Controversial Clinical Entity Research Articles

Pediatric Bipolar Disorder: A Practical Guide for Clinicians.

Pediatric bipolar disorder (PBD) is a controversial clinical entity and it still needs to be satisfactorily defined. Having a polymorphous presentation and associated with numerous symptoms of comorbid psychiatric illnesses often diagnosed during childhood and adolescence, including attention deficit hyperactivity disorder, its symptoms do not completely parallel those of bipolar disorder in adults. The clinician must be able to reach a diagnosis of PBD in the presence of fluctuating and atypical symptoms, especially in children, who tend to experience mixed episodes and very rapid cycles. Historically a key symptom for diagnosing PBD is episodic irritability. Proper diagnosis is critical due to the gravity of its prognosis. Clinicians may find supporting evidence for a diagnosis through careful study of the medical and developmental history of the young patient in addition to psychometric data. Treatment prioritizes psychotherapeutic intervention and assigns important roles to family involvement and a healthy lifestyle.

Nodal Merkel Cell Carcinoma with Unknown Primary Site and No Distant Metastasis: A Single-Center Series.

Simple SummaryMerkel cell carcinoma is a very rare and highly aggressive neuroendocrine carcinoma originating from the skin. Exceptionally it presents with a nodal localization without a cutaneous primary site and distant metastases. This entity is controversial in terms of origin and clinical management. The main histological differential diagnosis is that of small cell neuroendocrine carcinoma. As a referral center for neuroendocrine neoplasms with more than 20 years of experience we have dealt with patients showing this clinical context several times and we usually manage them within our dedicated multidisciplinary team. Due to the extreme rarity of the entity and undefined clinical management, we report our single-center series and detail some of the diagnostic and therapeutic aspects. Our analysis can be helpful for centers which manage these patients and future investigations on the topic.Merkel cell carcinoma (MCC) is a very rare and aggressive neuroendocrine carcinoma originating from Merkel cells, typically with a skin nodule; however, it exceptionally presents with only a basin lymph node localization, with neither a cutaneous primary site nor distant metastases. From 1996 to 2020, among patients with histologically confirmed MCC managed at a neuroendocrine neoplasm-referral center, we selected those with an exclusive nodal basin, no distant metastasis, and an unknown primary site defined by cross-sectional and physical examination. A total of 55 out of 310 patients fulfilled the selection criteria. The median age was 64 years and the majority were males. Inguinal lymph-nodes were the most common anatomic site. With a median follow-up of 4.3 years, the 5-year relapse-free survival (RFS) rate was 56.6 (95% CI 42.0–68.8%) and the 5-year cancer specific survival (CSS) rate was 68.5 (95% CI 52.8–79.9%) for the whole population. The 36 patients (65.5%) undergoing lymphadenectomy (LND) + radiotherapy (RT) ± chemotherapy had a 5-year RFS rate of 87.2% (95% CI 65.5–95.7%) and a 5-year CSS rate of 90.5% (95% CI 67.0–97.5), which were better than those receiving LND alone. In a multivariable analysis, the survival benefit for LND + RT remained significant. Results from one of the largest single-center series of nMCC-UP suggest that a curative approach including RT can be effective, similar to what is observed for stage IIIB MCC. Multicentric studies with homogenous populations should be carried out in this controversial clinical entity, to minimize the risk of biases and provide robust data.

Chronic Endometritis: Diagnostic Considerations in Patients With Infertility

Abstract Chronic endometritis (CE) is a controversial clinical and pathological entity. Although the presence of plasma cells (PCs) is the most frequently used diagnostic criterion for CE, the minimal diagnostic criteria remain controversial and undefined. The clinical setting of CE (asymptomatic, pelvic inflammatory disease, infertility) is an important consideration regarding the clinical significance of endometrial PCs. In the setting of infertility, specifically recurrent implantation failure (RIF) and recurrent pregnancy loss (RPL), CE may have a negative impact on endometrial receptivity, resulting in higher rates of implantation failure. The proposed pathophysiology is that a subclinical intrauterine infection alters the local inflammatory milieu resulting in a shift of the normal inflammatory cell profiles toward responses associated with implantation failure and poorer reproductive outcomes. Cure rates following antibiotic treatment are reported to be high (up to 90%). The results of meta-analyses describing the effect of CE on reproductive outcomes are mixed. There is evidence supporting CE as a treatable factor with improved reproductive outcomes following treatment and there are also results showing no differences in reproductive outcomes. Variable diagnostic criteria for CE, inclusion criteria, and treatment regimens between studies are the main limitations, hampering the ability to compare results across studies. Results regarding the optimal diagnostic criteria in the setting of RIF and RPL are also variable, with some authors recommending a cutoff of 1 or more CD138+ PCs per high-power field and some recommending a cutoff of 5 or more CD138+ PCs per high-power field. There are some studies indicating CE as a negative prognostic factor in patients with RIF and RPL, which may be reversible with antibiotic treatment. The optimal diagnostic criteria for CE in this clinical setting are undefined. For the investigation of infertility patients whom clinicians intend to treat, a descriptive diagnosis (indicating the presence/number of PCs and method of identification) is a reasonable approach. Routine use of CD138 immunohistochemistry is of limited value for the diagnosis of CE.

Surgery Versus Continued Conservative Treatment for Neurogenic Thoracic Outlet Syndrome: the First Randomised Clinical Trial (STOPNTOS Trial)

Neurogenic thoracic outlet syndrome (NTOS) is one of the most controversial clinical entities in medicine. Several major case series have shown promising results of surgery; however, solid scientific evidence is lacking. The aim of this trial was to objectify the effect of thoracic outlet decompression (TOD).

Surgery Versus Continued Conservative Treatment for Neurogenic Thoracic Outlet Syndrome: the First Randomised Clinical Trial (STOPNTOS Trial)

Neurogenic thoracic outlet syndrome (NTOS) is one of the most controversial clinical entities in medicine. Several major case series have shown promising results of surgery; however, solid scientific evidence is lacking. The aim of this trial was to objectify the effect of thoracic outlet decompression (TOD). A single centre (high volume, tertiary TOS centre), non-blinded, randomised controlled trial was conducted with parallel group design. Patients with a diagnosis of NTOS refractory to conservative therapy were randomised to one of two intervention arms, receiving either a transaxillary thoracic outlet decompression (TA-TOD) or continued conservative treatment. After three months, the conservative treated group was also offered a TA-TOD. The primary outcome was change in Disability of the Arm, Shoulder and Hand (DASH) questionnaire score. Secondary outcomes were changes in Cervical Brachial Symptoms Questionnaire (CBSQ), TOS disability scale, and quality of life scores. Outcomes were assessed at baseline, three, six, and 12 months after inclusion. Fifty patients were enrolled in this trial: 25 in the TA-TOD group and 25 in the continued conservative treatment group. Follow up was completed in 24 and 22 patients, respectively. At three months, there was a statistically significant difference in DASH scores (TA-TOD: mean 45.15, 95% confidence interval [CI] 38.08 - 52.21; conservative treatment: mean 64.92, 95% CI 57.54 - 72.30; p < .001). All patients in the conservative treatment group applied for surgery three months after randomisation. After surgery of the conservative treatment group, there was no statistically significant difference between the groups for all primary and secondary outcome measures. TA-TOD for NTOS is effective in patients who do not respond to conservative treatment. Trial register number: NL63986.100.17.

Metabolomic Characterization of Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS).

IntroductionPANS is a controversial clinical entity, consisting of a complex constellation of psychiatric symptoms, adventitious changes, and expression of various serological alterations, likely sustained by an autoimmune/inflammatory disease. Detection of novel biomarkers of PANS is highly desirable for both diagnostic and therapeutic management of affected patients. Analysis of metabolites has proven useful in detecting biomarkers for other neuroimmune-psychiatric diseases. Here, we utilize the metabolomics approach to determine whether it is possible to define a specific metabolic pattern in patients affected by PANS compared to healthy subjects.DesignThis observational case-control study tested consecutive patients referred for PANS between June 2019 to May 2020. A PANS diagnosis was confirmed according to the PANS working criteria (National Institute of Mental Health [NIMH], 2010). Healthy age and sex-matched subjects were recruited as controls.MethodsThirty-four outpatients referred for PANS (mean age 9.5 years; SD 2.9, 71% male) and 25 neurotypical subjects matched for age and gender, were subjected to metabolite analysis. Serum samples were obtained from each participant and were analyzed using Nuclear Magnetic Resonance (NMR) spectroscopy. Subsequently, multivariate and univariate statistical analyses and Receiver Operator Curves (ROC) were performed.ResultsSeparation of the samples, in line with the presence of PANS diagnosis, was observed by applying a supervised model (R2X = 0.44, R2Y = 0.54, Q2 = 0.44, p-value < 0.0001). The significantly altered variables were 2-Hydroxybutyrate, glycine, glutamine, histidine, tryptophan. Pathway analysis indicated that phenylalanine, tyrosine, and tryptophan metabolism, as well as glutamine and glutamate metabolism, exhibited the largest deviations from neurotypical controls.ConclusionWe found a unique plasma metabolic profile in PANS patients, significantly differing from that of healthy children, that suggests the involvement of specific patterns of neurotransmission (tryptophan, glycine, histamine/histidine) as well as a more general state of neuroinflammation and oxidative stress (glutamine, 2-Hydroxybutyrate, and tryptophan-kynurenine pathway) in the disorder. This metabolomics study offers new insights into biological mechanisms underpinning the disorder and supports research of other potential biomarkers implicated in PANS.

Compensated hypogonadism in men with sickle cell disease.

To evaluate the prevalence of hypogonadism in a population of men with SCD and characterize its aetiology. Sickle cell disease (SCD) is associated with the development of hypogonadism, but there is still controversy regarding its aetiology and clinical implications. We performed a cross-sectional study of 34 men with SCD aged > 18 years. Sociodemographic and clinical data, including anthropometric measurements (weight, height and BMI), were obtained. Early morning, blood samples were collected and total testosterone (TT), free testosterone (FT), luteinizing hormone (LH), follicle stimulating hormone (FSH), a complete blood count and haemoglobin electrophoresis were measured. Eugonadism was defined as T≥300ng/dL and LH≤9.4mUI/mL; primary hypogonadism as T< 300 ng/dL and LH>9.4mUI/mL; secondary hypogonadism as T<300ng/dL and LH≤9.4mUI/mL; and compensated hypogonadism as T≥300ng/dL and LH>9.4mUI/mL. Median age was 33 (26-41) years, and SS genotype was the most frequent (73.5%). The prevalence of eugonadism, compensated hypogonadism and secondary hypogonadism was 67.5%, 26.4% and 5.88%, respectively. No men with primary hypogonadism were identified in our sample. Those with compensated hypogonadism had also higher FSH levels (>7.8mUI/mL, P<.0001). In our study population of men with SCD, a high prevalence of compensated hypogonadism was identified, which is a controversial and distinct clinical entity that warrants monitoring and further research.

Trends of Cholecystectomies for Presumed Biliary Dyskinesia in Children in the United States.

Biliary dyskinesia (BD) is a controversial clinical entity. Standardized diagnostic test and management guidelines are lacking in children. Published data suggest that long-term outcomes of surgical and medical management are similar. We sought to determine national population-based trends of cholecystectomies performed in children for BD and associated healthcare expenditure in the United States during a 10-year period. Using Nationwide Inpatient Sample and the International Classification of Diseases, the 9th revision clinical modification codes, we identified children who had a cholecystectomy for BD from 2002 to 2011 in the United States. A total of 66,380 cholecystectomies were identified as primary procedural diagnosis using weighted analysis from 2002 to 2011 in children. BD was the primary indication for cholecystectomy in 6674 (10.8%) of the patients. During the study period, the number of cholecystectomies performed for BD in children increased from 6.6% in 2002 to 10.6% in 2011, and a majority were adolescent white females. The annual health care expenditure for surgical management of BD for children in the US was estimated to almost $16 million/year. Despite lack of standardized diagnostic criteria and variable outcomes of surgical intervention reported in pediatric literature, cholecystectomies are commonly performed for children with BD in the United States. Consensus guidelines for the diagnosis and management of this controversial disorder in children are needed.

Vocal fold paresis

Vocal fold paresis is a complex, controversial, and unique clinical entity. Guidance in assessing and evaluating these patients is provided in this comprehensive review of the current literature discussing the varying clinical presentation, the broad differential and general prognosis. Patients with vocal fold paresis can present with elements of hyperfunction, which can often mask an underlying paresis. As such, repetitive phonatory tasks and videostroboscopic examination are critical for the assessment of patients with a suspected paresis. When analyzing stroboscopic findings, anatomical and motion asymmetries can strongly suggest the presence of a paresis. However, it is important to remember that other disorders can sometimes mimic or create a visual asymmetry when a true paresis may not be present. Laryngeal electromyography (LEMG) can serve as a valuable adjunct to confirm a paresis with the most reliable indicator being a decreased recruitment pattern. The differential is vast, including infectious, iatrogenic, systemic rheumatologic, and neurologic conditions. LEMG along with time of onset and the underlying cause of the paresis can be valuable prognostic indicators. Patients with paresis often present with symptoms of a hyperkinetic voice disorder. Regardless of the myriad of causes, their assessment hinges upon close clinical evaluation with videostroboscopy aided with LEMG.

Thoracic Outlet Syndrome: Past and Present—88 Surgeries in 30 Years at Chang Gung

Background:Thoracic outlet syndrome (TOS) is a highly controversial clinical entity. There is much debate on its terminology, existence, diagnosis, and treatment. The purpose of this study was to describe our opinions about these controversial topics of TOS and the treatment of TOS over the past 30 years.Methods:From 1985 to 2014, a total of 80 patients underwent decompressive surgery for TOS. Eight patients requested a second surgery on the contralateral limb. They all had at least 1-year follow-up. Preoperative evaluation included provocative tests, plain X-ray, magnetic resonance angiography/computed tomography angiography, and electromyography. Surgical intervention for each patient involved a supraclavicular approach and near-total resection of the anterior scalene muscle and the first rib and of any cervical rib if it was present. Rib resection was performed with the use of Kerrison bone punch forceps. The operative time was typically 2 hours.Results:Major postoperative complications were rare. Nearly all patients (98%) experienced significant symptom relief, with improvement in soreness and tightness of the shoulder, neck, and arm immediately on the first postoperative day or within a few weeks thereafter. There were no cases with symptoms recurring.Conclusions:It is evident that decompressive surgery through a supraclavicular approach for TOS not only is an effective and safe procedure but also provides a diagnosis of the cause of TOS. For a patient who meets the criteria for surgical indication, decompressive surgery usually results in resolution of symptoms and no recurrence.

Carcinosarcoma of the thyroid gland.

Introduction. Surgeon has significant role in the management of such rare and controversial clinical entities related to thyroid gland. In this case report we have presented an elderly patient with rapid enlargement in thyroid that was related to carcinosarcoma. Case Presentation. A 60-year-old lady was presented with rapid enlargement of the thyroid gland. A fine needle aspiration of the nodule in right lobe was performed several weeks before presentation to our clinic. End diagnosis was a papillary carcinoma of the thyroid with spindle cell component. Thus the nodule was recognized to be a carcinosarcoma. Conclusion. Thyroid surgery merits a multidisciplinary approach. Indeed the surgeon should make a conclusive decision in some controversial and rare clinical entities such as carcinosarcoma.

Postural orthostatic tachycardia syndrome (POTS): association with Ehlers-Danlos syndrome and orthopaedic considerations.

Postural orthostatic tachycardia syndrome (POTS) is the most common of several types of dysautonomia, characterized by dysfunction of the autonomic nervous system manifesting with symptoms of orthostatic intolerance with or without associated orthostatic hypotension and excessive autonomic excitation. Given the numerous presenting musculoskeletal symptoms of POTS and its known associations with other clinical entities like Ehlers-Danlos syndrome, POTS constitutes an unusual treatment challenge of which the orthopaedic surgeon and other related healthcare providers should be aware. We describe two patients with a diagnosis of POTS and musculoskeletal manifestations. The first is a 13-year-old boy with a concurrent diagnosis of Ehlers-Danlos syndrome and worsening back pain. The patient had resolution of his musculoskeletal symptoms after treatment with a Boston Overlap LSO brace and physical therapy and resolution of his cardiovascular symptoms after pharmacologic treatment. The second patient is a 17-year-old girl with an L1 vertebral hemangioma and pain of sudden onset radiating to her lower extremities. Her symptoms were controlled with therapy and gabapentin. POTS is a poorly understood and controversial clinical entity with lack of awareness and knowledge regarding the syndrome by the majority of medical specialists. Its complex and wide range of clinical presentation has led to being commonly mistaken with malingering, depression and anxiety disorders. Review of the literature did not yield any previous studies addressing POTS and the special considerations for the orthopaedic surgeon caring for these patients; previous studies of large patient series focus on investigating the pathologic substrate of POTS and the efficacy of various treatment modalities on the cardiovascular dysregulation. These two cases show that understanding and knowledge of this syndrome and its comorbidities should guide any diagnostic approach or therapeutic intervention by the orthopaedic surgeon caring for patients with this syndrome. It is important that the surgeon is aware of the benefits of detailed patient education and physical conditioning, the increased perioperative complications, and the need for specialized anesthesia.

Post-traumatic headaches: a clinical overview.

Headache attributed to head and/or neck trauma or injury, the so-called post-traumatic headache (PTH), is the most common secondary headache disorder and one of the most controversial clinical entities in the headache field, due to its unclear pathophysiological mechanisms and the unsolved role of associated psychological and medico-legal aspects. PTH, as a significant cause of morbidity after traumatic brain injury, may occur as an isolated symptom or as one of a constellation of symptoms known as post-concussive syndrome. However, in many cases, PTH might also represent an accentuation of non-disabling, remote or infrequent pre-existing primary headaches rather than a new onset headache strictly related to the trauma. Recently, the International Classification of Headache Disorders attempted to classify PTH; however, many unsolved issues are still to be clarified. In this brief review, we will focus on PTH clinical aspects and diagnostic criteria.

Factors associated with the development of superficial vein thrombosis in patients with varicose veins

IntroductionSuperficial vein thrombosis (SVT) is a common and controversial clinical entity. Recent studies have demonstrated that SVT should be seen as a venous thromboembolism (VTE). The objective of this study was to investigate the prevalence of thrombophilia defects and to estimate the role of age, sex and body mass index (BMI) in patients with varicose veins (VVs) and SVT. Materials and MethodsA total of 230 patients with VVs, 128 with, and 102 without SVT underwent thrombophilia testing included factor V Leiden, prothrombin G20210A, methylenetetrahydrofolate reductase and plasminogen activator inhibitor- 1 mutations, protein C, protein S (PS), anti-thrombin III and plasminogen deficiencies and levels of A2 antiplasmin, activate protein C resistance and lupus anticoagulant. According to Clinical- Etiology- Anatomy- Pathophysiology (CEAP) classification patients were categorized in two subgroups: moderate disease (C2,3) and severe disease (C4,5,6). Age and body mass index were also assessed. ResultsThe prevalence of thrombophilia defects was significantly higher in patients with moderate disease and SVT (p=0.002). In the C2,3 group, SVT was associated with PS deficiency (p=0.018), obesity (p<0.001), male gender (p=0.047) and age (p<0.001). There were no significant differences in patients with severe disease. ConclusionsAge, male sex, obesity and PS deficiency are factors associated with SVT development among patients with VVs having moderate disease (C2,3).

Diabetic cardiomyopathy: ongoing controversies in 2012

Diabetic cardiomyopathy is a controversial clinical entity that in its initial state is usually characterized by left ventricular diastolic dysfunction in patients with diabetes mellitus that cannot be explained by coronary artery disease, hypertension, or any other known cardiac disease. It was reported in up to 52-60% of well-controlled type-II diabetic subjects, but more recent studies, using standardized tissue Doppler criteria and more strict patient selection, revealed a much lower prevalence. The pathological substrate is myocardial damage, left ventricular hypertrophy, interstitial fibrosis, structural and functional changes of the small coronary vessels, metabolic disturbance, and autonomic cardiac neuropathy. Hyperglycemia causes myocardial necrosis and fibrosis, as well as the increase of myocardial free radicals and oxidants, which decrease nitric oxide levels, worsen the endothelial function, and induce myocardial inflammation. Insulin resistance with hyperinsulinemia and decreased insulin sensitivity may also contribute to the left ventricular hypertrophy. Clinical manifestations of diabetic cardiomyopathy may include dyspnea, arrhythmias, atypical chest pain, and dizziness. Currently, there is no specific treatment of diabetic cardiomyopathy that targets its pathophysiological substrate, but various therapeutic options are discussed that include improving diabetic control with both diet and drugs (metformin and thiazolidinediones), the use of ACE inhibitors, beta blockers, and calcium channel blockers. Daily physical activity and a reduction in body mass index may improve glucose homeostasis by reducing the glucose/insulin ratio and the increase of both insulin sensitivity and glucose oxidation by the skeletal and cardiac muscles.

Les cénesthopathies : un trouble des émotions d’arrière plan. Regards croisés des sciences cognitives et de la phénoménologie

Cenesthesia and cenesthopathy have played a fundamental role in 19th and early 20th century French and German psychiatry. Cenesthesia refers to the internal, global, implicit and affective perception of one's own body. The concept of cenesthopathy was coined by Dupre and Camus in 1907 to describe a clinical entity characterized by abnormal and strange bodily sensations. In this review, we examine the history of these concepts and the influence they have had on clinical, nosographical and phenomenological psychiatry and on cognitive neuropsychiatry. We performed a narrative review of the published research literature. Classical French and German psychiatrists have written extensively on cenesthesia and cenesthopathy although these notions are no longer in the mainstream of contemporary psychiatry. However, they are still present in contemporary psychiatric nosography in the form of some controversial clinical entities clearly related to cenesthesia such as cenesthetic schizophrenia, hypochondriacal monothematic delusions, or the Capgras and the Cotard syndromes. These clinical entities are all associated with a state of depersonalization. We point out the similarities between Ey's description of the depersonalization syndrome, especially in psychosis, and the characteristics of cenesthesia. Philosophers like Sartre or Merleau-Ponty have developed the concept of cenesthesia, and in particular have added new concepts like "body schema". Similarly, phenomenological psychiatrists like Minkowski or Tellenbach have attempted to describe psychiatric disorders associated with cenesthesia and have also proposed new concepts (i.e. atmospheric sense) in order to understand them better. More recently, cognitive neuropsychiatry has tried to discover the mechanisms, which cause or contribute to the genesis of delusions. The majority of delusion theories developed in cognitive neuropsychiatry consider that the explanation of monothematic delusions involves one or two explanatory stages. The first stage corresponds to an abnormal experience (the experiential stage) while the second is related to abnormal reasoning (the inferential stage). This theoretical first stage has been considered to be the result of a highly unusual or bizarre perceptual experience. According to the authors, this experience refers to a phenomenon of depersonalization, a loss of cenesthesia, or a loss of a feeling of familiarity. For example, the neurocognitive models of the Capgras and Cotard syndromes have in common the belief that they are both based on various kinds of unusual experiences. These unusual experiences are thought to include affective or emotional experiences. Capgras' syndrome is possibly triggered by an abnormal affective experience in response to the sight of closed-person's face. Similarly, the Cotard syndrome may result from a general flattening of affective responses to external stimuli. The inferential stage can also differentiate between the two syndromes. Some empirical validation has already been obtained in Capgras' syndrome but not yet in Cotard's syndrome. This review illustrates that the historical descriptions of cenesthesia and cenesthopathy remain relevant in contemporary neurocognitive models and more generally suggests that the comprehension of quite complex phenomena like delusion requires a multidisciplinary approach.

Amnésie autobiographique isolée : une origine neurologique ?

Introduction The term “autobiographical memory” (AuM) refers to contextually bound experiences that occurred in a specific time, place, and affective setting. AuM is a component of memory commonly impaired in amnestic disorders. Alteration occurs rarely in isolation but rather in the setting of a larger memory impairment. Isolated AuM deficit is a controversial clinical entity, however, recently reported in the context of temporal lobe epilepsy. This study aims at characterizing this poorly documented clinical syndrome and at discussing its potential pathophysiological basis. Patients and methods We studied a group of three subjects with a history of pharmacosensitive epilepsy and severe AuM complaints. They all were submitted to a neuropsychological evaluation that included an extensive episodic memory assessment, along with wake/sleep electroencephalogram (EEG) and brain magnetic resonance imaging (MRI). Results We observed the following findings: preserved autonomy and intact global cognitive functioning; normal performance to standardized episodic memory assessment, contrasting with decreased performance to specific AuM evaluation; frontal and/or temporal epileptic activity on EEG; and normal structural brain MRI. Conclusion We reported on a group of patients exhibiting selective impairment of some components of personal memory, associated with interictal frontal and/or temporal abnormalities on EEG. To account for these findings, we hypothesise that interictal epileptic-related activity is impeding long-term consolidation or storage of autobiographical material.

Thoracic outlet syndrome.

Thoracic outlet syndrome (TOS) is one of the most controversial clinical entities in medicine. We provide a review of this difficult-to-treat disorder, including a brief overview, clinical presentations, surgical anatomy, treatment options, and outcomes. TOS represents a spectrum of disorders encompassing three related syndromes: compression of the brachial plexus (neurogenic TOS), compression of the subclavian artery or vein (vascular TOS), and the nonspecific or disputed type of TOS. Neurovascular compression may be observed most commonly in the interscalene triangle, but it also has been described in the costoclavicular space and in the subcoracoid space. Patients present with symptoms and signs of arterial insufficiency, venous obstruction, painless wasting of intrinsic hand muscles, paresthesia, and pain. A careful and detailed medical history and physical examination are the most important diagnostic tools for proper identification of TOS. Electromyography, nerve conduction studies, and imaging of the cervical spine and the chest also can provide helpful information regarding diagnosis. Clinical management usually starts with conservative treatment including exercise programs and physical therapy; when these therapies fail, patients are considered for surgery. Two of the most commonly used surgical approaches are the supraclavicular exposure and the transaxillary approach with first rib resection. On occasion, these approaches may be combined or, alternatively, posterior subscapular exposure may be used in selected patients. TOS is perhaps the most difficult entrapment neuropathy encountered by neurosurgeons. Surgical intervention is indicated for vascular and true neurogenic TOS and for some patients with the common or nonspecific type of TOS in whom nonoperative therapies fail. With careful patient selection, operative intervention usually yields satisfactory results.

The anterior and superior malleal ligaments in otosclerosis: a histopathologic observation.

The aim of this study was to assess, in otosclerosis, whether the anterior and superior malleal ligaments show histologic changes that can lead to reduced malleal mobility and eventual fixation, and also to evaluate whether these changes are related to the degree of histologic otosclerosis. Fixation of the malleus seems to be one of the most controversial clinical entities in the acquired condition of otosclerosis. It has even been postulated that persistent conductive deafness, or progression of conductive deafness after initial improvement after stapedotomy, could be due to unsuspected malleus fixation. Fifty eight temporal bones with known otosclerosis and 43 normal temporal bones were selected. In addition, 10 temporal bones of fetuses and children were also studied. Otosclerosis of the footplate and otic capsule was graded as none, mild, moderate, and severe. The histologic changes in the ligaments also were graded from none to severe. The median ages of patients in the otosclerotic and normal groups were 62 and 60 years, respectively. In the anterior malleal ligament of the otosclerotic bones, 10% mild (+), 60% moderate (++), and 30% severe (+++) degrees of hyalinization were observed. In the anterior malleal ligament of the nonotosclerotic bones, 14% showed no hyalinization, 24% had only a tinge of hyalinization (minimal), 51% had mild (+) hyalinization, and 11% had moderate (++) hyalinization. Superior ligament hyalinization appears to be related to the severity of anterior ligament hyalinization. The severity of otosclerosis in the footplate or the otic capsule did not appear to be related to the severity of hyalinization. From this study, it is apparent that otosclerotic bones have a significantly high incidence of hyalinization of the anterior malleal ligament. This seems to be related to the duration rather than the severity of otosclerosis. It is important to properly evaluate malleal mobility during all stapes surgery.

Magnetic resonance imaging findings in piriformis syndrome: A case report

Piriformis syndrome (PS) is an unusual cause of sciatica that, because of the lack of strict diagnostic criteria, remains a controversial clinical entity. The diagnosis of PS is still primarily clinical because no diagnostic tests have proven to be definitive. We report the case of a 30-year-old woman, affected by a severe scoliosis, who developed a persistent buttock pain resembling that of PS. The clinical suspicion was confirmed by magnetic resonance imaging (MRI) of the pelvis, which showed an enlargement of the left piriformis muscle with an anterior isplacement of the sciatic nerve. The role of MRI in the diagnosis, clinical definition, and therapeutic approach to PS is discussed.