cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.19927512.v1
title: Injecction of trigger points in the temporal muscles of patients with miofascial syndrome

description: Objective : The aim was to examine the effect of blocking trigger points in the temporal muscles of patients with masticatory myofascial pain syndrome, fibromyalgia and headache.Method : Seventy patients with one trigger point were randomly divided into 3 groups: injection with saline or anesthetic and non-injected (control).Results : Pain was reduced in 87.71% patients injected with saline and 100% injected with anesthetic. Similar results were obtained for headache frequency. With regard to headache intensity, the injection groups differed from the control group, but not between themselves.Conclusion : Treatment with injection at trigger points decreased facial pain and frequency and intensity of headache. Considering the injected substance there was no difference.
DOI: 10.6084/m9.figshare.19927518
title: Friedrich Nietzsche: the wandering and learned neuropath under Dionisius

description: Friedrich Nietzsche (1844-1900) was a remarkable philologist-philosopher while remaining in a condition of ill-health. Issues about his wandering/disruptive behavior that might be a consequence and/or protection against his cognitive decline and multifaceted disease are presented. The life complex that raises speculations about its etiology is constituted by: insight, creativity and wandering behavior besides several symptoms and signs of disease(s), mainly neurological one. The most important issue to be considered at the moment is not the disease diagnosis (Lissauer’s general paresis or CADASIL, e.g.), but the probable Nietzsche’s great cognitive reserve linked to the multifactorial etiology (genetic and environmental), and shared characteristics both to creativity and psychopathology. This makes any disease seems especial regarding Nietzsche, and whichever the diagnostic hypothesis has to consider the Nietzsche’s unique background to express any disease(s).
DOI: 10.6084/m9.figshare.19927518.v1
title: Friedrich Nietzsche: the wandering and learned neuropath under Dionisius

description: Friedrich Nietzsche (1844-1900) was a remarkable philologist-philosopher while remaining in a condition of ill-health. Issues about his wandering/disruptive behavior that might be a consequence and/or protection against his cognitive decline and multifaceted disease are presented. The life complex that raises speculations about its etiology is constituted by: insight, creativity and wandering behavior besides several symptoms and signs of disease(s), mainly neurological one. The most important issue to be considered at the moment is not the disease diagnosis (Lissauer’s general paresis or CADASIL, e.g.), but the probable Nietzsche’s great cognitive reserve linked to the multifactorial etiology (genetic and environmental), and shared characteristics both to creativity and psychopathology. This makes any disease seems especial regarding Nietzsche, and whichever the diagnostic hypothesis has to consider the Nietzsche’s unique background to express any disease(s).
DOI: 10.6084/m9.figshare.19927539
title: Extratemporal abnormalities in phosphorus magnetic resonance spectroscopy of patients with mesial temporal sclerosis

description: ABSTRACT Objective We evaluated extratemporal metabolic changes with phosphorus magnetic resonance spectroscopy (31P-MRS) in patients with unilateral mesial temporal sclerosis (MTS). Method 31P-MRS of 33 patients with unilateral MTS was compared with 31 controls. The voxels were selected in the anterior, posterior insula-basal ganglia (AIBG, PIBG) and frontal lobes (FL). Relative values of phosphodiesters- PDE, phosphomonoesters-PME, inorganic phosphate - Pi, phosphocreatine- PCr, total adenosine triphosphate [ATPt = γ- + a- + b-ATP] and the ratios PCr/ATPt, PCr/γ-ATP, PCr/Pi and PME/PDE were obtained. Results We found energetic abnormalities in the MTS patients compared to the controls with Pi reduction bilaterally in the AIBG and ipsilaterally in the PIBG and the contralateral FL; there was also decreased PCr/γ-ATP in the ipsilateral AIBG and PIBG. Increased ATPT in the contralateral AIBG and increased γ-ATP in the ipsilateral PIBG were detected. Conclusion Widespread energy dysfunction was detected in patients with unilateral MTS.
DOI: 10.6084/m9.figshare.19927540
title: Current concepts in the treatment of hereditary ataxias

description: ABSTRACT Hereditary ataxias (HA) represents an extensive group of clinically and genetically heterogeneous neurodegenerative diseases, characterized by progressive ataxia combined with extra-cerebellar and multi-systemic involvements, including peripheral neuropathy, pyramidal signs, movement disorders, seizures, and cognitive dysfunction. There is no effective treatment for HA, and management remains supportive and symptomatic. In this review, we will focus on the symptomatic treatment of the main autosomal recessive ataxias, autosomal dominant ataxias, X-linked cerebellar ataxias and mitochondrial ataxias. We describe management for different clinical symptoms, mechanism-based approaches, rehabilitation therapy, disease modifying therapy, future clinical trials and perspectives, genetic counseling and preimplantation genetic diagnosis.
DOI: 10.6084/m9.figshare.19927540.v1
title: Current concepts in the treatment of hereditary ataxias

description: ABSTRACT Hereditary ataxias (HA) represents an extensive group of clinically and genetically heterogeneous neurodegenerative diseases, characterized by progressive ataxia combined with extra-cerebellar and multi-systemic involvements, including peripheral neuropathy, pyramidal signs, movement disorders, seizures, and cognitive dysfunction. There is no effective treatment for HA, and management remains supportive and symptomatic. In this review, we will focus on the symptomatic treatment of the main autosomal recessive ataxias, autosomal dominant ataxias, X-linked cerebellar ataxias and mitochondrial ataxias. We describe management for different clinical symptoms, mechanism-based approaches, rehabilitation therapy, disease modifying therapy, future clinical trials and perspectives, genetic counseling and preimplantation genetic diagnosis.
DOI: 10.6084/m9.figshare.19927542
title: Presence or absence of cognitive complaints in Parkinson’s disease: mood disorder or anosognosia?

description: ABSTRACT We intended to evaluate whether non-demented Parkinsons’s disease (PD) patients, with or without subjective cognitive complaint, demonstrate differences between them and in comparison to controls concerning cognitive performance and mood. We evaluated 77 subjects between 30 and 70 years, divided as follows: PD without cognitive complaints (n = 31), PD with cognitive complaints (n = 21) and controls (n = 25). We applied the following tests: SCOPA-Cog, Trail Making Test-B, Phonemic Fluency, Clock Drawing Test, Boston Naming Test, Neuropsychiatric Inventory, Hospital Anxiety and Depression Scale (HADS) and Beck Depression Inventory. PD without complaints presented lower total score on Scales for outcome of Parkinson’s disease-cognition as compared to controls (p = 0.048). PD with complaints group showed higher scores on HADS (p = 0.011). PD without complaints group showed poorer cognitive performance compared to controls, but was similar to the PD with complaints group. Moreover, this group was different from the PD without complaints and control groups concerning mood.
DOI: 10.6084/m9.figshare.19927542.v1
title: Presence or absence of cognitive complaints in Parkinson’s disease: mood disorder or anosognosia?

description: ABSTRACT We intended to evaluate whether non-demented Parkinsons’s disease (PD) patients, with or without subjective cognitive complaint, demonstrate differences between them and in comparison to controls concerning cognitive performance and mood. We evaluated 77 subjects between 30 and 70 years, divided as follows: PD without cognitive complaints (n = 31), PD with cognitive complaints (n = 21) and controls (n = 25). We applied the following tests: SCOPA-Cog, Trail Making Test-B, Phonemic Fluency, Clock Drawing Test, Boston Naming Test, Neuropsychiatric Inventory, Hospital Anxiety and Depression Scale (HADS) and Beck Depression Inventory. PD without complaints presented lower total score on Scales for outcome of Parkinson’s disease-cognition as compared to controls (p = 0.048). PD with complaints group showed higher scores on HADS (p = 0.011). PD without complaints group showed poorer cognitive performance compared to controls, but was similar to the PD with complaints group. Moreover, this group was different from the PD without complaints and control groups concerning mood.
DOI: 10.6084/m9.figshare.19927543
title: Positive correlation between functional disability, excessive daytime sleepiness, and fatigue in relapsing-remitting multiple sclerosis

description: ABSTRACT Sleep disorders in patients with multiple sclerosis have various causes and interfere with daytime wakefulness. This study assessed the correlation between fatigue, excessive daytime sleepiness and level of disability. Method Retrospective review of medical records from patients with multiple sclerosis to collect data on severity of fatigue, disability, daytime sleepiness, and depression. From 912 medical records reviewed, 122 reported daytime sleepiness: 67% had relapsing remitting, 12% had primary progressive, and 21% had secondary progressive. Results In 95% of the patients with relapsing remitting who complained of daytime sleepiness and fatigue, association was found between these symptoms and neurological disability. Patients with relapsing remitting who complained of daytime sleepiness and fatigue also experienced depression (p = 0.001). No association between fatigue, excessive daytime sleepiness, depression, and disability was found in patients with progressive disease. Conclusion In relapsing remitting, there is correlation between functional disability, excessive daytime sleepiness and fatigue, a finding not confirmed in primary progressive and secondary progressive form.
DOI: 10.6084/m9.figshare.19927543.v1
title: Positive correlation between functional disability, excessive daytime sleepiness, and fatigue in relapsing-remitting multiple sclerosis

description: ABSTRACT Sleep disorders in patients with multiple sclerosis have various causes and interfere with daytime wakefulness. This study assessed the correlation between fatigue, excessive daytime sleepiness and level of disability. Method Retrospective review of medical records from patients with multiple sclerosis to collect data on severity of fatigue, disability, daytime sleepiness, and depression. From 912 medical records reviewed, 122 reported daytime sleepiness: 67% had relapsing remitting, 12% had primary progressive, and 21% had secondary progressive. Results In 95% of the patients with relapsing remitting who complained of daytime sleepiness and fatigue, association was found between these symptoms and neurological disability. Patients with relapsing remitting who complained of daytime sleepiness and fatigue also experienced depression (p = 0.001). No association between fatigue, excessive daytime sleepiness, depression, and disability was found in patients with progressive disease. Conclusion In relapsing remitting, there is correlation between functional disability, excessive daytime sleepiness and fatigue, a finding not confirmed in primary progressive and secondary progressive form.
DOI: 10.6084/m9.figshare.19927555
title: Aged Lewis rats exposed to low and moderate doses of rotenone are a good model for studying the process of protein aggregation and its effects upon central nervous system cell physiology

description: ABSTRACT Cell physiology is impaired before protein aggregation and this may be more relevant than inclusions themselves for neurodegeneration. The present study aimed to characterize an animal model to enable the analysis of the cell biology before and after protein aggregation. Ten-month-old Lewis rats were exposed either to 1 or 2 mg/kg/day of rotenone, delivered subcutaneously through mini-pumps, for one month. Hyperphosphorylated TAU, alpha-synuclein, amyloid-beta peptide and protein carbonylation (indicative of oxidative stress) were evaluated in the hippocampus, substantia nigra and locus coeruleus through immunohistochemistry or western blot. It was found that 2 mg/kg/day rotenone increased amyloid-beta peptide, hyperphosphorylation of TAU and alpha-synuclein. Rotenone at 1mg/kg/day did not alter protein levels. Protein carbonylation remained unchanged. This study demonstrated that aged Lewis rats exposed to a low dose of rotenone is a useful model to study cellular processes before protein aggregation, while the higher dose makes a good model to study the effects of protein inclusions.
DOI: 10.6084/m9.figshare.19927555.v1
title: Aged Lewis rats exposed to low and moderate doses of rotenone are a good model for studying the process of protein aggregation and its effects upon central nervous system cell physiology

description: ABSTRACT Cell physiology is impaired before protein aggregation and this may be more relevant than inclusions themselves for neurodegeneration. The present study aimed to characterize an animal model to enable the analysis of the cell biology before and after protein aggregation. Ten-month-old Lewis rats were exposed either to 1 or 2 mg/kg/day of rotenone, delivered subcutaneously through mini-pumps, for one month. Hyperphosphorylated TAU, alpha-synuclein, amyloid-beta peptide and protein carbonylation (indicative of oxidative stress) were evaluated in the hippocampus, substantia nigra and locus coeruleus through immunohistochemistry or western blot. It was found that 2 mg/kg/day rotenone increased amyloid-beta peptide, hyperphosphorylation of TAU and alpha-synuclein. Rotenone at 1mg/kg/day did not alter protein levels. Protein carbonylation remained unchanged. This study demonstrated that aged Lewis rats exposed to a low dose of rotenone is a useful model to study cellular processes before protein aggregation, while the higher dose makes a good model to study the effects of protein inclusions.
DOI: 10.6084/m9.figshare.19927557
title: Long term follow-up of growth hormone-secreting pituitary adenomas submitted to endoscopic endonasal surgery

description: ABSTRACT Objective The aim of this study was to evaluate the results of the endoscopic transsphenoidal technique for growth hormone (GH)-secreting adenomas. Methods A retrospective analysis based on medical records of 23 acromegalic patients submitted to endoscopic transsphenoidal surgery. Biochemical control was defined as basal GH &lt; 1ng/ml, nadir GH &lt; 0.4ng/ml after glucose load and age-adjusted IGF-1 normal at the last follow-up. Results The overall endocrinological remission rate was 39.1%. While all microademonas achieved a cure, just one third of macroadenomas went into remission. Suprasellar extension, cavernous sinus invasion and high GH levels were associated with lower rates of disease control. The most common complication was diabetes insipidus and the most severe was an ischemic stroke. Conclusion The endoscopic transsphenoidal approach is a safe and effective technique to control GH-secreting adenomas. The transcavernous approach may increase the risk of complications. Suprasellar and cavernous sinus extensions may preclude gross total resection of these tumors.
DOI: 10.6084/m9.figshare.19927557.v1
title: Long term follow-up of growth hormone-secreting pituitary adenomas submitted to endoscopic endonasal surgery

description: ABSTRACT Objective The aim of this study was to evaluate the results of the endoscopic transsphenoidal technique for growth hormone (GH)-secreting adenomas. Methods A retrospective analysis based on medical records of 23 acromegalic patients submitted to endoscopic transsphenoidal surgery. Biochemical control was defined as basal GH &lt; 1ng/ml, nadir GH &lt; 0.4ng/ml after glucose load and age-adjusted IGF-1 normal at the last follow-up. Results The overall endocrinological remission rate was 39.1%. While all microademonas achieved a cure, just one third of macroadenomas went into remission. Suprasellar extension, cavernous sinus invasion and high GH levels were associated with lower rates of disease control. The most common complication was diabetes insipidus and the most severe was an ischemic stroke. Conclusion The endoscopic transsphenoidal approach is a safe and effective technique to control GH-secreting adenomas. The transcavernous approach may increase the risk of complications. Suprasellar and cavernous sinus extensions may preclude gross total resection of these tumors.
DOI: 10.6084/m9.figshare.19927564
title: Revisiting head circumference of Brazilian newborns in public and private maternity hospitals

description: ABSTRACT Objective To revisit the head circumference (HC) of newborns in public and private maternity hospitals; to correlate our findings with the gestational age, gender, and type of delivery; and build and validate graphs and curves. Methods This was a prospective study performed on healthy newborns. Differences in HC were analyzed as a function of gestational age, gender, the healthcare system and the type of delivery. Smoothed percentile curves were created using the least mean squares method. Results Of the included newborns, 697 were born in private maternity hospitals and 2,150 were born in public maternity hospitals. In all, 839 were born by vaginal delivery, and 1,311 were born by cesarean delivery. At 37 to 42 weeks of gestation, male newborns had a larger HC than females. Infants born in private maternity and those born by cesarean delivery had a larger HC. Conclusion An important result of the present study is that our analyses allowed us to generate curves and statistically-validated graphs that can be used in clinical neonatal practice.
DOI: 10.6084/m9.figshare.19927564.v1
title: Revisiting head circumference of Brazilian newborns in public and private maternity hospitals

description: ABSTRACT Objective To revisit the head circumference (HC) of newborns in public and private maternity hospitals; to correlate our findings with the gestational age, gender, and type of delivery; and build and validate graphs and curves. Methods This was a prospective study performed on healthy newborns. Differences in HC were analyzed as a function of gestational age, gender, the healthcare system and the type of delivery. Smoothed percentile curves were created using the least mean squares method. Results Of the included newborns, 697 were born in private maternity hospitals and 2,150 were born in public maternity hospitals. In all, 839 were born by vaginal delivery, and 1,311 were born by cesarean delivery. At 37 to 42 weeks of gestation, male newborns had a larger HC than females. Infants born in private maternity and those born by cesarean delivery had a larger HC. Conclusion An important result of the present study is that our analyses allowed us to generate curves and statistically-validated graphs that can be used in clinical neonatal practice.
DOI: 10.6084/m9.figshare.19927566
title: The art and neurology of Paul Richer

description: ABSTRACT In the 1890s, one of Charcot’s most important protégés, Dr. Paul Richer (1849–1933), drew and sculpted a series of representations of the main types of nerve pathology. That series included drawings of pleomorphic hysterical crises and sculptures depicting patients suffering from labio-glosso-laryngeal paralysis and myopathy, as well as Parkinson’s disease. Richer was a resident at La Salpêtrière and, in 1882, became head of the Charcot museum. Early in his career, despite having no formal artistic training, he could represent masterfully, in drawings and sculptures, people’s tragic suffering from neurological diseases. Later on, with the same tools, he expressed the beauty of human movements in health.
DOI: 10.6084/m9.figshare.19927566.v1
title: The art and neurology of Paul Richer

description: ABSTRACT In the 1890s, one of Charcot’s most important protégés, Dr. Paul Richer (1849–1933), drew and sculpted a series of representations of the main types of nerve pathology. That series included drawings of pleomorphic hysterical crises and sculptures depicting patients suffering from labio-glosso-laryngeal paralysis and myopathy, as well as Parkinson’s disease. Richer was a resident at La Salpêtrière and, in 1882, became head of the Charcot museum. Early in his career, despite having no formal artistic training, he could represent masterfully, in drawings and sculptures, people’s tragic suffering from neurological diseases. Later on, with the same tools, he expressed the beauty of human movements in health.
DOI: 10.6084/m9.figshare.19927576
title: Brazilian consensus on Duchenne muscular dystrophy. Part 1: diagnosis, steroid therapy and perspectives

description: ABSTRACT Significant advances in the understanding and management of Duchenne muscular dystrophy (DMD) took place since international guidelines were published in 2010. Our objective was to provide an evidence-based national consensus statement for multidisciplinary care of DMD in Brazil. A combination of the Delphi technique with a systematic review of studies from 2010 to 2016 was employed to classify evidence levels and grade of recommendations. Our recommendations were divided in two parts. We present Part 1 here, where we describe the guideline methodology and overall disease concepts, and also provide recommendations on diagnosis, steroid therapy and new drug treatment perspectives for DMD. The main recommendations: 1) genetic testing in diagnostic suspicious cases should be the first line for diagnostic confirmation; 2) patients diagnosed with DMD should have steroids prescribed; 3) lack of published results for phase 3 clinical trials hinders, for now, the recommendation to use exon skipping or read-through agents.
DOI: 10.6084/m9.figshare.19927576.v1
title: Brazilian consensus on Duchenne muscular dystrophy. Part 1: diagnosis, steroid therapy and perspectives

description: ABSTRACT Significant advances in the understanding and management of Duchenne muscular dystrophy (DMD) took place since international guidelines were published in 2010. Our objective was to provide an evidence-based national consensus statement for multidisciplinary care of DMD in Brazil. A combination of the Delphi technique with a systematic review of studies from 2010 to 2016 was employed to classify evidence levels and grade of recommendations. Our recommendations were divided in two parts. We present Part 1 here, where we describe the guideline methodology and overall disease concepts, and also provide recommendations on diagnosis, steroid therapy and new drug treatment perspectives for DMD. The main recommendations: 1) genetic testing in diagnostic suspicious cases should be the first line for diagnostic confirmation; 2) patients diagnosed with DMD should have steroids prescribed; 3) lack of published results for phase 3 clinical trials hinders, for now, the recommendation to use exon skipping or read-through agents.
DOI: 10.6084/m9.figshare.19927578.v1
title: Guillain-Barré syndrome: celebrating a century

description: ABSTRACT A hundred years ago, Guillain, Barré and Strohl described a syndrome with a predominant motor acute or subacute polyneuritis, albumin-cytologic dissociation in the cerebrospinal fluid, and a benign course. Before them, many other authors, such as Landry, Duménil, Osler, and Grainger Stewart had described similar cases although they had not performed lumbar punctures. In this work, we outline certain features of the beginning of this famous syndrome.
DOI: 10.6084/m9.figshare.19927578
title: Guillain-Barré syndrome: celebrating a century

description: ABSTRACT A hundred years ago, Guillain, Barré and Strohl described a syndrome with a predominant motor acute or subacute polyneuritis, albumin-cytologic dissociation in the cerebrospinal fluid, and a benign course. Before them, many other authors, such as Landry, Duménil, Osler, and Grainger Stewart had described similar cases although they had not performed lumbar punctures. In this work, we outline certain features of the beginning of this famous syndrome.