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https://www.readbyqxmd.com/read/28214652/unilateral-oculomotor-palsy-in-charcot-marie-tooth-disease-1a-cmt-1a
#1
A Posa, A Emmer, M E Kornhuber
BACKGROUND: Charcot-Marie-Tooth disease (CMT) type 1A is the most common form of CMT 1 and one of the autosomal dominant demyelinating hereditary motor and sensory neuropathies (HMSN). Cranial nerves may be frequently subclinically affected in CMT disease. However manifest clinical signs of cranial nerve involvement are rare. METHODS: This case comprise neurological, ophthalmological, internal medicine and ear-nose-throat investigation, motor and sensory nerve conduction velocity, auditory evoked potentials and orbicularis-oculi reflex measurements, lumbar puncture and blood examination, inclusive molecular genetic testing, as well as electrocardiogram and cranial imaging such as computer tomography and magnetic resonance imaging RESULTS: The present case shows a Charcot-Marie-Tooth (CMT) 1A patient with complete unilateral oculomotor palsy in combination with predominant ipsilateral subclinical trigeminal demyelination...
February 13, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28214063/multiple-cranial-nerve-neuropathies-ataxia-and-areflexia-miller-fisher-syndrome-in-a-child-and-review
#2
Antonio E Muñiz
No abstract text is available yet for this article.
July 25, 2016: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28212886/primary-ameloblastoma-of-the-temporal-bone
#3
Andro Košec, Jakov Ajduk, Mihael Ries, Robert Trotić
Ameloblastoma is a locally aggressive tumor derived from odontogenic epithelium. Although benign, its clinical behavior can often exhibit malignant characteristics. It is marked by slow and persistent growth with infiltration of adjacent tissues. Almost 70% occur in the mandible in patients older than 30 years. Recurrence of ameloblastoma from inadequate treatment is frequent. Because of its slow growth, recurrences can present decades after primary surgery. A primary ameloblastoma in an area outside the mandibular, maxillary, and infratemporal fossa regions has not been described in detail to date, with only 1 possible case mentioned in the literature...
January 24, 2017: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28211985/a-girl-with-developmental-delay-ataxia-cranial-nerve-palsies-severe-respiratory-problems-in-infancy-expanding-ndst1-syndrome
#4
Linlea Armstrong, Maja Tarailo-Graovac, Graham Sinclair, Kimberly I Seath, Wyeth W Wasserman, Colin J Ross, Clara D M van Karnebeek
NDST1 encodes an enzyme involved in the first steps in the synthesis of heparan sulfate chains, proteoglycans that are regulators found on the cell surface and in the extracellular matrix. Eight individuals homozygous for one of four family-specific missense mutations in the sulfotransferase domain of the enzyme have been described. They have intellectual disability. Some additionally had hypotonia, ataxia. seizures, and/or short stature, but none had history of respiratory problems. No humans with homozygous null mutations are known...
March 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28210908/updates-in-outcomes-of-stereotactic-radiation-therapy-in-acromegaly
#5
REVIEW
Monica Livia Gheorghiu
Purpose Treatment of acromegaly has undergone important progress in the last 20 years mainly due to the development of new medical options and advances in surgical techniques. Pituitary surgery is usually first-line therapy, and medical treatment is indicated for persistent disease, while radiation (RT) is often used as third-line therapy. The benefits of RT (tumor volume control and decreased hormonal secretion) are hampered by the long latency of the effect and the high risk of adverse effects. Stereotactic RT methods have been developed with the aim to provide more precise targeting of the tumor with better control of the radiation dose received by the adjacent brain structures...
February 16, 2017: Pituitary
https://www.readbyqxmd.com/read/28210639/an-interesting-case-of-tolosa-hunt-syndrome-in-a-young-male
#6
Ghulam Murtaza, Nicholas Konowitz, Hannah Lu, Anadil Faqah, Aneesh Kuruvilla
Tolosa-Hunt syndrome is a rare disease with a limited number of cases reported in the literature. It typically presents with orbital pain associated with palsy of the third, fourth, or sixth cranial nerve. We present an interesting case of Tolosa-Hunt syndrome in a young male who responded well to high-dose steroids and in a few days had significant improvement in his retro-orbital pain and ocular movements.
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28210514/hemorrhagic-colloid-cyst-presenting-with-acute-hydrocephaly
#7
Reza Akhavan, Behrouz Zandi, Masoud Pezeshki-Rad, Donya Farrokh, Bita Abbasi
Colloid cysts are benign slow-growing cystic lesions located on the roof of the third ventricle that usually present with symptoms related to gradual rise of intracranial pressure. They mostly remain asymptomatic and sometimes grow progressively and cause diverse symptoms associated with increased intracranial pressure such as headache, diplopia, and sixth cranial nerve palsy. Here we report a 47-year-old female who presented to the emergency department with acute severe headache and nausea/vomiting. On MRI examination acute hydrocephaly due to hemorrhagic colloid cyst was detected...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/28209311/mri-findings-of-optic-pathway-involvement-in-miller-fisher-syndrome-in-3-pediatric-patients-and-a-review-of-the-literature
#8
Ajay Malhotra, Mia Zhang, Xiao Wu, Shanu Jindal, David Durand, Naila Makhani
BACKGROUND: Miller Fisher syndrome (MFS) is a rare demyelinating condition which may have involvement of cranial nerves. There are a few case reports of optic pathway involvement in children. We describe 3 patients with optic pathway enhancement in pediatric patients with MFS. CASE SERIES: We retrospectively reviewed brain imaging findings in 17 pediatric patients with of Guillain-Barré syndrome (GBS) meeting Brighton criteria who had brain MRIs performed during their acute illness...
February 10, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28208975/solid-cystic-hypoglossal-nerve-schwannoma-with-fluid-fluid-level-a-rare-case-report
#9
Kulamani Sahoo, Pramod Ramchand Shaha, Rahul Khetawat, Mohd Abbas Ilyas, Gaurav Rajendra Khairnar
Schwannomas (neurinoma, neurilemmoma) are benign slow-growing encapsulated tumours originating from well-differentiated myelin-producing Schwann cells or nerve fiber sheet cells at the glial-Schwann cell junction. Hypoglossal nerve schwannoma mostly develops in the intracranial and extra-cranial segment or in both intracranial and extra-cranial segment forming a dumbbell shape tumour. The peripheral hypoglossal schwannomas are very rare. We present a case of right hypoglossal nerve schwannoma in a 46-year-old female who presented with headache and neck pain since 2 weeks with deviation of tongue to right side since 1 week...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28208895/extracalvarial-meningioma-in-the-parapharyngeal-space-presentation-diagnosis-and-management
#10
Sirshak Dutta, Neeraj Aggarwal, Mainak Dutta, Saurav Kumar Ghosh, Ramanuj Sinha
Extracalvarial meningioma in the cervical region presenting as a parapharyngeal mass lesion is seldom encountered in clinical practice and poses great challenge in its diagnosis and surgical management. In this report, we present a case of extracranial meningioma in a middle-aged person who presented with a large, gradually progressing cervical swelling with multiple cranial nerve pareses. The difficulties in diagnosis and surgical management of this unusual neoplasm in the setting of partial encasement and thrombosis of the internal jugular vein have been discussed, along with the computed tomography and magnetic resonance imaging providing details of its extent and character...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28203388/on-the-development-of-the-chondrocranium-and-the-histological-anatomy-of-the-head-in-perinatal-stages-of-marsupial-mammals
#11
REVIEW
Marcelo R Sánchez-Villagra, Analía M Forasiepi
An overview of the literature on the chondrocranium of marsupial mammals reveals a relative conservatism in shape and structures. We document the histological cranial anatomy of individuals representing Monodelphis domestica, Dromiciops gliroides, Perameles sp. and Macropus eugenii. The marsupial chondrocranium is generally characterized by the great breadth of the lamina basalis, absence of pila metoptica and large otic capsules. Its most anterior portion (cupula nasi anterior) is robust, and anterior to it there are well-developed tactile sensory structures, functionally important in the neonate...
2017: Zoological Letters
https://www.readbyqxmd.com/read/28202828/-diagnosis-of-cochleovestibular-neurovascular-compression-syndrome-a-scoring-system-based-on-five-clinical-characteristics
#12
Tomomi Okamura, Takafumi Nishizaki, Norio Ikeda, Shigeki Nakano, Takanori Sakakura, Natsumi Fujii, Takeshi Okuda
: The optimal method for diagnosing cochleovestibular neurovascular compression syndrome(CNVC)remains controversial, and the aim of this study is to develop a standard diagnostic instrument for the condition. The clinical features of 53 sides of 50 patients with a combination of vertigo, tinnitus, and/or hearing loss were retrospectively evaluated. The following five clinical features were evaluated and given a score of 1 or 0:1)a long history of recurrent vertigo, tinnitus, or hearing loss;2)neurological findings related to positional vertigo, nystagmus, tinnitus, or hearing loss;3)neuro-otological findings involving tinnitus, audiometry, or vestibular testing;4)auditory brainstem response(ABR)evaluation with the detection of a low 25-75% II wave amplitude on the contralateral side and delayed interpeak latency in the I-III or I-V waves(usually low II wave amplitude combined with double peaks and a wide-based form)during ABR evaluation using 80 and 90dB clicks;and 5)the detection of vascular contact with the eighth cranial nerve on magnetic resonance imaging-constructive interference in steady state or air computed tomography imaging...
February 2017: No Shinkei Geka. Neurological Surgery
https://www.readbyqxmd.com/read/28202826/-hypogeusia-and-high-signals-in-the-nuclei-of-the-solitary-tract-on-mri-due-to-varicella-zoster-virus-infection-a-case-report
#13
Yuji Shiga, Yuhei Kanaya, Shinichi Takeshima, Yutaka Shimoe, Akio Tanaka, Masaru Kuriyama
A 63-year-old man noticed hypogeusia after presenting hiccups for several days. He was serologically diagnosed with varicella-zoster virus (VZV) infection, but had no skin lesions typical of herpes (zoster sine herpete). Hypogeusia was confirmed by electrogustometry and the filter-paper disk method, which showed damage in the areas innervated by the cord of tympanum, glossopharyngeal nerve, and greater petrosal nerve. High signals in the nuclei of the solitary tract of the medulla oblongata and the enhancement of the bilateral intracranial segments of the cranial nerve IX and X complex were observed by magnetic resonance imaging (MRI)...
February 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/28198523/diversity-among-posterior-thalamoperforating-branches-originated-from-p1-segment-systematic-review
#14
Cezary Grochowski, Ryszard Maciejewski
BACKGROUND: The P1 segment of the posterior cerebral artery (PCA) begins at the termination of the basilar artery and ends at the origin of posterior communicating artery (PCoM), within the interpeduncular cistern. Perforating branches arising from this segment are called posterior thalamoperforating arteries and the main and biggest artery among those is called thalamoperforating artery (TPA). Perforating branches are a crucial component of cerebrovascular system supplying the posterior part of the thalamus, subthalamus, hypothalamus, substantia nigra, perforated substance, posterior part of internal capsule and the nucleus of III and IV cranial nerve...
February 15, 2017: Folia Morphologica (Warsz)
https://www.readbyqxmd.com/read/28197624/-nerve-distribution-and-density-in-the-canine-hip-joint-capsule-comparison-of-healthy-and-dysplastic-hip-joints
#15
Felix Giebels, Andreas Prescher, Stefan Wagenpfeil, Arno Bücker, Sylvia Kinzel
OBJECTIVE: The hip-joint capsule is exposed to increased tension forces during canine hip dysplasia, resulting in inflammation of the capsular tissue. It has been postulated that inflammation is associated with an increased nerve-distribution density. Therefore, it could be supposed that the nerve-distribution density in the hip-joint capsule is higher in dogs with dysplastic hip compared to healthy dogs. MATERIAL AND METHODS: In 16 Labrador Retriever dogs that had been euthanised due to unrelated reasons, the hip joints were classified as normoplastic (group 1, n = 18) or dysplastic (group 2, n = 14) based on radiography...
February 15, 2017: Tierärztliche Praxis. Ausgabe K, Kleintiere/Heimtiere
https://www.readbyqxmd.com/read/28194509/fetal-facial-nerve-course-in-the-ear-region-revisited
#16
Zhe Wu Jin, Kwang Ho Cho, Hiroshi Abe, Yukio Katori, Gen Murakami, Jose Francisco Rodríguez-Vázquez
PURPOSE: The aim of this study was to re-examine the structures that determine course of the facial nerve (FN) in the fetal ear region. MATERIALS AND METHODS: We used sagittal or horizontal sections of 28 human fetuses at 7-8, 12-16, and 25-37 weeks. RESULTS: The FN and the chorda tympani nerve ran almost parallel until 7 weeks. The greater petrosal nerve (GPN) ran vertical to the distal FN course due to the trigeminal nerve ganglion being medial to the geniculate ganglion at 7 weeks...
February 14, 2017: Surgical and Radiologic Anatomy: SRA
https://www.readbyqxmd.com/read/28194134/chronic-trigemino-cardiac-reflex-an-underestimated-truth
#17
REVIEW
Tumul Chowdhury, Bernhard Schaller
The trigemino-cardiac reflex (TCR) is a brainstem reflex that manifests as adverse cardiorespiratory events upon the stimulation of sensory branches of the fifth cranial nerve. This reflex is mainly investigated in different neurosurgical procedures and intervention. This reflex is commonly considered as an acute and mild physiological response. On the other hand, more devastating and chronic nature of this reflex is largely underreported and unknown. Therefore, this article aims to provide the comprehensive understanding of the chronic form of TCR, its manifestations, and management by literature search...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28193760/teaching-neuroimages-cranial-nerve-hypertrophy-in-igg4-anti-neurofascin-155-antibody-positive-polyneuropathy
#18
Jérôme Franques, Frédérique Chapon, Jérôme Devaux, Stéphane Mathis
No abstract text is available yet for this article.
February 14, 2017: Neurology
https://www.readbyqxmd.com/read/28193110/pontine-tegmental-cap-dysplasia-in-an-extremely-preterm-infant-and-review-of-the-literature
#19
Sylvie Picker-Minh, Sebastian Hartenstein, Hans Proquitté, Sebastian Fröhler, Vera Raile, Nadine Kraemer, Sarah Apeshiotis, Michael Leipoldt, Karim D Kalache, Deborah Morris-Rosendahl, Eugen Boltshauser, Wei Chen, Angela M Kaindl
Pontine tegmental cap dysplasia is a rare hindbrain malformation syndrome with a hypoplastic pons, a tissue protrusion into the fourth ventricle, and cranial nerve dysfunction. We here report clinical, imaging, and genetic findings of the first extremely low-birth-weight preterm infant with pontine tegmental cap dysplasia born at 25 weeks of gestation and provide an overview of 29 sporadic cases. A prenatally diagnosed hypoplastic and rostrally shifted cerebellum was indicative of a hindbrain defect and later identified as an early sign of pontine tegmental cap dysplasia in our patient...
March 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28192045/radio-transmitter-implantation-and-movement-in-the-wild-timber-rattlesnake-crotalus-horridus
#20
Vanessa Hale, Brian MacGowan, Lorraine Corriveau, David Huse, Andrea F T Currylow, Steve Thompson
Radiotelemetry transmitters have become critical to studies of wildlife ecology. However, little is known about how transmitter implantation surgery affects the mobility of some species, including the timber rattlesnake, Crotalus horridus . Tracking snake movement can provide insights into the effects of transmitter implantation. During 2007-2011, 71 radio transmitters were surgically implanted intracoelomically in 47 timber rattlesnakes. Over 20 of these snakes underwent surgery at least twice in 5 yr to replace old transmitters...
February 13, 2017: Journal of Wildlife Diseases
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