scholarly journals Stiff-Eye Syndrome—Anti-GAD Ataxia Presenting with Isolated Ophthalmoplegia: A Case Report

2021 ◽  
Vol 11 (7) ◽  
pp. 932
Author(s):  
Abel Dantas Belém ◽  
Thaís de Maria Frota Vasconcelos ◽  
Rafael César dos Anjos de Paula ◽  
Francisco Bruno Santana da Costa ◽  
Pedro Gustavo Barros Rodrigues ◽  
...  
Keyword(s):  
Motor Neurons ◽  
Complete Resolution ◽  
Stiff Person Syndrome ◽  
Gad Antibodies ◽  
Spinal Motor Neurons ◽  
Immune Mediated ◽  
Progressive Ataxia ◽  
Gaze Holding ◽  
Cerebellar Ataxias ◽  
The Right

Anti-GAD ataxia is one of the most common forms of immune-mediated cerebellar ataxias. Many neurological syndromes have been reported in association with anti-GAD. Ophthalmoparesis has been described in stiff person syndrome. We report a case of anti-GAD ataxia presenting initially with isolated ophthalmoplegia and showing complete resolution after immunotherapy. A 26-year-old male patient presented with ophthalmoparesis characterized by tonic upwards deviation of the right eye. In the following month, he developed progressive ataxia with anti-GAD titers of 1972 UI/mL. After treatment with methylprednisolone and immunoglobulin, there was complete resolution of symptoms and anti-GAD titers decreased. This is the first report of isolated ophthalmoparesis due to tonic eye deviation associated with anti-GAD antibodies without stiff-person syndrome. Tonic eye deviation has been reported in SPS, possibly secondary to continuous discharge in gaze holding neurons in the brainstem (similar to what occurs in spinal motor neurons). With growing evidence for ocular abnormalitites in SPS, anti-GAD associated neurological syndromes should be included in the differential diagnosis of isolated ophthalmoplegia.

scholarly journals Restoration of breathing after opioid overdose and spinal cord injury using temporal interference stimulation

2021 ◽  
Vol 4 (1) ◽  
Author(s):  
Michael D. Sunshine ◽  
Antonino M. Cassarà ◽  
Esra Neufeld ◽  
Nir Grossman ◽  
Thomas H. Mareci ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Motor Neurons ◽  
Drug Overdose ◽  
Cervical Spinal Cord ◽  
Opioid Overdose ◽  
Electrode Position ◽  
Spinal Motor Neurons ◽  
Cord Injury ◽  
Low Amplitude

AbstractRespiratory insufficiency is a leading cause of death due to drug overdose or neuromuscular disease. We hypothesized that a stimulation paradigm using temporal interference (TI) could restore breathing in such conditions. Following opioid overdose in rats, two high frequency (5000 Hz and 5001 Hz), low amplitude waveforms delivered via intramuscular wires in the neck immediately activated the diaphragm and restored ventilation in phase with waveform offset (1 Hz or 60 breaths/min). Following cervical spinal cord injury (SCI), TI stimulation via dorsally placed epidural electrodes uni- or bilaterally activated the diaphragm depending on current and electrode position. In silico modeling indicated that an interferential signal in the ventral spinal cord predicted the evoked response (left versus right diaphragm) and current-ratio-based steering. We conclude that TI stimulation can activate spinal motor neurons after SCI and prevent fatal apnea during drug overdose by restoring ventilation with minimally invasive electrodes.

scholarly journals The atlas of RNase H antisense oligonucleotide distribution and activity in the CNS of rodents and non-human primates following central administration

2020 ◽  
Author(s):  
Paymaan Jafar-nejad ◽  
Berit Powers ◽  
Armand Soriano ◽  
Hien Zhao ◽  
Daniel A Norris ◽  
...  
Keyword(s):  
Motor Neurons ◽  
Muscular Atrophy ◽  
Neurological Diseases ◽  
Cell Types ◽  
Rnase H ◽  
Central Administration ◽  
Spinal Motor Neurons ◽  
New Class ◽  
Wide Range ◽  
Therapeutic Development

Abstract Antisense oligonucleotides (ASOs) have emerged as a new class of drugs to treat a wide range of diseases, including neurological indications. Spinraza, an ASO that modulates splicing of SMN2 RNA, has shown profound disease modifying effects in Spinal Muscular Atrophy (SMA) patients, energizing efforts to develop ASOs for other neurological diseases. While SMA specifically affects spinal motor neurons, other neurological diseases affect different central nervous system (CNS) regions, neuronal and non-neuronal cells. Therefore, it is important to characterize ASO distribution and activity in all major CNS structures and cell types to have a better understanding of which neurological diseases are amenable to ASO therapy. Here we present for the first time the atlas of ASO distribution and activity in the CNS of mice, rats, and non-human primates (NHP), species commonly used in preclinical therapeutic development. Following central administration of an ASO to rodents, we observe widespread distribution and target RNA reduction throughout the CNS in neurons, oligodendrocytes, astrocytes and microglia. This is also the case in NHP, despite a larger CNS volume and more complex neuroarchitecture. Our results demonstrate that ASO drugs are well suited for treating a wide range of neurological diseases for which no effective treatments are available.

scholarly journals Translatomic analysis of regenerating and degenerating spinal motor neurons in injury and ALS

iScience ◽  
2021 ◽  
pp. 102700
Author(s):  
Jennifer L. Shadrach ◽  
Wesley M. Stansberry ◽  
Allison M. Milen ◽  
Rachel E. Ives ◽  
Elizabeth A. Fogarty ◽  
...  
Keyword(s):  
Motor Neurons ◽  
Spinal Motor Neurons ◽  
Spinal Motor

A new method of isolating spinal motor neurons from fetal mouse

2017 ◽  
Vol 288 ◽  
pp. 57-61 ◽  
Author(s):  
Weifang Wang ◽  
Bao Qi ◽  
Hui Lv ◽  
Fei Wu ◽  
Lulu Liu ◽  
...  
Keyword(s):  
Motor Neurons ◽  
New Method ◽  
Fetal Mouse ◽  
Spinal Motor Neurons ◽  
Spinal Motor

scholarly journals Müllerian inhibiting substance contributes to sex-linked biases in the brain and behavior

2009 ◽  
Vol 106 (17) ◽  
pp. 7203-7208 ◽  
Author(s):  
Pei-Yu Wang ◽  
Anna Protheroe ◽  
Andrew N. Clarkson ◽  
Floriane Imhoff ◽  
Kyoko Koishi ◽  
...  
Keyword(s):  
Motor Neurons ◽  
Reproductive Tract ◽  
Behavioral Traits ◽  
Spinal Motor Neurons ◽  
Müllerian Inhibiting Substance ◽  
Males And Females ◽  
Brain And Behavior ◽  
And Behavior ◽  
The Brain ◽  
Mullerian Inhibiting Substance

Many behavioral traits and most brain disorders are common to males and females but are more evident in one sex than the other. The control of these subtle sex-linked biases is largely unstudied and has been presumed to mirror that of the highly dimorphic reproductive nuclei. Sexual dimorphism in the reproductive tract is a product of Müllerian inhibiting substance (MIS), as well as the sex steroids. Males with a genetic deficiency in MIS signaling are sexually males, leading to the presumption that MIS is not a neural regulator. We challenge this presumption by reporting that most immature neurons in mice express the MIS-specific receptor (MISRII) and that male Mis−/− and Misrii−/− mice exhibit subtle feminization of their spinal motor neurons and of their exploratory behavior. Consequently, MIS may be a broad regulator of the subtle sex-linked biases in the nervous system.

Relevant Biophysical Parameters Discrimination along Corticospinal Tract in Patients with Stroke Using Convolutional Neural Networks

2021 ◽  
Vol 51 ◽  
pp. 95-103
Author(s):  
Abderrazek Zeraii ◽  
Amine Ben Slama ◽  
Lazhar Rmili ◽  
Cyrine Drissi ◽  
Mokhtar Mars ◽  
...  
Keyword(s):  
Motor Neurons ◽  
Corticospinal Tract ◽  
Diffusion Tensor ◽  
Biophysical Parameters ◽  
Spinal Motor Neurons ◽  
Fiber Tracts ◽  
Subacute Stroke ◽  
Quantitative Metrics ◽  
White Matter Fiber Tracts

Stroke remains the leading source of long-term disability. As the only direct descending motor pathway, the corticospinal tract (CST) is the primary pathway to innervate spinal motor neurons and one of the most well studied tracts in human neuroanatomy. Its clinical significance can be demonstrated in many distinguished traumatic situations and diseases such as stroke. Along‐tract statistics analysis enables the extraction of quantitative diffusion metrics along specific white matter fiber tracts. Besides quantitative metrics derived from classical diffusion tensor imaging (DTI), such as fractional anisotropy and diffusivities. In this study, we extracted DTI derived quantitative microstructural diffusion metrics along the CST tract in patients with moderate to severe subacute stroke. Respectively DTI metric of individual patient's fiber tract was then plotted. This approach may be useful for future studies that may compare in two different time (acute and chronic). The contribution of this work presents a totally computerized method of DTI image recognition based on conventional neural network (CNN) in order to supply quantitative appraisal of clinical characteristics. The obtained results have achieved an important classification (Accuracy=94.12%) when applying the CNN. The proposed methodology enables us to assess the classification of the used DTI images database within a reduced processing time. Experimental results prove the success of the proposed rating system for a suitable analysis of microstructural diffusion when compared to previous work.

An initial investigation into the effects of the equine transeva technique (pulsating current electrotherapy) on the equine Gluteus superficialis

2021 ◽  
pp. 1-10
Author(s):  
H. Knaggs ◽  
G. Tabor ◽  
J.M. Williams
Keyword(s):  
Motor Neurons ◽  
Pretreatment Condition ◽  
Negative Change ◽  
Temporal Variables ◽  
Before And After ◽  
The Right ◽  
The Impact ◽  
Athletic Horse ◽  
Pulsating Current ◽  
Larger Sample

The equine transeva technique (ETT), is a novel electrotherapy, which utilises pulsating current electrotherapy to target sensory and motor neurons. The technique may facilitate increased circulation and correction of musculoskeletal issues and injuries, such as tendon and ligament tears and muscle atrophy. Despite the importance of understanding the impact of ETT on horses, no current scientific research exists in this area. This preliminary study investigated the effects of ETT on the musculoskeletal system of the horse, specifically within the Gluteus superficialis (GS). Using surface electromyography, muscle workload was measured in 11 sound and healthy horses of varying breeds and disciplines within the inclusion criteria. Integrated electromyography (iEMG) calculated the percentage change in maximal contractions before and after ETT treatment during one minute trials at 30 s intervals. An ANCOVA determined if these constituted significant changes (Bonferroni adjusted alpha: P≤0.02). Significant differences in muscle workload were found on the left side between pre- and post-treatment readings across trials (P≤0.02), however no significant changes occurred for the right side. The majority of horses (82%; n=9) experienced bilateral changes, with 78% of these (n=7) exhibiting a negative change in muscle workload recorded from the pretreatment condition, which may indicate muscular relaxation. The results suggest ETT may have some effect on muscle workload in the athletic horse, however further research is needed to confirm the effects observed. Future studies should include randomising the side which is treated first, a larger sample size, expansion of temporal variables and consideration of a longitudinal study to determine if these trends accrue over multiple maintenance-purposed treatments.

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