ECHOCARDIOGRAPHY, Issue 7 2008
Pierluigi Costanzo M.D.
Background: In idiopathic and ischemic dilated cardiomyopathy (DCM) there are differences in left atrial and ventricular relaxation. We assessed the hypothesis of an influence of these dissimilarities in assessing left ventricular filling pressure (LVFP) in these two DCMs by standard Doppler and tissue Doppler imaging. In particular, we focused on early transmitral flow to early diastolic motion velocity of mitral annulus ratio (E/Ea), useful to estimate normal or elevated LVFP. However, when found in intermediate range (8,15), its role is unclear. Methods and Results: We evaluated 26 patients with ischemic and 21 patients with idiopathic DCM. To validate the echocardiographic estimation of LVFP, a sample (12 patients) underwent LVFP assessment by catheterization. In idiopathic DCM, E/Ea directly related to duration of retrograde pulmonary venous flow (ARd) (r = 0.66 P = 0001). In ischemic DCM E/Ea inversely related only to systolic to diastolic velocity ratio of pulmonary venous flow (S/D) (r =,0.56 P = 0002). After a mean follow up of 6 months, by a second echocardiogram we observed a direct relation between E/Ea and ARd percentage variation (r = 0.52 P = 0.02) in idiopathic DCM group, whereas in the ischemic DCM group there was an inverse relation between E/Ea and S/D percentage variation (r =,0.59 P = 0.02).Conclusions: In conclusion, ARd in idiopathic and S/D in ischemic DCM might be used as specific additional information to estimate LVFP when E/Ea falls within intermediate range. [source]

Long-Term Results of Cardiac Transplantation

JOURNAL OF CARDIAC SURGERY, Issue 3 2003
Alberto Juffe M.D.
From April 1991 to December 2000, 345 patients underwent heart transplantation at the Juan Canalejo Hospital. The mean age of recipients was54.5 ± 11.4 years; 286 (83%) were male patients. Idiopathic (52.2%) and ischemic (34.9%) end-stage cardiomyopathy were the main causes leading to transplantation. Ninety-four patients had undergone a previous heart operation. The mean left ventricular ejection fraction was22.8 ± 11.4. Forty patients (11.5%) were transplanted in urgent (status I) condition. The mean time spent on the waiting list was 35.9 days. In-hospital mortality was 10.6% and 24% for transplantations performed on an elective and urgent basis, respectively. Operative (30-day), one-year and six-year survival was 87.2%, 81.3% and 64%, respectively. In terms of actuarial survival, there were no significant differences with regard to the recipient's age, sex, previous cardiac surgery, and the etiology of the end-stage cardiomyopathy. The six-year actuarial survival for recipients receiving hearts from female donors was 59% compared with 72% for male donors(p = 0.05). There has been a low incidence of rejection, as well as cardiac graft vasculopathy. Actuarial survival at six years was 66% for patients transplantated on an elective basis compared with 57% for patients transplanted on an urgent basis(p = 0.04). The aim of the study was to evaluate long-term results for patients who underwent orthotopic heart transplantation. In our experience, status I is associated with a higher mortality.(J Card Surg 2003;18:183-189) [source]

Idiopathic Left Ventricular Arrhythmias Originating Adjacent to the Left Aortic Sinus of Valsalva: Electrophysiological Rationale for the Surface Electrocardiogram

JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 2 2010
TAKUMI YAMADA M.D.
IVT Arising Adjacent to the Left Sinus of Valsalva.Background: Idiopathic ventricular arrhythmias (VAs) may be amenable to catheter ablation within or adjacent to the left sinus of Valsalva (LSOV). However, features that discriminate these sites have not been defined. The purpose of this study was to determine the electrocardiographic and electrophysiological features of VAs originating within or adjacent to the LSOV. Methods and Results: We studied 48 consecutive patients undergoing successful catheter ablation of idiopathic VAs originating from the left coronary cusp (LCC, n = 29), aortomitral continuity (AMC, n = 10) and great cardiac vein or anterior interventricular cardiac vein (Epi, n = 9). A small r wave, or rarely an R wave, was typically observed in lead I during the VAs and pacing in these regions. An S wave in lead V5 or V6 occurred significantly more often during both the VAs and pacing from the AMC than during that from the LCC and Epi (p < 0.05 to 0.0001). For discriminating whether VA origins can be ablated endocardially or epicardially, the maximum deflection index (MDI = the shortest time to the maximum deflection in any precordial lead/QRS duration) was reliable for VAs arising from the AMC (100%), but was less reliable for LCC (73%) and Epi (67%) VAs. In 3 (33%) of the Epi VAs, the site of an excellent pace map was located transmurally opposite to the successful ablation site (LCC = 1 and AMC = 2). Conclusions: The MDI has limited value for discriminating endocardial from epicardial VA origins in sites adjacent to the LSOV probably due to preferential conduction, intramural VA origins or myocardium in contact with the LCC. (J Cardiovasc Electrophysiol, Vol. 21, pp. 170-176, February 2010) [source]

Idiopathic Right Ventricular Outflow Tract Tachycardia: No Longer Idiopathic?

JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 7 2006
SUNIL SINHA M.D.
No abstract is available for this article. [source]

Idiopathic Left Ventricular Tachycardia Originating from the Mitral Annulus

JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 10 2005
KOJI KUMAGAI M.D.
Background: Radiofrequency catheter ablation (RFCA) can eliminate most idiopathic repetitive monomorphic ventricular tachycardias (RMVTs) originating from the right and left ventricular outflow tracts (RVOT, LVOT). Here, we describe the electrophysiological (EP) findings of a new variant of RMVT originating from the mitral annulus (MAVT). Methods and Results: MAVT was identified in 35 patients out of 72 consecutive left ventricular RMVTs from May 2000 to June 2004. All patients underwent an EP study and RFCA. The sites of origin of the MAVT were grouped into four groups according to the successful ablation sites around the mitral annulus. Group I included the anterior sites (n = 11), group II the anterolateral sites (n = 9), group III the lateral sites (n = 6), and group IV the posterior sites (n = 9). The MAVTs were a wide QRS tachycardia with a delta wave-like beginning of the QRS complex. The transitional zone of the R wave occurred between V1-V2 in all cases. The 12-lead electrocardiogram (ECG) pattern might reflect the site of the origin of MAVTs around the mitral annulus. We proposed an algorithm for predicting the site of the focus and the tactics needed for successful RFCA of the MAVT. Conclusions: We described the EP findings of the new variant of RMVT, MAVT. Most MAVTs could be eliminated by RF applications to the endocardial mitral annulus using our proposed tactics. [source]

Endocardial Noncontact Activation Mapping of Idiopathic Left Ventricular Tachycardia

JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 12 2000
JASBIR SRA M.D.
Mapping of Idiopathic Ventricular Tachycardia. Idiopathic left ventricular tachycardia with a right bundle, left-axis deviation is thought to originate from posterior fascicles. Recently, there has been considerable interest in the anatomic and mechanistic basis of this arrhythmia. We report our experience with a 26-year-old man in whom new noncontact mapping technology was used to acquire detailed data from the left ventricle, identify the mid-diastolic potential and part of the ventricular tachycardia circuit, and perform successful ablation. This information helped define the physiologic aspects of this unique tachycardia. [source]

Steroid-Responsive Meningitis-Arteritis in Dogs with Noninfectious, Nonerosive, Idiopathic, Immune-Mediated Polyarthritis

JOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 3 2002
Aubrey A. Webb
Signs related to spinal pain are commonly reported in dogs with noninfectious, nonerosive, idiopathic immune-mediated polyarthritis (IMPA). This study examined the prevalence and etiology of spinal pain in these dogs through a retrospective review of 62 case records of dogs with IMPA. All dogs with IMPA and signs suggestive of spinal pain were described with regard to age, gender, breed, physical stature, location of vertebral pain, rectal temperature, and clinical laboratory findings. The prevalence of spinal pain in these dogs was 29% (18 of 62). Fourteen of the 18 dogs with spinal pain and IMPA were male. Cerebrospinal fluid (CSF) from 11 dogs with signs of spinal pain was analyzed. Five of these (46%) had concurrent steroid-responsive meningitis-arteritis (SRMA). We concluded that SRMA does occur concurrently in some dogs having IMPA. Meningeal involvement may explain the origin of spinal pain observed in some of these dogs. [source]

Blood loss during posterior spinal fusion surgery in patients with neuromuscular disease: is there an increased risk?

PEDIATRIC ANESTHESIA, Issue 9 2003
Alice Edler MD, MA (EDUC)
Summary Background Scoliosis surgery in paediatric patients can carry significant morbidity associated with intraoperative blood loss and the resultant transfusion therapy. Patients with neuromuscular disease may be at an increased risk for this intraoperative blood loss, but it is unclear if this is because of direct vascular pathophysiological changes or the fact that neuromuscular patients typically have more extensive orthopaedic disease and more vertebral segments involved. This study examined the risk of extensive blood loss (>50% of total blood volume) in patients with neuromuscular disease compared with patients who did not have neuromuscular disease when the extent of the surgery (number of segments fused), age and preoperative coagulation profile where taken into consideration. Methods Retrospective chart review of 163 paediatric patients was preformed. Patients who carried a diagnosis of preexisting neuromuscular disease were classified as such. Idiopathic, traumatic and iatrogenic scoliosis were classified as nonneuromuscular. Extensive blood loss was defined as >50% of estimated total blood volume. Logistic regression was used to predict the risk of extensive blood loss between the two groups when age, weight, extent of surgery was controlled for and anaesthetic and surgical techniques remained similar. Results Patients with neuromuscular disease did not vary significantly in age, weight, or preoperative haematocrit and platelet count from patients without neuromuscular disease. Neuromuscular patients did have significantly more vertebral segments fused. When this difference was controlled for statistically, neuromuscular patients had an almost seven times higher risk (adjusted odds ration 6.9, P < 0.05) of losing >50% of their estimated total blood volume during scoliosis surgery. Conclusions Patients with neuromuscular disease can present various anaesthetic challenges during scoliosis surgery, among these is the inherent risk of extensive blood loss. Recognizing this may help anaesthesiologists and surgeons more accurately prepare for and treat intraoperative blood loss during scoliosis surgery in patients with neuromuscular disease. [source]

Idiopathic acquired generalized anhidrosis due to occlusion of proximal coiled ducts

BRITISH JOURNAL OF DERMATOLOGY, Issue 3 2004
J. Ogino
Summary Idiopathic acquired generalized anhidrosis is a very rare disease of unknown pathogenesis. We report a 25-year-old man with acquired generalized anhidrosis due to occlusion of the coiled ducts. He did not have sweat secretion over the entire surface of the body, including the palms and soles. Sweat-inducing stimuli provoked tingling pain on the skin. Pilocarpine iontophoresis on the forearm did not induce sweat secretion. Neurological examination did not reveal any abnormality in the central or peripheral nervous system. Skin biopsy showed that the coiled ducts were occluded by an amorphous eosinophilic substance. This amorphous eosinophilic substance was positive with periodic acid,Schiff (PAS) staining and was resistant to digestion by diastase. Electron microscopy demonstrated that the coiled ducts were completely occluded by an amorphous substance. The substance occluding the coiled ducts contained fibrous structures. These findings suggested that the acquired generalized anhidrosis in this patient was caused by occlusion of the coiled ducts by a PAS-positive substance probably derived from dark cell granules. [source]

Linear IgA bullous dermatosis induced by interferon-, 2a

CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 5 2009
P. Kocyigit
Summary Linear Ig A bullous dermatosis (LABD) is an acquired autoimmune subepidermal blistering disorder with linear deposits of IgA along the basement membrane zone. Its cause is unclear, although it appears to have an immune-mediated basis. Idiopathic, systemic disorder-related, and rarely drug-induced forms of LABD have been described. We describe a case of LABD associated with interferon-, 2A used for the treatment of Kaposi's sarcoma. [source]

Idiopathic and allergic rhinitis show a similar inflammatory response

CLINICAL OTOLARYNGOLOGY, Issue 6 2000
D.G. Powe
Hypothesis. Idiopathic and allergic rhinitics have similar mucosal mast cell and IgE+ cell distribution. Introduction. The pathophysiology of idiopathic rhinitis (IR) is unknown but patients differ from those with allergic rhinitis (AR) in that they do not express IgE. Our study is novel because we investigated: (1) three study groups chosen prospectively using strict selection criteria over a 4-year period; and (2) mast cell and IgE+ cell counts were on full-thickness, full-length inferior turbinate mucosa. Methods. Patient groups: allergic (n = 17); idiopathic: (n = 16); and normal controls (n = 9). Immunohistochemistry: mast cell and IgE+ cell detection using anti-mast cell tryptase and anti-IgE antibodies with an avidin-biotin (peroxidase) complex on paraffin processed tissue. Morphometry: sections were divided into three strata comprising an epithelial layer and two submucosal layers. Statistics: Mann,Whitney non-parametric analysis. ,,= 0.05, ,,= 0.2. Results. The power of the study was 89%. Mast cells (P = 0.03) and IgE+ cells (P < 0.05) were significantly increased in the epithelium of idiopahtic and allergic rhinitis mucosa compared to the normal control. More IgE+ cells were counted in the AR and IR groups compared to the controls in all three strata. Conclusion. Mast cells and IgE+ cells are involved in the pahtophysiology of IR. We propose that IR may be a variant form of AR involving a localized IgE-mediated inflammatory response. [source]

Fish Oil Supplementation Improves Left Ventricular Function in Children With Idiopathic Dilated Cardiomyopathy

CONGESTIVE HEART FAILURE, Issue 6 2007
Seref Olgar MD
Fish oil has a cardioprotective effect in adults with ischemic heart disease. The authors examined the effects of fish oil in children with idiopathic dilated cardiomyopathy (DCM). Eighteen DCM patients (group I) and 12 healthy children (group III) were given fish oil (10 mL/d). Their cardiac findings were compared with those of 11 patients with DCM who did not receive fish oil (group II). After 6.62±1.70 months, left ventricular ejection fraction had increased by 8.44%±3.80% (P<.05), in group I; 2.48%±3.85% (not statistically significant) in group II; and 0.84%±2.34% (not statistically significant) in group III. Left ventricular internal diastolic diameter (mm) was reduced by 4.36±4.86 (P=.001) in group I and 1.92±5.37 (P=.263) in group II, but increased by 0.22±2.54 (not statistically significant) in group III. The results suggest that fish oil leads to accelerated improvement of left ventricular function. The authors believe that if these results are confirmed in larger studies, fish oil should be added to the standard anticongestive therapy of children with DCM. [source]

Idiopathic Circumscripta Calcinosis Cutis of the Knee

DERMATOLOGIC SURGERY, Issue 12 2003
FACS, Luigi Valdatta MD
Background. Calcinosis cutis, a disease characterized by the presence of calcium deposits in the skin, is classified into four types according to etiology: dystrophic, metastatic, iatrogenic and idiopathic. The dystrophic form is the most common while the idiopathic one is the rarest, but specific incidence and frequency data are not available in scientific literature. Objective. Calcinosis cutis circumscripta is a very rare form of idiopathic calcinosis cutis arising in the second half of life. It typically involves the extremities and is associated with prior trauma and scleroderma. We dealt with a very rare form of calcinosis cutis circumscripta in a healthy patient, for whom surgical excision revealed to be an effective and successful treatment. Methods and materials. We present the case of a 46-year-old woman affected by idiopathic circumscripta calcinosis cutis of the left knee, successfully treated by surgical removal. Discussion. Medical and surgical treatment are options to cure calcinosis cutis. Medical therapy is not very effective. Surgical excision has shown to be beneficial, as it can provide a symptomatic relief. However, since calcinosis cutis limits are not always well defined a recurrence of the lesions may occur. [source]

Medical treatment of hirsutism

DERMATOLOGIC THERAPY, Issue 5 2008
Ulrike Blume-Peytavi
ABSTRACT:, Hirsutism is usually the result of an underlying adrenal, ovarian, or central endocrine abnormality mainly due to polycystic ovary syndrome but may also be idiopathic or drug induced. The aim of medical treatment of hirsutism is to rectify any causal hormonal balance, slow down or stop excessive hair growth, and improve the aesthetic appearance of hirsutism, thereby positively affecting the patient's quality of life. Today, for the majority of women, a monotherapy with oral contraceptives that have antiandrogenic activity is recommended as a first-line treatment for hirsutism. Combining an oral contraceptive pill with an antiandrogen is recommended if clinical improvement of hirsutism is insufficient after 6,9 months' monotherapy. In women who present with hirsutism, hyperandrogenism, and insulin resistance, insulin sensitizers are effective for the hirsutism as well as the hyperinsulinemia, hyperandrogenism, and infertility but there is no convincing evidence that they are effective for hirsutism alone. Topical eflornithine is a medical therapy that can be a useful adjuvant for hirsutism when used in conjunction with systemic medications or with laser/photoepilation. [source]

Contribution of neuroinflammation in burning mouth syndrome: indications from benzodiazepine use

DERMATOLOGIC THERAPY, Issue 2008
Fabrizio Guarneri
ABSTRACT: Characterized by burning and painful oral sensations in absence of clinically significant mucosal abnormalities, the burning mouth syndrome is, despite numerous researches made, basically idiopathic and, consequently, difficult to treat effectively. Therapy with tricyclic antidepressants and benzodiazepines has been proposed, although the exact pathomechanism is not clear. The objective of this study is to define the possible reasons for the efficacy of benzodiazepines in the treatment of the burning mouth syndrome. Starting from the report of eight cases successfully treated with prazepam, the present authors examined the clinical features and the evidence from literature that support the possibility of a role of neuroinflammation in the pathogenesis of the burning mouth syndrome. Available data suggest that the nervous system could be crucial in the pathogenesis of the syndrome (altered perception of pain, disturbance of neural transmission, increased excitability, negative involvement of trigeminal-vascular system), and the present authors' experience lets them suppose a role for neuroinflammation. This hypothesis could also explain the positive response to benzodiazepines in some patients. The important role of neuroinflammation in dermatologic and oral diseases has been only recently investigated and acknowledged. Further studies on the connection between neuroinflammation and burning mouth syndrome could open interesting perspectives in the understanding and management of this difficult clinical condition. [source]

Second- and third-generation antihistamines in the treatment of urticaria

DERMATOLOGIC THERAPY, Issue 4 2000
Anne K. Ellis
ABSTRACT: Chronic urticaria is mainly idiopathic in nature and can be difficult to treat. While less responsive to antihistamine therapy than acute urticaria, antihistamines still play a key role in the management of symptomatology. While many of the antihistamines still commonly used to treat urticaria are first generation H1 antagonists (e.g., diphenhydramine, hydroxyzine), the more recently developed second-generation agents (e.g., loratadine, cetirizine) and their metabolites,the third-generation antihistamines (e.g., fexofenadine, norastemizole, descarboxyloratadine),possess many of the desirable clinical effects of the first-generation agents with a more tolerable side effect profile. This review discusses the advantages and disadvantages of each of the various second- and third-generation agents available, and presents some of the data showing the differences among these agents in the treatment of chronic urticaria. [source]

Impact of congenital talipes equinovarus etiology on treatment outcomes

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 7 2008
Christina A Gurnett MD
Although congenital talipes equinovarus (CTEV) is often idiopathic, additional birth defects occur in some patients that may have an impact on the treatment of this disorder. The purpose of this study was to determine the prevalence of associated malformations, chromosomal abnormalities, or known genetic syndromes, and to compare treatment outcomes of children with idiopathic CTEV with children with non-idiopathic CTEV. Of 357 children evaluated, 273 (76%) had idiopathic CTEV (179 males, 94 females; mean age 2y 1mo [SD 1y 2mo], range 0,18y) and 84 (24%) had non-idiopathic CETV (51 males, 33 females; mean age 2y 5mo [SD 2y], range 0,16y). Disorders affecting the nervous system were found in 46 (54%) children with non-idiopathic CTEV. In a subgroup of patients treated entirely at our institution (n=196), children with non-idiopathic CTEV (n=47) required more casts for correction than those with idiopathic CTEV (n=149; 5.3 vs 4.6; p=0.016). There was also a greater risk of recurrence in non-idiopathic CTEV (14.9% vs 4%; p=0.009), but no significant difference in the need for extensive surgery (2.7% vs 8.5%; p=0.096). Treatment was initiated at a mean age of 13 weeks (range 1wk to 2y 6mo) for both idiopathic and non-idiopathic patients, and treatment was assessed during a minimum 2-year follow-up. Non-idiopathic CTEV can be successfully treated with the Ponseti method of serial casting, with low recurrence rates or need for surgery. [source]

Outcome after prolonged convulsive seizures in 186 children: low morbidity, no mortality

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 1 2004
Piia Metsäranta BM
Prolonged convulsive seizures are a common neurological emergency and a potential cause of neuronal damage and functional sequelae. We explored the role of seizure duration and various background factors for neurological sequelae in children with prolonged convulsive seizures. The population-base of this study was all children (age < 16 years) who had been admitted to the Tampere University Hospital, Finland between 1993 and 1999 with convulsive seizures lasting more than 5 minutes. Patients were followed up individually (mean length of follow-up 2 years 1 month, range 0 to 7 years 8 months). All available data on the prolonged seizure episodes and clinical follow-up were analyzed retrospectively by a detailed review of all medical charts and records. In 186 children (94 males, 92 females; mean age 4 years 5 months, SD 3 years 10 months, range 1 month to 15 years 4 months) there were 279 separate convulsive seizure episodes lasting over 5 minutes, yielding an annual incidence of 47.5 out of every 100000 episodes. Seizure aetiology was idiopathic in 26.2% of episodes, febrile in 41.9%, remote symptomatic in 28%, and acute symptomatic in 3.9% of episodes. Mean duration of all seizure episodes was 42.5 minutes (SD 46.1 minutes) and was significantly correlated with the aetiology: shortest in the febrile group (mean 35.4 minutes) and longest in the acute symptomatic group (mean 88.6 minutes; p < 0.001). There was no mortality related directly to these acute seizure episodes. The most common sequela was an onset of epilepsy in 40 children (22%). Permanent neurological sequelae were noted in only four patients (2.2%; mean seizure duration 16 minutes) and non-permanent sequelae in six patients (3.2%; mean seizure duration 38 minutes). Neurological sequelae of prolonged convulsive seizures in children are rare and are related to aetiological factors rather than the duration of a single seizure. The role of acute seizures in the evolution of epilepsy in children remains obscure. [source]

Differences in Echocardiographic Assessment with Standard Doppler and Tissue Doppler Imaging of Left Ventricular Filling Pressure in Idiopathic and Ischemic Dilated Cardiomyopathy

Right Ventricular Dimensions and Function in Isolated Left Bundle Branch Block: Is There Evidence of Biventricular Involvement?

ECHOCARDIOGRAPHY, Issue 5 2008
Jeroen Van Dijk M.D.
Background: Isolated left bundle branch block (LBBB) may be an expression of idiopathic cardiomyopathy affecting both ventricles. The present study was conducted to evaluate right ventricular (RV) dimensions and function in asymptomatic LBBB patients with mildly depressed left ventricular (LV) function. Methods: Fifteen patients with asymptomatic LBBB in whom coronary artery disease, hypertension, and valvular pathology was excluded were studied. Fifteen healthy volunteers and 15 idiopathic dilated cardiomyopathy LBBB patients served as controls. RV long axis and tricuspid annulus diameter were obtained, as were tricuspid annular plane systolic excursion (TAPSE) and peak systolic velocity (Sm) of the RV free wall annulus. Tricuspid regurgitation (TR) jets (peak TR jets) were used for RV pressure assessment. Results: RV dimensions were comparable between the asymptomatic LBBB patients and controls. RV functions of healthy volunteers and asymptomatic LBBB patients were similar (TAPSE: 24 ± 3 and 24 ± 4 mm, Sm: 13 ± 2 and 13 ± 3 cm/s, respectively), whereas functional parameters in idiopathic dilated cardiomyopathy patients were significantly reduced (TAPSE: 19 ± 5 mm, Sm: 9 ± 2 cm/s, both P < 0.01 by analysis of variance [ANOVA]). For the three groups combined, a significant inverse correlation between RV pressure (peak TR jets) and RV function (Sm) was observed (r =,0.52, P = 0.017). Conclusions: In patients with an asymptomatic LBBB, RV dimensions and function are within normal range. The present study suggests that screening of RV functional parameters in asymptomatic LBBB patients is not useful for identification of an early-stage cardiomyopathy, and RV dysfunction is merely a consequence of increased RV loading conditions caused by left-sided heart failure and does not indicate a generalized cardiomyopathy affecting both ventricles. [source]

C-Reactive Protein and Aortic Stiffness in Patients with Idiopathic Dilated Cardiomyopathy

ECHOCARDIOGRAPHY, Issue 1 2007
Feridun Kosar M.D.
Background: Previous studies have shown an association between C-reactive protein (CRP)and arterial stiffness in most cardiovascular diseases. Increased CRP levels and arterial stiffness have been considered independent predictors of cardiovascular mortality in cardiovascular disease and even in the general population. Objective: The aim of this study was to investigate the relationship between CRP, a marker of systemic inflammation and aortic stiffness in patients with idiopathic dilated cardiomyopathy (DCMP). Methods: Serum CRP levels and aortic stiffness parameters were measured in DCMP patients (n= 37) and age- and gender-matched control subjects (n= 30). High-sensitivity CRP levels were determined by an immunonephelometry assay. Aortic strain (AS) and aortic distensibility (AD) were calculated from the aortic diameters measured using M-mode echocardiography and blood pressure obtained by sphygmomanometry. Results: Serum levels of CRP in DCMP patients were higher than in the control subjects (5.47 ± 2.06 mg/L and 2.35 ± 0.47 mg/L, P < 0.001, respectively). AS and AD were significantly decreased in DCMP patients compared to the controls (P < 0.001 and P < 0.001, respectively). There were positive correlations between CRP, and (r = 0.3.64, P = 0.027) smoking (r = 0.3.56, P = 0.024), and increasing age (r = 0.587, P < 0.001), and negative correlations between CRP, and DBP (r =,0.485, P < 0.001), diameter change (DC; r =,0.493, P < 0.001), AS (r =,0.526, P < 0.001), and AD (r =,0.626, P < 0.001). Conclusion: We have shown that there is a significant relation between high serum CRP levels and impaired aortic stiffness in patients with idiopathic DCMP. These findings may indicate an important role of CRP in the pathogenesis of impaired aortic stiffness in idiopathic DCMP. [source]

Revised terminology and concepts for organization of seizures and epilepsies: Report of the ILAE Commission on Classification and Terminology, 2005,2009

EPILEPSIA, Issue 4 2010
Anne T. Berg
Summary The International League Against Epilepsy (ILAE) Commission on Classification and Terminology has revised concepts, terminology, and approaches for classifying seizures and forms of epilepsy. Generalized and focal are redefined for seizures as occurring in and rapidly engaging bilaterally distributed networks (generalized) and within networks limited to one hemisphere and either discretely localized or more widely distributed (focal). Classification of generalized seizures is simplified. No natural classification for focal seizures exists; focal seizures should be described according to their manifestations (e.g., dyscognitive, focal motor). The concepts of generalized and focal do not apply to electroclinical syndromes. Genetic, structural,metabolic, and unknown represent modified concepts to replace idiopathic, symptomatic, and cryptogenic. Not all epilepsies are recognized as electroclinical syndromes. Organization of forms of epilepsy is first by specificity: electroclinical syndromes, nonsyndromic epilepsies with structural,metabolic causes, and epilepsies of unknown cause. Further organization within these divisions can be accomplished in a flexible manner depending on purpose. Natural classes (e.g., specific underlying cause, age at onset, associated seizure type), or pragmatic groupings (e.g., epileptic encephalopathies, self-limited electroclinical syndromes) may serve as the basis for organizing knowledge about recognized forms of epilepsy and facilitate identification of new forms. [source]

Historical Aspects of Idiopathic Generalized Epilepsies

EPILEPSIA, Issue 2005
Peter Wolf
Summary:, Early in these proceedings, the origin of the three terms in the title, "idiopathic generalized epilepsy," is discussed with respect to their significance over time, and typical misunderstandings. In the mid-20th century, a rather chaotic use of a multitude of often loosely defined terms had developed, which increasingly became an obstacle to a meaningful international discussion. The International League against Epilepsy (ILAE) took the initiative to develop an internationally accepted terminology with a classification system consisting of a classification of seizures (1981) and a classification of syndromes (1989). The Idiopathic Generalized Epilepsies are one of its four major groups emerging from a double dichotomy of generalized versus localization-related and idiopathic versus symptomatic. The inclusion of biologic aspects such as syndrome-specific ages of onset ("age-related syndromes") or syndrome-specific relations of seizure occurrence to the sleep,wake cycle ("Epilepsy with Grand Mal on Awaking") meant that the syndrome classification merged the more biological views of the German school with the more neurophysiological ones of the French. Apart from establishing a common international language concerning epilepsy, the International Classification of Epilepsies and Epileptic Syndromes became an important stimulator of research, especially concerning the idiopathic epilepsies. In particular, genetic and functional imaging investigations aim at a better understanding of these conditions. It is now understood that most idiopathic syndromes have a,sometimes complex,genetic background, but we are also becoming aware of the inappropriateness of the time-honored term "generalized" and part of our dichotomies. Both localization-related and "generalized" idiopathic epilepsies seem to share a principle of ictogenesis based on functional anatomic pathogenic networks, and we seem to move toward understanding them as functional system disorders of the brain. [source]

Evidence-based Treatment of Idiopathic Generalized Epilepsies with Older Antiepileptic Drugs

EPILEPSIA, Issue 2005
Nikolas Hitiris
Summary:, Older antiepileptic drugs continue to play a major role in the treatment of the idiopathic generalized epilepsies. Comparative studies of ethosuximide and valproate have demonstrated equivalence in the treatment of childhood absence epilepsy. Valproate can be regarded as the recommended first-line treatment for juvenile myoclonic epilepsy based on case series reports. Studies in patients with generalized tonic-clonic seizures have not separated out idiopathic from secondary generalized events. Treatment for the other idiopathic generalized epilepsy syndromes lacks evidence other than a few case reports and diverse expert opinion. Further randomized controlled trials of older antiepileptic drugs are recommended to solidify the evidence-based treatment of the idiopathic generalized epilepsies. [source]

A Proton Magnetic Resonance Spectroscopy Study of Metabolites in the Occipital Lobes in Epilepsy

EPILEPSIA, Issue 4 2003
Robert J. Simister
Summary: ,Purpose: ,-Amino butyric acid (GABA) and glutamate, respectively the principal inhibitory and excitatory neurochemicals in the brain, are visible to proton magnetic resonance spectroscopy (MRS). We report a study of GABA+ (GABA plus homocarnosine) and GLX (glutamate plus glutamine) concentrations in the occipital lobes in patients with idiopathic generalised epilepsy (IGE) and in patients with occipital lobe epilepsy (OLE). Methods: Fifteen patients with IGE, 15 patients with OLE, and 15 healthy volunteers were studied. A single voxel was prescribed in the occipital lobes for each subject. PRESS localised short-echo-time MRS was performed to measure GLX by using LCModel. A double quantum GABA filter was used to measure GABA+. Segmented T1 -weighted images gave the tissue composition of the prescribed voxel. Results: Grey-matter proportion, GLX, and GABA+ were all elevated in IGE. However, analysis using grey-matter proportion as a covariable showed no significant group differences. No correlation was observed between GABA+ concentration and either seizure frequency or time since last seizure. Conclusions: GLX and GABA+ were elevated in IGE. Elevated grey-matter content in the IGE group despite normal MRI appearance can be expected to account for some or all of this observed elevation of GLX and GABA+. GABA+ concentration did not correlate with seizure control or duration since most recent seizure. [source]

Nonsymptomatic Generalized Epilepsy in Children Younger than Six Years: Excellent Prognosis, but Classification Should Be Reconsidered after Follow-up: The Dutch Study of Epilepsy in Childhood

EPILEPSIA, Issue 7 2002
C. M. Middeldorp
Summary: ,Purpose: To assess the prognosis and the accuracy of the epilepsy classification in young children with nonsymptomatic generalized epilepsy. Methods: Of the cohort of the Dutch Study of Epilepsy in Childhood (n = 466), all children younger than 6 years with a diagnosis of idiopathic (IGE) or cryptogenic (CGE) generalized epilepsy either at intake (n = 108) and/or after 2 years of follow-up (n = 102) were included. The number of reclassifications after 2 years was determined, and the reasons for reclassification were analyzed. All children receiving a diagnosis of IGE or CGE at 2 years were followed up for 5 years to study their outcome in terms of terminal remission (TR). Data on their level of intellectual functioning were collected at the start of this analysis. Results: The epilepsy syndrome was reclassified in 17 children. In 14 of them, the seizure type also was reclassified, and in three, the course of the epilepsy determined the new epilepsy type. Two other children had a reclassification of their seizure types without a change of the epilepsy type. Many children were categorized as having IGE not otherwise specified. In all probability, this is a heterogeneous group, containing patients with various epilepsy syndromes, with generalized tonic,clonic seizures as a common hallmark. Of the 102 children with IGE or CGE at 2 years of follow-up, 75% had a TR of >6 months after 2 years, and 85% a TR of ,1 year after 5 years. Conclusions: In a fair proportion of children with nonsymptomatic generalized epilepsy in this age group, it is not possible to classify firmly the epilepsy and/or the seizures immediately after the intake. Instead, they are reclassified during the course of the disease. This and the apparent heterogeneity of the category IGE not otherwise specified point to inherent drawbacks of the current International League Against Epilepsy (ILAE) classification of epilepsy and epileptic syndromes. The prognosis of IGE at this young age is generally excellent. [source]

New Insights into the Clinical Management of Partial Epilepsies

EPILEPSIA, Issue S5 2000
Prof. Edouard Hirsch
Summary The diagnosis, treatment, and prognosis of seizure disorders depend on the correct identification of epileptic syndromes. Partial epilepsies are heterogeneous and can be divided into idiopathic, cryptogenic, and symptomatic epilepsies. The most common of the idiopathic localization-related epilepsies is benign epilepsy with rolandic or centrotemporal spikes (BECTS). Seizures remain rare and the use of antiepileptic drug (AED) treatment in all patients does not appear justified. Children who present with some of the electroclinical characteristics of BECTS may also display severe unusual neurologic, neuropsychological, or atypical symptoms. In some cases, carbamazepine has been implicated as a triggering factor. Primary reading epilepsy and idiopathic occipital lobe epilepsies with photosensitivity are examples of an overlap between idiopathic localization-related and generalized epilepsies and respond well to sodium valproate. Autosomal dominant nocturnal frontal lobe epilepsy and benign familial infantile convulsions are recently described syndromes, differing in several ways from classical idiopathic localization-related epileptic syndromes. In cryptogenic or symptomatic epilepsy, the topography of the epileptogenic zone might influence drug efficacy. An individualized approach to AED selection, tailored to each patient's needs, should be used. Resistance of seizures to antiepileptic therapy may be due to diagnostic and/or treatment error or may be the result of noncompliance. Increasing the dosage, discontinuation or replacement of a drug, or addition of a second drug is indicated in truly resistant cases. The use of more than two AEDs rarely optimizes seizure control, and in some cases reduction of treatment may improve seizure control while lessening side effects. EEG-video assessment of patients with refractory epilepsy is important. Indications for and timing of epilepsy surgery should be reconsidered. Surgical therapy should probably be used more often and earlier than it is at present. [source]

A single question for the rapid screening of restless legs syndrome in the neurological clinical practice

EUROPEAN JOURNAL OF NEUROLOGY, Issue 9 2007
R. Ferri
The purposes of this study were to validate the use of a single standard question for the rapid screening of restless legs syndrome (RLS) and to analyze the eventual effects of the presence of RLS on self-assessed daytime sleepiness, global clinical severity and cognitive functioning. We evaluated a group of 521 consecutive patients who accessed our neurology clinic for different reasons. Beside the answer to the single question and age, sex, and clinical diagnosis, the following items were collected from all patients and normal controls: the four criteria for RLS, the Epworth Sleepiness Scale (ESS), the Clinical Global Impression of Severity (CGI-S), and the Mini-Mental State evaluation. RLS was found in 112 patients (70 idiopathic). The single question had 100% sensitivity and 96.8% specificity for the diagnosis of RLS. ESS and CGI-S were significantly higher in both RLS patient groups than in normal controls. RLS severity was significantly higher in idiopathic than in associated/symptomatic RLS patients. RLS can be screened with high sensitivity and good reliability in large patient groups by means of the single question; however, the final diagnosis should always be confirmed by the diagnostic features of RLS and accompanied by a careful search for comorbid conditions. [source]

A systematic review on the diagnosis and treatment of primary (idiopathic) dystonia and dystonia plus syndromes: report of an EFNS/MDS-ES Task Force

EUROPEAN JOURNAL OF NEUROLOGY, Issue 5 2006
A. Albanese chairman
To review the literature on primary dystonia and dystonia plus and to provide evidence-based recommendations. Primary dystonia and dystonia plus are chronic and often disabling conditions with a widespread spectrum mainly in young people. Computerized MEDLINE and EMBASE literature reviews (1966,1967 February 2005) were conducted. The Cochrane Library was searched for relevant citations. Diagnosis and classification of dystonia are highly relevant for providing appropriate management and prognostic information, and genetic counselling. Expert observation is suggested. DYT-1 gene testing in conjunction with genetic counselling is recommended for patients with primary dystonia with onset before age 30 years and in those with an affected relative with early onset. Positive genetic testing for dystonia (e.g. DYT-1) is not sufficient to make diagnosis of dystonia. Individuals with myoclonus should be tested for the epsilon-sarcoglycan gene (DYT-11). A levodopa trial is warranted in every patient with early onset dystonia without an alternative diagnosis. Brain imaging is not routinely required when there is a confident diagnosis of primary dystonia in adult patients, whereas it is necessary in the paediatric population. Botulinum toxin (BoNT) type A (or type B if there is resistance to type A) can be regarded as first line treatment for primary cranial (excluding oromandibular) or cervical dystonia and can be effective in writing dystonia. Actual evidence is lacking on direct comparison of the clinical efficacy and safety of BoNT-A vs. BoNT-B. Pallidal deep brain stimulation (DBS) is considered a good option, particularly for generalized or cervical dystonia, after medication or BoNT have failed to provide adequate improvement. Selective peripheral denervation is a safe procedure that is indicated exclusively in cervical dystonia. Intrathecal baclofen can be indicated in patients where secondary dystonia is combined with spasticity. The absolute and comparative efficacy and tolerability of drugs in dystonia, including anticholinergic and antidopaminergic drugs, is poorly documented and no evidence-based recommendations can be made to guide prescribing. [source]

Craniofacial morphology, dental occlusion, tooth eruption, and dental maturity in boys of short stature with or without growth hormone deficiency

EUROPEAN JOURNAL OF ORAL SCIENCES, Issue 5 2000
Heidrun Kjellberg
The aim of this project was to study the craniofacial morphology, dental occlusion, dental maturation and tooth eruption in short-statured boys with growth hormone secretion ranging from low to high. The measurements from lateral and postero-anterior cephalograms, orthopantomograms and plaster models were used. Almost all linear measurements of the facial structures were significantly smaller. A disproportionate growth in the cranial base structures as well as in the jaws resulted in facial retrognathia, a proportionately smaller posterior than anterior facial height, and a steep vertical inclination of the mandible. Dental crowding was more common and the overbite was small. Dental maturity and tooth eruption were delayed 1.2 and 1.3 yr, respectively. No significant differences between the idiopathic short-statured and the growth hormone-deficient group in any of the above-mentioned variables were found. It can be concluded that although most of the cephalometric variables measured differed significantly from the average, the facial appearance of the boys is not conspicuous and is of minor clinical importance. However, the short-statured boys might be in greater need of orthodontic treatment due to the higher percentage of dental crowding. [source]