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The most common cause of severe cognitive impairment in adults is Alzheimer's disease (AD). Depending on the age of onset, AD is divided into early (<65 years) and late (≥65 years) forms. Early-onset AD (EOAD) is significantly less common than late-onset AD, accounting for only about 5–10% of cases. However, its medical and social significance as a disease leading to loss of ability to work and legal capacity, as well as premature death in patients aged 40–64 years, is extremely high. Patients with EOAD, as compared with late-onset AD (LOAD), have a greater number of atypical clinical variants: 25% and 6–12.5%, respectively, which complicates the differential diagnosis of EOAD against other neurodegenerative diseases. Nonetheless, the typical amnestic variant predominates in both LOAD and EOAD. Also, patients with EOAD have characteristic neuroimaging features: brain MRI scans from patients with EOAD often have more severe parietal atrophy and less severe hippocampal atrophy than those from patients with LOAD. This report addresses the features of the clinical and neuroimaging picture in patients with EOAD. A clinical case of a patient with EOAD is presented. [ABSTRACT FROM AUTHOR]

Immunoglobulin G4-related disease (IgG4-RD) is a multi-organ disorder characterized by a highly variable clinical presentation depending on the affected organ/s, extent of tumefactive fibroinflammatory lesions, and associated functional impairment. The disease pursues a chronic, relapsing, often asymptomatic course and hence may pose a significant diagnostic challenge. Diagnostic delay can lead to progressive fibrosis and irreversible organ damage resulting into significant morbidity and even mortality. Given its broad clinical spectrum, physicians of all specialties may be the first clinicians facing this diagnostic challenge. Outside the pancreatobiliary system, the head and neck represents the major site of IgG4-RD with variable organ-specific diffuse or mass-forming lesions. In up to 75% of cases, elevated serum IgG4 levels are observed, but this figure possibly underestimates the fraction of seronegative cases, as the disease manifestations may present metachronously with significant intervals. Together with negative serology, this can lead to misdiagnosis of seronegative cases. A standardized nomenclature and diagnostic criteria for IgG4-RD were established in 2012 and revised in 2020 facilitating scientific research and expanding the range of diseases associated with IgG4 abnormalities. In addition to orbital pseudotumor, dacryoadenitis, Riedel thyroiditis, sinonasal manifestations, and rare miscellaneous conditions, IgG4-related sialadenitis is one of the most frequent presentations in the head and neck region. However, controversy still exists regarding the relationship between sialadenitis and IgG4-RD. This review focuses on the clinicopathological features of IgG4-related sialadenitis and its contemporary diagnostic criteria. [ABSTRACT FROM AUTHOR]

Objectives. To describe the features of the clinical picture and estimate the incidence of HIV-associated cerebellar degeneration in patients with progressive cerebellar ataxia. Materials and methods. A total of 377 patients with progressive cerebellar ataxia were examined using brain MRI, the Scale for the Assessment and Rating of Ataxia (SARA), and screening for cognitive impairment using the Montreal Cognitive Assessment test (MoCa). Autoimmune, deficiency, and other causes of ataxia, opportunistic infections, multiple system atrophy, and common forms of hereditary spinocerebellar ataxia were excluded in patients with HIV infection. Results. Five patients (1.3%) with combined ataxia and HIV infection were identified (two men, three women, aged 31–52 years). The median duration of HIV infection was five years and the duration of ataxia was one year. The clinical picture included not only progressive ataxia, but also pyramidal signs, dysphagia, and, less commonly, ophthalmoparesis, dystonia, postural hand tremor, and affective and moderate cognitive impairment. Brain MRI scans revealed signs of olivopontocerebellar atrophy in three patients and degeneration of the cerebellum (mainly the vermis) in two. All patients received combined antiretroviral therapy in various regimens, though despite this, ataxia was progressive. Conclusions. HIV infection is a rare cause of cerebellar degeneration. This diagnosis remains a diagnosis of exclusion. Cerebellar degeneration can occur and progress even after achieving stable remission of HIV infection on the background of highly active antiretroviral therapy. [ABSTRACT FROM AUTHOR]

Introduction: In Japan, epidemiological studies on type 1 diabetes (T1D) have mainly focused on the disease in childhood. Meanwhile, limited information is available regarding the clinical features of adolescent and adult T1D. Therefore, we aimed to investigate their current clinical state in Saitama prefecture near Tokyo, Japan. Materials and methods: We conducted a cross-sectional, hospital-based, multicenter study. Eight institutions participated in the study, all of which treated relatively large numbers of T1D patients. We identified 1241 T1D patients aged 16 or over: 814 with acute-onset T1D (AT1D), 362 with slowly progressive insulin-dependent diabetes mellitus (SPIDDM), and 65 with fulminant T1D (FT1D). Based on the patient's medical records, various clinical parameters and complications were investigated. Results: Of 1241 patients, 739 (59.5%) were females. Among all patients, the median age, onset age, and disease duration were 51, 38, and 13 years, respectively. The patients had a median BMI of 22.6 kg/m2, and 26.1% were obese, corroborating previous nationwide surveys. Moreover, the median HbA1c was 7.8%, similar to previous nationwide surveys. Among patients with AT1D, SPIDDM, and FT1D, 85.6%, 72.1%, and 81.5% carried out multiple daily insulin injection, respectively, while 10.3%, 2.2%, and 18.5% were subject to continuous subcutaneous insulin infusion. The proportions of retinopathy, nephropathy, and neuropathy were 26.3%, 20.8%, and 21.5%. Conclusions: The glycemic control in T1D patients in Saitama was equivalent to that observed in previous nationwide surveys. Moreover, approximately one-quarter of T1D patients had obesity. Future studies should address whether our findings reflect those throughout Japan. [ABSTRACT FROM AUTHOR]

Progressive supranuclear palsy (PSP) is a progressive atypical parkinsonian syndrome characterised by postural instability, supranuclear ophthalmoplegia, dysarthria, dysphagia, executive dysfunction and other features. This clinical presentation represents the classic PSP-Richardson syndrome (PSP-RS). However, several other clinical subtypes have been recognised, including PSP-parkinsonism (PSP-P), probably the second most common PSP variant. Unlike PSP-RS, PSP-P often presents with an asymmetric onset, tremor and a moderate initial response to levodopa, especially during the first years of the disease, thus resembling Parkinson's disease (PD). It runs a more favourable course, but over time, PSP-P may evolve clinically into PSP-RS. Therefore, it may seem that PSP-P stands clinically between PD and PSP. There are several peculiarities that can distinguish PSP-P from these entities. As there is lack of systematic reviews on PSP-P in the literature, we decided to summarise all the necessary data about the epidemiology, clinical picture, neuroimaging, genetics and other aspects of this PSP variant in order to provide complete information for the reader. [ABSTRACT FROM AUTHOR]

Background: The RDoC (research domain criteria) domain arousal and regulatory systems plays an important role for psychiatric disorders. Objective: What is the association between physiology and function of arousal, sleep and circadian rhythms and psychiatric disorders? What are the therapeutic consequences? Material and method: In this narrative review the most important findings related to the topic are presented briefly and concisely. First, the physiology and function of the systems are described. Then the association with various psychiatric disorders is elucidated and therapeutic approaches are discussed. Results: The treatment of disorders of the arousal system plays a role in a broad group of psychiatric disorders. It is especially important to incorporate the disturbance of the arousal system into therapeutic concepts of affective disorders, schizophrenia, attention deficit hyperactivity disorder (ADHD) and substance abuse. Conclusion: The dimensional concept of RDoC is reflected in DSM‑5 in the simultaneous registration of sleep disturbances and psychiatric disorders, which has important therapeutic consequences. The relationship between sleep function and the development of psychiatric disorders needs continued intensive research. [ABSTRACT FROM AUTHOR]

Copyright of Der Hautarzt is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: The objective was to determine if Spanish foster care children and Spanish non-foster children differ on sluggish cognitive tempo (SCT), ADHD-inattention (IN), ADHD-hyperactivity/impulsivity (HI), oppositional defiant disorder (ODD), limited prosocial emotions (LPE), anxiety, depression, social and academic impairment measures and if the duration of foster care predicts a reduction in symptom and impairment differences between foster and non-foster care children. Method: Foster care parents of 49 children (8 to 13 years, 57% girls) and non-foster care mothers and fathers of 1776 children (8 to 13 years, 49% girls) completed the Child and Adolescent Behavior Inventory (CABI). Results: Foster care children had significantly higher scores on all symptom and impairment measures than non-foster care mother and father groups (d values from 0.37 to 1.53). A longer duration in foster care (range 8 to 86 months) was also associated with significant lower scores on SCT, ADHD-IN, anxiety, depression, and academic impairment. In addition, while foster care children with a shorter duration in foster care (less than medium months) had significantly higher scores than the non-foster care groups on all measures (d values from 0.66 to 2.25), children with a longer duration in foster care did not differ from the non-foster care groups on anxiety, depression, ADHD-IN, ADHD-HI, LPE, social and academic impairment. Conclusions: Although foster care children had elevated psychopathology and impairment scores relative to non-foster care children, a longer stay in foster care was associated with the elimination of the difference on most symptom and impairment measures. [ABSTRACT FROM AUTHOR]

Purpose: Bacterial urinary tract infections (UTIs) are the most frequently occurring infectious diseases in the geriatric population. The aim of the study was to determine the prevalence and clinical features of UTIs in geriatric in-patients and their association with health and functional ability characteristics.Methods: A prospective cross-sectional cohort study was conducted among patients hospitalized on the geriatric ward. Patients were interviewed, examined, and had their hospital records analyzed. An uncontaminated midstream urine sample was collected and cultured in all of the cases suspected for UTI. Relative risks for UTI were counted and multivariable logistic regression model was built.Results: 246 patients were included, 179 (72.8%) women, 210 (85.4%) 75 + -year-olds. Bacterial UTIs occurred in 18.3% of the patients. The main etiological agent was Escherichia coli (73.3%). The most significant predictors of UTI were recurrent UTI and urinary catheter. The typical clinical UTI symptoms occurred in less than half of the cases (only in 11.1% of cases fever was observed). More often, than in patients without UTIs, they reported symptoms such as delirium (28.9% vs. 18%), tachycardia (11.1% vs. 1.5%) or hypotension (20% vs. 12.1%).Conclusions: Bacterial UTIs affect about 1/5 of hospitalized geriatric patients. The clinical picture of these infections very often is atypical and it indicates a need for diagnostic vigilance. [ABSTRACT FROM AUTHOR]

Copyright of Der Hautarzt is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Burning Mouth Syndrome (BMS) is a chronic pain condition characterized by persistent intraoral burning without related objective findings and unknown etiology that affects elderly females mostly. There is no satisfactory treatment for BMS. We aimed to observe the long-term efficacy of high velanfaxine doses combined with systemic and topical administered clonazepam in a particular subgroup of BMS patients who do not respond to current clinical management. Results: Eight (66.1 ± 6.2 years old females) out of 14 BMS patients fulfilled the inclusion criteria and were treated with venlafaxine (300 mg/d) and clonazepam (5 mg/d) for 35.4 ± 12.1 (mean ± SD) months. The average duration of the symptoms at baseline was 4.3 ± 1.4 years and the overall mean daily pain intensity score was 8.6 ± 1.3 (VAS); pain was in tongue and within the oral mucosa, accompanying by oral and facial dysesthesia. In five patients tasting was abnormal. All patients had positive history of concomitant primary headache. The average score of Hamilton Rating scale for Anxiety and Depression was 21 ± 4.2, and 26.1 ± 2.9, respectively. Previous ineffective treatments include anticonvulsants and anti-depressants. All patients responded (more than 50% decrease in VAS) after three months treatment (mean VAS 3.2 ± 2.2) with no remarkable adverse events. Conclusion: BMS deserves bottomless psychiatric evaluation and management when current available treatments fail. Treatment with venlafaxine combined with topical and systemic clonazepam may be effective in refractory BMS cases but further investigation in a large-scale controlled study is needed to confirm these results. [ABSTRACT FROM AUTHOR]

Alcohol-induced muscle damage (AIMD) is an umbrella term that includes all forms of alcoholic myopathy developing in acute or chronic alcohol intoxication. The most common form of destruction of skeletal muscles in alcoholism is chronic alcoholic myopathy, which develops independently of other alcohol-induced disorders, such as polyneuropathy, the malabsorption syndrome, and liver damage, but may be combined with them. The atrophy of muscle fibers underlies skeletal muscle destruction in chronic AIMD. Type II muscle fibers are affected to a greater degree than type I muscle fibers. To date, the pathogenesis of chronic alcoholic myopathy has been studied insufficiently. The imbalance between protein synthesis and proteolysis, as well as increased apoptosis rate, is discussed. [ABSTRACT FROM AUTHOR]

Narcolepsy-cataplexy (N-C) is a focal neurodegenerative disease with a genetic predisposition and autoimmune etiology; the pathogenesis of narcolepsy without cataplexy (Nw/oC) is less clear. One hundred and forty eight patients underwent clinical face-to face interviews, polysomnography, multiple sleep latency testing and HLA-DQB1*0602 typing. The cohort was divided into four age groups: children and adolescents under 19 years ( N = 31), adults aged 20-39 years ( N = 51), 40-59 years ( N = 28) and over 60 years ( N = 38). N-C was found in 93 adults (79.5 %) compared with 16 pediatric patients (51.6 %) ( p < 0.01), suggesting that at least some of the children were candidates for developing cataplexy in the future. Statistical evaluation showed an increasing age-related proportion of associated sleep disorders-obstructive sleep apnea, periodic leg movements and restless leg syndrome ( p < 0.001). Nw/oC patients showed sleep comorbidities less frequently than N-C group. A close connection with N-C was found particularly in REM behavior disorder (RBD) ( p < 0.05). RBD affected a third of the patients in the youngest as well as in the oldest groups. However, association with other sleep disorders had no significant effect on nocturnal sleep (with the exception of obstructive sleep apnea), and the sleep comorbidities under study had no noticeable effect on daytime sleepiness. [ABSTRACT FROM AUTHOR]

Cyanobacterial blooms exert negative impacts on fisheries and water management authorities. Recently, it has gained global attention, as elevated earth warming and environmental pollution are accelerating algal growth. Oreochromis niloticus ( O. niloticus) is a worldwide and the most commonly cultured fish in Egypt. The biological interaction of the living organisms to the surrounding environment must continuously be assessed to predict future effects of the ongoing hazards on fish. The study was designed to examine the possible biological and biochemical response of O. niloticus exposed to different concentrations of microcystins crude extract (containing microcystin-LR). Three equal groups of O. niloticus were assigned for intraperitoneal injection of three different doses: 100, 200, and 400 μg m dried aqueous microcystins extract, for 10 days. Clinical, condition factor (K) and hepatosomatic index (HIS) were estimated. Biochemical alterations were evaluated via lipid peroxidation, DNA fragmentation assay and electrophoretic analysis of fragmented DNA using agarose gel electrophoresis. The results showed that there were discernible behavioral and clinical alterations. Significant differences in K and HIS were observed between treatments. Also, significant elevations were observed in lipid peroxidation level and in the DNA fragmentation percentage in the exposed fish to the doses of 200 and 400 μg m of microcystins crude extract. The current study addresses the possible toxic effects of microcystins crude extract to O. niloticus. The results cleared that microcystins crude extract (containing MC-LR) is toxic to O. niloticus in time- and dose-dependent manners. [ABSTRACT FROM AUTHOR]

Anti-NMDA-receptor encephalitis is a severe and considerably underdiagnosed form of encephalitis with characteristic clinical features including psychiatric symptoms, decreased levels of consciousness, hypoventilation, epileptic seizures, autonomic dysfunction and dyskinesias. Most patients are primarily seen by psychiatrists, often on the assumption of a drug-induced psychosis. Anti-NMDA-receptor encephalitis had initially been described in young women with ovarian teratoma, but is also common in women without tumour, in men and in children. The diagnosis is based on the characteristic clinical picture, supporting findings of brain MRI, electroencephalogram and cerebrospinal fluid (CSF), and the presence of highly specific autoantibodies directed against the NR1 subunit of NMDA-type glutamate receptors in the serum or CSF. In particular, anti-NMDA-receptor encephalitis must be excluded in patients with 'encephalitis of unknown cause'. In principle, the prognosis is favourable and recovery from symptoms can be expected even after prolonged intensive care treatment and mechanical ventilation. However, improvement correlates with prompt identification of the disorder, early immunotherapy and - in the case of a malignancy - with complete tumour removal. Patient care requires an interdisciplinary approach including neurologists, psychiatrists, paediatricians, oncologists and gynaecologists. [ABSTRACT FROM AUTHOR]

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Copyright of Operative Orthopädie und Traumatologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

A retrospective study was carried out of the ten cases of osteopoikilosis seen at this Orthopedic Unit over a 15-year period in order to determine the reasons why patients seek consultation, preliminary diagnosis, and associated lesions. Eight patients consulted for problems not related to the locomotor apparatus, and diagnosis was by chance; the other two presented joint pain. The preliminary diagnosis was osteoblastic metastasis in five patients and osteopoikilosis in the other five. None of the patients displayed skin or visceral involvement, but three presented bone alterations. Definitive diagnosis was made by measurement of biochemical markers of bone remodeling, radiography of both hands, and bone scan. Bone biopsy was performed in one case. Although rare, the radiographic symptoms of osteopoikilosis are sufficiently specific to avoid false diagnoses, which may give rise to rigorous and expensive studies for other important disorders. [ABSTRACT FROM AUTHOR]

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29 patients with amebic liver abscess were evaluated in a study to examine clinical picture, laboratory data, epidemiology, radiologic methods, and therapy. Since the clinical picture was unspecific a considerable amount of misdiagnoses occurred, and often originated from pulmonary symptoms. To establish diagnosis one should rely on the triad with positive amebic serology, intrahepatic scanning defects, and clinical picture with fever, right upper quadrant pain, and hepatomegaly. Nearly all patients had an exposure history of travel or immigration from endemic areas in the tropics. Medical therapy with metronidazole alone is highly effective and leads to defervescence and clinical improvement usually within 3-5 days. Invasive procedures, such as needle aspiration or surgical drainage of the abscess are rarely needed; these invasive methods neither shorten the course of the disease nor improve prognosis. [ABSTRACT FROM AUTHOR]

The authors describe their findings in a study aimed at identifying clinical-prognostic factors in treatment of idiopathic normal-pressure hydrocephalus. The study comprised 18 adult patients submitted to surgery for ventriculo-peritoneal shunting. The findings that emerged from this series of patients were compared with those reported for the 381 published cases. In our group of 18 patients, average age was 65 years and the average duration of clinical history was 47 months (median 18 months). Follow-up ranged from 3 to 5 years (median 4.2 years): 12 patients improved (9 completely) and 6 presented stable neurological deficits. The factors that had a statistically significant influence on outcome were a short clinical history (less than 6 months) (p = 0.05) and a clinical onset without dementia (p = 0.03). Patients with medium-grade preoperative ventricular enlargement always made a complete functional recovery after surgery (p = 0.2). [ABSTRACT FROM AUTHOR]

Systemic candidiasis with Candida-induced abscesses, predominantly in the liver and the spleen, was diagnosed in 27 patients with haematologic malignancies after intensive cytostatic therapy. Specific features included septic fever unresponsive to antimicrobial therapy, hepatosplenomegaly with multiple lesions in the liver and spleen (diameter up to 2 cm) as detected by computed tomography (CT) or ultrasound, and an elevation in liver enzymes. During treatment, induced neutropenia, hepatic and splenic foci were poorly defined histologically and were not identified by imaging procedures. After granulocyte recovery these foci showed characteristic histological patterns. Ultrasound and/or CT investigations of the abdomen now revealed characteristic lesions in the liver and the spleen. Gamma-GT and alkaline phosphatase were early indicators of hepatic involvement in Candida septicaemia and were often already elevated in aplasia. [ABSTRACT FROM AUTHOR]

Four hundred and sixty-three new diagnoses of coeliac disease in children were made during 1973 to 1986 in our department. The distribution of new diagnoses per year did not show any decreasing trend in the period 1973 to 1986. No changing trend in breast feeding attitudes was found in the region during the same period. Analysis of the clinical pattern showed a constant distribution of age at diagnosis, onset of first symptoms and nature of symptoms throughout the period studied. These data suggest that coeliac disease is predominantly genetically determined and its expression is modulated by individual and environmental factors. The stability of the observed prevalence of the disease is parallel to the unchanging pattern of breast feeding attitudes in the region. [ABSTRACT FROM AUTHOR]

A high frequency of anti-Ro (SSA) circulating antibodies and lack of HLA-DR association described recently in Greek rheumatoid arthritis (RA) patients, prompted us to study their clinical and laboratory picture and compare it with that described in the literature. One hundred and ninety seven patients with definite or classical RA were divided into three groups A, B, and C with age at disease onset below 39, between 40 and 59, and above 60 years respectively. Disease duration below 5 and above 5 years resulted in further division of each group into subgroups 1 and 2. With few exceptions, there were no significant differences between the groups in terms of maximal articular index, lymphadenopathy, splenomegaly, hepatomegaly, frequency of rheumatoid nodules and lowest hematocrit, highest erythrocyte sedimentation rate, and C-reactive protein values. The presence of rheumatoid factor, antinuclear antibodies, cryoglobulins, and elevated levels of globulins, C and C in patients' sera were not significantly different among the groups. A statistically significant radiologic deterioration was observed with disease duration, common in all groups. Diffuse interstitial lung disease was the most common pulmonary abnormality noted. There were no differences between the groups. Penicillamine toxicity was independent of age and disease duration. This study suggests that the clinical picture of RA in Greece is similar to that in other populations and that there are no significant differences in general among its age groups. [ABSTRACT FROM AUTHOR]

The dynamics of infectious process was studied by modeling chronic herpesvirus infection in mice. The infectious process ran a wave-like course with periods of relapses and remissions. Clinical manifestations and severity and duration of relapses largely depended on the disease duration. Reactivation of herpesvirus led to systemic involvement, this indicating the dissemination of infection in animals and chronic transformation of the disease. [ABSTRACT FROM AUTHOR]