Analysis of the EEG Dynamics of Epileptic Activity in Gelastic Seizures Using Decomposition in Independent Components

Objective: Gelastic seizures are a frequent and well established manifestation of the epilepsy associated with hypothalamic hamartomas. The scalp EEG recordings very seldom demonstrate clear spike activity and the information about the ictal epilepsy dynamics is limited. In this work, we try to isolate epileptic rhythms in gelastic seizures and study their generators. Methods: We extracted rhythmic activity from EEG scalp recordings of gelastic seizures using decomposition in independent components (ICA) in three patients, two with hypothalamic hamartomas and one with no hypothalamic lesion. Time analysis of these rhythms and inverse source analysis was done to recover their foci of origin and temporal dynamics. Results: In the two patients with hypothalamic hamartomas consistent ictal delta (2–3 Hz) rhythms were present, with subcortical generators in both and a superficial one in a single patient. The latter pattern was observed in the patient with no hypothalamic hamartoma visible in MRI. The deep generators activated earlier than the superficial ones, suggesting a consistent sub-cortical origin of the rhythmical activity. Conclusions: Our data is compatible with early and brief epileptic generators in deep sub-cortical regions and more superficial ones activating later. Significance: Gelastic seizures express rhythms on scalp EEG compatible with epileptic activity originating in sub-cortical generators and secondarily involving cortical ones.

SIXTEEN YEARS OF PITYRIASIS VERSICOLOR IN METROPOLITAN AREA OF PORTO ALEGRE, SOUTHERN BRAZIL

SUMMARY Pityriasis versicolor is the most common of the diseases caused by Malasseziayeasts. The aim of this study is to determine the prevalence of pityriasis versicolor and its etiological aspects in the metropolitan area of Porto Alegre, Brazil. A retrospective crosssectional study with data from patients of a reference hospital from 1996 to 2011 was performed. Collected data included: date, age, gender, ethnicity, anatomical region of lesion and the direct mycological examination results. Among the positive results in the direct mycological examination, 5.8% (2,239) were positive for pityriasis versicolor. The angular coefficient (B) was -0.3%/year, showing a decrease over the years. The disease was more prevalent in men (7.1% of men versus 5.1% of women that underwent the direct mycological examination); younger age (median 31 years old); "pardo" and black people (3.7% more than expected in the sample); trunk (73.44% of the affected anatomic sites). Lesions in rare sites (groin, genitals, legs, feet and hands) were also observed in this study. In conclusion, due to the decrease in the prevalence of pityriasis versicolor, long-term epidemiological studies in the metropolitan area of Porto Alegre, Brazil, are needed to continue the monitoring of this disease.

Chronic Calcified Subdural Hematoma: Case Report and Review of the Literature

Background: Calcified chronic subdural hematoma is a rare but known entity, estimated to represent 0.3-2.7% of chronic subdural hematomas. Although surgical treatment is unanimous for chronic subdural hematomas, therein lies some doubt on it being applied to calcified chronic subdural hematomas. Case Description: We report a case of a 73‑year‑old male, presenting with deterioration of motor function in his right limbs since 18 months, with computed tomography (CT) scans and magnetic resonance imaging (MRI) documenting a large subdural collection of the left hemisphere, with calcified inner membrane, which was successfully and completely removed, with progressive clinical and radiological improvement. Conclusions: We report a case where this type of rare lesion was removed with a progressive and complete resolution of the patient’s symptoms, restoring his previous neurological condition. From the cases described in the literature and our own experience with this case, we think surgical treatment in these patients, when symptomatic, is necessary and viable, frequently resulting in the patient’s improvement.

NEW PRIMERS FOR DETECTION OF Leishmania infantum USING POLYMERASE CHAIN REACTION

SUMMARY Leishmania infantum causes visceral leishmaniasis (VL) in the New World. The diagnosis of VL is confirmed by parasitological and serological tests, which are not always sensitive or specific. Our aim was to design new primers to perform a Polymerase Chain Reaction (PCR) for detecting L. infantum. Sequences of the minicircle kinetoplast DNA (kDNA) were obtained from GenBank, and the FLC2/RLC2 primers were designed. Samples of DNA from L. infantum, Leishmania amazonensis, Leishmania braziliensis, Leishmania guyanensis, Leishmania naiffi, Leishmania lainsoni, Leishmania panamensis, Leishmania major and Trypanosoma cruzi were used to standardize the PCR. PCR with FLC2/RLC2 primers amplified a fragment of 230 bp and the detection limit was 0.2 fg of L. infantum DNA. Of the parasite species assayed, only L. infantum DNA was amplified. After sequencing, the fragment was aligned to GenBank sequences, and showed (99%) homology with L. infantum. In the analysis of blood samples and lesion biopsy from a dog clinically suspected to have VL, the PCR detected DNA from L. infantum. In biopsy lesions from humans and dogs with cutaneous leishmaniasis, the PCR was negative. The PCR with FLC2/RLC2 primers showed high sensitivity and specificity, and constitutes a promising technique for the diagnosis of VL.

AMERICAN CUTANEOUS LEISHMANIASIS WITH UNUSUAL CLINICAL PRESENTATION AND RESPONSE TO TREATMENT

The clinical manifestations and prognosis of cutaneous leishmaniasis (CL) can be influenced by the immune response of the patient and the species of the parasite. A case of atypical clinical presentation of CL, with development of non-characteristic lesions, poor response to therapy, and a long time to resolution is reported. Confirmatory laboratory tests included parasite detection, indirect immunofluorescence, Montenegro skin test, polymerase chain reaction, and parasite identification by multilocus enzyme electrophoresis. The parasite was identified as Leishmaniabraziliensis. The lesion was unresponsive to three complete courses of N-methylglucamine antimoniate intramuscular, and to treatment with pentamidine. The patient did not tolerate amphotericin B. The lesion finally receded after treatment with intravenous N-methylglucamine antimoniate. It is essential to ensure the accuracy of diagnosis and the appropriate treatment, which can include the use a second choice drug or a different route of administration.

Torpedo Maculopathy with an Anisometropic Amblyopia in a 5-Year-Old Caucasian Girl: Case Report

The aim of this study is to report a clinical case of asymptomatic female Caucasian children with torpedo maculopathy. A 5-year-old girl was referred to our clinic for routine evaluation. The ophthalmic examination revealed best-corrected visual acuity of 20/20 in both eyes, without any changes in the biomicroscopy. Fundus examination showed normal findings in one eye, whereas in the contralateral eye it disclosed, in the temporal sector of the macular region, a whitish, atrophic, oval chorioretinal lesion with clearly defined margins. Posterior evaluations documented the stability of the lesion. Torpedo maculopathy diagnosis is based on its characteristic shape and peculiar location. The differential diagnosis has to be established versus choroidal lesions (melanoma and nevus), congenital or iatrogenic hyperplasia of the retinal pigment epithelium (RPE) and particularly versus the congenital pigmented lesions associated with Gardner's syndrome.

Medullary Schistosomiasis

BACKGROUND: Schistosomal infestation of the central nervous system is a rare cause of cord compression, although a predominant one in endemic areas. CASE DESCRIPTION: A 38-year-old male, native of Ivory Coast, with a history of 1 month of progressive paraparesis, neurogenic bladder, diminished deep tendon reflexes of the lower limbs, and sensory level. The magnetic resonance imaging (MRI) showed a medullary lesion at D4-D5 level, suggestive of an intramedullary tumor. Laminotomy of D3 to D5 and excision of a grayish white lesion according to a preliminary histopathologic review suggestive of a high grade glioma. Definitive histopathology review established the diagnosis of medullary schistosomiasis. CONCLUSION: Schistosomal myeloradiculopathy should be considered in patients presenting with cord compression or features of transverse myelitis, especially in patients from endemic areas or low social economic settlements.

Efecto de la temperatura de la piel en la leishmaniasis cutanea experimental

The literature on the thermosensitive properties of strains or species of Leishmania and of other miercorganisms is revised. Cutaneous or mucocutaneous strains that infect animais in the coldest areas of the skin or mucosa in general can not grow in tissue culture at 37°C or higher temperatures and their respiratory metabolism decreases at these temperatures. These facts suggest a thermosensitive event in some important metabolism phase of the organisme. The strains or species that are able to produce visceral leishmaniasis were probably originated from cutaneous strains after genetioally determined physiological adaptation, to warmer temperatures. These strains can not only visceralize in animais and man but will also grow in tissue culture at 36-37°C and the respiratory metabolism will be higher at such temperatures. There are reasons to believe that intermediate strains, i. e., with properties of both groupsí do exist. A thermosensitive physiological event is a more general phenomenon and examples of it can also be found in the fields of virology, bacteriology and mycology. It has practical applications since some of the diseases produced by these agents can be cured by treatments with heat or artificial fever. Experiments along these line were performed on hamsters with a Costa Rican strain of L. braziliensis as an experimental model. Even after intraperitoneal inoculation lesions appear in the nose, ears, paws and tail with a subcutaneous temperature bellow 33°C at 22-24°C. Healing of the lesión is accomplished by increasing room temperature. A good lesión is produced in the rump of the animal if the area is depilated (comercial cream depilatory) previously and the naked skin cooled artificially. Elevated temperature, or the growing back of the hair will tend to diminish or cure the lesion.

Tumors of the Brachial Plexus in a Tertiary Referral Center: a Case Series and Literature Review

Introduction: Brachial plexus (BP) tumors are very rare tumors, with less than 800 cases been described in the literature worldwide since 1970. These tumors often present as local or radicular pain, with scant or no neurological deficits. These symptoms are shared by many other more common rheumatologic diseases, thus making their diagnosis difficult in most cases. Additionally, these tumors often present as lumps and are therefore biopsied, which carries a significant risk of iatrogenic nerve injury. Material and Methods: In this paper the authors describe their experience with the management of 5 patients with BP tumors followed up for at least 2 years. There were 4 males and 1 female. Median follow-up time was 41 ± 21 months. Average age at diagnosis was 40,0 ± 19,9 years. The most common complaints at presentation were pain and sensibility changes. All patients had a positive Tinel sign when the lesion was percussed. In all patients surgery was undertaken and the tumors removed. In 4 patients nerve integrity was maintained. In one patient with excruciating pain a segment of the nerve had to be excised and the nerve defect was bridged with sural nerve grafts. Results: Pathology examination of the resected specimens revealed a Schwannoma in 4 cases and a neurofibroma in the patient submitted to segmental nerve resection. Two years postoperatively, no recurrences were observed. All patients revealed clinical improvement. The patient submitted to nerve resection had improvement in pain, but presented diminished strength and sensibility in the involved nerve territory. Conclusion: Surgical excision of BP tumors is not a risk free procedure. Most authors suggest surgery if the lesion is symptomatic or progressing in size. If the tumor is stationary and not associated with neurological dysfunction a conservative approach should be taken.

Atheroembolic Renal Disease As a Cause of Allograft Primary Non-Function

Atheroembolic renal disease, also referred to as cholesterol crystal embolization, is a rare cause of renal failure, secondary to occlusion of renal arteries, renal arterioles and glomerular capillaries with cholesterol crystals, originating from atheromatous plaques of the aorta and other major arteries. This disease can occur very rarely in kidney allografts in an early or a late clinical form. Renal biopsy seems to be a reliable diagnostic test and cholesterol clefts are the pathognomonic finding. However, the renal biopsy has some limitations as the typical lesion is focal and can be easily missed in a biopsy fragment. The clinical course of these patients varies from complete recovery of the renal function to permanent graft loss. Statins, acetylsalicyclic acid, and corticosteroids have been used to improve the prognosis. We report a case of primary allograft dysfunction caused by an early and massive atheroembolic renal disease. Distinctive histology is presented in several consecutive biopsies. We evaluated all the cases of our Unit and briefly reviewed the literature. Atheroembolic renal disease is a rare cause of allograft primary non -function but may become more prevalent as acceptance of aged donors and recipients for transplantation has become more frequent.

Warm Antibody Autoimmune Haemolytic Anaemia Associated with Ovarian Teratoma

The ovarian cystic teratoma is a rare cause of autoimmune haemolytic anaemia by warm antibodies, resistant to corticotherapy, with few case reports published in the medical literature. We present a case of a 45-year-old woman admitted to hospital due to general weakness. Laboratory studies revealed macrocytic anaemia, biochemical parameters of haemolysis and peripheral spherocytosis. The direct Coombs test was positive. Viral serologies, anti-nuclear antibodies, anti-double-stranded DNA antibodies and β2-microglobulin were negative. CT scan of the thorax, abdomen and pelvis showed a heterogeneous right anexial lesion. The patient was treated with corticotherapy without improvement of anaemia. Regression of extra-vascular haemolysis and normalisation of haemoglobin was obtained only after laparoscopic splenectomy and right ooforectomy, and the histopathology of the right anexial mass revealed a cystic teratoma. Previously published cases controlled the haemolysis by surgically removing the lesion associated with splenectomy.

Melanoma and Satellite Blue Papule

The colors that are seen in dermoscopy depend on the anatomic level of the skin at which the chromophores are seen. Blue color can be found in a variety of melanocytic and nonmelanocytic lesions. An 89-year-old man presented with a 3-year history of a slow-growing, hyperpigmented patch located on the distal third of the right arm. Dermoscopy showed an atypical network, irregularly distributed globules, pigmented internal streaks and a milky-red area. Based on these findings a diagnosis of slow-growing malignant melanoma was made. Simultaneously, a well-defined blue papule was seen on the proximal third of the same arm. Dermoscopy disclosed a homogeneous blue pattern. After clinical and dermoscopic correlation our differential diagnosis for this blue lesion included cutaneous melanoma metastasis, blue nevus and foreign body reaction. The patient recalled its onset 75 years ago after a grenade explosion. We also discuss the blue lesion appearance under reflectance confocal microscopy and high-definition optical coherence tomography. Histopathological examination after excision of the hyperpigmented patch and blue papule revealed a melanoma in situ and a foreign body reaction, respectively. The diagnostic evaluation of a blue lesion should always rely on the integration of all data, especially clinical and dermoscopic features. Other non-invasive techniques, like reflectance confocal microscopy and high-definition optical coherence tomography can also be important aids for its differential diagnosis.

Intramural Hematoma of the Esophagus After Thrombolysis for Ischemic Stroke

Intramural dissecting hematoma is an unusual esophageal condition with a threatening presentation but excellent prognosis when managed conservatively.We report the case of an 88-year-old woman who developed an intramural hematoma of the esophagus after intravenous thrombolysis for an acute ischemic stroke. Before thrombolysis, nasogastric intubation was attempted unsuccessfully. She was kept on nil by mouth, intravenous hydration, proton pump inhibitor, antiemetics,and an antibiotic initiated 2 days before for periodontal disease. The esophageal hematoma regressed, and she resumed oral diet asymptomatically.To our knowledge, this is the first report of this type of lesion after thrombolysis for an ischemic stroke. A brief discussion and literature review are presented.

Hepatic Actinomycosis

Actinomycosis is a rare disorder caused by an anaerobic gram-positive bacillus (Actinomyces), predominantly by the Actinomyces israelii species. Only 20% of cases show an abdominal manifestation, the appendix and ileocecal valve being the most frequent locations. Definitive diagnosis is based on microbiological cultures, microscopy or macroscopy examination. Nevertheless, histological examination of the percutaneous biopsy and blood microbiological cultures are rarely positives. Preoperative diagnosis is hampered by the lack of specific clinical and imaging manifestations, which often mimic malignancy. The rate of preoperative diagnosis is less than 10%, however, the outcome is excellent, with a low mortality rate. The authors describe the case of a patient who was diagnosed with primary hepatic actinomycosis only by a histological examination of the surgical specimen of left hepatectomy extended to segments V and VIII, for suspected malignant lesion. This case demonstrates the difficulties in diagnosing hepatic actinomycosis.

Aortic and Tricuspid Endocarditis in Hemodialysis Patient with Systemic and Pulmonary Embolism

This is a case report of a 43-year-old Caucasian male with end-stage renal disease being treated with hemodialysis and infective endocarditis in the aortic and tricuspid valves. The clinical presentation was dominated by neurologic impairment with cerebral embolism and hemorrhagic components. A thoracoabdominal computerized tomography scan revealed septic pulmonary embolus. The patient underwent empirical antibiotherapy with ceftriaxone, gentamicin and vancomycin, and the therapy was changed to flucloxacilin and gentamicin after the isolation of S. aureus in blood cultures. The multidisciplinary team determined that the patient should undergo valve replacement after the stabilization of the intracranial hemorrhage; however, on the 8th day of hospitalization, the patient entered cardiac arrest due to a massive septic pulmonary embolism and died. Despite the risk of aggravation of the hemorrhagic cerebral lesion, early surgical intervention should be considered in high-risk patients.