RESUMEN
A 32-year-old male presented with unilateral orbital-temporal pulsatile headache, followed by fever in the 38°C range and nausea. The patient experienced two episodes of transient dysarthria and tinnitus, each lasting several minutes. MRI revealed swelling of the left cerebral cortex, enhancement of the leptomeninges, dilation of the left middle cerebral artery, and subcortical FLAIR hypointensity. The clinical presentation and MRI findings raised suspicions of myelin oligodendrocyte glycoprotein (MOG) antibody-associated cortical encephalitis. After two courses of steroid pulse therapy, the patient's headache subsided, and there was a significant improvement in the swelling of the left cerebral cortex. Subsequently, serum MOG antibody positivity was confirmed. While unilateral cortical FLAIR hyperintensity and increased blood flow can be observed in various diseases, MOG antibody-associated cortical encephalitis is notably characterized by subcortical FLAIR hypointensity, a finding more frequently observed in this condition compared to other diseases. In this case, the findings were useful for early diagnosis and intervention.
RESUMEN
Background: Extracellular adenosine 5'-triphosphate (ATP) acts as a signaling molecule in the peripheral nerves, regulating myelination after nerve injury. The present study examined whether the cerebrospinal fluid (CSF) ATP levels in patients with Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are related to disease severity. Methods: CSF ATP levels in 13 patients with GBS and 18 patients with CIDP were compared with those in a control group of 16 patients with other neurological diseases (ONDs). In patients with CIDP, CSF ATP levels were compared before and after treatment. The correlations between CSF ATP levels and other factors, including clinical data and CSF protein levels, were also evaluated. Results: Median CSF ATP levels were significantly higher in patients with GBS and CIDP than in those with ONDs. When patients with CIDP were classified into two groups depending on their responsiveness to immunotherapy, median CSF ATP levels were significantly higher in good responders than in ONDs. CSF ATP levels tended to decrease after treatment in patients with CIDP. In patients with CIDP, there is a negative correlation between CSF ATP and CSF protein levels. Conclusions: CSF ATP levels were increased in patients with GBS and CIDP. In particular, CSF ATP levels tended to decrease following treatment in patients with CIDP. CSF ATP levels may be useful biomarkers for the diagnosis or monitoring of therapeutic effects in patients with GBS and CIDP.
RESUMEN
An 83-year-old man with hepatocellular carcinoma developed muscle weakness, ptosis, and dyspnea 3 weeks after receiving atezolizumab. Soon after, mechanical ventilation was initiated, which was followed by marked blood pressure spikes. The levels of creatine kinase and troponin-I were significantly elevated, and acetylcholine receptor antibodies were positive. The patient was diagnosed with immune checkpoint inhibitor (ICI)-induced myositis, myasthenia gravis (MG), myocarditis, and suspected autoimmune autonomic ganglionopathy (AAG). After immunotherapy, the serum markers and blood pressure normalized, and he was weaned from the ventilator after five months. To our knowledge, this is the first reported case of AAG secondary to ICI-induced myositis, MG, and myocarditis.