Download full-text PDF

Source
http://dx.doi.org/10.1136/jnnp-2011-300783DOI Listing

Publication Analysis

Top Keywords

brainstem spinal
4
spinal cord
4
cord motor
4
motor neuron
4
neuron involvement
4
involvement optineurin
4
optineurin inclusions
4
inclusions proximal-dominant
4
proximal-dominant hereditary
4
hereditary motor
4

Similar Publications

Hodological patterning as an organizing principle in vertebrate motor circuitry.

Front Neuroanat

January 2025

Department of Molecular Medicine, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway.

Hodological patterning refers to developmental mechanisms that link the location of neurons in the brain or spinal cord to specific axonal trajectories that direct connectivity to synaptic targets either within the central nervous system or in the periphery. In vertebrate motor circuits, hodological patterning has been demonstrated at different levels, from the final motor output of somatic and preganglionic autonomic neurons targeting peripheral motoneurons and ganglion cells, to premotor inputs from spinal and brainstem neuron populations targeting the somatic motoneurons and preganglionic autonomic neurons, to cortical neurons that delegate movement commands to the brainstem and spinal neurons. In many cases molecular profiling reveals potential underlying mechanisms whereby selective gene expression creates the link between location and axon trajectory.

View Article and Find Full Text PDF

Cerebrospinal fluid-contacting neurons (CSF-cNs) exhibit neural stem cell (NSC) properties both in vitro and in vivo, and they may play a critical role in recovery after spinal cord injury (SCI). GABA receptors (GABABRs) are expressed in Pkd2l1 CSF-cNs. However, their role in Pkd2l1 CSF-cNs still needs to be discovered.

View Article and Find Full Text PDF

BRAF inhibitor monotherapy in BRAFV600E-mutated pediatric low-grade glioma: a single center's experience.

Front Oncol

January 2025

Department of Pediatrics, Children's Healthcare of Atlanta, Atlanta, GA, United States.

Background: Pediatric low-grade gliomas (pLGGs) have an overall survival of over 90%; however, patients harboring a BRAF alteration may have worse outcomes, particularly when treated with classic chemotherapy. Combined BRAF/MEK inhibition following incomplete resection demonstrated improved outcome in BRAF altered pLGG compared to combined carboplatin/vincristine chemotherapy and is now considered the standard FDA-approved treatment for this group of tumors. The aim herein was to investigate the efficacy and tolerability of single agent BRAF inhibitor treatment in BRAF altered pLGG.

View Article and Find Full Text PDF

Bladder dysfunction is a common clinical problem in stroke patients and a strong prognostic factor of disability and exerts an enormous impact on health and economy. The aim of this narrative review was tο examine the pathophysiological mechanisms of lower urinary tract symptoms after stroke, as well as the relevant clinical anatomy. Normal micturition is achieved through complex coordination between brain regions, spinal cord, and peripheral nerves, and anatomic brain connectivity is crucial to lower urinary tract physiology.

View Article and Find Full Text PDF

Central projections of nociceptive input originating from the low back and limb muscle in rats.

Sci Rep

January 2025

Department of Physical Therapy, Faculty of Rehabilitation, Niigata University of Health and Welfare, 1398 Shimami-cho, Kita-ku, Niigata, 950-3198, Japan.

Since clinical features of chronic muscle pain originating from the low back and limbs are different (higher prevalence and broader/duller sensation of low back muscle pain than limb muscle pain), spinal and/or supraspinal projection of nociceptive information could differ between the two muscles. We tested this hypothesis using c-Fos immunohistochemistry combined with retrograde-labeling of dorsal horn (DH) neurons projecting to ventrolateral periaqueductal grey (vlPAG) or ventral posterolateral nucleus of the thalamus (VPL) by fluorogold (FG) injections into the vlPAG or VPL. C-Fos expression in the DH was induced by injecting 5% formalin into the multifidus (MF, low back) or gastrocnemius-soleus (GS, limb) muscle.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!