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Cranial Nerve 10

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9821. A novel form of aberrant innervation in congenital cranial dysinnervation disorder. (Abstract)

A novel form of aberrant innervation in congenital cranial dysinnervation disorder. Certain forms of congenital incomitant strabismus are associated with abnormal cranial nerve development and resultant abnormal orbital innervation (eg, Duane retraction syndrome, congenital fibrosis of the extraocular muscles [CFEOM]); such conditions can be considered congenital cranial dysinnervation disorders (CCDDs). In addition to duction limitation and/or ptosis, orbital CCDD phenotypes include (...) inappropriate extraocular muscle and/or levator innervation by nerves intended for innervation of other structures (eg, some of the innervation intended for the medial rectus muscle inappropriately innervating the ipsilateral lateral rectus muscle in Duane retraction syndrome). This report documents a unique orbital dysinnervational pattern-supraduction during attempted adduction and infraduction during attempted abduction in the left affected eye of a girl with exotropia and enophthalmos.

2008 JAAPOS - Journal of the American Association for Pediatric Ophthalmology and Strabismus

9822. Cystic olfactory schwannoma of the anterior cranial base. (Abstract)

olfactory nerve. It was removed completely and histology showed it to be a schwannoma. Olfactory groove schwannomas are rare lesions and should be differentiated from meningiomas, neuroblastomas and dural-based metastatic lesions of the anterior cranial base. (...) Cystic olfactory schwannoma of the anterior cranial base. Olfactory groove schwannomas are extremely uncommon and less than 30 cases are reported in the literature. We report a 21-year-old developmentally-retarded boy who experienced severe headache and aggressive behaviour for 5 months. Imaging showed a cystic mass in the subfrontal region, which was removed by craniotomy. The lesion had a vascular supply from the anterior ethmoidal arteries and it was noted to be attached to the right

2008 British Journal of Neurosurgery

9823. Congenital unilateral multiple cranial neuropathy: an etiology shared with Mobius syndrome? (Abstract)

Congenital unilateral multiple cranial neuropathy: an etiology shared with Mobius syndrome? Mobius syndrome was originally described as bilateral 6th and 7th cranial nerve palsy and has since been expanded to include involvement of nearly all cranial nerves, limb deformities, orofacial anomalies and deficits in intellectual function. The etiology of Mobius syndrome remains elusive. Here we present a case with unilateral 5th, 7th, 9th, 10th and 12th cranial nerve palsy along with MRI evidence

2006 International Journal of Pediatric Otorhinolaryngology

9824. Hypertrophic cranial pachymeningitis associated with middle ear inflammation. (Abstract)

imaging scans of the brain in all patients.Contrast-enhanced magnetic resonance imaging (MRI) of the brain, dural biopsy, and otomicroscopic examination.Thickening and abnormal enhancement of the dura mater on the MRI of the brain.All of the six patients had chronic inflammatory middle ear diseases. Three patients had otitis media with effusion, two had chronic otitis media, and one had postoperative otorrhea. The most common presentations of HCP were headache and cranial nerve palsies. Cranial Nerves (...) Hypertrophic cranial pachymeningitis associated with middle ear inflammation. To describe a series of patients with hypertrophic cranial pachymeningitis (HCP) associated with chronic middle ear inflammation.Retrospective case review.University Hospital.Six consecutive patients between the years 2000 and 2004 who had both middle ear disease and HCP (three men and three women; age range, 36-72 yr, mean age, 58.2 yr). The diagnosis of HCP was made by the use of contrast-enhanced magnetic resonance

2006 Otology and Neurotology

9825. New method of identifying the internal auditory canal as seen from the middle cranial fossa approach. Full Text available with Trip Pro

New method of identifying the internal auditory canal as seen from the middle cranial fossa approach. The middle cranial fossa approach allows one to remove acoustic tumors and preserve the facial nerve and hearing. However, there are no consistent landmarks on the surface of the temporal bone to identify the internal auditory canal. This study was designed to identify the internal auditory canal by use of external and internal references as seen during the middle cranial fossa approach.We (...) dissected 32 temporal bones using the middle cranial fossa approach and measured the distances from the posterior origin of the zygomatic arch to an imaginary coronal line between the foramen spinosum and the foramen ovale. We measured the angle between the lines drawn from the posterior origin of the zygomatic root to the foramen spinosum and from the foramen spinosum to the porus of the internal auditory canal.The distances were 14.7 mm and 22.9 mm, respectively, and the angle was roughly 90

2006 Rhinology and Laryngology

9826. Circumferential petrosectomy for petrous apicitis and cranial base osteomyelitis. (Abstract)

therapy (duration, 6-10 weeks) was administered after surgery, which resulted in the complete resolution of the disease and the associated symptoms in all five patients. No patient experienced hearing loss or facial nerve dysfunction as a result of the surgery within at least 1 year of follow-up in four of the five patients in this series.The circumferential petrosectomy is a potential treatment option when medical treatment fails in patients with petrositis or cranial base osteomyelitis. It permits (...) Circumferential petrosectomy for petrous apicitis and cranial base osteomyelitis. Petrous apicitis and cranial base osteomyelitis are life-threatening conditions. A surgical management may be necessary in cases that progress, in conditions that fail to improve with medical treatment, or in cases with impending complications. In this study, we describe a technique to remove the maximum amount of infected temporal bone while preserving the integrity of the peripheral auditory pathway and facial

2006 Otology and Neurotology

9827. Cranial ultrasound scanning and prediction of outcome in newborns with congenital cytomegalovirus infection. (Abstract)

Cranial ultrasound scanning and prediction of outcome in newborns with congenital cytomegalovirus infection. To report the accuracy of ultrasound scanning (US) in predicting neurodevelopmental and sensorineural outcome in patients with congenital cytomegalovirus (CMV) infection.Fifty-seven neonates with congenital CMV infection underwent brain US and were observed prospectively for motor skills, developmental quotient, and hearing function.Abnormal results on US were found in 12 of 57 neonates (...) . US lesions were more frequent in newborns with clinical and laboratory signs of congenital CMV infection at birth (10/18) than in newborns who had no symptoms at birth (2/39; P < .001). At least 1 sequela developed in all neonates with symptoms who had abnormal US results, whereas none of the neonates with symptoms who had normal US results had long-term sequelae (P < .001). In the population without symptoms, sensorineural hearing loss developed in 3 of 37 (8.1%) neonates with normal US results

2007 Journal of Pediatrics

9828. RAB23 mutations in Carpenter syndrome imply an unexpected role for hedgehog signaling in cranial-suture development and obesity. Full Text available with Trip Pro

RAB23 mutations in Carpenter syndrome imply an unexpected role for hedgehog signaling in cranial-suture development and obesity. Carpenter syndrome is a pleiotropic disorder with autosomal recessive inheritance, the cardinal features of which include craniosynostosis, polysyndactyly, obesity, and cardiac defects. Using homozygosity mapping, we found linkage to chromosome 6p12.1-q12 and, in 15 independent families, identified five different mutations (four truncating and one missense) in RAB23 (...) , which encodes a member of the RAB guanosine triphosphatase (GTPase) family of vesicle transport proteins and acts as a negative regulator of hedgehog (HH) signaling. In 10 patients, the disease was caused by homozygosity for the same nonsense mutation, L145X, that resides on a common haplotype, indicative of a founder effect in patients of northern European descent. Surprisingly, nonsense mutations of Rab23 in open brain mice cause recessive embryonic lethality with neural-tube defects, suggesting

2007 American Journal of Human Genetics

9829. Assessment of the cranial involvement pattern of giant cell arteritis with 3T magnetic resonance imaging. Full Text available with Trip Pro

thickness and lumen diameter of the major cranial arteries on both sides of the head. In all cases, MRI results were compared with findings of clinical examination and laboratory tests. In addition, temporal artery biopsy specimens from 10 patients were examined by histology.MRI sharply revealed all of the major superficial cranial arteries, allowing for an evaluation of their lumen and vessel wall. Nine of the 21 patients were diagnosed as having GCA according to the criteria of the American College (...) Assessment of the cranial involvement pattern of giant cell arteritis with 3T magnetic resonance imaging. To noninvasively determine the involvement pattern of the cranial arteries in giant cell arteritis (GCA), with high-resolution magnetic resonance imaging (MRI).The superficial cranial arteries of 21 patients with suspected GCA were examined using a 3T high-field MRI scanner. Postcontrast T1-weighted spin-echo images were acquired with submillimeter spatial resolution, to assess mural

2005 Arthritis and Rheumatism

9830. Penetrating orbital trauma by stiletto causing complex cranial neuropathies. Full Text available with Trip Pro

, and was treated with corticosteroids, however the globes escaped direct injury. Injuries to the IIIrd and VIth cranial nerves were also observed. Deep orbital injuries must be excluded in patients presenting with small eyelid wounds caused by sharp penetrating objects. (...) Penetrating orbital trauma by stiletto causing complex cranial neuropathies. Penetrating orbital injuries pose a serious threat to vision, ocular motility, and in some cases, life. Long, sharp stiletto objects may penetrate deeply, causing catastrophic damage to orbital structures, despite seemingly trivial entry wounds. The authors present two cases of penetrating orbital injuries by stiletto objects, both entering via small eyelid wounds. Traumatic optic neuropathy occurred in both cases

2006 Emergency Medicine Journal

9831. Epidemiology of blunt head injury victims undergoing ED cranial computed tomographic scanning. (Abstract)

of TBI, among patients selected for scanning, included the following: age below 10 years (relative risk [RR] = 1.44, 95% confidence interval [CI], 1.19-1.77); age above 65 years (RR = 1.59; 95% CI, 1.40-1.80), and male sex (RR = 1.27; 95% CI, 1.30-1.43).Among patients selected for cranial CT scanning after blunt head injury, men, patients younger than 10 years, and those older than 65 years have an increased likelihood of significant TBI. (...) Epidemiology of blunt head injury victims undergoing ED cranial computed tomographic scanning. We sought to describe the epidemiology of emergency department (ED) patients with blunt head injury undergoing cranial computed tomography (CT) scanning for the evaluation of possible traumatic brain injury (TBI).Prospective, multicenter, observational study of ED patients undergoing cranial CT after blunt head injury. Patient's date of birth, sex, and race/ethnicity were documented before CT scanning

2006 American Journal of Emergency Medicine

9832. Cranial irradiation and growth hormone neurosecretory dysfunction: a critical appraisal. Full Text available with Trip Pro

Cranial irradiation and growth hormone neurosecretory dysfunction: a critical appraisal. It has been suggested that radiation-induced GH neurosecretory dysfunction exists in children; however, the pathophysiology is poorly understood, and it is unknown if such a phenomenon exists in adult life.Twenty-four-hour spontaneous GH secretion was studied by 20-min sampling both in the fed state (n = 16; six women) and the last 24 h of 33-h fast (n = 10; three women) in adult cancer survivors of normal (...) GH status defined by two GH provocative tests, 13.1 +/- 1.6 (range, 3-28) yr after cranial irradiation (18-40 Gy) for nonpituitary brain tumors (n = 12) or leukemia (n = 4) in comparison with 30 (nine women) age- and body mass index-matched normal controls (fasting, 11 men and three women).Using previously published diagnostic thresholds, all patients had stimulated peak GH responses in the normal range to both the insulin tolerance test and the combined GHRH plus arginine stimulation test

2007 Journal of Clinical Endocrinology and Metabolism

9833. Erythema multiforme associated with phenytoin and cranial radiation therapy: a report of three patients and review of the literature. (Abstract)

Erythema multiforme associated with phenytoin and cranial radiation therapy: a report of three patients and review of the literature. Intracranial malignancies (primary and metastatic) are often complicated by seizure activity. Phenytoin (Dilantin) is typically employed as prophylactic anticonvulsant in this setting. Uncommonly, erythema multiforme (EM) can develop in such patients at the port site during or soon after cranial radiation and can rapidly progress to EM major. Herein, in addition (...) to a comprehensive literature review of this entity, three additional patients are presented. The acronym 'EMPACT' is suggested (E: erythema; M: multiforme; associated with P: phenytoin; A: and; C: cranial, radiation; T: therapy) to best describe this disorder.An extensive review of the English medical literature through the National Library of Medicine (PUBMED) was performed to identify patients who had received or continued to receive radiation therapy while on phenytoin. A total of 24 patients were identified

2004 International Journal of Dermatology

9834. Absence of adrenocorticotropin (ACTH) neurosecretory dysfunction but increased cortisol concentrations and production rates in ACTH-replete adult cancer survivors after cranial irradiation for nonpituitary brain tumors. Full Text available with Trip Pro

neurosecretory dysfunction that might explain the excessive fatigue suffered by some cancer survivors.Cortisol profiling at 20-min intervals over 24 h during the fed state was undertaken in 34 patients (10 females), aged 17-53.7 yr (median, 21.5 yr), 2-29 yr (median, 11.5 yr) after receiving conventional cranial irradiation for nonpituitary brain tumors or leukemia (n = 5) and in 33 age-, gender-, and body mass index-matched normal controls, of whom 23 patients and 17 controls were also profiled in the last (...) Absence of adrenocorticotropin (ACTH) neurosecretory dysfunction but increased cortisol concentrations and production rates in ACTH-replete adult cancer survivors after cranial irradiation for nonpituitary brain tumors. For the first time, physiological cortisol secretion has been studied in ACTH-replete adult cancer survivors to explore any discrepancy between stimulated (during insulin-induced hypoglycemia) and spontaneous cortisol secretion and, in particular, the possible existence of ACTH

2005 Journal of Clinical Endocrinology and Metabolism

9835. Orbital varices, cranial defects, and encephaloceles: an unrecognized association. (Abstract)

anomalies.All available orbital imaging for patients with orbital venous anomalies was examined. For those with defects of the cranial base, the radiologic characteristics were noted and the clinical details were reviewed.The presence and type of orbital roof or medial wall defects and associated nasal or other cranial anomalies.Imaging was adequate for review in 222 of 310 patients (72%), and anomalies of the neighboring cranium or cerebral structure were found in 10 of 222 patients (4.5%). In the group (...) with cranial anomalies, the proportion of men (7/10; 70%) did not significantly differ from that in the group with varices but without cranial anomalies (93/212; 44%; P = 0.19, Fisher exact test). Orbital varices were associated with 3 types of cranial anomaly: major midline encephaloceles (type I anomaly; 4 cases), large superomedial defects of the orbital wall (type II; 3 cases), or defects of the greater wing of the sphenoid (type III; 3 cases).Clinicians should be aware of the possibility

2004 Ophthalmology

9836. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or cranial autonomic features (SUNA)--a prospective clinical study of SUNCT and SUNA. Full Text available with Trip Pro

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or cranial autonomic features (SUNA)--a prospective clinical study of SUNCT and SUNA. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic features (SUNA) are described, although SUNA is rarely reported. The phenotype of SUNCT and SUNA was characterized from (...) a large series of patients (43 SUNCT, 9 SUNA). Three attack types were identified: stabs, groups of stabs and saw-tooth attacks. The mean duration of stabs was 58 s (1-600 s); stab groups, 396 s (10-1200 s); and saw-tooth, 1160 s (5-12 000s). The attack frequency was a mean of 59 attacks/day (2-600), and this depended largely on the type of attack. The pain was orbital, supraorbital or temporal in 38 (88%) SUNCT and 7 (78%) SUNA, and also occurred in the retro-orbital region, side, top, back of head

2006 Brain

9837. Geste antagonistes in idiopathic lower cranial dystonia. (Abstract)

Geste antagonistes in idiopathic lower cranial dystonia. Geste antagonistes, or sensory tricks, are well described in focal dystonia affecting the neck, hand, and face. Improvement in dystonic movements is typically maintained while the trick is performed, but disappears when the geste ends. We investigated the phenomenological features of geste antagoniste maneuvers in 19 patients with idiopathic lower cranial dystonia who were prospectively evaluated over a period of 6 years. Of the 19, 10 (...) were men, mean age of onset was 49.8 years, and the most commonly involved lower cranial area was the jaw (10 patients). In most patients, dystonia was task-specific. Taking advantage of the improvement with a sensory geste, we manufactured oral appliances that mimicked the geste in 8 patients, and 3 continue to use it.(c) 2007 Movement Disorder Society.

2007 Movement Disorders

9838. Comparison of long-term neurocognitive outcomes in young children with acute lymphoblastic leukemia treated with cranial radiation or high-dose or very high-dose intravenous methotrexate. (Abstract)

Comparison of long-term neurocognitive outcomes in young children with acute lymphoblastic leukemia treated with cranial radiation or high-dose or very high-dose intravenous methotrexate. Cranial radiation therapy (CRT) is associated with neurocognitive morbidity in survivors of childhood acute lymphoblastic leukemia (ALL). For most patients, CRT has been replaced with intensified systemic and intrathecal chemotherapy, often including methotrexate (MTX). The impact of chemotherapy-only (...) protocols on neurocognitive outcomes is unclear, and the importance of systemic MTX dose has not been established.Seventy nine of 120 eligible children diagnosed with high-risk ALL between the ages of 1.0 and 4.9 years participated in this retrospective cohort study. All patients were treated on a uniform chemotherapy protocol with one of three modalities of CNS prophylaxis, depending on their treatment era. In addition to intrathecal therapy, CNS-directed therapy consisted of CRT (18 Gy in 10 fractions

2006 Journal of Clinical Oncology

9839. Concurrent chemotherapy and reduced-dose cranial spinal irradiation followed by conformal posterior fossa tumor bed boost for average-risk medulloblastoma: efficacy and patterns of failure. (Abstract)

Concurrent chemotherapy and reduced-dose cranial spinal irradiation followed by conformal posterior fossa tumor bed boost for average-risk medulloblastoma: efficacy and patterns of failure. To review the efficacy and patterns of failure in average-risk medulloblastoma patients treated with concurrent chemotherapy and reduced-dose cranial spinal irradiation and a conformal tumor bed boost.Thirty-three patients with average risk (defined as < or =1.5 cm(2) of residual tumor after resection, age (...) >3 years, and no involvement of the cerebrospinal fluid or spine [M0]) medulloblastoma were diagnosed at our institution between January 1994 and December 2001. They were enrolled in an institutional pilot protocol consisting of concurrent chemotherapy (vincristine), reduced-dose cranial spinal irradiation (2340 cGy), a conformal primary tumor bed boost (3240 cGy), followed by eight cycles of chemotherapy (vincristine, cisplatin, and lomustine or cyclophosphamide). The median age at diagnosis

2004 Biology and Physics

9840. Pial arterial response to topical verapamil in acute closed cranial windows in rabbits. Full Text available with Trip Pro

the large proximal arteries. We tested our hypothesis in acute cranial windows implanted in 10 New Zealand White rabbits. After predrug measurements, we superfused 4 increasing concentrations of verapamil or nitroglycerin (10(-8), 10(-6), 10(-4), and 10(-3) M) in the cranial windows for 5 min each. The maximum increase in diameter was expressed as a percentage change from predrug diameters. There was a 30-min period of rest between the two drug challenges. Topical verapamil increased the arterial (...) Pial arterial response to topical verapamil in acute closed cranial windows in rabbits. We have previously observed that intraarterial verapamil increases cerebral blood flow, whereas nitric oxide donors, such as nitroglycerin, do not. Clinically, both verapamil and nitroglycerin dilate large cerebral arteries. Therefore, we hypothesized that topical verapamil would dilate both the large proximal and the small distal cerebral arteries, whereas nitroglycerin would preferentially dilate only

2005 Anesthesia and Analgesia

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