Ossicular erosions in the dry ear: CT diagnosis

Erosions of the ossicular chain that occurred as a complication of noncholesteatomatous chronic otitis media were studied with computed tomography (CT) in 55 patients. The incus (particularly the long and lenticular processes) was the ossicle most commonly involved (50 cases). Coronal and axial CT sections were complementary in the diagnosis of these erosions. Fibrous replacement of the incudostapedial articulation was diagnosed in four cases on axial CT scans when an unusually wide joint was present.     

Absent CNKSR2 causes seizures and intellectual,attention, and language deficits

Synaptic function is central to brain function. Understanding the synapse is aided by studies of patients lacking individual synaptic proteins. Common neurological diseases are genetically complex. Their understanding is likewise simplified by studies of less common monogenic forms. We detail the disease caused by absence of the synaptic protein CNKSR2 in 8 patients ranging from 6 to 62 years old. The disease is characterized by intellectual disability, attention problems, and abrupt lifelong language loss following a brief early childhood epilepsy with continuous spike‐waves in sleep. This study describes the phenotype of CNKSR2 deficiency and its involvement in systems underlying common neurological disorders. Ann Neurol 2014;76:758–764     

Prevalence of a history of prior varicella/herpes zoster infection in multiple sclerosis

Varicella zoster virus (VZV) infection has been implicated in multiple sclerosis (MS), but direct causal involvement has been disputed. Nevertheless, knowledge of VZV exposure is important, given the risk of serious complications of first exposure while undergoing immunosuppressive treatment, in particular with fingolimod. We distributed questionnaires to MS clinic patients, requesting information about history of chickenpox, sibling/household/occupational exposure, history of zoster (shingles), and disease-modifying treatment. A random, proportionally representative sample of 51 patients that included patients with positive, negative, and unknown chickenpox history were selected for determination of VZV IgG by ELISA. Of 1206 distributed questionnaires, 605 were returned (50% response rate). Of these, 86% reported history of chickenpox, 5.6% gave negative history, and 8.5% did not know. Of 594 who answered the zoster question, 78% gave a negative response, 4% did not know, and 104 (17%) answered yes. Of these, 83 reported 1 episode; 12 had 2; 5 had 3; and 1 each reported 5, 6, and 15 episodes. Of 51 patients tested for VZV IgG (44 “yes,” 4 “no,” and 3 “I don’t know” answers to the question of whether they had chickenpox), 48 were seropositive; the 3 seronegative all had reported having had chickenpox. The high rate of MS patients reporting prior chickenpox infection is comparable with previous reports. A substantial proportion of MS patients, estimated to be higher than an age-matched general population, report single or multiple episodes of zoster. These data are useful for consideration of immunosuppressive treatments and/or VZV and zoster vaccination.     

Tracheal Stenting has Minimal Impact on Survival in Anaplastic Thyroid Carcinoma

Background

Anaplastic thyroid carcinoma (ATC) is the most aggressive form of thyroid cancer and it has an extremely poor prognosis. Rapid airway compromise is the main cause of death in patients with ATC.

Methods

The present study is based on a retrospective review of clinical data of patients with ATC treated over the last decade in a tertiary referral center.

Results

Between January 1998 and December 2010, 31 patients (13 males: 18 females) with a mean age of 74 years (range: 54–90 years) had a diagnosis of ATC made on fine-needle aspiration (n = 29) or biopsy (n = 2). Eight patients underwent total thyroidectomy and lymph node dissection, and five patients had attempted surgery but the procedure was abandoned because of inoperability. Airway compromise was clinically apparent in 11 patients. Five patients had tracheal stents inserted, with a median survival of 2 months (range: 1–36 months). In the whole cohort, 29 patients died of the disease, with a mean survival of 7 months (range: 1–36 months).

Conclusions

Survival of patients with undifferentiated thyroid cancer is severely compromised by airway obstruction. Palliation with tracheal stenting can rescue catastrophic airway obstruction but offers minimal survival benefit.     

Post‐mastectomy Radiotherapy for pT3N0 Breast Cancers: A Retrospective,Multi‐Institution Review

The role of post‐mastectomy radiotherapy for pT3N0 breast cancers remains undefined. The purpose of this study was to report institutional outcomes for women with pT3N0 breast cancers treated with and without post‐mastectomy radiotherapy. We collected data from two large tumor registries on pT3N0 breast cancers diagnosed between 1985 and 2014. Kaplan–Meier estimates were used to analyze freedom from local‐regional recurrence (FFLR), relapse free survival, and overall survival. This analysis identified 93 women with pT3N0 breast cancers. Of these, 53 received post‐mastectomy radiotherapy and 40 did not. Median follow‐up was 6.2 years and 5.3 years in the non‐post‐mastectomy radiotherapy and post‐mastectomy radiotherapy cohorts, respectively. Women not undergoing post‐mastectomy radiotherapy were more likely to be diagnosed in the 1980s and 1990s and were less likely to receive systemic therapies than women receiving post‐mastectomy radiotherapy (p < 0.05). There was a trend toward increased FFLR in the women receiving post‐mastectomy radiotherapy (p = 0.15). FFLR in the post‐mastectomy radiotherapy cohort was 98% at both 5 and 10 years. For women not receiving post‐mastectomy radiotherapy, FFLR was 88% at both 5 and 10 years. Women not receiving post‐mastectomy radiotherapy in our study had an isolated local‐regional failure rate of 12% at 10 years, despite receiving inferior systemic treatment by current standards. Local‐regional control after post‐mastectomy radiotherapy for pT3N0 breast cancers was excellent. Further research is needed to define post‐mastectomy radiotherapy indications for this patient population when receiving chemotherapy and endocrine therapy in line with current guidelines.     

Une forme limite de délire et son rapport au conflit psychique

The relation between delirium and psychic conflict is considered from a psycho-analytical angle, using a clinical vignette as a starting point. The patient has a particular form of delirium: he claims a bond with a dead girl, whom he has never met. He was observed during several years in individual psychoanalytic psychodrama. His delirium is first discussed as a symptom. Secondly like his bond with the dead person, his acoustic hallucinations - mocking voices - reflect his difficult relationship with his father, also dead. At a third level, the issue of psychic conflict in psychosis is raised, because of the contradictions in the patient's mental functioning. Thus, the conflict can be located at different levels, as a consequence of splitting, or as a manifestation of instinctual defusion. But above all, the patient's delirium seems to hide an identification conflict. Piera Aulagnier also pointed out the importance of identification difficulties in psychosis. From a metapsychological stance, Freud regarded the conflict between Ego and reality as the characteristic for psychosis. This difference of outlook is treated here as a problem whose solution consists in situating the object on the same side as reality. The conflict with reality thus also becomes a conflict with the object, which is the root cause of the psychotic's identification difficulties.     

Levels of brain related proteins in cerebrospinal fluid: an aid in the differential diagnosis of parkinsonian disorders

Parkinsonian disorders such as Parkinson's disease (PD), multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD), are a large group of common neurodegenerative diseases. The initial differential diagnosis can be extremely challenging with major implications for prognosis. The 42 amino acid fragment of amyloid-beta (Abeta42), neurofilament light chain (NFL), neurofilament heavy chain (pNFH), tau protein, glial fibrillary acidic protein (GFAP), neuron specific enolase (NSE), S-100B protein, and myelin basic protein (MBP) are brain related proteins (BRP) present in neurons and glia cells. They are released in the cerebrospinal fluid (CSF) after brain tissue damage caused by a variety of neurological diseases, including the parkinsonian disorders. A review of the literature shows that, carefully interpreted, the CSF levels of BRP can be of value in the differential diagnosis of parkinsonian disorders.     

Diagnosis of laryngopharyngeal reflux

PURPOSE OF REVIEW: Laryngopharyngeal reflux is of great interest to otolaryngologists, speech and language therapists, and gastroenterologists. This is a brief review of recent publications in the diagnosis of laryngopharyngeal reflux. RECENT FINDINGS: Otolaryngologic signs and symptoms can be found in 4 to 10% of patients with gastroesophageal reflux and those presenting for ear, nose, and throat evaluations. Laryngeal signs are not pathognomonic for laryngopharyngeal reflux because many of these signs can be found in healthy volunteers. A combination of signs and symptoms should be sought before suspecting this diagnosis. Most investigators consider pH monitoring the best currently available instrument to diagnose gastroesophageal reflux, even though it is not considered to be 100% sensitive and specific. Studies in normal volunteers indicate that a minimal number of reflux episodes reach the hypopharynx. The correlation between laryngeal signs and symptoms and pH-documented reflux is less than perfect, whereas the combination of pH testing and signs and symptoms is better in detecting patients with a favorable response to acid-suppressing therapy. Using an empiric trial of high-dose proton pump inhibitors over a prolonged period of time to diagnose laryngopharyngeal reflux is supported mainly by uncontrolled studies. To date, double-blind, placebo-controlled studies suggest that empiric trials of proton pump inhibitors may not have high accuracy for the diagnosis of laryngopharyngeal reflux. SUMMARY: Multidisciplinary trials are needed to establish the optimal combination of sign and symptom scores, reflux monitoring results, and empiric treatment trials for the most accurate diagnosis of laryngopharyngeal reflux.