We report an atypical Wallenberg syndrome presented with an acute vertigo, preceded by headache, simulating a peripheral vestibular pathology. The first Magnetic Resonance Imaging (MRI) study done in urgencies is negative. Presence of headache, strong lateral ataxia and secondary focally neurological symptoms 48h after the beginning of the crises, lead to a repetition of the MRI which shows a right lateral bulbar infarct. We focus on the semiological elements that can guide us to a lateral bulbar pathology in patients with pseudoperypheric symptoms (axial latero-pulsion, ocular laterodeviation, posterior headache, oculographic test, etc.).
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http://dx.doi.org/10.1016/j.otorri.2009.06.001 | DOI Listing |
Muscle Nerve
January 2025
Division of Neurology, Department of Medicine, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.
Introduction/aims: Spirometry is the conventional means to measure lung function in amyotrophic lateral sclerosis (ALS), but is dependent on patient effort and bulbar strength. We aimed to use electric impedance tomography (EIT), an emerging non-invasive imaging modality, to measure dynamic lung volume changes.
Methods: Twenty-one patients with ALS underwent sitting and supine spirometry for forced vital capacity (FVC), and sitting and supine EIT.
J Mol Neurosci
January 2025
Department of Neurology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science/Peking Union Medical College, Beijing, 100730, China.
CSF1R-related leukoencephalopathy (CSF1R-L) and AARS2-related leukoencephalopathy (AARS2-L) were two disease entities sharing similar phenotype and even pathological changes. Although clinically, radiologically, and pathologically similar, they were caused by mutation of two different genes. As the rarity of the two diseases, the differential diagnosis of them was difficult.
View Article and Find Full Text PDFBMJ Case Rep
January 2025
Department of Rehabilitation Physiotherapy, National Center of Neurology and Psychiatry, Tokyo, Japan.
We report a case of amyotrophic lateral sclerosis (ALS) in a patient in their 50s, presenting with spastic paraparesis and bulbar palsy, treated with lung volume recruitment therapy (LVRT). From early stage in the disease, vital capacity (VC), lung insufflation capacity (LIC) and ALS Functional Rating Scale-Revised scores were regularly measured, and LVRT was continuously performed at home. After 10 years, the patient had complete limb function loss and required nutritional management via gastrostomy and full assistance with daily activities.
View Article and Find Full Text PDFPLoS Genet
January 2025
Department of Pediatric and Adolescent Medicine, Mayo Clinic, 200 1st St. SW, Rochester, Minnesota 55905, United States of America.
Motor neuron diseases, such as amyotrophic lateral sclerosis (ALS) and progressive bulbar palsy, involve loss of muscle control resulting from death of motor neurons. Although the exact pathogenesis of these syndromes remains elusive, many are caused by genetically inherited mutations. Thus, it is valuable to identify additional genes that can impact motor neuron survival and function.
View Article and Find Full Text PDFBrain Commun
January 2025
Neuromuscular Department, Motor Neuron Disease Centre, Queen Square Institute of Neurology, University College London, London WC1N 3BG, UK.
Neuroinflammation impacts on the progression of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder. Specialized pro-resolving mediators trigger the resolution of inflammation. We investigate the specialized pro-resolving mediator blood profile and their receptors' expression in peripheral blood mononuclear cells in relation to survival in ALS.
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