and postoperative radiation. Of the 33 patients, 19 (58%) were male, and 14 (42%) were female. The average age of presentation was 49 (range 22-81) years. Of the 29 patients fully staged at the time of diagnosis, 7 (24%) presented at American Joint Committee on Cancer stage I, 9 (31%) at stage II, 4 (14%) at stage III, and 9 (31%) at stage IV. The cribriform histologic subtype was predominant (64%). The majority originated in the parotid gland (21, 64%), with the remaining originating in either the submandibular gland (10, 30%) or the sublingual gland (2, 6%). Local control was 94% at 5 years and 73% at 10 years. Metastatic control was 82% at 5 years and 63% at 10 years. Four patients failed locally and nine failed distally. Overall survival was 85% at 5 years and 69% at 10 years, with a median of 12.9 years. Surgical excision with postoperative radiation provides a long period of disease-free survival. Patients were more likely to fail with metastases than with local recurrence.

Colonic metastasis from squamous carcinoma of the lung: report of a case and review of the literature

We describe a rare case with metastatic involvement of the gastrointestinal tract (colonic metastasis) secondary to lung cancer. After diagnostic work-up, a left hemicolectomy was performed. Patient has been alive and healthy 9 months after operation. We report the first case with the longest survival period after the diagnosis of metastasis, although the symptomatic gastrointestinal metastases from lung cancer generally follow a poor prognosis because of their advanced state and multiplicity.

Long-term reinnervation effects after sciatic nerve lesions in adult rats

Transection of the sciatic nerve in adult rats induces drastic changes in hindleg muscles. Earlier, we demonstrated that the reinnervated soleus (SOL) muscle, 21 weeks after a transection mainly contains type II fibers. This is in striking contrast to normal muscle, which consists to 80% of type I muscle fibers. Also we observed 13.9% of the fibers to be polyneurally innervated. The problem of the present study is whether these changes are reversible after Longer survival periods. Therefore, the SOL was studied 60 weeks after transection and reconstruction by an autologous nerve graft. In six rats, we studied muscle fiber distributions by monoclonal antibodies, and innervation patterns by cholinesterase staining and AgNO3 impregnation. Still at 60 weeks, only 20% of the muscle fibers are of type I and this is similar to results at 21 weeks, indicating that no recovery to the normal has been reached by that age. Furthermore, 20% of the endplates in the reinnervated SOL were polyneurally innervated, but we also observed this in 10% of the endplates on the control side. These increases, compared to data at 21 weeks, are interpreted as an aging effect.

Pathophysiology of human paralytic rabies

Furious rabies is a well-recognized clinical disorder in humans but the paralytic form is not as easily identified. The mechanisms responsible for the weakness and longer survival periods are not clear. Several hypotheses have been proposed, including rabies virus variants associated with a particular vector, location of wounds, incubation period, influence of prior rabies vaccination, and virus localization in the central nervous system (CNS). However, none of these have been substantiated. Regarding molecular analyses of rabies viruses isolated from both furious and paralytic rabies patients, only minor genetic variations with no specific patterns in glyco- (G), phospho- (P), and nucleoprotein (N) sequences have been identified and arginine 333 in G protein was present in all samples. Regional distribution of rabies virus antigenin rabies patients whose survival periods were 7 days or less and magnetic resonance imaging (MRI) of the CNS indicated brainstem and spinal cord as predilection sites regardless of clinical presentations. There are clinical, electrophysiological, and pathological indications that peripheral nerve dysfunction is responsible for weakness in paralytic rabies whereas in furious rabies, even in the absence of clinical weakness, abundant denervation potentials with normal sensory nerve conduction studies and proximal motor latencies suggest anterior horn cell dysfunction. The lack of cellular immunity to rabies virus antigen accompanied by an absence of cerebrospinal fluid (CSF) rabies neutralizing antibody in most paralytic rabies patients may argue against role of an immune response against rabies virus-positive axons. Aberrant immune responses to peripheral nerve antigen, in particular those mediated by one or more cellular-dependent mechanisms, may be involved as is supported by the absence of putative anti-ganglioside antibodies commonly found in immune-mediated peripheral nerve diseases. Longer survival period in paralytic rabies may possibly be related to currently unidentified mechanism(s) on neuronal gene expression, required for virus transcription/replication and for maintaining neuronal survival.

Long-term changes of c-Fos expression in the rat spinal cord following chronic constriction injury

The expression of c-Fos protein has been used as a relative marker of nociceptive neuronal activity in the spinal cord following various noxious stimuli. Experiments were conducted to examine c-Fos expression in lumbar spinal cord (L3–L6) following chronic constriction injury (CCI) in relation to nociceptive behavior over longer survival period up to 28 days. Development of mechanical allodynia was observed in the ipsilateral hind paw of CCI rats at day 3 and lasted up to 28 days. In contrast, the spinal c-Fos expression in CCI rats appeared in a biphasic manner. The highest number of c-Fos positive neurons occurred during the first week, followed by a decline at 7 and 14 days and reappearance at day 28 following injury. The early increase of c-Fos expression correlated with allodynia development, however, at longer survival period (28 days) c-Fos positivity become comparable in both CCI and sham groups despite their obvious behavior differences. Our results suggest that, at least in the CCI model, the c-Fos protein expression should not be considered as a reliable index of pain sensation disorders.

Cervical Spine Metastasis of Multiple Myeloma: A Case Report With 16 Years of Follow-up

Presented is a case report of a 16-year clinical course of a patient with a multiple myeloma of the third cervical vertebra. To describe a rare 16-year follow-up of a cervical multiple myeloma lesion that forced several surgical interventions. Multiple myeloma is a systemic malignancy. It slowly causes bone destruction due to bone marrow infiltration. It is more common in the elderly and rarely affects the young. In patients with continuing neck pain and headache, one has to bear in mind the rare possibility of multiple myeloma or also other malignancies affecting the cervical spine. Clinical case analysis, radiographs, magnetic resonance images, and histological sections of the lesion are discussed. Repeated surgical interventions were required due to progressive disease. Initial surgical treatment was aimed at palliation. However, this was in contrast to the slowly progressive clinical course. Thus, further surgical interventions were necessary. The patient died 16 years after the first procedure due to progression of the disease. Even in young patients presenting with neck pain and minor neurological symptoms, one should bear in mind the rare possibility of multiple myeloma. At the very least, a radiograph of the cervical spine and a routine blood test should be performed. Surgical interventions must take into account the relatively long period of survival in multiple myeloma patients compared to patients with other secondary bone tumors.

Intratumoral induction of thymidylate synthase mRNA by 5-FU in colorectal cancer patients: association with survival

Dihydropyrimidine dehydrogenase (DPD) is the rate-limiting enzyme in 5-fluorouracil (5-FU) catabolism. Thymidylate synthase (TS) is inhibited to form an inactive ternary complex by 5-fluoro-dUMP and is considered to be a target enzyme of 5-FU treatment. Two enzymes, DPD and TS, have been reported to be major determinants of individual sensitivity to 5-FU, and it has been reported that TS mRNA levels are modified by 5-FU treatment. We investigated their impact on treatment efficacy in colorectal cancer patients. TS and DPD mRNA levels, which correlated to the corresponding enzyme activities, were quantified in tumor tissues before and after treatment in 40 advanced colorectal cancer patients who had been treated with Doxifluridine (5'-DFUR) for 14 days before surgery. Furthermore inter-individual variations of TS mRNA levels after 5-FU treatment were found, and the individual TS induction varied between patients (0.2-2.4). Increased TS mRNA levels were found in 19 out of 40 cases. The samples were divided into two groups according to their TS mRNA induction (< or >1; TS/beta-actin ratio after treatment divided by values prior treatment) and compared with tumor reduction and survival. TS and DPD mRNA levels in tumor biopsies before treatment were not related to 5-FU responses by histological evaluations in this study. However the efficacy of 5-FU treatment was enhanced in patients with no or low TS mRNA induction (odds ratio: 6.2, p<0.05). Furthermore, longer periods of survival were observed in the group without increased TS mRNA levels. These findings suggest that TS mRNA was induced by 5-FU treatment, and the overall induction level varied between individuals. Therefore, the estimation of TS mRNA induction may be useful to predict the efficacy of 5-FU treatment.

Invasion Pathways of Karnal Bunt of Wheat into the United States.

Karnal bunt of wheat (caused by Tilletia indica) was first detected in the United States in Arizona in 1996. The seed lots of infected, spring-habit, durum wheat associated with the initial detection were traced to planted fields in California, Arizona, New Mexico, and Texas. However, in the summer of 1997, the disease appeared in unrelated, winter-habit, bread wheat located over 700 km from the nearest potentially contaminated wheat from 1996 (and destroyed prior to reinfection). Here, we examined potential invasion pathways of the fungus associated with the movement of wheat into the United States. We analyzed the USDA/APHIS Port Information Network (PIN) database from 1984 through 2000 to determine likely pathways of introduction based on where, when, and how the disease was intercepted coming into the United States. All interceptions were made on wheat transported from Mexico, with the majority (98.8%) being intercepted at land border crossings. Karnal bunt was not intercepted from any other country over the 17-year period analyzed. Most interceptions were on wheat found in automobiles, trucks, and railway cars. The majority of interceptions were made at Laredo, Brownsville, Eagle Pass, and El Paso, TX, and Nogales, AZ. Karnal bunt was intercepted in all 17 years; however, interceptions peaked in 1986 and 1987. Averaged over all years, more interceptions (19.2%) were made in the month of May than in any other month. Our results indicate that Karnal bunt has probably arrived in the United States on many occasions, at least since 1984. Because of the relatively unaggressive nature of the disease and its reliance on rather exacting weather conditions for infection, we surmised that it is possible this disease has a long period of latent survival between initial arrival and becoming a thriving, established disease.

Third model of the undulation pump total artificial heart.

The undulation pump is a small, continuous flow displacement type blood pump, and the undulation pump total artificial heart (UPTAH) is a unique, implantable total artificial heart based on this pump. To improve the durability of the UPTAH for investigating long-term pathophysiology with UPTAH, a third model (UPTAH3) has been developed. UPTAH3 was designed to separate the left and right undulation shafts and to be more durable. The undulation pumps were also redesigned. UPTAH3 was implemented with a diameter of 76 mm, width of 78 or 79 mm, total volume of 292 ml, and weight of 620 g. The priming volumes of the left and right pumps are 26 and 21 ml, respectively. The atrial cuffs and outflow cannulae were also redesigned for UPTAH3. The maximum output against an arterial pressure load of 100 mm Hg is about 11 L/min. The maximum pump efficiency is about 15% in the left pump and 18% in the right pump, giving a maximum total efficiency for both of about 11%. To date, UPTAH3 has been tested in 17 goats, and the longest survival period was 46 days. This third model will be useful for investigating pathophysiology with UPTAH.

Galectin-1 modulates human glioblastoma cell migration into the brain through modifications to the actin cytoskeleton and levels of expression of small GTPases.

We show that high-grade astrocytic tumors with high levels of galectin-1 expression are associated with dismal prognoses. The immunohistochemical analysis of galectin-1 expression of human U87 and U373 glioblastoma xenografts from the brains of nude mice revealed a higher level of galectin-1 expression in invasive areas rather than non-invasive areas of the xenografts. Nude mice intracranially grafted with U87 or U373 cells constitutively expressing low levels of galectin-1 (by stable transfection of an expression vector containing the antisense mRNA of galectin-1) had longer survival periods than those grafted with U87 or U373 cells expressing normal levels of galectin-1. Galectin-1 added to the culture media markedly and specifically increased cell motility levels in human neoplastic astrocytes. These effects are related to marked modifications in the organization of the actin cytoskeleton and the increase in small GTPase RhoA expression. All the data obtained indicate that galectin-1 enhances the migratory capabilities of tumor astrocytes and, therefore, their biological aggressiveness.

Voltammetric studies of basal and electrically evoked cathecolamine release from long‐term cultures of chromaffin cells

Previous in vitro studies have revealed basal and evoked release from cultures of chromaffin cells (CC). However no basal and evoked release of catecholamines from long‐term permanent cultures of CC have been studied. The objectives of the present study were to compare basal and evoked release of catecholamines from these type of cultures and to achieve a long period of survival of permanent microcultures. CC were obtained from adult bovine adrenal glands and plated (50 k cells, n = 3; 100 k cells, n = 9) in microculture dishes with a platinum sputter‐coated base. Basal and electrical evoked release (50 Hz, 100–250 mA/15 s) from CC microcultures were recorded using fast cyclic voltammetry with carbon‐fiber microelectrodes at different intervals of time (20, 40, 60, 90 days). Cell counts at each time period were assessed using an inverted microscope. After at least 120 days, CC were fixed, marked for TH, PNMT, and the nuclei stained. CC count decreased slightly over the entire period of culture. The basal release of electroactive compounds from the long‐term microcultures was always present although a decrease was associated with the decrease in CC count. The electrical stimulation of cells did show an increased release (best between 200 and 250 mA stimuli) in the nanomolar range compared to that of the basal release. Confocal microscopy of labeled cultures revealed that most of the CC present were adrenergic and noradrenergic. This study shows that permanent populations of CC microcultures can be maintained long‐term and the catecholamine release enhanced with electrical stimulation.

Phase II Study of Repeated Intrapleural Chemotherapy Using Implantable Access System for Management of Malignant Pleural Effusion

To examine the effectiveness of repeated intrapleural chemotherapy using an implantable access system (INFUSE-A-PORT; Horizon Medical Products; Manchester, GA) for the management of a malignant pleural effusion (MPE). Twenty-two patients with histologically proven MPEs (11 men and 11 women; mean age, 63.8 years; age range, 51 to 81 years; performance status, less-than-or-equal 3) were enrolled in this study. There were 17 patients with MPEs resulting from primary lung cancer; of whom 7 had metastatic disease (stage IV), 3 had stage IIIB disease, and 7 had postoperative recurrences. Three patients had MPEs that were caused by mesothelioma, and two had MPEs caused by breast cancer. Intrapleural catheters were placed by a video-assisted thoracoscopic procedure. The intrapleural administration of 5-fluorouracil (5-FU; 250 mg per body) and cisplatin (10 mg per body) using the implantable access system was performed biweekly at the outpatient clinic. Drainage of the MPEs through the port was performed only when the patients had some manifestations that occurred due to increasing effusion. The mean total administered doses of 5-FU and cisplatin were 3,290 and 124 mg, respectively. The mean follow-up period was 431 days (range, 209 to 792 days). The median survival period was 403 days, and the longest survival period was 792 days. No treatment-related mortality, renal dysfunction, bone marrow suppression, or infection occurred. One patient experienced a hemothorax after eight instances of intrapleural administration. The port and the catheter were involved with the tumor in one patient. Repeated intrapleural chemotherapy using the implantable access system is useful and safe for patients with MPEs. In the future, prospective randomized studies will be necessary to compare the efficacy of therapy for MPE using the implantable access system with that of pleurodesis.

Surgical resection combined with chemotherapy for advanced hepatocellular carcinoma with tumor thrombus: Report of 19 cases

Background. Prognosis of hepatocellular carcinoma (HCC) with tumor thrombus in the main portal vein (MPV), inferior vena cava (IVC), or extrahepatic bile duct (EBD) treated by conventional therapies has been considered poor. This study aimed to evaluate the efficacy of hepatic arterial infusion chemotherapy after surgical resection as an adjuvant therapy or as a treatment for intrahepatic recurrence of HCC with tumor thrombus in MPV, IVC, or EBD. Methods. Nineteen patients with HCC and tumor thrombus in the MPV, IVC, or EBD who underwent hepatectomy with thrombectomy were reviewed retrospectively. Results. The overall 3-year survival rate was 48.5%. Two patients with postoperative residual tumor thrombus died within 6 months owing to rapid progression of the residual tumor thrombus. Five patients survived more than 5 years after their operations. Tumors disappeared completely in 3 patients after hepatic arterial infusion chemotherapy with a combination of cisplatinum and 5-fluorouracil, and the longest survival period was 17 years and 11 months in a patient with EBD thrombus. Conclusions. If hepatic reserve is satisfactory, an aggressive surgical approach combined with chemotherapy seems to be of benefit for patients having HCC with tumor thrombus in the MPV, IVC, or EBD. (Surgery 2002;131:300-10.)

Alterations in ApoE and ApoJ in Relation to Degeneration and Regeneration in a Mouse Model of Entorhinal Cortex Lesion

Apolipoproteins are primarily involved in the transport of lipid and cholesterol within the central nervous system (CNS) and are thought to play a role in synaptic remodeling, repair, and regeneration after brain injury. In the present study, alterations in apolipoproteins E (apoE) and J (apoJ) were examined in the molecular layers of the dentate gyrus after unilateral chemical lesioning of the entorhinal cortex (ECL), at days 0, 1, 3, 7, 28, and 90 days following injury. Alterations in immunostaining for these proteins were assessed in relation to accumulation of silver-labeled degeneration products and alterations in synaptophysin and GAP-43 immunoreactivity. Quantitative analysis of synaptophysin and GAP-43 immunostaining highlighted synaptic loss and fiber degeneration initially (3–7 days post-ECL), with subsequent terminal sprouting and reactive synaptogenesis occurring at longer survival periods (28–90 days post-ECL). Increased apoE and apoJ immunoreactivity was evident first within the neuropil (*P < 0.05 and **P < 0.01) followed by intense glial staining by day 7 post-ECL. By day 28 apoE and apoJ immunostaining had returned almost to baseline levels. However, at day 90 post-ECL, neuropil apoE and apoJ immunoreactivity was dramatically increased compared to contralateral levels (**P < 0.01 and ***P < 0.0001, respectively). Silver-labeled degeneration products were found to be in abundance at day 3 postlesion; however, by day 7 this was reduced leaving only a thin band of material within the MML and at day 90 post-ECL, dentate silver staining was similar to that of controls. The results indicate that apoE and apoJ are upregulated after injury and parallel clearance of cholesterol and lipid debris from the site of injury. This coordinated alteration in apolipoproteins may redistribute lipid material to sprouting fibers to promote neurite extension and may play an important role in long-term plasticity changes following injury.

Second Surgery for Recurrent Pilocytic Astrocytoma in Children

Pilocytic astrocytoma (PA) is the most common childhood brain tumor. In cases where the tumor progresses or recurs following primary surgical resection, the appropriate treatment is unclear. Options include chemotherapy, radiation therapy, surgical resection or a combination thereof. To analyze the utility of further surgery, we performed a retrospective, single-institution review of pediatric patients with recurrent PAs from 1990 to 1999 who were treated with a second surgical resection. Patients were excluded if they received adjuvant chemotherapy or radiation therapy. Twenty cases were identified. Tumor locations included: cerebral hemisphere (3), cerebellum (7), optic pathway/hypothalamus (5), thalamus (1) and brainstem (4). The indication for 4 surgeries included an enlarging tumor-associated cyst. At second surgery, 10 of 20 patients had a gross total resection (GTR), 2 a near total resection (NTR), and the remaining 8 patients had a subtotal resection (STR). No patients have died. Two of 10 tumors after GTR, 0 of 2 tumors after NTR, and 7 of 8 tumors after STR had second recurrence/progression at a mean of 15 months (range 4–33 months) following second surgery. The remaining 11 patients are recurrence/progression-free at a mean of 40.7 months (range 19–119 months). Surgery for tumors or midline structures rarely resulted in a GTR (1 of 10 cases). Surgery for tumors located in the cerebral hemispheres or cerebellum resulted in GTR or NTR in all cases and can result in long periods of progression-free survival without further adjuvant treatment.

Experimental study of an artificial esophagus using a collagen sponge, a latissimus dorsi muscle flap, and split-thickness skin.

The time and effort spent trying to devise an artificial esophagus have not yet resulted in success, and leakage and strictures at the anastomotic sites remain the most frequent complications. We developed an artificial esophagus with a bilayered structure made of porous collagen sponge (artificial dermis; AD), a latissimus dorsi muscle flap (LD), and split-thickness skin (STS). We investigated whether the use of AD prevented the contraction of grafted skin and its effects on the extensibility of the neoesophagus in rabbits. We experimented with two groups. In the AD group, AD was applied to the surface of the LD. Three weeks later, the STS was grafted. In the control group, the STS was grafted directly onto the LD. The sizes of the STS in both groups 3 weeks after the graft were, respectively, 56.6% +/- 4.1% and 39.0% +/- 10.2% of the initial surface area of the STS (P < 0.01). The roll made in the AD group had better extensibility than that in the control group. We replaced the cervical esophagus in 12 rabbits with the neoesophagus made from AD, STS, and LD. The longest survival period was 16 days. Esophagography did not reveal either anastomotic leakage or stenosis in any of the five rabbits in the experiments. These findings suggested that AD can thus be used to create a more suitable hybrid artificial esophagus.

The effectiveness of preoperative intraluminal brachytherapy in preventing wall penetration and nodal involvement of rectal carcinomas

A retrospective review was conducted of 115 patients with middle or lower rectal carcinoma, without distant metastasis, who were consecutively treated with preoperative intraluminal brachytherapy and radical surgery (IBT group). Another 115 rectal carcinoma patients treated with surgery alone were reviewed as a control (NIBT group). We investigated whether conventional pathologic stagings were correlated with prognosis. All tumors were diagnosed by the TNM classification. According to the distribution of pathologic staging in the resected specimens, the prevalence of totally ablated tumors (stage T0) in the IBT group was significantly higher than that in the NIBT group, but there were no significant differences in the distribution of other T stages between the two groups. Good local control was achieved for the stage-T3 tumors in the IBT group, similarly to the T < or = 2 tumors in both groups. In the IBT group, the survival period of the patients with stage-T4 tumors even after radiotherapy was significantly shorter than that of those with T3 tumors (P = 0.042), while those with N < or = 2-stage tumors had significantly longer survival periods than those with N3-stage tumors (P = 0.047). These findings demonstrate the usefulness of analyzing clinicopathologic parameters to predict local control and the prolongation of survival. Determining these factors will also help to identify high-risk patients likely to require more effective adjuvant chemotherapy.

Soporte ventilatorio en pacientes con esclerosis lateral amiotrófica

In the final stage of amyotrophic lateral sclerosis, the majority of patients develop chronic respiratory failure. If these patients are not informed about the acute respiratory insufficiency, they run the risk of having to be intubated and stay in Intensive Care Unit over a long term; many of them die of respiratory failure without being informed about the available options. Prognosis and treatment options should be discussed with the patient and family. Their informed consent to prolonged mechanical ventilation of the lungs and stopped it under certain circumstances would be obtained before the condition progress to critical. The non invasive artificial home ventilation has led to advances in the management of patients with amyotrophic lateral sclerosis, as a longer period of survival with a higher level of quality of life. We also have analyzed ethical and social aspects and the results of long term mechanical ventilation in the literature.