scholarly journals A Case of Frontotemporal Lobar Degeneration with Progressive Dysarthria

2006 ◽  
Vol 17 (2) ◽  
pp. 97-104 ◽  
Author(s):  
Nami Ihori ◽  
Shigeo Araki ◽  
Kenji Ishihara ◽  
Mitsuru Kawamura
Keyword(s):  
Clinical Symptoms ◽  
Frontotemporal Degeneration ◽  
Behavioral Abnormalities ◽  
Personality Changes ◽  
Initial Stage ◽  
Neuropsychological Characteristics ◽  
Opercular Syndrome ◽  
Rate Of Speech ◽  
Clinical Subtype ◽  
Post Onset

We investigated the evolution of the neurological and neuropsychological characteristics in a right-handed woman who was 53-years-old at the onset and who showed personality changes and behavioral disorders accompanied by progressive dysarthria. She had hypernasality and a slow rate of speech with distorted consonants and vowels, which progressed as motor disturbances affecting her speech apparatus increased; finally, she became mute two years post onset. Her dysarthria due to bilateral voluntary facio-velo-linguo-pharyngeal paralysis accompanied with automatic-voluntary dissociation fit the description of anterior opercular syndrome. She showed personality changes and behavioral abnormalities from the initial stage of the disease, as is generally observed in frontotemporal degeneration (FTD), and her magnetic resonance image showed progressive atrophy in the frontotemporal lobes; thus, she was clinically diagnosed with FTLD. This patient’s symptoms suggest that FTLD, including bilateral anterior operculum degeneration, causes progressive pseudobulbar paretic dysarthria accompanied by clinical symptoms of FTD, which raises the possibility of a new clinical subtype in the FTLD spectrum.

scholarly journals Some Predictors of Depressive Disorders in the Context of Clinical Patomorphosis

2021 ◽  
Vol 6 (4) ◽  
pp. 77-82
Author(s):  
O. O. Belov ◽  
Keyword(s):  
Cognitive Impairment ◽  
Clinical Picture ◽  
Depressive Disorders ◽  
Clinical Symptoms ◽  
Emotional Lability ◽  
Obsessive Compulsive ◽  
Diagnostic Strategies ◽  
Low Mood ◽  
Self Blame ◽  
Initial Stage

The purpose of the study was to study the clinical and psychopathological phenomenology of the initial stage of depressive disorders in the context of clinical pathomorphosis. Materials and methods. Features of clinical symptoms of the initial stage of depressive disorders in the comparative aspect in the context of clinical pathomorphosis based on the analysis of medical records of 236 patients who were treated for depressive disorders in 1971-1995 (ICD-9 codes 296.1, 296.3) and clinical examination of 245 patients with depressive disorders in 2015-2019 (ICD-10 codes F 31.3, F 31.4, F 32.0, F 32.1, F 32.2, F 33.0, F 33.1, F 33.2) are considered. Results and discussion. It was established that there is a predominance in the clinical picture of modern depressive disorders of low mood (in general in 91.4% of patients, 91.6% of men and 91.3% of women, p>0.05), dyssomnia (93.1%, 92.5% and 93.5%, respectively, p>0.05), anxiety, fear (84.5%, 78.5%, 89.1%, respectively, p<0.01), asthenia (82.4%, 77.6% and 86.2%, respectively, p>0.05), somatic vegetative symptoms (82.9%, 77.6% and 87.0%, respectively, p<0.01), apathy (78.8%, 69.2% and 86.2%, respectively, p<0.01) and ideas of self-humiliation and self-blame (69.8%, 72.9% and 67.4%, respectively, p<0.01), and the relatively low prevalence of obsessive symptoms (55.1%, 54.2% and 55.8%, respectively, p<0.05), emotional lability (51.0%, 54.2% and 48.6%, respectively, p<0.01) and cognitive impairment (45.3%, 43.9% and 46.4%, respectively, p<0.05) with a predominance of emotional lability and ideas of self-humiliation and self-blame in men, and manifestations of anxiety, fear, apathy, cognitive impairment, obsessive and somatic vegetative symptoms in women, which gives grounds to consider that the main predictors of depressive disorder at the initial stage of low mood are dyssomnia, anxiety fear, asthenia and somatic vegetative symptoms. The revealed features suggest the presence of a clinical pathomorphosis of depressive disorders. The clinical pathomorphosis of the initial stage of depressive disorders is in a significant reduction in the clinical picture of low mood, ideas of self-abasement and self-blame, emotional lability and cognitive impairment, and an increase in anxiety, fear, asthenia, apathy, obsessive symptoms and obsessive-compulsive symptoms, with significantly greater gender differentiation of clinical symptoms of depression. Conclusion. The identified patterns are embedded in the general trend towards polymorphism and clinical undifferentiation of modern depressive disorders, significant involvement of patients with sleep disorders, asthenic, apathetic and somatic vegetative symptoms, which requires revision of diagnostic strategies and individualization of diagnosis. The identified patterns can be used for early diagnosis of depressive disorders and prevention of depression

Approaches for ear-targeted delivery systems in neurosensory disorders to avoid chronic hearing loss mediated neurological diseases

2021 ◽  
Vol 20 ◽  
Author(s):  
Rishabh Verma ◽  
Preeti Vyas ◽  
Jasmeet Kaur ◽  
Md. Noushad Javed ◽  
Mohammad Sarafroz ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Targeted Delivery ◽  
Clinical Symptoms ◽  
Neurological Diseases ◽  
Delivery Systems ◽  
Small Dimension ◽  
Initial Stage ◽  
Prolonged Duration ◽  
Progression Of Disease

Background & Objective: Hearing loss is a common audio-vestibular-related neurosensory disability of inner ears, in which patients exhibit clinical symptoms of dizziness, gait unsteadiness, and oscillopsia, at an initial stage. While, if such disorders are untreated for a prolonged duration then the progression of disease into a chronic state significantly decreases GABA level as well as an alteration in the neurotransmission of CNS systems. Hence, to control the progression of disease into a chronic state, timely and targeted delivery of the drug into the site of action in the ear is now attracting the interest of neurologists for effective and safe treatment of such disorders. Among delivery systems, owing to small dimension, better penetration, rate-controlled release, higher bioavailability; nanocarriers are preferred to overcome delivery barriers, improvement in residence time, and enhanced the performance of loaded drugs. Subsequently, these carriers also stabilize encapsulated drugs while the opportunity to modify the surface of carriers favors guided direction for site-specific targeting. Conventional routes of drug delivery such as oral. intravenous, and intramuscular are poorer in performance because of inadequate blood supply to the inner ear and limited penetration of blood–inner ear barrier. Conclusion: This review summarized novel aspects of non-invasive and biocompatible nanoparticles-based approaches for targeted delivery of drugs into the cochlea of the ear to reduce the rate, and extent of the emergence of any hearing loss mediated neurological disorders.

scholarly journals A Case of Previously Unsuspected Huntington Disease Diagnosed at Autopsy

2017 ◽  
Vol 7 (1) ◽  
pp. 136-144
Author(s):  
Catherine R. Miller ◽  
Nobby C. Mambo ◽  
Jianli Dong ◽  
Gerald A. Campbell
Keyword(s):  
Genetic Testing ◽  
Huntington Disease ◽  
Clinical Symptoms ◽  
Neurodegenerative Disorder ◽  
Case Reports ◽  
Neuronal Loss ◽  
Cag Repeat ◽  
Cag Repeats ◽  
Psychiatric Disturbances ◽  
Personality Changes

Huntington disease (HD) is a neurodegenerative disorder with a worldwide prevalence of four to ten per 100 000. It is characterized by choreiform movements, behavioral/psychiatric disturbances, and eventual cognitive decline. Symptoms usually present between 30 and 50 years of age and the diagnosis is based on the combination of clinical symptoms, family history, and genetic testing. A variation of HD, juvenile Huntington disease (JHD), presents earlier, with more severe symptoms and with a worse prognosis. Symptoms are different in JHD, with personality changes and learning difficulties being the predominant presenting features. Seizures are common in JHD, and chorea is uncommon; movement disorders at presentation of JHD are predominantly nonchoreiform. The inheritance pattern for both HD and JHD is autosomal dominant and the disease is caused by an elongation of the CAG repeat in the huntingtin gene. There are many published case reports of Huntington disease that were confirmed at autopsy, but to our knowledge, there are no reports in the literature where the diagnosis of Huntington disease was first made at autopsy. We present a case of a 28-year-old African-American male who was in a state of neglect due to a lifetime of abuse, cognitive difficulties, and seizures, whose cause of death was pneumonia. The gross autopsy findings included bilateral caudate nucleus atrophy and lateral ventricular dilation. Microscopically, severe bilateral neuronal loss and gliosis of the caudate and putamen nuclei were seen. Genetic testing for the number of CAG repeats confirmed the diagnosis and was consistent with JHD.

Uncommon neurodegenerative causes of dementia

2005 ◽  
Vol 17 (s1) ◽  
pp. S35-S49 ◽  
Author(s):  
Alexander F. Kurz
Keyword(s):  
Clinical Symptoms ◽  
Corticobasal Degeneration ◽  
Cortical Atrophy ◽  
Behavioral Alterations ◽  
Autosomal Dominant Disorder ◽  
Personality Changes ◽  
Subcortical Nuclei ◽  
Vertical Gaze ◽  
The Brain ◽  
Visual Disturbances

A group of neurodegenerative diseases is outlined that affect cortical and subcortical areas of the brain. These diseases give rise to atypical forms of dementia and, unlike Alzheimer's disease (AD), are often associated with neurological symptoms. Clinical symptoms reflect the localization of the degenerative process rather than the nature of the underlying histopathology. Degeneration of the frontal and anterior temporal lobe presents initially with behavioral alterations, but later in the course, impairment of cognition and activities of daily living develops. Posterior cortical atrophy affects the parietal and occipital association cortices and causes complex visual disturbances. In corticobasal degeneration (CBD) the focus of pathology includes the frontoparietal cortex and several subcortical nuclei, causing symmetrical rigidity, bradykinesia, myoclonus and dystonia. Progressive supranuclear palsy (PSP) involves the frontal, temporal and parietal cortex as well as parts of the brain stem. Clinical features include a hypokinetic rigid syndrome with nuchal dystonia and vertical gaze palsy. Huntington's disease is a prototypical autosomal dominant disorder that affects the extrapyramidal system and causes choreatic movements in combination with personality changes and cognitive deterioration. Amyotrophic lateral sclerosis (ALS) with dementia is a neurodegeneration of the frontotemporal cortex and of the anterior horn of the spinal cord. Behavioral change similar to frontotemporal dementia (FTD) is paralleled or followed by the classic features of motor neuron disease.

scholarly journals Long-term Outcomes of Patients with Anti–Leucine-Rich Glioma-Inactivated 1 Encephalitis

2020 ◽  
Author(s):  
Shan Qiao ◽  
Huai-kuan Wu ◽  
Ling-ling Liu ◽  
Ke-jun Zang ◽  
Xuewu liu
Keyword(s):  
Clinical Symptoms ◽  
Early Recognition ◽  
Chinese Patients ◽  
Long Term Outcomes ◽  
Ancillary Test ◽  
Behavioral Abnormalities ◽  
Long Term Follow Up ◽  
And Behavior

Abstract Background: This report aims to provide a detailed description of the clinical manifestation, immunotherapy, and long-term outcomes of 117 Chinese patients with anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis. Methods: We retrospectively selected 117 patients diagnosed with anti-LGI1 encephalitis from the databases of multiple clinical centers from September 2014 to December 2019. The clinical features, ancillary test results, and details of long-term outcomes were analyzed.Results: Among the 117 anti-LGI1 encephalitis patients, 81 (69%) were male and 36 (31%) were female; the median onset age was 51 years (range: 30-77 years). The median time from symptom onset until diagnosis was 8.7 weeks (range: 2-49 weeks). The main features evaluated in our cohort were seizures, cognitive impairment, and mental and behavioral abnormalities. One hundred and nine patients were treated with immunotherapy, After 3-5 days of treatment, the clinical symptoms were somewhat alleviated in all the patients, and their memory, mental ability, and behavior improved. The median follow-up time was 33 months (range: 6-59 months). A total of 19 (16%) patients experienced a relapse; the median duration from onset to the first relapse was 5 (0.3-27) months. There were no mortalities during the follow-up period.Conclusions: The outcome of patients with anti-LGI1 encephalitis is mostly favorable, although some patients continue to suffer from cognitive dysfunction. Early recognition is of great significance for the treatment of anti-LGI1 encephalitis. Prompt adequate immunotherapy has positive implications for the improvement of clinical symptoms of anti-LGI1 encephalitis. Long-term follow-up is important for the assessment of LGI1 antibody-mediated encephalitis.

Clinical characteristics of alcoholism with different rates of progression

1985 ◽  
Vol 66 (6) ◽  
pp. 440-442
Author(s):  
D. D. Enikeeva
Keyword(s):  
Clinical Characteristics ◽  
Subsequent Development ◽  
Clinical Material ◽  
Personality Changes ◽  
Initial Stage ◽  
Rate Of Progression

In order to study the rate of progression of alcoholism, an analysis of a large clinical material was carried out: the timing of the appearance and subsequent development of the axial symptomatology of the disease, the duration of the stages and the rate of transition of the initial stage to the late ones, as well as the time of the onset of the consequences and complications of the disease, personality changes and social decompensation.

scholarly journals Fecal calprotectin and alpha-1 antitrypsin predict severity and response to corticosteroids in gastrointestinal graft-versus-host disease

Blood ◽  
2012 ◽  
Vol 119 (24) ◽  
pp. 5909-5917 ◽  
Author(s):  
Paula Rodriguez-Otero ◽  
Raphael Porcher ◽  
Régis Peffault de Latour ◽  
Margarita Contreras ◽  
Yoram Bouhnik ◽  
...  
Keyword(s):  
Prognostic Value ◽  
Clinical Symptoms ◽  
Complete Response ◽  
Fecal Calprotectin ◽  
Steroid Resistant ◽  
Graft Versus Host ◽  
Initial Stage ◽  
Alpha 1 Antitrypsin ◽  
Multiple Regression Modeling ◽  
Stage 1

AbstractDiagnosis of gastrointestinal GVHD (GI-GVHD) is based on clinical symptoms and histologic findings. No biomarkers predicting responses to treatment are routinely available even though 30% to 50% of patients will not respond to corticosteroids. In this study, we aimed to evaluate fecal calprotectin, α-1-antitrypsin (α1-AT), and elastase at the time of first symptoms as diagnostic and prognostic tools for GI-GVHD in 72 consecutive patients, of whom 51 developed GI-GVHD. The prognostic value of markers was evaluated by their association with complete response (CR) and steroid-resistant (SR) GVHD. Calprotectin and α1-AT concentrations increased with GI-GVHD initial stages but patients with initial stage 1 GI-GVHD had similar marker levels to patients without GI-GVHD, so sensitivity to diagnose GI-GVHD was weak. In contrast, calprotectin and α1-AT were predictors for SR-GVHD and CR. Multiple regression modeling identified calprotectin and α1-AT concentration as independently predicting SR-GVHD together with initial stage > 2 GI-GVHD. Our results showed that fecal calprotectin and α1-AT levels at the time of diagnosis are predictive for responses to treatment but are not diagnostic markers for initial stage 1 to 3 GI-GVHD.

The Therapeutic Factors of Group-Analytical Treatment

1950 ◽  
Vol 96 (405) ◽  
pp. 976-997 ◽  
Author(s):  
F. Kräupl Taylor
Keyword(s):  
Environmental Factors ◽  
Clinical Symptoms ◽  
Therapeutic Process ◽  
Therapeutic Factors ◽  
Analytical Treatment ◽  
Personality Changes ◽  
Environmental Agencies

The term “therapeutic factor” will be used to denote any agency which is potentially capable of producing such changes in the personality of a patient that an alleviation or cure of clinical symptoms may result. Such agencies originate either in the environment of the patient or in his organism. In psychotherapy we are primarily concerned with environmental agencies, namely those which are introduced, regulated and controlled by the therapist, and which are therapeutic only if the patient responds to them in a manner that is conducive to producing the desired changes in his personality. We can therefore distinguish three elements in a therapeutic process: environmental factors, responses by the patient, and ultimate personality changes.

scholarly journals Lasting effects of a single psilocybin dose on resting-state functional connectivity in healthy individuals

2021 ◽  
pp. 026988112110264
Author(s):  
Drummond E-Wen McCulloch ◽  
Martin Korsbak Madsen ◽  
Dea Siggaard Stenbæk ◽  
Sara Kristiansen ◽  
Brice Ozenne ◽  
...  
Keyword(s):  
Functional Connectivity ◽  
Resting State ◽  
Clinical Symptoms ◽  
Brain Connectivity ◽  
Well Being ◽  
Long Term Effects ◽  
Resting State Functional Connectivity ◽  
Positive Effects ◽  
Personality Changes ◽  
Psychedelic Drug

Background: Psilocybin is a psychedelic drug that has shown lasting positive effects on clinical symptoms and self-reported well-being following a single dose. There has been little research into the long-term effects of psilocybin on brain connectivity in humans. Aim: Evaluate changes in resting-state functional connectivity (RSFC) at 1 week and 3 months after one psilocybin dose in 10 healthy psychedelic-naïve volunteers and explore associations between change in RSFC and related measures. Methods: Participants received 0.2–0.3 mg/kg psilocybin in a controlled setting. Participants completed resting-state functional magnetic resonance imaging (fMRI) scans at baseline, 1-week and 3-month post-administration and [11C]Cimbi-36 PET scans at baseline and 1 week. We examined changes in within-network, between-network and region-to-region RSFC. We explored associations between changes in RSFC and psilocybin-induced phenomenology as well as changes in psychological measures and neocortex serotonin 2A receptor binding. Results: Psilocybin was well tolerated and produced positive changes in well-being. At 1 week only, executive control network (ECN) RSFC was significantly decreased (Cohen’s d = −1.73, pFWE = 0.010). We observed no other significant changes in RSFC at 1 week or 3 months, nor changes in region-to-region RSFC. Exploratory analyses indicated that decreased ECN RSFC at 1 week predicted increased mindfulness at 3 months ( r = −0.65). Conclusions: These findings in a small cohort indicate that psilocybin affects ECN function within the psychedelic ‘afterglow’ period. Our findings implicate ECN modulation as mediating psilocybin-induced, long-lasting increases in mindfulness. Although our findings implicate a neural pathway mediating lasting psilocybin effects, it is notable that changes in neuroimaging measures at 3 months, when personality changes are observed, remain to be identified.

Neurological soft signs significantly differentiate schizophrenia patients from healthy controls

2017 ◽  
Vol 30 (2) ◽  
pp. 97-105 ◽  
Author(s):  
Konstantinos N. Fountoulakis ◽  
Panagiotis Panagiotidis ◽  
Xenia Gonda ◽  
Vasilios Kimiskidis ◽  
Ioannis Nimatoudis
Keyword(s):  
Clinical Symptoms ◽  
Linear Regression Analysis ◽  
Age At Onset ◽  
Correlation Coefficients ◽  
Analysis Of Covariance ◽  
Neurological Soft Signs ◽  
Dsm Iv ◽  
Medication Status ◽  
Clinical Subtype ◽  
Normal Controls

ObjectiveNeurological soft signs (NSS) are a group of minor non-localisable neurological abnormalities found more often in patients with schizophrenia. The aim of the current study was to test for the effect of gender, age, parental age, age at onset and clinical symptomatology on NSS.Material and methodsThe study sample included 133 patients suffering from schizophrenia according to DSM-IV-TR (77 males and 56 females; aged 33.55±11.22 years old) and 122 normal controls (66 males and 56 females; aged 32.89±9.91 years old). The assessment included the Neurological Evaluation Scale (NES), and a number of scales assessing the clinical symptoms and adverse effects especially extrapyramidal. The statistical analysis included exploratory t-test, simple linear regression analysis, analysis of covariance and the calculation of correlation coefficients.ResultsThe results of the current study confirm that NSS are more frequent in patients with schizophrenia in comparison with normal controls (Wilks=0.622, p<0.0001), but do not support an effect of gender, age, age at onset, paternal or maternal age, education, medication status or clinical subtype of schizophrenia on NES scores.DiscussionOverall these results suggest that NSS constitute an independent (from the rest of symptoms), core (present in the vast majority of patients) and trait (unrelated to age and probably to the stage of schizophrenia) symptom of schizophrenia which could be of value in the clinical assessment and research of schizophrenia. Overall these results are not in full accord with the literature, but they could serve to fill in gaps and inconsistencies observed so far.

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