Secondary Disorders Research Articles

Depletion and Supplementation of Coenzyme Q10 in Secondary Deficiency Disorders

Coenzyme Q10 (CoQ10) deficiency is broadly divided into two types, primary and secondary. Primary CoQ10 deficiencies are relatively rare disorders resulting from mutations in genes directly involved in the CoQ10 biosynthetic pathway, and are not a subject of this article. Secondary CoQ10 disorders are relatively common, and may occur for a variety of reasons; these include mutations in genes not directly related to the synthetic pathway, oxidative stress induced reduction of CoQ10, and the effects of pharmacological agents such as statins. CoQ10 is of key importance in cell metabolism; in addition to its role in mitochondrial oxidative phosphorylation, it is a major endogenous antioxidant, and has a role in the metabolism of sulphides, lipids and amino acids. Given its importance in cell metabolism, it is unsurprising that secondary CoQ10 deficiency has been linked with a wide range of disorders. In this article, we have reviewed evidence of secondary CoQ10 deficiency in both common and less common disorders, and highlighted those disorders in which CoQ10 supplementation has been shown to be of significant clinical benefit.

Phenomenology, quality of life, and predictors of reversibility in patients with drug-induced movement disorders: a prospective study.

Drug-induced movement disorders (DIMDs) form an important subgroup of secondary movement disorders, which despite conferring a significant iatrogenic burden, tend to be under-recognized and inappropriately managed. We aimed to look into phenomenology, predictors of reversibility, and its impact on the quality of life of DIMD patients. We conducted the study in the Department of Neurology at a tertiary-care centre in India. The institutional ethics-committee approved the study. We assessed 55-consecutive DIMD patients at presentation to our movement disorder clinic. Subsequently, they followed up to evaluate improvement in severity-scales (UPDRS, UDRS, BARS, AIMS) and quality of life (EuroQol-5D-5L). Wilcoxan-signed-rank test compared the scales at presentation and follow-up. Binary-logistic-regrerssion revealed the independent predictors of reversibility. Fourteen patients (25.45%) had acute-subacute DIMD and 41 (74.55%) had tardive DIMD. Tardive-DIMD occurred more commonly in the elderly (age 50.73±16.92 years, p<0.001). Drug-induced-Parkinsonism (DIP) was the most common MD, followed by tardivedyskinesia. Risperidone and levosulpiride were the commonest culprit drugs. Patients in both the groups showed a statistically significant response to drug-dose reduction /withdrawal based on follow-up assessment on clinical-rating-scales and quality of life scores (EQ-5D-5L). DIMD was reversible in 71.42% of acute-subacute DIMD and 24.40% of patients with chronic DIMD (p=0.001). Binary-logistic-regression analysis showed acute-subacute DIMDs and DIP as independent predictors of reversibility. DIP is the commonest and often reversible drug-induced movement disorder. Levosulpiride is notorious for causing DIMD in the elderly, requiring strict pharmacovigilance.

Prevalence of stress disorders in frontline healthcare students

Emerging evidence indicates that some healthcare students are developing stress disorders, due to factors relating to their chosen field of study. If accurate, this could have a major impact not only on those students, but also on the professions into which they are entering. To understand the extent of the problem, a literature review was conducted to identify the overall prevalence of stress disorders in healthcare students who are exposed to occupational stress/trauma, and/or course-related stress. Two databases were searched to include studies reporting on the presence of primary or secondary stress disorders in healthcare students. Six studies were reviewed, which included primary outcomes of depression, anxiety and stress. The pooled prevalence of primary outcomes was 41.4%. Pooled prevalence of depression was 49.58%, anxiety 54.55% and stress 20.2%. High prevalence figures suggest the need for adequate provision of mental health resources.

STATE OF COCHLEOVESTIBULAR DISORDERS IN PATIENTS WITH ARTERIAL HYPERTENSION

The article presents the otoneurological characteristics of pre-stroke cerebrovascular disorders in patients with essential hypertension. Among the problems of modern otorhinolaryngology, one of the leading places belongs to cochleovestibular disorders in some cardiovascular diseases, in particular, hypertension. The state of vestibular function in arterial hypertension was studied by many authors who noted a decrease in resistance in such patients. It has been established that one of the reasons for the formation of secondary cochleovestibular disorders is chronic hypertensive encephalopathy, which develops in patients with arterial hypertension, often already in the early stages of the disease. In the study of the peculiarities of the pathogenesis of cochleovestibular disorders, certain results have made it possible to study the nature of the vestibulo-vascular interaction. It is shown, in particular, the difference in vestibulo-vascular reactions in the case of central and peripheral lesions of the vestibular analyzer, which is important for the differential diagnosis of these pathological conditions.

Cerebral palsy: review of epidemiology, etiology, clinical features, classification and prevention

Cerebral Palsy (CP) is a neurodevelopment disorder caused by improper brain development or harm to the developing brain and is the underlying cause of the most common motor disability in children. The clinical symptoms vary between subjects because the etiology is complex and can affect a variety of anatomical structures and each of these can lead to a different symptom. The motor dysfunction is often associated with sensory, perceptual, cognitive, communication and behaviour impairments as well as epilepsy and secondary musculoskeletal disorders which have a significant influence on the child’s quality of life, activity, and participation. The risk of developing CP is present in infants born preterm, but these children sum up less than 50% of cases. The factors that cause CP in children born at term are grouped in antenatal, perinatal, neonatal, some of them can be modified like alcohol consumption, maternal smoking, infections, but others like genetic factor cannot be modified. CP can be classified in different ways depending on the clinical manifestation. Throughout time classification was based on the type and distribution of motor anomalies, which often corresponded to the area of injury. Spastic subtypes, dyskinetic subtypes, and ataxic subtypes are the three basic forms of motor dysfunction. The most common conditions associated with CP are pain, intellectual disability, speech disorder, bladder control problems, epilepsy, and behaviour disorders. Early intervention is thought to be the most effective treatment for CP. As soon as the diagnosis is determined, rehabilitation treatment should begin. The earlier a rehabilitation intervention begins, the better the prospects of improving the child’s functional abilities and independence.

Фактори ризику, асоційовані з діабетичною нефропатією та їх клінічне значення

The purpose of the study was to highlight the most studied risk factors associated with the development and/or progression of diabetic nephropathy, with an emphasis on some important aspects that should be kept in mind by the physician. Materials and methods. Research materials are publications of national and foreign authors. The methods used were: system approach and system theoretical retrospective analysis of selected materials; generalization; medical and statistical method. Results and discussion. Timely diagnostics of diabetic nephropathy and elimination of risk factors of its development and progression are emphasized to be a difficult task of clinics of internal diseases particularly in conditions when amount of these risk factors is constantly growing. The most investigated risk factors associated with development and progression of diabetic nephropathy such as age, arterial hypertension, disorders of carbohydrate and lipid exchange, proteinuria are discussed in the article. Some peculiarities of correction of metabolic risk factors such as hyperglycemia and dyslipidemia as well as hemodynamic risk factors such as arterial hypertension and intraglomerular hypertension participated in development and progression of diabetic nephropathy are discussed with consideration of acting recommendations. Some aged and gender peculiarities of change of glomerular filtration rate as well as a role of arterial hypertension in progression of diabetic nephropathy through albuminuria are considered. A significance of glycosylated hemoglobin level as an independent risk factors of microalbuminuria is demonstrated. A role of secondary lipid exchange disorders due to type 2 diabetes mellitus in reduction of glomerular filtration rate and elevation of albumin/creatinine ratio as well as albuminuria presence is emphasized. A necessity of urinal detection of nephrin and podocin levels for an early diagnostics of diabetic nephropathy and monitoring of renal glomerular dysfunction in diabetes mellitus is discussed. Some risk factors related to appearance of albuminuria as well as association of albuminuria and proteinuria with glomerular and tubular structural changes in kidney are considered. A clinical significance of albumin/creatinine ratio calculation as an alternative to daily urinal protein concentration measuring for diabetic nephropathy screening is emphasized. Some risk factors related to close positive correlation with this ratio are considered. A role of genetic factors in the development of diabetic nephropathy with a participation of known today some genes candidates and a necessity of genealogical anamnesis definition to reveal a patient’s susceptibility to development of diabetic nephropathy is discussed. Conclusion. The contemporary recommendations for control of glycemia, correction of dyslipidemia and arterial hypertension which allow significantly reduce a risk of kidney impairment in diabetes mellitus or inhibit a progression of diabetic nephropathy in patients with albuminuria and proteinuria are given. A significance of detailed investigation of principles of development and progression of diabetic nephropathy for collaboration of effective diagnostic, treatment and preventive measures is emphasized

Incidence and Medical Hospitalization Rates of Patients With Pediatric Eating Disorders.

Objective Eating disorders typically onset in preadolescence and adolescence and cause negative mental and physical health sequelae over the life span. This study examined the incidence and medical hospitalization rates of pediatric eating disorders in an integrated health system in the United States. Methods This retrospective cohort study examined 4883 Kaiser Permanente Northern California members 8-18 years of age with an eating disorder diagnosis from January 2015 to June 2019. Medical hospitalizations include admissions at any of the 13 Kaiser Permanente Northern California hospitals with a primary or secondary eating disorder diagnosis. Results Incidence rates ranged between 177 and 205 per 100,000 adolescents per year. More than half the adolescents were non-White: 10.8% Asian, 4.3% Black, 26.7% Hispanic/Latinx, 8.4% multiracial, 0.3% Native American/Alaskan Native, and 0.5% Native Hawaiian/Pacific Islander. Thirteen percent had a body mass index (BMI) below the 5th percentile, 61.8% had a BMI between the 5th and the 84th percentiles, 19.7% had a BMI above the 85th percentile, and 5.6% had an unknown BMI. During the 12-month follow-up period, 5.4% of adolescents had medical hospitalizations. Conclusions This study adds to the evidence that eating disorders affect children/adolescents across all weight/BMI ranges and racial/ethnic backgrounds. Future studies call for exploration on treatment strategies that tailor to the diverse populations.

An Unusual Cause of Acute Gastroenteritis, Ascites, and Eosinophilia in a 29-Year-Old Woman

An Unusual Cause of Acute Gastroenteritis, Ascites, and Eosinophilia in a 29-Year-Old Woman

PSAT038 A Case of Spontaneous Atraumatic Unilateral Adrenal Hemorrhage

Abstract Background Adrenal hemorrhage is a rare disorder with an incidence of 0.1 to 1% for which a cause is identified in most cases. It is usually classified as traumatic or atraumatic, unilateral or bilateral and can result in serious consequences such as adrenal crisis and death if not diagnosed and treated properly. We describe a patient with an atraumatic unilateral adrenal hemorrhage without a known coagulopathy Case presentation: A 51-year-old man with history of COPD and Reynaud's phenomenon presented to the hospital for severe right upper quadrant pain. CT of the abdomen demonstrated a right adrenal hemorrhage of 5.2×4.0×2.6 cm in size, for which Endocrinology was consulted. The patient denied recent trauma or anticoagulation use. Physical examination exhibited generalized abdominal tenderness, without hypotension, or symptoms of acute adrenal insufficiency. Biochemical evaluation showed an AM Cortisol level of 14.3 ug/dL (4.2 - 22.4 ug/dL) and ACTH 67.1 pg/mL (7.2 - 63.3 pg/mL). MRI abdomen showed a decrease in size of the hemorrhage measuring 3.1×1.7×5.2 cm. He was referred to Hematology/Oncology to evaluate for coagulopathy which was unrevealing. About one month from hospital discharge, the patient reported voice hoarseness and was diagnosed with moderately differentiated keratinizing squamous cell carcinoma of larynx. Ten months later PET-CT scan showed hypermetabolic laryngeal mass with bilateral cervical lymphadenopathy without evidence of adrenal gland metastasis with resolution of adrenal hemorrhage, indicative that the hemorrhagic episode is unrelated to this newly diagnosed malignancy. Discussion Adrenal hemorrhage is a rare disorder that overall is associated with a 10-20% mortality rate, it is usually classified as traumatic and no traumatic. Most times it is caused by secondary disorders such as trauma, surgery, anticoagulants, infections, but in rare instances is idiopathic. Clinical manifestations of adrenal hemorrhages vary depending on extension of the hemorrhage and early diagnosis is crucial for better outcomes. As per current guidelines, assessment for adrenal insufficiency including hemodynamics, biochemical evaluation and imaging is paramount. To date, clear etiology of primary adrenal hemorrhage is lacking. This case demonstrated an unusual presentation of idiopathic unilateral adrenal hemorrhage without evidence of adrenal insufficiency. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

The correlation between anxiety, depression, and vertigo: a cross-sectional study

BackgroundDizziness, including vertigo, affects approximately 15% to over 20% of adults each year. A significant proportion of patients with vertigo develop secondary psychiatric disorders throughout their disease. On the other hand, patients with psychiatric disorders frequently report dizziness as a co-occurring symptom of their illness. The purpose of this study was to screen the prevalence of depression and anxiety indices in dizzy patients and their relation to the severity of their dizziness handicap by administering two different questionnaires: The dizziness handicap inventory (DHI) and the Hospital Anxiety and Depression Scale (HADS). ResultsPsychological assessments revealed that 49% of the study group was normal, 29% had anxiety, 7% had depression, and 15% had both anxiety and depression. Besides, vestibular neuritis was associated with a higher mean anxiety score, whereas Meniere's disease was associated with a higher mean depression score. ConclusionAs this study has shown, there was a statistically significant positive correlation between anxiety and depression scores and physical, emotional, functional, and total DHI scores. Screening and treatment of co-morbid mental health disorders are critical for appropriate management of vertigo disability that leads to a higher quality of life and fewer complications.

THE USE OF ELEMENTS OF ART THERAPY IN CORRECTIVE-DEVELOPING WORK WITH CHILDREN

The article is devoted to the current problem of modern special pedagogy - the use of elements of art therapy in corrective and developmental work, because these elements are considered the most acceptable and effective in working with children with special educational needs. Rozkriti Zmіst is an individual Vikoristannye Elementiv art-therapy in the core-nitrum robot with a special dedication, the yak is able to be in the urahuvannya ditini, the type of jerk off piston. The directions of using elements of art therapy in correctional and developmental work are highlighted: psychophysiological (related to the correction of psychosomatic disorders), psychotherapeutic (related to the impact on the cognitive and emotional spheres), social-psychological (performs catharsis of aesthetic, regulatory, communicative functions ), socio-pedagogical (related to the development of aesthetic needs, the expansion of the general and artistic-aesthetic horizons, and the activation of the child's potential in practical artistic activity and creativity). Various aspects of the use of elements of art therapy are analyzed as one of the most interesting and creative methods of work, which helps to use the available opportunities of art to achieve positive changes in the emotional, intellectual and personal development of a child with special educational needs. It is noted that the use of elements of art therapy in corrective and developmental work with children with special educational needs allows for more effective influence on the formation of the emotional sphere, encourages the further development of compensatory properties of preserved functional systems, and improves the child's s it possible to carry out a simultaneous corrective effect on both primary, secondary and tertiary disorders, as well as to maximally mobilize the child's creative potential and find those ways of creative self-expression that best correspond to his capabilities and needs.

Epidemiology of Disorders Associated with Short Stature in Childhood: A 20-Year Birth Cohort Study in Finland.

BackgroundMany primary and secondary disorders disturb growth and cause short stature (height below −2 SDS) in childhood. Growth monitoring programs aim at their early detection but are not evidence-based: epidemiology of childhood growth disorders is poorly characterized, and no consensus exists on priority target conditions. Herein, we describe population-based epidemiological data on several primary and secondary growth disorders associated with short stature in childhood.Materials and MethodsThis retrospective population-based 20-year birth cohort study examined 1 144 503 children (51% boys) born in Finland between 1998 and 2017, with 16.5 million care notifications including medical diagnoses. The first occurrences of key primary or secondary growth disorders were identified in multiple registers. Median ages at diagnosis (MAD), and age- and sex-specific cumulative incidences (CMI) from birth until 16 years of age were determined.ResultsTurner syndrome was the most common primary growth disorder (CMI 52 per 100 000 at 16 years, MAD 4.0 years). Most primary growth disorders were diagnosed before the age of 4 years, and thereafter, secondary growth disorders increased in number. MAD of growth hormone deficiency (GHD) was 8.7 (boys) and 7.2 years (girls). At 16 years, the CMI of GHD was higher in boys than in girls (127 versus 93 per 100 000, respectively), whereas the CMI of hypothyroidism was higher in girls (569 versus 306 per 100 000). Celiac disease was the most common secondary growth disorder and more common in girls than in boys (988 versus 546 per 100 000 at 16 years, respectively).ConclusionThese population-based epidemiological data indicate that childhood growth monitoring should be age- and sex-specific. In the early childhood, the focus should be on primary growth disorders, and from preschool age also on secondary growth disorders. These results provide evidence for improving growth monitoring programs and diagnostic practices targeting on Turner syndrome, GHD, hypothyroidism, and celiac disease.

S2466 Pelvic Floor Dysfunction as a Cause of Chronic Intestinal Pseudo Obstruction

Introduction: Chronic intestinal pseudo-obstruction (CIPO) is a rare motility disorder with a variety of secondary causes, including genetics, metabolic disease, paraneoplastic syndromes, inflammatory disorders, and neurologic disorders. We present a case of CIPO secondary to pelvic floor dyssynergia. Case Description/Methods: A 64 year-old male with a past medical history of hypertension, atrial tachycardia, and heavy alcohol use was referred for further GI workup by his primary care physician following a 5 month history of worsening constipation after a viral infection. Prior to the infection, he had a bowel movement 2-3 times per week. However, he now describes one Bristol 4 or 7 bowel movement with straining per month. Symptoms were refractory to Dulcolax and Colace. Initial workup included a normal extended electrolyte panel, normal TSH, and negative Celiac disease serologies. A CT abdomen and pelvis revealed extensive dilation of proximal colon, compression of descending and sigmoid colon, and small bowel fecalization. A representative coronal image is shown in Figure 1. Subsequent colonoscopy did not reveal any evidence of obstruction. Anorectal manometry (ARM) findings included elevated resting pressure (74.94 mmHg), decreased sensation, paradoxical contraction with Valsalva, and inability to pass the balloon catheter after 2.5 minutes. Normal rectal capacity (280 mL) and excellent squeeze pressure but paradoxical contraction with Valsalva is consistent with pelvic floor dyssynergia. Biofeedback therapy was recommended. Discussion: Evaluating for secondary causes of CIPO (i.e., obstruction, metabolic disturbances, autoimmune disorders, neurologic disorders, and musculoskeletal disorders) is an important diagnostic step. Key tests include imaging, colonoscopy, electrolytes and autoimmune panels, and anorectal manometry. This case highlights long-standing pelvic floor dyssynergia as an underlying cause of CIPO.Figure 1.: CT abdomen and pelvis with extensive dilation of proximal colon and small bowel fecalization.

Active transport nanochelators for the reduction of liver iron burden in iron overload.

Liver dysfunction and failure account for a major portion of premature deaths in patients suffering from various iron associated pathogeneses, particularly primary and secondary iron overload disorders, despite intensive treatment. The liver is a central player in iron homeostasis and a major iron storage organ, and currently, there are no active approaches for the excretion of excess liver iron. Herein, we report a new method for the rapid reduction of iron burden in iron overload diseases by developing a new class of liver targeted nanochelators with favorable pharmacokinetics and biodistribution. The new nanochelators bypass the reticuloendothelial system and specifically target hepatocytes without non-specific accumulation in other organs. The targeted nanochelators bound and neutralized excess iron in the liver and from the vasculature and, eventually leading to rapid hepatobiliary excretion of labile iron. Further, these rapidly excreted nanochelators did not induce toxicity in the liver, were highly cytocompatible in both iron overload and non-loaded conditions, and were promising in mitigating iron triggered free radical oxidative damage. These studies provide key insights into the development of organ targeted nanochelating systems and the rapid reduction of iron burden in vivo. This methodology allows for further development of nanotherapeutics for specific iron overload diseases.

Headache and acute cerebrovascular disease: How do we differentiate primary and secondary headache disorders in the emergency setting?

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Судинні порушення у хворих на ревматичні хвороби після перенесеного COVID-19

The article briefly summarizes data on the role of micro- and macrocirculatory disorders in the pathogenesis of COVID-19. Focused attention on similarities of clinical and pathogenetic features of rheumatic diseases, COVID-19 and its complications. The review analyzed and compared the data of the assessment of the main instrumental studies of the functional state of blood vessels in patients with rheumatic diseases in general and in patients with rheumatic diseases who suffered from COVID-19 (capillaroscopy and occlusion test). A conclusion was made about the presence of endothelial dysfunction in rheumatic diseases and its features in patients with rheumatic diseases after transmission of COVID-19. This leads to the opinion about the expediency of timely detection of secondary disorders of the functional state of blood vessels in patients with rheumatic diseases who are convalescents of COVID-19, and justifies the use of an occlusion test for this purpose. No conflict of interests was declared by the authors.

Patient-reported long-term outcome following allogeneic hematopoietic stem cell transplantation in pediatric chronic myeloid leukemia.

BackgroundPediatric CML is very rare. Before the introduction of tyrosine kinase inhibitors (TKIs), allogeneic hematopoietic stem cell transplantation (HSCT) from a donor -if available- was the standard cure attempt. Data on the long-term outcome and health-related quality of life (HRQOL) in former pediatric CML patients undergoing HSCT are lacking.Study questionWe investigated long-term survivors’ self-reporting to a questionnaire sent out to patients formerly enrolled in pediatric CML-HSCT trials.MethodsIndividuals with CML transplanted at age <18 years were identified from the German Childhood Cancer Registry database. Long-term survivors received a questionnaire based on the SF-36 and FACT-BMT asking them to self-report HRQOL issues. (Ethical vote #541_20 B, Medical Faculty, University of Erlangen-Nürnberg).Results111/171 (64.9%) individuals survived HSCT long-term and 86/111 (77.5%) fulfilled all inclusion criteria and received the questionnaire. 37/86 (43%) participants (24 female, 13 male, median age at HSCT 12 years [range 2-18], median age at the time of the survey 29 years [range 18-43]) responded after a median follow-up period of 19 years (range 4-27) after HSCT. 10/37 (27%) participants underwent no regular medical follow-up examinations. Self-reported symptoms like chronic graft-versus-host disease (cGvHD)-associated organ impairments and conditioning regimen consequences could causatively not sharply be separated in each case. Complains comprised hypothyroidism (N=11, 30%), infertility (N=9, 24%), lung problems, dry eyes (each N=7, 19%), skin alterations (N=6, 17%), hair problems (N=4, 11%), and sexual dysfunction (N=3, 9%). 10 (27%) participants experienced 13 CML relapses after a median interval from HSCT of 31 months (range 2-93). Only one patient underwent 2nd SCT after failure of relapse treatment with TKIs. Six secondary malignancies (dysplastic melanocytic nevus and ALL, basal cell carcinoma (N=2), rhabdomyosarcoma, and thyroid carcinoma developed in 5 (13%) participants. As assessed by the SF-36 questionnaire, impaired physical health was mainly associated with cGvHD. The mental component summary score showed that also participants without cGvHD scored significantly lower than the general population. When assessed by the FACT-BMT, participants with cGvHD scored significantly lower while participants without cGvHD scored even 5 points higher than the data from controls. 18 (49%) participants considered the sequelae of HSCT an obstacle to education. Out of the total cohort, N=20 (54%), N=7 (19%), N=5 (14%), and N=4 (11%) participants worked full time, part-time, were unemployed, or had not yet finalized their education, respectively. 20 (54%) participants lived as singles, 8 (22%) lived in a partnership, 6 (16%) were married, and 3 (8%) had been divorced. Four (11%) participants reported a total number of 7 children.ConclusionThis first assessment of HRQOL in former pediatric patients with CML surviving HSCT for more than two decades demonstrates self-reported satisfactory well-being only in the absence of cGvHD. Research-based on self-reported outcomes sheds light on former patients’ perspectives and provides an additional layer of valuable knowledge for pediatric and adult hematologists. Regular follow-up examinations are mandatory helping to avoid that late secondary neoplasias, CML-relapse, and disorders forming the broad range of possible long-term consequences of HSCT are not detected too late.

Gaps, Controversies, and Proposed Roadmap for Research in Poststroke Movement Disorders

Poststroke movement disorders (PSMDs) are a common cause of secondary movement disorders. Although prior studies have highlighted the clinical spectrum and phenomenology of PSMDs, there are many knowledge gaps worth addressing. Some of the most important include lack of clinical definitions, variable stroke symptom latencies, and lack of biomarkers for vulnerability for or resilience against developing PSMDs. Collectively, the association between stroke localization and phenomenology is less than 30%, and the long-term clinical prognosis and treatment responses are highly variable. After summarizing the accumulated evidence regarding the phenomenology, pathophysiology, neuroimaging, and treatment of PSMDs, highlighting the many gaps and controversies including diagnostic challenges, we propose a roadmap for future research to fill these gaps and resolve the related controversies. More research is warranted concerning the phenomenology, classification, diagnostic criteria, and pathophysiology of PSMDs. Further, there is an urgent need for treatment guidelines for the management of PSMDs. © 2022 International Parkinson and Movement Disorder Society.

What respiratory physicians should know about narcolepsy and other hypersomnias.

Narcolepsy and related central disorders of hypersomnolence may present to the sleep clinic with excessive daytime sleepiness. A strong clinical suspicion and awareness of the diagnostic clues, such as cataplexy, are essential to avoid unnecessary diagnostic delay. This review provides an overview of the epidemiology, pathophysiology, clinical features, diagnostic criteria and management of narcolepsy and related disorders, including idiopathic hypersomnia, Kleine-Levin syndrome (recurrent episodic hypersomnia) and secondary central disorders of hypersomnolence.

Headache and Autonomic Dysfunction: a Review.

We explore the anatomy of the central and peripheral autonomic pathways involved in primary headache as well as the mechanisms for secondary headache associated with disorders of the autonomic nervous system. The prevalence and clinical presentation of cranial and systemic autonomic symptoms in these conditions will be discussed, with a focus on recent studies. Several small studies have utilized the relationship between headache and the autonomic nervous system to identify potential biomarkers to aid in diagnosis of migraine and cluster headache. Headache in postural orthostatic tachycardia syndrome (POTS) has also been further characterized, particularly in its association with orthostatic headache and spontaneous intracranial hypotension (SIH). This review examines the pathophysiology of primary and secondary headache disorders in the context of the autonomic nervous system. Mechanisms of headache associated with systemic autonomic disorders are also reviewed.