Hypertrophic olivary degeneration (HOD) arises from lesions of the dentato-rubro-olivary pathway (Guillain–Mollaret triangle), and bilateral HOD is the rarest. Our patient, a 42-year-old man with bilateral HOD caused by unilateral midbrain infarction, had both increased dizziness and ataxia as the first symptoms. HOD has no effective treatment and is easily misdiagnosed as other diseases in clinical practice. Our case demonstrated unique HOD symptomatology and emphasizes the important role of magnetic resonance imaging in diagnosing HOD. The use of gabapentin relieved nystagmus in our patient and may provide a reference for the future treatment of such patients.
Although dyskinesia is well recognized in Parkinson's disease, it is generally under acknowledged in multiple system atrophy (MSA). Reported cases of dyskinesia primarily manifest in patients with MSA with predominant parkinsonism (MSA-P), and characteristically present as orofacial dystonia. However, we have observed other manifestations of dyskinesia in our clinical practice. The current report aims to present the specific manifestations of dyskinesia in MSA-P, with videos.
We enrolled six patients with MSA-P with dyskinesia from Xuanwu Hospital. Of these, four had clinically established MSA-P and two had clinically probable MSA-P according to the 2022 Movement Disorder Society criteria for MSA diagnosis. All six patients underwent an acute levodopa challenge test, and videos were recorded during the process.
Dyskinesia had a unilateral distribution in four patients. Three patients presented with peak-dose orofacial dystonia; of these, two were associated with blepharospasm and two were associated with limb dystonia. In addition, we observed that one patient had peak-dose distal lower limb dystonia with upper limb chorea, one patient had wearing-off dystonia of the eyelids, and one patient had diphasic generalized chorea mimicking that of Parkinson's disease.
In addition to orofacial dystonia, the topographic patterns of dyskinesia in MSA-P can manifest as limb dystonia, blepharospasm, and generalized chorea. Moreover, the temporal patterns of dyskinesia in MSA-P can be peak-dose, wearing-off, or diphasic.
Inflammatory reactions are recognized as pivotal in spinal cord injury (SCI), with the anti-inflammatory role of polarized microglia crucial in mitigating such injury. The present study aimed to determine the protective effects of GsMTx4 on functional recovery in a mouse model of SCI and investigate the role of GsMTx4 in cytokine-induced microglial activation and associated molecular mechanisms.
We assessed the effects of GsMTx4 on motor function in a mouse model of SCI, including neuronal survival and activated microglia in the vicinity of the injury after SCI. We also investigated the effects of GsMTx4 on expression of relevant inflammatory factors involved in cytokine-induced microglial activation and the associated signaling pathways.
GsMTx4 effectively promoted functional recovery in mice and alleviated nerve damage after SCI. Additionally, GsMTx4 facilitated the transition of microglia from the M1 phenotype to the M2 phenotype, suppressed microglial activation, and reduced the expression of corresponding inflammatory mediators. These effects may involve modulation of neurogenic inflammation through the Piezo1/NFκB/STAT6 pathway, at least in part.
GsMTx4 safeguards against SCI by regulating microglial polarization, potentially via the Piezo1/NFκB/STAT6 pathway, offering initial evidence supporting the potential therapeutic efficacy of GsMTx4 for treatment of SCI.