The Premenstrual Syndrome and Fibromyalgia—Similarities and Common Features

The aim of the study was to assess the clinical similarities and common features of fibromyalgia syndrome (FM) and premenstrual dysphoric syndrome (PMDD). Thirty young patients who met the diagnostic criteria for PMDD were included in the study and compared to 26 women belonging to the medical staff of a general psychiatry department. All enrollees were interviewed and examined by a skilled physician. They completed the following nine survey items: demographic information, clinical health assessment questionnaire, fibromyalgia impact questionnaire, sleep and fatigue questionnaires, Sheehan disability scales, SF-36 assessment for quality of life, visual analog scale for pain, Mini International Neuropsychiatric Interview (MINI) questionnaire (assessment of coexistent psychiatric conditions), and the premenstrual severity scale. Additionally, each individual underwent a physical examination measuring the classical tender points and was asked to describe the distribution and continuum of her pain or tenderness. The PMDD group scored significantly higher in the measures pain and tenderness as well as in severity of premenstrual symptoms compared to the control group. Five patients in the PMDD group and none in the control group had FM. Quality of life measured by the SF-36 was higher in the control group than in the PMDD group and correlated with the degree of tenderness reported. Psychiatric comorbidity was significantly more common in the PMDD group, affecting 16 of the 30 PMDD patients compared to only three of the 26 control patients. In this study, patients with PMDD were found to have higher levels of tenderness, higher psychiatric comorbidity, greater level of physical disabilities, and a lower quality of life. These parameters were highly correlated with a lower pain threshold.     

Adrenocortical Oncocytoma Presenting as Cushing’s Syndrome: An Additional Report of a Paediatric Case

Oncocytomas are tumours predominantly or exclusively composed of oncocytes, cells with granular and eosinophilic cytoplasm filled with mitochondria. Although they can occur in every organ, they are rare in adrenal glands, and in paediatric patients they are even rarer, with only three case reports previously published. We present a preschool child developing Cushing’s syndrome due to an adrenocortical oncocytoma, which was confirmed immunohistochemically with antibodies to the mitochondrial electron complex 2. A 5.8-year-old girl presented with clinical features of Cushing’s syndrome. ACTH-independent hypercortisolism was confirmed biochemically and a left adrenal mass was detected by imaging and removed by laparotomy. Histopathological analysis revealed a tumour composed of more than 95 % of oncocytes, confirmed immunohistochemically with antibodies to subunits A and B of the mitochondrial enzyme succinate dehydrogenase. Using the Lin–Weiss–Bisceglia score system and the reticulin algorithm, this tumour was categorized as a benign adrenocortical oncocytoma. The patient currently has 64 months of follow-up, without any evidence of relapse of symptoms. To our knowledge, we herein present the youngest patient developing an adrenocortical oncocytoma and the first manifestation of Cushing’s syndrome due to this rare neoplasm in paediatric patients. We also emphasize the clinical usefulness of immunohistochemistry to the mitochondrial enzyme succinate dehydrogenase to confirm the oxyphilic nature of adrenocortical oncocytomas.     

Fibromyalgia: a stress disorder? Piecing the biopsychosocial puzzle together

Fibromyalgia (FM) is a controversial syndrome, characterised by persistent widespread pain, abnormal pain sensitivity and additional symptoms such as fatigue and sleep disturbance. The syndrome largely overlaps with other functional somatic disorders, particularly chronic fatigue syndrome (CFS). Although the exact aetiology and pathogenesis of FM are still unknown, it has been suggested that stress may play a key role in the syndrome. This article first reviews the function of the stress response system, placing special emphasis on the relationships between adverse life experiences, stress regulation and pain-processing mechanisms, and summarising the evidence for a possible aetiopathogenetic role of stress in FM. Finally, an integrative biopsychosocial model that conceptualizes FM as a stress disorder is proposed, and the clinical and research implications of the model are discussed.     

Does Restless Legs Syndrome increase cardiovascular risk in Attention-Deficit/Hyperactivity Disorder?

Preliminary evidence suggests a possible association between Attention-Deficit/Hyperactivity Disorder and Restless Legs Syndrome with or without Periodic Limb Movements during Sleep. When comorbid, Restless Legs Syndrome/Periodic Limb Movements during Sleep might aggravate Attention-Deficit/Hyperactivity Disorder symptoms. Pharmacological treatment of Attention-Deficit/Hyperactivity Disorder may be associated, at least in some cases, with adverse cardiovascular events, including clinically significant elevation in heart rate and systemic blood pressure. However, the characteristics of patients with Attention-Deficit/Hyperactivity Disorder at risk for cardiovascular events during pharmacological treatment are poorly understood. Here, we hypothesize that Restless Legs Syndrome and/or Periodic Limb Movements during Sleep comorbid with Attention-Deficit/Hyperactivity Disorder increase cardiovascular risk via imbalance in activity of the autonomic nervous system. Such an imbalance of the could be related to alterations of sleep microarchitecture also detected by cyclic alternating pattern analysis. If empirical studies confirm our hypothesis, the clinician would be advised to systematically screen for and effectively treat Restless Legs Syndrome/Periodic Limb Movements during Sleep even before starting treatment with Attention-Deficit/Hyperactivity Disorder drugs. The management of Restless Legs Syndrome/Periodic Limb Movements during Sleep might reduce cardiovascular risk during pharmacological treatment of Attention-Deficit/Hyperactivity Disorder.     

My Voice or Yours? An Electrophysiological Study

This study examined the neural processes underlying own voice discrimination using electrophysiological methods. Event-related potentials were recorded while healthy subjects (n = 17) heard passively three oddball sequences composed of recordings of the French vowel/a/pronounced either by the participant her/himself or by two unknown persons. The results indicated that, although the mismatch negativity (MMN) displayed similar peak latency and amplitude in both conditions, the subsequent P3a clearly distinguished the two conditions since its amplitude was significantly smaller for own voice discrimination than for that of unknown voices. Moreover, the own voice discriminative response was associated with an early pre-MMN response. This early response involved a left inferior frontal component, the activity of which lasted throughout the time course of the discriminative response, which included both MMN and P3a.     

Experiences and lessons learned as a Chair of anatomy—An 18-year journey

In 1998, I was appointed Chair of the Department of Anatomy at Monash University in Melbourne, Australia. On commencing as Chair, I had three main goals: (a) to maintain and extend the high quality of anatomy teaching in the medical program; (b) to introduce significantly more developmental biology, cell biology, and neuroscience into our existing Bachelor of Science major in human anatomy; and (c) to establish an active research program in the department. Over the next 18 years, I worked with staff and students at all levels of the university to turn this vision into a reality, with the Monash Department of Anatomy and Developmental Biology now arguably the top ranked anatomy department in Australia. During my tenure, countless challenges were faced and while some errors were made, and a good number of goals were never realized the general outcome was a vibrant scholarly environment where that rich nexus of research and teaching was realized. This personal account provides some insights into that 18-year journey, which I hope may prove useful for current and future Chairs of anatomy. For me personally, it was definitely a journey worth taking.     

An FBN1 Deep Intronic Mutation in a Familial Case of Marfan Syndrome: An Explanation for Genetically Unsolved Cases?

Marfan syndrome (MFS) is caused by mutations in the FBN1 (fibrillin‐1) gene, but approximately 10% of MFS cases remain genetically unsolved. Here, we report a new FBN1 mutation in an MFS family that had remained negative after extensive molecular genomic DNA FBN1 testing, including denaturing high‐performance liquid chromatography, Sanger sequencing, and multiplex ligation‐dependent probe amplification. Linkage analysis in the family and cDNA sequencing of the proband revealed a deep intronic point mutation in intron 56 generating a new splice donor site. This mutation results in the integration of a 90‐bp pseudo‐exon between exons 56 and 57 containing a stop codon, causing nonsense‐mediated mRNA decay. Although more than 90% of FBN1 mutations can be identified with regular molecular testing at the genomic level, deep intronic mutations will be missed and require cDNA sequencing or whole‐genome sequencing.     

Is the Inattentive Type of ADHD a Separate Disorder?

The contention of Milich, Balentine, and Lynam that the inattentive type of ADHD is a disorder distinct from and unrelated to the combined type bears scrutiny. After commenting on the optimal means of validating syndromes and disorders, I concur with Milich et al. that, under current definitions, the inattentive type subsumes both children who are subthreshold for the combined type and those who display sluggish cognitive tempo and underactivity. In all probability, only the latter subgroup may be distinct, in any qualitative sense, from current conceptions of ADHD. Yet with respect to a key criterion variable, cognitive processing/ neuropsychological functioning, there is currently little definitive evidence for clear differentiation. Until nosologies allow for better phenotypic differentiation, which includes broader sets of symptoms to index inatten-tiveness, research will continue to confound these two subgroups, precluding advances regarding possible genetic, psychobiologic, neuropsychologic, cognitive, familial, developmental, and treatment-related distinctions between them. Milich et al. have prompted needed research on diagnostic reformulations and external validation regarding this crucial aspect of developmental psychopathology.     

Sjögren’s Syndrome—Study of Autoantigens and Autoantibodies

The presence of autoantibodies is the hallmark of systemic autoimmune diseases. During the past 30 years, intense clinical and basic research have dissected the clinical value of autoantibodies in many autoimmune diseases and offered new insights into a better understanding of the molecular and functional properties of the targeted autoantigens. Unraveling the immunologic mechanisms underlying the autoimmune tissue injury, provided useful conclusions on the generation of autoantibodies and the perpetuation of the autoimmune response. Primary Sjögren’s syndrome (pSS) is characterized by the presence of autoantibodies binding on a vast array of organ and non-organ specific autoantigens. The most common autoantibodies are those targeting the Ro/La RNP complex, and they serve as disease markers, as they are included in the European–American Diagnostic Criteria for pSS. Other autoantibodies are associated with particular disease manifestations, such as anti-centromere antibodies with Raynaud’s phenomenon, anti-carbonic anhydrase II with distal renal tubular acidosis, anti-mitochondrial antibodies with liver pathology, and cryoglobulins with the evolution to non-Hodgkin’s lymphoma. Finally, autoantibodies against autoantigens such as alpha- and beta-fodrin, islet cell autoantigen, poly(ADP)ribose polymerase (PARP), NuMA, Golgins, and NOR-90 are found in a subpopulation of SS patients without disease specificity, and their utility remains to be elucidated. In this review, the molecular and clinical characteristics (divided according to their clinical utility) of the autoantigens and autoantibodies associated with pSS are discussed.     

Trauma and PTSD – An overlooked pathogenic pathway for Premenstrual Dysphoric Disorder?

Summary ¶Background: A recent epidemiological analysis on premenstrual dysphoric disorder (PMDD) in the community revealed increased rates of DSM-IV posttraumatic stress disorder (PTSD) among women suffering from PMDD. Aims: To explore whether this association is artifactual or might have important pathogenic implications. Methods: Data come from a prospective, longitudinal community survey of an original sample of N=1488 women aged 14–24, who were followed-up over a period of 40 to 52 months. Diagnostic assessments are based on the Composite International Diagnostic Interview (CIDI) using the 12-month PMDD diagnostic module. Data were analyzed using logistic regressions (odds ratios) and a case-by-case review. Results: The age adjusted odds ratio between PTSD and threshold PMDD was 11.7 (3.0–46.2) at baseline. 10 women with full PTSD and at least subthreshold PMDD were identified at follow-up. Most reported an experience of abuse in childhood before the onset of PMDD. Some had experienced a life-threatening experience caused by physical attacks, or had witnessed traumatic events experienced by others. 3 women reported more than one traumatic event. Conclusions: A case-by-case review and logistic regression analyses suggest that women with traumatic events and PTSD have an increased risk for secondary PMDD. These observations call for more in-depth analyses in future research.Received March 3, 2003; accepted August 4, 2003 Published online October 22, 2003     

Interleukin-16 as a Marker of S��zary Syndrome Onset and Stage

Introduction

Sézary syndrome is one of the most common forms of cutaneous T cell lymphoma (CTCL). It is characterized by skin infiltration of malignant T cells. We examined interleukin-16, a potent T cell chemoattractant and cell-cycle regulator, as a prospective marker of disease onset and stage.

Methods

The correlation of total intracellular interleukin-16 and surface CD26 was studied by flow cytometry. Confocal microscopy was performed to determine localization of interleukin-16 at different stages of the disease. The levels of interleukin-16 in plasma and culture supernatants were examined by enzyme-linked immunoassay. Additionally, lymphocytes from stage IB patients were cultured in the presence of interleukin-16 alone and in combination with interleukin-15, and their ability to survive and proliferate was determined by cell counts and [3H]TdR incorporation.

Results

The data indicate that loss of both nuclear and intracellular pro-interleukin-16 highly correspond to disease stage, with a concomitant increase in secreted mature interleukin-16 in both culture supernatants and patients?? plasma that peaks at stage IB. Loss of intracellular interleukin-16 strongly corresponded to loss of surface CD26, which has been shown to occur with more advanced stage of CTCL. Nuclear translocation of pro-interleukin-16 was not observed in late stages of Sézary syndrome, indicating this loss is not reversible.

Conclusions

We propose that it is feasible to use plasma levels of IL-16 as a potential diagnostic marker of Sézary syndrome and to use loss of intracellular IL-16 as a prognostic indicator of disease severity and stage.     

Pediatric Acute Respiratory Distress Syndrome in India: Time for Collaborative Study?

How to cite this article: Solomon R. Pediatric Acute Respiratory Distress Syndrome in India: Time for Collaborative Study? Indian J Crit Care Med 2022;26(8):896–897.