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Cranial Nerves (cranial + nerve)
Terms modified by Cranial Nerves Selected AbstractsDirect Infiltration of Brainstem Glioma Along the Cranial NervesJOURNAL OF NEUROIMAGING, Issue 2 2005Alexander Ree ABSTRACT The authors describe a case of a low-grade brainstem glioma extending along the cranial nerves without any evidence of leptomeningeal spread. The tumor extended directly along the VII-VIIIth cranial nerve complex and also along the trigeminal nerve, which is quite an unusual characteristic of the glial tumors. [source] Intraoperative cranial nerve monitoringMUSCLE AND NERVE, Issue 3 2004C. Michel Harper MD Abstract The purpose of intraoperative monitoring is to preserve function and prevent injury to the nervous system at a time when clinical examination is not possible. Cranial nerves are delicate structures and are susceptible to damage by mechanical trauma or ischemia during intracranial and extracranial surgery. A number of reliable electrodiagnostic techniques, including nerve conduction studies, electromyography, and the recording of evoked potentials have been adapted to the study of cranial nerve function during surgery. A growing body of evidence supports the utility of intraoperative monitoring of cranial nerve nerves during selected surgical procedures. Muscle Nerve 29: 339,351, 2004 [source] Neurological aspects of osteopetrosisNEUROPATHOLOGY & APPLIED NEUROBIOLOGY, Issue 2 2003C. G. Steward The osteopetroses are caused by reduced activity of osteoclasts which results in defective remodelling of bone and increased bone density. They range from a devastating neurometabolic disease, through severe malignant infantile osteopetrosis (OP) to two more benign conditions principally affecting adults [autosomal dominant OP (ADO I and II)]. In many patients the disease is caused by defects in either the proton pump [the a3 subunit of vacuolar-type H(+)-ATPase, encoded by the gene variously termed ATP6i or TCIRG1] or the ClC-7 chloride channel (ClCN7 gene). These pumps are responsible for acidifying the bone surface beneath the osteoclast. Although generally thought of as bone diseases, the most serious consequences of the osteopetroses are seen in the nervous system. Cranial nerves, blood vessels and the spinal cord are compressed by either gradual occlusion or lack of growth of skull foramina. Most patients with OP have some degree of optic atrophy and many children with severe forms of autosomal recessive OP are rendered blind; optic decompression is frequently attempted to prevent the latter. Auditory, facial and trigeminal nerves may also be affected, and hydrocephalus can develop. Stenosis of both arterial supply (internal carotid and vertebral arteries) and venous drainage may occur. The least understood form of the disease is neuronopathic OP [OP and infantile neuroaxonal dystrophy, MIM (Mendelian inheritance in man) 600329] which causes rapid neurodegeneration and death within the first year. Although characterized by the finding of widespread axonal spheroids and accumulation of ceroid lipofuscin, the biochemical basis of this disease remains unknown. The neurological complications of this disease and other variants are presented in the context of the latest classification of the disease. [source] Neurological complications in two children with Lemierre syndromeDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 8 2010BASHEER PEER MOHAMED Lemierre syndrome is a distinct clinical syndrome comprising oropharyngeal sepsis and fever, internal jugular vein thrombosis and remote septic metastases caused by Fusobacterium species. The mortality rate was historically high and although use of antibiotics led to a dramatic fall in incidence, a resurgence has been seen recently. A 14-year-old male developed Lemierre syndrome after tonsillitis. There was extensive leptomeningitis, especially over the clivus, causing 6th and 12th cranial nerve palsies, a clinical feature termed the ,clival syndrome'. He also developed an epidural abscess in the cervical spine, which was unsafe for surgical drainage. Conservative treatment with an extended course of antibiotics and anticoagulation for jugular vein thrombosis led to a good recovery. A 15-year-old female developed Lemierre syndrome after a persistent sore throat lasting 7 weeks. She had palsy of the 12th cranial nerve from clival osteomyelitis. She was treated with a 6-week course of antibiotics and anticoagulants leading to almost full recovery at 3-month review. Awareness of the potential neurological complications of Lemierre syndrome and prompt management are crucial in reducing morbidity and mortality in this ,forgotten disease'. [source] Generation of a transgenic mouse line expressing GFP-Cre protein from a Hoxb4 neural enhancerGENESIS: THE JOURNAL OF GENETICS AND DEVELOPMENT, Issue 2 2008Elena Rivkin Abstract Here, we describe a transgenic mouse line, in which expression of green fluorescent protein fused to Cre recombinase (GFP-Cre) is directed by the early neuronal enhancer (ENE) of Hoxb4. In E9.0,13.5 transgenic embryos, Cre activity coincided with endogenous Hoxb4 throughout the neural tube up to the r6/r7 boundary in the hindbrain, the dorsal root ganglia, and the Xth cranial ganglia. Unexpectedly, Cre activity was also consistently detected in the trigeminal (Vth) cranial nerve, which is devoid of endogenous Hoxb4 expression. Strong GFP dependent fluorescence appeared slightly later in E9.5,E11.5 embryos, and reflected the later expression pattern expected for Hoxb4-ENE directed expression in the neural tube up to the r7/r8 not r6/r7 boundary. Thus, with the exception of the trigeminal nerve, this reporter faithfully reproduces endogenous embryonic neural Hoxb4 expression, and provides an excellent reagent for in vivo gene manipulations in neuronal Hoxb4 positive cells as well as the developing trigeminal nerve. This transgenic mouse line should prove especially useful for determining the fate map of neuronal populations arising in rhombomeres 7 and 8 on its own and in combination with the small set of other existing rhombomere-specific Cre recombinase expressing lines. genesis 46:119,124, 2008. © 2008 Wiley-Liss, Inc. [source] Neurological signs and late-life depressive symptoms in a community population: the ESPRIT studyINTERNATIONAL JOURNAL OF GERIATRIC PSYCHIATRY, Issue 7 2010Mishael Soremekun Abstract Objective Depression in the elderly is common and often resistant to treatment. It has been suggested that late-life depression may be related to underlying neurobiological changes. However, these observations are derived from diverse clinical samples and as yet have not been confirmed in a more representative population study. Our aim was to investigate associations between neurological signs as markers of underlying brain dysfunction and caseness for depression in an elderly community sample, controlling for physical health and comorbid/past neurological disorders. Method A cross-sectional analysis of 2102 older people without dementia from the ESPRIT project. Depressive symptomatology was ascertained using the CES-D and abnormal neurological signs/comorbidity from a full neurological examination according to ICD-10 criteria. Results Pyramidal, extrapyramidal, cranial nerve and sensory deficit signs were significantly associated with case-level depressive symptoms. However, all odds ratios were close to null values in participants who did not have previous neurological disorder. Conclusions We confirmed previous findings of an association between neurological signs and case-level depressive symptoms in late life. However, this association may simply reflect the impact of more severe comorbid neurological disorder. Copyright © 2009 John Wiley & Sons, Ltd. [source] The terminal nerve and its relation with extrabulbar "olfactory" projections: Lessons from lampreys and lungfishesMICROSCOPY RESEARCH AND TECHNIQUE, Issue 1-2 2004Christopher S. Von BartheldArticle first published online: 29 NOV 200 Abstract The definition of the terminal nerve has led to considerable confusion and controversy. This review analyzes the current state of knowledge as well as diverging opinions about the existence, components, and definition of terminal nerves or their components, with emphasis on lampreys and lungfishes. I will argue that the historical terminology regarding this cranial nerve embraces a definition of a terminal nerve that is compatible with its existence in all vertebrate species. This review further summarizes classical and more recent anatomical, developmental, neurochemical, and molecular evidence suggesting that a multitude of terminalis cell types, not only those expressing gonadotropin-releasing hormone, migrate various distances into the forebrain. This results in numerous morphological and neurochemically distinct phenotypes of neurons, with a continuum spanning from olfactory receptor-like neurons in the olfactory epithelium to typical large ganglion cells that accompany the classical olfactory projections. These cell bodies may lose their peripheral connections with the olfactory epithelium, and their central projections or cell bodies may enter the forebrain at several locations. Since "olfactory" marker proteins can be expressed in bona fide nervus terminalis cells, so-called extrabulbar "olfactory" projections may be a collection of disguised nervus terminalis components. If we do not allow this pleiomorphic collection of nerves to be considered within a terminal nerve framework, then the only alternative is to invent a highly species- and stage-specific, and, ultimately, thoroughly confusing nomenclature for neurons and nerve fibers that associate with the olfactory nerve and forebrain. Microsc. Res. Tech. 65:13,24, 2004. © 2004 Wiley-Liss, Inc. [source] Trigeminal nerve repetitive stimulation in myasthenia gravisMUSCLE AND NERVE, Issue 4 2004Devon I. Rubin MD Abstract The aim of this study was to evaluate the utility of repetitive nerve stimulation (RNS) of the trigeminal nerve in assessing patients with myasthenia gravis (MG). In 26 normal controls and 21 patients with myasthenia gravis (MG), 2-Hz repetitive stimulation of the trigeminal nerve was performed using a monopolar needle for percutaneous nerve stimulation and recording over the surface of the masseter. In the MG patients, repetitive stimulation of the ulnar, spinal accessory, and facial nerves was also performed. The mean percent decrement in the compound muscle action potential (CMAP) amplitude among the different nerves at rest were: ulnar, 4.3%; spinal accessory, 10.1%; facial, 14%; and trigeminal, 17.3%. The facial nerve demonstrated abnormal decrement in 57% of all patients, compared with the spinal accessory (48%), trigeminal (43%), and ulnar (20%) nerves. All patients tolerated trigeminal RNS better than or as well as facial RNS. The study demonstrates that trigeminal RNS is a safe, reliable, efficient, and well-tolerated technique that provides another cranial nerve,muscle combination that can be used to supplement repetitive stimulation of other limb or cranial nerves in the evaluation of patients with bulbar or generalized MG. Muscle Nerve 29: 591,596, 2004 [source] Epidural haemorrhage of the cervical spinal cord: a post-mortem artefact?NEUROPATHOLOGY & APPLIED NEUROBIOLOGY, Issue 3 2005G. N. Rutty Spinal epidural haemorrhage is a rare entity that occurs uncommonly in adults and rarely in children. It has a typical clinical presentation, although to date, the cause for the majority of cases remains unknown. We present a series of cases where epidural haemorrhage was identified at post-mortem, principly to the cervical cord, in cases outside the age range usually reported for clinical epidural haemorrhage, and with no underlying pathology to account for the finding. We present a hypothesis for a post-mortem cause for this finding and consider that, in the absence of any other identifiable causation, then this is a post-mortem occurrence similar to that of the Prinsloo,Gordon artefact of the soft tissues of the neck. This finding must be interpreted with care so as not to make the mistaken diagnosis of a nonaccidental head injury based on its finding, especially in the absence of intracranial, cranial nerve, optic nerve or eye pathologies. [source] Diameter of the Cochlear Nerve in Endolymphatic Hydrops: Implications for the Etiology of Hearing Loss in Ménière's Disease,THE LARYNGOSCOPE, Issue 9 2005Cliff A. Megerian MD Abstract Objective/Hypothesis: Endolymphatic hydrops (ELH) is an important histopathological hallmark of Ménière's disease. Experimental data from human temporal bones as well as animal models of the disorder have generally failed to determine the mechanism by which ELH or related pathology causes hearing loss. Hair cell and spiral ganglion cell counts in both human and animal case studies have not, for the most part, shown severe enough deterioration to explain associated severe sensorineural hearing loss. However a limited number of detailed ultrastructural studies have demonstrated significant reductions in dendritic innervation densities, raising the possibility that neurotoxicity plays an important role in the pathology of Ménière's disease (MD) as well as experimental endolymphatic hydrops (ELH). This study tests the hypothesis that neurotoxicity is an important primary mediator of injury to the hydropic ear and is reflected in measurable deterioration of the cochlear nerve in the animal model of ELH. This study also explores the previously presented hypothesis that cochlear injury in ELH is mediated through the actions of nitric oxide (NO) by evaluating whether hearing loss or various measures of cochlear damage can be ameliorated by administration of an agent that limits excess production of NO. Study Design: Part one of the project involves the surgical induction of endolymphatic hydrops and correlation of long term hearing loss with histological parameters of ELH severity as well as cochlear nerve and eighth cranial nerve diameter measurements. In part two, aminoguanidine is administered orally to a separate set of hydropic animals in an attempt to limit cochlear injury presumably mediated by NO. Methods: Guinea pigs are subjected to surgical induction of unilateral endolymphatic hydrops after establishing baseline ABR thresholds at 2, 4, 8, 16, and 32 kHz. Threshold shifts are established prior to sacrifice at 4 to 6 months and temporal bones processed for light microscopy. Measurements of cochlear nerve and eighth cranial nerve maximal diameters as well as average maximal diameters are carried out and correlated to hearing loss and a semi-quantitative measure of hydrops severity. The identical experiments are carried out in animals treated with aminoguanidine, an inhibitor of inducible nitric oxide synthase. Results: The mean maximal diameter (n = 14) of the hydropic cochlear nerve was significantly reduced (432.14 ± 43.18 vs. 479.28 ± 49.22 microns, P = .0025) as compared to the control nerve. This was also seen in measures of the eighth cranial nerve (855.71 ± 108.82 vs. 929 ± 81.53 microns, P = 0.0003). Correlation studies failed to show correlation between hydrops severity and a cochlear nerve deterioration index (r = -0.0614, P = .8348). Similarly, hearing loss severity failed to correlate with cochlear nerve deterioration (r = 0.1300, P = .6577). There was a significant correlation between hearing loss and hydrops severity (r = 0.6148, P = .0193). Aminoguanidine treated animals (n = 5) also sustained nerve deterioration to the same degree as non-treated animals and there appeared to be no protective effect (at the dosage administered) against ELH related hearing loss, hydrops formation, or nerve deterioration. Conclusion: ELH results in significant deterioration of cochlear nerve and eighth cranial nerve maximal diameters in the guinea pig model. These findings are in accord with previous studies which detected ultrastructural evidence of dendritic damage and indicate that neural injury is of sufficient severity to result in light microscopic evidence of cochlear nerve and eighth cranial nerve deterioration. These data support the concept that the principle pathological insult in ELH is a form of neurotoxicity, especially in light of previous studies which indicate relative preservation of hair cells at similar points in time. The lack of correlation between the severity of hydrops and nerve deterioration suggests that nerve deterioration is independent of hydrops severity. [source] 3163: Post-traumatic oculomotor disordersACTA OPHTHALMOLOGICA, Issue 2010E EGGENBERGER Trauma is a common ocular motor pathophysiology with a predilection for younger patients. Although afferent visual dysfunction is well known following trauma, the efferent conditions are perhaps less familiar. Trauma has a predilection to produce diplopia, and this may result from muscle, cranial nerve, nuclear, internucelar or supranuclear means. Orbital trauma commonly affects the extraocular muscles with signs to include proptosis and orbital bony abnormalities. CN4 is the most common ocular motor palsy post-trauma, perhaps due to its long course around the midbrain; CN3 or 6 palsies are well know following trauma but often require more severe trauma than CN4 palsies. Supranuclear ocular motor dysfunction is common after trauma (e.g., skew deviation). Oscillopsia has been reported post-trauma, most commonly related to vestibular ocular reflex abnormalities or nystagmus. [source] The formation of the superior and jugular ganglia: Insights into the generation of sensory neurons by the neural crestDEVELOPMENTAL DYNAMICS, Issue 2 2010Hannah Thompson Abstract The superior and jugular ganglia (S/JG) are the proximal ganglia of the IXth and Xth cranial nerves and the sensory neurons of these ganglia are neural crest derived. However, it has been unclear the extent to which their differentiation resembles that of the Dorsal Root Ganglia (DRGs). In the DRGs, neural crest cells undergo neuronal differentiation just after the onset of migration and there is evidence suggesting that these cells are pre-specified towards a sensory fate. We have analysed sensory neuronal differentiation in the S/JG. We show, in keeping with previous studies, that neuronal differentiation initiates long after the cessation of neural crest migration. We also find no evidence for the existence of migratory neural crest cells pre-specified towards a sensory phenotype prior to ganglion formation. Rather our results suggest that sensory neuronal differentiation in the S/JG is the result of localised spatiotemporal cues. Developmental Dynamics 239:439,445, 2010. © 2009 Wiley-Liss, Inc. [source] Pre-/post-otic rhombomeric interactions control the emergence of a fetal-like respiratory rhythm in the mouse embryoDEVELOPMENTAL NEUROBIOLOGY, Issue 12 2006C. Borday Abstract How regional patterning of the neural tube in vertebrate embryos may influence the emergence and the function of neural networks remains elusive. We have begun to address this issue in the embryonic mouse hindbrain by studying rhythmogenic properties of different neural tube segments. We have isolated pre- and post-otic hindbrain segments and spinal segments of the mouse neural tube, when they form at embryonic day (E) 9, and grafted them into the same positions in stage-matched chick hosts. Three days after grafting, in vitro recordings of the activity in the cranial nerves exiting the grafts indicate that a high frequency (HF) rhythm (order: 10 bursts/min) is generated in post-otic segments while more anterior pre-otic and more posterior spinal territories generate a low frequency (LF) rhythm (order: 1 burst/min). Comparison with homo-specific grafting of corresponding chick segments points to conservation in mouse and chick of the link between the patterning of activities and the axial origin of the hindbrain segment. This HF rhythm is reminiscent of the respiratory rhythm known to appear at E15 in mice. We also report on pre-/post-otic interactions. The pre-otic rhombomere 5 prevents the emergence of the HF rhythm at E12. Although the nature of the interaction with r5 remains obscure, we propose that ontogeny of fetal-like respiratory circuits relies on: (i) a selective developmental program enforcing HF rhythm generation, already set at E9 in post-otic segments, and (ii) trans-segmental interactions with pre-otic territories that may control the time when this rhythm appears. © 2006 Wiley Periodicals, Inc. J Neurobiol, 2006 [source] Clinical anatomy of the equine sphenopalatine sinusEQUINE VETERINARY JOURNAL, Issue 6 2004J. L. McCANN Summary Reasons for performing study: Disorders of the equine sphenopalatine sinus, including empyema and neoplasia, have been reported to cause damage to cranial nerves II and V. However, the clinical anatomy of these sinuses is not well described in horses. Objective: To examine the anatomy of the sphenopalatine sinuses in a range of equidae and, in particular, to examine the relationship of these sinuses to adjacent major nerves and vessels. Methods: The anatomy of the sphenoidal and palatine paranasal sinuses was examined in 16 equidae, primarily using transverse skull sections. Relevant structures were documented and photographed. Results: There was much variation between individual horses in sphenopalatine sinus anatomy. The sphenoidal sinuses were small in young horses and appeared to become larger and more complex with age. Variation was present in the extent that the sphenopalatine sinus extended into the basisphenoid bone. The septum dividing left and right sphenoidal sinuses was frequently not midline, but was intact in all cases. The sphenoidal and palatine sinuses communicated in most horses. In such cases, what could accurately be termed the (combined) sphenopalatine sinuses usually drained directly into the caudal maxillary sinuses. Additionally, in 5 out of 16 cases, some compartments of the sphenoidal sinus also drained into the ethmoidal sinus. The dorsal and lateral walls of the sphenoidal sinus were very thin and directly adjacent to cranial nerves II, III, IV, V and VI and major blood vessels. Conclusions: The equine sphenoidal and palatine sinuses are very variable in their anatomy, but are always in close proximity to multiple cranial nerves and major blood vessels. Potential relevance: Many cranial nerves and blood vessels could be damaged with disorders involving the sphenopalatine sinus, potentially causing major and variable neurological syndromes, haemorrhage and extension of sepsis. [source] Palatal adhesion: The treatment of unilateral palatal paralysis after high vagus nerve injuryHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 8 2002James L. Netterville MD Abstract Background Resection of skull base tumors commonly necessitates intraoperative sacrifice of lower cranial nerves at the level of the jugular foramen. Sequelae of unilateral vagus nerve loss include ipsilateral laryngeal paralysis, ipsilateral palatal and pharyngeal paralysis, and velopharyngeal incompetence (VPI) marked by hypernasal speech and nasopharyngeal reflux of liquids during swallowing. Methods Palatal adhesion (PA), a procedure whereby the unilaterally paralyzed palate is attached to the posterior pharyngeal wall, decreases the size of the velopharyngeal port and minimizes the symptoms. This study assessed the outcome of PA in 31 patients with VPI secondary to proximal vagus nerve injury. Results PA decreased postoperative nasality in 96% of patients. Nasopharyngeal reflux was significantly improved in 83%. Three patients (11%) had minor wound breakdown postoperatively, all of which healed completely with conservative management. Conclusion PA offers a favorable result with minimal concomitant morbidity and is recommended for patients with VPI secondary to unilateral proximal vagus nerve paralysis. © 2002 Wiley Periodicals, Inc. Head Neck 24: 721,730, 2002 [source] Combined endovascular and surgical treatment of head and neck paragangliomas,A team approach,HEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 5 2002Mark S. Persky MD Abstract Background Paragangliomas are highly vascular tumors of neural crest origin that involve the walls of blood vessels or specific nerves within the head and neck. They may be multicentric, and they are rarely malignant. Surgery is the preferred treatment, and these tumors frequently extend to the skull base. There has been controversy concerning the role of preoperative angiography and embolization of these tumors and the benefits that these procedures offer in the evaluation and management of paragangliomas. Methods Forty-seven patients with 53 paragangliomas were treated from the period of 1990,2000. Initial evaluation usually included CT and/or MRI. All patients underwent bilateral carotid angiography, embolization of the tumor nidus, and cerebral angiography to define the patency of the circle of Willis. Carotid occlusion studies were performed with the patient under neuroleptic anesthesia when indicated. The tumors were excised within 48 hours of embolization. Results Carotid body tumors represented the most common paraganglioma, accounting for 28 tumors (53%). All patients underwent angiography and embolization with six patients (13%), demonstrating complications (three of these patients had embolized tumor involving the affected nerves). Cerebral angiography was performed in 28 patients, and 5 of these patients underwent and tolerated carotid occlusion studies. The range of mean blood loss according to tumor type was 450 to 517 mL. Postoperative cranial nerve dysfunction depended on the tumor type resected. Carotid body tumor surgery frequently required sympathetic chain resection (21%), with jugular and vagal paraganglioma removal frequently resulting in lower cranial nerve resection. These patients required various modes of postoperative rehabilitation, especially vocal cord medialization and swallowing therapy. Conclusions The combined endovascular and surgical treatment of paragangliomas is acceptably safe and effective for treating these highly vascular neoplasms. Adequate resection may often require sacrifice of one or more cranial nerves, and appropriate rehabilitation is important in the treatment regimen. © 2002 Wiley Periodicals, Inc. [source] First missense mutation in the SOST gene causing sclerosteosis by loss of sclerostin function,HUMAN MUTATION, Issue 7 2010Elke Piters Abstract Sclerosteosis is a rare bone dysplasia characterized by greatly increased bone mass, especially of the long bones and the skull. Patients are tall, show facial asymmetry and often have syndactyly. Clinical complications are due to entrapment of cranial nerves. The disease is thought to be due to loss-of-function mutations in the SOST gene. The SOST gene product, sclerostin, is secreted by osteocytes and transported to the bone surface where it inhibits osteoblastic bone formation by antagonizing Wnt signaling. In a small Turkish family with sclerosteosis, we identified a missense mutation (c.499T>C; p.Cys167Arg) in exon 2 of the SOST gene. This type of mutation has not been previously reported and using different functional approaches, we show that it has a devastating effect on the biological function of sclerostin. The affected cysteine is the last cysteine residue of the cystine-knot motif and loss of this residue leads to retention of the mutant protein in the ER, possibly as a consequence of impaired folding. Together with a significant reduced ability to bind to LRP5 and inhibit Wnt signaling, the p.Cys167Arg mutation leads to a complete loss of function of sclerostin and thus to the characteristic sclerosteosis phenotype. © 2010 Wiley-Liss, Inc. [source] Ventrally emigrating neural tube (VENT) cells: a second neural tube-derived cell populationJOURNAL OF ANATOMY, Issue 2 2004Douglas P. Dickinson Abstract Two embryological fates for cells of the neural tube are well established. Cells from the dorsal part of the developing neural tube emigrate and become neural crest cells, which in turn contribute to the development of the peripheral nervous system and a variety of non-neural structures. Other neural tube cells form the neurons and glial cells of the central nervous system (CNS). This has led to the neural crest being treated as the sole neural tube-derived emigrating cell population, with the remaining neural tube cells assumed to be restricted to forming the CNS. However, this restriction has not been tested fully. Our investigations of chick, quail and duck embryos utilizing a variety of different labelling techniques (DiI, LacZ, GFP and quail chimera) demonstrate the existence of a second neural tube-derived emigrating cell population. These cells originate from the ventral part of the cranial neural tube, emigrate at the exit/entry site of the cranial nerves, migrate in association with the nerves and populate their target tissues. On the basis of its site of origin and route of migration we have named this cell population the ventrally emigrating neural tube (VENT) cells. VENT cells also differ from neural crest cells in that they emigrate considerably after the emigration of neural crest cells, and lack expression of the neural crest cell antigen HNK-1. VENT cells are multipotent, differentiating into cell types belonging to all four basic tissues in the body: the nerve, muscle, connective and epithelium. Thus, the neural tube provides at least two cell populations , neural crest and VENT cells , that contribute to the development of the peripheral nervous system and various non-neural structures. This review describes the origin of the idea of VENT cells, and discusses evidence for their existence and subsequent fates. [source] Direct Infiltration of Brainstem Glioma Along the Cranial NervesJOURNAL OF NEUROIMAGING, Issue 2 2005Alexander Ree ABSTRACT The authors describe a case of a low-grade brainstem glioma extending along the cranial nerves without any evidence of leptomeningeal spread. The tumor extended directly along the VII-VIIIth cranial nerve complex and also along the trigeminal nerve, which is quite an unusual characteristic of the glial tumors. [source] Paediatric Tolosa-Hunt syndromeJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 7 2004MC Yeung Abstract: Tolosa-Hunt syndrome is characterized by a dull, persistent pain around the affected eye, ophthalmoplegia and, sometimes, involvement of other cranial nerves passing through the cavernous sinus. Corticosteroid administration is valuable in the treatment and frequently has a dramatic effect. We report a boy with Tolosa-Hunt syndrome who fails to respond to the initial steroid treatment. The role of the MRI in the management of this condition is discussed. [source] Polyneuritis cranialis with contrast enhancement of cranial nerves on magnetic resonance imagingJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 1 2003A Morosini Abstract: The disorder of multiple cranial nerve palsies without spinal cord involvement is referred to as polyneuritis cranialis (PC) and is rare. It is thought to be an acute post-infective polyneuropathy or a variant of Guillain,Barré syndrome. Electrophysiological evidence of demyelination has been reported, but no radiological abnormalities of the affected cranial nerves have been noted. We report a case of PC where contrast enhanced magnetic resonance imaging (MRI) showed enhancement of the peripheral segments of the oculomotor and abducens nerves. This case illustrates the utility of MRI in the assessment of cranial nerve palsies. [source] Infantile botulism: Clinical and laboratory observations of a rare neuroparalytic diseaseJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 2 2000E Urdaneta-Carruyo Abstract: A 3-month-old male infant was admitted to the University Hospital of Los Andes with a history of constipation, weak crying, poor feeding, flaccidity and later bilateral ptosis and hyporeflexia. The admission diagnosis was septicaemia until an electrophysiological study reported postetanic facilitation with 50 Hz/seg stimulations four days later. The Clostridium botulinum toxin type B was isolated from the infant's stool samples and the organism grew in anaerobic cultures. The patient recovered completely and was discharged 2 months later. Although infant botulism is an uncommon disease in our environment, this diagnosis must be suspected in all afebrile infants with constipation, affected cranial nerves and generalized hypotonia. The principal differential diagnoses are Landry-Guillain,Barré syndrome, poliomyelitis, myasthenia gravis and infant muscular atrophy. [source] Dejerine-Sottas Neuropathy with Multiple Nerve Roots Enlargement and Hypomyelination Associated with a Missense Mutation of the Transmembrane Domain of MPZ/P0JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 2 2003A Simonati In a patient affected with a slowly progressive, severe form of Dejerine-Sottas syndrome, symmetric enlargement of cranial nerves and focal hypertrophy of cervical and caudal roots were detected following MRI. Neuropathological features of the sural nerve disclosed a dramatic loss of myelinated fibres, with skewed-to-the-left, unimodal distribution of the few residual fibres, consistent with the diagnosis of congenital hypomyelination neuropathy. Genetic analysis revealed this condition to be associated with a heterozygous G to A transition at codon 167 in the exon 4 of the MPZ/P0 gene causing a Gly138Arg substitution in the transmembrane domain of the mature MPZ/P0 protein. Focal enlargement of the nerve trunks in demyelinating, hereditary motor and sensory neuropathies (HMSN) was previously reported in both asymptomatic and symptomatic cases with root compression, but peculiar to this case is the diffuse involvement of both cranial and spinal nerves. We believe that the relevance of nerve trunk hypertrophy in HMSN is probably underevaluated: therefore MRI investigation of the head and spine should be included in the diagnostic study of selected HMSN patients. Molecular analysis of peripheral myelin genes will help to rule out misdiagnosed cases. [source] Lymphoma and peripheral neuropathy: A clinical reviewMUSCLE AND NERVE, Issue 3 2005John J. Kelly MD Abstract Lymphoma occasionally affects the peripheral nervous system. When it does, the diagnosis can be elusive since many patients present without known lymphoma. Most peripheral nerve complications are due to non-Hodgkin's lymphoma (NHL), which infiltrates nerves causing axonal damage. This disorder can affect nerve roots and cranial nerves, often associated with lymphomatous meningitis. NHL may also infiltrate peripheral nerves and cause plexopathy, mononeuropathy, or generalized neuropathy. These neuropathies may resemble an asymmetric mononeuropathy multiplex or a generalized disorder such as chronic inflammatory demyelinating polyradiculoneuropathy. When NHL infiltrates diffusely, the term neurolymphomatosis is used. Hodgkin's lymphoma (HL), by contrast, rarely infiltrates nerves. More often, HL causes immunological disorders of the peripheral nervous system such as inflammatory plexopathy or Guillain,Barré syndrome. Other rare lymphomas such as intravascular lymphoma and Waldenström's macroglobulinemia can also affect peripheral nerves in specific ways. In addition, other malignant and nonmalignant lymphoproliferative disorders enter into the differential diagnosis of lymphomatous neuropathy. This review discusses the multiple peripheral nerve presentations of lymphoma from the clinician's point of view and provides a guide to the evaluation and diagnosis of these uncommon, challenging disorders. Muscle Nerve, 2005 [source] Trigeminal nerve repetitive stimulation in myasthenia gravisMUSCLE AND NERVE, Issue 4 2004Devon I. Rubin MD Abstract The aim of this study was to evaluate the utility of repetitive nerve stimulation (RNS) of the trigeminal nerve in assessing patients with myasthenia gravis (MG). In 26 normal controls and 21 patients with myasthenia gravis (MG), 2-Hz repetitive stimulation of the trigeminal nerve was performed using a monopolar needle for percutaneous nerve stimulation and recording over the surface of the masseter. In the MG patients, repetitive stimulation of the ulnar, spinal accessory, and facial nerves was also performed. The mean percent decrement in the compound muscle action potential (CMAP) amplitude among the different nerves at rest were: ulnar, 4.3%; spinal accessory, 10.1%; facial, 14%; and trigeminal, 17.3%. The facial nerve demonstrated abnormal decrement in 57% of all patients, compared with the spinal accessory (48%), trigeminal (43%), and ulnar (20%) nerves. All patients tolerated trigeminal RNS better than or as well as facial RNS. The study demonstrates that trigeminal RNS is a safe, reliable, efficient, and well-tolerated technique that provides another cranial nerve,muscle combination that can be used to supplement repetitive stimulation of other limb or cranial nerves in the evaluation of patients with bulbar or generalized MG. Muscle Nerve 29: 591,596, 2004 [source] Localization of the mRNA encoding prolyl endopeptidase in the rat brain and pituitaryTHE JOURNAL OF COMPARATIVE NEUROLOGY, Issue 2 2004Gaelle Bellemère Abstract Prolyl endopeptidase (EC 3.4.21.26, PEP), a serine protease that hydrolyzes peptides at the carboxyl side of proline residues, is involved in the breakdown of several proline-containing neuropeptides and, thus, may contribute to the regulation of behavioral activities. In this study, the distribution of PEP mRNA was investigated in the central nervous system and pituitary of rat by means of quantitative reverse transcriptase-polymerase chain reaction analysis and in situ hybridization histochemistry. High densities of PEP transcripts were found in cerebellar Purkinje and granule cells, within most hypothalamic nuclei, in pyramidal neurons of the Ammon's horn, in granule cells of the dentate gyrus, and within the basolateral complex of the amygdala. Moderate levels of PEP mRNA were observed in layers 3,5 of the cerebral cortex, the anterior thalamic group, the septal region, the substantia nigra, the magnocellular neurons of the red nucleus, and the motor nuclei of the cranial nerves. Low concentrations of PEP mRNA were detected in the deep mesencephalic nuclei, the reticular formation, the pretectum, and the tectum. A high density of PEP mRNA was found in the intermediate and the anterior lobes of the pituitary, while the neural lobe was devoid of labeling. In several brain regions, the distribution pattern of PEP mRNA overlapped that of various neuropeptide receptors, suggesting that PEP is actually involved in the inactivation of regulatory neuropeptides. J. Comp. Neurol. 471:128,143, 2004. © 2004 Wiley-Liss, Inc. [source] Surgical Management of Jugular Foramen Meningiomas: A Series of 13 Cases and Review of the Literature,THE LARYNGOSCOPE, Issue 10 2007Mario Sanna MD Abstract Objective: Primary meningiomas occurring within the jugular foramen are exceedingly rare lesions presumed to originate from arachnoid-lining cells situated within the jugular foramen. The objective of this study is to analyze the management and outcome in a series of 13 primary jugular foramen meningiomas collected at a single center. Study Design: Retrospective study. Setting: Quaternary referral otology and skull base private center. Methods: Charts belonging to 13 consecutive patients with pathologically confirmed jugular foramen meningioma surgically treated between September 1991 and May 2005 were examined retrospectively. The follow-up of the series ranged from 12 to 120 (mean, 42.8 ± 27.5) months. Results: Four (28.5%) patients underwent single-stage tumor removal through the petro-occipital transigmoid (POTS) approach. In two patients with preoperative unserviceable hearing, a combined POTS-translabyrinthine approach was adopted. Two patients underwent a combined POTS-transotic approach because of massive erosion of the carotid canal. A modified transcochlear approach type D with posterior rerouting of the facial nerve and transection of the sigmoid sinus and jugular bulb was performed in two patients with a huge cerebellopontine angle tumor component with extension to the prepontine cistern together with massive involvement of the petrous bone and middle ear and encasement of the vertical and horizontal segments of the intrapetrous carotid artery. In one patient with evidence of a dominant sinus on the site of the tumor, a subtotal tumor removal via an enlarged translabyrinthine approach (ETLA) was planned to resect the intradural component of the tumor. Two patients in our series underwent a planned staged procedure on account of a huge tumor component in the neck. One of these patients underwent a first-stage infratemporal fossa approach type A to remove the tumor component in the neck; the second-stage intradural removal of the tumor was accomplished via an ETLA. The last patient underwent a first-stage modified transcochlear type D approach to remove the intradural tumor component followed by a second-stage transcervical procedure for removal of the extracranial component. Gross total tumor removal (Simpson grade I,II) was achieved in 11 (84.6%) cases. Subtotal removal of the tumor was accomplished in two patients. Good facial nerve function (grades I and II) was achieved in 46.1% of cases, whereas acceptable function (grade III) was achieved in the remaining cases 1 year after tumor removal. Hearing was preserved at the preoperative level in all four patients who underwent surgery via the POTS approach. After surgery, no patient recovered function of the preoperatively paralyzed lower cranial nerves. A new deficit of one or more of the lower cranial nerves was recorded in 61.5% of cases. Conclusions: Surgical resection is the treatment of choice for jugular foramen meningiomas. Among the various surgical techniques proposed for dealing with these lesions, we prefer the POTS approach alone or combined with the translabyrinthine or transotic approaches. Despite the advances in skull base surgery, new postoperative lower cranial nerve deficits still represent a challenge. [source] Neuropathological Findings in 9 Cases of Listeria Monocytogenes Brain Stem EncephalitisBRAIN PATHOLOGY, Issue 3 2005Ellen-Ann Antal Brain stem encephalitis is a particular manifestation of infection with the bacterium Listeria monocytogenes. Here, we present the neuropathological findings in 9 such cases. In the brain stem, the inflammatory infiltrates were located predominantly within nuclei and tracts of cranial nerves innervating the oropharynx. These findings support the hypothesis that the food-borne bacterium Listeria monocytogenes invades the brain stem along cranial nerves. [source] | |||||||||||||||||||||||||