A 57‐year‐old man who suffered from a headache for 1 year, accompanied by blurred vision for 7 months and numbness in his left face for 1 week was admitted to the Affiliated Hospital of Zunyi Medical University on May 7, 2022. One year ago, the patient had no obvious precipitating factor of paroxysmal stabbing pain in the whole skull with dizziness, which could be relieved by oneself after lasting for 1–2 min each time, with about 20 episodes per day. The cranial magnetic resonance imaging revealed changes in bilateral frontal lobe ischemic foci, bilateral frontal, ethmoid, sphenoid and maxillary sinusitis, and retinal macular degeneration. After hormone shock treatment, the condition improved. He suffered from headaches again with blurred vision in the right eye 7 months ago and was initially diagnosed with multiple sclerosis. He then was discharged after improvement due to hormone shock therapy. Oral hormone therapy was continued outside the hospital, but he stopped it due to drug side effects (details remained unclear). After cutting off, he developed a headache and visited our hospital once more, the relevant tests were performed and the patient was diagnosed with idiopathic hypertrophic pachymeningitis (IHP). The symptoms were slightly abated after hormone therapy. We hope that through this case report, we can deepen the clinicians' understanding of IHP, and improve the diagnosis rate of the disease through relevant examinations in future clinical work, so that patients can receive timely treatment and the mental pressure and economic burden caused by the disease on patients are reduced.

Mitophagy is a process of removing damaged mitochondria, transferring damaged mitochondria to lysosomes for degradation, thereby regulating the quality of mitochondria and maintaining the stability of the intracellular environment. CME(Clathrin-mediated-endocytosis)plays an important role in the endocytosis and recovery of vesicles. It is also the main way for large parts of substances to enter cells, and it is widely involved in the signal transduction of various physiological activities of cells. Therefore, this study used pentylenetetrazol to ignite the abnormal mitochondrial autophagy in the chronic epilepsy model and the epilepsy model without magnesium-induced neurons. Subsequently, the autophagy inhibitor 3-MA was used to further observe that the inhibition of autophagy caused a decrease in the fluorescence uptake of Tf-A488 in the primary cultured neurons and the hippocampus of experimental mice, and the inhibition of mitochondrial autophagy could inhibit the function of CME. Finally, through PTZ epilepsy model and kainic acid model, we observed the effect of 3-MA on epilepsy behavior and its in-body field potential, and finally clarify the possible mechanism of mitochondrial autophagy regulating CME and participating in epilepsy.

The aim of this article was to analyze the clinical and genetic characteristics of a patient with Huntington's disease and her family. We analyzed the clinical data of a patient with Huntington's disease and her family members in the Department of Neurology of our hospital, drew the genetic pedigree, and used gene fragment analysis to detect and analyze the genes of three people in the family according to the principle of informed consent. The genetic pedigree of the family was consistent with that of autosomal dominant diseases. A total of five people in this family developed the disease, two died, and the main clinical manifestations were dystonia, ataxia, and cognitive impairment. Three people in this family underwent genetic testing, and two exhibited normal genotypes. The cytosine-adenine-guanine trinucleotide (CAG) repeats of the proband were abnormally amplified, and the number of repeats reached 43. The main clinical features of the patient included chronic obscure onset, obvious positive family genetic history, clinical features of involuntary limb movement with cognitive impairment, rapid disease progression, poor treatment effect, and abnormal amplification of CAG repeats as shown through genetic testing. All the above features met the diagnostic criteria of Huntington's disease.

Rationale: Hereditary motor-sensory peripheral neuropathy, or Charot-Marie-Tooth (CMT) Charcot-Marie-Tooth disease is an inherited peripheral neuropathy characterized by progressive limb weakness and muscle atrophy. As the disease progresses, sensory and autonomic involvement may occur. We report a case of CMT associated with SOD1 gene mutation, in order to provide new ideas for clinical disease diagnosis.Patient concerns: A 50-years-old female patient was admitted to the hospital with "progressive weakness of the right lower extremity for 5 years, aggravating, and weakness of the left lower extremity for 4 months".Diagnosis: The patient was diagnosed CMT.Intervention: Nerve nutrition and rehabilitation therapy were given, but the patient's condition still did not improve significantly.Outcomes: The improvement of symptoms was not obvious.Lessons: The clinical manifestations and electromyography results of this patient are consistent with the characteristics of CMT. The peripheral nerve-related hereditary gene test found mutation in SOD1. It is possible that this mutation is linked to CMT. The disease is a neurodegenerative disease, that may be slowed by physical therapy and rehabilitation, but could not be healed.

Background Peroxisome proliferator-activated receptor γ coactivator-1α (PGC-1α), regulated by AMPK, is an important regulator of mitochondrial fusion. At present, whether the AMPK/PGC-1α signaling pathway regulates mitochondrial dynamics in epileptic rats is still unknown. Methods Adult male Sprague-Dawley (SD) rats were randomly divided into five groups: the control group (0.9% saline, n = 5), the EP groups (lithium-pilocarpine was used to induce epilepsy, and tissues were harvested at 6 and 24 h, every time point n = 5), the EP + Compound C group (the specific inhibitor of PGC-1α, 15 mg/kg in 2% DMSO, n = 5), and the EP + DMSO group (0.9% saline + 2% DMSO, n = 5). To observe the seizure susceptibility of the rats to epilepsy by behavioral study. MFN1, MFN2, and PGC-1α were measured using the Western blot and the immunofluorescence analysis. Results In this study, the behavioral results indicate that the seizure susceptibility of the rats to epilepsy was increased when the expression of PGC-1α was inhibited. Subsequently, Western blot results suggested that the expression level of both MFN1 and MFN2 in the hippocampus was higher at 6 and 24 h after an epileptic seizure. Besides, the expression of PGC-1α and MFN2 was significantly decreased in the hippocampus when the epileptic rats were treated with Compound C. Furthermore, the immunofluorescence analysis of the localization of MFN1/2 and PGC-1α showed that MFN1/2 was mainly expressed in neurons but not astrocytes in the hippocampus and cerebral cortex of rats. Meanwhile, PGC-1α colocalized with the excitatory post-synaptic marker PSD95, suggesting that PGC-1α may regulate the seizure susceptibility of the rats by mediating excitatory post-synaptic signaling. Conclusion The AMPK/PGC-1α signaling pathway may play an important role in the lithium-pilocarpine-induced epileptic rat model by mediating the expression of fusion proteins.