We report clinical and detailed nerve conduction findings in case of polyneuropathy associated with kappa light chains monoclonal gammopathy of undetermined significance with progression to lymphoproliferative disorder. A 55-year-old man had a predominantly distal, chronic (5 years duration), slowly progressive, symmetric, predominantly sensory impairment with sensory ataxia, and mild weakness. M protein was identified by serum protein electrophoresis. The kappa/lambda ratio of free light chains was significantly elevated to 11.96. The cerebrospinal fluid protein level was elevated at 3.5 g/L. This case study has revealed 2 unusual electrophysiological phenomena-a very unusual prolongation of distal motor latencies of compound muscle action potentials (CMAP) up to 86.5 ms and impaired excitability of the distal segments of the peripheral nerves. The distal CMAP areas were considerably lower compared with the proximal CMAP areas. Radiography of the skull revealed osteolytic lesions.
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http://dx.doi.org/10.1097/CND.0000000000000357 | DOI Listing |
Brain
March 2025
School of Life Science and Technology, ShanghaiTech University, Shanghai 201210, China.
Patients with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, often present with severe psychiatric symptoms, yet the neuropathological mechanisms underlying their cognitive deficits remain insufficiently understood. In this study, we constructed an animal model using anti-NMDAR IgG purified from the serum of patients with anti-NMDAR encephalitis, and we used IgG obtained from healthy individuals as a control. Daily administration of anti-NMDAR IgG into the medial prefrontal cortex (mPFC) of mice for 7 days resulted in cognitive impairments resembling clinical symptoms, which spontaneously resolved 30 days after discontinuing the injections.
View Article and Find Full Text PDFMol Autism
March 2025
Children's Hospital Affiliated to Zhengzhou University, Henan Children's Hospital, Zhengzhou Children's Hospital, Henan Key Laboratory of Children's Genetics and Metabolic Diseases, Henan Children's Neurodevelopment Engineering Research Center, Zhengzhou, China.
Dysfunction in social interactions is a core symptom of autism spectrum disorder (ASD). Nevertheless, the neural mechanisms underlying social deficits in ASD are poorly understood. By integrating electrophysiological, in vivo fiber photometry, viral-mediated tracing, optogenetic and pharmacological stimulation, we show reduced intrinsic excitability and hypoactivity of SOM interneurons in medial prefrontal cortex (mPFC) in Magel2-deficient mice, an established ASD model, were required to social defects.
View Article and Find Full Text PDFeNeuro
March 2025
Michael Smith Laboratories, University of British Columbia. 2185 East Mall. Vancouver, B.C., V6T 1Z4, Canada.
T-type calcium channels shape neuronal excitability driving burst firing, plasticity and neuronal oscillations that influence circuit activity. The three biophysically distinct T-type channel subtypes (Cav3.1, Cav3.
View Article and Find Full Text PDFMult Scler Relat Disord
March 2025
Faculty of Sport, University of Ljubljana, Gortanova 22, Ljubljana 1000, Slovenia. Electronic address:
Background: Submotor-threshold electrical stimulation (subES) can enhance motoneuron excitability, presenting a potential strategy for improving exercise efficiency in people with multiple sclerosis (PwMS).
Objective: This study aimed to investigate the effects of superimposed subES of the tibial nerve during voluntary isometric contraction on ankle plantarflexor torque production.
Methods: A total of 48 participants, comprising both PwMS and healthy participants, performed plantarflexion at three voluntary isometric contraction (MVIC) levels: 20, 60, and 100 %.
Epilepsia
March 2025
Department of Physiology, Niigata University School of Medicine, Niigata, Japan.
Objective: Clinical investigators have hypothesized that interictal epileptiform discharges (IEDs) generated by hypothalamic hamartoma (HH) lead to cognitive dysfunction in patients with drug-resistant gelastic seizures. Herein we provide causal evidence supporting this hypothesis by demonstrating that excitatory neural bursts, when propagating from the HH to the mediodorsal thalamus during the encoding period, impair working memory.
Methods: By employing channelrhodopsin-2 photostimulation, we induced excessive neural excitation in Long-Evans rats, resembling IEDs, at the axon terminals of the lateral hypothalamus projecting toward the mediodorsal thalamus and prelimbic cortex.
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