Eosinophilic cellulitis (Wells' syndrome) in a female pediatric patient. / Celulitis eosinofílica (síndrome de Wells) en una paciente en edad pediátrica.

Wells' syndrome, or eosinophilic cellulitis, is an inflammatory disease of unknown origin, uncommon in the pediatric age. It usually appears clinically as erythematous and edematous plaques, nodules, papules, blisters, among other symptoms. Here we describe the case of a female pediatric patient with generalized, asymptomatic subcutaneous nodules associated with severe eosinophilia. The histopathological examination of the lesions was compatible with Wells' syndrome. An interdisciplinary evaluation was performed to establish the cause and look for associated eosinophilic disorders; the results were negative. Systemic corticosteroids were indicated and the patient had a good response; however, in view of the recurrence of the lesions after treatment discontinuation, dapsone was indicated as a second-line treatment, with subsequent improvement of the lesions and eosinophilia. The aim of this report was to describe the case of a female patient with an atypical manifestation of Wells' syndrome and the resulting therapeutic challenge.

El síndrome de Wells o celulitis eosinofílica es una enfermedad inflamatoria de origen desconocido, de aparición infrecuente en la edad pediátrica. Suele manifestarse clínicamente como placas eritematoedematosas, nódulos, pápulas, ampollas, entre otros. Se presenta una paciente en edad pediátrica con nódulos subcutáneos asintomáticos generalizados asociados a eosinofilia grave. El estudio histopatológico de las lesiones fue compatible con celulitis de Wells. Se realizó una evaluación interdisciplinaria en busca de la causa y trastornos eosinofílicos asociados, sin resultados positivos. Se indicó tratamiento sistémico con corticoides y presentó buena respuesta, pero, ante la recidiva de las lesiones tras su suspensión, se indicó dapsona como tratamiento de segunda línea, con mejoría posterior de las lesiones y de la eosinofilia. El objetivo del reporte es presentar una paciente con una manifestación atípica de síndrome de Wells y su desafío terapéutico.

Generalized Lymphatic Anomaly as a Differential Diagnosis of Lytic Lesions.

Generalized lymphatic anomaly (GLA) is an infrequent multiorgan disease characterized by the presence of abnormal proliferation of lymphatic vessels. The diagnosis requires histological confirmation, and the treatment is controversial. We are presenting a case of a 28-year-old male patient who was diagnosed with an extragonadal mediastinal nonseminomatous germ cell tumor. He underwent chemotherapy, and during this treatment, radiologic findings evidenced lytic lesions. Multiple biopsies were performed, which revealed the presence of abnormal lymphatic vessels, characteristic of GLA. There are different etiologies of osteolytic lesions, and on some occasions, they mimic a tumoral entity. The clinical suspicion of GLA is the first step in approaching the diagnosis, particularly in young adult patients.

Tumor de células yuxtaglomerulares (reninoma) como causa de hipertensión arterial en la adolescencia. A propósito de un caso / Juxtaglomerular cell tumor (reninoma) as a cause of arterial hypertension in adolescents. A case report

La hipertensión arterial (HTA) grave en pediatría responde fundamentalmente a causas secundarias. Presentamos una paciente adolescente de 14 años con HTA grave, alcalosis metabólica e hipopotasemia, secundaria a un tumor de células yuxtaglomerulares productor de renina, diagnosticado luego de dos años de evolución de HTA.

Severe arterial hypertension (HTN) in pediatrics is mainly due to secondary causes. Here we describe the case of a 14-year-old female adolescent with severe HTN, metabolic alkalosis, and hypokalemia, secondary to a renin-secreting juxtaglomerular cell tumor diagnosed after 2 years of HTN progression.

Juxtaglomerular cell tumor (reninoma) as a cause of arterial hypertension in adolescents. A case report. / Tumor de células yuxtaglomerulares (reninoma) como causa de hipertensión arterial en la adolescencia. A propósito de un caso.

Severe arterial hypertension (HTN) in pediatrics is mainly due to secondary causes. Here we describe the case of a 14-year-old female adolescent with severe HTN, metabolic alkalosis, and hypokalemia, secondary to a renin-secreting juxtaglomerular cell tumor diagnosed after 2 years of HTN progression.

La hipertensión arterial (HTA) grave en pediatría responde fundamentalmente a causas secundarias. Presentamos una paciente adolescente de 14 años con HTA grave, alcalosis metabólica e hipopotasemia, secundaria a un tumor de células yuxtaglomerulares productor de renina, diagnosticado luego de dos años de evolución de HTA.

Influence of personality profile in patients with drug-resistant epilepsy on quality of life following surgical treatment: A 1-year follow-up study.

The objectives of this study are to determine the influence of personality profile in patients with drug-resistant epilepsy on quality of life (QoL) after surgical treatment and compare the results with a non-surgical control group at the 1-year follow-up. We conducted a prospective, comparative, controlled study, including 70 patients suffering from drug-resistant epilepsy. Demographic, psychiatric, neurological, and psychological data were recorded at the baseline and at the 1-year follow-up. Assessment of personality dimensions was performed using the NEO-FFI-R questionnaire; severity of anxiety and depression were assessed by the Hospital Anxiety and Depression Scale (HADS), and QoL was evaluated using the QOLIE-31. At the 1-year follow-up, comparing the control and the surgical groups, we detected differences in scores of most items of QoL, which were higher in those patients who had undergone surgery. High levels of Conscientiousness and Openness to experience at the baseline in patients who underwent surgery predicted better post-surgical outcomes in the QoL scores, whereas high neurotic patients showed worse QoL results. Postoperative changes in QoL in patients were associated with the personality profile at the baseline. QoL measures significantly improved in the surgical group compared with the non-surgical group but were not associated with baseline or postoperative seizure frequency at 1 year.

Long-range action of an HDAC inhibitor treats chronic pain in a spared nerve injury rat model.

Histone deacetylase inhibitors (HDACi) that modulate epigenetic regulation and are approved for treating rare cancers have, in disease models, also been shown to mitigate neurological conditions, including chronic pain. They are of interest as non-opioid treatments, but achieving long-term efficacy with limited dosing has remained elusive. Here we utilize a triple combination formulation (TCF) comprised of a pan-HDACi vorinostat (Vo at its FDA-approved daily dose of 50mg/Kg), the caging agent 2-hydroxypropyl-ß-cyclodextrin (HPBCD) and polyethylene glycol (PEG) known to boost plasma and brain exposure and efficacy of Vo in mice and rats, of various ages, spared nerve injury (SNI) model of chronic neuropathic pain. Administration of the TCF (but not HPBCD and PEG) decreased mechanical allodynia for 4 weeks without antagonizing weight, anxiety, or mobility. This was achieved at less than 1% of the total dose of Vo approved for 4 weeks of tumor treatment and associated with decreased levels of major inflammatory markers and microglia in ipsilateral (but not contralateral) spinal cord regions. A single TCF injection was sufficient for 3-4 weeks of efficacy: this was mirrored in repeat injections, specific for the injured paw and not seen on sham treatment. Pharmacodynamics in an SNI mouse model suggested pain relief was sustained for days to weeks after Vo elimination. Doubling Vo in a single TCF injection proved effectiveness was limited to male rats, where the response amplitude tripled and remained effective for > 2 months, an efficacy that outperforms all currently available chronic pain pharmacotherapies. Together, these data suggest that through pharmacological modulation of Vo, the TCF enables single-dose effectiveness with extended action, reduces long-term HDACi dosage, and presents excellent potential to develop as a non-opioid treatment option for chronic pain.

Basaloid follicular hamartoma associated with follicular mucinosis and inflammation

Abstract Basaloid follicular hamartoma is a benign, superficial malformation of hair follicles that can be mistaken both clinical and histopathologically for basal cell carcinoma. Basaloid follicular hamartoma has been linked to a mutation in the PTCH-1 gene, which is part of the same pathway involved in Gorlin-Goltz syndrome. Here we present a 9-year-old patient with an asymptomatic congenital lesion on the forehead, which increased in size over the years. Histopathology showed a basaloid follicular hamartoma associated with follicular mucinosis and inflammation. Gorlin-Goltz syndrome was ruled out by clinical examination.

Basaloid follicular hamartoma associated with follicular mucinosis and inflammation.

Basaloid follicular hamartoma is a benign, superficial malformation of hair follicles that can be mistaken both clinical and histopathologically for basal cell carcinoma. Basaloid follicular hamartoma has been linked to a mutation in the PTCH-1 gene, which is part of the same pathway involved in Gorlin-Goltz syndrome. Here we present a 9-year-old patient with an asymptomatic congenital lesion on the forehead, which increased in size over the years. Histopathology showed a basaloid follicular hamartoma associated with follicular mucinosis and inflammation. Gorlin-Goltz syndrome was ruled out by clinical examination.

Personality changes in patients suffering from drug-resistant epilepsy after surgical treatment: a 1-year follow-up study.

OBJECTIVE: To determine changes in dimensions of personality in a sample of patients suffering from drug-resistant epilepsy at the 1-year follow-up following surgery, compared to non-surgically treated controls. METHODS: We conducted a prospective comparative controlled study, including drug-resistant epilepsy surgery candidates. Demographic, psychiatric, neurological, and psychological data were recorded. Presurgical and 12-months follow-up evaluations were performed. Personality dimensions were measured by the NEO Five-Factor Inventory, Revised version (NEO-FFI-R), anxiety and depression symptoms were assessed by the Hospital Anxiety and Depression Scale (HADS: HADA-Anxiety and HADD-Depression), psychiatric evaluations were performed using the Diagnostic and Statistical Manual of Mental Disorders, fourth edition (DSM-IV) Axis-I disorders classification. Statistical analysis consisted of comparative tests, correlation analysis, and the stepwise multiple regression test (ANOVA). RESULTS: A 1-year follow-up was completed by 70 out of 80 patients. Through the study, the surgical group decreased in neuroticism and increased in agreeableness. The controls increased in consciousness, and these changes were predicted by the earlier age of epilepsy onset and lesser score in HADD at the baseline. No personality changes were associated with seizure frequency. The presurgical evaluation concluded that both groups had no differences in demographic, psychiatric, or neurological variables with the only exception being for the number of seizures per month, which was higher in the surgical group. Psychiatric comorbidity in patients was associated with their higher degree of neuroticism and agreeableness at the baseline. Comparing control and surgical groups at the one-year follow-up, the agreeableness personality variable was higher in the surgical group, and as expected, HADS scores were higher in the control group, and seizure frequency was also higher in the control group. SIGNIFICANCE: Higher agreeableness was the most relevant difference in personality dimensions in patients who underwent surgical treatment compared with the non-surgical treatment group. After surgery patients decreased in neuroticism and increased in agreeableness scores.

Mapping Epileptic Networks Using Simultaneous Intracranial EEG-fMRI.

Background: Potentially curative epilepsy surgery can be offered if a single, discrete epileptogenic zone (EZ) can be identified. For individuals in whom there is no clear concordance between clinical localization, scalp EEG, and imaging data, intracranial EEG (icEEG) may be needed to confirm a predefined hypothesis regarding irritative zone (IZ), seizure onset zone (SOZ), and EZ prior to surgery. However, icEEG has limited spatial sampling and may fail to reveal the full extent of epileptogenic network if predefined hypothesis is not correct. Simultaneous icEEG-fMRI has been safely acquired in humans and allows exploration of neuronal activity at the whole-brain level related to interictal epileptiform discharges (IED) captured intracranially. Methods: We report icEEG-fMRI in eight patients with refractory focal epilepsy who had resective surgery and good postsurgical outcome. Surgical resection volume in seizure-free patients post-surgically reflects confirmed identification of the EZ. IEDs on icEEG were classified according to their topographic distribution and localization (Focal, Regional, Widespread, and Non-contiguous). We also divided IEDs by their location within the surgical resection volume [primary IZ (IZ1) IED] or outside [secondary IZ (IZ2) IED]. The distribution of fMRI blood oxygen level-dependent (BOLD) changes associated with individual IED classes were assessed over the whole brain using a general linear model. The concordance of resulting BOLD map was evaluated by comparing localization of BOLD clusters with surgical resection volume. Additionally, we compared the concordance of BOLD maps and presence of BOLD clusters in remote brain areas: precuneus, cuneus, cingulate, medial frontal, and thalamus for different IED classes. Results: A total of 38 different topographic IED classes were identified across the 8 patients: Focal (22) and non-focal (16, Regional = 9, Widespread = 2, Non-contiguous = 5). Twenty-nine IEDs originated from IZ1 and 9 from IZ2. All IED classes were associated with BOLD changes. BOLD maps were concordant with the surgical resection volume for 27/38 (71%) IED classes, showing statistical global maximum BOLD cluster or another cluster in the surgical resection volume. The concordance of BOLD maps with surgical resection volume was greater (p < 0.05) for non-focal (87.5%, 14/16) as compared to Focal (59%, 13/22) IED classes. Additionally, BOLD clusters in remote cortical and deep brain areas were present in 84% (32/38) of BOLD maps, more commonly (15/16; 93%) for non-focal IED-related BOLD maps. Conclusions: Simultaneous icEEG-fMRI can reveal BOLD changes at the whole-brain level for a wide range of IEDs on icEEG. BOLD clusters within surgical resection volume and remote brain areas were more commonly seen for non-focal IED classes, suggesting that a wider hemodynamic network is at play.

Dolor abdominal y parálisis flácida, una aproximación a las porfirias: serie de casos y revisión de la literatura / Flaccid paralysis and abdominal pain, an approach to porphyrias: a literature review and case series

RESUMEN Las porfirias son trastornos metabólicos hereditarios causados por deficiencias enzimáticas de la biosíntesis del grupo HEM. Con presentación en distintos grupos de edades, más común en infancia y tercera a cuarta década de la vida, se caracterizan por elevación de porfirinas, y manifestaciones variadas cutáneas y neuro viscerales. Describimos una serie de 3 casos de pacientes femeninas en tercera década de la vida con dolor abdominal severo e inespecífico y una amplia gama de manifestaciones clínicas con sus complicaciones a corto y largo plazo en quienes se diagnosticó porfiria aguda intermitente (PAI). Se hará revisión en la literatura para aportar al reconocimiento temprano de estas condiciones e instaurar de forma temprana el manejo específico e impactar en desenlaces irreversibles.

ABSTRACT Porphyrias are inherited metabolic disorders caused by enzymatic deficiencies of HEM group biosynthesis. Most common in childhood at the third and fourth decade of life. They are characterized by increased levels of porphyrins, and various cutaneous, neurological, and visceral manifestations. We describe a series of 3 cases of female patients in the third decade of life with abdominal pain and a wide range of clinical manifestations and short and long-term complications. Our review contributes to the early recognition of these diseases to establish early specific managements to impact on irreversible outcomes.

Liquen plano, estudio retrospectivo en un hospital pediátrico / Lichen planus, retrospective study in a pediatric hospital

Introducción: El liquen plano (LP) es una enfermedad inflamatoria crónica, de etiología desconocida, que puede afectar la piel, los anexos y las mucosas. Objetivo: Describir las características epidemiológicas y clínicas y el tratamiento realizado en los pacientes con diagnóstico clínico e histopatológico de LP, valorados en el Servicio de Dermatología del Hospital de Pediatría "Prof. Dr. Juan P. Garrahan" en los últimos 33 años. Diseño:Estudio descriptivo, retrospectivo y transversal. Materiales y métodos: Se revisaron las historias clínicas de los pacientes con diagnóstico histopatológico de LP atendidos en el Hospital desde agosto de 1987 hasta febrero de 2020 y se analizaron las variables epidemiológicas, clínicas y de tratamiento. Resultados: Se incluyeron datos de 26 pacientes, 18 de los cuales eran varones (69,3%). La media de edad en el momento del diagnóstico fue de 9,6 años. Presentaron la variedad de LP clásico 24 pacienes (93%). El compromiso ungueal y de la mucosa bucal se observó en 2 pacientes (7,7%). Ninguno tuvo asociación a fármacos ni a infecciones. En cuanto al tratamiento recibido, 4 pacientes (15,4%) tuvieron pérdida del seguimiento; 13 (50%) realizaron tratamiento tópico; 6 (23%), tratamiento sistémico y 3 (11,5%), tratamiento combinado. El promedio de seguimiento fue de 6 meses y se obtuvo una respuesta favorable en el 68% de los pacientes. Conclusiones: El LP es una patología de baja frecuencia en la infancia. No se encontró asociación a medicamentos ni a enfermedades infecciosas como se describe en la población adulta. Su curso es crónico y, en los pacientes que tuvieron un adecuado seguimiento, se obtuvo una buena respuesta al tratamiento indicado.

Introduction: Lichen planus (LP) is a chronic, inflammatory disease of unknown etiology, which can affect skin, adnexal tissues and mucosa. Objective: to describe the clinical, epidemiological characteristics and the treatment received by patients with clinical and histopathological diagnosis of LP, seen at the Dermatology Service of Pediatric Hospital "Prof. Dr. Juan P. Garrahan" in the last 33 years. Design: Descriptive, retrospective and cross-sectional study. Materials y methods: We reviewed the clinical history of patients with histopathological diagnosis of LP that were treated at our hospital from August 1987 to February 2020 and analysed the epidemiological, clinical and treatment variables. Results: Data of 26 patients were included in this study 18 of which are males (69.3%). The mean age of diagnosis was 9.6 years. 24 patients (93%) presented the classical type of LP. Ungual and mucosal compromise were observed in 2 patients (7.7%) and none had associations with infections or drugs. Regarding treatment, 4 patients (15.4%) did not continue follow-ups, 13 patients (50%) had topical treatment, 6 (23%) had systemic treatment and 3 patients (11.5%) received combined treatment. The average follow-up time was 6 months and 68% of patients had favorable outcome. Conclusions: LP is an uncommon pathology in children. During this study we did not find any etiologic relations with use of drugs or infectious diseases as is described in de adult population. Its course is chronic and in the patients that had a proper follow-up a good response to the indicated treatment was obtained.

Psychotic symptoms in drug resistant epilepsy patients after cortical stimulation.

PURPOSE: The use of invasive EEG (iEEG) recordings before epilepsy surgery has increased as more complex focal epilepsies are evaluated. Psychotic symptoms (PS) during iEEG have been scarcely reviewed. We aim to report our series of patients with psychotic symptoms (PS) brought about by cortical stimulation (CS) and to identify triggers. METHODS: Retrospective cohort of patients who underwent iEEG and CS. We report patients who developed delusional thinking and/or disorganized behaviour within 24 h after CS. Exclusion criteria were primary psychiatric disorders or absence of CS. RESULTS: We evaluated 32 (SEEG 23; subdural 9) patients with a median age of 38 years, 6 with PS. Patients underwent 2586 stimulations over 1130 contacts. Age at CS was significantly higher in patients with PS. Temporal lobe epilepsy was significantly more often documented in patients with PS (χ2: 3.94; p< 0.05). We found no correlation between stimulation of the limbic system and development of psychosis. Four (66.7 %) patients were stimulated in the non-dominant limbic system and developed psychosis compared to 7 (27 %) who did not [χ2: 3.41; p= 0.06].Epilepsy duration was significantly higher in PS patients (p=0.002). Patients with history of postictal psychosis were twice more likely to experience PS(p=0.04). CONCLUSIONS: PS may arise more frequently in patients with PIP history, older age and longer epilepsy duration. The neurobiology and physiology of psychosis, that may share common mechanisms with epilepsy, is yet to be identified but we hypothesize that it may be triggered by CS due to alteration of brain networks dynamics.

Psychiatric disorders in patients with resistant temporal lobe epilepsy two years after undergoing elective surgery. A longitudinal study.

PURPOSE: Psychiatric morbidity in temporal lobe epilepsy (TLE) is frequent and negatively affects patients' life quality. Surgery is the procedure of choice when treating seizures, although the effects on psychiatric disorders remain unclear. We evaluate the effect of surgery on psychiatric disorders in patients with TLE two years after the intervention, to then shed light on how these are related to anxiety and depression symptoms, and Interictal Dysphoric Disorder (IDD). METHODS: We included data from 65 patients with TLE whose psychiatric evaluations were performed according to DSM-IV criteria. Anxiety and depression symptoms were assessed using the Hospital Anxiety and Depression Scale (HADS) test. RESULTS: At 2-year follow-up, anxiety and depressive disorders decreased, and psychotic disorders augmented without statistical significance. Baseline psychiatric disorders predisposed to psychiatric pathology at 2-year follow-up and did not correlate with epilepsy outcome after surgery. Postoperative psychiatric disorders correlated with the seizure incidence two years after the intervention, suggesting that epilepsy and psychiatric disorders were associated in processes such as surgery. De novo psychiatric disorders represented 52% of postoperative psychiatric pathology, 62% being psychotic disorders. De novo psychiatric disorders became more frequent from the first year of surgery, occurring mainly in patients free of seizures. The HADS test scores and IDD correlated with psychiatric disorders at 2-year follow-up. CONCLUSIONS: Baseline psychiatric disorders did not influence surgery outcome, but correlated with psychiatric disorders' prevalence two years after surgery. Despite not finding statistical significance, surgery reduced the prevalence of psychiatric disorders, and de novo psychiatric disorders were associated with an improvement in the epilepsy course at 2-year follow-up.

[The impact of COVID-19 on surgical waiting lists.] / Impacto de la COVID-19 sobre las listas de espera quirúrgicas.

OBJECTIVE: In Spain, the number of persons that are in a surgery waiting list as well as the available surgery resources, differ across autonomous communities. The pandemic generated by COVID-19 has increased these waiting lists. In this study two objectives were pursued: on the one hand, to determine which are the resources that are determining the number of persons that are in a surgery waiting list per 1,000 inhabitants; on the other hand, to estimate the impact that the current pandemic has on the latter. METHODS: To estimate which are the resources that are having a greater impact on the waiting lists and to forecast the effect that the COVID-19 has on them, we use dynamic panel data models. The data on the surgery resources and on the waiting lists by autonomous communities is obtained from the Surveys on Health, Hospital Statistics and reports on waiting lists of the Ministry of Health, Consumption and Social Well Being and the Counsels. The sample period is 2012-2017 (last published year for surgery resources). In addition, a literature review is conducted and it shows the important and complexity of waiting list like a gestion tool of health system (Science, SciELO and Dialnet web data bases). RESULTS: COVID-19 will increase the waiting lists by approximately 7.6% to 19.14%, depending on the autonomous community. Not all the available surgery resources have the same relevance nor an equal effect on the reduction of the waiting lists. The most significant resources are the beds and operating rooms per 1,000 inhabitants. The hospital expenditure is not so relevant. CONCLUSIONS: The panel data models estimate the relation between the surgery resources and the waiting list. The latter is deemed complex and different across autonomous communities. In addition, these models allow to predict the expected increase in the waiting lists and are, thus, a useful instrument for their management.

OBJETIVO: En España, tanto el número de personas que están en espera de intervención quirúrgica, como los recursos quirúrgicos disponibles difieren entre comunidades autónomas. La pandemia ocasionada por la COVID-19 ha incrementado estas listas de espera. En este trabajo se persiguió un doble objetivo, por un lado, determinar cuáles son los recursos que tiene mayor influencia en el número de personas por mil habitantes que se encuentran en espera quirúrgica y, por otro, estimar el impacto que la pandemia actual ha generado. METODOS: Para estimar cuales son los recursos que más están repercutiendo en las listas de espera y predecir el efecto que la COVID-19 ejerce en ellas, utilizamos un modelo de datos de panel dinámico. Los datos de recursos quirúrgicos y listas de espera por comunidades autónomas se han obtenido de Encuestas de Salud, Estadísticas Hospitalarias e informes de listas de espera del Ministerio de Sanidad Consumo y Bienestar Social y de las Consejerías. El periodo muestral es 2012-2017 (último año publicado para recursos quirúrgicos). Además, se llevó a cabo una revisión de la literatura que permite corroborar la importancia y complejidad de las listas de espera como instrumento de gestión del sistema sanitario (bases de datos Web of Science, SciELO y Dialnet). RESULTADOS: La COVID-19 incrementará las listas de espera, aproximadamente entre el 7,6% y el 19,4%, dependiendo de la comunidad. No todos los recursos quirúrgicos disponibles tienen la misma importancia ni influyen de la misma manera en la reducción de las personas en espera. Los recursos más significativos son las camas y quirófanos por mil habitantes y no tanto el gasto en hospitales del Sistema Nacional de Salud. CONCLUSIONES: Los modelos de datos de panel permiten conocer la relación entre recursos quirúrgicos y lista de espera, la cual parece ser compleja y diferente entre comunidades. Además, estos modelos ayudan a predecir el incremento esperable y, por lo tanto, son un instrumento útil en la gestión de listas de espera.

The nature, frequency and value of stimulation induced seizures during extraoperative cortical stimulation for functional mapping.

PURPOSE: The aim of this retrospective service evaluation was to determine the nature, frequency and clinical value of seizure occurrence during extraoperative direct cortical stimulation for functional mapping in patients undergoing invasive recordings (icEEG) for epilepsy surgery workup. METHODS: We reviewed 145 sequential cases of patients with refractory focal epilepsy who underwent intracranial electrode implantation and extraoperative direct cortical stimulation (CS) for functional mapping. CS intended for mapping can elicit as a by-product electrical or electroclinical events, such as afterdischarges, subclinical EEG seizures, and stimulation-induced seizures (SIS). SIS may have habitual or non-habitual semiology (as defined by comparison to the patient's spontaneous events). RESULTS: In our cohort, electrical (subclinical EEG seizures) or electroclinical events, (SIS) were recorded in 34.5% (50/145) patients during CS. SIS occurred in 23.4% (34/145) of all patients, of which over half were habitual SIS (SIShab). In most cases the location of contacts eliciting habitual SIS originated from the same location as the spontaneous ictal onset zone in icEEG. Of those with SIS hab undergoing surgery (n = 13), seizure freedom was achieved in 61.5%, and of those with SISNH undergoing surgery (n = 10), 40% became seizure free (ns). CONCLUSIONS: Electroclinical SIS occur in about a quarter of CS for functional mapping; SIS are habitual in the majority of cases, and where elicited, SIS in icEEG could be an additional diagnostic tool to localize the seizure onset zone. However, a significant minority of stimulations lead to non-habitual SIS.

Caspr2 antibody-associated limbic encephalitis: contribution of visual aided analysis of 18F-FDG PET images using normal database comparison. / Encefalitis límbica asociada a anti-Caspr2: contribución del análisis visual ayudado de las imágenes PET con 18F-FDG mediante comparación con una base de datos de normalidad.

Early immunotherapy is of paramount importance for a positive outcome in patients suffering acute encephalitis of autoimmune origin (AIE). A new approach for early diagnosis based on clinical presentation and complementary tests has been proposed, but not all these tests show positive findings in the first weeks. While common forms of AIE (anti-LGI-1 and anti-NMDAR antibodies) exhibit consistent 18Fluor-fluorodeoxiglucose (FDG-PET) patterns in many cases, the anti-Caspr2 form of AIE is infrequent and FDG-PET patterns have not been well characterized. In our experience, FDG-PET in anti-Caspr2 limbic encephalitis shows medial temporal hypermetabolism and diffuse cortical hypometabolism, even in the absence of findings in these tests. However, it is necessary to standardize PET image analysis by means of visual and voxel-based methods compared to normal databases to define the areas of pathological metabolism that may go unnoticed when using visual analysis exclusively.

Pilomatrixomas en niños: a propósito de 149 casos. Estudio retrospectivo en dos centros pediátricos / Pilomatrixomas in children: Report of 149 cases. A retrospective study at two children's hospitals

El pilomatrixoma es un tumor benigno cutáneo, de frecuente aparición en los niños, que se origina en las células de la matriz del folículo piloso. Se presenta como un nódulo o tumor, aproximadamente, de 0,5 a 3 cm de tamaño, de consistencia cálcica, de bordes facetados, con piel suprayacente eritematoazulada. En ocasiones, la piel puede atrofiarse, dar la apariencia de una ampolla e, incluso, extruir material cálcico. El diagnóstico se sospecha por la presentación clínica característica y los estudios de imágenes complementarios. El diagnóstico definitivo es por los hallazgos histológicos. La extirpación quirúrgica es el tratamiento de elección. Se presentan los datos epidemiológicos, clínicos, estudios complementarios y técnicas quirúrgicas empleadas de 149 pilomatrixomas resecados en 137 pacientes tratados en los Servicios de Dermatología de los hospitales de pediatría Dr. Pedro de Elizalde y Prof. Dr. Juan P. Garrahan.

A pilomatrixoma is a benign skin tumor common in children, which develops from the matrix cells of hair follicles. It presents as a nodule or tumor of approximately 0.5-3 cm in size, with calcium-like consistency, faceted edges, and blue erythematous overlying skin. Sometimes, the skin may atrophy and look like a blister or even extrude calcium. Diagnosis is suspected based on the typical clinical presentation and supplementary imaging tests. The definite diagnosis is made according to histological findings. Surgical removal is the treatment of choice. Here we describe the epidemiological and clinical data, supplementary tests, and surgical techniques in relation to 149 pilomatrixomas resected in 137 patients treated at the Departments of Dermatology of two children's hospitals: Dr. Pedro de Elizalde and Prof. Dr. Juan P. Garrahan.