Satoyoshi's syndrome related muscle spasms: functional study.

Satoyoshi syndrome is a rare disease presumed to be immunologically mediated, characterized by muscle spasms, alopecia and diarrhea. We describe the case of a female in whom the muscle spasms were the predominant feature and we analyze the changes in cortical and in spinal excitability under the paired pulses paradigm. Hyperexcitability was present in the H-reflex study, thus suggesting that the spinal cord is the structure most likely responsible for the spasms. This is the first reported case in Spain.

Granulomatous cheilitis: a report of 6 cases and a review of the literature.

INTRODUCTION: Granulomatous cheilitis (Miescher cheilitis), a condition characterized by recurrent swelling of the lips, is the most common monosymptomatic form of the Melkersson-Rosenthal syndrome. The aim of this study was to study the characteristics of patients diagnosed with granulomatous cheilitis at the dermatology department of our hospital over a period of 17 years. MATERIAL AND METHODS: We performed a descriptive study of patients diagnosed with granulomatous cheilitis at our hospital between January 1993 and January 2010. RESULTS: The condition was diagnosed in 6 patients (4 women and 2 men), with a mean age of 49 years at the time of diagnosis. All the patients had recurrent swelling of the upper lip and 2 also had swelling in other parts of the face. The mean time from the onset of symptoms to the initial visit was approximately 16 months. There were no cases of facial palsy, and just 1 patient had a fissured tongue. None of the patients developed Crohn disease or any other granulomatous disorders during follow-up. CONCLUSIONS: Granulomatous cheilitis is a rare disease. None of the patients in our series had gastrointestinal or neurologic symptoms. Accordingly, we believe that granulomatous cheilitis is an independent orofacial granulomatous disease which most often presents without accompanying signs or symptoms.

[Referrals to dermatology: proportion of banal disorders]. / Demanda derivada a Dermatología: peso de la patología banal.

BACKGROUND AND OBJECTIVE: Skin diseases account for a large number of consultations in primary care. The objective of this study was to determine the characteristics and cost of referrals from primary care to a dermatology clinic. MATERIAL AND METHODS: Descriptive cross-sectional study of referrals from a primary care health center to a dermatology clinic. The dermatology clinic was situated in the same health center and was attended by a dermatologist from Complejo Hospitalario Universitario in Albacete, Spain. The study was performed on 10 days selected at random between April 21, 2009, and June 26, 2009. The data gathered included age, sex, use of cryotherapy, and diagnostic group. Patients were divided into 4 diagnostic groups: A) benign degenerative disease or trivial disorders whose treatment may not merit involvement of the national health service, B) diseases resolved with a single dermatology consultation at the health center, C) diseases requiring evaluation in hospital-based dermatology outpatients, and D) diseases referred for surgical treatment. RESULTS: Data were gathered on 257 patients with a mean age was 41.18 years and there was a slight female predominance. The majority of patients were in diagnostic group B (53.7%), followed by groups A (19.1%), C (19.1%), and D (8.2%). The total estimated cost of these 257 visits was €29 750.32, of which €5672.24 was for trivial disorders. CONCLUSIONS: The current high prevalence of trivial disorders in the caseload of dermatology clinics by trivial disorders makes it necessary to control referrals from primary care more strictly.

[Dermatology consultations in an emergency department prior to establishment of emergency dermatology cover]. / Consultas dermatológicas en el Servicio de Urgencias: situación previa a la instauración de guardias de la especialidad.

BACKGROUND AND OBJECTIVE: an increasing number of patients seek emergency treatment for dermatologic complaints. The aim of this study was to assess the characteristics of skin complaints seen in an emergency department prior to establishment of specialist dermatology cover. MATERIALS AND METHODS: a retrospective, descriptive study was undertaken using data on urgent dermatology cases seen by nonspecialist physicians in the emergency department of Hospital General Universitario de Albacete, Spain, in 2008. RESULTS: a total of 3662 patients with skin diseases were seen (2.59% of all emergency cases; approximately 10 patients per day). The mean age was 27.73 years and there was a slight predominance of female patients. Children and adolescents accounted for 5.85% of cases. A total of 96 different conditions were diagnosed and 84% of cases corresponded to one of 21 different diagnostic entities, urticaria being the most frequent (19.27%). The 96 diagnoses were grouped into 16 categories to facilitate analysis. According to this classification, most patients had infectious diseases (47.49%), followed by urticaria and angioedema (20.13%), "nonspecific diagnosis" (11.93%), and "descriptive diagnosis" (6.49%). In 4.8% of cases, the patient was admitted, most frequently for cellulitis. CONCLUSIONS: in nonspecialist emergency services, the number of different diagnoses is small in relation to the number of patients seen and the proportion of nonspecific and descriptive diagnoses is relatively large. In our opinion, an on-call dermatologist should be made available within emergency departments in order to offer a higher quality of care to patients with skin conditions.

[Leprosy and severe neuropathy in two native Spaniards]. / Lepra y neuropatía grave en dos nativos españoles.

INTRODUCTION: Leprosy is a widespread infectious disease in humans that is endemic to regions with poor sanitary conditions, especially in cases of overcrowding, malnutrition and bad hygiene. The disease is characterised by dermopathy, which is quite typical, but above all by neuropathy, which often becomes the most important element. In most cases, alterations to nerves are defined by sensory deficits that are predominantly distal and multiple neuritis in areas where nerve entrapment has taken place. CASE REPORTS: Two patients, both native Spaniards, presented largely overlapping clinical pictures, that is, a history of 'glove and stocking' type paresthesias and dysesthesias going back months or even years and functional impotence, which gave rise to a very pronounced gait disorder. In the two cases, the immunological situation was determined to be borderline lepromatous leprosy. The neurophysiological study revealed the presence of severe, diffuse sensory-motor axonal polyneuropathy that was predominantly distal, and several entrapped nerves. The dermatological illness was greatly improved by the treatment. The same was partially true, although to a satisfactory extent, of the neurological disease. CONCLUSION: We describe the cases of two Spaniards with borderline lepromatous leprosy with no past history of the disease, in whom neuropathy was the predominant symptom. We highlight the speed with which the neuropathies progressed, probably due to a change in 'polarity', and the severity of the neurological deficits in comparison with the dermopathy, in an unusual immunological situation. The growing number of native patients in the first world, even when there is no relevant history, suggests that we should not think of leprosy as something only occurring in immigrant patients from places where it is endemic, although the epidemiological relationship has still not been determined.

The Ginkgo biloba extract (GbE) protects the kidney from damage produced by a single and low dose of carbon tetrachloride in adult male rats.

Gingko biloba leaves have been used as herbal medicine in China for 5000 years, and the standardized leaf extract (GB-STE) has some beneficial effects in the treatment of age-related, cardiovascular, and neurological diseases. The aim of this study was to investigate the renoprotective effects of the Gingko biloba extract (GbE) against the toxicity of a single and relatively low dose of carbon tetrachloride (CCl4). In male adult Wistar rats, we determined the urine flux, the concentration of total proteins in urine, the concentration of glucose in urine, and the concentration of malondialdehyde (MDA) in renal cortex as well as two markers of renal function (clearance of inulin and p-aminohippurate); we also compared the histological lesions caused by CCl4. Carbon tetrachloride increased the urinary concentration of total proteins, and the renal concentration of MDA; however, it did not modify the urine flux, urinary concentration of glucose, nor the inuline or the p-aminohipurate clearances. Morphologically, CCl4 generated some tubular damage that was more intense in the inner cortex of kidneys. The GbE extract counteracted the effects of CCl4 on the concentration of total proteins in urine, the concentration of renal MDA, and the renal histological changes. In conclusion the main toxic effects produced by CCl4 were prevented by the GbE, probably due to their antioxidant properties and the inhibition of the main P450 isoenzyme (CYP2E1) that metabolize CCl4.

[Embryopathy due to valproic acid with severe malformations in the central nervous system]. / Embriopatía por ácido valproico con malformaciones graves del sistema nervioso central.

INTRODUCTION: Embryogenetic disorders are one of the most serious problems in the life of an epileptic. Over the last few decades many antiepileptic drugs, including valproic acid, have been shown to have teratogenic properties. Embryopathy due to valproate, also known as fetal valproate syndrome, is a well-known and documented example of these conditions. CASE REPORT: We report the case of a preterm newborn infant who, at birth, exhibited a syndrome characterised by facial dysmorphia, gingival hyperplasia, neurological hyperexcitability and multiple malformations, the most striking of which was the presence of predominantly temporal atrophy in the left brain hemisphere. The most significant event in the medical history of the case was the mother's taking valproate in monotherapy throughout the entire period of gestation as treatment for generalised idiopathic epilepsy that was diagnosed during adolescence. Screening precluded the most common metabolic, hereditary or infectious causes that can cause embryopathies. CONCLUSIONS: The mother's history of taking valproic acid and the specific findings that coincided in the peculiar embryopathy of this patient enabled us to link them.

[Pure neural leprosy. Diagnostic aspects of a clinical case]. / Lepra neural pura. Aspectos diagnosticos en un caso clinico.

INTRODUCTION: Leprosy is an infectious disease caused by the bacteria Mycobacterium leprae. It is particularly prone to affect the skin and the nerve trunks and, in fact, both are compromised in most infected patients. It is transmitted by exposure to those with the disease and sometimes by reactivation. One uncommon possibility is pure neural leprosy, which is characterised by neuropathy, but without skin lesions. We report the case of a patient with pure neural leprosy and review the diagnostic aspects. CASE REPORT: A 40-year-old male, an immigrant who was diagnosed and treated for leprosy 20 earlier. The patient visited due to painful paraesthesias and dysesthesias in the hands and legs without the presence of any skin lesions. Acute multiple mononeuritis with mainly ulnar involvement was observed. The disease, typified as paucibacillary/tuberculoid, was treated and in a few weeks there was a clear improvement. CONCLUSIONS: In this case of pure neural leprosy due to reactivation, early diagnosis allowed timely treatment to be established. Evaluation of neuropathy together with clinical, electrophysiological and ultrasound criteria is recommended. By so doing, a high degree of sensitivity is achieved as well as allowing early diagnosis and treatment, and therefore a better functional recovery.

TITLE: Lepra neural pura. Aspectos diagnosticos en un caso clinico.Introduccion. La lepra es una enfermedad infecciosa causada por la bacteria Mycobacterium leprae. Presenta especial avidez por la piel y los troncos nerviosos, y, de hecho, ambos se afectan en la mayor parte de los infectados. Se trasmite por exposicion con enfermos y en ocasiones por reactivacion. Una posibilidad inhabitual es la lepra neural pura, caracterizada por neuropatia, pero sin lesiones en la piel. Se describe un paciente con lepra neural pura y se revisan los aspectos diagnosticos. Caso clinico. Varon de 40 años, inmigrante, diagnosticado y tratado de lepra 20 años antes. Acudio por parestesias y disestesias dolorosas en las manos y las piernas sin lesiones en la piel. Se demostro mononeuritis multiple aguda con principal afectacion de cubitales. La enfermedad, tipificada como tuberculoide paucibacilar, se trato y en pocas semanas la mejoria fue evidente. Conclusiones. En este caso de lepra neural pura por reactivacion, el diagnostico temprano permitio un rapido tratamiento. Es recomendable la evaluacion de la neuropatia integrada con criterios clinicos, electrofisiologicos y ecograficos. De este modo se consigue una alta sensibilidad y especialmente una precocidad en el diagnostico y la instauracion del tratamiento, y por consecuencia una mejor recuperacion funcional.

Five-year experience of tele-ophthalmology for diabetic retinopathy screening in a rural population. / Análisis de un programa de teleoftalmología para el cribado de retinopatía diabética en área rural tras cinco años.

OBJECTIVE: To identify the prevalence and risk factors of diabetic retinopathy (DR) among rural inhabitants included in a tele-ophthalmology program. To analyse diagnostic accuracy among primary care physicians, concordance with ophthalmologists, and financial savings. MATERIAL AND METHODS: An observational randomised study was conducted on 394 patients included in a tele-ophthalmology program (from January 2010 to January 2015). An analysis was performed on the clinical characteristics, DR findings in retinography images, and correspondence between the request for a second interpretation by an ophthalmologist, and previously established criteria for it: presence of moderate to severe DR, vision loss, poor image quality and/or intraocular pressure >22mmHg. Statistical analysis was performed using the SPSS program (Student t and χ(2) tests). RESULTS: DR prevalence was 12.1%. Patients with glycosylated haemoglobin values >7.68% or those treated with a combination of insulin and oral antidiabetic drugs showed a higher risk of DR (P<.05). 43.3% of patients correctly referred to ophthalmologists showed moderate to severe DR. Unnecessary referrals to specialists were improved from 91.7% in 2010 to 98.6% in 2014. It is estimated that the program has made a total saving of €152,550.45. CONCLUSIONS: Tele-ophthalmology programs are a useful tool in DR screening. Primary care physicians are able to distinguish patients who need specialist care, avoiding unnecessary referrals to ophthalmologists, and saving costs.

The ATP levels in kidneys and blood are mainly decreased by acute ingestion of tullidora (Karwinskia humboldtiana).

Our previous acute toxicity studies with Karwinskia humboldtiana (Kh) in rats showed renal hemodynamic changes with a marked increase in the fractional excretion of sodium and morphological damage. To analyse the effects of Kh or 'tullidora' on energetic metabolism, a single dose of an oral preparation from the seed fruits was given to Wistar rats (1.25 g/kg). In tullidora-treated rats there was 8% mortality. ATP concentrations in renal tissue decreased significantly (control: 53.85+/-3.34, tullidora 38.28+/-5.31 micromol/g fresh tissue, P<0.05). Total blood (54.8+/-0.96, tullidora: 40.2+/-1.55 micromol/dL, P<0.01) and haemoglobin-ATP concentrations (3.69+/-0.12, tullidora: 2.56+/-0.11 micromol/g, P<0.01) were also significantly diminished. Moreover, the total protein in renal cortex from tullidora-treated rats decreased as compared to control group (control: 71.43+/-2.88, tullidora: 55.20+/-4.06 mg/g fresh tissue, P<0.05). In contrast, Na+-K+-ATPase activity in tullidora-treated animals was not different from control rats. These findings might partially explain the acute effects and mortality observed in the Kh treated rats.

Glutathione S-transferases and esterases in placenta after normal and pre-eclamptic pregnancies.

Severe pre-eclampsia reduced significantly (P<0.05) by 68+/-6 per cent (mean+/-sem, n=10) the maximal velocity (V(max)) and, consequently, reduced significantly by 60+/-7 per cent the catalytic efficiency (C(E)) of placental glutathione transferase pi, assayed with ethacrynic acid. Mild and severe pre-eclampsia reduced significantly by 82+/-5 per cent (mean+/-sem, n=5) and by 41+/-5 per cent (mean+/-sem, n=10), respectively, the V(max)and, consequently, reduced significantly by 72+/-7 and by 33+/-13 per cent, respectively, the C(E)of esterase, assayed with p-nitrophenyl acetate. Furthermore, severe pre-eclampsia increased significantly by 296+/-78 per cent the Michaelis-Menten constant (K(m)) of total GST, assayed with chlorodinitrobenzene and, consequently, decreased significantly the C(E)by 83+/-3 per cent. On the other hand, the concentrations of total and non-protein thiols did not change significantly in placental homogenates from patients with mild or severe pre-eclampsia compared to normal pregnancies. These findings would indicate a decreased capacity of the glutathione transferases and esterase detoxification systems to protect the fetus from drugs prescribed to pregnant women suffering pre-eclampsia, mainly in the severe phase.

[Traumatic maculopathies]. / Maculopatías traumáticas.

PURPOSE/METHODS: We reviewed all patients seen urgently on our Hospital and we studied those that present contusive pathology of the anterior pole with macular repercusion. RESULTS/CONCLUSIONS: Post-traumatic maculopathies are usually produced by contusion, namely: rupture of the choroid, chorioretinitis sclopetaria, postraumatic macular hole, commotio retinae (Berlin's oedema) and Purtscher's retinopathy was reported. With the presentation of these clinical cases, some of which are very typical, we aim to show the functional sequelae some patients suffer.