Olfaction and incident Parkinson disease in US white and black older adults
Conclusions:
Poor olfaction predicts PD in short and intermediate terms; the possibility of stronger associations among men and white participants warrants further investigation. (Source: Neurology)
Source: Neurology - October 2, 2017 Category: Neurology Authors: Chen, H., Shrestha, S., Huang, X., Jain, S., Guo, X., Tranah, G. J., Garcia, M. E., Satterfield, S., Phillips, C., Harris, T. B., For the Health ABC Study Tags: Parkinson's disease/Parkinsonism ARTICLE Source Type: research
Sickle cell disease and the unmet challenges of neurologic complications
Sometimes an important scientific report is more notable for what it cannot tell us than for what it can. In this issue of Neurology®, Noubiap and colleagues1 provide a comprehensive, well-conducted systematic meta-analysis of the neurologic complications of sickle cell disease (SCD) that incorporates rigorous design and advanced analytic methods to delineate in substantial detail what is known about the prevalence of neurologic injury among people with SCD in Africa—where over half the world's population of people with SCD reside. As one might expect, based upon what is known from more medically developed region...
Source: Neurology - October 2, 2017 Category: Neurology Authors: Birbeck, G. L., Green, R. Tags: EDITORIALS Source Type: research
Amyloid-PET in cerebral amyloid angiopathy: Detecting vascular amyloid deposits, not just blood
Sporadic cerebral amyloid angiopathy (CAA) is a common age-related cerebral small vessel disease characterized by progressive deposition of amyloidal protein made up of (with rare exception) amyloid-β (Aβ) in the media and adventitia of cortical and leptomeningeal vessels.1 Lobar intracerebral hemorrhage (ICH) represents a late, often devastating manifestation of the disease, leading to high mortality, poor functional outcome, and dementia. Definite CAA diagnosis requires a full postmortem neuropathologic examination. However, brain MRI can help to diagnose CAA during life by detecting hemorrhagic lesions such as...
Source: Neurology - October 2, 2017 Category: Neurology Authors: Raposo, N., Sonnen, J. A. Tags: EDITORIALS Source Type: research
The yin and yang of gastrostomy in the management of ALS: Friend or foe?
Though we do not yet have a cure for amyotrophic lateral sclerosis (ALS), we can provide treatment, and the host of medical and other interventions provided by ALS specialists and multidisciplinary care teams increases survival and substantially improves quality of life for patients and their families. Dysphagia is one of the most consequential symptoms in ALS, and ultimately affects the majority of patients. It causes dehydration, weight loss, choking, and chronic aspiration, which substantially increase the risk of potentially fatal aspiration pneumonia. Weight loss alone worsens progression and survival in ALS,1 pr...
Source: Neurology - October 2, 2017 Category: Neurology Authors: de Carvalho, M., Gooch, C. L. Tags: EDITORIALS Source Type: research
Biomarkers for early detection of Parkinson disease: A scent of consistency with olfactory dysfunction
There are approximately 1,000 genes involved in odor recognition and 347 that code for functional receptors. This enables our distinguishing of approximately 4,000–10,000 distinct odorous ligands, a scientific discovery that earned a Nobel Prize in 2004.1 The olfactory nerve (CN1) contains 6–10 million receptor cells whose ciliated dendrites and cell bodies are contained within a specialized (neuro)epithelium located within the posterior nasal cavity (figure).2 These olfactory receptor cells are interspersed with sustentacular cells for stability of the epithelium and basal cells (stem cells) that provide a reg...
Source: Neurology - October 2, 2017 Category: Neurology Authors: Bowman, G. L. Tags: EDITORIALS Source Type: research
Spotlight on the October 3 issue
(Source: Neurology)
Source: Neurology - October 2, 2017 Category: Neurology Authors: Gross, R. A. Tags: IN FOCUS Source Type: research
Teaching Video NeuroImages: Palsy of conjugate horizontal gaze and face due to isolated abducens nuclear infarction
A 58-year-old woman presented leftward conjugate gaze palsy and left facial nerve palsy of a peripheral pattern due to acute infarction restricted to the area of left abducens nucleus (figure, A–C; video at Neurology.org). (Source: Neurology)
Source: Neurology - October 2, 2017 Category: Neurology Authors: Kim, J.-S., Jeong, S.-H., Choi, J.-Y., Kim, H.-J. Tags: Peripheral neuropathy, Ocular motility, Diplopia (double vision), Infarction RESIDENT AND FELLOW SECTION Source Type: research
Teaching Video NeuroImages: Spastic ataxia syndrome: The Friedreich-like phenotype of ARSACS
A 24-year-old Chilean man with slowly progressive ataxia since age 2 presented with spastic ataxia, hyperreflexia, pes cavus, axonal polyneuropathy, incomplete right-bundle branch block on ECG, and impaired glucose tolerance test, suggesting Friedreich ataxia (figure; video at Neurology.org). However, the combination of hyperreflexia and cerebellar (rather than cervical cord) atrophy with T2-weighted linear hypointensity in the pons on brain MRI suggested autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). Biallelic mutations were found (c.4492C>T p.[R1498X] and c.2388dupA p.[L797Ifs*4]) in the SACS gene...
Source: Neurology - October 2, 2017 Category: Neurology Authors: Saffie, P., Kauffman, M. A., Fernandez, J. M., Acosta, I., Espay, A. J., de la Cerda, A. Tags: All Clinical Neurology, Gait disorders/ataxia RESIDENT AND FELLOW SECTION Source Type: research
Teaching NeuroImages: Typical neuroimaging features in high-altitude cerebral edema
A 61-year-old man presented with thunderclap headache followed by loss of consciousness, 2 days after arriving in Atacama Desert, Andes Mountains, Chile, at 4,000 meters. Examination showed coma. Mechanical ventilation was necessary. He had progressive improvement after transfer to low altitudes. Brain MRI showed diffuse vasogenic edema and microhemorrhages (figure), and high-altitude cerebral edema (HACE) was diagnosed. (Source: Neurology)
Source: Neurology - October 2, 2017 Category: Neurology Authors: Marussi, V. H. R., Pedroso, J. L., Piccolo, A. M., Barsottini, O. G., Moraes, F. M. d., Oliveira, A. S. B., Freitas, L. F., Amaral, L. L. F. d. Tags: MRI, Other cerebrovascular disease/ Stroke, All Clinical Neurology RESIDENT AND FELLOW SECTION Source Type: research
Teaching NeuroImages: Rare cause of Horner syndrome in Loeys-Dietz syndrome
A 36-year-old woman with Loeys-Dietz syndrome presented with left eye ptosis, anisocoria, and shoulder pain. The diagnosis of left-sided Horner syndrome was made (figure 1). The patient had a known left subclavian artery aneurysm with percutaneous stent graft placement. CT angiography revealed a large left subclavian aneurysm sac, consistent with endoleak type I (figure 2). Horner syndrome and shoulder pain improved following common carotid to axillary artery bypass in addition to thoracic endovascular aortic repair surgery. (Source: Neurology)
Source: Neurology - October 2, 2017 Category: Neurology Authors: Cho, S.-M., Di Lorenzo, R., Mathew, J., Buletko, A. B. Tags: RESIDENT AND FELLOW SECTION Source Type: research
Teaching NeuroImages: Figure of 8: The clue to the diagnosis of AMPD2 pontocerebellar hypoplasia (PCH9)
A 2-year-old girl, born after uneventful pregnancy from healthy nonconsanguineous parents, presented with failure to thrive, microcephaly, facial dysmorphism, strabismus, nystagmus, axial hypotonia, lower limb spasticity/hyperreflexia, dystonic movements, and no active postures. Since the first months of life, developmental delay without any motor skills acquisition, drug-resistant epilepsy, and progressive spasticity had emerged. Neuroimaging revealed pontocerebellar hypoplasia with the figure of 8 midbrain appearance (figure), a distinctive sign for PCH8 or AMPD2 deficiency.1,2 Sanger sequencing of the adenosine monophos...
Source: Neurology - October 2, 2017 Category: Neurology Authors: Severino, M., Zara, F., Rossi, A., Striano, P. Tags: MRI, All Clinical Neurology, All Pediatric, All Genetics RESIDENT AND FELLOW SECTION Source Type: research
White matter microstructure, cognition, and molecular markers in fragile X premutation females
(Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Tags: CORRECTIONS Source Type: research
Author response: Physician-assisted death in chronic neurologic diseases
We thank Dr. Bier for the correction that the law in Belgium allows only active euthanasia and not physician-assisted death (PAD). Our statement remains true that, in the Netherlands, where both activities are lawful, active euthanasia is more commonly practiced than PAD.1 (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Bernat, J. L., McQuillen, M. P. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research
Author response: High hypothetical interest in physician-assisted death in multiple sclerosis
We appreciate Dr. Masdeu's interest in our article,1 and Dr. Sethi's comments on our article and the related editorial by Drs. Bernat and McQuillen.2 (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Marrie, R. A., Salter, A., Tyry, T., Cutter, G. R., Fox, R. J. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research
Letter re: High hypothetical interest in physician-assisted death in multiple sclerosis and Physician-assisted death in chronic neurologic diseases
I read with interest the editorial by Drs. Bernat and McQuillen,1 accompanying the article by Marrie et al.2 I share some of the concerns highlighted in the editorial, but want to highlight the other side of the argument. (Source: Neurology)
Source: Neurology - September 25, 2017 Category: Neurology Authors: Sethi, N. K. Tags: WRITECLICK & amp;reg; EDITOR ' S CHOICE Source Type: research
