Case Reports in Neurology最新文献

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.

Dementia is a gradual and irreversible loss of higher mental function, particularly memory. Dural arteriovenous fistulas (DAVFs) are one of the rare causes of a rapid decline in cognitive function, which can be curable. DAVFs are pathological shunts between the dural artery and the dural venous sinus, dural vein, or cortical vein. Here, we present a case that initially manifested nausea and dizziness and developed rapidly progressive dementia caused by DAVFs in the left transverse sinus-sigmoid sinus junction area and the sinus confluence area, combined with cerebral venous sinus thrombosis. Moreover, our case has multiple DAVFs that cause bilateral thalamic lesions and rapidly progressive dementia called thalamic dementia, which is infrequent and often misdiagnosed. His symptoms have improved after receiving endovascular embolization treatment. In addition to presenting our case, we conducted a systemic literature review to summarize how familiarity with the manifestation and early diagnosis of bilateral thalamic lesions caused by DAVFs can lead to earlier and more effective therapy.

COVID-19 has disrupted the routine flow of patients through emergency departments (EDs) across the globe, including the need to consider COVID-19 for nearly all presenting complaints. The constraints of mask wearing and isolation have created inherent barriers to timely stroke care. We present a case that highlights one of the many ways in which the pandemic has negatively impacted the care of the non-COVID patient. A patient presented to the ED with a chief complaint of diffuse weakness and a new-onset cough on awakening. His daughter noted that he was slurring his words. An emergency medicine resident evaluated him, ordered laboratory studies, and decided to monitor the patient. The same resident later noted the patient veering to the left when walking, prompting a more detailed neurological examination. On removing the patient's facemask, a left lower facial weakness was evident. The resident called a Code Stroke roughly 50 min after the patient initially presented to the ED. The patient proved to have an acute infarct at the right thalamocapsular junction. Universal masking policies during the COVID-19 pandemic should not prevent the routine assessment of cranial nerve function for all patients presenting to an ED.

Despite the clinical impact of dysphagia in myasthenia gravis (MG), a standard protocol for diagnosing dysphagia reliably has not yet been established. High-resolution manometry (HRM) provides precise information on pharyngeal pressure. We hypothesized that swallowing pressure assessment using HRM during the edrophonium chloride (EC) test could identify mild bulbar symptoms with no abnormalities on videoendoscopic (VE) and videofluorographic (VF) examination of swallowing, and we tested this hypothesis on a 72-year-old female patient diagnosed with ocular MG who developed slight pharyngeal discomfort over 3 months. The patient's ocular symptoms were stable with pyridostigmine medication. VE and VF revealed no abnormalities. The swallowing pressure along the pharynx was measured using HRM during the EC test. HRM parameters, including velopharyngeal contractile integral and meso-hypopharyngeal contractile integral, were evaluated. These parameters were assessed for three swallows using 3 mL of water. After EC injection, the values of the velopharyngeal contractile integral (78.0 ± 5.4 vs. 134.7 ± 1.3 mm Hg cm·s) and the meso-hypopharyngeal contractile integral were both higher (130.6 ± 1.5 vs. 284.2 ± 11.9 mm Hg cm·s) than those observed before EC injection. Chest computed tomography revealed a thymoma that had not been observed in previous examinations. The patient was diagnosed with thymoma-associated MG. Intravenous immunoglobulin therapy improved the mild dysphagia. We concluded that swallowing pressure assessment during the EC test may be helpful in identifying mild bulbar symptoms in patients with MG.

Recombinant tissue plasminogen activator (r-tPA) is the first-line drug for the treatment of acute ischemic stroke, despite it may lead to a variety of complications in some cases. In patients with extensive stroke, infarction of the brain can cause suppression of the respiratory center in the brain leading to neurogenic pulmonary edema that potentially causes respiratory failure. Its etiology is either due to a neurogenic or non-neurogenic process. Nevertheless, the definite pathophysiology of these circumstances remains unclear. In this study, we reported four cases of post-thrombolytic ischemic stroke patients who suffer from pulmonary edema with different symptoms and onset times as well as we discuss the possible explanation behind these different outcomes.

An otherwise healthy 63-year-old woman was given a diagnosis of normal tension glaucoma (NTG) in the right eye (OD) 2 months before presentation. Standard computerized perimetry showed a unilateral right hemianoptic temporal field defect. On examination visual acuity was preserved, intraocular pressure was normal, there was a right relative afferent pupillary defect (RAPD) with an asymmetric cupping of the disc, but no pallor. Brain magnetic resonance imaging (MRI) showed a meningioma compressing the right optic nerve at its junction with the chiasm. Compressive disorders on the anterior chiasm, albeit rarely, may cause cupping of the disc and unilateral temporal visual field defect (junctional scotoma of Traquair) with normal visual acuity that should be considered in the differential diagnosis of NTG.