MRI Findings and Evoked Potentials in Patients with Myotonic Dystrophy versus Facioscapulohumeral Dystrophy

1997 ◽  
Vol 10 (4) ◽  
pp. 437-441
Author(s):  
L. Manfrè ◽  
A. Banco ◽  
M. Midiri ◽  
G. Sparacia ◽  
S. Pappalardo ◽  
...  
Keyword(s):  
Evoked Potentials ◽  
Muscular Atrophy ◽  
Facioscapulohumeral Muscular Dystrophy ◽  
Voluntary Contraction ◽  
Clinical Severity ◽  
Mri Findings ◽  
Auditory Evoked Responses ◽  
The Face ◽  
The Central Nervous System ◽  
Severity Of The Disease

Evoked potentials recordings have been applied to many neurological disorders, localizing the lesions in the central nervous system (CNS) pathways. Facioscapulohumeral muscular dystrophy (FSHD) is a slowly progressive degenerative disease involving the muscles of the face and shoulders. On the contrary, myotonic distrophy (MD), the most frequent and severe myotonic disease, is caracterized by myotonia (delay of relaxation after voluntary contraction), muscular atrophy and dystrophic changes in non-muscular tissues. In the present investigation, patients with clinically and electromyographically verified FSHD and MD were examined using somatosensory evoked potentials (SEP) and brainstem auditory evoked responses (BAEP). The main purpose of the investigation was to determine whether impaired central conduction in patients with MD or FSHD could be associated to specific cerebral abnormalities detected by MRI. Nine patients with FSHD (7 males, 2 females) 22 to 43 y.o. (mean age 35.11 +/– 9.32 y.o.) and ten patients with MD (8 males, 2 females), aged 24 to 48 (mean age 36.27 +/– 11.80 y.o.), were examined. Our results suggest that patients with FSHD may present subclinical involvement of the WM on MRI examinations and of the afferent sensory and auditory system at SEP/BAEP analysis irrespective of age, duration or clinical severity of the disease.

scholarly journals Viruses and Endogenous Retroviruses as Roots for Neuroinflammation and Neurodegenerative Diseases

2021 ◽  
Vol 15 ◽  
Author(s):  
Christine Römer
Keyword(s):  
Neurodegenerative Diseases ◽  
Viral Infections ◽  
Endogenous Retroviruses ◽  
Clinical Severity ◽  
Inflammation Markers ◽  
The Central Nervous System ◽  
Severity Of The Disease ◽  
Line Elements ◽  
The Brain ◽  
Existing Data

Many neurodegenerative diseases are associated with chronic inflammation in the brain and periphery giving rise to a continuous imbalance of immune processes. Next to inflammation markers, activation of transposable elements, including long intrespersed nuclear elements (LINE) elements and endogenous retroviruses (ERVs), has been identified during neurodegenerative disease progression and even correlated with the clinical severity of the disease. ERVs are remnants of viral infections in the human genome acquired during evolution. Upon activation, they produce transcripts and the phylogenetically youngest ones are still able to produce viral-like particles. In addition, ERVs can bind transcription factors and modulate immune response. Being between own and foreign, ERVs are reviewed in the context of viral infections of the central nervous system, in aging and neurodegenerative diseases. Moreover, this review tests the hypothesis that viral infection may be a trigger at the onset of neuroinflammation and that ERVs sustain the inflammatory imbalance by summarizing existing data of neurodegenerative diseases associated with viruses and/or ERVs.

Effects of Alcohol on Evoked Potentials of Various Parts of the Central Nervous System of Cat

1968 ◽  
Vol 29 (1) ◽  
pp. 20-37 ◽  
Author(s):  
Raoul DiPerri ◽  
Anant Dravid ◽  
Arlene Schweigerdt ◽  
Harold E. Himwich
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Evoked Potentials ◽  
The Central Nervous System

Serial CT and MRI findings in a patient with isolated angiitis of the central nervous system associated with cerebral amyloid angiopathy

Amyloid ◽  
2002 ◽  
Vol 9 (4) ◽  
pp. 256-262 ◽  
Author(s):  
Takashi Oide ◽  
Takahiko Tokuda ◽  
Yo-Ichi Takei ◽  
Hitoshi Takahashi ◽  
Kenjiro Ito ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cerebral Amyloid Angiopathy ◽  
Amyloid Angiopathy ◽  
Mri Findings ◽  
The Central Nervous System ◽  
Serial Ct ◽  
Cerebral Amyloid ◽  
Ct And Mri

The cephalic neural crest provides pericytes and smooth muscle cells to all blood vessels of the face and forebrain

Development ◽  
2001 ◽  
Vol 128 (7) ◽  
pp. 1059-1068 ◽  
Author(s):  
H.C. Etchevers ◽  
C. Vincent ◽  
N.M. Le Douarin ◽  
G.F. Couly
Keyword(s):  
Smooth Muscle ◽  
Smooth Muscle Cells ◽  
Neural Crest ◽  
Muscle Cells ◽  
Embryonic Cell ◽  
Avian Embryo ◽  
Connective Tissues ◽  
The Face ◽  
Mesodermal Origin ◽  
The Central Nervous System

Most connective tissues in the head develop from neural crest cells (NCCs), an embryonic cell population present only in vertebrates. We show that NCC-derived pericytes and smooth muscle cells are distributed in a sharply circumscribed sector of the vasculature of the avian embryo. As NCCs detach from the neural folds that correspond to the future posterior diencephalon, mesencephalon and rhombencephalon, they migrate between the ectoderm and the neuroepithelium into the anterior/ventral head, encountering mesoderm-derived endothelial precursors. Together, these two cell populations build a vascular tree rooted at the departure of the aorta from the heart and ramified into the capillary plexi that irrigate the forebrain meninges, retinal choroids and all facial structures, before returning to the heart. NCCs ensheath each aortic arch-derived vessel, providing every component except the endothelial cells. Within the meninges, capillaries with pericytes of diencephalic and mesencephalic neural fold origin supply the forebrain, while capillaries with pericytes of mesodermal origin supply the rest of the central nervous system, in a mutually exclusive manner. The two types of head vasculature contact at a few defined points, including the anastomotic vessels of the circle of Willis, immediately ventral to the forebrain/midbrain boundary. Over the course of evolution, the vertebrate subphylum may have exploited the exceptionally broad range of developmental potentialities and the plasticity of NCCs in head remodelling that resulted in the growth of the forebrain.

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA IN CHILDHOOD AND ADOLESCENCE

PEDIATRICS ◽  
1967 ◽  
Vol 39 (5) ◽  
pp. 675-688
Author(s):  
Denis R. Miller ◽  
Robert L. Baehner ◽  
Louis K. Diamond
Keyword(s):  
Fetal Hemoglobin ◽  
Normal Activity ◽  
Glycolytic Enzyme ◽  
Clinical Severity ◽  
Childhood And Adolescence ◽  
Red Cell ◽  
Red Cell Membrane ◽  
Severity Of The Disease ◽  
Increased Activity ◽  
Aldolase A

Two cases of PNH in adolescence and childhood are reported. The first presented at age 7½ years with aplastic anemia and improved after splenectomy performed at age 14. The second, a 15-year-old girl, presented with a Coombs-positive hemolytic anemia and has had a course complicated by multiple peripheral thromboses. The clinical and laboratory manifestations, complications, and certain therapeutic aspects of PNH are discussed. Anticoagulant therapy appears indicated in the presence of multiple thrombotic episodes. Erythrocyte metabolic studies revealed normal glycolysis, ATP stability, and GSH content in the cells of a child with a normal reticulocyte count. Mild elevations of glycolysis, noted in the child with a reticulocytosis, was ascribed to a younger mean red cell population since further elevations found in the "top" reticulocyte-rich layer after centrifugation. Heparin, the anticoagulant used in these studies, had no adverse effect on glycolysis but did inhibit hemolysis and minimize ATP instability when compared to cells suspended in defibrinated serum. Erythrocytes fractionated by centrifugation revealed increased glycolytic enzyme activities of hexokinase, G3PD, PGK, TPI, PK, LDH, G6PD, and 6PGD in the reticulocyte-rich layer. Normal, rather than increased activity of aldolase, a membrane enzyme, may reflect damage to the red cell membrane. PFK, known to be decreased in the erythrocyte of neonates, showed normal activity, but it was lowest in the reticulocyte-rich layer. Fetal hemoglobin was elevated in this layer. AChE deficiency and increased suceptibility to hydrogen perioxide and acid hemolysis confirmed previous reports and were most marked in the young cell layer. The level of increased glycolytic rates and enzyme activity, AChE deficiency, acid hemolysis and peroxide hemolysis were related to the clinical severity of the disease.

scholarly journals Corticospinal excitability of the biceps brachii is shoulder position dependent

2017 ◽  
Vol 118 (6) ◽  
pp. 3242-3251 ◽  
Author(s):  
Brandon Wayne Collins ◽  
Edward W. J. Cadigan ◽  
Lucas Stefanelli ◽  
Duane C. Button
Keyword(s):  
Evoked Potentials ◽  
Maximal Voluntary Contraction ◽  
Biceps Brachii ◽  
Motor Evoked Potentials ◽  
Voluntary Contraction ◽  
Corticospinal Excitability ◽  
State Dependent ◽  
Shoulder Flexion ◽  
Erb’S Point ◽  
Shoulder Position

The purpose of this study was to examine the effect of shoulder position on corticospinal excitability (CSE) of the biceps brachii during rest and a 10% maximal voluntary contraction (MVC). Participants ( n = 9) completed two experimental sessions with four conditions: 1) rest, 0° shoulder flexion; 2) 10% MVC, 0° shoulder flexion; 3) rest, 90° shoulder flexion; and 4) 10% MVC, 90° shoulder flexion. Transcranial magnetic, transmastoid electrical, and Erb’s point stimulation were used to induce motor-evoked potentials (MEPs), cervicomedullary MEPs (CMEPs), and maximal muscle compound potentials (Mmax), respectively, in the biceps brachii in each condition. At rest, MEP, CMEP, and Mmax amplitudes increased ( P < 0.01) by 509.7 ± 118.3%, 113.3 ± 28.3%, and 155.1 ± 47.9%, respectively, at 90° compared with 0°. At 10% MVC, MEP amplitudes did not differ ( P = 0.08), but CMEP and Mmax amplitudes increased ( P < 0.05) by 32.3 ± 10.5% and 127.9 ± 26.1%, respectively, at 90° compared with 0°. MEP/Mmax increased ( P < 0.01) by 224.0 ± 99.1% at rest and decreased ( P < 0.05) by 51.3 ± 6.7% at 10% MVC at 90° compared with 0°. CMEP/Mmax was not different ( P = 0.22) at rest but decreased ( P < 0.01) at 10% MVC by 33.6 ± 6.1% at 90° compared with 0°. EMG increased ( P < 0.001) by 8.3 ± 2.0% at rest and decreased ( P < 0.001) by 21.4 ± 4.4% at 10% MVC at 90° compared with 0°. In conclusion, CSE of the biceps brachii was dependent on shoulder position, and the pattern of change was altered within the state in which it was measured. The position-dependent changes in Mmax amplitude, EMG, and CSE itself all contribute to the overall change in CSE of the biceps brachii. NEW & NOTEWORTHY We demonstrate that when the shoulder is placed into two common positions for determining elbow flexor force and activation, corticospinal excitability (CSE) of the biceps brachii is both shoulder position and state dependent. At rest, when the shoulder is flexed from 0° to 90°, supraspinal factors predominantly alter CSE, whereas during a slight contraction, spinal factors predominantly alter CSE. Finally, the normalization techniques frequently used by researchers to investigate CSE may under- and overestimate CSE when shoulder position is changed.

Clinical Features and Endolymphatic Hydrops in Patients With MRI Evidence of Hydrops

2018 ◽  
Vol 128 (4) ◽  
pp. 286-292 ◽  
Author(s):  
Suming Shi ◽  
Ping Guo ◽  
Wenquan Li ◽  
Wuqing Wang
Keyword(s):  
Diagnostic Criteria ◽  
Clinical Features ◽  
Clinical Characteristics ◽  
Clinical Symptoms ◽  
Endolymphatic Hydrops ◽  
Low Frequency ◽  
Clinical Severity ◽  
Mri Findings ◽  
Vestibular Symptoms ◽  
Magnetic Resonance Imaging Mri

Objectives: The purpose of this study was to investigate the correlation between grades of endolymphatic hydrops (ELH) and clinical characteristics and determine the detailed clinical characteristics of Ménière’s disease (MD) patients with evidence of hydrops based on magnetic resonance imaging (MRI). Methods: One hundred ninety-eight MD patients (396 ears) with MRI evidence of hydrops were included. ELH grades were evaluated using the Nakashima grading standard. Correlations between the extent of ELH and clinical features were evaluated. Detailed clinical characteristics were analyzed to assess the clinical diagnostic criteria. Results: Of 198 patients, ELH was observed in 100% of cases on the clinically affected side and 8.6% of cases on the asymptomatic side. In addition, 98.5% of ELH was classified as moderate or significant grade. Low-frequency hearing loss was significantly correlated with the extent of both vestibular and cochlear hydrops, whereas the vertigo attack frequency showed no significant correlation with ELH grades. The disease duration of MD with bilateral ELH was longer than that with unilateral ELH. The clinical characteristics were variant and did not completely fit the proposed diagnostic criteria. Conclusions: MRI findings have relevance to the clinical severity, to a certain extent, but not vestibular symptoms. The proposed diagnostic criteria based on clinical characteristics may be partially effective; analysis of the detailed clinical characteristics of MD was meaningful. Diagnosis of MD based on both MRI and clinical symptoms could facilitate an early diagnosis.

Cerebral blood flow and evoked potentials during Cushing response in sheep

1989 ◽  
Vol 256 (3) ◽  
pp. H779-H788
Author(s):  
R. C. Koehler ◽  
J. E. Backofen ◽  
R. W. McPherson ◽  
M. D. Jones ◽  
M. C. Rogers ◽  
...  
Keyword(s):  
Blood Flow ◽  
Cardiac Output ◽  
Cerebral Blood Flow ◽  
Intracranial Pressure ◽  
Arterial Pressure ◽  
Evoked Potentials ◽  
Aortic Pressure ◽  
Base Line ◽  
Auditory Evoked Responses ◽  
Cushing Response

We determined how alterations in systemic hemodynamics, characteristic of the Cushing response, are related to changes in cerebral blood flow (CBF), cerebral metabolic rate of O2 (CMRO2), and brain electrical conductive function, as assessed by somatosensory-evoked potentials (SEP) and brain stem auditory-evoked responses (BAER). In three groups of eight pentobarbital-anesthetized sheep, intracranial pressure was gradually elevated to within 50, 25, or 0 mmHg of base-line mean arterial pressure and then held constant for 40 min by intraventricular infusion of mock cerebrospinal fluid. Microsphere-determined CBF fell when cerebral perfusion pressure was less than 50 mmHg. CMRO2 fell when CBF fell greater than 30-40%. Mean aortic pressure and cardiac output increased when CBF fell greater than 40%, i.e., at approximately the level at which CMRO2 fell. Furthermore, the magnitude of the increase in arterial pressure and cardiac output correlated with the reduction of CMRO2. SEP latency did not increase unless CBF fell greater than 55-65%, corresponding to a 20-30% reduction of CMRO2. Increased latency of BAER wave V was associated with a fall in midbrain blood flow of greater than 65-70%. Thus increase in SEP and BAER latencies required reductions of flow greater than those required to elicit a systemic response. This demonstrates that there is a range of intracranial pressure over which the increase in arterial pressure preserves sufficient CBF to sustain minimal electrical conductive function. The best predictor of the onset and magnitude of the Cushing response in adult sheep is the decrease in CMRO2.

scholarly journals Investigation of association between ABO blood groups and COVID-19 clinical severity

2021 ◽  
Vol 8 (12) ◽  
pp. 673-676
Author(s):  
Arzu İrvem ◽  
Abdurrahman Sarmış ◽  
Özlem Akgün Doğan ◽  
Jale Yıldız ◽  
Zafer Habib ◽  
...  
Keyword(s):  
Risk Factors ◽  
Blood Group ◽  
Blood Groups ◽  
Ct Imaging ◽  
Clinical Severity ◽  
Imaging Findings ◽  
Mortality And Morbidity ◽  
Significant Difference ◽  
Important Center ◽  
Severity Of The Disease

Objective: COVID-19 has been detected in Turkey since March 11, 2020. Istanbul has become an important center of the pandemic in Turkey. Various risk factors for COVID-19 infection, mortality, and morbidity are under investigation. Recent studies have suggested that certain blood groups are risk factors for the disease. The aim of this study is the evaluation the relationship between blood groups and the risk of contracting COVID-19 disease, clinical severity of the disease, and CT (computed tomography) imaging findings. Material and Methods: Age, gender, blood group data, clinical severity and CT images of 300 patients who were positive with RT PCR (Reverse transcription-polymerase chain reaction) and were followed up in the clinic were retrospectively scanned and recorded. The clinical severity of the disease and CT imaging findings were scored, and the data were evaluated statistically. Results: While the incidence of COVID-19 was high in the A blood group, it was low in the 0 blood group. Although there was no significant difference between blood types and clinical severity, the involvement in the B blood group was more severe on CT imaging. Conclusion: People with A blood group should pay more attention to protection and isolation. Investigating this difference and underlying pathogenic mechanisms can guide science with advanced studies.

scholarly journals Multifaceted roles of microRNAs: From motor neuron generation in embryos to degeneration in spinal muscular atrophy

eLife ◽  
2019 ◽  
Vol 8 ◽  
Author(s):  
Tai-Heng Chen ◽  
Jun-An Chen
Keyword(s):  
Nervous System ◽  
Neurodegenerative Diseases ◽  
Spinal Muscular Atrophy ◽  
Motor Neuron ◽  
Motor Neurons ◽  
Muscular Atrophy ◽  
Cell Types ◽  
Spinal Motor Neurons ◽  
Potential Applications ◽  
The Central Nervous System

Two crucial questions in neuroscience are how neurons establish individual identity in the developing nervous system and why only specific neuron subtypes are vulnerable to neurodegenerative diseases. In the central nervous system, spinal motor neurons serve as one of the best-characterized cell types for addressing these two questions. In this review, we dissect these questions by evaluating the emerging role of regulatory microRNAs in motor neuron generation in developing embryos and their potential contributions to neurodegenerative diseases such as spinal muscular atrophy (SMA). Given recent promising results from novel microRNA-based medicines, we discuss the potential applications of microRNAs for clinical assessments of SMA disease progression and treatment.

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