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cranial nervs syndromes

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https://www.readbyqxmd.com/read/28099131/turner-syndrome-and-pituitary-adenomas-a-case-report-and-review-of-literature
#1
Tiffany Yeh, Angela Ganan Soto, Jose Bernardo Quintos, Lisa Swartz Topor
BACKGROUND: Turner syndrome (TS) is the most common sex chromosome abnormality in females, typically associated with primary amenorrhea and premature ovarian failure due to gonadal dysgenesis. The association of TS with hypopituitarism is an uncommon finding. The objective of the study was to describe an adolescent with TS with hypergonadotropic hypogonadism and subsequent hypogonadotropic hypogonadism. CASE PRESENTATION: A 16-year-old female with primary amenorrhea was diagnosed with TS based on karyotype 45,XO...
January 18, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28097083/recurrent-squamous-cell-carcinoma-of-the-eyelid-presenting-as-trigeminal-neuralgia
#2
Nicole Spitzer, Naazli Shaikh, Leah Strickland, Son Ho
This paper describes two patients with squamous cell carcinoma (SCC) of the periocular and periorbital skin who presented with trigeminal neuralgia. Both patients had previous cutaneous SCC of the scalp treated successfully with surgical resection but later presented with neuro-ophthalmic findings suggesting perineural invasion (PNI) of SCC. PNI of SCC in the periocular skin or orbit can lead to devastating effects if malignant cells seed into the orbit and adjacent cranial nerves as our two patients developed an orbital apex syndrome...
December 16, 2016: Curēus
https://www.readbyqxmd.com/read/28064284/surgery-modified-pi-with-triple-bonnet-flap-and-fronto-orbital-advancement
#3
Uday Singh Raswan, Sarbjit Singh Chhiber, Altaf Umar Ramzan
INTRODUCTION: Craniosynostosis is the premature fusion of one or more of the cranial sutures and can occur as part of a syndrome or as an isolated defect. Pansynostosis is a rare form of craniosynostosis that involves premature fusion of all the cranial sutures (coronal, sagittal, metopic, and occipital). Particularly in cases of late presentation, there are heightened clinical concerns, both functional and aesthetic. In untreated cases of pansynostosis and increased intracranial pressure, optic nerve damage progresses to optic atrophy and then blindness...
January 7, 2017: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28062188/a-comprehensive-analysis-and-immunobiology-of-autoimmune-neurological-syndromes-during-the-zika-virus-outbreak-in-c%C3%A3%C2%BAcuta-colombia
#4
Juan-Manuel Anaya, Yhojan Rodríguez, Diana M Monsalve, Daniel Vega, Ernesto Ojeda, Diana González-Bravo, Mónica Rodríguez-Jiménez, Carlos A Pinto-Díaz, Pablo Chaparro, María L Gunturiz, Aftab A Ansari, M Eric Gershwin, Nicolás Molano-González, Carolina Ramírez-Santana, Yeny Acosta-Ampudia
We have focused on the epidemiology and immunobiology of Zika virus (ZIKV) infection and factors associated with the development of Guillain-Barré syndrome (GBS) and other neurological syndromes in Cúcuta, the capital of North Santander department, Colombia. Data of patients with ZIKV disease reported to the national population-based surveillance system were used to calculate the basic reproduction number (R0) and the attack rates (ARs) as well as to develop epidemiological maps. Patients with neurological syndromes were contacted and their diagnoses were confirmed...
January 3, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28032327/emerging-zika-virus-infection-a-rapidly-evolving-situation
#5
Bordi Licia, Avsic-Zupanc Tatjana, Lalle Eleonora, Vairo Francesco, Capobianchi Maria Rosaria, Pedro Fernando da Costa Vasconcelos
Zika virus is a mosquito-borne flavivirus, firstly identified in Uganda and responsible for sporadic human cases in Africa and Asia until recently, when large outbreak occurred in Pacific Ocean and the Americas. Since the main vectors during its spread outside of Africa have been Ae. albopictus and Ae. aegypti mosquitoes, which are widely distributed all over the world, there is urgent need for a coordinated response for prevention and spread of ZIKV epidemics.Despite clinical manifestation of Zika virus infection are usually mild and self limiting, there are reports suggesting, during the recent epidemic, an association of ZIKV infection with severe consequences, including fetal/newborn microcephaly, due to vertical in utero transmission, autoimmune-neurological presentations including cranial nerve dysfunction, and Guillain-Barré Syndrome in adults...
December 29, 2016: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28012056/pachymeningitis-in-granulomatosis-with-polyangiitis-case-series-with-earlier-onset-in-younger-patients-and-literature-review
#6
Violeta Higuera-Ortiz, Abraham Reynoso, Natllely Ruiz, Rosa Delia Delgado-Hernández, Gilberto Gómez-Garza, Luis Felipe Flores-Suárez
The objective of this study is to describe the characteristics of patients with pachymeningitis (PM) in granulomatosis with polyangiitis (GPA) from Latin America, including three young patients. This is a retrospective case series. Patients were classified according to the ACR criteria, the 2012 Chapel Hill Consensus Conference Nomenclature and the EMA algorithm. Demographic, clinical, serological, and neuroimaging characteristics are described. Thirteen patients (nine females, four males) were identified. Mean age ± SD of PM diagnosis was 35...
December 23, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/28009406/the-frequency-and-causes-of-abnormal-head-position-based-on-an-ophthalmology-clinic-s-findings-is-it-overlooked
#7
Kadriye Erkan Turan, Hande Taylan Sekeroglu, Irem Koc, Meltem Kilic, Ali S Sanac
PURPOSE: To determine the frequency of abnormal head position (AHP) and identify the underlying causes in patients who presented to an ophthalmology clinic due to any ophthalmologic complaint. METHODS: The medical records of patients who presented with any ophthalmologic symptoms during a 6-month period were prospectively evaluated. In all, 2,710 patients (1,492 female and 1,218 male) aged 6 months-91 years were included in the study. Each patient underwent complete ophthalmologic evaluation...
December 2, 2016: European Journal of Ophthalmology
https://www.readbyqxmd.com/read/28005197/sbf1-mutations-associated-with-autosomal-recessive-axonal-neuropathy-with-cranial-nerve-involvement
#8
Andreea Manole, Alejandro Horga, Josep Gamez, Nuria Raguer, Maria Salvado, Beatriz San Millán, Carmen Navarro, Alan Pittmann, Mary M Reilly, Henry Houlden
Biallelic mutations in the SBF1 gene have been identified in one family with demyelinating Charcot-Marie-Tooth disease (CMT4B3) and two families with axonal neuropathy and additional neurological and skeletal features. Here we describe novel sequence variants in SBF1 (c.1168C>G and c.2209_2210del) as the potential causative mutations in two siblings with severe axonal neuropathy, hearing loss, facial weakness and bulbar features. Pathogenicity of these variants is supported by co-segregation and in silico analyses and evolutionary conservation...
January 2017: Neurogenetics
https://www.readbyqxmd.com/read/27957617/neuromyotonia-as-an-unusual-neurological-complication-of-primary-sj%C3%A3-gren-s-syndrome-case-report-and-literature-review
#9
REVIEW
Fei Xiao
Primary Sjögren's syndrome (PSS) is a systemic autoimmune disorder characterized by chronic inflammation of exocrine glands such as the lachrymal and salivary glands, leading to xerophthalmia and xerostomia. Neurological manifestations are sometimes found in patients with PSS. A variety of neurological complications has been reported in patients with PSS, and both the central nervous system (CNS) and peripheral nervous system (PNS) can be involved in PSS. Several forms of neuropathy, including polyneuropathy, cranial neuropathy, and multiple mononeuropathy, are often seen in PSS patients...
December 12, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27956341/brainstem-and-cranial-nerve-disorders-of-ruminants
#10
REVIEW
Mélanie J Boileau, John Gilliam
Asymmetrical signs of brainstem disease occur relatively infrequently in ruminants. The most common differential diagnoses include listeriosis, otitis media/interna, and pituitary abscess syndrome. Although these conditions produce signs of brainstem dysfunction, the diseases can usually be differentiated based on historical findings and subtle clinical differences. Basic laboratory diagnostic tests are often not specific in the definitive diagnosis but may be supportive. Advanced imaging techniques have proven to be useful in the diagnosis of otitis media/interna...
December 9, 2016: Veterinary Clinics of North America. Food Animal Practice
https://www.readbyqxmd.com/read/27942479/posterior-fossa-syndrome-a-narrative-review
#11
REVIEW
Salima S Wahab, Samantha Hettige, Kshtij Mankad, Kristian Aquilina
Posterior fossa syndrome (PFS), or cerebellar mutism syndrome (CMS), is a collection of neurological symptoms that occur following surgical resection of a posterior fossa tumour, and is characterised by either a reduction or an absence of speech. Some authors suggest that CM is only one symptom of the CMS complex that also includes ataxia, hypotonia and irritability as well as cranial nerve deficits, neurobehavioral changes and urinary retention or incontinence. It is seen almost exclusively in children. In 1985 Rekate et al...
October 2016: Quantitative Imaging in Medicine and Surgery
https://www.readbyqxmd.com/read/27939798/diplopia-and-sjogren-s-disease-a-rare-case-report
#12
Kaushalendra Tripathi, Richa Tripathi, Navid Seraji-Bozorgzad
Sjogren's syndrome is a chronic autoimmune disorder which affects the exocrine glands with lymphocytic infiltration, and occasionally involves central nervous system. It is usually rare and manifests as a lesion in the trigeminal nerve. Our case discusses the involvement of the oculomotor and abducens nerves along with the prevalence of such cases as seen on literature review. We describe a case of a middle aged woman who presented with ophthalmoplegic symptoms. The symptoms resolved in response to steroid therapy and serum analysis was positive for anti SSA antibodies...
January 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/27929599/surgical-management-of-parapharyngeal-space-tumors-the-role-of-cervical-and-lateral-skull-base-approaches
#13
Arturo Mario Poletti, Siba P Dubey, Giovanni Colombo, Giovanni Cugini
We conducted a retrospective study to analyze the role of the cervical and lateral skull base approaches in the surgical excision of parapharyngeal space (PPS) tumors. Our study population was made up of 34 patients-15 males and 19 females, aged 13 to 73 years (mean: 50.6)-who had presented to us with a PPS tumor during a 9-year period. The 34 cases included 23 benign tumors and 11 malignancies. The 23 benign tumors consisted of 11 pleomorphic adenomas, 5 schwannomas, 2 paragangliomas, 2 Warthin tumors, 1 oncocytoma, 1 hamartoma, and 1 osteochondroma...
December 2016: Ear, Nose, & Throat Journal
https://www.readbyqxmd.com/read/27903844/therapeutic-approaches-to-pediatric-pseudotumor-cerebri-new-insights-from-literature-data
#14
Giovanna Vitaliti, Piero Pavone, Nassim Matin, Omidreza Tabatabaie, Salvatore Cocuzza, Michele Vecchio, Luigi Maiolino, Paola Di Mauro, Angelo Conti, Riccardo Lubrano, Agostino Serra, Raffaele Falsaperla
The pseudotumor cerebri syndrome (PTCS), also known as idiopathic intracranial hypertension (IIH), is characterized by signs and symptoms of increased intracranial pressure such as headache and cranial nerve palsies, in the absence of any space-occupying mass. This condition commonly affects overweight women of childbearing age, even if it is also frequent in men and children. Children with PTCS may present with atypical signs and symptoms, with a different prognosis compared to adults. However, the treatment is the same for both children and adults, even if there are no strict treatment guidelines in regards...
November 30, 2016: International Journal of Immunopathology and Pharmacology
https://www.readbyqxmd.com/read/27891417/a-commonly-missed-well-known-entity-acute-intermittent-porphyria-a-case-report
#15
Smilu Mohanlal, Radha Gulati Ghildiyal, Alpana Kondekar, Poonam Wade, Richa Sinha
Acute Intermittent Porphyria (AIP) usually presents with abdominal pain, peripheral neuropathy and psychiatric manifestations. Incidence of AIP being 5 in 1,00,000. We present a case of an 11-year-old male child with multiple cranial nerve involvement, quadriparesis, focal convulsions, hypertension, hyponatremia with history of recurrent abdominal pain. His complete haemogram, ultrasonography (USG) abdomen, renal function tests were normal, he was also evaluated for tuberculosis which was negative. On further evaluation Electroencephalography (EEG) was suggestive of a generalised seizure disorder, MRI Brain suggestive of Posterior Reversible Encephalopathy Syndrome (PRES), Electromyography revealed a sensory motor axonal polyneuropathy and urine UV fluoresence test was positive for porphobilinogen which clinched the diagnosis of AIP...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27890834/eagle-syndrome-presenting-after-blunt-trauma
#16
Ashley Mann, Scott Kujath, Mark L Friedell, Scott Hardouin, Chalmers Wood, Robert Carter, Karl Stark
As classically described, Eagle syndrome is an entity where patients develop pain or neurologic manifestations arising from an elongated styloid process and/or an ossified stylohyoid ligament irritating or compressing adjacent cranial nerves or the carotid arteries. Over the past few years, there have been reports of actual injury to the internal carotid artery with dissection, occlusion and strokes. We present three cases identified after blunt trauma-one due to carotid compression and two due to actual injury to the internal carotid artery...
November 24, 2016: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/27890181/imaging-of-vascular-compression-syndromes
#17
REVIEW
Joseph H Donahue, David A Ornan, Sugoto Mukherjee
Trigeminal neuralgia, hemifacial spasm, vestibulocochlear neuralgia and glossopharyngeal neuralgia represent the most common neurovascular compression syndromes. Repeated vascular pulsations at the vulnerable transitional zone of the individual cranial nerves lead to focal axonal injury and demyelination. High-resolution 3-D T2-weighted MR imaging is essential in detecting and mapping neurovascular compression for directed therapy. Knowledge of the specific nerve root exit, the transitional zones, and the adjacent vasculature is critical in proper management...
January 2017: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/27858390/eagle-syndrome-presented-with-neurological-symptoms
#18
Elçin Aydin, Hüseyin Quliyev, Celal Çinar, Halil Bozkaya, İsmail Oran
AIM: Eagle syndrome is a rare entity that causes recurrent throat pain, neck pain, dysphagia, or facial pain by elongated styloid process or calcified stylohyoid ligament. Clinical findings related to the lower cranial nerves compression have also been reported. In some cases it is reported that carotid artery compression or dissection can be seen due to elongated styloid process and it is called carotid artery syndrome. Carotid artery compression causes flow reduction and carotidynia or neurological symptoms can be seen...
October 14, 2016: Turkish Neurosurgery
https://www.readbyqxmd.com/read/27837354/congenital-cranial-dysinnervation-disorders
#19
REVIEW
Anupam Singh, P K Pandey, Ajai Agrawal, Sanjeev Kumar Mittal, Kartik Maheshbhai Rana, Chirag Bahuguna
The European Neuromuscular Centre (ENMC) derived the term Congenital Cranial Dysinnervation Disorders in 2002 at an international workshop for a group of congenital neuromuscular diseases. CCDDs are congenital, non-progressive ophthalmoplegia with restriction of globe movement in one or more fields of gaze. This group of sporadic and familial strabismus syndromes was initially referred to as the 'congenital fibrosis syndromes' because it was assumed that the primary pathologic process starts in the muscles of eye motility...
November 11, 2016: International Ophthalmology
https://www.readbyqxmd.com/read/27829262/-upper-airway-stimulation-in-osa
#20
B Bender
Anatomical basis of obstructive sleep apnea (OSA) is the collapse of the pharynx during sleep. It is considered as a result of complex interactions of structural and neuromuscular factors. Depth of sleep and body position have modulating effects. Although different areas of obstruction were identified, studies show that the sleep-mediated obstruction frequently occurs in the base of tongue area.Continuous positive airway pressure (CPAP) is the standard treatment for obstructive sleep apnea syndrome (OSAS) since more than 30 years...
November 2016: Laryngo- Rhino- Otologie
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