Clinical Features and Prognosis in Chinese Patients With Dipeptidyl–Peptidase–Like Protein 6 Antibody–Associated Encephalitis

Objectives:Anti-dipeptidyl–peptidase–like protein 6 (anti-DPPX) encephalitis an extremely rare type of immune-mediated encephalitis. This study aimed to analyze the electroclinical characteristics and prognosis of anti-DPPX encephalitis.Methods:Five patients (all male) with anti-DPPX encephalitis in East China from January 2016 to October 2021 was retrospective analyzed. Electroclinical features and outcomes were reviewed.Results:All five patients were male. The media age at disease onset was 32 years old with a range of 14–56 years. The main symptoms included psychiatric disturbances (2/5), amnesia (4/5), confusion (3/5), and seizures (3/5). Migrating myoclonus were identified in patient 4 with positive DPPX and contactin-associated protein-like 2 antibodies in blood. All of the patients had positive DPPX antibodies in serum. Only one of them had positive antibody in the cerebrospinal fluid. EEG showed diffuse slowing in two patients, but no epileptiform discharges were observed. Eighty percent (4/5) of the patients showed normal brain magnetic resonance imaging. After immunotherapy, improvement of neuropsychiatric symptoms from all of the patients was observed. Over a mean follow-up of 30.8 weeks, all of the patients had marked improvement in the modified Rankin Scale. To date, no tumors were not observed in any patients.Conclusions:Anti-DPPX encephalitis mainly presents as neuropsychiatric symptoms. Cooperation of DPPX antibodies and CASPR2 antibodies might have contributed to the migration of myoclonus in the patient 4. Prompt immunotherapy often results in improvement.

Papillary craniopharyngioma: a type of tumor primarily impairing the hypothalamus. A comprehensive anatomo-clinical characterization of 350 well-described cases

Objective: Papillary craniopharyngiomas (PCPs) represent a rare histological variant of craniopharyngiomas (CPs) usually involving the hypothalamus. This study systematically analyzes the clinical-anatomical correlation between tumor topography and symptoms related to hypothalamic dysfunction in the largest series of PCPs ever gathered. Methods: From 5,346 CP reports published from 1856 to 2021, we selected 350 well-described cases of the squamous-papillary type. Clinical presentation, tumor topography, severity of hypothalamic adhesion, patient outcome and tumor recurrence were thoroughly analyzed. Results: PCPs predominantly occur in adult (96.3%), male (61.7%) patients presenting with headache (63.4%), visual alterations (56.2%) and psychiatric disturbances (50.4%). Most PCPs are solid (50%), round (50%) lesions that occupy the third ventricle (3V, 94.8%) and show low-risk severity adhesions to the hypothalamus (66.8%). Two major topographical categories can be found: strictly 3V (57.5%), growing above an intact 3V floor (3VF) and not-strictly or infundibulo-tuberal (32.9%), expanding at the infundibulum and/or tuber cinereum. The hypothalamic syndrome predominated among strictly 3V PCPs (p<0.001). Psychiatric symptoms (p<0.001) and high-risk hypothalamic attachments (p=0.031) related to unfavorable postoperative outcomes among patients treated from 2006 onwards. The not-strictly 3V topography was identified as the major predictor of high-risk hypothalamic attachments (71.2% correctly predicted), which, along with incomplete tumor removal (p=0.018), underlies the higher tumor recurrence of this topography (p=0.001). Conclusions: This systematic review evidences that PCP topography is a major determinant of hypothalamic-related symptoms, type of hypothalamic attachments and tumor recurrence rate. Accurate preoperative definition of PCP-hypothalamus relationships is essential for the judicious, safe management of these complex lesions.

The Role of Autophagy and Mitophagy in Huntington’s Disease

Huntington’s disease (HD) is an inherited autosomal-dominant neurodegenerative disorder that occurs due to mutations in the polyglutamine expansions of the Huntingtin protein (Htt). HD is characterised by the loss of cognitive and motor functions, as well as the development of emotional and psychiatric disturbances. The HD pathology is manifested through the cellular changes that arise due to the toxic functions of mutant Htt (mHtt). Autophagy is a lysosomal pathway that functions to remove damaged intracellular components while mitophagy is a selective form of autophagy involving mitochondria; and PINK1/Parkin-mediated mitophagy is the most well-understood pathway. Mitochondrial dysfunction and defects in mitophagy can be linked to the pathogenesis of HD. Previous research has shown that the presence of mHtt hinders mitophagy; while PINK1/Parkin-mediated mitophagy provides neuroprotection in HD. Hence, this review discusses the roles and regulations of mitophagy, along with an overview of mitophagy in HD.

Longitudinal Analyses of the Reciprocity of Depression and Anxiety after Traumatic Brain Injury and Its Clinical Implications

Depression and anxiety are common following traumatic brain injury (TBI). Understanding their prevalence and interplay within the first year after TBI with differing severities may improve patients’ outcomes after TBI. Individuals with a clinical diagnosis of TBI recruited for the large European collaborative longitudinal study CENTER-TBI were screened for patient-reported major depression (MD) and generalized anxiety disorder (GAD) at three, six, and twelve months post-injury (N = 1683). Data were analyzed using autoregressive cross-lagged models. Sociodemographic, premorbid and injury-related factors were examined as risk factors. 14.1–15.5% of TBI patients reported moderate to severe MD at three to twelve months after TBI, 7.9–9.5% reported GAD. Depression and anxiety after TBI presented high within-domain persistency and cross-domain concurrent associations. MD at three months post-TBI had a significant impact on GAD at six months post-TBI, while both acted bidirectionally at six to twelve months post-TBI. Being more severely disabled, having experienced major extracranial injuries, an intensive care unit stay, and being female were risk factors for more severe MD and GAD. Major trauma and the level of consciousness after TBI were additionally associated with more severe MD, whereas being younger was related to more severe GAD. Individuals after TBI should be screened and treated for MD and GAD early on, as both psychiatric disturbances are highly persistent and bi-directional in their impact. More severely disabled patients are particularly vulnerable, and thus warrant timely screening and intensive follow-up treatment.

Psychological Morbidity After Critical Illness

Patients with critical illnesses face a number of severe psychic and physical stressors. Survivors often have long-term cognitive and physical impairments, as well as family, financial, and other stressors. These potential stressors increase the risk of psychiatric disturbances substantially. This chapter describes the burden of distress-related psychiatric morbidity in patients who survive critical illnesses, as well as risk factors for this morbidity. This knowledge serves as the motivation to develop new approaches that can ameliorate, or even prevent, long-term distress in survivors. The chapter also presents information about early attempts to reduce, prevent, and manage long-term psychological morbidity.

Obesity Exacerbates Irritable Bowel Syndrome-Related Sleep and Psychiatric Disorders in Women With Polycystic Ovary Syndrome

Background/ObjectivesPolycystic ovary syndrome (PCOS) and irritable bowel syndrome (IBS) share similar clinical and psychosocial features. We aimed to investigate the clinical characteristics of IBS in women with PCOS, and its relationship with obesity, metabolic and hormonal profiles, as well as sleep and psychiatric disorders.Subjects/MethodsThis is a cross-sectional case-control study of 431 untreated women with PCOS and 259 healthy volunteers. All participants were assessed with a comprehensive clinical evaluation and two questionnaires: the Athens Insomnia Scale (AIS) and the Brief Symptom Rating Scale (BSRS-5). IBS was diagnosed using the Rome III criteria. Obesity was defined as a BMI ≥30 kg/m2. Anthropometric measurements, metabolic, hormonal profiles, and psychosocial morbidities were compared.ResultsWomen with PCOS were more likely to have IBS (10.7% vs 5.8%, p=0.029) and obesity (29% vs 4%, p&lt;0.001) than healthy volunteers. Mixed-type IBS (IBS-M) was the most common subtype (74%) among patients with PCOS and IBS. There was a higher prevalence of psychiatric morbidities (total BSRS-5 score ≥10) in women with PCOS than in healthy women (11.4% vs 3.5%, p&lt;0.001). Women with PCOS and IBS were more likely to have sleep difficulties (67.4% vs 30.9%, p&lt;0.001) and psychiatric morbidities (21.7% vs 10.1%, p=0.019) than those without IBS. Anthropometrics, metabolic and hormonal profiles were similar between PCOS women with and without IBS. Among women with PCOS, those with both IBS and obesity had the highest risk of developing sleep difficulties (odds ratio: 5.91; 95% confidence interval: 1.77–19.77) and psychiatric distress (odds ratio: 4.39; 95% confidence interval: 1.26–15.29) than those without.ConclusionWomen with PCOS have increased IBS, obesity, sleep and psychiatric disturbances. The presence of IBS in PCOS women is associated with sleep and psychiatric disorders. The coexistence of obesity and IBS exacerbates sleep difficulties and psychiatric distress. Screening and management of IBS and obesity might be warranted to improve sleep and psychiatric disturbances in women with PCOS.

Mania in an individual with Systemic Lupus Erythematosus – A case report

Neuropsychiatric manifestations are common and occur in around 14-80% of patients with SLE. No particular neurologic or psychiatric manifestation is characteristic of SLE and the form and pattern of neuropsychiatric symptoms vary significantly. The American College of Rheumatology (ACR) Nomenclature provides case definition for 19 neuropsychiatric syndromes seen in SLE. However, these case definitions were not found to be effective in differentiating neuropsychiatric SLE (NPSLE) patients from those with neuropsychiatric manifestations not associated with SLE.Here we present a case of mania in a patient with SLE and discuss the differential diagnosis of neuropsychiatric manifestations of SLE and primary mood disorder. Symptoms of neuropsychiatric SLE vary significantly and psychiatric disturbances in a patient with SLE is a diagnosis of exclusion where other possibilities have to be considered including an independent comorbid psychiatric disorder. This case highlights the difficulty in the diagnostic process and the need for more studies on the differences between primary psychiatric disorders and neuropsychiatric SLE.

Oromandibular Dystonia: A Clinical Examination of 2,020 Cases

Objective: The goal of this study is to better characterize the phenotypic heterogeneity of oromandibular dystonia (OMD) for the purpose of facilitating early diagnosis.Methods: First, we provide a comprehensive summary of the literature encompassing 1,121 cases. Next, we describe the clinical features of 727 OMD subjects enrolled by the Dystonia Coalition (DC), an international multicenter cohort. Finally, we summarize clinical features and treatment outcomes from cross-sectional analysis of 172 OMD subjects from two expert centers.Results: In all cohorts, typical age at onset was in the 50s and 70% of cases were female. The Dystonia Coalition cohort revealed perioral musculature was involved most commonly (85%), followed by jaw (61%) and tongue (17%). OMD more commonly appeared as part of a segmental dystonia (43%), and less commonly focal (39%) or generalized (10%). OMD was found to be associated with impaired quality of life, independent of disease severity. On average, social anxiety (LSA score: 33 ± 28) was more common than depression (BDI II score: 9.7 ± 7.8). In the expert center cohorts, botulinum toxin injections improved symptom severity by more than 50% in ~80% of subjects, regardless of etiology.Conclusions: This comprehensive description of OMD cases has revealed novel insights into the most common OMD phenotypes, pattern of dystonia distribution, associated psychiatric disturbances, and effect on QoL. We hope these findings will improve clinical recognition to aid in timely diagnosis and inform treatment strategies.

Effect of Botulinum Toxin on Non-Motor Symptoms in Cervical Dystonia

Patients with cervical dystonia (CD) may display non-motor symptoms, including psychiatric disturbances, pain, and sleep disorders. Intramuscular injection of botulinum toxin type A (BoNT-A) is the most efficacious treatment for motor symptoms in CD, but little is known about its effects on non-motor manifestations. The aim of the present study was to longitudinally assess BoNT-A’s effects on CD non-motor symptoms and to investigate the relationship between BoNT-A-induced motor and non-motor changes. Forty-five patients with CD participated in the study. Patients underwent a clinical assessment that included the administration of standardized clinical scales assessing dystonic symptoms, psychiatric disturbances, pain, sleep disturbances, and disability. Clinical assessment was performed before and one and three months after BoNT-A injection. BoNT-A induced a significant improvement in dystonic symptoms, as well as in psychiatric disturbances, pain, and disability. Conversely, sleep disorders were unaffected by BoNT-A treatment. Motor and non-motor BoNT-A-induced changes showed a similar time course, but motor improvement did not correlate with non-motor changes after BoNT-A. Non-motor symptom changes after BoNT-A treatment are a complex phenomenon and are at least partially independent from motor symptom improvement.

STEROID INDUCED PSYCHOSIS IN A CASE OF ERYTHEMA MULTIFORME MAJOR.

Corticosteroids have been documented to cause psychiatric disturbances to various degree. We report a case of psychosis in a case of erythema multiforme major treated with steroids that presented to psychiatric outpatient department requiring medical management. A 32 years old female was brought to psychiatry outpatient department following behavioural disturbances. Patient had a history of recently suffering from Erythema Multiforme major following a dose of injectable ceftriaxone. After being treated with injectable and oral dose of corticosteroids she developed behavioural disturbances. On detailed evaluation patient was diagnosed to be suffering from Substance/medication-induced psychotic disorder as per DSM-5. She was started on treatment with Tab. Olanzapine (5mg) HS and Tab. Clonazepam (0.5 mg) HS with which her symptoms improved and remitted completely within few weeks. Corticosteroid induced psychosis can be managed with tapering of steroid dosages and / or addition of psychotropic medication. This case emphasizes on the need of liaison-based approach for a holistic management in such cases.Further research is essential to establish the course of illness and long-term prognosis in these patients