Present and future of subthalamotomy in the management of Parkinson´s disease: a systematic review.

Introduction: The subthalamic nucleus (STN) is known to be involved in the pathophysiology of Parkinson´s disease and by reducing its abnormal activity, normal output of basal ganglia can be restored along with improvement in PD cardinal motor features. Deep brain stimulation of the STN is currently the main surgical procedure for PD with motor complications, but lesioning can be an alternative.Areas covered: Here, the authors systematically review the current evidence regarding subthalamotomy both with radiofrequency and, more recently, with focused ultrasound (FUS) for the treatment of PD.Expert opinion: Unilateral subthalamotomy for the treatment of PD motor features can be considered a viable option in asymmetric patients, particularly with FUS which allows a minimally invasive safe and effective ablation of the STN. Risk of inducing dyskinesia (i.e., hemichorea/ballism) may be strikingly reduced when lesions enlarge dorsally to impinge on pallidothalamic fibers.

[Clinical experience in the treatment of motor fluctuations in Parkinson's disease. Delphi consensus of a group of experts in movement disorders]. / Experiencia clínica en el tratamiento de las fluctuaciones motoras en la enfermedad de Parkinson. Consenso Delphi de un grupo de expertos en trastornos del movimiento.

INTRODUCTION: Motor fluctuations are one of the most common complications of Parkinson's disease and their treatment is still a complex matter. Therefore, from the Neurology Movement Disorders Group we present our clinical experience in the treatment of these complications, with the intention of it being useful in decision-making in daily clinical practice. DEVELOPMENT: Nineteen questions were developed based on a literature review and an open survey answered by members of this group. These issues were discussed in two phases, using the Delphi methodology. Considering the results of the survey, levodopa dose adjustment and dopamine agonists are the option with the best efficacy/tolerability ratio in the treatment of motor fluctuations. Rotigotine is useful in the motor fluctuations associated with gastroparesis, and intermittent subcutaneous apomorphine has positive effects in patients with unpredictable off periods. The most relevant adverse effect associated with dopamine agonists is impulse control disorder. Catechol-O-methyltransferase inhibitors are useful in the initial stages of motor fluctuations, especially in wearing off. Monoamine oxidase inhibitors are generally drugs that are well-tolerated and useful in motor fluctuations. If these measures are not effective, second-line treatments should be indicated on a case-by-case basis. CONCLUSION: The clinical profile of patients with Parkinson's disease is paramount in deciding the most appropriate therapy for the treatment of motor fluctuations.

TITLE: Experiencia clínica en el tratamiento de las fluctuaciones motoras en la enfermedad de Parkinson. Consenso Delphi de un grupo de expertos en trastornos del movimiento.Introducción. Las fluctuaciones motoras son una de las complicaciones más frecuentes en la enfermedad de Parkinson y su tratamiento sigue siendo complejo. Por ello, desde el Grupo de Trastornos del Movimiento de la Asociación Madrileña de Neurología presentamos nuestra experiencia clínica en el tratamiento de estas complicaciones, con la intención de que sea de utilidad en la toma de decisiones en la práctica clínica diaria. Desarrollo. Se elaboraron 19 preguntas a partir de una revisión bibliográfica y una encuesta abierta respondida por los miembros de dicho grupo. Dichas cuestiones se debatieron en dos fases, utilizando la metodología Delphi. Considerando los resultados de la encuesta, el ajuste de la dosis de levodopa y los agonistas dopaminérgicos son la opción con mejor relación eficacia/tolerabilidad en el tratamiento de las fluctuaciones motoras. La rotigotina es útil en las fluctuaciones motoras asociadas a gastroparesia, y la apomorfina subcutánea intermitente, en pacientes con off impredecible. El efecto adverso más relevante asociado a los agonistas dopaminérgicos es el trastorno del control de impulsos. Los inhibidores de la catecol-O-metiltransferasa son útiles en las fluctuaciones motoras de inicio, especialmente en el wearing off. Los inhibidores de la monoaminooxidasa son fármacos, en general, bien tolerados y útiles en las fluctuaciones motoras. En caso de que estas medidas no resulten eficaces, se deben indicar terapias de segunda línea de manera individualizada. Conclusión. El perfil clínico del paciente con enfermedad de Parkinson es primordial para decidir la terapia más adecuada en el tratamiento de las fluctuaciones motoras.

[A protocol for the treatment of retinopathy of prematurity in Spain]. / Protocolo de tratamiento de la retinopatía del prematuro en España.

OBJECTIVE: To prepare a protocol for the treatment of retinopathy of prematurity (ROP) agreed by the majority of Spanish ophthalmologists dedicated to this topic. MATERIAL AND METHOD: A draft of the protocol was produced taking into account the experience of the participants and up to date publications. This draft was corrected by all the ophthalmologists participating in the project, and the final document was agreed by all of them. RESULTS: We present general guidelines as an aid for the treatment of ROP, including treatment criteria, treatment methods, a calendar of action, and follow-up. CONCLUSIONS: It is important to have a common working protocol for the treatment of ROP to improve care and to avoid mistakes. Although individual Hospitals may adapt the protocol to their daily activity, it is recommended that there is a minimal working protocol agreed by most of professionals dedicated to pediatric ophthalmology in Spain.

Screening program for retinopathy of prematurity in Spain.

OBJECTIVE: To prepare a retinopathy of prematurity (ROP) screening program as agreed by most of Spanish ophthalmologists dedicated to this topic. MATERIALS AND METHODS: A draft of the protocol was produced taking into account the experience of the participants and current publications. This draft was corrected by all the ophthalmologists participating in the project and the final document produced was agreed by all of them. RESULTS: We present general guidelines to help in the screening of ROP, including treatment criteria, treatment methods, and a calendar of action. CONCLUSIONS: It is important to have a common working protocol in the screening of ROP to improve the action and to avoid mistakes. Although individual Hospitals may adapt the protocol to their daily activity, it is recommended that there is a minimal working protocol agreed by most of professionals dedicated to pediatric ophthalmology in Spain.

[X linked retinoschisis, unusual presentation: strabismus]. / Retinosquisis ligada al cromosoma X, presentación inusual: estrabismo.

CASE REPORT: X linked retinoschisis is a recessively inherited degenerative retinopathy. We report two cases that debuted with an unusual presentation (strabismus) in early childhood (months). Both of them presented with vitreous veils in the retinal periphery. Mutation in the XLRS1 gene was detected in both cases. DISCUSSION: X linked retinoschisis is one of the leading causes of macular degeneration in male children. Clinical features include a stellate foveal schisis, with or without peripheral retinoschisis. Clinical diagnosis is often difficult because of a high degree of phenotype variability. Furthermore, ERG and OCT may be normal in early stages of the disease. In our opinion, the XLRS1 gene mutation screening provides a powerful clinical tool for evaluating clinically ambiguous cases of X linked retinoschisis.

Use of fully covered self-expanding metal stents for the management of benign biliary conditions.

BACKGROUND AND AIM: biliary self-expanding metal stents (SEMS) have the advantage of being inserted undeployed with very small sizes and provide, when fully opened, large diameters for biliary drainage. However, their use in benign conditions has been very limited, mainly because of difficulty in their extraction. We present our initial experience with a fully covered SEMS (Wallflex) for the management of benign problems of the bile duct. PATIENTS AND METHODS: in a prospective study, stents of 8 mm in diameter and 4, 6 or 8 cm long were inserted by means of ERCP. These SEMS were chosen when according to medical judgement it was thought that diameters greater than 10 French (3.3 mm) were needed for proper biliary drainage. Stents were extracted also endoscopically, several months later when deemed clinically appropriate. RESULTS: twenty biliary SEMS were inserted. Reasons for insertion were: large intrahepatic biliary fistula after hydatid cyst surgery (1), perforation of the papillary area following endoscopic sphincterotomy (2), coaxial insertion to achieve patency in obstructed uncovered stents inserted in benign conditions (3), benign strictures (7), multiple and large common bile duct stones that could not be extracted because of tapering and stricturing of the distal common bile duct (7). In all cases, successful biliary drainage was achieved and there were no complications from insertion. Stents were easily extracted after a mean time of 132 days (36-270) in place. Complete resolution of biliary problems was obtained in 14 patients (70%). CONCLUSIONS: in our initial experience, the fully covered Wallflex biliary stent was removed without any complication after being in place in the common bile duct for a mean time of over four months. Therefore, it could be used in the management of benign biliary conditions.

[Palliative management of malignant gastric outlet obstruction with endoscopically inserted self-expanding metal stents].

AIM AND BACKGROUND: the insertion of self-expanding metal stents to palliate malignant gastric outlet obstruction is a minimally invasive procedure that is being increasingly used. We discuss experience with this technique in a level-II hospital in the Spanish National Health System. PATIENTS AND METHODS: a retrospective five-year study (2003-2007) was conducted in 23 patients who underwent 27 procedures aimed at resolving malignant gastric outlet obstruction (mean, 0.45 procedures per month) using endoscopically inserted noncovered stents (Wallstent and Wallflex). RESULTS: insertion was technically feasible in all 27 (100%) attempts, with satisfactory clinical results in 25 cases (92.5%). Endoscopy alone was used 10 times (37%), and both endoscopy and fluoroscopy on 17 (63%) occasions. After stent insertion, one patient was intervened for treatment, and a patient with an unsuccessful prosthesis received a palliative surgical bypass. Four stents became obstructed by tumoral ingrowth, and patency was reestablished by inserting a new stent. Obstructive jaundice caused by stents covering the papilla of Vater occurred in three cases. There were no other complications or mortality due to the procedure. Mean survival was 104 days (range 28-400, SD +/- 94). CONCLUSIONS: in our experience endoscopic insertion of self-expanding metal stents appears to be a safe and efficient palliative method for malignant gastric outlet obstruction, and can be performed successfully in a center with our characteristics.

[Prognostic factors in patients admitted with type 2 diabetes in Internal Medicine Services: hospital mortality and readmission in one year (DICAMI study)]. / Factores pronósticos en pacientes con diabetes mellitus tipo 2 ingresados en Servicios de Medicina Interna: mortalidad y reingreso hospitalario en un año (estudio DICAMI).

INTRODUCTION: Type II diabetes mellitus (T2DM) is a prevalent Public Health Care problem that causes an increase in morbidity, mortality and number of hospital admissions as well as increased costs in care services in this population group. The clinical indicator that determine readmission and/or death are analyzed in a 12 month follow-up period. METHODS: All T2DM patients admitted in Spanish Internal Medicine Services between two different periods (june 1-15, 2003 and november 1-15) were enrolled in a prospective cohort study. Primary endpoint were readmission and/or death in the year following the first admission. RESULTS: Population of the study (n = 482) was distributed in 229 males (47.5%) and 253 females (52.5%). Mean age was 73.48 +/- 8.86 years. A total of 210 (43.6%) were not readmitted to the hospital and/or died in the follow-up and 272 (56.4%) were readmitted and/or died. The latter 272 patients had a significantly greater percentage of heart failure (odds ratio [OR] 1.760; 1.073-2.886), atrial fibrillation (OR 1.747; 1.010- 3.022) and previous history of systolic blood pressure (OR 0.400; 0.241-0.666). They also showed increased levels of plasma glucose (OR 1.004; 1.001-1.007), and lower concentration of plasma hemoglobin (OR 0.756; 0.677-0.845) and creatinine clearance (OR 0.985; 0.976-0.994). CONCLUSIONS: T2DM patients who are admitted to the Internal Medicine Services in Spain are elderly patients with elevated indices of readmission and death in a short follow-up period (one year). The coexistence of heart failure, atrial fibrillation, renal dysfunction deterioration and decrease in hemoglobin levels may predict this worse outcome.

[Ocular manifestations in Proteus syndrome]. / Manifestaciones oculares en el síndrome de Proteus.

CASE REPORT: Congenital disfiguring malformations are rare and usually have a multifactorial aetiology. Here we report on the ocular manifestations seen in a patient with Proteus syndrome. The retina showed retinal dysgenesia, retinal pigmentary abnormalities and optic nerve hypoplasia. Other abnormalities included strabismus and high myopia. DISCUSSION: Proteus syndrome is a complex hamartomatous disorder defined by local overgrowth, subcutaneous tumours and various bone, cutaneous and/or vascular anomalies. The incidence of ocular malformations in Proteus syndrome is unknown, however a meticulous cranio-facial examination and a systematic study of the eye is required to improve the medical care of these patients.

[Cavitated pneumonia and toxic shock syndrome: presentation of a case]. / Neumonía cavitada y síndrome del shock tóxico: presentación de un caso.

Toxic shock syndrome (TSS) is a potentially fatal acute disease preferentially affecting menstruating women and is related to the use of vaginal tampons seems to be due to an specific exotoxin produced by some Staphylococcus aureus strains. We present here the case of a male patients suffering cavitated pneumonia who developed a systemic clinical picture comparable to TSS. This unusual form of presentation is discussed as well as the diagnostic criteria which define this entity.

Cyclosporine in the treatment of pure red cell aplasia.

We present a patient with severe idiopathic pure red cell aplasia who responded spectacularly to cyclosporin after failure of corticoid treatment and cyclophosphamide. We conclude that cyclosporin should be considered a viable therapeutic alternative to cytotoxic immunosuppressive treatments in this disease.

Visceral leishmaniasis (kala-azar) as an opportunistic infection in patients infected with the human immunodeficiency virus in Spain.

In an area endemic for visceral leishmaniasis, 16 patients with human immunodeficiency virus (HIV) infection developed the disease. All belonged to populations at risk for AIDS (15 were intravenous drug abusers). Five patients fulfilled the criteria for full-blown AIDS, and two more fulfilled them after diagnosis of leishmaniasis. All presented with the classic manifestations of visceral leishmaniasis, but leishmania serology was negative in 15 patients (93%). Leishmania donovani amastigotes were identified in the bone marrow in all cases. Most patients responded initially to treatment with pentavalent antimonial drugs, but seven (43%) followed a chronic course, with multiple relapses in five, despite alternative treatments. Visceral leishmaniasis occurred in patients with different levels of depression of the CD4 to CD8 lymphocyte ratio. Mortality was 37% (six patients) and was independent of the chronic-relapsing course of the disease. In no case was leishmaniasis the primary cause of death. Our data establish that visceral leishmaniasis is an opportunistic infection in HIV-infected patients, and we suggest that in endemic areas it should be considered an indicator disease for the diagnosis of AIDS.

[Bacteremia caused by Salmonella non-typhi in patients infected with HIV]. / Bacteriemia por Salmonella no-typhi en pacientes infectados por VIH.

All the cases of HIV infected patients diagnosed in our department during 1987 of Salmonella bacteremia are included in this report. Out of a total of 10 patients with both conditions, six were diagnosed of AIDS, three of AIDS related complex, and one presented an asymptomatic HIV infection. The mean age was 32.9 years (range 22 to 56). Eight of them were males, seven were intravenous drug addicts, and three were homosexual. Fever was observed in all cases and diarrhea in four of them. Salmonella enteritidis was isolated from the blood culture of nine patients and salmonella typhimurium from one. Salmonella enteritidis was isolated from the stool culture of 5 patients and Salmonella typhimurium and Campylobacter from the stool culture of one patient. Of the 10 patients 6 have died, 4 of them within the three weeks after the diagnosis of Salmonella non-typhi bacteremia, 2 have not relapsed and two patients were lost during follow up.