Tremor as the first neurological manifestation of Sneddon's syndrome.

We report on a 54-year-old woman with Sneddon's syndrome manifested by livedo reticularis, fetal losses, hypertension, and high antinuclear antibody titres. At the age of 42 years she developed tremor of the trunk, limbs, and head only in the standing position that interfered with walking, followed some years later by cognitive decline and a parkinsonian syndrome. T2-weighted brain magnetic resonance imaging showed high signal in cortical areas, basal ganglia, midbrain, and cerebellum.

Hipoparatiroidismo e hipercalcemia / Hypoparathyroidism and hypercalcamia

Presentamos el caso clínico de una mujer de 68 años de edad que tenía convulsiones debido a una hipocalcemia como consecuencia de un hipoparatiroidismo primario, y que posteriormente evoluciona a una hipercalcemia debido a un deterioro de la función renal por deshidratación, uso de inhibidores de la enzima convertidora de la angiotensina y posiblemente una intoxicación por vitamina D (AU)

Correlation of genotypes and route of transmission with histologic activity and disease stage in chronic hepatitis C.

Our objective was to evaluate the histopathological features of chronic hepatitis C of 64 liver biopsies and to correlate this with the route of transmission of hepatitis C virus, the genotype of HCV, and the patient's age. Moderate chronic hepatitis was the most frequently observed (62.5%). Cirrhosis was observed in 14 patients (21.9%) and was more frequently found among patients over 40 years of age (34.3% vs. 6.9%, P = 0.025). The mean histopathological activity index (HAI) was significantly higher in the sporadic (10+/-3.1) than the posttransfusional (7.5+/-3.7) and the intravenous drug use (IVDU) groups (6.3+/-2.8) (P<0.02). Moreover the sporadic group showed more fibrosis (P<0.04) than the posttransfusional group. No liver cirrhosis was found in the IVDU group. The overall prevalence of HCV variants was: 54.7% type 1b, 4.6% type 1a, 37.5% type 2c, 1.6% type 2b, 1.6% type 2. The genotype distribution showed no relation to the HAI, hepatitis activity (grade), and fibrosis (stage) of the liver disease. In conclusion, the sporadic route of transmission of HCV was related to a more severe chronic hepatic disease, a finding that could influence future antiviral therapies. The predominance of HCV type 1b in this study reflects the higher frequency of this variant in our area. Our data suggests that the ultimate consequence of HCV chronic infection depends on patient age rather than on HCV genotype.

DYSBOT: a single-blind, randomized parallel study to determine whether any differences can be detected in the efficacy and tolerability of two formulations of botulinum toxin type A--Dysport and Botox--assuming a ratio of 4:1.

BACKGROUND: Elston and Russell discovered a difference in the biological potency of the English formulation of botulinum toxin type A or BTX-A (Dysport) and the American formulation (Botox). Potency of both is expressed in LD50 mouse units, but because of assay differences, these units are not equivalent. Since the first warning by Quinn and Hallet on the clinical importance of this issue, it has been impossible to reach a consensus on the conversion factor for the potency of these formulations. OBJECTIVE: To test the hypothesis that the conversion factor for the clinical potency of Dysport to Botox is approximately 4:1. DYSBOT is an acronym that results from adding "DYS" from Dysport with "BOT" from Botox. DESIGN: A single-blind, randomized, parallel comparison. A total of 91 patients with blepharospasm or hemifacial spasm were randomized to treatment with Dysport or Botox using a fixed potency ratio of 4:1. Clinical evaluations: The patients were evaluated at baseline (day of the treatment). 1 month after treatment, and whenever the effect was judged to be fading. Objective and functional rating scales were used as quantitative measures of the change in clinical status. Adverse reactions were collected using a systematic questionnaire. RESULTS: Using this ratio between products, both Dysport and Botox groups produced similar clinical efficacy and tolerability. For patients showing a positive response without the need of a booster, the duration of effect was 13.3 +/- 5.9 weeks for the Dysport group and 11.2 +/- 5.8 weeks for the Botox group. Of 48 patients, 11 (23%) needed booster treatment in the Dysport group compared with five (12%) of 43 in Botox group. Adverse events were noted in 24 (50%) of 48 patients in the Dysport group and 20 (47%) of 43 of the Botox-treated group. CONCLUSIONS: Using a 4:1 conversion ratio for Dysport and Botox, similar results were obtained for the two treatments in an appropriately powered study, suggesting that this conversion factor is a good estimate of their comparative clinical potencies.

An aggressive protocol of ESWL and dissolution therapy of gallbladder stones.

OBJECTIVES: to assess the overall efficacy and which factors are independent predictors of success of ESWL and oral dissolution therapy of gallbladder stones using an aggressive protocol (high energy shock waves -median 22 Kv- and allowance to up to 6 sessions with an electro-hydraulic lithotripter). PATIENTS AND METHODS: inclusion criteria were 1) biliary pain; 2) 1 to 3 radiolucent stones or with slight calcification; 3) total stone volume under 15 cm3, equivalent to a single stone 3 cm diameter and 4) opacified cholecystography. Data was collected prospectively for 139 consecutive patients undergoing this treatment and the stone-free curves up to 12 months were analyzed as a function of age, sex, body-mass index, total stone volume, number of stones and the presence of slight calcification. RESULTS: patients underwent a mean of 2.6 sessions (range: 1-6) and 2834 shock waves (range: 589-8175). The global stone-free rate at 12 months was 54% (95% confidence interval: 45-64%). Factors that significantly -and adversely- influenced outcome were total stone volume (P < 0.001), number of stones (P = 0.005) and slight calcification (P = 0.038), using Cox's regression. Beyond significance, these three factors showed a marked effect on the stone-free curves. CONCLUSIONS: our data suggest that, even with this aggressive protocol, these factors are clearly detrimental. Thus, the results of our study agree with the current trend to restrict this combination therapy to patients with single, non-calcified stones with a small volume, or up to 2 cm diameter as is usually quoted.

Migraine and intracranial vascular malformations.

The relationships between migraine and A-V Malformations is a subject of controversy and the arguments are mainly based on case reports and retrospective data. To clarify this subject a structured inquiry and classification of headaches in large samples of patients with intracranial vascular malformations (IVM) is essential. The authors studied the prevalence of headaches in 51 patients with IVM admitted to our Department, between 1984 and 1992. The methods used were a review of medical records followed by a self-administered headache questionnaire and clinical interview using the IHS criteria for the diagnostic classification of headaches. The relative frequency of the different types of headaches was calculated and compared with the general population data. A correlative study of the headache characteristics with the type and location of the IVM was made. A high prevalence (47%) of migraine type headaches and a strong positive correlation (88.8%) between the site of AVM and side of the pain was found. This is highly suggestive but not conclusive of a pathophysiologic relationship between these entities. The conclusion drawn is that a prospective study of headaches by questionnaire or semi-structured clinical interview in patients with IVM is essential to discover the effective prevalence and characteristics of headaches associated with IVM and their relationships.

[Extracorporeal shockwave lithotripsy in the treatment of gallbladder lithiasis. The first 109 patients]. / A litotrícia extra-corporal por ondas de choque no tratamento da litíase vesicular. Os primeiros 109 doentes.

The AA report their 15-month experience with extracorporeal shock-wave lithotripsy (ESWL) in the treatment of gallbladder stones (GS). The selection criteria included symptomatic patients, with 1 to 3 radiolucent stones in a functioning gallbladder. All patients were put on adjuvant therapy with 10 mg/Kg weight/day of ursodeoxycholic acid (ursodiol). ESWL sessions were performed in an ambulatory setting, using high-energy shock-waves (mean: 23 Kv) until fragments < or = 4mm were obtained, if possible. By the end of December 1992, 109 patients had completed the ESWL protocol, undergoing a total of 265 sessions (mean: 2.4 sessions per patient, variation 1-5). The stone-free (SF) rates and respective 95% confidence intervals were 31% (22%-43%) at 6 months and 59% (38%-76%) at 1 year of follow-up. For the subset of patients with a single stone < or = 2 cm (n = 59), were 47% (33%-63%) at 6 months and 77% (40%-95%) at 1 year. The most significant complication was acute biliary pancreatitis, of which we report 4 cases (4%). They were always mild and non-complicated. There was no mortality. The AA conclude that ESWL is a safe and effective treatment for selected patients with GS.

[Endoscopic sphincterotomy in choledocholithiasis: analysis of an experience of 530 interventions]. / Esfincterotomia endoscópica na coledocolitíase: análise de uma experiência de 530 intervenções.

We report the experience of 530 consecutive cases of choledocolithiasis treated through Endoscopic Sphincterotomy (ES) between January 1980 and January 1988 (74% of total patients submitted to ES in the same period). 73% had more than 60 years and 47% more than 70 years. A slight majority (52%) had previous cholecystectomy (1/3 still with T-tube drainage) and the remaining had gallbladders insitu (lithiasic in 82%). Therapeutic goals (achieved drainage and complete stone removal) have been achieved in 95% of patients with a low immediate morbidity (9%) and mortality (1%). Delayed mortality, until 30th day (3%) includes 6 cases of obstructive cholangitis with sepsis, unaltered by emergent ES. Follow-up showed a 3% incidence of reintervention from restenosed ES. Patients with lithiasic gallbladder had a 33% cholecistectomy rate, mostly elective during first month post-ES. Our experience confirms ES as an efficient and safe routine treatment for choledocolithiasis. It should be the first choice in patients with previous cholecystectomy or alithiasic gallbladder and a largely applicable tool in those with associated gallbladder stones. Decision about post-ES cholecystectomy in these patients must be weighed on clinical grounds and according to surgical risk groups.

Floxacillin-induced cholestatic hepatitis with evidence of lymphocyte sensitization.

A severe and prolonged form of biopsy-proved cholestatic hepatitis occurred in a 45-year-old man who had received floxacillin for two weeks preceding the episode of drug-related cholestatic injury. Immunologic tests revealed evidence of in vitro sensitization to the drug as well as to the serum of a normal subject collected after ingestion of floxacillin. Floxacillin should be added to the list of drugs causing cholestatic hepatitis, most likely by an immunologic mechanism.