Leiomyosarcoma of the inguinal canal.

We describe a rare case of a leiomyosarcoma in the inguinal canal in a patient presenting clinically with an inguinal hernia. The clinical details, histological findings and surgical management are reviewed.

Outcome reporting in neoadjuvant surgical trials: a systematic review of the literature and proposals for new standards.

BACKGROUND: The use of neoadjuvant therapy before surgery for gastrointestinal cancer is increasing; however, patients may not complete both treatment components. Understanding completion rates of each treatment stage is necessary for treatment evaluation and to inform decision-making. This study evaluates reporting for recent neoadjuvant surgical trials, focusing on treatment progression and other key outcomes. METHODS: Systematic literature searches identified randomized and nonrandomized phase II and III studies evaluating neoadjuvant treatment and surgery for esophageal, stomach, and colorectal cancer, and colorectal liver metastases. Rates of reporting of failure to complete neoadjuvant treatment, nonprogression to surgery after neoadjuvant treatment, and nonresection at planned surgery were assessed. For each measure, reporting was categorized as "full," "partial," and "absent" according to predefined criteria, and reasons for nonprogression at each stage of treatment were examined to inform proposed standards. RESULTS: Of 9854 abstracts, 123 papers were reviewed and 62 articles were included, reporting outcomes for 9126 patients. Details of noncompletion of neoadjuvant treatment and nonprogression to surgery were completely absent in 21 (33.9%) and 19 (30.6%) studies, respectively. Reporting of nonresection at planned surgery was also deficient, with 21 (33.9%) studies providing no information about this outcome. Reasons for noncompletion and nonprogression were similar and included disease progression, treatment toxicity, and patient choice. Common reasons for nonresection were locally advanced disease and the discovery of unsuspected metastases. CONCLUSIONS: Reports of recent neoadjuvant surgical trials often fail to include treatment progression and other key outcomes. These findings support the need for minimum reporting standards.

Comparación farmacocinética de Sinemet y Grifoparkin (levodopa/carbidopa 250/25 mg) en pacientes con enfermedad de Parkinson avanzada: un estudio con dosis única / Pharmacokinetic comparison of Sinemet and Grifoparkin (levodopa/carbidopa 250/25 mg) in Parkinson s disease: a single dose study

BACKGROUND: There are doubts wether generic medications have the same bioavailability and efficacy compared with the original drugs developed by pharmaceutical companies with research capabilities. AIM: To compare the pharmacokinetics and clinical (motor) responses of Sinemet and Grifoparkin (generic carbidopa/levodopa 250/25 mg) in patients with advanced Parkinson's disease. PATIENTS AND METHODS: Patients were randomly assigned to Sinemet (15 patients 62 +/- 12 years old; mean disease duration 11 +/- 7 years) or Grifoparkin (15 patients, 64 +/- 11 years old; mean disease duration 12 +/- 4 years) groups. Medication and food were withheld 12 h before the study. Fifteen blood samples were collected (starting 9 AM) immediately before (sample 1, t = 0 min) and after (samples 2-15, t = 20-360 min) oral administration of a single dose of Sinemet or Grifoparkin, and plasmatic L-DOPA was quantified using HPLC with electrochemical detection. Additionally, each patient was clinically evaluated every 20 minutes, using the tapping test and the unified Parkinson's disease scale Hoehn & Yarh. RESULTS: Tmax (time at which the maximal L-DOPA concentration was reached) were 69 +/- 12 min and 64 +/- 11 min for Sinemet and Grifoparkin respectively (NS). Cmax (maximal L-DOPA concentration reached) was 3161 +/- 345 ng/ml for Sinemet and 3274 +/- 520 ng/ml for Grifoparkin (NS). The t1/2 (half life time), CL (clearance) and volume of distribution (Vd) values calculated were 159 +/- 32 min, 51.7 +/- 5.1 1/h and 3.6 +/- 1.2 l/kg for Sinemet and 161 +/- 48 min, 58.7 +/- 8 l/h and 3.0 +/- 0.7 l/kg for Grifoparkin (NS). UPDRS-III value for the best on state and for the worst off state were 23 +/- 11 and 50 +/- 19 for Sinemet and 20 +/- 7 and 46 +/- 13 for Grifoparkin respectively (NS). CONCLUSION: The results obtained showed that both drugs are bioequivalent in patients with advanced Parkinson's disease.

The evolution and origin of motor complications in Parkinson's disease.

Levodopa is the major symptomatic therapy for Parkinson's disease (PD), having revolutionized the treatment of PD and provided benefit to virtually all patients. However, after 5-10 years of treatment, levodopa therapy is complicated by the development of motor complications, which include dyskinesia and motor fluctuations. The initial long duration response to a dose of levodopa becomes progressively shorter, and periods in which the patient responds to the drug become complicated by involuntary dyskinetic movements. Thus, patients may cycle between "on" periods that are complicated by dyskinesia and "off" periods in which they are severely parkinsonian. As a consequence they may experience profound disability despite the fact that levodopa remains an effective anti-parkinson agent throughout the course of the disease. In this article we review the various motor complications associated with the treatment of PD and present current concepts on the origin of these problems.

[Tics disease (Gilles de la Tourette syndrome): clinical characteristics of 70 patients]. / Enfermedad de los tics (síndrome de Gilles de la Tourette): características clínicas de 70 pacientes.

BACKGROUND: Tourette's syndrome is a childhood-onset hereditary neurobehavioural disorder believed to occur without geographical restrictions. Although there have been reports of this disorder worldwide just a few are from Latin America. AIM: To report a preliminary experience with a series of 70 patients and to review recent advances in this disorder. PATIENTS AND METHOD: We reviewed patients seen in pediatric and adult neurological clinics in Santiago, Chile, all of whom fulfilled clinical diagnostic criteria for Tourette Syndrome. RESULTS: Seventy patients were studied, 54 males (77.1%) and 16 females (22.8%), their mean age at first evaluation was 13.6 years (range 2-46). The mean age of onset of symptoms was 6.4 (range 2-20), the mean time of follow-up was 3 years. Fifty-eight patients showed simple motor tics (blinking, facial grimacing, shoulder shrugging), whereas dystonic tics like head jerking were seen in 38 patients, torticollis in 6 and oculogyric movements in 2. Complex motor tics like jumping, antics, trunk bending and head shaking were present in 16 subjects. Vocal tics were predominantly of the simple type: sniffing, throat clearing, blowing, and whistling. Complex vocal tics were seen in 12 patients, five cases showed palilalia, 3 echolalia and only six displayed coprolalia (8.5%). Tics were of mild to moderate severity in most patients. Obsessive-compulsive disorder was observed in 22.8% and attention deficit and hyperactivity disorder were present in 35.7%. Forty-five patients (64.2%) had a first degree relative with tics, nine patients (12.8%) had a family history of obsessive-compulsive disorder. The current evidence involving desinhibition of cortico-striatum-thalamic-cortical neuronal circuits in the pathogenesis of this disorder is analyzed. CONCLUSION: Our report supports the recognized clinical homogeneity and genetical basis of Tourette's syndrome regardless of geographical region and ethnic origin.

[Accessibility to the specific pharmacotherapy for Parkinson disease, in Santiago de Chile]. / Accesibilidad a la farmacoterapia específica de la enfermedad de Parkinson, en Santiago de Chile.

BACKGROUND: The high costs of adequate pharmacological treatment for Parkinson disease preclude the universal access of patients to this medication. AIM: To assess the accessibility to pharmacological treatment of Chilean patients with Parkinson disease. PATIENTS AND METHODS: An inquiry about socioeconomic stratification, medical controls, disease features and costs of pharmacological therapy, was applied to 95 patients (56 male, aged 32 to 89 years old) attending the Chilean League Against Parkinson Disease. RESULTS: Among studied patients, the disease had a mean duration of 7.9 +/- 6 years. There was a direct relationship between family income, the frequency of visits to physicians and the dose and cost of pharmacological therapy. CONCLUSIONS: The accessibility to pharmacological therapy among patients with Parkinson disease, depends on their socioeconomic level. Measures to correct this situation and improve the quality of life of these patients should be undertaken by health services.

Complicaciones motoras precoces en parkisonianos de novo tratados con L-Dopa más inhibidor periférico de la decarboxilasa / Early motor complications in parkisonians of novo treated with L-Dopa plus descarboxylase inhibitor

En una serie de 27 pacientes con enfermedad de Parkinson esencial de novo, en grado 2,7 de Hoehn y Yahr como promedio, con un rango de 1 a 4, tratados con levodopa más benserazida durante 16 semanas, se observaron efectos motores adversos leves a moderados en 12 casos (44,4 por ciento): 8 pacientes presentaron diskinesias, 7 distonías y un deterioro de final de dosis. La dosis máxima de levodopa ID utilizada fue de 750 mg los que alcanzaron a la tercera semana, previo ascenso paulatino desde 125 mg inicial. Posteriormente, se buscó la mínima dosis efectiva la cual correspondió a una cifra de 475 mg como media con un rango de 250 a 750 mg correspondiente a 49,7 por ciento de reducción, lográndose un efecto antiparkinsoniano similar al obtenido con la dosis máxima. Se discuten los posibles factores que hayan influido en el porcentaje alto de efectos motores adversos encontrados en esta serie

[The hepatic porphyrias: experience with 105 cases]. / Las porfirias hepáticas: experiencia con 105 casos.

Hepatic porphyria is a rare metabolic syndrome caused by abnormal enzyme activity in heme biosynthesis. Between 1974 and 1991; 105 patients have met criteria for diagnosis of hepatic porphyria based on typical clinical findings and/or laboratory abnormalities. According to type, 42% had porphyria cutanea tarda, 21% porphyria variegate, 15% protoporphyria, 6.7% acute intermittent porphyria, 6.7% coproporphyria and 1.9% porphyria due to porphobilinogen deficit. A proper classification was not established in 6.7% of patients. Porphyria cutanea tarda was more common in males (70%) and porphyria variegata, in females (90%). A family history of the disease was present in 33% of patients; 20% of patients were of European descent and 4% of Mapuche descent. Diagnosis was usually established in the third decade, somewhat later in porphyria cutanea tarda (45 years of age) and very early in protoporphyria. 10% of patients were asymptomatic and 29 patients developed at least one porphyric crisis. These were related to pregnancy in 6 patients, to hormone administration in 7, to antibiotics in 5. No cause was established in 21 cases. Severe crisis were successfully treated with Hematin. Venipuncture was used to treat 50% of patients with porphyria cutanea tarda with 95% success. Thus, hepatic porphyria is recognized with increasing frequency and can be treated successfully in most cases.

[Convulsive electrotherapy in Parkinson disease. Presentation of a clinical case]. / Electroterapía convulsiva en la enfermedad de Parkinson. Presentación de un caso clínico.

Electroshock therapy was performed in a patient with Parkinson disease who had shown intolerance to treatment with levodopa. Evaluation at 1 and 6 months post therapy revealed mild and short lived antiparkinsonian effects. The theoretical basis for this therapy are discussed.

[Levodopa and controlled release benserazide in the handling of motor fluctuations in Parkinson's disease]. / Levodopa más benserazida de liberación controlada en el manejo de lasfluctuaciones motoras en la enfermedad de Parkinson.

Ten advanced Parkinson pts (mean 8 years since diagnosis), 6 male and 4 female, 57 to 69 years old, mean 5.9 years on L-Dopa therapy, were put on Madopar HBS to assess the efficacy of the drug. All the pts had levodopa end-of-dose wearing-off type secondary motor fluctuations, 9 of them with dyskinesia and dystonia. Clinical evaluation was performed in basal conditions (pts on standard L-Dopa therapy) 1.6 and 12 months on Madopar HBS therapy. Parkinson signology was quantified with the modified Columbia scale (0 to 44), and motor fluctuations and dyskinesia with a scale 0 to 4 according to intensity and frequency. Pts received mean 1.150 mg. HBS daily dose plus 100 to 200 mg standard L-Dopa added to the early morning dose for a faster effect. At 12 months, a 60% decrease in "off" periods, a 50% decrease in feet dystonia, with no change in orofacial dystonia were observed. Dyskinesia decreased in intensity but not in frequency. There was a 50% decrease of Parkinson signology in "on" periods. In conclusion, Madopar HBS reduces the signology of the long-term Levodopa therapy syndrome and therefore is commendable in pts with advanced Parkinsonism.