Case Reports in Neurology最新文献

Epstein-Barr virus (EBV) encephalitis is caused by initial infection or reactivation of EBV. In adults, the risk factors of EBV encephalitis include human immunodeficiency virus infection, immunosuppressant drugs, congenital immune deficiencies, post-stem cell transplantation, and post-solid organ transplantation. However, a few cases of adult-onset EBV encephalitis without these risk factors were also reported. The efficacy of steroid or intravenous immunoglobulin remains unclear in the treatment of EBV encephalitis. Herein, we report a case of an 82-year-old man with fever and disturbance of consciousness who was diagnosed as having EBV encephalitis. Gadolinium-enhanced magnetic resonance imaging showed punctate enhancement in the bilateral basal ganglia, which resembled chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Symptoms and enhanced lesions improved after immunotherapy. Immunotherapy may be effective in the treatment of EBV encephalitis with CLIPPERS-like lesions.

Rhabdomyolysis is an acute disruption in skeletal muscle integrity, leading to the rapid release of 4 muscle contents into the bloodstream, such as creatine kinase (CK). It can have various causes, including infections. Throughout the pandemic, multiple cases of rhabdomyolysis following COVID-19 infections have been reported. However, rhabdomyolysis subsequent to COVID-19 vaccinations appears to be relatively rare. Here, we report such a case after a second COVID-19 Comirnaty (BioNTech/Pfizer) vaccination. Our patient developed rhabdomyolysis 1 day after the second Comirnaty vaccination with high creatine kinase (CK) levels, generalized weakness, and kidney failure. CK levels and muscle weakness resolved after treatment with intravenous fluids, but unfortunately, he remained hemodialysis dependent after discharge. To our knowledge, this is one of the first case reports describing a patient with rhabdomyolysis after a Comirnaty vaccination. However, as millions of people have received the Comirnaty vaccine, it is unclear whether the rhabdomyolysis in our patient is a rare side effect or an unrelated, coincidental event. Large observational studies are needed to elucidate the causality between the Comirnaty vaccination and rhabdomyolysis. Awareness is warranted in patients with myalgia and muscle weakness shortly after COVID-19 vaccination, in order to initiate treatment early and prevent life-threatening complications.

Idiopathic intracranial hypertension (IIH) is a condition of elevated intracranial pressure commonly seen in obese women of childbearing age. Fulminant IIH is a rare subset of IIH that is characterized by rapidly progressive vision loss in less than 4 weeks, and typically requires surgical intervention for treatment. We describe a 36-year-old man with a 3-week history of acute onset vision loss and fulminant IIH in whom severe bilateral hemorrhagic optic disk edema was identified. There were also associated moderate visual field defects. Given the rapid onset of symptoms and severity of papilledema, surgical management was discussed but the patient had opted for medical management and close follow-up. He began oral acetazolamide, which was escalated to the maximal dose of 4 g and seen regularly with close follow-up. Four months after presentation, he was completely symptom free and the bilateral optic disk edema had resolved. His visual fields had also improved. We emphasize the importance of close follow-up in fulminant IIH and highlight that although most cases often require surgical intervention, some patients may show improvement with medical management only.

Pedicle or lateral mass screws, which are usually used to fix atlantoaxial instability, increase the risk of vertebral artery (VA) injury in patients with bone or arterial anomalies or osteoporotic bone. Here, we report the use of a unilateral C1 posterior arch screw-C2 laminar screw posterior fixation with a contralateral C1 lateral mass screw for VA preservation in a patient with bow hunter's syndrome (BHS). A 65-year-old male presented with recurrent loss of consciousness in the right rotational and backward-bending head positions for 1 year. Cerebral angiography in the same head position showed that the left VA was disrupted at C1/2 and the right VA was hypoplastic. The patient was diagnosed with BHS. C1-2 posterior fixation and iliac bone grafting were performed. The left VA was on the dominant side, and the VA was in a high position; thus, a C1 posterior arch screw was selected for the left side, a C1 lateral mass screw was selected for the right side, and a C2 laminar screw with O-arm navigation and a C-arm was used to prevent arterial injury. Intraoperative findings revealed no VA injury, and postoperative computed tomography showed the screw at the planned site. In a patient with BHS, posterior fixation with a unilateral C1 posterior arch screw-C2 laminar screw prevented VA injury because the screw could be inserted while avoiding the VA.

Cetuximab is a chimeric IgG1 monoclonal antibody against epidermal growth factor receptor. It is approved by the European medical agency for the treatment of RAS wild-type metastatic colorectal cancer and metastatic squamous cell cancer of the head and neck. Few cases of aseptic meningitis, primarily associated with the first administration of cetuximab in patients with squamous cell cancer, have been reported. So far, there was only 1 case in a patient with metastatic colorectal cancer. We report on a 50-year-old Caucasian patient with metastatic rectum carcinoma who suffered from headache, fever, and neck stiffness 3 h after the first administration of cetuximab (400 mg/m²). CSF examination revealed an excessive pleocytosis with a white blood cell count of 2,433/µL. He was diagnosed with cetuximab-induced aseptic meningitis since clinical symptoms and CSF pleocytosis resolved within days, and further diagnostic workup revealed no infectious cause. Cetuximab-induced aseptic meningitis is a rare and severe drug reaction with predominance in treating squamous cell cancer of the head and neck. Clinical presentation and CSF findings suggest acute meningoencephalitis. In all reported cases, the course of the disease was benign and self-limited. Empiric antimicrobial and antiviral therapy are suggested until infectious causes can be ruled out. A lower dosage of cetuximab and a premedication including antihistamines and glucocorticosteroids may lower the risk of a re-occurrence if cetuximab therapy is continued.

Rapidly progressive dementia (RPD) is a heterogeneous group of diseases characterized by cognitive impairment and other neurological disorders developed in a short span of fewer than 2 years. Currently viewed as new and infrequent entities, most medical personnel have little understanding of it. Nevertheless, they significantly compromise many patients' quality of life. Here, we drive 3 clinical cases that evolve as RPD with different etiologies.

Case 1: 70-year-old woman presented to the emergency with neuropsychiatric syndrome for 18 days. The researchers identified inflammatory cerebrospinal fluid (CSF), protein 14-3-3-positive T-tau protein, MRI: T2 and FLAIR hyperintensities in bilateral caudate nuclei with diffusion restriction, EEG shows a generalized periodic pattern with triphasic wave morphology.

Case 2: 29-year-old man with cognitive impairment and faciobrachial dystonia seizure. The diagnosis was confirmed by achieving elevated antibodies against voltage-gated potassium channels.

Case 3: A 49-year-old woman with encephalopathy and myoclonic seizures; EEG and MRI showed subtle changes. The patient also had a normal CSF but a positive CBA serologic NMDA-R antibody test. We described fundamental aspects of RPD to allow made differential diagnoses in patients with cognitive impairment and encephalopathy. Establishing an early and accurate diagnosis can benefit patients with RPD etiologies that are treatable and even reversible, decreasing in morbidity and mortality.

Eteplirsen is an antisense oligonucleotide used in the treatment of Duchenne muscular dystrophy (DMD). The safety of eteplirsen use in individuals with rare comorbid conditions is not known. We present the case of a 4-year-old boy with a DMD exon deletion amenable to treatment with eteplirsen and comorbid sickle cell anemia. He has received eteplirsen treatment for 3 years with no clear adverse effects, including no increase in sickle cell crises.

Cancer immunotherapies have been revolutionary treatments in oncological disease. Such therapies include immune checkpoint inhibitors that target programmed cell death protein, ligands, and cytotoxic T-lymphocyte-associated antigen (CTLA-4). Increased use has led to recognition of immune-related adverse events. Such events are often distinct from the typical adverse events of traditional cancer therapies. Immune-related adverse events are more commonly found to affect the skin, gastrointestinal tract, and endocrine system. The incidence of these adverse events remains low for central nervous system effects. This article describes a case of atezolizumab-associated encephalitis in a patient with metastatic small cell lung cancer.

F-waves are used to measure the excitability of spinal motor nerve function. This study aimed to investigate the F-wave patterns in a patient with cerebrovascular disease who had no voluntary movement of the hand, particularly the thumb, caused by a considerably increased tone of the thenar muscles. A patient with right hemiplegia caused by left cerebral hemorrhage (putamen) showed a considerably increased tone of the thumb flexors and no voluntary movements. F-waves were recorded from the affected thenar muscles with median nerve stimulation in the supine lying position during the first trial. Exercise therapy that included stretching of the affected thenar muscles was performed twice a week for 20 min for 8 months. Subsequent changes in the F-wave waveform were examined and considered as second trial. The latency and persistence of the F-wave and F-wave conduction velocity did not show any significant change between the two trials. Compared with the first trial, the F/M amplitude ratio in the second trial was increased. Following 8 months of exercise therapy, muscle tone improved slightly, and minimal voluntary movements of the affected thumb were noted. Since motor function of the affected thumb improved with exercise therapy but there was no improvement in F-wave data, it was determined that the main factor underlying the hypertonicity of the thenar muscles in this patient was more likely due to secondary muscle shortening than to spasticity. Unclear waves that possibly were F-waves were also observed approximately 20 ms after the appearance of the M-wave in the first trial but not in the second trial. Because exercise therapy showed muscle tone improvement and did not result in the appearance of unclear waves, F-wave patterns should be monitored for evaluating spasticity, which markedly increases muscle tone in patients with cerebrovascular disease.