These are representative sample records from Science.gov related to your search topic.
For comprehensive and current results, perform a real-time search at Science.gov.
1

Bilateral agenesis of arcuate fasciculus demonstrated by fiber tractography in congenital bilateral perisylvian syndrome.  

PubMed

Congenital bilateral perisylvian syndrome (CBPS) is a type of cortical developmental abnormality associated with distinctive clinical and imaging features. Clinical spectrum of this syndrome is quite heterogeneous, with different degrees of neurological impairment in affected individuals. High-definition magnetic resonance imaging (MRI) has a great importance in revealing the presence of CBPS, but is limited in elucidating the heterogeneous clinical spectrum. The arcuate fasciculus (AF) is a prominent language tract in the perisylvian region interconnecting Broca and Wernicke areas, and has a high probability of being affected developmentally in CBPS. Herein, we report a case of CBPS with investigation of AF using diffusion tensor imaging (DTI) and fiber tractography in relation to clinical findings. We postulated that proven absence of AF on DTI and fiber tractography would correlate with a severe phenotype of CBPS. PMID:24852949

Kilinc, Ozden; Ekinci, Gazanfer; Demirkol, Ezgi; Agan, Kadriye

2015-03-01

2

Congenital bilateral perisylvian syndrome (CBPS): do concomitant esophageal malformations indicate a poor prognosis?  

PubMed

Congenital bilateral perisylvian syndrome (CBPS) is a syndrome of cortical malformation characterized by faciopharyngoglossomasticatory diplegia. We report on two cases of CBPS with associated esophageal malformations and a poor mental and motor development. The association of CBPS and esophageal malformations may indicate a subgroup of patients with a very early prenatal injury, characterised by a bad prognosis due to severe cortical disorganization. However, it can not be excluded that the association of CBPS and esophageal malformation is purely coincidental. PMID:11508551

Küker, W; Friese, S; Riethmüller, J; Krägeloh-Mann, I

2000-12-01

3

Bilateral perisylvian infarct: a rare cause and a rare occurrence.  

PubMed

Foix-Chavany-Marie opercular syndrome is a severe form of pseudobulbar palsy occurring due to bilateral anterior opercular lesions. We report a case of a 51-year-old man with sudden onset of inability to speak and dysphagia, and a history of synovial sarcoma of the right hand. Detailed language evaluation was normal. The patient had right upper motor neuron facial paresis and absent gag reflex bilaterally. Magnetic resonance (MR) imaging revealed acute and subacute infarcts involving the bilateral insular cortex. Two-dimensional echocardiography and cardiac MR imaging showed a mobile mass in the left atrium attached to the interatrial septum, which was likely a myxoma. Chest radiograph and computed tomography imaging of the chest revealed multiple cannonball shadows that were suggestive of secondaries in the lung. The probable cause of the cerebral lesions was the mass lesion in the heart or metastatic lesions from the synovial sarcoma. The cardiac surgeon and surgical oncologist recommended palliative care. PMID:21552775

Singh, A; Kate, M P; Nair, M D; Kesavadas, C; Kapilamoorthy, T R

2011-04-01

4

Ischemic Bilateral Opercular Syndrome  

PubMed Central

Opercular syndrome, also known as Foix-Chavany-Marie syndrome, is a paralysis of the facial, pharyngeal, masticatory, tongue, laryngeal, and brachial muscles. It is a rare cortical form of pseudobulbar palsies caused by vascular insults to bilateral operculum. Its clinical presentations include anarthria, weakness of voluntary muscles involving face, tongue, pharynx, larynx, and masticatory muscles. However, autonomic reflexes and emotional activities of these structures are preserved. In the present case, an 81-year-old male presented with acute onset of anarthria with difficulties in chewing, speaking, and swallowing that was diagnosed with opercular syndrome. PMID:23476665

Milanlioglu, Aysel; Ayd?n, Mehmet Nuri; Gökgül, Alper; Hamamc?, Mehmet; Erkuzu, Mehmet Atilla; Tombul, Temel

2013-01-01

5

Acral osteolysis in bilateral carpal tunnel syndrome  

PubMed Central

Carpal tunnel syndrome is a common neurological condition with rare yet potentially serious cutaneous and skeletal complications. We present a case of mutilating/ulcerating bilateral carpal tunnel syndrome in a 63 year old female. Radiographs showed symmetrical acral osteolysis in the index and middle fingers distal phalanges bilaterally. Carpal tunnel decompressions provided symptomatic relief. PMID:22470585

Khan, Sameer K; Saeed, Iram

2008-01-01

6

[Bilateral acute depigmentation of the iris syndrome].  

PubMed

Bilateral acute depigmentation of the iris syndrome (BADI syndrome) is a new clinical entity. Young females from 20 to 45 years of age are most commonly affected. It is characterized by bilateral nontransilluminating depigmentation of the iris stroma. During the acute phase, this clinical entity also combines with red painful eye, pigmentation of the trabecular meshwork, anterior chamber flare, circulating pigment, and pigmented deposit on the endothelium cornea. At the acute stage, the symptoms are controlled with topical corticosteroid treatment. The prognosis is good. We report a 41-year-old woman presenting with BADI syndrome. PMID:21531477

Portmann, A; Gueudry, J; Siahmed, K; Muraine, M

2011-05-01

7

Atypical Cogan's syndrome presenting as bilateral endogenous endophthalmitis.  

PubMed

We report a case of atypical Cogan's syndrome presenting as bilateral endogenous endophthalmitis in a woman with ovarian cancer. A 62-year-old woman with ovarian cancer developed bilateral interstitial keratitis and panuveitis accompanied by bilateral sensorineural hearing loss and chondritis. Auricular cartilage biopsy ruled out relapsing polychondritis and the diagnosis of atypical Cogan's syndrome was set clinically. PMID:22908938

Georgakopoulos, Constantine D; Makri, Olga E; Exarchou, Artemis M; Pharmakakis, Nikolaos

2014-01-01

8

The perisylvian epileptic network. A unifying concept.  

PubMed

In this work the authors provide evidences for a unifying concept of the syndromes of benign focal childhood epilepsies, Landau-Kleffner syndrome, and electrical status epilepticus in sleep treating them as a spectrum of disorders with a common transient, age dependent, non lesional, genetically based epileptogenic abnormality, the nature of which is still not known. The electro-clinical features of these syndromes are congruent with the different degree involvement of the perisylvian cognitive network and with the involvement of the thalamo-cortical associative system of variable degree. These epilepsies are characterized by the abundance of regional epileptiform discharges in sharp contrast with the rare and in several cases lacking seizures. The nature and severity of interictal cognitive symptoms are closely related to localization within the network and amount of epileptic interictal discharges. Spike-wave discharges are attributed to an alternation of overexcitation (spikes) and overinhibition (waves). The recurrent overinhibition represented by the wave of the discharges may interfere with the continuous depolarization of the cells of a large population of neurons, which is a requirement of the overt seizures. The overinhibition also interfere with cognitive processes which are correlated with the continuous presence of the fast (gamma) activity, binding the required cortical areas. Hence the recurrent inhibition works against the existence of the binding fast frequency activity. This is the assumed reason for the co-existence of the relative lack of overt seizures and in the same time for the frequently observed epileptogenic cognitive deficit symptoms ("cognitive epilepsies"). The time course of these syndromes overlaps with important developmental milestones. The frequent epileptic discharges alters the evolution of the perisylvian network developing late after early childhood and is very vulnerable for any interference in this imprinting time for speech and other cognitive functions. This spectrum of disorders represents a type of age linked, mild to severe 'epileptic encephalopathy' limited to the perisylvian network, where the cognitive impairment is caused by epileptic discharges interfering with cognitive development. PMID:15884395

Halász, Péter; Kelemen, Anna; Clemens, Béla; Saracz, Judit; Rosdy, Beáta; Rásonyi, György; Szücs, Anna

2005-01-20

9

Anton's Syndrome due to Bilateral Ischemic Occipital Lobe Strokes  

PubMed Central

We present a case of a patient with Anton's syndrome (i.e., visual anosognosia with confabulations), who developed bilateral occipital lobe infarct. Bilateral occipital brain damage results in blindness, and patients start to confabulate to fill in the missing sensory input. In addition, the patient occasionally becomes agitated and talks to himself, which indicates that, besides Anton's syndrome, he might have had Charles Bonnet syndrome, characterized by both visual loss and hallucinations. Anton syndrome, is not so frequent condition and is most commonly caused by ischemic stroke. In this particular case, the patient had successive bilateral occipital ischemia as a result of massive stenoses of head and neck arteries. PMID:25530893

Zuki?, Sanela; Sinanovi?, Osman; Hodži?, Renata; Mujagi?, Svjetlana; Smajlovi?, Edina

2014-01-01

10

Bilateral Horner's syndrome and hoarseness complicating lumbar epidural anesthesia.  

PubMed

A case of bilateral Horner's syndrome associated with hoarseness resulting from single-dose lumbar epidural analgesia for relief of low back pain in a 57-year-old man is reported. The pathophysiology of Horner's syndrome and hoarseness is discussed. PMID:2535019

Tabatabia, M; Mazloomdoost, M; Kirimli, B

1989-01-01

11

Bilateral adrenal haemorrhagic infarction in a patient with antiphospholipid syndrome.  

PubMed

A 68-year-old woman with antiphospholipid syndrome presented with a 3-day history of bilateral loin pain, vomiting, fever and confusion. On examination she was febrile, hypotensive and tachycardic. Investigations revealed raised inflammatory markers, renal impairment and hyponatraemia. Abdominal ultrasound revealed two well-defined heterogeneous areas bilaterally in the region of the adrenal glands. This prompted serum cortisol measurement and a CT of the abdomen. Cortisol was low in the context of sepsis at 48?nmol/L, and CT confirmed bilateral heterogeneous adrenal pathology. The patient was managed for septic shock and adrenal insufficiency. She was recognised to have several risk factors for haemorrhagic infarction of the adrenals: antiphospholipid syndrome, sepsis, postoperative state and anticoagulant therapy. She was discharged well on glucocorticoid and mineralocorticoid therapy and a repeat CT at 4?weeks confirmed the diagnosis of bilateral adrenal infarct and haemorrhage. PMID:25410037

Godfrey, Rebecca Louise; Clark, James; Field, Benjamin

2014-01-01

12

Perisylvian language networks of the human brain  

Microsoft Academic Search

Early anatomically based models of language consisted of an arcuate tract connecting Broca's speech and Wernicke's comprehension centers; a lesion of the tract resulted in conduction aphasia. However, the heterogeneous clinical pre- sentations of conduction aphasia suggest a greater complexity of perisylvian anatomical connections than allowed for in the classical anatomical model. This article re-explores perisylvian language connectivity using in

Marco Catani; Derek K. Jones; Dominic H. ffytche

2005-01-01

13

Bilateral Horner's syndrome in cluster type headaches.  

PubMed

A patient with cluster type headaches demonstrated bilateral and alternating ocular sympathetic dysfunction during a spontaneous as well as a nitroglycerin-induced attack. Biochemical evaluation revealed postganglionic pupillary dysfunction on the symptomatic side and preganglionic pupillary dysfunction contralaterally. These findings defy a simple explanation regarding a central or peripheral origin of the oculocephalic sympathetic dysfunction. PMID:8262788

Khurana, R K

1993-09-01

14

Buschke-Ollendorff syndrome and bilateral cutaneous syndactyly.  

PubMed

A 3-year-old boy presented with asymptomatic elastomas on the posterior trunk. Radiographic studies revealed osteopoikilosis, confirming the diagnosis of Buschke-Ollendorff syndrome. The patient had a history of bilateral simple cutaneous syndactyly, which has not been previously reported with this condition. Buschke-Ollendorff syndrome is a rare autosomal-dominant disorder characterized by connective tissue nevi and osteopoikilosis. Several associated systemic abnormalities have been reported, but morbidity and mortality are generally not affected. PMID:22150319

Fernández-Faith, Esteban; Kress, Douglas; Piliang, Melissa; Sachdeva, Mandi; Vidimos, Allison

2012-01-01

15

Bilateral carpal tunnel syndrome associated with interleukin 2 therapy.  

PubMed Central

We report the development of synchronous bilateral carpal tunnel syndrome in a woman with metastatic colorectal cancer, undergoing treatment with recombinant interleukin 2. A carpal tunnel decompression was carried out on the hand which was more severely affected, with a gradual recovery in median nerve function. To the best of our knowledge, this is the first reported case of carpal tunnel syndrome in association with recombinant interleukin 2. PMID:1437960

Heys, S. D.; Mills, K. L.; Eremin, O.

1992-01-01

16

Bow hunter's syndrome secondary to bilateral dynamic vertebral artery compression.  

PubMed

Bow hunter's syndrome is a condition in which vertebrobasilar insufficiency is resultant from head rotation, clinically manifested by presyncopal sensation, syncope, dizziness, and nausea. It is usually diagnosed clinically, with supporting vascular imaging demonstrating an occluded or at the very least compromised unilateral vertebral artery, while the dominant vertebral artery remains patent in the neutral position. Dynamic imaging is utilized to confirm the rotational compression of the dominant vertebral artery. We present the rare case of a patient with typical Bow hunter's symptoms, bilaterally patent vertebral arteries on neutral imaging, and bilateral compromise with head rotation. Our patient underwent posterior decompression of the culprit atlanto-axial transverse foramen and subaxial cervical fusion, with resolution of his symptoms. Our patient exemplifies the possibility of bilateral dynamic vertebral artery occlusion. We show that Bow hunter's syndrome cannot be ruled out in the setting of bilaterally patent vertebral arteries on neutral imaging and that severe cervical spondylosis should impart further clinical suspicion of this unusual phenomenon. PMID:25070633

Healy, Andrew T; Lee, Bryan S; Walsh, Kevin; Bain, Mark D; Krishnaney, Ajit A

2015-01-01

17

Bilateral Type-I Duane Syndrome with Multiple Anamolies: A Case Report  

PubMed Central

The Duane syndrome is a strabismus syndrome which is characterized by congenital non-progressive horizontal ophthalmoplegia which primarily affects the abducens nerve. Approximately 70% of the individuals with the Duane syndrome have an isolated disease. We have described here, a case of bilateral Duane syndrome with associated anamolies. PMID:23205369

Varma, Chaitanya; Aroor, Shrikiran; Mundkur, Suneel C; Annamalai, Karthick

2012-01-01

18

Coexisting first and bilateral second branchial fistulas in a child with nonfamilial branchio-otic syndrome.  

PubMed

We describe what we believe is only the third reported case of coexisting first and bilateral second branchial fistulas associated with nonfamilial branchio-otic syndrome. The patient was a 6-year-old girl who presented with bilaterally draining anterior neck puncta, a preauricular sinus, and moderately severe bilateral hearing loss. She had no family history of branchial anomalies. Compared with branchial cysts and sinuses, branchial fistulas are rare. Even more rare are bilateral second branchial fistulas coexisting with first branchial anomalies, as only 10 cases have been previously reported in the English-language literature. Of these 10 cases, 5 were associated with either branchio-otic syndrome or branchio-oto-renal syndrome; 2 patients had familial branchio-otic syndrome, 2 had nonfamilial branchio-otic syndrome, and 1 had nonfamilial branchio-oto-renal syndrome. PMID:23904306

Lapeña, Jose F; Jimena, Genilou Liv M

2013-07-01

19

Bilateral Coronary Artery Dilatation and Supravalvular Pulmonary Stenosis in a Child with Noonan Syndrome  

Microsoft Academic Search

Noonan syndrome is the second most frequent congenital malformation syndrome, after Down syndrome, associated with cardiovascular\\u000a abnormalities. The most prevalent cardiovascular abnormalities in Noonan syndrome are pulmonary stenosis and hypertrophic\\u000a cardiomyopathy. We report the case of a 12-year-old girl with Noonan syndrome who had multiple cardiovascular abnormalities,\\u000a including extensive bilateral coronary artery dilatation, valvular and supravalvular pulmonary stenosis, atrial septal

T. Uçar; S. Atalay; M. Tekin; E. Tutar

2005-01-01

20

Compound heterozygosity in GPR56 with bilateral frontoparietal polymicrogyria.  

PubMed

Polymicrogyria is caused by a diverse etiology, one of which is gene mutation. At present, only one gene (GPR56) is known to cause polymicrogyria, which leads to a distinctive phenotype termed bilateral frontoparietal polymicrogyria (BFPP). BFPP is an autosomal recessive inherited human brain malformation with abnormal cortical lamination. Here, we identified compound heterozygous GPR56 mutations in a patient with BFPP. The proband was a Japanese female born from non-consanguineous parents. She presented with mental retardation, developmental motor delay, epilepsy exhibiting the feature of Lennox-Gastaut syndrome, exotropia, bilateral polymicrogyria with a relatively spared perisylvian region, bilateral patchy-white-matter MRI signal changes, and hypoplastic pontine basis. GPR56 sequence analysis revealed a c.107G>A substitution leading to a p.S36N, and a c.113G>A leading to a p.R38Q. Although affected individuals with compound heterozygosity in GPR56 have not been previously described, we presume that compound heterozygosity of these two mutations in a ligand binding domain within the extracellular N-terminus of protein could result in BFPP. In addition, we observed unusually less involvement of perisylvian cortex for polymicrogyria, and Lennox-Gastaut syndrome for epilepsy, which are likely common features in patients with BFPP caused by GPR56 mutations. PMID:23981349

Fujii, Yuji; Ishikawa, Nobutsune; Kobayashi, Yoshiyuki; Kobayashi, Masao; Kato, Mitsuhiro

2014-06-01

21

Bilateral Functional Thoracic Outlet Syndrome in a Collegiate Football Player  

PubMed Central

Background Thoracic Outlet Syndrome (TOS) involves compression of the brachial plexus, subclavius artery and vein. Many studies discuss efficacy of surgery and few discuss conservative treatment. It is unknown what specific forms of conservative treatment are best. Objective Describe conservative management for TOS using unique exercises. Case Description A collegiate football player reported numbness/tingling down his right arm after a right brachial plexus stretch injury. Seven months later, he was diagnosed with recurrent cervical traction neuropraxia. Two months later, he reported bilateral symptoms and was diagnosed with functional TOS. The athlete began shoulder strengthening (deltoid, middle trapezius, rhomboids, pectoralis major, latissimus dorsi, biceps, upper trapezius and rotator cuff) and stretching (pectoralis, scalene and upper trapezius) which failed to resolve his symptoms after four weeks. Surgical resection of bilateral first ribs and quitting football was recommended by four physicians. Unique therapeutic exercises developed by the Postural Restoration Institute™ were used to optimize respiration/posture via muscle activation and inhibition. After six weeks, the athlete was asymptomatic and returned to football but still experienced paresthesia with contact. Additional exercises were prescribed and remaining symptoms were abolished. Outcomes The Northwick Park Neck Pain Questionnaire was 55.5% at initial and 0% at four weeks and discharge. Discussion Athlete did not demonstrate relief of symptoms from shoulder stretching and strengthening. Intervention designed to optimize respiration/posture by repositioning the pelvis/trunk via specific muscle inhibition and activation resulted in abolishing the athlete's symptoms. Management that aims to optimize respiration via muscle inhibition, activation, and repositioning warrants further research. PMID:21509101

Robey, Jason H.

2009-01-01

22

Bilateral multifocal hemangiomas of the orbit in the blue rubber bleb nevus syndrome  

Microsoft Academic Search

PurposeTo report the clinical findings and surgical treatment of multifocal, bilateral orbital hemangiomas in a patient with the blue rubber bleb nevus syndrome, a rare vascular disorder characterized by distinctive cutaneous and visceral hemangiomas.

Eli L Chang; Peter A. D Rubin

2002-01-01

23

Bilateral carpal tunnel syndrome and multiple trigger fingers in a child with mucolipidosis Type III disease  

PubMed Central

This report presents a five-year-old girl with mucolipidosis Type III disease who had bilateral carpal tunnel syndrome and eight trigger fingers. Bilateral open carpal tunnel release was performed. The trigger fingers were treated with A1 and A3 pulleys release and the ulnar slips of the flexor digitorum superficialis tendons were removed. PMID:22279294

Afshar, Ahmadreza

2011-01-01

24

Bilateral coracoclavicular joints as a rare cause of bilateral thoracic outlet syndrome and shoulder pain treated successfully by conservative means.  

PubMed

The coracoclavicular joint (CCJ) is a rare anomalous joint. Symptomatic CCJ, being an exceptional rarity, makes it difficult to formulate a standard set of practice or guidelines. We report a 50-year-old Indian man, a machine operator by profession, who experienced bilateral shoulder pain and arm paraesthesia for two years, and was diagnosed with bilateral CCJ. The symptoms gradually increased, affecting his daily activities. Dynamic magnetic resonance imaging revealed the compression of the brachial plexus in extreme shoulder abduction. After a thorough search of the literature, we retrieved four similar cases, all of them treated with individualised protocols. None of the cases was bilateral. The lack of clear evidence in any particular direction and the patient's medical condition prompted us to give a conservative trial, before embarking on more invasive methods. He showed rapid response to the conservative treatment with remission of all symptoms. To the best of our knowledge, this is the first reported case of bilateral symptomatic CCJ with bilateral thoracic outlet syndrome, that was managed conservatively. PMID:19551300

Singh, V K; Singh, P K; Balakrishnan, S K

2009-06-01

25

Bilateral internal carotid artery dissection presenting as isolated unilateral Horner syndrome.  

PubMed

A 52-year-old man developed a unilateral Horner syndrome following a skiing accident. He was otherwise asymptomatic. Neuroimaging with magnetic resonance revealed bilateral internal carotid artery dissections, and he was urgently treated with anticoagulation. Immediate neuroimaging should be performed in any patient with Horner syndrome following trauma, regardless of other symptoms or signs. PMID:19935255

Demetriades, Anna M; Miller, Neil R; Garibaldi, Daniel C

2009-01-01

26

Central bilateral sixth nerve palsy associated with a unilateral preganglionic Horner's syndrome.  

PubMed

Unilateral peripheral sixth nerve palsy associated with an ipsilateral postganglionic Horner's syndrome has previously been described. Isolated bilateral sixth nerve palsy due to a pontine hemorrhagic lesion, however, is a rare occurrence. We describe such a case whose only other neurological finding was a unilateral preganglionic Horner's syndrome. PMID:2971682

Kellen, R I; Burde, R M; Hodges, F J; Roper-Hall, G

1988-09-01

27

Bilateral posterior semicircular canal dehiscence in the setting of Hallermann-Streiff syndrome.  

PubMed

Hallermann-Streiff syndrome, also known as oculomandibulofacial syndrome, is a rare congenital disorder affecting growth and cranial, dental, ocular, pilocutaneous, and mental development. In addition to routine audiologic testing in patients with this syndrome, high-resolution computed tomography of the temporal bones should be performed in those with documented or suspected sensorineural hearing loss. Cochlear implantation may be considered, as in other children with syndromic hearing loss and certain inner ear abnormalities, if the appropriate audiologic, psychosocial, and medical criteria are met. The current case report radiographically and clinically characterizes inner ear dysplasia in an 8-year-old patient with Hallermann-Streiff syndrome. High-resolution computed tomography of the temporal bones revealed a hypoplastic bony island between the vestibule and horizontal semicircular canals, as well as incomplete bony coverage of the posterior semicircular canal crura bilaterally. To our knowledge, this is the first report of a pediatric patient demonstrating bilateral posterior semicircular canal dehiscence. PMID:22996707

Goddard, John C; Oliver, Eric R; Meyer, Ted A

2012-09-01

28

Reversible cerebral vasoconstriction syndrome and bilateral vertebral artery dissection presenting in a patient after cesarean section  

PubMed Central

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden-onset thunderclap headache and focal neurologic deficits. Once thought to be a rare syndrome, more advanced non-invasive imaging has led to an increase in RCVS diagnosis. Unilateral vertebral artery dissection has been described in fewer than 40% of cases of RCVS. Bilateral vertebral artery dissection has rarely been reported. We describe the case of a patient with RCVS and bilateral vertebral artery dissection presenting with an intramedullary infarct treated successfully with medical management and careful close follow-up. This rare coexistence should be recognized as the treatment differs. PMID:23354867

Mitchell, Lex A; Santarelli, Justin G; Singh, Inder Paul; Do, Huy M

2013-01-01

29

Metachronous bilateral adrenocortical functional adenomas causing adrenocorticotropic hormone-independent Cushing’s syndrome  

PubMed Central

Metachronous bilateral adrenocortical adenomas causing adrenocorticotropic hormone-independent Cushing’s syndrome is a surgical case that scarcely occurs. A 38-year-old woman diagnosed with bilateral adrenocortical adenomas one by one in 2006 and 2013. Both of the two adenomas were functional and caused typical Cushingoid symptoms. The patient underwent a laparoscopic adrenolectomy in 2006 and a laparoscopic adrenocortical adenoma resection in 2013. After surgery, the symptoms of Cushing’s syndrome withdrew and the woman started steroid replacement therapy as a following treatment. PMID:25197412

Cheng, Kang; Cao, Wanli; Dai, Jun; Huang, Xin; Huang, Baoxing; Su, Henchuan; Sun, Fukang

2014-01-01

30

Metachronous Bilateral Posterior Tibial Artery Aneurysms in Ehlers-Danlos Syndrome Type IV  

SciTech Connect

Ehlers-Danlos syndrome type IV is a life-threatening genetic connective tissue disorder. We report a 24-year-old woman with EDS-IV who presented with metachronous bilateral aneurysms/pseudoaneurysms of the posterior tibial arteries 15 months apart. Both were treated successfully with transarterial coil embolization from a distal posterior tibial approach.

Hagspiel, Klaus D., E-mail: kdh2n@virginia.edu [University of Virginia, Department of Radiology (United States); Bonatti, Hugo [Vanderbilt University, Department of Surgery (United States); Sabri, Saher [University of Virginia, Department of Radiology (United States); Arslan, Bulent [Moffitt Cancer Center (United States); Harthun, Nancy L. [Johns Hopkins University, Division of Vascular Surgery, Department of Surgery (United States)

2011-04-15

31

Bilateral familial vertical Duane Syndrome with synergistic convergence, aberrant trigeminal innervation, and facial hypoplasia  

PubMed Central

A 5-year-old girl presented with bilateral familial vertical Duane retraction syndrome with alternating esotropia, elevation deficit, Marcus gunn phenomenon, and facial hypoplasia. Abnormal adducting downshoots on attempting abduction suggestive of a synergistic convergence were noted. Hypothesis suggests aberrant innervations or peripheral anatomic connections between inferior and medial recti. PMID:25378878

Gupta, Malvika; Gupta, Om Prakash; Vohra, Vishal

2014-01-01

32

Bilateral trigeminal nerve paralysis and Horner's syndrome associated with myelomonocytic neoplasia in a dog.  

PubMed

A 5-year-old male Doberman Pinscher had nasal stenosis, dropped mandible, bilateral atrophy of masseter and temporalis muscles, and Horner's syndrome caused by aleukemic myelomonocytic leukemia. Neoplastic cellular neurotropism, diffuse turbinate and nodular peribronchial infiltrate, and a hepatic portal infiltrative pattern similar to that of lymphoma were microscopic features of interest. PMID:3480282

Carpenter, J L; King, N W; Abrams, K L

1987-12-15

33

Cerebellar Ataxia with Bilateral Vestibulopathy: Description of a Syndrome and Its Characteristic Clinical Sign  

ERIC Educational Resources Information Center

We report four patients with the syndrome of cerebellar ataxia with bilateral vestibulopathy (CABV) and, using search coil oculography, we validate its characteristic clinical sign, namely impairment of the visually enhanced vestibulo-ocular reflex (VVOR) or doll's head reflex. In our four patients, CABV began in the sixth decade of life; they are…

Migliaccio, Americo A.; Halmagyi, G. Michael; McGarvie, Leigh A.; Cremer, Phillip D.

2004-01-01

34

Bilateral squamosal suture synostosis: A rare form of isolated craniosynostosis in Crouzon syndrome.  

PubMed

Craniosynostosis is a pathologic condition which is characterized by the premature fusion of cranial sutures. It may occur alone or in association with other anomalies making up various syndromes. Crouzon syndrome is the most common craniosynostosis syndrome. Bicoronal sutures fusion is most commonly involved in Crouzon syndrome. There have only been a handful of cases of squamosal suture synostosis described in the surgery literature with the few ones described in Crouzon syndrome associated with other types of craniosynostosis. To the best of our knowledge, we are presenting the first case of isolated bilateral squamosal suture synostosis in a patient with Crouzon syndrome in a radiology journal with emphasis on its radiological appearance. PMID:25071892

Tandon, Yasmeen K; Rubin, Michael; Kahlifa, Mohamed; Doumit, Gaby; Naffaa, Lena

2014-07-28

35

Bilateral striatal necrosis in two subjects with Aicardi-Goutières syndrome due to mutations in ADAR1 (AGS6).  

PubMed

Aicardi-Goutières syndrome (AGS) is a genetic inflammatory disease. The classic neuroradiological picture mimics that of congenital infections in that Aicardi-Goutières syndrome is characterized by leukoencephalopathy, brain atrophy and intracranial calcifications. To date, bilateral striatal necrosis has not been reported in patients with AGS. We report on two patients with clinical diagnosis of Aicardi-Goutières syndrome in which brain MRI and CT scans demonstrated bilateral striatal necrosis. The diagnosis of Aicardi-Goutières syndrome in these two patients was genetically confirmed after the recent discovery that mutations in the ADAR1 (AGS6) gene may cause Aicardi-Goutières syndrome. This is the first report of bilateral striatal necrosis in association with Aicardi-Goutières syndrome. These results expand the neuroradiological phenotype of Aicardi-Goutières syndrome. PMID:24376015

La Piana, Roberta; Uggetti, Carla; Olivieri, Ivana; Tonduti, Davide; Balottin, Umberto; Fazzi, Elisa; Orcesi, Simona

2014-03-01

36

Bilateral hand\\/wrist heat and cold hyperalgesia, but not hypoesthesia, in unilateral carpal tunnel syndrome  

Microsoft Academic Search

The aim of the current study was to evaluate bilaterally warm\\/cold detection and heat\\/cold pain thresholds over the hand\\/wrist\\u000a in patients with carpal tunnel syndrome (CTS). A total of 25 women with strictly unilateral CTS (mean 42 ± 10 years), and\\u000a 20 healthy matched women (mean 41 ± 8 years) were recruited. Warm\\/cold detection and heat\\/cold pain thresholds were assessed\\u000a bilaterally over the carpal tunnel and

Ana Isabel de la Llave-Rincón; César Fernández-de-las-Peñas; Josué Fernández-Carnero; Luca Padua; Lars Arendt-Nielsen; Juan A. Pareja

2009-01-01

37

Klüver-Bucy Syndrome with Isolated Bilateral Hippocampal Atrophy Following Status Epilepticus  

PubMed Central

Klüver-Bucy syndrome may result from affection of various location of brain. We report a case of Klüver-Bucy syndrome associated with isolated bilateral hippocampal atrophy without any abnormal lesion in other areas following status epilepticus. A 31-year-old man who had no significant medical history presented with status epilepticus after encephalitis of unknown etiology. He had been recovered from status epilepticus three weeks later, but afterwards he developed Klüver-Bucy syndrome: hyperphagia, hypersexuality, hypermetamorphosis, anterograde amnesia and dysosmia. Initial brain MRI showed T2 hyperintensity and swelling of isolated bilateral hippocampus, especially CA1 region without any abnormal lesion in other areas. One month later, follow-up brain MRI showed isolated bilateral hippocampal atrophy. This is a meaningful case report because this case differs from other reports of Klüver-Bucy syndrome in humans in that the anatomic abnormalities revealed by MRI were very selective. We report this case because this case is very educative for above reason. Moreover, this report would give us additional information of the relationship between human behavior and limbic system. PMID:24649454

Park, Hong-Kyun; Kim, Kyeong-Joon; Moon, Hye-Jin; Kim, Seon-Jeong; Yun, Chang-Ho; Park, Seong-Ho

2012-01-01

38

Branchiooculofacial syndrome and bilateral ectopic thymus: report of a family.  

PubMed

Branchiooculofacial syndrome (BOFS) is a rare autosomal-dominant condition characterized by branchial cleft sinus and ophthalmologic and craniofacial abnormalities that can range from mild to severe forms. Ectopic thymus, an uncommon condition with just over 100 cases reported in the literature, is seen in BOFS. We report a family with BOFS affecting the mother and two daughters, showing the dominant inheritance and variable phenotypical expression of BOFS. PMID:23106675

Loh, James; El-Hakim, Hamdy; Sergi, Consolato Maria; Fiorillo, Loretta

2012-01-01

39

46,XY female sex reversal syndrome with bilateral gonadoblastoma and dysgerminoma.  

PubMed

Sex reversal syndrome is a rare congenital condition of complete or disordered gonadal development leading to discordance between the genetic, gonadal and phenotypic sexes, including 46,XX and 46,XY. The gonadoblastoma on the Y-chromosome (GBY) region is associated with an increased risk of developing type II germ cell tumors/cancer. The present study reports a unique case of a phenotypically normal female (age 17 years), presenting with primary amenorrhea and later diagnosed with 46,XY female sex reversal syndrome. Following bilateral gonadectomy, bilateral gonadoblastoma and dysgerminoma were diagnosed. Thus, estrogen replacement therapy was administered periodically to promote the development of secondary sexual characteristics and menstruation, and to prevent osteoporosis. A four year follow-up showed no tumor recurrence and a regular menstrual cycle in this patient. PMID:25187804

DU, Xue; Zhang, Xuhong; Li, Yongmei; Han, Yukun

2014-10-01

40

46,XY female sex reversal syndrome with bilateral gonadoblastoma and dysgerminoma  

PubMed Central

Sex reversal syndrome is a rare congenital condition of complete or disordered gonadal development leading to discordance between the genetic, gonadal and phenotypic sexes, including 46,XX and 46,XY. The gonadoblastoma on the Y-chromosome (GBY) region is associated with an increased risk of developing type II germ cell tumors/cancer. The present study reports a unique case of a phenotypically normal female (age 17 years), presenting with primary amenorrhea and later diagnosed with 46,XY female sex reversal syndrome. Following bilateral gonadectomy, bilateral gonadoblastoma and dysgerminoma were diagnosed. Thus, estrogen replacement therapy was administered periodically to promote the development of secondary sexual characteristics and menstruation, and to prevent osteoporosis. A four year follow-up showed no tumor recurrence and a regular menstrual cycle in this patient. PMID:25187804

DU, XUE; ZHANG, XUHONG; LI, YONGMEI; HAN, YUKUN

2014-01-01

41

Ovarian remnant syndrome after laparoscopic hysterectomy and bilateral salpingo-oophorectomy for severe pelvic endometriosis.  

PubMed

Ovarian remnant syndrome is a rare complication of total abdominal hysterectomy and bilateral salpingo-oophorectomy (BSO). Ovarian enlargement and dense periovarian adhesions are the predisposing factors. Recurrent ovarian remnant syndrome was associated with recurrence of symptomatic endometriosis in a woman who underwent laparoscopic supracervical hysterectomy and BSO for severe endometriosis and extensive pelvic adhesions. After primary surgery she required five additional procedures for complete resection of all ovarian remnants. Definitive surgery for advanced endometriosis with extensive periovarian adhesions may be complicated by ovarian remnant syndrome and reactivation of the disease. Careful retroperitoneal resection of all ovarian tissue is of paramount importance in preventing the syndrome. This, however, may be a limitation of laparoscopic surgery. The choice between laparoscopy and laparotomy in such cases should be individualized and based on the degree of surgical difficulty and the surgeon's level of experience. PMID:9050667

Rana, N; Rotman, C; Hasson, H M; Redwine, D B; Dmowski, W P

1996-05-01

42

Bilateral pleural effusion with APLA positivity in a case of rhupus syndrome  

PubMed Central

Rhupus syndrome is a rare syndrome characterized by overlap of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Our patient was a diagnosed case of RA and developed SLE 2 years after. She was a middle-aged woman, presented with bilateral pleural effusion with exacerbation of skin and joint symptoms of SLE. We diagnosed the case as tubercular pleural effusion by positive Mycobacterium tuberculosis in bactec 460 culture. She had also anti-phospholipid antibody positivity without any symptoms and signs of thrombosis. PMID:25378850

Saha, Kaushik; Saha, Arnab; Mitra, Mrinmoy; Panchadhyayee, Prabodh

2014-01-01

43

Bilateral Renal Tumour as Indicator for Birt-Hogg-Dubé Syndrome  

PubMed Central

Birt-Hogg-Dubé (BHD) syndrome is a cancer disorder caused by a pathogenic FLCN mutation characterized by fibrofolliculomas, lung cysts, pneumothorax, benign renal cyst, and renal cell carcinoma (RCC). In this case we describe a patient with bilateral renal tumour and a positive familial history for pneumothorax and renal cancer. Based on this clinical presentation, the patient was suspected for BHD syndrome, which was confirmed after molecular testing. We discuss the importance of recognizing this autosomal dominant cancer disorder when a patient is presented at the urologist with a positive family history of chromophobe renal cell cancer or a positive familial history for renal cell cancer and pneumothorax. PMID:24772173

Johannesma, P. C.; van Moorselaar, R. J. A.; Horenblas, S.; van der Kolk, L. E.; Thunnissen, E.; van Waesberghe, J. H. T. M.; Menko, F. H.; Postmus, P. E.

2014-01-01

44

Treatment of hyperacusis in Williams syndrome with bilateral conductive hearing loss  

Microsoft Academic Search

Williams syndrome (WS) is a rather rare congenital disorder characterised by a series of cardiovascular, maxillo-facial and\\u000a skeletal abnormalities. It sometimes displays otorhinolaryngological symptoms because of the relatively high incidence of\\u000a secretory otitis media and hyperacusis, which may be present in up to 95% of patients. The present paper describes a case\\u000a of WS associated with bilateral conductive hearing loss

Cesare Miani; Paolo Passon; Anna Maria Bergamin Bracale; Alessandra Barotti; Nadia Panzolli

2001-01-01

45

Bilateral brachial plexus palsy and right Horner syndrome due to congenital cervicothoracal syringomyelia.  

PubMed

Syringomyelia (SM) is a disorder in which a cyst forms within the spinal cord. This cyst, called a syrinx, expands and elongates over time, destroying the center of the cord. Horner syndrome is an infrequent illness caused by a lesion of the cervical sympathetic nerve fiber. Its clinical features are facial anhidrosis, ptosis, miosis, and hypochromia iridis of the affected side. A full-term male newborn infant was admitted with weakness in bilateral upper extremities and narrowing of the palpebral fissure on the right side. Ophthalmologic examination revealed a smaller right pupil. Muscle power in bilateral upper limbs was 1/5. Chest X-ray and cranial magnetic resonance imaging were normal. Magnetic resonance imaging of the cervicothoracic spine showed SM at C4-T2 level. Electromyographic examination revealed bilateral brachial plexus palsy. The diagnosis was of brachial plexus palsy and congenital Horner syndrome due to congenital cervicothoracic SM. According to our best knowledge, this association has not been reported in the literature. PMID:19765926

Ca?an, Eren; Sayin, Refah; Do?an, Murat; Peker, Erdal; Ca?an, Havva Hasret; Caksen, Hüseyin

2010-08-01

46

Bilateral deficits in fine motor control ability and manual dexterity in women with fibromyalgia syndrome.  

PubMed

The aim of the current study was to investigate fine motor control ability and manual dexterity women with fibromyalgia syndrome (FMS) without symptoms in the upper extremity compared to healthy women. Subtests of the Purdue Pegboard Test (one-hand, bilateral and assembly) and of the Jebsen-Taylor hand-function test (writing, turning cards, picking up small, light and large heavy objects, simulated feeding and stacking checkers) were evaluated bilaterally in 20 women with FMS (aged 35-55 years) without symptoms in the upper limb and 20 age- and hand dominance-matched healthy women. Differences between sides and groups were analysed with several analysis of variance (ANOVA). The ANOVA revealed significant differences between groups (P < 0.001) and sides (P = 0.007) for one-hand pin placement subtest: women with FMS showed bilateral worse scores than controls. Patients also exhibited significantly lower scores in bilateral pin placement and assembly subtests when compared to healthy controls (P < 0.001). The ANOVA also revealed significant differences between groups for writing, turning over cards, picking up small objects, stacking checkers, picking up large light objects and picking up large heavy objects (all, P < 0.001): women with FMS needed more time for these subtests than healthy women with both hands. No difference for simulated feeding was found between groups. Our findings revealed bilateral deficits in fine motor control ability and manual dexterity in patients with FMS without symptoms in the upper extremity. These deficits are not related to the clinical features of the symptoms supporting an underlying central mechanism of altered motor control. PMID:23354668

Pérez-de-Heredia-Torres, Marta; Martínez-Piédrola, Rosa M; Cigarán-Méndez, Margarita; Ortega-Santiago, Ricardo; Fernández-de-Las-Peñas, César

2013-04-01

47

Coats’ disease and bilateral cataract in a child with Turner syndrome: a case report  

Microsoft Academic Search

Purpose  To report the first case in which Coats’ disease was observed with infantile cataract in a girl with Turner syndrome (TS).\\u000a \\u000a \\u000a \\u000a Materials and methods  We examined a 4-year-old female infant with TS who was referred with a diagnosis of leukocoria in the left eye.\\u000a \\u000a \\u000a \\u000a Results and discussion  Examination under anaesthesia revealed a bilateral punctate cataract and left eye fundus showed vascular retinal

F. Beby; O. Roche; C. Burillon; P. Denis

2005-01-01

48

Bilateral Upper Extremity DVT in a 43-Year-Old Man: Is It Thoracic Outlet Syndrome?!  

PubMed Central

Recurrent deep venous thrombosis, involving bilateral upper extremities, is an extremely rare phenomenon. We are presenting a 43-year-old man who was diagnosed with left upper extremity deep vein thrombosis (UEDVT) and was treated with anticoagulation and surgical decompression in 2004. 9 years later, he presented with right arm swelling and was diagnosed with right UEDVT using US venous Doppler. Venogram showed compression of the subclavian vein by the first rib, diagnosing thoracic outlet syndrome (TOS). He was treated with anticoagulation and local venolysis and later by surgical decompression of the subclavian vein. Bilateral UEDVT, as mentioned above, is an extremely rare condition that is uncommonly caused by TOS. To our knowledge, we are reporting the first case of bilateral UEDVT due to TOS. Diagnosis usually starts with US venous Doppler to detect the thrombosis, followed by the gold standard venogram to locate the area of obstruction and lyse the thrombus if needed. The ultimate treatment for TOS remains surgical decompression of the vascular bundle at the thoracic outlet. PMID:25140182

Sanghavi, Devang; Chalise, Shyam

2014-01-01

49

Bilateral frontoparietal polymicrogyria, Lennox-Gastaut syndrome, and GPR56 gene mutations  

PubMed Central

Summary Purpose Bilateral frontoparietal polymicrogyria (BFPP) has been reported in sporadic patients and in recessive pedigrees. Eleven mutations in GPR56, a gene encoding an evolutionarily dynamic G-protein–coupled receptor, have been identified in 29 patients from 18 families. The clinical features of BFPP include severe mental retardation, motor and language impairment, and epilepsy. No detailed description of the epilepsy is available for the patients reported to date. We report three consanguineous families in which four affected individuals with BFPP and GPR56 mutations had Lennox-Gastaut syndrome. Methods Family studies, brain magnetic resonance imaging (MRI), electroencephalography (EEG)-video recordings, and mutation analysis. Results In Family 1, with one affected proband, we found an R565W change in the second extracellular loop of GPR56, involving a highly conserved aminoacidic residue. In Family 2, with one affected proband, we found an R79X change affecting the protein N-terminus and predicted to cause a premature truncation with loss of the G-protein–coupled receptor proteolytic site. In family 3, with two affected siblings, we found an R33P substitution in the protein N-terminus, involving a highly conserved aminoacidic residue. Epilepsy, present in all four patients, had started between ages 1 and 8 years, with infantile spasms in one patient and with de novo Lennox-Gastaut syndrome in the remaining three. All patients had Lennox-Gastaut syndrome when last observed, at ages 13 to 32 years. Discussion Several genes, when mutated, can cause malformations of cortical development that have been associated with the Lennox-Gastaut syndrome. BFPP caused by GPR56 mutations represents an additional, although rare, genetically determined cause of Lennox-Gastaut syndrome. PMID:19016831

Parrini, Elena; Ferrari, Anna Rita; Dorn, Thomas; Walsh, Christopher A.; Guerrini, Renzo

2014-01-01

50

Bilateral striopallidodentate calcification (Fahr's syndrome) and multiple system atrophy in a patient with longstanding hypoparathyroidism.  

PubMed

Recently, a family with idiopathic brain calcification was reported, in which one family member was diagnosed with multiple system atrophy (MSA) at autopsy. We report here a case showing similar neuropathological features in a patient with longstanding hypoparathyroidism. Our female patient had a history of hypoparathyroidism with hypocalcaemia and tetany since the age of 9 years. In her 50s she developed dementia and parkinsonism. She died of myocardial infarction aged 65 years. Neuropathology showed severe brain calcifications of the Fahr type in the basal ganglia, thalami, cerebral and cerebellar white matter and dentate nuclei. Additionally, there was prominent alpha-synucleinopathy of the multiple system atrophy type (MSA). The patient has a healthy identical twin and there is no family history of hypoparathyroidism or neurological disease. Data on alpha-synuclein accumulation in various cases of Fahr's syndrome are needed to establish the correlation between alpha-synucleinopathy and bilateral striopallidodentate calcification. PMID:18018479

Preusser, Matthias; Kitzwoegerer, Melitta; Budka, Herbert; Brugger, Stefan

2007-10-01

51

A rare differential diagnosis to occupational neck pain: bilateral stylohyoid syndrome  

PubMed Central

Chronic neck pain is widely prevalent and a common source of disability in the working-age population. Etiology of chronic neck pain includes neck sprain, mechanical or muscular neck pain, myofascial pain syndrome, postural neck pain as well as pain due to degenerative changes. We report the case of a 42 year old secretary, complaining about a longer history of neck pain and limited movement of the cervical spine. Surprisingly, the adequate radiologic examination revealed a bilateral ossification of the stylohyoid ligament complex. Her symptoms remained intractable from conservative treatment consisting of anti-inflammatory medication as well as physical therapy. Hence the patient was admitted to surgical resection of the ossified stylohyoid ligament complex. Afterwards she was free of any complaints and went back to work. Therefore, ossification of the stylohyoid ligament complex causing severe neck pain and movement disorder should be regarded as a rare differential diagnosis of occupational related neck pain. PMID:16800878

Kirchhoff, Gertrud; Kirchhoff, Chlodwig; Buhmann, Sonja; Kanz, Karl-Georg; Lenz, Miriam; Vogel, Tobias; Kichhoff, Rainer Maria

2006-01-01

52

Unbalanced der(5)t(5;20) translocation associated with Megalencephaly, perisylvian Polymicrogyria, Polydactyly and Hydrocephalus  

PubMed Central

The combination of megalencephaly, perisylvian polymicrogyria, polydactyly and hydrocephalus (MPPH) is a rare syndrome of unknown cause. We observed two first cousins affected by an MPPH-like phenotype with a submicroscopic chromosome 5q35 deletion as a result of an unbalanced der(5)t(5;20)(q35.2;q13.3) translocation, including the NSD1 Sotos syndrome locus. We describe the phenotype and the deletion breakpoints of the two MPPH-like patients and compare these with five unrelated MPPH and Sotos patients harboring a 5q35 microdeletion. Mapping of the breakpoints in the two cousins was performed by MLPA, FISH, high density SNP-arrays and Q-PCR for the 5q35 deletion and 20q13 duplication. The 5q35 deletion area of the two cousins almost completely overlaps with earlier described patients with an atypical Sotos microdeletion, except for the DRD1 gene. The five unrelated MPPH patients neither showed submicroscopic chromosomal aberrations nor DRD1 mutations. We reviewed the brain MRI of 10 Sotos patients and did not detect polymicrogyria in any of them. In our two cousins, the MPPH-like phenotype is probably caused by the contribution of genes on both chromosome 5q35 and 20q13. Some patients with MPPH may harbor a submicroscopic chromosomal aberration and therefore high-resolution array analysis should be part of the diagnostic workup. PMID:20503325

Verkerk, Annemieke J.M.H.; Schot, Rachel; van Waterschoot, Laura; Douben, Hannie; Poddighe, Pino J.; Lequin, Maarten H.; de Vries, Linda S.; Terhal, Paulien; Hahnemann, Johanne M.D.; de Coo, Irenaeus F.M.; de Wit, Marie-Claire Y.; Wafelman, Leontien S.; Garavelli, Livia; Dobyns, William B.; Van der Spek, Peter J.; de Klein, Annelies; Mancini, Grazia M.S.

2010-01-01

53

Mosaic Chromosome 18q Partial Deletion Syndrome with Bilateral Full-thickness Corneal Disease: Surgical Intervention and Histopathology.  

PubMed

Abstract A 2-month-old boy diagnosed with a mosaic chromosome 18q partial deletion syndrome was referred for bilateral cloudy corneas. The abnormal metaphases had a terminal deletion of the long arm of chromosome 18 as clonal abnormality. The cytogenetics findings were 46,XY, del (18)(q21.2)[12]/46,XY[20]. Ocular findings included bilateral microcornea with dense opacification and unilateral iris and chorioretinal coloboma. Penetrating keratoplasty (PK) was performed on both eyes. Histopathology of the host corneal button showed complete loss of Bowman's layer, hyperkeratosis of the epithelium, stromal neovascularization, and leukocyte infiltration. Descemet's membrane and endothelium were irregular in both specimens. CD45 stain for leukocytes confirmed perivascular and epithelial leukocytes infiltration. Mosaic chromosome 18q deletion syndrome is a rare genetic abnormality with a variable phenotype - including ocular findings - and hence, warrants an ophthalmic evaluation and genetic counseling. PMID:24024746

Galvin, Jennifer A; LeBoyer, Russell M; Michelotti, Monica; Monte, Monte A Del; Elner, Victor M; Mian, Shazad I

2015-03-01

54

[A male case of acute sarcoidosis with fever, polyarthralgia, erythema nodosum, and bilateral hilar lymphadenopathy: Löfgren's syndrome].  

PubMed

A 27-year-old man initially had low back pain and ankle arthralgia. He was admitted because fever, cough, and polyarthralgia developed and continued for three months. Chest X-ray and CT revealed bilateral hilar and mediastinal lymphadenopathy with pulmonary lesions. Furthermore, elevated serum-ACE level and noncaseating epitheloid cell granuloma obtained by TBLB confirmed the diagnosis of sarcoidosis. After hospitalization, erythema nodosum appeared and ocular involvement was demonstrated. As a result, this case fulfilled the criteria of Löfgren's syndrome (arthritis, erythema nodosum, and BHL). Löfgren's syndrome is not uncommon in European countries, but is extremely rare in Japan. So far, only six cases with Löfgren's syndrome were reported in Japan, and all were female cases. This is the first male case in Japan. Löfgren's syndrome is usually a self-limiting disease. We used steroids for this case and remission has been maintained after the beginning of the treatment for the past one year. PMID:16457340

Izumo, Mayu; Sekiya, Kiyoshi; Sakai, Toshihiko; Tojima, Hirokazu

2005-12-01

55

Eagle's syndrome: treatment by intraoral bilateral resection of the ossified stylohyoid ligament. A review and report of two cases.  

PubMed

The complexities of Eagle's syndrome are examined according to anatomical, historical, clinical, and treatment aspects. There appears to be little correlation between the extent, form, and size of the anomalies of the styloid process and the stylohyoid ligament and the predictability of patients with related symptoms. Surgical treatment and the advantages and disadvantages of each surgical approach (intra- and extraoral) are discussed. The probable causes of enlargement of the styloid process and ossification of the stylohyoid ligament are addressed. Two cases of Eagle's syndrome are presented: one unilateral and the other bilateral. An intraoral modified surgical technique is presented. PMID:23971163

Martins, Wilson Denis; Ribas, Marina de Oliveira; Bisinelli, Julio; França, Beatriz Helena Sottile; Martins, Guilherme

2013-07-01

56

Hallermann–Streiff syndrome with severe bilateral enophthalmos and radiological evidence of silent brain syndrome: a new congenital silent brain syndrome?  

PubMed Central

Background We present the first case of a congenital form of silent brain syndrome (SBS) in a young patient affected by Hallermann–Streiff syndrome (HSS) and the surgical management of the associated eyelid anomalies. Methods HSS signs were evaluated according to the Francois criteria. Orbital computed tomography (CT) and genetic analysis were performed. An upper eyelid retractor-free recession was performed. Follow-up visits were performed at day 1, weeks 1 and 3, and months 3, 6, 9 (for both eyes), and 12 (for left eye) after surgery. Results The patient exhibited six of the seven signs of HSS. Orbital CT showed bilateral enophthalmos and upward bowing of the orbital roof with air entrapment under the upper eyelid as previously described for SBS. Genetic analysis showed a 2q polymorphism. During follow-up, the cornea showed absence of epithelial damage and the upper eyelids were lowered symmetrically, with a regular contour. Conclusion Our HSS patient shares features with SBS. We postulate that SBS could include more than one pattern, ie, an acquired form following ventriculoperitoneal shunting and this newly reported congenital form in our HSS patient in whom typical syndromic skull anomalies led to this condition. The surgical treatment has been effective in restoring an appropriate lid level, with good globe apposition and a good cosmetic result. PMID:21792277

Nucci, Paolo; de Conciliis, Carlo; Sacchi, Matteo; Serafino, Massimiliano

2011-01-01

57

Perisylvian Functional Connectivity during Processing of Sentential Negation.  

PubMed

Every language has the means to reverse the truth value of a sentence by using specific linguistic markers of negation. In the present study we investigated the neural processing costs afforded by the construction of meaning in German sentences containing negation in different clause types. We studied negations within and across clause boundaries as well as single and double negations. Participants read German sentences comprising of affirmations, single negations in the main or in the subordinate clause, or double negations. As a result, we found a network including the left inferior frontal gyrus (pars triangularis, BA 45), and the left inferior parietal gyrus (BA 40) to be activated whenever negations in the main clause had to be processed. Additionally, we found increased functional coupling between the left pars triangularis (BA 45), left pars opercularis (BA 44), left SMA (BA 6), and left superior temporal gyrus (BA 42) during the processing of main clause negations. The study shows that in order to process negations that require semantic integration across clause boundaries left BA 45 interplays with other areas that have been related to language processing and/or the processing of cognitive demands and logical/conditional reasoning. Thus, the results indicate that the left perisylvian language network synchronizes in order to resolve negations, in particular, whenever requirements on meaning integration are enhanced. PMID:21687464

Bahlmann, Jörg; Mueller, Jutta L; Makuuchi, Michiru; Friederici, Angela D

2011-01-01

58

Acute bilateral exercise-induced medial compartment syndrome of the thigh. Correlation of repeated MRI with clinicopathological findings.  

PubMed

We present a case report of acute bilateral excercise-induced compartment syndrome in the adductor longus muscles, which was treated with bilateral medial fasciotomies. Postoperatively, the healing process of the adductor muscles was followed up by repeated MR imagings over six months. Myonecrosis was found in peroperative muscle biopsies. Pain and muscle swelling subsided soon after the fasciotomy, correlating with the early postoperative MR findings. Four months postoperatively, the signal intensity of the adductor muscles was normal in T1- and T2-weighted images, but the normal fibre structure of the adductor muscles could only be seen 6 months postoperatively. At six month's control checkup there was no subjective weakness of the adductors, and hyperesthesia had disappeared and the patient was capable of normal activities. PMID:12439779

Leppilahti, J; Tervonen, O; Herva, R; Karinen, J; Puranen, J

2002-11-01

59

Bilateral lid/brow elevation procedure for severe ptosis in Kearns-Sayre syndrome, a mitochondrial cytopathy  

PubMed Central

Background The purpose of this work was to determine the effectiveness and possible complications encountered with bilateral fascia lata lid suspension used to correct blepharoptosis in patients with Kearns-Sayre syndrome. Methods This was a retrospective study of seven patients with Kearns-Sayre syndrome who had a minimum of 1 year of follow-up. A bilateral fascia lata sling was used to correct the ptosis. Preoperative and postoperative measurements of the vertical lid fissure width (VFW) and marginal reflex distance (MRD) were performed. The Student’s t-test was used to analyze the results. Results The mean preoperative VFW and MRD measurements were 4±2.45 mm and 0.14±0.92 mm, respectively. The mean postoperative VFW and MRD measurements were 7.71±1.85 mm, and 2.86±1.69 mm, respectively. All preoperative and postoperative values were considered to be statistically significant (P<0.01). Adequate elevation of the lids was obtained in all patients, both functionally and aesthetically. All of the patients showed a mild symmetric postoperative inferior version lagophthalmos, and one patient developed corneal ulceration and scarring due to corneal exposure and a weak Bell’s phenomenon. Conclusion The surgical technique described to correct the blepharoptosis found in patients with Kearns-Sayre syndrome was found to be efficient and relatively safe. The correction should be conservative to decrease the risk of postoperative corneal damage that occurred in one patient. PMID:25565765

Sebastiá, Roberto; Fallico, Ester; Fallico, Matteo; Fortuna, Eduardo; Lessa, Sergio; Neto, Guilherme Herzog

2015-01-01

60

Bilateral claw hand: an uncommon presentation of regional Guillain-Barré syndrome.  

PubMed

We present an uncommon case of a 38-year-old man presented with bilateral subacute weakness of intrinsic hand muscles, manifesting as bilateral claw-hand, without sensory deficits and absent tendon reflexes in upper arms. Nerve conduction studies showed findings consistent with demyelinating GBS. During the fourth day of hospitalization the patient presented symmetrical distal leg weakness and was treated with intravenous immunoglobulin. PMID:23927940

Tsivgoulis, Georgios; Tsakaldimi, Soultana; Vadikolias, Konstantinos; Mantatzis, Michalis; Katsanos, Aristeidis H; Heliopoulos, Ioannis; Piperidou, Charitomeni

2013-11-15

61

Fluctuating Kluver-Bucy syndrome in a child with epilepsy due to bilateral anterior temporal congenital malformations.  

PubMed

An 11-year-old boy with epilepsy due to congenital bilateral anterior temporal lobe malformations presented with fluctuating Kluver-Bucy syndrome (KBS). Since the age of 2, he had experienced clusters of three or four daily complex partial seizures over 2-3 days in a month, followed by a seizure-free interval of 3-4 weeks. During the seizure-free period, the patient exhibited hyperorality, sniffing, irritability alternating with placidity, anxiety, unsolicited sexual gestures, and unusual calmness after eating. KBS features escalated up to the onset of the seizure cluster, and remitted after the seizures. Brain MRI revealed bilateral anterior temporal cortical dysplasia with enlarged and dysmorphic amygdalar-hippocampal complex. Brain [(18)F]fluorodeoxyglucose positron emission tomography PET showed bilateral anterior and mesial temporal hypometabolism. Video/EEG monitoring revealed independent right and left temporal lobe seizures. This is the first reported case of KBS due to congenital bitemporal malformations. Also, KBS behavior phenotype in this patient fluctuated, with escalation during the seizure-free period and remission induced by the monthly seizure cluster. This fluctuating pattern could represent forced normalization. PMID:17218157

Pestana, Elia M; Gupta, Ajay

2007-03-01

62

Bilateral ovarian carcinoma with bilateral uveal melanoma.  

PubMed Central

A case of bilateral uveal melanoma in a 60-year-old woman in association with primary bilateral ovarian carcinoma is described. This is the first case in which ultrastructural studies have been performed on the ocular tumours. Seven previously described cases are summarised, and the extreme rarity of such reports would suggest that this may indeed be a new syndrome. Images PMID:6704361

Mullaney, J; Mooney, D; O'Connor, M; McDonald, G S

1984-01-01

63

[A case of Shy-Drager syndrome complicated with syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and incomplete paralysis of bilateral vocal cords].  

PubMed

A 67-year-old man with SIADH complicated by slowly progressing autonomic failure was described. The patient noticed constipation at the age of 57. In the following years, he suffered from urinary incontinence, depletion of sweating, impotence, sleeplessness with snore, and dizziness while walking. Physical examination revealed a masked oily face with slight cerebellar disturbance. Abnormality of autonomic function tests was recognized and he was diagnosed as Shy-Drager syndrome with gradually progressing, diffuse autonomic failure accompanied by slight cerebellar ataxia and Parkinsonism. Both serum sodium level and plasma osmotic pressure were reduced, whereas daily sodium excretion was more than 100mEq and urinary osmolality was about 500mOsm/kgH2O. His renal function was intact, and the adrenocortical and thyroid hormone levels were normal, then criteria of SIADH was fulfilled. SIADH was thought to have occurred on the basis of Shy-Drager syndrome. Water load test showed failure of adequate water diuresis, but intravenous phenytoin administration following the water load test ameliorated the diuresis to normal. The relationship between plasma osmolality and the ADH response indicates that ADH was adequately secreted in response to the increase in plasma osmolality but not suppressed in response to the decrease in plasma osmolality below 280mOsm/kgH2O. These results suggest that ADH synthesis in the hypothalamus and its secretion from the pituitary gland were both intact. The response of ADH secretion to the orthostatic hypotension induced by head-up tilt was quite blunted, being compatible with Shy-Drager Syndrome. Sleep disturbance was studied by polysomnography and laryngoscopy, and was revealed to be based upon severe sleep apnea due to incomplete paralysis of the bilateral vocal cords. Sleep apnea due to vocal cord paralysis is sometimes found to be complicated in patients with multiple system atrophy (MSA) including Shy-Drager syndrome, and is known as Gerhardt syndrome. This is the first report on a case of Shy-Drager syndrome complicated with SIADH and bilateral vocal cord paralysis. In this case, SIADH is caused by impaired afferent pathways from baroreceptors to the hypothalamus, which transfer inhibitory stimuli on ADH secretion. It is suggested that Shy-Drager syndrome should be considered one of the causes of SIADH. PMID:7958087

Sone, H; Okuda, Y; Bannai, C; Asano, M; Asakura, Y; Yamaoka, T; Suzuki, S; Kawakami, Y; Odawara, M; Matsushima, T

1994-03-20

64

Good visual outcome in an immunocompromised patient with bilateral acute retinal necrosis syndrome: A case report  

PubMed Central

Acute retinal necrosis (ARN) is an uncommon necrotizing, fulminant retinopathy caused by the herpes simplex virus types 1 or 2 or by the varicella zoster vírus with visually devastating consequences. Generally it occurs in patients who are systemically healthy, but occasionally occurs in immunocompromised host. We report a case of bilateral ARN in a patient with AIDS with a good final visual outcome. PMID:25278806

Marrocos de Aragão, Ricardo E.; Barreira, Ieda M.A.; Arrais, Barbara L.A.; Pereira, Leidiane A.; Ramos, Carine S.

2013-01-01

65

Restoration of Severely Impaired Eyesight in an Adolescent with Down Syndrome and Bilateral Cataract in South Asia  

PubMed Central

Purpose To report the recovery of visual function after phacoemulsification in a 16-year-old adolescent with Down syndrome (DS) from Saharsa, India. Methods Interventional case report and short review of the pertinent literature. Results A 16-year-old South Asian male with DS and bilateral cataract underwent successful surgery in both eyes after considerable difficulty for his parents including cross-border travel from India into Nepal. After the operation, the patient responded to visual stimuli (e.g. movement of hands) that had eluded him prior to surgery, and thus the additional obstacle to social participation imposed by the impaired eyesight could be removed. Conclusions The establishment of basic ophthalmological surgery in developing countries is feasible and can yield extraordinary benefits for individual patients. PMID:24403902

Schönfeld, Carl-Ludwig; Hennig, Albrecht

2013-01-01

66

Anatomical characteristics and surgical treatment of bilateral congenital upper eyelid entropion in an infant with neonatal progeroid syndrome.  

PubMed

Neonatal progeroid syndrome (NPS) is a rare, autosomal recessive disorder characterized by an aged face, wrinkled skin, decreased subcutaneous fat, and neonatal teeth. The authors describe herein a 2-month-old infant with NPS who underwent eyelid surgery for bilateral upper eyelid entropion and stromal keratitis. The preoperative examination revealed thin, narrow, and soft tarsal plates. The eyelids were large and floppy, and there was no preaponeurotic fat tissue. The entropion was successfully treated by the levator muscle advancing onto the tarsus, anterior lamellar repositioning, horizontal eyelid shortening, and canthal resuspension. Upper eyelid entropion may occur relatively frequently in infants with NPS, and the special anatomical features of the eyelid should be considered while performing entropion surgery. PMID:24807800

Yaz?c?, Bülent; Toka, Fatih; Çömez, Arzu T

2014-01-01

67

Bone-anchored maxillary expansion and bilateral interoral mandibular distraction osteogenesis in adult with severe obstructive sleep apnea syndrome.  

PubMed

Severe obstructive sleep apnea syndrome (OSAS) threatens patients' lives. To solve ventilation problem, snoring, and avoid another orthognathic surgery for mandibular advancement, bone-anchored rapid maxillary expansion and bilateral interoral mandibular distraction osteogenesis were tried on a 20-year-old Chinese male patient with severe skeletal class II malocclusion and OSAS.The patient had polysomnography (apnea-hypopnea index 54.2), body mass index measurement (19.7 kg/m), and cephalometry before the treatment. Bone-anchored rapid maxillary expansion was performed for the correction of maxillary transverse and minor sagittal deficiency and the improvement of nasal airflow by decreasing nasal resistance. Bilateral interoral mandibular distraction osteogenesis was operated to lengthen the small, retruded mandible by 15 mm. Orthodontic treatment after the maxillary expansion and mandibular distraction osteogenesis can help obtain stable occlusion.The Epworth Sleepiness Scale, a questionnaire for temporomandibular joint, cephalometric analysis, polysomnography, acoustic rhinometry, and multislice spiral computed tomography were performed to evaluate changes from the treatment. All the results showed that the patient had a significantly alleviated OSAS. In addition, an acceptable occlusion was also obtained. PMID:23714918

Nie, Ping; Zhu, Min; Lu, Xiao-Feng; Fang, Bing

2013-05-01

68

Neurophysiological evidence for generalized sensory neuronopathy in cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS).  

PubMed

Introduction: Cerebellar Ataxia with Neuropathy and bilateral Vestibular Areflexia Syndrome (CANVAS) is a recently described multi-system ataxia defined by the presence of cerebellar ataxia, bilateral vestibulopathy, and a somatic sensory deficit. The characteristic clinical sign is an abnormal visually enhanced vestibulo-ocular reflex. The somatic sensory deficit contributes to a significant level of disability in CANVAS. Methods & results: Neurophysiological investigation in 14 patients revealed uniformly absent sensory nerve action potentials in all limbs, abnormal blink reflexes in 13/14, and abnormal masseter reflexes in 6/11. Tibial H-reflexes were absent in 11/14. Somatosensory evoked potentials were abnormal in 10/11 tested, and brainstem audiometry evoked responses were abnormal in 3/8. Cutaneous silent period responses were abnormal in 7/14. Discussion: We suggest that a sensory neuronopathy should be sought in cerebellar and/or vestibular ataxias, particularly where the degree of ataxia is out of proportion to the clinically identified cerebellar and/or vestibular dysfunction. © 2014 Wiley Periodicals, Inc. PMID:25130975

Szmulewicz, David J; Seiderer, Linda; Halmagyi, G Michael; Storey, Elsdon; Roberts, Leslie

2014-08-16

69

Bilateral reactive lymphoid hyperplasia of the orbit in a child with Russell-Silver syndrome.  

PubMed

Russell-Silver syndrome (RSS) primarily includes intrauterine growth retardation, postnatal growth failure, body asymmetry, and craniofacial disproportion. Four types of malignancies have been reported in patients with RSS: craniopharyngioma, testicular seminoma, hepatocellular carcinoma, and Wilms tumor. PMID:15616510

Dang, Yen N T; Shin, In C; Gordon, Robert A; Karcioglu, Zeynel A

2004-12-01

70

Horner's syndrome associated with a mandibular symphyseal fracture and bilateral temporomandibular luxation.  

PubMed

Interruption of the postganglionic neuron in the sympathetic pathway to the eye is reported to be a common cause of Horner's syndrome in the cat, although there are few clinical reports identifying the site and nature of the lesion responsible. A case of Horner's syndrome resulting from trauma to the periorbital structures following reduction of temporomandibular joint luxation is presented. In this case, the ophthalmic abnormalities resolved spontaneously over eight days. PMID:11791777

Baines, S J; Langley-Hobbs, S

2001-12-01

71

Substance-P-induced protein extravasation is bilaterally increased in complex regional pain syndrome  

Microsoft Academic Search

Pain, mechanical hyperalgesia, edema, increased skin temperature, and skin reddening are characteristic symptoms of acute complex regional pain syndrome (CRPS). We have recently demonstrated facilitated neurogenic inflammation on the affected limb. To further elucidate the underlying mechanisms, exogenous substance P (SP) in ascending concentrations (10?9, 10?8, 10?7, 10?6 M) was intradermally applied to the affected and the unaffected limbs, respectively,

Stefan Leis; Margarete Weber; Andreas Isselmann; Martin Schmelz; Frank Birklein

2003-01-01

72

Fibrolipomatous hamartoma of the median nerve: A cause of acute bilateral carpal tunnel syndrome in a three-year-old child: A case report and comprehensive literature review.  

PubMed

A three-year-old boy was investigated for inexplicable incessant crying. On examination, his left wrist was mildly swollen (three to four months) and sensitive. Exploration and carpal tunnel decompression of the left wrist with incisional biopsy was performed for the presence of a fusiform swelling intimately associated with the median nerve. Histopathology revealed the presence of enlarged nerve bundles admixed with mature fat cells and diffuse fibroblastic proliferation. Three months later, he underwent urgent contralateral carpal tunnel decompression for a similar presentation. The final diagnosis was bilateral fibrolipomatous hamartoma (FLH) of the median nerves causing acute bilateral compression neuropathy. FLH of the median nerve is an extremely unusual cause of acute bilateral carpal tunnel syndrome in a young child presenting with 'incessant crying'. A comprehensive review of FLH including epidemiology, etiology, clinical presentation, differential diagnosis, imaging, pathology, treatment and prognosis is discussed. PMID:25332651

Senger, Jenna-Lynn; Classen, Dale; Bruce, Garth; Kanthan, Rani

2014-01-01

73

POST?SURGICAL REHABILITATION FOLLOWING FASCIOTOMIES FOR BILATERAL CHRONIC EXERTIONAL COMPARTMENT SYNDROME IN A SPECIAL FORCES SOLDIER: A CASE REPORT  

PubMed Central

Background and Purpose: The etiology of Chronic Exertional Compartment Syndrome (CECS) is still unclear. The most commonly accepted theory suggests that it is a transient but debilitating process where there is an abnormally increased intracompartmental pressure during exercise/exertion due to non?compliant expansion of the osteofascial tissues. This most commonly occurs in the lower leg. Surgical intervention is often performed for symptom relief. However, there has been limited scientifically?based publication on post?surgical rehabilitation, especially with regard to return to function in the military population. The purpose of this case report is to demonstrate the utilization of a recommended post?operative protocol in a Special Forces Soldier. Case Description: The subject presented as a 25?year?old US Army Special Forces Soldier, who failed 8 weeks of conservative management for the diagnosis of CECS and subsequently underwent bilateral lower leg fasciotomies of the anterior and lateral compartments. Outcomes: Following recommended protocol guidelines he was progressed rapidly and within three months deployed without restriction or complications in a demanding combat zone. Discussion: This case report illustrates that following clearly defined, scientifically?based rehabilitation guidelines helped in addressing all of the involved structures and musculoskeletal dysfunctions that presented following the surgical intervention for CECS in a unique subject. Level of Evidence: 5 PMID:24175149

Miller, Joseph

2013-01-01

74

Surgical-orthodontic management of bilateral multiple impactions in non-syndromic patient.  

PubMed

Several surgical and orthodontic treatment options are available to disimpact the impacted teeth. But the closed eruption technique has the best long-term prognosis. The tooth is surgically exposed, an attachment is bonded to it, flap is resutured over it and an orthodontic extrusive force is delivered to bring the tooth into occlusion. This case report presents a case with multiple impacted teeth in which no syndrome or systemic conditions were detected. A 20-year-old female patient reported for orthodontic treatment with chief complaint of multiple unerupted permanent teeth and retained primary teeth. Radiographic examination revealed impacted 14, 15, 24, 25, 33, 34, 43 and 44. Surgical exposure of the impacted teeth was done after extraction of retained primary teeth. Forced eruption of these teeth was done by applying traction with closed eruption technique. After careful treatment planning followed by guided eruption of impacted teeth, patient finished with a significantly improved functional and aesthetic result. PMID:23329714

Agrawal, Jiwanasha Manish; Agrawal, Manish Suresh; Nanjannawar, Lalita Girish

2013-01-01

75

Surgical-orthodontic management of bilateral multiple impactions in non-syndromic patient  

PubMed Central

Several surgical and orthodontic treatment options are available to disimpact the impacted teeth. But the closed eruption technique has the best long-term prognosis. The tooth is surgically exposed, an attachment is bonded to it, flap is resutured over it and an orthodontic extrusive force is delivered to bring the tooth into occlusion. This case report presents a case with multiple impacted teeth in which no syndrome or systemic conditions were detected. A 20-year-old female patient reported for orthodontic treatment with chief complaint of multiple unerupted permanent teeth and retained primary teeth. Radiographic examination revealed impacted 14, 15, 24, 25, 33, 34, 43 and 44. Surgical exposure of the impacted teeth was done after extraction of retained primary teeth. Forced eruption of these teeth was done by applying traction with closed eruption technique. After careful treatment planning followed by guided eruption of impacted teeth, patient finished with a significantly improved functional and aesthetic result. PMID:23329714

Agrawal, Jiwanasha Manish; Agrawal, Manish Suresh; Nanjannawar, Lalita Girish

2013-01-01

76

Long-term outcome study of bilateral mandibular distraction: a comparison of Treacher Collins and Nager syndromes to other types of micrognathia.  

PubMed

A long-term follow-up study of patients who underwent bilateral mandibular distraction is presented, and the results of patients with Treacher Collins syndrome and Nager syndrome are compared with results for other forms of congenital micrognathia. It was hypothesized that the factors responsible for the predetermined, syndrome-specific shape of the mandible in patients with Treacher Collins and Nager syndromes would alter the long-term results of linear (uniplanar) distraction of the mandible. Thus, over time, the mandibles would remodel to preoperative form while maintaining the increase in volume. To investigate this hypothesis, all patients treated with bilateral mandibular distraction who had at least 1.5 years of follow-up, including satisfactory cephalometric examinations, were retrospectively reviewed. Two groups were identified. Group 1 (n = 6) were Treacher Collins and Nager syndrome patients (ages, 2 to 13 years; mean, 5.2 years) and group 2 (n = 6) included other forms of bilateral, congenital micrognathia (ages, 1.5 to 19 years; mean, 8.4 years). Serial cephalometric measurements were recorded before distraction, after distraction, and at least 18 months after distraction. Mandibular mean linear distraction distance (as recorded on the device) averaged 24.5 mm in group 1 and 26.2 mm in group 2. In group 1, the antegonial angle (angle from the mandibular plane to the top of the antegonial notch) decreased after distraction by 3.8 degrees, and the antegonial notch height was reduced by 1.6 mm. The posttreatment morphologic change was modified significantly over time, with a 3.7-degree increase of the antegonial angle and a 1.2-mm deepening of the antegonial notch. In group 2, the immediate reduction in height of the antegonial notching was subtler; however, long-term recurrence of the antegonial notching was also observed. At the end of distraction, the mean group 1 gonial angle became 8 degrees more obtuse. In contrast, patients in group 2 developed a more acute angle (mean, 8 degrees). The mandibles of the Treacher Collins syndrome patients (group 1) maintained their more obtuse postdistraction gonial angle during the period of follow-up, whereas over time this change was reversed in group 2 patients. In conclusion, experience with bilateral mandibular distraction has demonstrated that long-term determination of mandibular form is more complex than either the amount of distraction or the direction of the distraction vector. The underlying genotype and the musculoskeletal milieu must be taken into account when planning distraction, as these factors tend to remodel the mandible into its preoperative shape over time, despite the fact that the increased mandibular volume and projection are maintained. PMID:11994578

Stelnicki, Eric J; Lin, Wen-Yuan; Lee, Catherine; Grayson, Barry H; McCarthy, Joseph G

2002-05-01

77

Multiple bilateral supernumerary mandibular premolars in a non-syndromic patient with associated orthokeratised odontogenic cyst- A case report and review of literature  

PubMed Central

Multiple supernumerary teeth are very rare, accounting for less than 1% of cases. They are commonly associated with syndromes like Gardner's syndrome and cleidocranial dysostosis and cleft lip and palate. Non-syndromic multiple supernumerary teeth have a predilection to occur in the mandibular premolar region. Orthokeratinized odontogenic cyst (OOC) is a relatively uncommon developmental cyst comprising about 10% of the cases that had been previously implied as odontogenic keratocysts. More than half of the cases of OOC are associated with impacted tooth; but not a single case of OOC associated with supernumerary teeth is reported. Hence, the purpose of this article is to report the first case of multiple supernumerary mandibular premolars associated with OOC in a 35-year-old male and to review the literature associated with multiple bilateral supernumerary mandibular premolars. PMID:23230374

Kasat, Vikrant O.; Saluja, Harish; Kalburge, Jitendra V.; Kini, Yogesh; Nikam, Atul; Laddha, Ruchi

2012-01-01

78

Adrenal Cushing's syndrome due to bilateral macronodular adrenal hyperplasia: prediction of the efficacy of beta-blockade therapy and interest of unilateral adrenalectomy.  

PubMed

Bilateral adrenalectomy is the standard treatment for Cushing's syndrome (CS) related to ACTH-independent bilateral macronodular hyperplasia (AIMAH), although it imposes life-long adrenal insufficiency. This study reports a clinical case in order to discuss the clinical interest of pharmacological beta-blockade of illegitimate membrane receptors and unilateral adrenalectomy as alternatives to bilateral adrenalectomy for treatment of CS due to AIMAH. Evidence for cortisol stimulation by upright posture and insulin-induced hypoglycemia in a patient with CS related to AIMAH led us to try beta-blockers as a therapeutic test and then as a first line treatment. Thus, a 3-day beta-blocker test (320 mg/d propranolol) induced normalization of cortisol secretion, with return of hypercortisolism at the end of the test. A long term treatment with 320 mg/d propranolol allowed sustained normalization of cortisol secretion and progressive disappearance of Cushingoid features but after 8 months the patient complained of Raynaud's syndrome and fatigue. Lowering propranolol dosage or switching to atenolol was less efficient to reduce cortisol levels. Unilateral adrenalectomy was then performed as a second line treatment, leading to normalisation of the 24h urinary cortisol without adrenal insufficiency. Long term control of blood pressure and glycemia were observed during a 7-year follow-up without beta-blocker. In conclusion, a 3-day propranolol test may identify patients with AIMAH who can benefit from a long term beta-blocker treatment. In case of intolerance to beta-blocking agents, unilateral adrenalectomy may allow for long term control of Cushing's syndrome related to AIMAH without adrenal insufficiency. PMID:19564707

Mazzuco, Tânia L; Chaffanjon, Philippe; Martinie, Monique; Sturm, Nathalie; Chabre, Olivier

2009-01-01

79

Pulmonary artery rupture after bilateral pulmonary artery banding in a neonate with Loeys-Dietz syndrome and an interrupted aortic arch complex: report of a case.  

PubMed

Loeys-Dietz syndrome (LDS) is a recognized connective tissue disorder characterized by progressive aortic aneurysm and dissection. Patients are at high risk of aortic dissection or rupture at an early age, but to our knowledge, surgery on the great arteries has never been attempted in the neonatal period. We report a case of LDS with dilated pulmonary arteries and an interrupted aortic arch complex in a neonate. We performed bilateral pulmonary artery banding, but 12 days after the procedure, the infant died of rupture of the distal portion of the banding sites following massive dilatation. PMID:24817126

Ozawa, Hideto; Kawata, Hiroaki; Iwai, Shigemitsu; Yamauchi, Sanae; Kanaya, Tomomitsu; Kishimoto, Hidefumi

2014-05-11

80

Non-surgical therapy for bilateral glossopharyngeal neuralgia caused by Eagle's syndrome, diagnosed by three-dimensional computed tomography: a case report.  

PubMed

Eagle's syndrome is an uncommon sequela of elongation of the styloid process. Symptoms include recurrent throat pain and anterolateral neck pain, with referred pain to the ear. We report a 65-year-old man who presented with bilateral glossopharyngeal neuralgia. We performed three-dimensional computed tomography which revealed that the right styloid process was 35.1 mm and the left process was 29.6 mm, leading to diagnosis of an elongated styloid process, i.e. Eagle's syndrome. Because the patient refused surgical treatment, conservative therapy was selected. Oral gabapentin, stellate ganglion block, and 8 % lidocaine spray on the tonsillar branches of the glossopharyngeal nerve resulted in complete resolution of the paroxysms of pain in approximately 3 weeks. PMID:22760524

Kawasaki, Masanori; Hatashima, Sunao; Matsuda, Tomio

2012-12-01

81

The Relationship between Perisylvian Morphology and Verbal Short-Term Memory Functioning in Children with Neurodevelopmental Disorders  

ERIC Educational Resources Information Center

Although children with neurodevelopmental disorders frequently present with reduced short-term memory functioning, the relationship between perisylvian morphology and verbal short-term memory functioning has received limited attention. Thus, examining this relationship in children with neurodevelopmental disorders was the focus of this exploratory…

Kibby, Michelle Y.; Kroese, Judith M.; Morgan, Allison E.; Hiemenz, Jennifer R.; Cohen, Morris J.; Hynd, George W.

2004-01-01

82

Auditory verbal hallucinations and brain dysconnectivity in the perisylvian language network: a multimodal investigation.  

PubMed

Neuroimaging studies of schizophrenia have indicated that the development of auditory verbal hallucinations (AVHs) is associated with altered structural and functional connectivity within the perisylvian language network. However, these studies focussed mainly on either structural or functional alterations in patients with chronic schizophrenia. Therefore, they were unable to examine the relationship between the 2 types of measures and could not establish whether the observed alterations would be expressed in the early stage of the illness. We used diffusion tensor imaging and functional magnetic resonance imaging to examine white matter integrity and functional connectivity within the left perisylvian language network of 46 individuals with an at risk mental state for psychosis or a first episode of the illness, including 28 who had developed AVH group and 18 who had not (nonauditory verbal hallucination [nAVH] group), and 22 healthy controls. Inferences were made at P < .05 (corrected). The nAVH group relative to healthy controls showed a reduction of both white matter integrity and functional connectivity as well as a disruption of the normal structure-function relationship along the fronto-temporal pathway. For all measures, the AVH group showed intermediate values between healthy controls and the nAVH group. These findings seem to suggest that, in the early stage of the disorder, a significant impairment of fronto-temporal connectivity is evident in patients who do not experience AVHs. This is consistent with the hypothesis that, whilst mild disruption of connectivity might still enable the emergence of AVHs, more severe alterations may prevent the occurrence of the hallucinatory experience. PMID:24361862

Benetti, Stefania; Pettersson-Yeo, William; Allen, Paul; Catani, Marco; Williams, Steven; Barsaglini, Alessio; Kambeitz-Ilankovic, Lana M; McGuire, Philip; Mechelli, Andrea

2015-01-01

83

Auditory Verbal Hallucinations and Brain Dysconnectivity in the Perisylvian Language Network: A Multimodal Investigation  

PubMed Central

Neuroimaging studies of schizophrenia have indicated that the development of auditory verbal hallucinations (AVHs) is associated with altered structural and functional connectivity within the perisylvian language network. However, these studies focussed mainly on either structural or functional alterations in patients with chronic schizophrenia. Therefore, they were unable to examine the relationship between the 2 types of measures and could not establish whether the observed alterations would be expressed in the early stage of the illness. We used diffusion tensor imaging and functional magnetic resonance imaging to examine white matter integrity and functional connectivity within the left perisylvian language network of 46 individuals with an at risk mental state for psychosis or a first episode of the illness, including 28 who had developed AVH group and 18 who had not (nonauditory verbal hallucination [nAVH] group), and 22 healthy controls. Inferences were made at P < .05 (corrected). The nAVH group relative to healthy controls showed a reduction of both white matter integrity and functional connectivity as well as a disruption of the normal structure?function relationship along the fronto-temporal pathway. For all measures, the AVH group showed intermediate values between healthy controls and the nAVH group. These findings seem to suggest that, in the early stage of the disorder, a significant impairment of fronto-temporal connectivity is evident in patients who do not experience AVHs. This is consistent with the hypothesis that, whilst mild disruption of connectivity might still enable the emergence of AVHs, more severe alterations may prevent the occurrence of the hallucinatory experience. PMID:24361862

Pettersson-Yeo, William; Allen, Paul; Catani, Marco; Williams, Steven; Barsaglini, Alessio; Kambeitz-Ilankovic, Lana M.; McGuire, Philip; Mechelli, Andrea

2015-01-01

84

Bilateral Optic Neuropathy with Bilateral Putaminal Lesions: A Case Report.  

PubMed

Bilateral optic neuropathy with bilateral putaminal lesions may be caused by methanol or cyanide poisoning or mitochondrial disorders including Leber hereditary optic neuropathy and Leigh syndrome. We report the case of a 34-year-old Japanese man who developed bilateral visual loss 5 days after the development of gastrointestinal symptoms. Magnetic resonance imaging of the brain on admission revealed high-intensity signal areas in the bilateral putamina on diffusion-weighted and T2-weighted images as well as a high-intensity signal area in the left middle cerebellar peduncle that had been identified 3 years previously. We diagnosed bilateral optic neuropathy with bilateral putaminal lesions caused by preceding infection-triggered demyelination. We administered methylprednisolone, but his vision did not recover. PMID:25052423

Togawa, Jumpei; Ohi, Takekazu

2014-07-23

85

A Rare Interstitial Duplication of 8q22.1–8q24.3 Associated with Syndromic Bilateral Cleft Lip/Palate  

PubMed Central

We present a rare case of 8q interstitial duplication derived from maternal balanced translocations in a patient with bilateral cleft lip and palate in syndromic form associated with other congenital malformations. G-banding cytogenetic analysis revealed a chromosomal abnormality in the form of the karyotype 46,XX der(22)t(8;22)(q22.1;p11.1)mat. Chromosome microarray analysis evidenced a 49?Mb duplicated segment of chromosome 8q with no pathogenic imbalances on chromosome 22. Two siblings also carry the balanced translocation. We have compared this case with other “pure” trisomies of 8q patients reported in the literature and with genome wide association studies recently published. This work highlights the involvement of chromosome 8q in orofacial clefts. PMID:25506438

Rezek, Regina Ferreira; Rodrigues Abbas, Ana Angélica; Forte Mazzeu, Juliana; Duarte Miranda, Siliana Maria; Velloso-Rodrigues, Cibele

2014-01-01

86

[Successful treatment of massive thrombosis of the vena cava inferior with nephrotic syndrome and chronic bilateral pulmonary artery thromboembolism in a patient with genetic thrombophilia].  

PubMed

A case is reported of a 23-year-old male patient who developed, after severe blunt injury of the lumbar region, massive thrombosis of the vena cava inferior (VCI), both renal veins, bilateral pulmonary artery thromboembolism (PATE), nephrotic syndrome (NS). In spite of anticoagulant therapy, the condition of the patient progressively aggravated for 1.5 year: thrombosis involved the ileac and femoral arteries on the right, thrombus floated in the right atrium with PATE recurrent episodes, pulmonary hypertension reached 120 mm Hg with formation of decompensated cor pulmnonale, proteinuria and hypoalbuminemia deteriorated, anasarca edema developed Multigenic thrombophilia was diagnosed (1 homozygous and 5 heterozygous mutations). A radical one-stage operation was successful: thromboectomy from the VCI, right ileac and left renal veins, thrombendarterectomy from the pulmonary arteries, suture of the interatrial septum defect, installation of cava-filter After the operation pulmonary pressure lowered to 40-45 mm Hg, right heart volume normalized, immunosuppressive therapy with prednisolone and cyclosporine led to nephropathy remission. The discussion covers mechanisms and factors (including genetic) of thrombosis progression, correlations between intravascular thrombosis, NS and chronic glomerulonephritis (possible NS development due to bilateral thrombosis of the renal veins and nephropathy role in thrombosis progression), approaches to conservative and surgical treatment of such patients. Global experience in conduction of pulmonary thrombendarterectomy and thrombectomy from VCI is reviewed (one-stage operations were not described earlier). PMID:22616531

Blagova, O V; Dzemeshkevich, S L; Kozlovskaia, N L; Nedostup, A V; Sarkisova, N D; Frolova, Iu V; Raskin, V V; Dzemeshkevich, A S; Abugov, S A; Skipenko, O G; Shilov, E M; Sedov, V P; Gagarina, N V; Sinitsyn, V E; Mershina, E A; Volkova, E Iu

2012-01-01

87

Urine alkalinization may be enough for the treatment of bilateral renal pelvis stones associated with Lesch-Nyhan syndrome.  

PubMed

Lesch-Nyhan syndrome is a rare sex-linked disorder of purine metabolism that is caused by a mutation in the hypoxanthine-guanine phosphoribosyltransferase (HPRT) gene which causes marked hyperuricemia and hyperuricosuria, with signs of gouty arthritis and uric acid stone disease in early childhood. We report a case of renal pelvis calculi which was dissolved within 10 days of urine alkalinization and hydration. PMID:21331772

Oh, Mi Mi; Ham, Byeong Kuk; Kang, Seok Ho; Bae, Jae Hyun; Kim, Je Jong; Yoo, Ki Hwan; Yoon, Duck Ki; Moon, Du Geon

2011-10-01

88

Complete Bilateral Ophthalmoplegia with Unilateral Facial Palsy in a Child with Anti-GQ1b Syndrome.  

PubMed

The classical phenotype of Miller Fisher syndrome is characterized by ophthalmoplegia, ataxia and areflexia. However, less extensive forms have been described. The authors report a 14-y-old boy with positive anti-GQ1b antibodies with unusual clinical findings. He presented with headache, double vision and vomiting for 7 d. Examination revealed complete opththalmoplegia, right lower-motor-neuron facial palsy, no limb weakness or cerebellar signs and normal fundus. CSF examination and MRI brain were normal. Electrophysiological studies showed normal limb nerve conduction studies, low CMAP amplitude of right facial nerve, abnormal blink reflex and negative repetitive-nerve-stimulation test. Anti-GQ1b antibodies were positive. The child was managed conservatively with gradual complete recovery. The patients with positive anti-GQ1b antibodies who do not demonstrate the full complement of the Miller Fisher syndrome triad have been reported previously. However, unilateral facial palsy has not been reported previously. This report further expands the phenotypic spectrum of anti-GQ1b syndrome. PMID:24997143

Kauser, Hina; Jain, Puneet; Sharma, Suvasini; Aneja, Satinder

2015-02-01

89

Bilateral lung 99mTc-MDP uptake on the bone scintigraphy in the myelodysplastic syndromes (MDS).  

PubMed

We report a case of myelodysplastic syndrome (MDS) with unusual abnormal 99mTc-MDP activity throughout both lungs on whole-body bone scan. To explain the pancytopenia, bone marrow examination was carried out which showed hypocellularity in addition to large abnormal megakaryocytes indicating myelodysplastic changes. His whole-body bone scan showed increased 99mTc-MDP activity in both lungs, kidneys, and also along the proximal two thirds of the femora. It was concluded that lung uptake in addition to skeletal uptake on scintigraphic bone scanning should be kept in mind in patients with MDS. PMID:23531771

Mogharrabi, Mehdi; Javadi, Hamid; Assadi, Majid

2013-05-01

90

Bilateral ankle edema with bilateral iritis  

Microsoft Academic Search

I report two patient presented to me with bilateral symmetrical ankle edema and bilateral acute iritis. A 42-year-old female\\u000a of Indian origin and 30-year-old female from Somalia both presented with bilateral acute iritis. In the first patient, bilateral\\u000a ankle edema preceded the onset of bilateral acute iritis. Bilateral ankle edema developed during the course of disease after\\u000a onset of ocular

Sunil Kumar

2007-01-01

91

Bilateral ankle pain secondary to sarcoidosis.  

PubMed

Sarcoidosis has been described as a multisystem granulomatous disorder of unknown etiology that usually affects young adults. It can affect the foot and ankle. When erythema nodosum is present, the sarcoidosis usually is characterized by bilateral hilar lymphadenopathy, uveitis, fever, arthralgia, or arthritis. This symptom complex is referred to as Löfgren's syndrome. The author describes a patient who presented with bilateral medial ankle pain secondary to sarcoidosis. PMID:2724109

Julsrud, M

1989-03-01

92

Bilateral ankle edema with bilateral iritis.  

PubMed

I report two patient presented to me with bilateral symmetrical ankle edema and bilateral acute iritis. A 42-year-old female of Indian origin and 30-year-old female from Somalia both presented with bilateral acute iritis. In the first patient, bilateral ankle edema preceded the onset of bilateral acute iritis. Bilateral ankle edema developed during the course of disease after onset of ocular symptoms in the second patient. Both patients did not suffer any significant ocular problem in the past, and on systemic examination, all clinical parameters were within normal limit. Lacrimal gland and conjunctival nodule biopsy established the final diagnosis of sarcoidosis in both cases, although the chest x-rays were normal. PMID:16633708

Kumar, Sunil

2007-07-01

93

Somato-Motor Haptic Processing in Posterior Inner Perisylvian Region (SII/pIC) of the Macaque Monkey  

PubMed Central

The posterior inner perisylvian region including the secondary somatosensory cortex (area SII) and the adjacent region of posterior insular cortex (pIC) has been implicated in haptic processing by integrating somato-motor information during hand-manipulation, both in humans and in non-human primates. However, motor-related properties during hand-manipulation are still largely unknown. To investigate a motor-related activity in the hand region of SII/pIC, two macaque monkeys were trained to perform a hand-manipulation task, requiring 3 different grip types (precision grip, finger exploration, side grip) both in light and in dark conditions. Our results showed that 70% (n?=?33/48) of task related neurons within SII/pIC were only activated during monkeys’ active hand-manipulation. Of those 33 neurons, 15 (45%) began to discharge before hand-target contact, while the remaining neurons were tonically active after contact. Thirty-percent (n?=?15/48) of studied neurons responded to both passive somatosensory stimulation and to the motor task. A consistent percentage of task-related neurons in SII/pIC was selectively activated during finger exploration (FE) and precision grasping (PG) execution, suggesting they play a pivotal role in control skilled finger movements. Furthermore, hand-manipulation-related neurons also responded when visual feedback was absent in the dark. Altogether, our results suggest that somato-motor neurons in SII/pIC likely contribute to haptic processing from the initial to the final phase of grasping and object manipulation. Such motor-related activity could also provide the somato-motor binding principle enabling the translation of diachronic somatosensory inputs into a coherent image of the explored object. PMID:23936121

Ishida, Hiroaki; Fornia, Luca; Grandi, Laura Clara; Umiltà, Maria Alessandra; Gallese, Vittorio

2013-01-01

94

Bilateral congenital cataract with suspected lens-induced granulomatous uveitis.  

PubMed

A healthy newborn baby girl presented with congenital bilateral cataract. Within a few days of presenting she also developed bilateral granulomatous uveitis, a condition generally linked in newborns to congenital infections, most frequently TORCHES syndrome (toxoplasmosis, rubella, cytomegalic inclusion disease, herpesvirus, including Epstein-Barr, syphilis). Extensive investigation did not reveal any underlying etiologic mechanism. Treatment with topical and systemic steroids did not improve the uveitis. However, bilateral lens extraction resulted in a quick resolution of the uveitis. PMID:25262559

Vandenbroucke, Stéphanie; Foets, Beatrijs; Wouters, Carine; Casteels, Ingele

2014-10-01

95

Bilateral Mandibular Supernumerary Canines: A Case Report  

PubMed Central

Supernumerary teeth are defined as the teeth developed in excess of the number found in a normal dentition. Supernumerary canine is an extremely rare finding particularly in the mandible. This case report presents a 25-year-old female patient with the unique feature of bilateral mandibular supplemental supernumerary canines. The patient was non-syndromic without any other supernumerary teeth. PMID:23346342

Abouei Mehrizi, Ehsan; Semyari, Hassan; Eslami Amirabadi, Gholamreza

2010-01-01

96

Bilateral Molariform Mandibular Second Premolars  

PubMed Central

Macrodontia is a rare dental anomaly that refers to teeth that appear larger than normal. Generalised macrodontia can be associated with certain medical conditions and syndromes. This case report presents clinical and radiographic findings of isolated bilateral macrodontia in a 14-year-old child. The patient was referred to the clinic with local crowding of maxillary and mandibular teeth. Radiographic findings revealed the presence of impacted macrodont mandibular second premolar on one side and erupted macrodontic premolar on the other side and their distinct morphological appearance, characterized by large, multitubercular, and molariform crowns and tapering, single roots.

Acharya, Sonu; Kumar Mandal, Pradip; Ghosh, Chiranjit

2015-01-01

97

Plasma ACTH responses to cortisol infusion are similar in patients with primary hypoadrenalism and patients studied some years after bilateral adrenalectomy for Cushing's syndrome.  

PubMed

It has been suggested that the first-phase feedback of steroid feedback on ACTH secretion is abnormal in Cushing's disease patients studied after adrenalectomies. We have performed hydrocortisone infusions (100 mg over 2 h) in 15 patients who had previously undergone bilateral adrenalectomy for Cushing's disease. None had had any pituitary-directed therapy and none had a pituitary macroadenoma. Their responses were compared with a group of seven hypoadrenal patients. A significant rise in serum cortisol was observed between 5 and 10 min in both groups. The first significant fall in ACTH occurred between 30 and 45 min in both groups. There was no abnormality of first-phase feedback in the Cushing's group and our results do not therefore support previous speculation that such an abnormality provides evidence for a higher central nervous system (CNS) aetiology of Cushing's disease. PMID:2557986

Atkinson, A B; Beacom, R; Kennedy, A L; Hadden, D R; Sheridan, B

1989-05-01

98

Bilateral taurodontism in primary dentition with hypodontia  

PubMed Central

Taurodontism is a rare dental anomaly in which there is an enlarged pulp chamber at the expense of roots with apical displacement of the pulpal floor, giving it a rectangular shape. It is caused by the failure of Hertwig's epithelial root sheath to invaginate at the proper horizontal level. Taurodontism has been reported as an intraoral feature of several syndromes like Down syndrome, Klinefelter syndrome, Smith-Magenis syndrome, Hurler syndrome, etc. Association of taurodontism with hypodontia in permanent dentition has also been reported. Taurodontism in primary dentition and its association with hypodontia is very rarely reported in the literature. The present case illustrates bilateral taurodontism of primary mandibular molars with hypodontia in maxilla. PMID:23345504

Surendar, Marappan Natarajan; Pandey, Ramesh Kumar; Khanna, Richa

2013-01-01

99

Neuroimaging Aspects of Aicardi Syndrome  

PubMed Central

Aicardi syndrome is a rare neurodevelopmental disorder characterized by congenital chorioretinal lacunae, corpus callosum dysgenesis, seizures, polymicrogyria, cerebral heterotopias, intracranial cysts, and costovertebral defects. Cerebellar abnormalities have been described occasionally. Aicardi syndrome is sporadic and has been observed only in females and 47,XXY males. Therefore, it is thought to result from a mutation in an X-linked gene. Improved definition of the clinical phenotype should focus the selection of functional candidate genes for mutation analysis. Because central nervous system abnormalities are the most prominent component of the phenotype, we performed a detailed characterization of abnormalities identified on magnetic resonance neuroimaging studies from 23 girls with Aicardi syndrome, the largest cohort to undergo such review by a single group of investigators. All patients had polymicrogyria that was predominantly frontal and perisylvian and often associated with underopercularization. Periventricular nodular heterotopias, present in all patients, were more frequent than previously reported; 10 had single and 11 had multiple intracranial cysts. Posterior fossa abnormalities were also more frequent than previously described. Cerebellar abnormalities were noted in 95% of studies where they could be evaluated. As a novel finding, we noted tectal enlargement in 10 patients. Since mildly-affected girls with variable callosal dysgenesis have now been reported, the constellation of frontal-dominant and perisylvian polymicrogyria, periventricular nodular heterotopias, intracranial cysts, and posterior fossa abnormalities, including tectal enlargement, should prompt consideration of the diagnosis of Aicardi syndrome. We further propose that improved characterization of the neurological phenotype will benefit the selection of candidate genes for mutation analysis. PMID:18925666

Hopkins, Bobbi; Sutton, V. Reid; Lewis, Richard Alan; Van den Veyver, Ignatia; Clark, Gary

2008-01-01

100

Bilateral Ovarian Fibrothecoma Associated with Ascites, Bilateral Pleural Effusion, and Marked Elevated Serum CA-125  

PubMed Central

Background. The risk of ovarian cancer is increased in the association of ovarian tumor, ascites, and hydrothorax with the significant elevated tumor marker CA-125. However, this association can be observed in a rare clinical and benign pathological entity, that is Demons-Meigs' syndrome. Objective. To describe a rare case of Demons-Meigs' syndrome observed in our department. Methods. A black African woman of 35 years old, seventh gravida and fourth parous, underwent a total abdominal hysterectomy with bilateral salpingoophorectomy for large bilateral ovarian masses associated with significant ascites, bilateral pleural effusion, and particular highly elevated tumor marker CA-125 (1835?UI/mL) in a pronounced general alteration condition. Results. The postoperative course was uneventful characterized by a complete remission of hydrothorax and ascites with normal level of CA-125 three months after tumor excision. Histology of both masses revealed a bilateral ovarian fibrothecoma, a benign tumor of the ovary, thus confirming the diagnosis of Demons-Meigs' syndrome. Conclusion. The Demons-Meigs syndrome, although it strongly mimics the clinical picture of malignant metastatic ovarian cancer, remains a disease with benign prognosis after surgical tumor resection. This is a rare condition that must be known and recognized by practitioners to avoid unnecessary practices. PMID:23431489

Loué, Védi André Serges; Gbary, Eléonore; Koui, Sylvanus; Akpa, Bédi; Kouassi, Adélaide

2013-01-01

101

Bilateral assymetric epidural hematoma  

PubMed Central

Background: Acute bilateral extradural hematoma is a rare presentation of head trauma injury. In sporadic cases, they represent 0.5–10% of all extradural hematomas. However, higher mortality rates have been reported in previous series. Case Description: The authors described the case of a 28-year-old male presenting head injury, comatose, Glasgow Coma Scale of 6, anisocoric pupils without puppilary light reflex. Computed tomography showed asymmetric bilateral epidural hematomas, effacement of the lateral ventricles and sulci, midline shift and a bilateral skull fracture reaching the vertex. Surgical evacuation was performed with simultaneous hematoma drainage. Patient was discharged on the 29th postoperative day with no neurological deficit. Conclusion: The correct approach on bilateral epidural hematomas depends on the volume, moment of diagnosis, and neurological deficit level. Simultaneous drainage of bilateral hematomas has been demonstrated to be an effective technique for it, which soon decreases the intracranial pressure and promotes an efficient resolution to the neurological damage. PMID:25657867

Pereira, Edmundo Luis Rodrigues; Rodrigues, Daniella Brito; Lima, Lorena Oliveira; Sawada, Luis Armando; Hermes, Mário de Nazareth

2015-01-01

102

Syndrome in question. MAGIC syndrome.  

PubMed

The authors present a male 40-year-old patient with established diagnosis of Behçet's disease which had evolved to recurrent bilateral auricular polychondritis crises. MAGIC syndrome (mouth and genital ulcers with inflamed cartilage) is rare and groups together patients with this clinical picture without necessarily fulfilling the clinical criteria for Behçet's disease or relapsing polychondritis, demonstrating an independent disorder. PMID:24626673

Nascimento, Ana Cláudia Mendes do; Gaspardo, Daniela Barros Cortez; Cortez, Tatiana Mimura; Miot, Hélio Amante

2014-01-01

103

Articulatory/phonetic sequencing at the level of the anterior perisylvian cortex: a functional magnetic resonance imaging (fMRI) study.  

PubMed

Damage to the anterior peri-intrasylvian cortex of the dominant hemisphere may give rise to a fairly consistent syndrome of articulatory deficits in the absence of relevant paresis of orofacial or laryngeal muscles (apraxia of speech, aphemia, or phonetic disintegration). The available clinical data are ambiguous with respect to the relevant lesion site, indicating either dysfunction of the premotor aspect of the lower precentral gyrus or the anterior insula in the depth of the Sylvian fissure. In order to further specify the functional anatomic substratum of this syndrome, functional magnetic resonance imaging (fMRI) was performed during reiteration of syllables differing in their demands on articulatory/phonetic sequencing (CV versus CCCV versus CVCVCV). Horizontal tongue movements and a polysyllabic lexical item served as control conditions. Repetition of the CV and CCCV monosyllables elicited a rather bilateral symmetric hemodynamic response at the level of the anterior and posterior bank of the central sulcus (primary sensorimotor cortex), whereas a more limited area of neural activity arose within this domain during production of lexical and nonlexical polysyllables, significantly or exclusively lateralized toward the left hemisphere. There is neurophysiological evidence that primary sensorimotor cortex mediates the "fractionation" of movements. Assuming that the polysyllables considered are organized as coarticulated higher-order units, the observed restricted and lateralized cortical activation pattern, most presumably, reflects a mode of "nonindividualized" motor control posing fewer demands on "movement fractionation." These findings may explain the clinical observation of disproportionately worse repetition of trisyllabic items as compared to monosyllables in apraxia of speech. The various test materials failed to elicit significant activation of the anterior insula. If at all, only horizontal tongue movements yielded a hemodynamic reaction extending beyond the sensorimotor cortex to premotor areas. Since limbic projections target the inferior dorsolateral frontal lobe, the enlarged region of activation during horizontal tongue movements might reflect increased attentional requirements of this task. PMID:11049668

Riecker, A; Ackermann, H; Wildgruber, D; Meyer, J; Dogil, G; Haider, H; Grodd, W

2000-11-01

104

Complete bilateral horizontal gaze paralysis disclosing multiple sclerosis  

PubMed Central

Two women presented with bilateral internuclear ophthalmoplegia evolving in a few days to complete bilateral horizontal gaze paralysis. Convergence and vertical eye movements were normal. Cerebral MRI showed a few small white matter lesions in the lateral ventricle regions, and, at the brainstem level, a single, small, bilateral lesion affecting the posterior part of the medial pontine tegmentum and responsible for the clinical syndrome. The condition gradually improved in both patients, following a similar progression as at the onset: improvement first involved the adduction movements in both eyes, whereas bilateral abduction paresis still persisted for a few weeks, before complete recovery of eye movements. Bilateral damage to the medial longitudinal fasciculus and subsequent lateral extent of damage to the region of the two abducens emerging fibres may explain the clinical findings. In both cases, the cause was probably multiple sclerosis.?? PMID:11160480

Milea, D; Napolitano, M; Dechy, H; Le Hoang, P; Delattre, J; Pierrot-Deseillig..., C

2001-01-01

105

Sirenomelia: the mermaid syndrome  

Microsoft Academic Search

Sirenomelia, or mermaid syndrome, is the extreme form of caudal regression syndrome. We present another case of this fascinating anomaly with fused lower limbs, absent external genitalia, and absent genitourinary system. The patient could not be salvaged because of bilateral renal agenesis. Detailed autopsy findings and a review of the literature are presented.

I. V. Meisheri; V. S. Waigankar; M. P. Patel; A. Naregal; S. Ramesh; P. Muthal

1996-01-01

106

Mermaid and Potter's Syndrome Occurring Simultaneously  

Microsoft Academic Search

We herein report a case of a female embryo who died in utero and at autopsy she was found to have bilateral renal agenesis with the extrarenal manifestations of Potter's syndrome together with mermaid syndrome which is a rare combination. From all the anomalies of the upper urinary tract bilateral renal agenesis seems to have a cardinal role in the

E. N. Liatsikos; P. Perimenis; K. Dandinis; E. Kaladelfou; G. A. Barbalias

1999-01-01

107

Bilateral pisotriquetral loose bodies  

PubMed Central

Case reports detailing diagnosis and effective treatment of pisotriquetral loose bodies are scarce. This article describes an even rarer case of bilateral pisotriquetral joint loose bodies, explores the relative diagnostic roles of magnetic resonance imaging versus computed tomography, and outlines effective strategies used for the management of this condition drawn from the literature and our own experience. PMID:22507708

Williams, GR; Holland, P; Beazley, J; Hyder, N

2012-01-01

108

Bilateral clicking ribs.  

PubMed Central

Congenital abnormalities of the ribs, including slipping or clicking rib, are well recognised but rarely give rise to symptoms. Slipping rib has previously been described as a unilateral condition. We report an unusual case of symptomatic bilateral slipping ribs treated successfully by surgery. PMID:2928991

Parry, W; Breckenridge, I; Khalil, Y F

1989-01-01

109

Bilateral temporalis muscle hypertrophy--two case reports.  

PubMed

Isolated idiopathic bilateral temporalis muscle hypertrophy is a very rare clinical entity. It can change the facial appearance that manifests itself as a morphopsychological conflict for the subject, which is termed as 'Minotaur syndrome' in the medical literature. Here one such case is reported. The second patient sought medical attention for fear of malignancy. PMID:23360032

Kar, Jugal Kishore; Kar, Manoranjan; Maiti, Saswati; Sen, Eva

2012-05-01

110

Coloration of Fundus Lesions in Bilateral Diffuse Uveal Melanocytic Proliferation  

Microsoft Academic Search

Purpose: To report differences in the coloration of fundus lesions between Asian and Caucasian patients with bilateral diffuse uveal melanocytic proliferation (BDUMP). Cases: This syndrome was detected in 2 Japanese patients, 69 and 73 years old, with lung cancer who visited our department complaining of visual disturbances. The coloration of the fundus lesions was investigated in these 2 patients. Results:

Saiyuu Yu; Tomohiro Ikeda; Naohiro Ikeda; Osamu Mimura; Keiko Sato

111

Bilateral Hypermetropia, Myelinated Retinal Nerve Fibers, and Amblyopia  

PubMed Central

A 14-year-old hyperopic female with poor vision in both eyes was evaluated for ophthalmic and systemic diseases. The patient had bilateral retinal fiber myelination and greater vision loss in the more hyperopic eye. This was a rare case of reverse Straatsma syndrome, the clinical presentation which may be accompanied with significant vision loss. PMID:21572738

Shenoy, Radha; Bialasiewicz, Alexander A.; Al Barwani, B.

2011-01-01

112

Bilateral hypermetropia, myelinated retinal nerve fibers, and amblyopia.  

PubMed

A 14-year-old hyperopic female with poor vision in both eyes was evaluated for ophthalmic and systemic diseases. The patient had bilateral retinal fiber myelination and greater vision loss in the more hyperopic eye. This was a rare case of reverse Straatsma syndrome, the clinical presentation which may be accompanied with significant vision loss. PMID:21572738

Shenoy, Radha; Bialasiewicz, Alexander A; Al Barwani, B

2011-01-01

113

Eagle syndrome.  

PubMed

Eagle syndrome, also known as elongated styloid process, is a condition first described by Watt Eagle in 1937. It occurs when an elongated styloid process or calcified stylohyoid ligament causes recurrent throat pain or foreign body sensation, dysphagia, or facial pain. Additional symptoms may include neck or throat pain with radiation to the ipsilateral ear. It is usually hard to diagnose because the symptoms related to this condition can be confused with those attributed to a wide variety of facial neuralgias. In this article, a case of Eagle syndrome exhibiting unilateral symptoms with bilateral elongation of styloid process is reported. PMID:24406612

Ferreira, Pedro Costa; Mendanha, Mário; Frada, Tiago; Carvalho, Jorge; Silva, Alvaro; Amarante, José

2014-01-01

114

Bilateral Mandibular Paramolars  

PubMed Central

ABSTRACT Supernumerary tooth is a developmental anomaly and has been argued to arise from multiple etiologies. These teeth may remain embedded in the alveolar bone or can erupt into the oral cavity. They can cause a variety of complications in the develo­ping dentition. Supernumerary teeth can present in various forms and in any region of the mandible or maxilla, but have a predisposition for the anterior maxilla. Here is the presentation of a case of unusual location of supernumerary teeth located in between mandibular first and second molar region bilaterally. How to cite this article: Dhull KS, Dhull RS, Panda S, Acharya S, Yadav S, Mohanty G. Bilateral Mandibular Paramolars. Int J Clin Pediatr Dent 2014;7(1):40-42. PMID:25206236

Dhull, Rachita Singh; Panda, Swagatika; Acharya, Sonu; Yadav, Shweta; Mohanty, Gatha

2014-01-01

115

Bilateral Sagittal Split Osteotomy  

PubMed Central

The bilateral sagittal split osteotomy is an indispensable tool in the correction of dentofacial abnormalities. The technique has been in practice since the late 1800s, but did not reach widespread acceptance and use until several modifications were described in the 1960s and 1970s. Those modifications came from a desire to make the procedure safer, more reliable, and more predictable with less relapse. Those goals continue to stimulate innovation in the field today and have helped the procedure evolve to be a very dependable, consistent method of correction of many types of malocclusion. The operative surgeon should be well versed in the history, anatomy, technical aspects, and complications of the bilateral sagittal split osteotomy to fully understand the procedure and to counsel the patient. PMID:24872760

Monson, Laura A.

2013-01-01

116

Bilateral sagittal split osteotomy.  

PubMed

The bilateral sagittal split osteotomy is an indispensable tool in the correction of dentofacial abnormalities. The technique has been in practice since the late 1800s, but did not reach widespread acceptance and use until several modifications were described in the 1960s and 1970s. Those modifications came from a desire to make the procedure safer, more reliable, and more predictable with less relapse. Those goals continue to stimulate innovation in the field today and have helped the procedure evolve to be a very dependable, consistent method of correction of many types of malocclusion. The operative surgeon should be well versed in the history, anatomy, technical aspects, and complications of the bilateral sagittal split osteotomy to fully understand the procedure and to counsel the patient. PMID:24872760

Monson, Laura A

2013-08-01

117

Bilateral calcaneal stress fractures: a case report.  

PubMed

The majority of plantar heel pain is diagnosed as plantar fasciitis or heel spur syndrome. When history or physical findings are unusual or when routine treatment proves ineffective, one should consider an atypical cause of heel pain. Stress fractures of the calcaneus are a frequently unrecognized source of heel pain. In a normal populat?on, the possibility of calcaneal stress fractures must be borne in mind with patients who have bilateral heel pain. When a stress fracture is considered, clinicans have different imaging options. First of all, x-rays must be used to evaluate for any visible osseous pathology. If plain films are inconclusive, the clinician can proceed with a bone scan or Magnetic Resonance Imaging. In the literature, calcaneal stress fractures are mostly reported in soldiers or athletes, but our case is one of a 44-year-old housewife with bilateral heel pain treated as Achilles tendinitis and plantar faciitis for a long time. Her final diagnosis was bilateral calcaneal fracture by Magnetic Resonance Imaging. PMID:23208939

Imerci, Ahmet; Incesu, Mustafa; Bozoglan, Muhammet; Canbek, Umut; Ursavas, Hüseyin Tamer

2012-01-01

118

Bilateral Testicular Epidermoid Cysts  

PubMed Central

Testicular epidermoid cysts are the most common benign tumors of the testes, but account for only 1-2% of all testicular tumors. In a young man presenting with a testicular mass, a high index of suspicion must be maintained for the malignant testicular germ cell tumor, which is 50-times more common than testicular epidermoid cyst. Bilateral testicular epidermoid cysts are a very rare condition, with only a few reports in the literature. It is extremely important in this condition to make a correct pre-operative diagnosis on imaging to enable a testis-sparing surgery. PMID:21915385

Loberant, Norman; Bhatt, Shweta; Messing, Edward; Dogra, Vikram S.

2011-01-01

119

Oto-onycho-peroneal syndrome: confirmation of a syndrome.  

PubMed Central

We report two sibs with a similar syndrome of abnormal external ears, peculiar facial features, nail hypoplasia, a bilateral fibrous fusion of the outer third of the clavicle and the scapular spine, and the absence of a normal acromioclavicular joint. The present patients represent the fourth and fifth cases of the oto-onycho-peroneal syndrome (MIM 259780). Images PMID:9643295

Devriendt, K; Stoffelen, D; Pfeiffer, R; Leys, A; Fryns, J P

1998-01-01

120

Löfgren's syndrome misdiagnosed as cellulitis.  

PubMed

Löfgren's syndrome is an acute-onset presentation of sarcoidosis that can be easily missed in an ED setting. A case is reported of Löfgren's syndrome presenting as erythema nodosum with bilateral ankle oedema. Although rare, this diagnosis should be considered when examining a patient with erythema nodosum and articular symptoms. PMID:21668726

Cheng, Daryl Ray; Maini, Amit

2011-06-01

121

International relations office BILATERAL AGREEMENTS  

E-print Network

International relations office BILATERAL AGREEMENTS UdeM CANDIDATES BILATERAL STUDENT EXCHANGE PROGRAMS APPLICATION FOR PARTICIPATION YEAR 2014-2015 2015-2016 CANDIDATE IDENTIFICATION Last name : ________ Winter semester : (Jan. to April) Number of UdeM credits : ________ Summer semester : (May to Aug

Parrott, Lael

122

Silicosis with bilateral spontaneous pneumothorax  

PubMed Central

Presentation with simultaneous bilateral pneumothorax is uncommon and usually in the context of secondary spontaneous pneumothorax. The association of pneumothorax and silicosis is infrequent and most cases are unilateral. Bilateral pneumothorax in silicosis is very rare with just a few reports in medical literature. PMID:20931041

Fotedar, Sanjay; Chaudhary, Dhruva; Singhla, Vikas; Narang, Rajat

2010-01-01

123

Bilateral cholesteatoma and habitual sniffing  

Microsoft Academic Search

Objective: To examine the clinical findings of acquired bilateral cholesteatoma with special reference to incidence of habitual sniffing and sniff-related negative middle ear pressure. Methods: Eighty-eight fresh cases of unilateral cholesteatoma and 33 fresh cases of bilateral cholesteatoma, which were operated on at Department of Otolaryngology, Hyogo College of Medicine, were examined in this study. Responses to a detailed questionnaire

Kojiro Tsuji; Mieko Sone; Masao Kakibuchi; Masafumi Sakagami

2002-01-01

124

Ovarian remnant syndrome: a case report and review of the literature.  

PubMed

The ovarian remnant syndrome in an unusual complication of bilateral oophorectomy, usually presenting with pelvic mass and pain. A case of the syndrome is described in a 35-year-old woman with a history of abdominal hysterectomy and bilateral oophorectomy. We suggest that ovarian remnant syndrome should be considered in the differential diagnosis of chronic pelvic pain after recorded oophorectomy. PMID:10968351

Vavilis, D; Loufopoulos, A; Agorastos, T; Vakiani, M; Intzesiloglou, L; Karavida, A; Bontis, J N

2000-01-01

125

Letter to the Editor Bilateral striopallidodentate calcinosis (Fahr's disease)  

Microsoft Academic Search

Madam, Bilateral striopallidodentate calcinosis (Fahr's disease), is a rare syndrome characterized by symmetrical calcification over the basal ganglion and dentate nucleus.1-4We report the case of a young female who met with a road traffic accident and CT scan showed calcification in basal ganglia. A 40 year old female presented with the history of a fall from an auto-rickshaw. Following the

Amit Agrawal

126

[Total bilateral carpal coalition with carpometacarpal fusion: one case].  

PubMed

Carpal coalition may involve more than two carpal bones which is more common when coalition occurs as part of a syndrome of congenital malformations. We describe an unusual case of congenital complete entire carpal coalition with massive carpometacarpal coalition except the first carpometacarpal joint and abnormal distal radioulnar joint bilaterally. In addition, radiocarpal joint surfaces were slightly irregular but compatible. There were abnormal distal radioulnar joints and ulnar styloids articulated with the ulnar side of the lunate of the both wrists. PMID:11845077

Tuncay, I; Akpinar, F; Unal, O; Aydinlioglu, A

2001-11-01

127

Association of cryptogenic organizing pneumonia in bilateral anterior uveitis.  

PubMed

Two female patients with histories of cancer who showed cryptogenic organizing pneumonia (COP) complications and bilateral anterior uveitis with hypopyon were examined. Both patients had suffered from COP and received intermitted systemic corticosteroid administration (SCA). The first patient, a 65-year-old woman with a history of breast cancer, showed bilateral uveitis with hypopyon. The topical corticosteroid treatment was ineffective. After SCA for the treatment of COP was started, the hypopyon gradually dissipated. Upon termination of SCA, uveitis relapses were controlled by renewed SCA. The other patient, a 69-year-old woman with a history of ovarian cancer, showed bilateral anterior uveitis with hypopyon. Her intraocular outcome did not improve by the topical corticosteroid administration, but SCA that was applied to treat COP led to remission of uveitis. Imaging examinations, biochemical analysis, symptoms or HLA-B27 antigen screenings in either patient did not explain the development of uveitis. Bilateral anterior uveitis is commonly related to autoimmune disease or systemic syndrome. We report two cases with COP that developed bilateral anterior uveitis with hypopyon resistant to topical administration but responsive to systemic administration of corticosteroid. These findings suggest that COP can be associated with the etiology of anterior uveitis. PMID:25520652

Fujimoto, Kaori; Hiraoka, Miki; Inatomi, Shuichiro; Ohguro, Hiroshi

2014-09-01

128

Syndrome In Question*  

PubMed Central

The authors present a male 40-year-old patient with established diagnosis of Behçet's disease which had evolved to recurrent bilateral auricular polychondritis crises. MAGIC syndrome (mouth and genital ulcers with inflamed cartilage) is rare and groups together patients with this clinical picture without necessarily fulfilling the clinical criteria for Behçet's disease or relapsing polychondritis, demonstrating an independent disorder. PMID:24626673

do Nascimento, Ana Cláudia Mendes; Gaspardo, Daniela Barros Cortez; Cortez, Tatiana Mimura; Miot, Hélio Amante

2014-01-01

129

Spontaneous bilateral internal carotid artery dissection.  

PubMed

Spontaneous internal carotid artery dissection is not an uncommon cause of ischaemic stroke in younger patients, but multiple cervical arterial dissections at presentation are uncommon. Recurrence of dissection in a previously normal artery is common. In this case report we review the history, clinical findings and management of a 42-year-old woman who presented with stroke and Horner syndrome and was found to have spontaneous bilateral internal carotid artery dissection. She was not anticoagulated due to concerns relating to the size of her infarct. She was treated with a combination of aspirin and clopidogrel. We use dual antiplatelets for the management of cervical dissections as a part of the CADISS trial. The patient made good progress with the multidisciplinary team and was discharged on day 22 with support from the community stroke team. PMID:22110554

Ardhalapudi, Srujan; Addy, Victoria; Da Costa, David

2009-01-01

130

Patient with bilateral breast enlargement and hypospadias.  

PubMed

A 20 year old man was admitted in the surgical unit of Dhaka Medical College Hospital on August 2007 presented with Hypospadias & underdeveloped penis from childhood, gradual enlargement of both breasts for the last 10 years, absence of axillary and pubic hair from puberty & painful swelling of both testes for the last 3 years. The patient was regarded as a female upto the age of 10 years. He has got no voice change since puberty. On examination he had average body built, there was bilateral gynaecomastia, hypospadias, rudimentary penis & absence of pubic and axillary hair. His testosterone level, serum prolactin level, serum progesterone level, serum estradiol level was done. Cytology on buccal smear done and ultrasonography revealed no ovary and uterus. Ultimately patient was diagnosed as a case of androgen insensitivity syndrome (AIS). The case is reported for clinical awareness & to share our experience. PMID:19182761

Ali, A A; Ahmed, T; Rashid, M M

2009-01-01

131

Bilateral mental nerve neuropathy in an adolescent during sickle cell crises.  

PubMed

Mental nerve neuropathy causes the "numb chin" syndrome and is usually associated with mandibular bone injury or disease in adults. It has been reported in adults during sickle cell crises. We describe a 15-year-old boy who developed bilateral mental nerve neuropathies during a sickle cell crisis. This case is unusual because of the simultaneous bilateral involvement and because of the age. PMID:22290855

Hamdoun, Elwaseila; Davis, Lamar; McCrary, Sara Jane; Eklund, Neva Penton; Evans, Owen B

2012-08-01

132

Primary ovarian adenocarcinoma developing in ovarian remnant tissue ten years after laparoscopic hysterectomy and bilateral salpingo-oophorectomy for endometriosis.  

PubMed

Ovarian remnant syndrome is a rare but known complication of bilateral salpingo-oophorectomy associated or not with hysterectomy. This complication is frequently related to a history of multiple surgery, pelvic inflammatory disease, or endometriosis. Here we report the eighth documented case of a primary ovarian adenocarcinoma developing in an ovarian remnant but, to our knowledge, the first case to occur after laparoscopic hysterectomy and bilateral salpingo-oophorectomy. We discuss the management of pelvic masses suspected of malignancy after bilateral salpingo-oophorectomy and the possible role of endometriosis in the development of malignancy in ovarian remnant syndrome. PMID:17980339

Donnez, Olivier; Squifflet, Jean; Marbaix, Etienne; Jadoul, Pascale; Donnez, Jacques

2007-01-01

133

Bilateral internal laryngoceles mimicking asthma  

PubMed Central

Laryngocele is an air-filled, abnormal dilation of the laryngeal saccule that extends upward within the false vocal fold, in communication with the laryngeal lumen. A case of 43-year-old male with bilateral internal laryngoceles, who has been treated as asthma for 4 years, is presented. The patient had dyspnea, cough, and excessive phlegm for a month and a late onset stridor. Flexible nasopharyngolaryngoscopy showed bilateral cystic enlargements of the false vocal folds and true vocal folds could not be visualized. Laryngeal CT without contrast enhancement showed bilateral internal laryngoceles. Submucosal total excision of bilateral cystic masses including parts of false vocal folds was performed. The symptoms resolved immediately after surgery. Although the incidence of internal laryngocele is rare, it should be remembered in the differential diagnosis of upper airway problems and diagnostic flexible nasopharnygolaryngoscopy is routinely indicated for airway evaluation in at-risk patients. PMID:24174956

Aksoy, Elif A.; Elsürer, Ça?da?; Serin, Gediz M.; Ünal, Ö. Faruk

2013-01-01

134

BILATERAL WASTEWATER LAND TREATMENT RESEARCH  

EPA Science Inventory

Diplomatic relations between the United States and China, established in 1979, opened the door for development of 1985 bilateral environmental research and technology transfer between the USEPA's Robert S. Kerr Environmental Research Laboratory, Ada, Oklahoma, and China's Beijing...

135

Perioperative spontaneous bilateral suprachoroidal hemorrhage  

PubMed Central

Summary Suprachoroidal hemorrhage is a rare condition, occurring most commonly in the perioperative period, although cases of unilateral spontaneous hemorrhages have been reported. We report a case of bilateral spontaneous suprachoroidal hemorrhage and discuss the potential causative factors. PMID:23362387

Haridas, Anjana; Litwin, Andre S.; Coker, Timothy

2011-01-01

136

Prune belly syndrome in an adult Nigerian: case report.  

PubMed

Prune Belly Syndrome is a rare congenital anomaly characterized by deficient anterior abdominal wall musculature, bilateral cryptorchidism, bilateral megaureters and often unilateral or bilateral vesico-ureteric junction obstruction. The report of prune belly syndrome in the adult is scanty. We report a case of prune belly syndrome in a 24 year old Nigerian who presented with 3 year history of recurrent right loin pain. Examination showed wrinkled abdominal skin, bilateral undescended testes and an hypoplastic rectus abdominis, below the umbilicus. Further evaluation revealed enlarged bladder, bilateral megaureters and right intra-abdominal testis. A diagnosis of Prune Belly Syndrome was made. The challenges in the diagnosis and management of this rare condition are highlighted in this presentation. PMID:20499630

Salako, A A; Takure, A O; Olajide, A O; Aarowolo, O A; Egberongbe, A A

2009-12-01

137

Heterogeneity in Waardenburg syndrome.  

PubMed Central

Heterogeneity of Waardenburg syndrome is demonstrated in a review of 1,285 patients from the literature and 34 previously unreported patients in five families in the Netherlands. The syndrome seems to consist of two genetically distinct entities that can be differentiated clinically: type I, Waardenburg syndrome with dystopia canthorum; and type II, Waardenburg syndrome without dystopia canthorum. Both types have an autosomal dominant mode of inheritance. The incidence of bilateral deafness in the two types of the syndrome was found in one-fourth with type I and about half of the patients with type II. This difference has important consequences for genetic counseling. Images Fig. 7 Fig. 8 Fig. 9 PMID:331943

Hageman, M J; Delleman, J W

1977-01-01

138

Achondroplasia Associated with Bilateral Keratoconus  

PubMed Central

We report a rare case of bilateral keratoconus in association with achondroplasia. A 26-year-old male, with a known case of achondroplasia, complained of bilateral gradual deterioration in vision for the past few years. Slit lamp biomicroscopy showed bilateral central corneal protrusion and stromal thinning at the apex consistent with keratoconus. a trial of hard contact lens fitting failed to improve VA in the left eye (LE). Right eye (RE) improved to 20/25. The patient underwent penetrating keratoplasty (PKP) in his LE. Twenty-seven months postoperatively, uncorrected visual acuity (UCVA) was 20/30. Ophthalmologists should be aware that patients with achondroplasia who complain of poor vision should be suspected of having keratoconus once other more common conditions are ruled out. PMID:23259098

Al Mahmood, Ammar M.; Al Katan, Hind M.; Al Bin Ali, Ghada Y.; Al-Swailem, Samar A.

2012-01-01

139

Bilateral breast in brothers - abreast  

PubMed Central

Gynecomastia is a common occurrence in pubertal age group, and is physiological in up to 65 percent of cases. When occurs in the family it should be investigated in order not to miss on a treatable etiology. Two brothers within the same family, presenting with bilateral gynecomastia of different causes and requiring different treatment are presented. PMID:24251182

Shaikh, Altamash Mohammed Yusuf; Yadav, Sameer; Moulick, Nivedita D.

2013-01-01

140

Telepresence index for bilateral teleoperations.  

PubMed

This paper proposes a performance index called telepresence index for bilateral teleoperation, which can be used both for the performance evaluation of bilateral control architectures and for design purposes. This index is intended to represent a comprehensive performance objective consisting of transparency and kinematic correspondence, which are two major performance objectives of bilateral teleoperation. In order to quantify the performance objective, telepresence index has employed the error vector magnitude, which enables a seamless combination of magnitude and phase errors and the accommodation of time delay. In comparison with existing performance indices, it was observed that telepresence index possesses the comprehensiveness of performance objectives, magnitude/phase integrity, and the capacity to include time delay, which the others lack in one way or another. The index was applied to evaluate the performances of two widely known control architectures: PD-type bilateral control and Ueda's ideal control. In all cases, telepresence index has been compared favorably with the other indices in terms of clarity, convenience, and accuracy, thereby demonstrating its superiority. PMID:21824852

Chang, Pyung Hun; Kim, Jonghyun

2012-02-01

141

Bilateral and symmetrical tinea mammae.  

PubMed

Tinea corporis has rarely been reported in some locations such as on the breast skin as unilaterally. Herein, we present a case of bilateral tinea mammae, which has not been reported before in English language literature to our knowledge. PMID:24050298

Yilmaz, Murat; Kavak, Ayse; Yamaner, Nalan Jale

2013-09-01

142

An Animal Model of Tourette's Syndrome  

Microsoft Academic Search

Method: Sera from 12 patients with Tourette's syndrome with high levels of antineural or antinuclear antibodies were in- fused bilaterally into the ventrolateral striatum of rats. Sera from 12 additional Tourette's syndrome patients and 12 nor- mal subjects (both groups with low levels of autoantibodies) were infused for comparison. Rates of oral stereotypies were recorded by observers who were blind

J. R. Taylor; Plunket KD; Lehmann JM; Syed A. Morshed; Salina Parveen; Marcos T. Mercadante; Bradley S. Peterson; Robert A. King; James F. Leckman; Paul J. Lombroso

2002-01-01

143

Major vascular complications in Behçet's syndrome  

Microsoft Academic Search

Two patients with Behçet's syndrome are described. One patient developed spontaneous bilateral popliteal artery aneurysms and the other spontaneous thrombosis of the superior vena cava. These cases emphasize that primary inflammatory lesions of large vessels, with arterial aneurysm formation and thrombosis may be a feature of the syndrome.

B. Anthony Enoch; T. C. L. Khoo; J. L. Castillo-Olivares; R. G. Grainger; L. Henry

1968-01-01

144

Rubinstein-Taybi syndrome: Cranial MR imaging findings  

Microsoft Academic Search

Rubinstein-Taybi syndrome is a syndrome of mental retardation associated with broad thumbs and big toes. The patients have a characteristic flat face with a beaked nose. This paper deals with brain changes in a patient with this syndrome, studied by MR imaging, which mainly consist of bilateral rolandic cortical clefts and diminished white matter, which probably account for mental retardation.

R. Nuri Sener

1995-01-01

145

Interictal Slow-Wave Focus in Left Medial Temporal Lobe during Bilateral Electroconvulsive Therapy  

Microsoft Academic Search

The interictal state between two electroconvulsive therapy (ECT) sessions is clinically characterised by possible cognitive adverse effects like mild amnestic syndrome. ECT-induced mnestic deficits can persist for several weeks after ECT. Electrophysiologically, slowing of brain electrical activity in the interictal state has often been reported. Especially, for bilateral ECT a correlation between enhanced left frontotemporal theta activity and retrograde amnesia

Andres H. Neuhaus; Juergen Gallinat; Malek Bajbouj; Friedel M. Reischies

2005-01-01

146

VOIDING DYSFUNCTION AFTER BILATERAL EXTRAVESICAL URETERAL REIMPLANTATION  

Microsoft Academic Search

PurposeVoiding dysfunction has been reported after bilateral extravesical ureteral reimplantation. We evaluate the incidence and duration of voiding dysfunction, and the effects of minimizing surgical dissection in a cohort of children after bilateral extravesical reimplantation.

BETH A. LIPSKI; MICHAEL E. MITCHELL; MARK W. BURNS

1998-01-01

147

Note: Unshielded bilateral magnetoencephalography system using two-dimensional gradiometers  

NASA Astrophysics Data System (ADS)

Magnetoencephalography (MEG) noninvasively measures neuronal activity with high temporal resolution. The aim of this study was to develop a new type of MEG system that can measure bilateral MEG waveforms without a magnetically shielded room, which is an obstacle to reducing both the cost and size of an MEG system. An unshielded bilateral MEG system was developed using four two-dimensional (2D) gradiometers and two symmetric cryostats. The 2D gradiometer, which is based on a low-Tc superconducting quantum interference device and wire-wound pickup coil detects a magnetic-field gradient in two orthogonal directions, or ?/?x(?2Bz/?z2), and reduces environmental magnetic-field noise by more than 50 dB. The cryostats can be symmetrically positioned in three directions: vertical, horizontal, and rotational. This makes it possible to detect bilateral neuronal activity in the cerebral cortex simultaneously. Bilateral auditory-evoked fields (AEF) of 18 elderly subjects were measured in an unshielded hospital environment using the MEG system. As a result, both the ipsilateral and the contralateral AEF component N100m, which is the magnetic counterpart of electric N100 in electroencephalography and appears about 100 ms after the onset of an auditory stimulus, were successfully detected for all the subjects. Moreover, the ipsilateral P50m and the contralateral P50m were also detected for 12 (67%) and 16 (89%) subjects, respectively. Experimental results demonstrate that the unshielded bilateral MEG system can detect MEG waveforms, which are associated with brain dysfunction such as epilepsy, Alzheimer's disease, and Down syndrome.

Seki, Yusuke; Kandori, Akihiko; Ogata, Kuniomi; Miyashita, Tsuyoshi; Kumagai, Yukio; Ohnuma, Mitsuru; Konaka, Kuni; Naritomi, Hiroaki

2010-09-01

148

Foix-Chavany-Marie syndrome in a 17-year-old female with congenital cytomegalovirus infection  

PubMed Central

Foix-Chavany-Marie syndrome is characterized by bilateral facio-glosso-pharyngo-masticatory paralysis of voluntary movement due to bilateral anterior opercular lesions. We describe the case of a 17-year-old female affected by Foix-Chavany-Marie syndrome and congenital cytomegalovirus infection, evaluating the possible etiopathogenetic correlation between cerebral cortical dysplasia and intrauterine infections. PMID:25429223

Conforti, Renata; Capasso, Raffaella; Capaldo, Guglielmo; Dato, Clemente; Saracino, Dario; Di Iorio, Giuseppe; Melone, Mariarosa A

2014-01-01

149

Laparoscopic Cortical Sparing Adrenalectomy for Pediatric Bilateral Pheochromocytoma: Anesthetic Management  

PubMed Central

Introduction: Pheochromocytoma is a catecholamine-secreting tumor, which is seen rarely in children. These tumors predominantly secrete norepinephrine and epinephrine. They might be familial and associated with hereditary tumors such as Von Hippel-Lindau syndrome and multiple endocrine neoplasia type II. Case Presentation: The child might present with a spectrum of clinical manifestation including hypertension, headache, visual disturbances, and behavioral problems. A meticulous preoperative preparation is essential for a stable intraoperative and postoperative outcome Conclusions: We described successful perioperative management of a child who underwent bilateral laparoscopic cortical sparing adrenalectomy and a repeated surgery for the residual tumor removal. PMID:24790902

Rajappa, Geetha Chamanhalli; Anandaswamy, Tejesh Channasandra

2014-01-01

150

Reversible posterior leukoencephalopathy syndrome  

Microsoft Academic Search

Reversible posterior leukoencephalopathy syndrome (RPLS) is characterized by subacute onset of headache, decreased alertness,\\u000a vomiting, seizures, visuoperceptual disturbances, together with bilateral white matter lesions in posterior brain regions\\u000a on brain imaging. The most frequently associated conditions related to RPLS are arterial hypertension and the use of immunosuppressive\\u000a or cytotoxic treatment. T2-, Fluid Attenuation Inversion Recovery (FLAIR), and Apparent Diffusion Coefficient

Dimitri Renard; Pierre Labauge; Rik Vandenberghe

2010-01-01

151

Tumors of Bilateral Streak Gonads in Patients with Disorders of Sex Development Containing Y Chromosome Material  

PubMed Central

Abstract The presence of Y chromosome material in patients with disorders of sex development (DSD) has been associated with a high risk of gonadoblastoma. Therefore, gonadectomy is recommended in females with bilateral streak gonads and Y chromosome material. The aim of this study was to present our experience with prophylactic gonadectomy in those patients and evaluate their risk of gonadal tumors. We reviewed the charts of 11 female patients who had bilateral gonadectomy (by laparoscopically in 9 patients, by laparotomy in 2 patients) between 1991 and 2012 at our hospital. Seven patients with Turner syndrome (TS) who carry a Y mosaic karyotype in peripheral blood, 3 patients with Swyer syndrome and one patient with Frasier syndrome were included. All patients had an unambiguous female phenotype. Age at surgery and follow-up ranged from 2 to 23 (mean 11) and 0.5 to 20 (mean 8) yr, respectively. Pathologic examination revealed gonadal tumors in 6 of 11 patients (56%), including 4 with TS, the youngest of which was 2 yr old, one with Swyer syndrome and one with Frasier syndrome. A gonadoblastoma was detected in 8 gonads, and an association of dysgerminoma with gonadoblastoma was detected in 2 gonads. Imaging studies showed no metastasis, and the postoperative course was uneventful in all patients. In our series of DSD patients with bilateral streak gonads and Y chromosome material, the risk of gonadal tumor was high. Considering the early occurrence of gonadoblastoma and its high potential for malignant transformation, early prophylactic gonadectomy is strongly recommended. PMID:25110393

Matsumoto, Fumi; Shimada, Kenji; Ida, Shinobu

2014-01-01

152

Bilatral auriculotemporal syndrome in childhood  

Microsoft Academic Search

A case of bilateral Auriculotemporal or Frey's syndrome in childhood is unique in the World literature. The clinical presentation and management is discussed. A conservative approach is advocated but other therapeutic options are considered. The literature is reviewed, only 17 cases all unilateral are known, and from the study the probable aetiology and long term follow-up is reported.

I. J. M. Johnson; J. P. Birchall

1995-01-01

153

Multiresolution Bilateral Filtering for Image Denoising  

PubMed Central

The bilateral filter is a nonlinear filter that does spatial averaging without smoothing edges; it has shown to be an effective image denoising technique. An important issue with the application of the bilateral filter is the selection of the filter parameters, which affect the results significantly. There are two main contributions of this paper. The first contribution is an empirical study of the optimal bilateral filter parameter selection in image denoising applications. The second contribution is an extension of the bilateral filter: multiresolution bilateral filter, where bilateral filtering is applied to the approximation (low-frequency) subbands of a signal decomposed using a wavelet filter bank. The multiresolution bilateral filter is combined with wavelet thresholding to form a new image denoising framework, which turns out to be very effective in eliminating noise in real noisy images. Experimental results with both simulated and real data are provided. PMID:19004705

Zhang, Ming; Gunturk, Bahadir K.

2008-01-01

154

FDG PET findings of the brain in sudden blindness caused by bilateral central retinal artery occlusion revealing giant cell arteritis.  

PubMed

We report the case of a 73-year-old woman presenting sudden blindness caused by bilateral simultaneous central retinal artery occlusion revealed by ophthalmoscopy. Temporal artery biopsy confirmed the giant cell arteritis. The patient was treated with a systemic steroid without visual recovery. FDG PET/CT was performed 6 months later in the context of persistent inflammatory syndrome. This case shows the close relationship between functional activity and glucose energy metabolism. We observed both bilateral occipital hypometabolism corresponding to loss of functional activity and bilateral temporal (auditory areas) and orbitofrontal hypermetabolism related to compensatory neuronal plasticity. PMID:25275418

Dietemann, Sébastien; Noblet, Vincent; Imperiale, Alessio; Blondet, Cyrille; Namer, Izzie Jacques

2015-01-01

155

Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal Hyperplasia  

PubMed Central

Myelolipomas are rare and benign neoplasms, predominant of the adrenal glands, consisting of adipose and mature hematopoietic tissue, commonly discovered incidentally with increased use of radiologic imaging. Few cases of giant bilateral adrenal masses are reported, especially in the setting of congenital adrenal hyperplasia (CAH). We report the case of a 39-year-old male with a history of CAH secondary to 21-? hydroxylase deficiency on steroids since childhood, self-discontinued during adolescence, presenting with abdominal distension, fatigue, decreased libido, and easy bruising. Imaging revealed giant bilateral adrenal masses. He subsequently underwent bilateral adrenalectomy found to be myelolipomas measuring 30 × 25 × 20?cm on the left and weighing 4.1?kg and 25 × 20 × 13?cm on the right and weighing 2.7?kg. Adrenal myelolipomas are found to coexist with many other conditions such as Cushing's syndrome, Addison's disease, and CAH. We discuss the association with high adrenocorticotropic hormone (ACTH) states and review the studies involving ACTH as proponent leading to myelolipomas. Massive growth of these tumors, as in our case, can produce compression and hemorrhagic symptoms. We believe it is possible that self-discontinuation of steroids, in the setting of CAH, may have resulted in the growth of his adrenal masses. PMID:25140269

Al-Bahri, S.; Tariq, A.; Lowentritt, B.; Nasrallah, D. V.

2014-01-01

156

Eagle's syndrome: signs and symptoms.  

PubMed

Eagle's syndrome, an uncommon sequela of an elongated styloid process, can manifest itself as a sensation of a foreign body in the throat and a retrogoniac or anterolateral neck pain often referred to the TMJ and the ear. When treating patients affected by temporomandibular disorders (TMD), complaining of atypical orofacial pain, and with a concomitant radiographic finding of a bilaterally elongated styloid, the possible coexistence of Eagle's syndrome should always be considered. The aim of this review is to present an overview of the epidemiology, pathophysiology, diagnosis, and therapy for Eagle's syndrome and to help the clinician in differential diagnosis with other conditions that can provide similar signs and symptoms. PMID:23461263

Costantinides, Fulvia; Vidoni, Gabriele; Bodin, Christiane; Di Lenarda, Roberto

2013-01-01

157

Loin pain hematuria syndrome.  

PubMed

Loin pain hematuria syndrome is a rare disease with a prevalence of ?0.012%. The most prominent clinical features include periods of severe intermittent or persistent unilateral or bilateral loin pain accompanied by either microscopic or gross hematuria. Patients with loin pain hematuria syndrome initially present with hematuria, flank pain, or most often both hematuria and flank pain. Kidney biopsies from patients with loin pain hematuria typically reveal only minor pathologic abnormalities. Further, loin pain hematuria syndrome is not associated with loss of kidney function or urinary tract infections. Loin pain hematuria syndrome-associated hematuria and pain are postulated to be linked to vascular disease of the kidney, coagulopathy, renal vasospasm with microinfarction, hypersensitivity, complement activation on arterioles, venocalyceal fistula, abnormal ureteral peristalsis, and intratubular deposition of calcium or uric acid microcrystals. Many patients with loin pain hematuria syndrome also meet criteria for a somatoform disorder, and analgesic medications, including narcotics, commonly are used to treat loin pain hematuria syndrome-associated pain. Interventional treatments include renal denervation, kidney autotransplantation, and nephrectomy; however, these methods should be used only as a last resort when less invasive measures have been tried unsuccessfully. In this review article, we discuss and critique current clinical practices related to loin pain hematuria syndrome pathophysiology, diagnosis, treatment, and prognosis. PMID:24725981

Taba Taba Vakili, Sahar; Alam, Tausif; Sollinger, Hans

2014-09-01

158

Independent bilateral primary bronchial carcinomas  

PubMed Central

Independent bilateral primary bronchial carcinomas are not common. Since Beyreuther's description in 1924, 16 well-documented cases of independent primary bronchial carcinomas of different histology have been described. From 1965 to 1970, eight cases were seen at the London Chest Hospital. In order to make the diagnosis of a second primary bronchial carcinoma, each tumour should be malignant and neither should be a metastasis from the other. To meet this last criterion, the histopathological features of the two tumours must be different. Many cases have been described in the literature as double primary bronchial carcinomas where the second primary had the same histological features as the first. Images PMID:4327711

Chaudhuri, M. Ray

1971-01-01

159

[Bilateral multilobular cystic adenomatoid malformation].  

PubMed

We reported a case of bilateral and multilobar Congenital Cystic Adenomatoid Malformation (C.C.A.M.) in a four months old child with good clinical results after resections of the lesions. This is a relatively rare form of pulmonary illness. The final prognosis, in those patients, depends on the type of malformation, the presence or absence of fetal hydrops and on the degree of affected lung. There have been reported a few cases of multiple affectation. We will consider the physiopathological aspects of the case, late clinical presentation and treatment and the positive surgical response based in the findings of the functional and anatomic imagen studys. PMID:8679395

Tristán, J U; Gracía Urgellés, X; Wiehoff Neumann, A; Hernández Briz Estévez Rosas, S; Ruiz, M P; Pavcovich Calvo, F M

1995-10-01

160

Gender specific manifestations of Löfgrens syndrome  

Microsoft Academic Search

Abstract Background: Löfgren´s syndrome,is commonly regarded as a distinct clinical entity. Methods: We have in detail evaluated a large group of patients (n=150) with an acute onset of sarcoidosis with bilateral hilar lymphadenopathy (BHL), in most with cases fever, erythema nodosum (EN) and\\/or bilateral ankle arthritis or periarticular inflammation. Within this group, 87 patients had EN (EN pos), while 63

Johan Grunewald; Anders Eklund

2006-01-01

161

Fahr's Syndrome- An Interesting Case Presentation.  

PubMed

Fahr's syndrome refers to a rare syndrome which is characterized by symmetrical and bilateral intracranial calcification. We are presenting a 63 year old male, who complained of progressive dysarthria of 6 months, which was associated with slowness of movements. His neurological examination revealed Parkinsonian features. His CT scan revealed a symmetrical large area of calcification over the basal ganglia, the thalamus and the cerebellum. The secondary causes of the bilateral calcification were ruled out to make the clinical diagnosis of Idiopathic Bilateral Striopallidodentate Calcinosis, which is otherwise called as the Fahr's syndrome. Deposits of calcium and minerals cannot be linked to a single chromosomal locus and further genetic studies are in progress for identifying the chromosomal locus of the disease. PMID:23634413

Asokan, Athulya Geetha; D'souza, Sydney; Jeganathan, Jayakumar; Pai, Shivananda

2013-03-01

162

Sequential bilateral retinal artery occlusion  

PubMed Central

An 86 year old woman experienced a sequential bilateral loss of vision over a period of less than 24 hours. Clinical findings and complementary studies suggested a bilateral atherogenic embolic event. Initially, she presented a superior branch retinal artery occlusion in her right eye followed by a central retinal artery occlusion with cilioretinal artery sparing in her left eye. Some conservative maneuvers performed did not improve visual acuity in the left eye. Supra-aortic Doppler ultrasonography revealed mild right internal carotid artery stenosis and moderate left internal carotid artery stenosis with a small, smooth, and homogeneous plaque. The transthoracic echocardiography showed a severe calcification of the mitral valve with a mild-moderate rim of stenosis. Central retinal artery occlusion and branch retinal artery occlusion are characterized by painless monocular loss of vision. Clinical approach and management attempt to treat the acute event, find the source of the vascular occlusion, and prevent further vascular events from occurring. Giant cell arteritis is a potentially treatable cause of central retinal artery occlusion and should be excluded in every single patient over 50 years old. PMID:24748768

Padrón-Pérez, Noel; Aronés, Janny Rosario; Muñoz, Silvia; Arias-Barquet, Luis; Arruga, Jorge

2014-01-01

163

Fast bilateral filtering using recursive moving sum  

NASA Astrophysics Data System (ADS)

We propose a constant-time algorithm for a bilateral filter. Bilateral filter can be converted into the operation of three-dimensional (3D) convolution. By using recursive moving sum, we can reduce the number of calculations needed to construct a pseudo-Gaussian filter. Applying one-dimensional Gaussian filter to the 3D convolution, we achieved a constant-time bilateral filter. We used a 3-GHz CPU without SIMD instructions, or multi-thread operations. We confirmed our proposed bilateral filter to be processed in constant time. In practical conditions, high PSNR values over 40 dB are obtained.

Igarashi, Masaki; Ikebe, Masayuki; Shimoyama, Sohsuke; Motohisa, Junichi

164

Bilateral eosinophilic mastitis: An uncommon unheard entity.  

PubMed

We are reporting a case of bilateral eosinophilic mastitis which is rare and hardly heard. It is a mimicker of carcinoma breast both clinically & radiologically. A 30 years old non diabetic female presented with bilateral breast lumps with history of rhinitis off & on and peripheral eosinophilia. Mammography was suspicious while ultrasonography was diagnostic of bilateral mastitis. Aspiration cytology exhibited inflammatory lesion rich in eosinophils. Histopathology revealed the diagnosis of eosinophilic mastitis. Eosinophilic infiltration of the breast is a rare manifestation of tissue involvement in peripheral eosinophilia and bilateralism is even rarer. PMID:25171213

Singh, Aminder; Kaur, Pavneet; Sood, Neena; Puri, Harpreet; Garg, Bhavna

2015-01-01

165

New findings in short rib syndrome.  

PubMed

This white infant, born at 37 weeks of gestation, had a large cranium, bilateral anophthalmia, a midline cleft lip and palate, hypoplastic chest with short ribs, slightly protuberant abdomen, short limbs, bilateral single transverse palmar creases, a single umbilical artery, normal female external genitalia, normal (46 XY) chromosomes, and radiographic findings suggesting a short-rib (polydactyly) syndrome type IV (Beemer-Langer). Autopsy showed pulmonary hypoplasia, bilateral renal cystic dysplasia, intrahepatic bile duct cysts with periportal fibrosis, pancreatic cysts, absent internal genitalia, an atrophic optic chiasm, absent optic nerves, a single left anterior cerebral artery, polymicrogyria, and fusion of the frontal lobes, preoptic region, mammillary bodies, and thalami. PMID:8488867

Cideciyan, D; Rodriguez, M M; Haun, R L; Abdenour, G E; Bruce, J H

1993-05-15

166

Ovarian remnant syndrome.  

PubMed

Twenty-seven patients with ovarian remnant syndrome were operated on by the author in a 7-year period. All patients presented with pelvic pain as their major symptom. A mass was palpable in 20 patients. In 3 patients, no mass was palpable but a lesion was visible on computed tomographic scanning. In 4 patients, there was no palpable mass and scans were negative, yet an ovarian remnant was found at laparotomy. Segmental bowel resection was necessary to obtain clearance in 8 patients, and partial bladder resection was performed in 2. Previous pelvic operations (mean, 4.3) in this group of patients probably contributed to the development of this syndrome. The ovarian remnant syndrome occurs in a patient who has previously had a bilateral salpingo-oophorectomy. A piece of ovarian tissue remains, and this remnant often produces symptoms, usually pelvic pain and associated dyspareunia. Usually the patient has had a hysterectomy as well as bilateral salpingo-oophorectomy, so that the pain is often dismissed as 'not gynaecological' in origin. This syndrome is not synonymous with residual ovary syndrome, in which an ovary that has previously been intentionally conserved either develops an abnormal pathological process or causes symptoms necessitating its surgical removal (6). Unfortunately, awareness of the problems caused by an ovarian remnant is not widespread, and often these patients have been to many gynaecologists, general surgeons, or even psychiatrists in an attempt to get relief from their symptoms. It is not possible to estimate the incidence of symptomatic ovarian remnants, but certainly when an awareness of the syndrome develops, referral of patients with the problem is common. PMID:2631678

Webb, M J

1989-11-01

167

Harlequin syndrome as a complication of epidural anesthesia.  

PubMed

Harlequin syndrome is a rare neurological condition that results in unilateral facial flushing and sweating. Although the syndrome is generally a benign condition with complete resolution if appropriate treatment is initiated, unilateral facial flushing can be a sign of several serious conditions and should be thoroughly investigated. Sudden onset of facial flushing related to harlequin syndrome developed in a patient who had bilateral lung transplant with postoperative epidural anesthesia for pain control. Differential diagnosis includes neurovascular disease (acute stroke), malignant neoplasm of brain or lung, Horner syndrome, idiopathic hyperhidrosis, and Frey syndrome. Harlequin syndrome is often easily treated by discontinuing the anesthetic or adjusting placement of the epidural catheter. PMID:24882829

Boling, Bryan; Key, Christopher; Wainscott, Justin; Rebel, Annette

2014-06-01

168

A rare case of Kartagener's syndrome  

PubMed Central

A young boy presented with cough and intermittent breathlessness for 3 months. He used to suffer from frequent cough and cold since childhood. Clinical examination revealed bilateral coarse basal crepitations and rhonchi. His apex beat was on right 5th intercostal space in mid-clavicular line. Investigation revealed situs inversus, bi-lateral bronchiectasis, and chronic sinusitis. His semen analysis revealed the complete absence of sperm. The Saccharin test revealed impaired nasal ciliary movement. Considering all the finding, he was diagnosed as a case of Kartagener's syndrome. We are reporting this case because of its rarity and rare presence of aspermia in Kartagener's syndrome. PMID:24678221

Pandit, Sudipta; Choudhury, Sabyasachi; Das, Anirban; Basuthakur, Sumitra; Das, Sibes Kumar

2014-01-01

169

Fahr's disease: bilateral symmetrical striopallidodentate calcification in two brothers with two distinct presentations.  

PubMed

Bilateral striopallidodentate calcinosis, commonly known as Fahr's disease is a rare clinical entity present mainly with extrapyramidal signs and accompanied with metabolic, biochemical, neuroradiological and neuropsychiatric situations. It is characterised by the symmetrical and bilateral intracranial deposition of calcium associated with cell loss in the basal ganglia, cerebral cortex and cerebellum.In this study, we discussed two brothers' cases of Fahr's diseases who presented with different symptomatology. The first presented with walking difficulty, cramps and dysarthria and moderate memory impairment whereas the second with vertigo, ataxia, forgetfulness and headache. CT scans of both patients revealed intracranial diffuse bilateral calcifications in the basal ganglia and the cerebellum. The second patient revealed progressive cerebral atrophy but reduction in the calcification. Fahr's disease, although encountered rarely, should also be taken into account in the differential diagnosis of cases with abnormal intracranial calcifications along with other familial, congenital and metabolic diseases and syndromes. PMID:24014337

Bowirrat, Abdalla; Yassin, Mustafa; Artoul, Faozi; Artul, Suheil

2013-01-01

170

Submaximal Expression of the Bilateral Deficit  

ERIC Educational Resources Information Center

Thirty-six participants performed bilateral and unilateral isometric elbow flexion trials at what they perceived to be 100, 75, 50, and 25% of maximal effort. Absolute bilateral deficits ranged from -16% at 25% effort to -10% at 100% effort. The deficit included a component independent of consciousness and a component inversely related to…

McLean, Scott P.; Vint, Peter F.; Stember, Amanda J.

2006-01-01

171

HERITABILITY ESTIMATE FOR BILATERAL OVULATION IN HEIFERS  

Technology Transfer Automated Retrieval System (TEKTRAN)

Recent studies have reported that cows giving birth to twin calves as a result of bilateral ovulations have lower incidences of dystocia and higher calf survival than twins born from the same uterine horn. Therefore, the objective of this study was to determine if bilateral ovulation in heifers is g...

172

Vertigem posicional paroxística benigna bilateral monocanal  

Microsoft Academic Search

Purpose: To determine the prevalence of the simultaneous bilateral involvement of the same semicircular canal in patients with benign paroxysmal positional vertigo (BPPV). Method: Files from 2345 consecutive patients with of BPPV were analyzed. Results: Bilateral monocanal BPPV occurred in 252 (10.9%) patients, with age ranging from 39 to 81 years (average of 59.0 years), prevailing in the feminine gender

Marcela Rosana; Maia da Silveira; Heloísa Helena Caovilla

2007-01-01

173

Bilateral sporadic aniridia: review of management  

PubMed Central

Purpose To report a rare case of bilateral sporadic aniridia in an African child and review the management modalities. Presentation We report a case of bilateral sporadic aniridia with horizontal nystagmus, axial cataract optic disc, and fovea hypoplasia in a 5-year-old female patient. She was managed conservatively. Various modalities of treatment are reviewed. PMID:20957053

Adeoti, Caroline O; Afolabi, Adeyinka A; Ashaye, Adebimpe O; Adeoye, Adenike O

2010-01-01

174

Bilateral cystic nephroma with pleuropulmonary blastoma  

Microsoft Academic Search

Cystic nephroma is a rare benign renal neoplasm that is purely cystic and is lined by an epithelium. Bilateral cystic nephromas are even rarer with only a handful cases reported in the literature. A case of a 2-year-old male child who presented with bilateral renal cystic masses later diagnosed as cystic nephromas is presented here. Ultrasound, CT scan and histopathological

Anand Kumar Bhardwaj; Prem Dass Sharma; Amit Mittal; Akshay Sharma

2011-01-01

175

Eagle syndrome revisited: cerebrovascular complications.  

PubMed

Cervical pain caused by the elongation of the styloid process (Eagle syndrome) is well known to otolaryngologists but is rarely considered by vascular surgeons. We report two patients with cerebrovascular symptoms of Eagle syndrome treated in our medical center in the past year. Case 1: an 80-year-old man with acromegaly presented with dizziness and syncope with neck rotation. The patient was noted to have bilateral elongated styloid processes impinging on the internal carotid arteries. After staged resections of the styloid processes through cervical approaches, the symptoms resolved completely. Case 2: a 57-year-old man presented with acute-onset left-sided neck pain radiating to his head immediately after a vigorous neck massage. Hospital course was complicated by a 15-minute transient ischemic attack resulting in aphasia. Angiography revealed bilateral dissections of his internal carotid arteries, with a dissecting aneurysm on the right. Both injuries were immediately adjacent to the bilateral elongated styloid processes. Despite immediate anticoagulation therapy, he experienced aphasia and right hemiparesis associated with an occlusion of his left carotid artery. He underwent emergent catheter thrombectomy and carotid stent placement, with near-complete resolution of his symptoms. Elongated styloid processes characteristic of Eagle syndrome can result in both temporary impingement and permanent injury to the extracranial carotid arteries. Although rare, Eagle syndrome should be considered in the differential diagnosis in patients with cerebrovascular symptoms, especially those induced by positional change. PMID:22664285

Todo, Tsuyoshi; Alexander, Michael; Stokol, Colin; Lyden, Patrick; Braunstein, Glenn; Gewertz, Bruce

2012-07-01

176

Non Syndromic Familial Bilateral Decidious Taurodontism – A First Case Report  

PubMed Central

Taurodontism is anatomical and morphological alteration in the shape of the tooth. In taurodontism the crowns appear larger and the roots shorter compared to the normal anatomy of the respective tooth. The incidence of taurodontism in deciduous is very low and is also not very common in permanent dentition. The endodontic treatment protocol of taurodontic tooth is challenging. Taurodontic tooth requires a very cautious approach in identifying the root canal orientation and presence of any extra or lateral canals. PMID:25654041

Panigrahi, Rajat G.; K. T., Srilatha; Bhuyan, Ruchi; Bhuyan, Sanat K.

2014-01-01

177

[Bilateral carotid artery dissection in a severe preeclamptic setting: an unusual cause of postpartum headache].  

PubMed

A 30-year-old woman with severe preeclampsia presented at 27 weeks of amenorrhea with left headache, neck pain, blurred vision and numbness of left hemiface that resolved spontaneously within 2 hours. A week later, hypertension remained poorly controlled despite combination of nicardipine and labetalol intravenous therapy; an urgent caesarean section was eventually performed due to onset of HELLP syndrome. At day 5 postpartum, the patient had a Horner syndrome with right ipsilateral disabling tinnitus. A CT-angiography of supra-aortic trunks was performed urgently; it showed a bilateral carotid arterial dissection without stroke, which was subsequently confirmed by MRI angiography. The patient was transferred in neurovascular intensive care unit. Anticoagulant therapy was implemented to prevent cerebral and retinal ischemic lesions. Symptoms resolved quickly and the patient was discharged at day 7 postpartum. MR-angiography performed 4 months later showed a full resolution of the bilateral carotid dissection. Anticoagulant therapy was therefore discontinued. PMID:23498556

Hoffmann, C; Augé, M; Falzone, E; Martel-Jacob, S; Mercier, F J

2013-04-01

178

Tourette Syndrome  

MedlinePLUS

NINDS Tourette Syndrome Information Page Condensed from Tourette Syndrome Fact Sheet Table of Contents (click to jump to sections) ... Trials Organizations Additional resources from MedlinePlus What is Tourette Syndrome? Tourette syndrome (TS) is a neurological disorder ...

179

Tourette Syndrome  

MedlinePLUS

What Is Tourette Syndrome? Tourette syndrome is a condition that affects a person's central nervous system and causes tics. Tics are ... few months or a year. Continue Who Gets Tourette Syndrome? Tourette syndrome can affect people of all ...

180

Wallenberg's Syndrome  

MedlinePLUS

NINDS Wallenberg's Syndrome Information Page Synonym(s): Lateral Medullary Syndrome Table of Contents (click to jump to sections) What is ... is being done? Clinical Trials Organizations What is Wallenberg's Syndrome? Wallenberg’s syndrome is a neurological condition caused ...

181

Heterochronic bilateral ectopic pregnancy after ovulation induction*  

PubMed Central

Ectopic pregnancy is identified with the widely-applied assisted reproductive technology (ART). Bilateral ectopic pregnancy is a rare form of ectopic pregnancy which is difficult to be diagnosed at the pre-operation stage. In this paper, we presented an unusual case of heterochronic bilateral ectopic pregnancy after stimulated intrauterine insemination (IUI), where there has been a delay of 22 d between the diagnoses of the two ectopic pregnancies. Literature was reviewed on the occurrence of bilateral ectopic pregnancy during the past four years in the MEDLINE database. We found 16 cases of bilateral ectopic pregnancy reported since 2008, and analyzed the characteristics of those cases of bilateral ectopic pregnancy. We emphasize that ovulation induction and other ARTs may increase the risk of bilateral ectopic pregnancy. Because of the difficulty in identification of bilateral ectopic pregnancy by ultrasonography, the clinician should be aware that the treatment of one ectopic pregnancy does not preclude the occurrence of a second ectopic pregnancy in the same patient and should pay attention to the intra-operation inspection of both side fallopian tubes in any ectopic pregnancy case. PMID:25091994

Zhu, Bo; Xu, Gu-feng; Liu, Yi-feng; Qu, Fan; Yao, Wei-miao; Zhu, Yi-min; Gao, Hui-juan; Zhang, Dan

2014-01-01

182

Impaired threat prioritisation after selective bilateral amygdala lesions.  

PubMed

The amygdala is proposed to process threat-related information in non-human animals. In humans, empirical evidence from lesion studies has provided the strongest evidence for a role in emotional face recognition and social judgement. Here we use a face-in-the-crowd (FITC) task which in healthy control individuals reveals prioritised threat processing, evident in faster serial search for angry compared to happy target faces. We investigate AM and BG, two individuals with bilateral amygdala lesions due to Urbach-Wiethe syndrome, and 16 control individuals. In lesion patients we show a reversal of a threat detection advantage indicating a profound impairment in prioritising threat information. This is the first direct demonstration that human amygdala lesions impair prioritisation of threatening faces, providing evidence that this structure has a causal role in responding to imminent danger. PMID:25282058

Bach, Dominik R; Hurlemann, Rene; Dolan, Raymond J

2015-02-01

183

Impaired threat prioritisation after selective bilateral amygdala lesions  

PubMed Central

The amygdala is proposed to process threat-related information in non-human animals. In humans, empirical evidence from lesion studies has provided the strongest evidence for a role in emotional face recognition and social judgement. Here we use a face-in-the-crowd (FITC) task which in healthy control individuals reveals prioritised threat processing, evident in faster serial search for angry compared to happy target faces. We investigate AM and BG, two individuals with bilateral amygdala lesions due to Urbach–Wiethe syndrome, and 16 control individuals. In lesion patients we show a reversal of a threat detection advantage indicating a profound impairment in prioritising threat information. This is the first direct demonstration that human amygdala lesions impair prioritisation of threatening faces, providing evidence that this structure has a causal role in responding to imminent danger. PMID:25282058

Bach, Dominik R.; Hurlemann, Rene; Dolan, Raymond J.

2015-01-01

184

Bilateral Renal Infarction in a Lupus Patient: An Unusual Pathology  

PubMed Central

Acute renal infarction is still an underdiagnosed pathology. Most cases are secondary to arterial embolism in patients with atrial fibrillation or other cardiac illnesses; however, a less known etiology is the vascular affection of systemic lupus erythematosus (SLE). Renal infarction in lupus patients normally appears with positive antiphospholipid antibodies or lupus anticoagulant in the context of an antiphospholipid syndrome (APS). This is characterized by a state of hypercoagulability potentially affecting all segments of the vascular bed with thrombosis. A differential diagnosis with lupus nephritis, a very common pathology in SLE patients, must be carried out. We have to suspect this pathology in patients with SLE and APS who come to the emergency department complaining of abdominal pains or a renal colic. We present the case of a 69-year-old woman who was diagnosed of bilateral segmental renal infarction in the context of recently diagnosed SLE with no other vascular manifestations. PMID:23761987

Padilla-Fernández, Bárbara; García-Casado, Diana; Martín-Izquierdo, Manuela; Manzano-Rodríguez, Carmen; García-García, Javier; Lorenzo-Gómez, María F.

2013-01-01

185

The guided bilateral filter: when the joint/cross bilateral filter becomes robust.  

PubMed

The bilateral filter and its variants, such as the joint/cross bilateral filter, are well-known edge-preserving image smoothing tools used in many applications. The reason of this success is its simple definition and the possibility of many adaptations. The bilateral filter is known to be related to robust estimation. This link is lost by the ad hoc introduction of the guide image in the joint/cross bilateral filter. We here propose a new way to derive the joint/cross bilateral filter as a particular case of a more generic filter, which we name the guided bilateral filter. This new filter is iterative, generic, inherits the robustness properties of the robust bilateral filter, and uses a guide image. The link with robust estimation allows us to relate the filter parameters with the statistics of input images. A scheme based on graduated nonconvexity is proposed, which allows converging to an interesting local minimum even when the cost function is nonconvex. With this scheme, the guided bilateral filter can handle non-Gaussian noise on the image to be filtered. A complementary scheme is also proposed to handle non-Gaussian noise on the guide image even if both are strongly correlated. This allows the guided bilateral filter to handle situations with more noise than the joint/cross bilateral filter can work with and leads to high peak signal-to-noise ratio values as shown experimentally. PMID:25585418

Caraffa, Laurent; Tarel, Jean-Philippe; Charbonnier, Pierre

2015-04-01

186

A longitudinal study of the bilateral benefit in children with bilateral cochlear implants.  

PubMed

Objective: To study the development of the bilateral benefit in children using bilateral cochlear implants by measurements of speech recognition and sound localization. Design: Bilateral and unilateral speech recognition in quiet, in multi-source noise, and horizontal sound localization was measured at three occasions during a two-year period, without controlling for age or implant experience. Longitudinal and cross-sectional analyses were performed. Results were compared to cross-sectional data from children with normal hearing. Study sample: Seventy-eight children aged 5.1-11.9 years, with a mean bilateral cochlear implant experience of 3.3 years and a mean age of 7.8 years, at inclusion in the study. Thirty children with normal hearing aged 4.8-9.0 years provided normative data. Results: For children with cochlear implants, bilateral and unilateral speech recognition in quiet was comparable whereas a bilateral benefit for speech recognition in noise and sound localization was found at all three test occasions. Absolute performance was lower than in children with normal hearing. Early bilateral implantation facilitated sound localization. Conclusions: A bilateral benefit for speech recognition in noise and sound localization continues to exist over time for children with bilateral cochlear implants, but no relative improvement is found after three years of bilateral cochlear implant experience. PMID:25428567

Asp, Filip; Mäki-Torkko, Elina; Karltorp, Eva; Harder, Henrik; Hergils, Leif; Eskilsson, Gunnar; Stenfelt, Stefan

2014-11-27

187

[Symptomatic cyst of the pancreas and asymptomatic bilateral phaeochromocytoma  

PubMed

Symptomatic cyst of the pancreas and asymptomatic bilateral phaeochromocytoma. HISTORY AND CLINICAL FINDINGS: A 39-year-old woman was admitted to our department of gastroenterology with recurrent epigastrical pain. Ten years previously the diagnosis of von Hippel-Lindau (VHL)-syndrome has been established. Two years before a germ line mutation in exon 3 of the VHL-tumour suppressor gene has been detected. The patient has a healthy son with a normal VHL-gene and four healthy siblings who had refused a genetic blood test. INVESTIGATIONS: At abdominal ultrasound at the head of the pancreas three 4 2 cm large cysts and in the region of the left adrenal gland a 2,9 2,7 cm large tumor were visible. MRI of the abdomen revealed in addition a 2,2 1,5 cm large tumour of the right adrenal gland. An asymptomatic biadrenal phaeochromocytoma was detected by elevated urine catecholamines and 123I-MIBG-scintigraphy. TREATMENT AND COURSE: The cysts of the pancreas were punctured under endosonographical control and analysis of the cyst fluid was not suspicious of a malignant cystic tumour. The patient had no further abdominal complaints. After oral treatment with the alpha-blocker phentolamine the biadrenal phaeochromocytoma was treated by retroperitoneal laparoscopic surgery in an organ-sparing fashion. Postoperatively ACTH-stimulating test revealed a normal cortisol response. CONCLUSION: Adrenocortical function can be preserved by la-paroscopic adrenal-sparing surgery in bilateral phaeochromocytoma. PMID:12751023

Karvar, S; Breidert, M; Nagel, M; Kirsch, C; Pinkert, J; Ehninger, G

2001-01-01

188

Fibromuscular Dysplasia Presenting with Bilateral Renal Infarction  

SciTech Connect

Fibromuscular dysplasia (FMD) describes a group of conditions which cause nonatheromatous arterial stenoses, most commonly of the renal and carotid arteries, typically in young women. We report a rare case of bilateral segmental renal infarction secondary to FMD in a young male patient. His initial presentation with loin pain and pyrexia resulted in a delay in the definitive diagnosis of FMD. He was successfully treated with bilateral balloon angioplasty. The delayed diagnosis in this patient until the condition had progressed to bilateral renal infarcts highlights the need for prompt investigation and diagnosis of suspected cases of FMD.

Doody, O., E-mail: orla_doody@hotmail.co [Alfred Hospital, Department of Radiology (Australia); Adam, W. R. [University of Melbourne and Goulburn Valley Health, School of Rural Health (Australia); Foley, P. T.; Lyon, S. M. [Alfred Hospital, Department of Radiology (Australia)

2009-03-15

189

Adult-onset bulbar ptosis in Joubert syndrome.  

PubMed

In this case report, we describe a case of adult-onset bulbar ptosis in a patient with Joubert syndrome. Joubert syndrome is a rare neurodevelopmental disorder with malformations in cerebellum and brainstem. Many ocular abnormalities have been noted in Joubert syndrome, but the association of this syndrome with adult-onset ptosis has not been described to date. This 24-year-old Joubert patient developed a cerebrospinal fluid cyst in her midbrain. She had signs of bilateral third nerve palsy and abducens palsy in the left eye. The bilateral central third nerve palsy causing functional blindness secondary to severe bilateral levator palsy was treated successfully with silicone sling frontalis suspension, as the seventh nerve nucleus was not involved. PMID:22291457

Burt, Benjamin; Levine, Johanan; Le, Kim

2012-01-01

190

Bilateral pneumothorax after orthognatic surgery  

PubMed Central

Among complications in orthognathic surgery, the insurgence of pneumothorax is very rare. Pneumothorax is the presence of air or gas in the pleural cavity and it is rare complications in the postoperative oral and maxillofacial surgery patient. The clinical results are dependent on the degree of collapse of the lung on the affected side. Pneumothorax can impair oxygenation and/or ventilation. If the pneumothorax is significant, it can cause a shift of the mediastinum and compromise haemodynamic stability. While 10% of pneumothoraces are asymptomatic, patients often complain of acute chest pain and difficulty breathing. There is a reduction in vital capacity, tachycardia, tachypnoea and a decrease in partial pressure of oxygen with an inability to maintain oxygen saturations. We observed this unusual surgical consequence in a 28-year-old female with negative clinical history and instrumental evaluation after Le Fort I osteotomy and bilateral sagittal split osteotomy (BSSO). No further consequences, no neurological sequelae, no infections and no other osteotomies sequelae were seen. Sudden post-surgical dispnea associated to sub-cutaneous emphysema of the neck and of the thorax must be adequately observed with the aim of monitoring further severe sequelae. The anaesthetic management of the emergency difficult airway in any post-surgical orthognatic treatment can be extremely difficult requiring a multi-disciplinary approach. PMID:23814593

Bertossi, Dario; Malchiodi, Luciano; Turra, Matteo; Bondi, Vincenzo; Albanese, Massimo; Lucchese, Alessandra; Carinci, Francesco; Nocini, Pierfrancesco

2012-01-01

191

Bilateral symmetry across Aphrodite Terra  

NASA Technical Reports Server (NTRS)

There are three main highland areas on Venus: Beta Regio, Ishtar Terra and Aphrodite Terra. The latter is least known and the least mapped, yet existing analyses of Aphrodite Terra based on available Pioneer-Venus orbiter data suggest that it may be the site of extensive rifting. Some of the highest resolution (30 km) PV data (SAR) included most of the western half of Aphrodite Terra. Recent analysis of the SAR data together with Arecibo range-doppler topographic profiling (10 X 100 km horizontal and 10 m vertical resolution) across parts of Aphrodite, further characterized the nature of possible tectonic processes in the equatorial highlands. The existence of distinct topographic and radar morphologic linear discontinuities across the nearly east-west strike of Aphrodite Terra is indicated. Another prominent set of linear features is distinctly parallel to and orthogonal to the ground tracks of the PV spacecraft and are not included because of the possibility that they are artifacts. Study of the northwest trending cross-strike discontinuities (CSD's) and the nature of topographic and morphologic features along their strike suggest the presence of bilateral topographic and morphologic symmetry about the long axis of Aphrodite Terra.

Crumpler, L. S.; Head, J. W.; Campbell, D. B.

1987-01-01

192

Slipping-rib syndrome.  

PubMed

A survey of 46 patients with the slipping-rib syndrome shows that it is a condition which affects mainly middle-aged people. It is equally common in men and women. The main symptom is upper abdominal pain, equally common on either side and occasionally bilateral. The pain is precipitated by movement and certain postures and is faithfully reproduced by pressure at one or more points on the costal margin. The cause of the "slipping rib" is not known. Symptoms are relieved by reassurance and in some cases by injection of local anaesthetic into painful sites. PMID:6107417

Wright, J T

1980-09-20

193

Prune belly syndrome.  

PubMed

The majority of paediatric surgeons will encounter a patient with prune belly syndrome (PBS) only a few times in their clinical practice. There have been many opposing views in the literature regarding the pathogenesis and management of this complex condition. A detailed review was conducted using PubMed to identify key publications involving PBS. This article discusses the evolution of our understanding of the pathogenesis and diagnosis of PBS, including its typical characteristics. We describe the management options available for bilateral intra-abdominal testes, the deficient abdominal wall, the dilated urinary system and examine the evidence base used to support the current approaches employed. PMID:22198807

Hassett, S; Smith, G H H; Holland, A J A

2012-03-01

194

Bilateral, Zero-Impedance Static Semiconductor Switch  

NASA Technical Reports Server (NTRS)

Static semiconductor switching circuit eliminates the undesirable features of electromechanical relays and conventional semiconductor switching circuits. There is a net zero voltage drop at the terminals and thus a zero impedance for bilateral currents there.

Doughman, C. L.

1968-01-01

195

Management of ovarian remnant syndrome.  

PubMed

Ovarian remnant syndrome is a rare condition which develops when functional ovarian tissue which is left in situ after intended bilateral oophorectomy produces clinically significant symptoms, namely chronic pelvic pain and/or dyspareunia. Women with this syndrome may have a pelvic mass palpable on bimanual examination, or visible on transvaginal ultrasound. However, visualization of an ovarian remnant is sometimes difficult because it is small and multiple previous pelvic operations have usually produced dense adhesions and distorted pelvic anatomy. Identification of these remnants may be facilitated by ovarian stimulation. Surgical removal is the optimal treatment, but hormonal suppression of ovarian activity may provide temporary relief of pain. PMID:9006836

Orford, V P; Kuhn, R J

1996-11-01

196

Renal function studies in an infant with 4p (-) syndrome.  

PubMed Central

An infant with the syndrome of deletion of the short arm of chromosome 4 is described. In addition, this child had renal insufficiency, which is found rarely in association with the 4p(--) syndrome. Previous reports of this syndrome have described only isolated gross structural abnormalites of the urinary tract. In the case discussed here, we present clinical and functional data which indicate that this patient had bilateral renal dysplasia. Images PMID:1018312

Schoeneman, M; Nitowsky, M M

1976-01-01

197

Cowden Syndrome Presenting as Breast Cancer: Imaging and Clinical Features  

PubMed Central

Cowden syndrome is an uncommon, autosomal dominant disease which is characterized by multiple hamartomas of the skin, mucous membrane, brain, breast, thyroid, and gastrointestinal tract. The diagnosis of Cowden syndrome implicates an increased risk of developing breast cancer. We report a case of a 22-year-old woman with Cowden syndrome that presented as breast cancer with concomitant bilateral exuberant benign masses in both breasts. PMID:25246819

Seo, Mirinae; Ahn, Hye Shin; Moon, Hyeong-Gon

2014-01-01

198

Bilateral hippocampal dysfunction in schizophrenia.  

PubMed

The hippocampus has long been known to be important for memory, with the right hippocampus particularly implicated in nonverbal/visuo-spatial memory and the left in verbal/narrative or episodic memory. Despite this hypothesized lateralized functional difference, there has not been a single task that has been shown to activate both the right and left hippocampi differentially, dissociating the two, using neuroimaging. The transverse patterning (TP) task is a strong candidate for this purpose, as it has been shown in human and nonhuman animal studies to theoretically and empirically depend on the hippocampus. In TP, participants choose between stimuli presented in pairs, with the correct choice being a function of the specific pairing. In this project, TP was used to assess lateralized hippocampal function by varying its dependence on verbal material, with the goal of dissociating the two hippocampi. Magnetoencephalographic (MEG) data were collected while controls performed verbal and nonverbal versions of TP in order to verify and validate lateralized activation within the hippocampi. Schizophrenia patients were evaluated to determine whether they exhibited a lateralized hippocampal deficit. As hypothesized, patients' mean level of behavioral performance was poorer than controls' on both verbal and nonverbal TP. In contrast, patients had no decrement in performance on a verbal and nonverbal non-hippocampal-dependent matched control task. Also, controls but not patients showed more right hippocampal activation during nonverbal TP and more left hippocampal activation during verbal TP. These data demonstrate the capacity to assess lateralized hippocampal function and suggest a bilateral hippocampal behavioral and activation deficit in schizophrenia. PMID:21763438

Hanlon, Faith M; Houck, Jon M; Pyeatt, Clinton J; Lundy, S Laura; Euler, Matthew J; Weisend, Michael P; Thoma, Robert J; Bustillo, Juan R; Miller, Gregory A; Tesche, Claudia D

2011-10-15

199

Bilateral tension pneumothorax related to acupuncture.  

PubMed

We report on a patient with a rare case of bilateral tension pneumothorax that occurred after acupuncture. A 69-year-old large-bodied man, who otherwise had no risk factors for spontaneous pneumothorax, presented with chest pressure, cold sweats and shortness of breath. Immediately after bilateral pneumothorax had been identified on a chest radiograph in the emergency room, his blood pressure and percutaneous oxygen saturation suddenly decreased to 78 mm Hg and 86%, respectively. We confirmed deterioration in his cardiopulmonary status and diagnosed bilateral tension pneumothorax. We punctured his chest bilaterally and inserted chest tubes for drainage. His vital signs promptly recovered. After the bilateral puncture and drainage, we learnt that he had been treated with acupuncture on his upper back. We finally diagnosed a bilateral tension pneumothorax based on the symptoms that appeared 8 h after the acupuncture. Because the patient had no risk factors for spontaneous pneumothorax, no alternative diagnosis was proposed. We recommend that patients receiving acupuncture around the chest wall must be adequately informed of the possibility of complications and expected symptoms, as a definitive diagnosis can be difficult without complete information. PMID:23449179

Tagami, Rumi; Moriya, Takashi; Kinoshita, Kosaku; Tanjoh, Katsuhisa

2013-06-01

200

[Diagnostic image (70). A man with fever, swollen joints and erythema nodosum. Loefgren's syndrome].  

PubMed

A 39-year-old man presented with fever, arthritis of knees and wrists, periarticular ankle inflammation, erythema nodosum, bilateral hilar adenopathy and diffuse pulmonary parenchymal changes, due to Löfgren's syndrome. PMID:11802332

Hofstee, H M A; Folmer, S C C Reinders

2002-01-01

201

Prenatal diagnosis of VACTERL syndrome and partial caudal regression syndrome: a previously unreported association.  

PubMed

We describe a case of VACTERL syndrome associated with type 1 unilateral caudal regression syndrome. The abnormal sonographic findings at 26 weeks included hemivertebrae, scoliosis, hypoplastic and deformed lumbar spine and sacrum, preaxial polydactyly on the left hand, duplicated hallux on the left foot and hemihypoplasia of the left lower limb, bilateral club foot, and single umbilical artery. Postmortem examination confirmed prenatal sonographic findings with additional findings of supernumerary rib at the lumbar level and anal atresia. PMID:19536863

Gedikbasi, Ali; Yararbas, Kanay; Yildirim, Gokhan; Yildirim, Dogukan; Arslan, Oguz; Gul, Ahmet; Ceylan, Yavuz

2009-10-01

202

Prenatal diagnosis of the Meckel syndrome.  

PubMed

Prenatal diagnosis of the Meckel syndrome was made at 20 weeks of gestation from the findings of a biparietal diameter smaller than expected for gestational age, a grossly raised amniotic fluid alphafetoprotein level and a rapid growth of foetal macrophages after 20 hours culture. Termination at 23 weeks of gestation resulted in a male foetus with an occipital encephalocele, microcephaly, polydactyly, and bilateral polycystic kidneys. This case report emphasies the importance for genetic counselling of delineating the Meckel syndrome from the multifactorial cases of neural tube defects, and also illustrates, at least in some cases, that the syndrome can be diagnosed in utero. PMID:83212

Nevin, N C; Thompson, W; Davison, G; Horner, W T

1979-01-01

203

Syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and Gerhardt syndrome associated with Shy-Drager syndrome.  

PubMed

This is the first report on a case of syndrome of inappropriate secretion of antidiuretic hormone (SIADH) associated with Gerhardt syndrome (paralysis of bilateral vocal cords). A 67-year-old Japanese man suffering from progressive autonomic failure was diagnosed as having Shy-Drager syndrome (SDS) with hyponatremia due to SIADH and severe sleep apnea caused by a bilateral recurrent nerve palsy. Water load test showed alteration in diuresis which was corrected by phenytoin. Arginine vasopressin secretion was not suppressed by plasma osmolality below 280 mOsm/kgH2O. Impairment of the afferent pathways of baroreceptors, or impairment of the osmoreceptors could be speculated as the etiological factor of the SIADH observed in this case. PMID:7718959

Sone, H; Okuda, Y; Bannai, C; Suzuki, S; Yamaoka, T; Asakura, Y; Kawakami, Y; Odawara, M; Matsushima, T; Kawai, K

1994-12-01

204

Laparoscopic simultaneous bilateral adrenalectomy for testosterone-secreting bilateral adrenal tumors.  

PubMed

Laparoscopic adrenalectomy is widely accepted as a safe and minimally-invasive procedure. Although it is a standard procedure for the surgical treatment of adrenal tumors, its simultaneous use with bilateral adrenalectomy is relatively rare. A 21-year-old woman was referred to Hamamatsu University School of Medicine University Hospital complaining of a deepening voice, hirsutism and secondary amenorrhea. Abdominal computed tomography showed bilateral adrenal tumors, and hormonal examinations showed that the tumors secreted excessive testosterone, resulting in virilizing symptoms. Laparoscopic simultaneous bilateral adrenalectomy was carried out. Postoperatively, serum testosterone levels immediately recovered to within the normal range. Menstruation began the month after the operation, and the hirsutism gradually regressed. This is the third reported case of bilateral virilizing adrenal tumors, and the first to be successfully treated with laparoscopic simultaneous bilateral adrenalectomy. PMID:23601096

Suzuki, Takahisa; Furuse, Hiroshi; Kurita, Yutaka; Ushiyama, Tomomi; Mugiya, Soichi; Ozono, Seiichiro; Oki, Yutaka

2014-01-01

205

GPR56-related bilateral frontoparietal polymicrogyria: further evidence for an overlap with the cobblestone complex.  

PubMed

GPR56 mutations cause an autosomal recessive polymicrogyria syndrome that has distinctive radiological features combining bilateral frontoparietal polymicrogyria, white matter abnormalities and cerebellar hypoplasia. Recent investigations of a GPR56 knockout mouse model suggest that bilateral bifrontoparietal polymicrogyria shares some features of the cobblestone brain malformation and demonstrate that loss of GPR56 leads to a dysregulation of the maintenance of the pial basement membrane integrity in the forebrain and the rostral cerebellum. In light of these findings and other data in the literature, this study aimed to refine the clinical features with the first description of a foetopathological case and to define the range of cobblestone-like features in GPR56 bilateral bifrontoparietal polymicrogyria in a sample of 14 patients. We identified homozygous GPR56 mutations in 14 patients from eight consanguineous families with typical bilateral bifrontoparietal polymicrogyria and in one foetal case, out of 30 patients with bifrontoparietal polymicrogyria referred for molecular screening. The foetal case, which was terminated at 35 weeks of gestation in view of suspicion of Walker Warburg syndrome, showed a cobblestone-like lissencephaly with a succession of normal, polymicrogyric and 'cobblestone-like' cortex with ectopic neuronal overmigration, agenesis of the cerebellar vermis and hypoplastic cerebellar hemispheres with additional neuronal overmigration in the pons and the cerebellar cortex. The 14 patients with GPR56 mutations (median 8.25 years, range 1.5-33 years) were phenotypically homogeneous with a distinctive clinical course characterized by pseudomyopathic behaviour at onset that subsequently evolved into severe mental and motor retardation. Generalized seizures (12/14) occurred later with onset ranging from 2.5 to 10 years with consistent electroencephalogram findings of predominantly anterior bursts of low amplitude ?-like activity. Neuroimaging demonstrated a common phenotype with bilateral frontoparietally predominant polymicrogyria (13/13), cerebellar dysplasia with cysts mainly affecting the superior vermis (11/13) and patchy to diffuse myelination abnormalities (13/13). Additionally, the white matter abnormalities showed a peculiar evolution from severe hypomyelination at 4 months to patchy lesions later in childhood. Taken as a whole, these observations collectively demonstrate that GPR56 bilateral bifrontoparietal polymicrogyria combines all the features of a cobblestone-like lissencephaly and also suggest that GRP56-related defects produce a phenotypic continuum ranging from bilateral bifrontoparietal polymicrogyria to cobblestone-like lissencephaly. PMID:20929962

Bahi-Buisson, Nadia; Poirier, Karine; Boddaert, Nathalie; Fallet-Bianco, Catherine; Specchio, Nicola; Bertini, Enrico; Caglayan, Okay; Lascelles, Karine; Elie, Caroline; Rambaud, Jérôme; Baulac, Michel; An, Isabelle; Dias, Patricia; des Portes, Vincent; Moutard, Marie Laure; Soufflet, Christine; El Maleh, Monique; Beldjord, Cherif; Villard, Laurent; Chelly, Jamel

2010-11-01

206

Bilateral normal filtering for mesh denoising.  

PubMed

Decoupling local geometric features from the spatial location of a mesh is crucial for feature-preserving mesh denoising. This paper focuses on first order features, i.e., facet normals, and presents a simple yet effective anisotropic mesh denoising framework via normal field denoising. Unlike previous denoising methods based on normal filtering, which process normals defined on the Gauss sphere, our method considers normals as a surface signal defined over the original mesh. This allows the design of a novel bilateral normal filter that depends on both spatial distance and signal distance. Our bilateral filter is a more natural extension of the elegant bilateral filter for image denoising than those used in previous bilateral mesh denoising methods. Besides applying this bilateral normal filter in a local, iterative scheme, as common in most of previous works, we present for the first time a global, noniterative scheme for an isotropic denoising. We show that the former scheme is faster and more effective for denoising extremely noisy meshes while the latter scheme is more robust to irregular surface sampling. We demonstrate that both our feature-preserving schemes generally produce visually and numerically better denoising results than previous methods, especially at challenging regions with sharp features or irregular sampling. PMID:21173457

Zheng, Youyi; Fu, Hongbo; Au, Oscar Kin-Chung; Tai, Chiew-Lan

2011-10-01

207

[Familial Wolfram syndrome].  

PubMed

Wolfram syndrome (WS) is a rare autosomal recessive progressive neurodegenerative disorder, and it is mainly characterized by the presence of diabetes mellitus and optic atrophy. Other symptoms such as diabetes insipidus, deafness, and psychiatric disorders are less frequent. The WFS1 gene, responsible for the disease and encoding for a transmembrane protein called wolframin, was localized in 1998 on chromosome 4p16. In this report, we present a familial observation of Wolfram syndrome (parents and three children). The propositus was a 6-year-old girl with diabetes mellitus and progressive visual loss. Her family history showed a brother with diabetes mellitus, optic atrophy, and deafness since childhood and a sister with diabetes mellitus, optic atrophy, and bilateral hydronephrosis. Thus, association of these familial and personal symptoms is highly suggestive of Wolfram syndrome. The diagnosis was confirmed by molecular analysis (biology), which showed the presence of WFS1 homozygous mutations c.1113G>A (p.Trp371*) in the three siblings and a heterozygote mutation in the parents. Our observation has demonstrated that pediatricians should be aware of the possibility of Wolfram syndrome when diagnosing optic atrophy in diabetic children. PMID:25282462

Bessahraoui, M; Paquis, V; Rouzier, C; Bouziane-Nedjadi, K; Naceur, M; Niar, S; Zennaki, A; Boudraa, G; Touhami, M

2014-11-01

208

Down Syndrome  

MedlinePLUS

... Information Clinical Trials Resources and Publications En Español Down Syndrome: Condition Information Skip sharing on social media links Share this: Page Content What is Down syndrome? Down syndrome describes a set of cognitive and ...

209

Brown Syndrome  

MedlinePLUS

... Does Brown syndrome cause eye problems besides abnormal eye movements? Some children with Brown syndrome have poor binocular ... In the congenital form of Brown syndrome, the eye movement problem is usually constant and unlikely to resolve ...

210

Metabolic Syndrome  

MedlinePLUS

... is a signal that someone could be on the road to serious health problems. Diagnosing metabolic syndrome helps ... like heart disease or type 2 diabetes down the road. What Exactly Is Metabolic Syndrome? Metabolic syndrome is ...

211

Williams syndrome  

MedlinePLUS

Williams-Beuren syndrome ... Williams syndrome is a rare condition caused by missing a copy of several genes. Parents may not have ... history of the condition. However, a person with Williams syndrome has a 50% chance of passing the disorder ...

212

Angelman Syndrome  

MedlinePLUS

... being done? Clinical Trials Organizations What is Angelman Syndrome? Angelman syndrome is a genetic disorder that causes developmental ... and ultimately cure them. NIH Patient Recruitment for Angelman Syndrome Clinical Trials At NIH Clinical Center Throughout the ...

213

Bilateral self-inflicted infectious dacryoadenitis.  

PubMed

The aim of this report is to present a case of a patient with bilateral lacrimal gland abscesses in the course of dacryoadenitis. A 45-year-old female patient with a long history of cocaine abuse presented with bilateral bacterial dacryoadenitis and upper lid inflammation with purulent discharge from a palpebral wound of the right upper lid. The diagnosis was confirmed with microbiology culture and an orbital CT scan, which revealed lacrimal gland abscesses. The patient admitted to vigorous eye scratching, which we believe was the mechanism responsible for the process. The infection resolved on targeted antibiotic therapy. This is the first reported case of bilateral infectious dacryoadenitis produced in a self-inflicted mechanism in a cocaine addict. PMID:25208047

Latasiewicz, Marta; Chang-Sotomayor, Meilin; Alonso-Caldarelli, Claudia; Farias-Plazas, Fabian; Leszczynska, Anna; Gonzalez-Candial, Miguel

2014-12-01

214

Bilateral filtering of magnetic resonance phase images.  

PubMed

High-pass filtering is required for the removal of background field inhomogeneities in magnetic resonance phase images. This high-pass filtering smooths across boundaries between areas with large differences in phase. The most prominent boundary is the surface of the brain where areas with large phase values inside the brain are located close to areas outside the brain where the phase is, on average, zero. Cortical areas, which are of great interest in brain MRI, are therefore often degraded by high-pass filtering. Here, we propose the use of the bilateral filter for the high-pass filtering step. The bilateral filter is essentially a Gaussian filter that stops smoothing at boundaries. We show that the bilateral filter improves image quality at the brain's surface, without sacrificing contrast within the brain. PMID:21664782

McPhee, Kelly C; Denk, Christian; Al-Rekabi, Zeinab; Rauscher, Alexander

2011-09-01

215

Bilateral olfactory sensory input enhances chemotaxis behavior.  

PubMed

Neural comparisons of bilateral sensory inputs are essential for visual depth perception and accurate localization of sounds in space. All animals, from single-cell prokaryotes to humans, orient themselves in response to environmental chemical stimuli, but the contribution of spatial integration of neural activity in olfaction remains unclear. We investigated this problem in Drosophila melanogaster larvae. Using high-resolution behavioral analysis, we studied the chemotaxis behavior of larvae with a single functional olfactory neuron on either the left or right side of the head, allowing us to examine unilateral or bilateral olfactory input. We developed new spectroscopic methods to create stable odorant gradients in which odor concentrations were experimentally measured. In these controlled environments, we observed that a single functional neuron provided sufficient information to permit larval chemotaxis. We found additional evidence that the overall accuracy of navigation is enhanced by the increase in the signal-to-noise ratio conferred by bilateral sensory input. PMID:18157126

Louis, Matthieu; Huber, Thomas; Benton, Richard; Sakmar, Thomas P; Vosshall, Leslie B

2008-02-01

216

Fahr’s Syndrome- An Interesting Case Presentation  

PubMed Central

Fahr’s syndrome refers to a rare syndrome which is characterized by symmetrical and bilateral intracranial calcification. We are presenting a 63 year old male, who complained of progressive dysarthria of 6 months, which was associated with slowness of movements. His neurological examination revealed Parkinsonian features. His CT scan revealed a symmetrical large area of calcification over the basal ganglia, the thalamus and the cerebellum. The secondary causes of the bilateral calcification were ruled out to make the clinical diagnosis of Idiopathic Bilateral Striopallidodentate Calcinosis, which is otherwise called as the Fahr’s syndrome. Deposits of calcium and minerals cannot be linked to a single chromosomal locus and further genetic studies are in progress for identifying the chromosomal locus of the disease. PMID:23634413

Asokan, Athulya Geetha; D’souza, Sydney; Jeganathan, Jayakumar; Pai, Shivananda

2013-01-01

217

Bilateral Femoral Neck Fracture-Related Hyperparathyroidism  

PubMed Central

Bilateral femoral neck fracture is not common as unilateral femoral fracture. Femoral neck fracture generally occurs by the high energized traumas. Traffic accidents and fallings are the most common reason for this fracture kind. But suddenly and minor traumatic fractures is not common. Especially, in the hormonal and pathogenic fractures is not common. In this case minor traumatic bilateral femoral fracture is presented. The fracture occurs in the background of critical medical condition by hyperparathyroidism. It can be said chronic hyperparathyroidism conditions must be determined for femoral neck fracture. Because these patients many times fell little disturbed by this fracture, diagnosis can be missed many times.

Ezirmik, Naci; Yildiz, Kadri; Cadirci, Kenan

2011-01-01

218

Spontaneous Bilateral Meningoencephalocoeles of the Temporal Bones  

PubMed Central

Spontaneous tegmen tympani defects are rare with even rarer bilateral cases. The symptoms are nonspecific; hence, a high index of suspicion is required to prevent serious intracranial complications. We present a case of spontaneous bilateral tegmen tympani defects with associated meningoencephalocoeles in a 54-year-old male who presented with the signs and symptoms of severe meningitis. After careful workup which included a lumbar puncture, CT and MRI scans, both defects were repaired using a middle fossa approach. The patient made an uneventful recovery with complete cessation of otorrhoea and improvement in his hearing. PMID:24288640

2013-01-01

219

Isolated Bilateral Congenital Iris Sphincter Agenesis  

PubMed Central

Purpose. We herein report a patient with bilateral congenital total iris sphincter agenesis with no other abnormality detected on systemic examination. Methods. A 24-year-old laborer presented to us for a routine checkup with complaint of photophobia and inability to work under sunlight. Examination revealed bilateral absence of sphincter and 6.5?mm pupil in both eyes in the undilated state. Results. Accommodation was poor in both eyes. Systemic examination was within normal limits. He was prescribed bifocal photochromic glasses for constant wear. Conclusions. Congenital sphincter agenesis can occur in an isolated form without systemic abnormalities which can be managed conservatively. PMID:22606462

Rao, Aparna

2011-01-01

220

Bilateral Metallic Stenting in Malignant Hilar Obstruction  

PubMed Central

Endoscopic palliative biliary drainage is considered as a gold standard treatment in advanced or inoperable hilar cholangiocarcinoma. Also, metal stents are preferred over plastic stents in patients with >3 months life expectancy. However, the endoscopic intervention of advanced hilar obstruction is often more challenging and complex than that of distal malignant biliary obstructions. In this literature review, we describe the issues commonly encountered during endoscopic unilateral (single) versus bilateral (multiple) biliary stenting for malignant hilar obstruction. Also, we provide technical guidance to improve the technical success rates and patient outcomes, focusing on bilateral metallic stenting techniques such as stent-in-stent or side-by-side deployment. PMID:25325005

Moon, Jong Ho; Park, Sang-Heum

2014-01-01

221

Bilateral Hypertrophic Olivary Degeneration in Wilson Disease  

PubMed Central

Hypertrophic olivary degeneration resulting from lesions of the dento-rubro-olivary pathway, also called Guillain-Mollaret-triangle, has been described previously in a number of cases. Reports about bilateral hypertrophic olivary degeneration of the inferior olivary nuclei are very limited, and the magnetic resonance imaging findings of hypertrophic olivary degeneration in Wilson disease have not yet been described to the best of our knowledge. Herein, we present the first report of bilateral hypertrophic olivary degeneration diagnosed by magnetic resonance imaging in a patient suffering from Wilson disease. PMID:23482821

Guenther, Peter; Hoffmann, Karl-Titus

2013-01-01

222

Bilateral Simultaneous Stage 1 Macular Hole  

PubMed Central

The authors describe two cases of bilateral simultaneous stage 1 macular hole diagnosed via optical coherence tomography (OCT). Vitreomacular traction, foveal pseudo cysts, and outer retinal changes were present initially. Resolution of the foveal pseudo cysts and outer retinal changes occurred in cases where a complete posterior vitreous detachment was noted. The initial step in the pathogenesis of macular holes is “traction” from the detaching posterior hyaloid. However, there are missing links in the exact inciting events and in the progression of the disease. The development of bilateral stage 1 macular holes simultaneously is unique and interesting because an unknown common inciting factor might be at play in these cases. PMID:23053850

Alwassia, Ahmad A.; Adhi, Mehreen; Duker, Jay S.

2014-01-01

223

Second-Side Surgery in Superior Canal Dehiscence Syndrome  

PubMed Central

Objective Bilateral superior canal (SC) dehiscence syndrome poses a challenge because bilateral SC dehiscence (SCD) plugging might be expected to result in oscillopsia and disability. Our aims were as follows: 1) to evaluate which symptoms prompted patients with bilateral SCD syndrome (SCDS) to seek second-side surgery, and 2) to determine the prevalence of disabling imbalance and oscillopsia after bilateral SC plugging. Study Design Prospective observational study. Setting Tertiary referral center. Patients Five patients with bilateral SCDS based on history, audiometric and physiologic testing, and computed tomographic findings. This includes all of our patients who have had second-side plugging surgery to date. Intervention(s) Bilateral sequential middle fossa craniotomy and plugging of SCs. Main Outcome Measure(s) Cochleovestibular symptoms, cervical and ocular vestibular-evoked myogenic potential testing, dizziness handicap inventory, short-form 36 Health Survey, dynamic visual acuity testing. Results The most common symptoms prompting second-side surgery were sound- and pressure-induced vertigo and autophony. Three of the 5 patients reported that symptoms shifted to the contralateral ear immediately after plugging the first side, whereas in 2 patients, contralateral symptoms developed several years after the first SC plugging. Two of 4 patients experienced ongoing oscillopsia after bilateral SCDS surgery; however, all patients reported relief from their SCD symptoms and were glad that they had pursued bilateral surgery. Conclusion In patients with bilateral SCDS, sound- and pressure-induced vertigo most commonly prompted second-side surgery. Despite some degree of oscillopsia after bilateral SCDS surgery, patients were very satisfied with second-side surgery, given their relief from other SCDS symptoms. PMID:22158019

Agrawal, Yuri; Minor, Lloyd B.; Schubert, Michael C.; Janky, Kristen L.; Davalos-Bichara, Marcela; Carey, John P.

2014-01-01

224

Bilateral Simultaneous Disc Edema and Cataract Associated with Albright Hereditary Osteodystrophy  

PubMed Central

A 16-year-old female presented with poor vision in both eyes. On clinical examination, she had bilateral cataracts and optic disc edema bilaterally on ultrasound examination. Extensive intracranial calcification was evident on computerized tomography. Physical examination revealed short stature, rounded chubby face, dental abnormalities, brachydactyly, and obesity. Laboratory evidence of hypocalcemia, hyperphosphatemia, elevated parathyroid hormone level (indicative of pseudohypoparathyroidism) along with the constellation of phenotypical characteristics lead to a diagnosis of Albright's hereditary osteodystrophy. This case is being presented to increase awareness regarding presence of coexisting and previously undiagnosed hypocalcemic syndromes in pediatric cataracts. The role of an ophthalmologist may be pivotal in diagnosing such an entity as documented in the present case. PMID:22346135

Sengupta, Sabyasachi; Ravindran, Ravilla D.; Kannusamy, Veena; Tamrakar, Varsha

2012-01-01

225

Neu-Laxova syndrome: a case report.  

PubMed

Neu-Laxova syndrome (NLS) is a rare lethal syndrome found in both consanguinous and non-consanguinous couple. This is characterized by terrible face with unusual craniofacial appearance with exophthalmos, spectrum of central nervous system malformation, like microcaphaly, hypoplastic cerebellum, cleft lip/palate, ichthyosis and oedema. The diagnosis is made on the basis of clinical parameter. We report a 4 hour old male term newborn with IUGR of Neu-Laxova syndrome presented with anencephaly, rudimentary cerebellum, exophthalmos of right eye, bilateral cleft lip and palate and cryptorchidism. Anencephaly and cryptorchidism are two recently reported findings of NLS. We are presenting this case in addition from Bangladesh to lend further support to those two new findings as component of Neu-Laxova syndrome. Outcome of this syndrome is not good. Most of the patients are died of infection within hours to days. PMID:24584393

Roy, S; Begum, J; Sharifunnaher, B; Saha, A K; Afroza, S; Islam, M Z

2014-01-01

226

Chromatin and transcriptional regulators act in a cascade to establish a bilateral asymmetry of the C. elegans nervous system  

E-print Network

Neuroanatomical bilateral asymmetry is a widespread feature in both vertebrates and invertebrates. Although mostly bilaterally symmetric, the nervous system of Caenorhabditis elegans displays bilateral asymmetry. Bilateral ...

Nakano, Shunji, Ph. D. Massachusetts Institute of Technology

2011-01-01

227

Bilateral synergies in foot force production tasks.  

PubMed

We analysed the effects of task symmetry during bilateral accurate force production tasks performed by the two feet. In particular, we tested a hypothesis that bilateral deficit would lead to higher indices of synergies defined as co-varied adjustments in the two forces across trials that reduced total force variability. The subjects produced steady-state force followed by a quick force pulse into the target. The two feet could be acting both into plantar flexion and into dorsiflexion (symmetrical tasks), or in opposite directions (asymmetrical task). We used the framework of the uncontrolled manifold hypothesis to quantify two variance components, one of which did not change total force (V UCM), while the other did (V ORT). Synergy indices during the asymmetrical task were higher than in either symmetrical task. The difference was due to higher V UCM (compared to the symmetrical plantar flexion task) or lower V ORT (compared to the symmetrical dorsiflexion task). The synergy index showed a drop (anticipatory synergy adjustment, ASA) starting 100-150 ms prior to the force pulse initiation. The ASA tended to be shorter and of a smaller magnitude for the asymmetrical task. This is the first demonstration of bilateral synergies during accurate force production by the legs. We conclude that bilateral deficit has no or weak effects on two-leg synergies. The results fit the earlier introduced scheme with two groups of neural variables defining average performance of a redundant system and patterns of co-variation among its elemental variables, respectively. PMID:23568657

Sarabon, Nejc; Markovic, Goran; Mikulic, Pavle; Latash, Mark L

2013-05-01

228

Improving a CAD system using bilateral information  

Microsoft Academic Search

Computer Aided Detection (CAD) mammographic systems are used in medicine to assist radiologists in the evaluation of mammographic images. The aim of this work is to compare the results of a developed single-image CAD system with a new one, dual-image CAD, that adds registration information of bilateral mammographic images in the training step of the former system. The evaluation of

Meritxell Tortajada; Arnau Oliver; Y. Di?ez; R. Marti?; J. C. Vilanova; J. Freixenet

2010-01-01

229

Bilateral breast cancer after cured Hodgkin's disease  

Microsoft Academic Search

Three patients developed bilateral breast cancer at 10 to 24 years after mantle irradiation for locally or systemically advanced Hodgkin's disease (HD). Four of the six cancers in the three patients were detected only by mammography. Pathologically, five of the cancers were intraductal carcinomas (four with an invasive component) with one being a lobular carcinoma. Five of the six lesions

Norwood Anderson; Jacob Lokich

1990-01-01

230

Bilateral brain processes for comprehending natural language  

E-print Network

Bilateral brain processes for comprehending natural language Mark Jung-Beeman Department 60208-2710, USA Comprehension of natural language ­ stories, conversa- tions, text ­ is very simple-established facts about the brain; yet, when it comes to comprehending complex, natural language, the right

Jung-Beeman, Mark

231

Bilateral field advantage in visual crowding  

PubMed Central

Thirty randomly oriented T’s were presented in a circle around fixation at an eccentricity of 11 degrees such that each T was crowded by its neighbors. Two locations within the same hemifield (unilateral condition) or one location in each hemifield (bilateral condition) were precued for subsequent probing. Observers were then asked to report the orientation of a target T at one of these locations. A bilateral field advantage was found: target identification was better when the two precued targets were in different hemifields than when they were within the same hemifield. This bilateral advantage was absent when only targets were presented, without any distracters. Further controls showed that this advantage could not be attributed to differences between horizontal and vertical target alignments or to visual field anisotropies. A similar bilateral advantage has been reported for multiple object tracking (Alvarez & Cavanagh, 2005) and other attentional tasks. Our results suggest that crowding also demonstrates separate attentional resources in the left and right hemifields. There was a cost to attending to two targets presented unilaterally over attending to a single target. However, this cost was reduced when the two crowded targets were in separate hemifields. PMID:19362572

Chakravarthi, Ramakrishna; Cavanagh, Patrick

2009-01-01

232

Experiences with Bilateral Art: A Retrospective Study  

Microsoft Academic Search

Recent advances in neuroscience describe the effect of experience on neural architecture. Paralleling these advances in neuroscience, recent explorations in the field of art therapy speculate on the relationship between specific therapeutic interventions and neuroplasticity, which underlies the changes in neural architecture. One such intervention, bilateral art, purposefully engages both left and right hemispheres of the brain, as well as

Carole M. McNamee

2006-01-01

233

Bilateral integration in the common chemical sense.  

PubMed

Human participants judged the pungency of various concentrations of carbon dioxide presented to one nostril, the other nostril, or both (dichorhinic stimulation). Perceived magnitude grew sharply with concentration over the range explored. The combinatorial rule of bilateral integration followed the Pythagorean theorem: the effective mass in a bilateral stimulus equalled the square root of the sum of squares of the mass entering each nostril. This simple rule implies a possible mutual inhibitory interaction between the two nostrils, but it also implies partial spatial summation of the sensory impact of bilaterally presented concentrations. When persons inhale a relatively strong irritant, such as the higher concentrations used here, they will exhibit a very brief reflex interruption of inhalation. An experiment on the threshold for the reflex implied that it too followed the Pythagorean rule of integration. This occurred for both males and females, though females displayed a lower threshold for the reflex, whether evoked unilaterally or bilaterally. This reflex may hold promise as an objective indicator of functional status of the nasal common chemical sense. PMID:7146140

Garcia Medina, M R; Cain, W S

1982-08-01

234

Branchiootic syndrome--a clinical case report and review of the literature.  

PubMed

Branchiootoic syndrome is part of the spectrum of brachiootorenal disorders. Brachiootorenal disorder is a rare autosomal dominant condition, characterized by malformations of the outer, middle, and inner ear, which are associated with branchial and renal anomalies. We describe a case of bilateral branchiootoic syndrome and discuss the anatomy of second branchial cleft fistulae and the surgical management of this uncommon condition. We report the case of a 6-year-old girl referred to our department with bilateral intermittently discharging neck swellings. Clinical examination revealed bilateral branchial fistulae and preauricular sinuses, on a background of a positive family history of branchial fistulae. A magnetic resonance imaging scan confirmed the diagnosis of bilateral second branchial cleft fistulae. In view of her symptoms, she underwent bilateral branchial fistula excision and tonsillectomy with an uneventful postoperative recovery. PMID:22901925

Amer, Imad; Falzon, Adam; Choudhury, Natasha; Ghufoor, Khalid

2012-08-01

235

Catel-Manzke palatodigital syndrome in a second trimester female foetus with nuchal oedema, costovertebral anomalies and radial ray defect.  

PubMed

We present a female fetus with combination of Pierre Robin anomaly and nuchal oedema, bilateral radial defects, multiple hand malformations including bilateral hyperphalangy, brachymesophalangy, costovertebral abnormalities, and complex cardiac malformation. The present findings constitute a true MCA syndrome with uncertain pattern of inheritance. PMID:7888142

Petit, P; Moerman, P; Legius, E; Fryns, J P

1994-01-01

236

Sequential Bilateral Cochlear Implantation in a Patient with Bilateral Meniere’s Disease  

PubMed Central

This case study describes a 45 year old female with bilateral, profound sensorineural hearing loss due to Meniere’s disease. She received her first cochlear implant in the right ear in 2008 and the second cochlear implant in the left ear in 2010. The case study examines the enhancement to speech recognition, particularly in noise, provided by bilateral cochlear implants. Speech recognition tests were administered prior to obtaining the second implant and at a number of test intervals following activation of the second device. Speech recognition in quiet and noise as well as localization abilities were assessed in several conditions to determine bilateral benefit and performance differences between ears. The results of the speech recognition testing indicated a substantial improvement in the patient’s ability to understand speech in noise and her ability to localize sound when using bilateral cochlear implants compared to using a unilateral implant or an implant and a hearing aid. In addition, the patient reported considerable improvement in her ability to communicate in daily life when using bilateral implants versus a unilateral implant. This case suggests that cochlear implantation is a viable option for patients who have lost their hearing to Meniere’s disease even when a number of medical treatments and surgical interventions have been performed to control vertigo. In the case presented, bilateral cochlear implantation was necessary for this patient to communicate successfully at home and at work. PMID:22463939

Holden, Laura K.; Neely, J. Gail; Gotter, Brenda D.; Mispagel, Karen M.; Firszt, Jill B.

2012-01-01

237

Hardware Implementation of a Bilateral Subtraction Filter  

NASA Technical Reports Server (NTRS)

A bilateral subtraction filter has been implemented as a hardware module in the form of a field-programmable gate array (FPGA). In general, a bilateral subtraction filter is a key subsystem of a high-quality stereoscopic machine vision system that utilizes images that are large and/or dense. Bilateral subtraction filters have been implemented in software on general-purpose computers, but the processing speeds attainable in this way even on computers containing the fastest processors are insufficient for real-time applications. The present FPGA bilateral subtraction filter is intended to accelerate processing to real-time speed and to be a prototype of a link in a stereoscopic-machine- vision processing chain, now under development, that would process large and/or dense images in real time and would be implemented in an FPGA. In terms that are necessarily oversimplified for the sake of brevity, a bilateral subtraction filter is a smoothing, edge-preserving filter for suppressing low-frequency noise. The filter operation amounts to replacing the value for each pixel with a weighted average of the values of that pixel and the neighboring pixels in a predefined neighborhood or window (e.g., a 9 9 window). The filter weights depend partly on pixel values and partly on the window size. The present FPGA implementation of a bilateral subtraction filter utilizes a 9 9 window. This implementation was designed to take advantage of the ability to do many of the component computations in parallel pipelines to enable processing of image data at the rate at which they are generated. The filter can be considered to be divided into the following parts (see figure): a) An image pixel pipeline with a 9 9- pixel window generator, b) An array of processing elements; c) An adder tree; d) A smoothing-and-delaying unit; and e) A subtraction unit. After each 9 9 window is created, the affected pixel data are fed to the processing elements. Each processing element is fed the pixel value for its position in the window as well as the pixel value for the central pixel of the window. The absolute difference between these two pixel values is calculated and used as an address in a lookup table. Each processing element has a lookup table, unique for its position in the window, containing the weight coefficients for the Gaussian function for that position. The pixel value is multiplied by the weight, and the outputs of the processing element are the weight and pixel-value weight product. The products and weights are fed to the adder tree. The sum of the products and the sum of the weights are fed to the divider, which computes the sum of products the sum of weights. The output of the divider is denoted the bilateral smoothed image. The smoothing function is a simple weighted average computed over a 3 3 subwindow centered in the 9 9 window. After smoothing, the image is delayed by an additional amount of time needed to match the processing time for computing the bilateral smoothed image. The bilateral smoothed image is then subtracted from the 3 3 smoothed image to produce the final output. The prototype filter as implemented in a commercially available FPGA processes one pixel per clock cycle. Operation at a clock speed of 66 MHz has been demonstrated, and results of a static timing analysis have been interpreted as suggesting that the clock speed could be increased to as much as 100 MHz.

Huertas, Andres; Watson, Robert; Villalpando, Carlos; Goldberg, Steven

2009-01-01

238

Familial spastic paraplegia, bilateral sensorineural deafness, and intellectual retardation associated with a progressive nephropathy.  

PubMed Central

We present a family in which at least four persons have evidence of an inherited disorder comprising a variable spastic paraplegia, bilateral sensorineural deafness, intellectual retardation, and a progressive nephropathy. Focal segmental proliferative lesions with sclerosis suggestive of mesangial IgA nephropathy (Berger's disease) were found on renal renal biopsy in two affected persons. The glomerular basement membrane showed none of the changes characteristic of Alport's syndrome. Males and females are affected and the segregation of the disease is consistent with dominant transmission. Images PMID:3351903

Fitzsimmons, J S; Watson, A R; Mellor, D; Guilbert, P R

1988-01-01

239

Wall-eyed bilateral internuclear ophthalmoplegia: review of pathogenesis, diagnosis, prognosis and management.  

PubMed

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is an uncommon disorder of ocular motility that possesses a unique spectrum of clinical findings, consisting of primary gaze exotropia, adduction impairment and nystagmus of the abducting eye. WEBINO is a variant of internuclear ophthalmoplegia (INO) sharing similar pathophysiology and aetiologies. Much of the literature published on internuclear ophthalmoplegia and its variants focuses on aetiology and pathophysiology, whereas there has been less information addressing prognosis and management. This review will provide current perspectives on the pathogenesis, prognosis and management of WEBINO syndrome. PMID:25308307

Wu, Yu-Tai; Cafiero-Chin, Malinda; Marques, Cathy

2015-01-01

240

Asymptomatic bilateral elongated and mineralized stylohyoid complex: report of one case.  

PubMed

The stylohyoid process is a cylindrical bony structure surrounded by important anatomical structures including vessels and nerves. Calcification and elongation of the stylohyoid ligament complex over 30 mm may be associated with neck and facial pain, known as Eagle's syndrome. However, a bilateral ossified and elongated stylohyoid complex may be devoid of symptoms. We report a 79-year-old symptom-free female who presented on a routine conventional dental radiographic exam an 80.96 mm pseudoarticulated stylohyoid complex in the left side and an 75.85 mm on the other. On CAT scan, both processes were calcified. PMID:24121584

Chaves, Hellíada; Costa, Fábio; Cavalcante, Daniel; Ribeiro, Thyciana; Gondim, Delane

2013-06-01

241

Aortic calcification and renal cysts demonstrated by CT in a teenager with Alagille syndrome.  

PubMed

Alagille syndrome, or arteriohepatic dysplasia, is a disorder characterized by paucity of intrahepatic bile ducts, peculiar facies and skeletal anomalies. We report a typical case of this syndrome in an 18-year-old girl, in whom abdominal CT showed bilateral renal cysts and aortic wall calcification, findings unreported in the radiological literature. PMID:7567250

Pombo, F; Isla, C; Gayol, A; Bargiela, A

1995-01-01

242

Bilateral Macular Hole Following Myopic Photorefractive Keratectomy  

PubMed Central

A 42-year-old man was admitted to our clinic complaining of visual distortion in his left eye two months after bilateral myopic photorefractive keratectomy (PRK). Macular optical coherence tomography (OCT) showed a stage II macular hole in the left eye. Simultaneous OCT in the right eye showed vitreous traction and distortion of the outer retina. One month later, the patient underwent vitrectomy for the left eye, and the macular hole was closed. Two months after that, the patient complained of visual distortion in the right eye, and OCT revealed increased traction and accentuated outer retinal distortion indicating a stage IB macular hole. Traction attenuated later without any intervention. The short interval between PRK and hole formation, bilateral involvement, and the moderate refractive error in this case highlight the possible role of PRK in aggravating vitreoretinal interface abnormalities. We recommend the addition of PRK to the list of procedures that may be associated with the formation of a macular hole. PMID:24882962

Shoeibi, Nasser; Abrishami, Majid; Ansari-Astaneh, Mohammad-reza

2014-01-01

243

Bilateral Nocardia Keratitis after Photorefractive Keratectomy  

PubMed Central

Purpose To report the clinical, confocal scan, and histopathologic features of nocardia keratitis in a patient who developed bilateral infection following photorefractive keratectomy (PRK). Case Report A 23-year-old woman underwent bilateral PRK for low myopia. On postoperative day 3, dense central stromal infiltrates were noticed in both eyes. Empirical antibiotic therapy was initiated which was converted into specific therapy after a definite diagnosis was made based on clinical features and confirmed by confocal scan and histopathologic findings. Clinical and confocal scan features were consistent with the diagnosis of Nocardia keratitis, and topical 2% amikacin eye drops were started. Because of poor response to medical therapy, lamellar keratectomy was performed in both eyes which shortened the treatment course. Histopathologic examination reconfirmed the initial diagnosis. Conclusion Familiarity with clinical and confocal scan features facilitates early diagnosis of Nocardia keratitis leading to proper management and hence a rapid therapeutic response. PMID:23275825

Faramarzi, Amir; Feizi, Sepehr; Javadi, Mohammad-Ali; Rezaei Kanavi, Mozhgan; Yazdizadeh, Forouzan; Moein, Hamid-Reza

2012-01-01

244

Bilateral pneumothorax following a blunt trachea trauma  

PubMed Central

A-13 year old boy had an accident with his bike with a blunt thorax trauma and presented shortly after with facial swelling. Due to respiratory insufficiency, intubation was done during the transport to the clinic. First, a chest radiograph was performed, which showed a unilateral pneumothorax. Later a CT scan revealed bilateral pneumothorax and pneumomediastinum. Bilateral chest tube insertions improved the respiratory situation. Bronchoscopy showed a tracheal lesion two cm posterior to the main carina. After good wound healing, the patient was dismissed after 21 days in good health. Conservative treatment can be recommended in selected patients with a tracheal lesion when having a stable respiratory situation. If the patient does not improve after 48 h or if the clinical condition worsens, surgical management should be considered.

Schwarz, C.; Usemann, J.; Stephan, V.; Kaiser, D.; Rothe, K.; Rückert, J.C.; Neudecker, J.

2013-01-01

245

Bilateral pulmonary sequestration in the elderly adult  

PubMed Central

Pulmonary sequestration (PS) is a rare malformation consisting of aberrant lung tissue which is not affiliated with the normal bronchial system and is fed by an aberrant artery that derives from systemic arteries. However, PS is usually seen unilaterally but, only rarely, it is bilateral. Most patients with PS are diagnosed because of symptoms due to pulmonary infection or cardiac disease, while a small portion of patients are asymptomatic and diagnosed incidentally. In this report, we present an extremely rare case of asymptomatic bilateral PS which was diagnosed at advanced age. To our knowledge, this case represents the oldest patient in the literature, and the second case that was diagnosed in a patient over the age of 50. PMID:23088281

2012-01-01

246

Bilateral Asymmetrical Traumatic Sternoclavicular Joint Dislocations  

PubMed Central

Unilateral and bilateral sternoclavicular joint (SCJ) dislocations are rare injuries. The difficulty in assessing this condition often leads to delay in diagnosis and treatment. We report a rare case of bilateral asymmetrical traumatic SCJ dislocations in a 45-year-old male. The right anterior SCJ dislocation was reduced in the emergency room (ER) and resulted in residual instability. The left posterior SCJ dislocation was asymptomatic and unnoticed for six months. It is important for ER physicians and orthopaedic surgeons to be able identify and treat this condition. All suspected SCJ dislocations should be evaluated by computed tomography (CT) scan for confirmation of the diagnosis and evaluation of both SCJs. Posterior SCJ dislocation is a potentially fatal injury and should not be overlooked due to the presence of other injuries. Surgical intervention is often necessary in acute and old cases. PMID:23275851

Albarrag, Mohammed K.

2012-01-01

247

Bilateral macular hole following myopic photorefractive keratectomy.  

PubMed

A 42-year-old man was admitted to our clinic complaining of visual distortion in his left eye two months after bilateral myopic photorefractive keratectomy (PRK). Macular optical coherence tomography (OCT) showed a stage II macular hole in the left eye. Simultaneous OCT in the right eye showed vitreous traction and distortion of the outer retina. One month later, the patient underwent vitrectomy for the left eye, and the macular hole was closed. Two months after that, the patient complained of visual distortion in the right eye, and OCT revealed increased traction and accentuated outer retinal distortion indicating a stage IB macular hole. Traction attenuated later without any intervention. The short interval between PRK and hole formation, bilateral involvement, and the moderate refractive error in this case highlight the possible role of PRK in aggravating vitreoretinal interface abnormalities. We recommend the addition of PRK to the list of procedures that may be associated with the formation of a macular hole. PMID:24882962

Shoeibi, Nasser; Jabbarpoor Bonyadi, Mohammad Hossein; Abrishami, Majid; Ansari-Astaneh, Mohammad-Reza

2014-06-01

248

Susac syndrome: diverse clinical findings and treatment.  

PubMed

We report a case of a 19-year-old woman presenting bilateral neurosensorial hearing loss, mental abnormalities, and loss of visual field in the left eye. Visual acuity was 20/20 in OD and 20/25 in OS. Patient was examined systemically. Audiometry showed sensorineural hearing loss in both ears. The magnetic resonance imaging (MRI) of brain revealed multiple small lesions in the white matter in both cerebral hemispheres and at the corpus callosum. Fundoscopy showed bilateral normal optic disc and sheathing of the arterioles in the middle periphery of OD. Retinal edema and cotton-wool spots were observed. Fluorescein angiography showed bilateral peripheral occlusive arterial vasculopathy. The patient was diagnosed with Susac syndrome and treated with quetiapine fumarate, flunitrazepam, and prednisone, which resulted in stabile outcome. This case shows that a high index of suspicion leading to early recognition and treatment is important to avoid irreversible damage. PMID:25295909

Bardal, Ricardo Canto; Badaro, Emmerson; Arana, Jayme; Alves, Fabio; Souza, Eduardo Cunha de; Bonomo, Pedro Paulo; Portella, Ezequiel; Maia, Mauricio

2014-01-01

249

Multiple osteochondroses of bilateral knee joints  

Microsoft Academic Search

We experienced a patient with a combination of multiple osteochondroses: Blount's disease, bipartite patella, and Sinding-Larsen-Johansson\\u000a disease in the left knee, and a combination of bipartite patella and Osgood-Schlatter disease in the right knee. The patient\\u000a was a healthy, active 12-year-old boy with bilateral knee pain. He had been diagnosed with Blount's disease of the left tibia\\u000a at 2 years

Hiroyuki Segawa; Go Omori; Yoshio Koga

2001-01-01

250

[Case of cystitis glandularis causing bilateral hydronephrosis].  

PubMed

A 38 year-old male was referred to our hospital due to bilateral hydronephrosis. Cystoscopy revealed multiple edematous papillary tumors that exist on the prostatic urethra, bladder neck, trigone, and lateral wall on both sides. He underwent a transurethral resection of bladder tumors. The pathological diagnosis was cystitis glandularis. He was given steroid orally for 6 months and had no sign of recurrence after 2 years. PMID:24187856

Maeda, Motohiro; Hirabayashi, Takaki; Inuzuka, Yoshihiro; Kondo, Atsuya; Tanaka, Kuniaki

2013-09-01

251

Bilateral pregnancy luteoma: a case report.  

PubMed

Pregnancy luteoma is a non-neoplastic lesion of the ovary occurring during pregnancy and is usually discovered incidentally at the time of a cesarean section or during postpartum tubal ligation. An accurate diagnosis is important for the mother and the fetus as it can be confused with ovarian malignancy leading to unnecessary surgery. We report a case of a pregnant female who was discovered to have bilateral enlarged ovaries at the time of emergency cesarean section. PMID:25337317

Nanda, Annu; Gokhale, Uday A; Pillai, G Rajasekharan

2014-09-01

252

Bilateral Pregnancy Luteoma: A Case Report  

PubMed Central

Pregnancy luteoma is a non-neoplastic lesion of the ovary occurring during pregnancy and is usually discovered incidentally at the time of a cesarean section or during postpartum tubal ligation. An accurate diagnosis is important for the mother and the fetus as it can be confused with ovarian malignancy leading to unnecessary surgery. We report a case of a pregnant female who was discovered to have bilateral enlarged ovaries at the time of emergency cesarean section. PMID:25337317

Nanda, Annu; Gokhale, Uday A.; Pillai, G Rajasekharan

2014-01-01

253

Diagnosis and management of bilateral nasolabial cysts  

PubMed Central

Nasolabial cysts are painless, submucosal, non-odontogenic jaw cysts presenting as soft tissue swellings in the maxillary anterior mucolabial fold lateral to midline, leading to elevation of nasal ala. Present case documents bilateral nasolabial cysts in a 69-year-old Asian female patient. In the present case, extraoral swelling of maxillary lip and elevation of nasal ala was observed on right side of the face. Intraorally, soft and fluctuant bilateral cysts were observed. Straw-colored fluid was aspirated from the right cyst. Radiographically, erosion of bone in a “cupping” fashion was observed in the region of left cyst. The cysts were enucleated using intraoral approach. Histopathology of the right-sided cyst revealed a cystic cavity lined by stratified squamous cells along with a few mucosal cells. At few places, stratified squamous and pseudostratified columnar epithelia with many cilia and goblet cells were also evident. Capsule was loosely arranged with fibrous tissue and chronic inflammatory infiltrate. Left-sided cystic specimen showed two or more layered stratified squamous lining epithelium with thin capsule. Diagnosis of bilateral nasolabial/nasoalveolar cysts was confirmed. PMID:24574670

Parwani, Rajkumar; Parwani, Simran; Wanjari, Sangeeta

2013-01-01

254

Diagnosis and management of bilateral nasolabial cysts.  

PubMed

Nasolabial cysts are painless, submucosal, non-odontogenic jaw cysts presenting as soft tissue swellings in the maxillary anterior mucolabial fold lateral to midline, leading to elevation of nasal ala. Present case documents bilateral nasolabial cysts in a 69-year-old Asian female patient. In the present case, extraoral swelling of maxillary lip and elevation of nasal ala was observed on right side of the face. Intraorally, soft and fluctuant bilateral cysts were observed. Straw-colored fluid was aspirated from the right cyst. Radiographically, erosion of bone in a "cupping" fashion was observed in the region of left cyst. The cysts were enucleated using intraoral approach. Histopathology of the right-sided cyst revealed a cystic cavity lined by stratified squamous cells along with a few mucosal cells. At few places, stratified squamous and pseudostratified columnar epithelia with many cilia and goblet cells were also evident. Capsule was loosely arranged with fibrous tissue and chronic inflammatory infiltrate. Left-sided cystic specimen showed two or more layered stratified squamous lining epithelium with thin capsule. Diagnosis of bilateral nasolabial/nasoalveolar cysts was confirmed. PMID:24574670

Parwani, Rajkumar; Parwani, Simran; Wanjari, Sangeeta

2013-09-01

255

Bilateral Descemet membrane detachment after canaloplasty.  

PubMed

We report a case of bilateral Descemet membrane detachment (DMD) after canaloplasty in a 70-year-old Portuguese man with primary open-angle glaucoma. The patient developed bilateral DMD immediately following consecutive (1 week apart) canaloplasty surgery in both eyes. Slitlamp biomicroscopy, gonioscopy, and Fourier-domain optical coherence tomography (FD-OCT) findings are described. On postoperative day 1, in both cases, slitlamp biomicroscopy revealed an unscrolled inferonasal DMD and a clear cornea with deep and quiet anterior chambers. Gonioscopy showed an intact, lightly pigmented, and distended trabecular meshwork with no evidence of suture extrusion. High-resolution FD-OCT revealed a widely dilated canal of Schlemm, trabecular distention, and a retrocorneal hyperreflective membrane corresponding to a DMD. At 3 months, the DMD resolved spontaneously in both eyes. Although DMD is a known complication of canaloplasty, the occurrence of bilateral symmetrically located DMDs in our case suggests a possible anatomical predisposition in addition to factors induced by the surgical technique. PMID:20202554

Palmiero, Pat-Michael; Aktas, Zeynep; Lee, Olivia; Tello, Celso; Sbeity, Zaher

2010-03-01

256

Multilateral, regional and bilateral energy trade governance  

SciTech Connect

The current international energy trade governance system is fragmented and multi-layered. Streamlining it for greater legal cohesiveness and international political and economic cooperation would promote global energy security. The current article explores three levels of energy trade governance: multilateral, regional and bilateral. Most energy-rich countries are part of the multilateral trading system, which is institutionalized by the World Trade Organization (WTO). The article analyzes the multilateral energy trade governance system by focusing on the WTO and energy transportation issues. Regionally, the article focuses on five major regional agreements and their energy-related aspects and examines the various causes that explain the proliferation of regional trade agreements, their compatibility with WTO law, and then provides several examples of regional energy trade governance throughout the world. When it comes to bilateral energy trade governance, this article only addresses the European Union’s (EU) bilateral energy trade relations. The article explores ways in which gaps could be filled and overlaps eliminated whilst remaining true to the high-level normative framework, concentrating on those measures that would enhance EU energy security.

Leal-Arcas, Rafael; Grasso, Costantino; Rios, Juan Alemany (Queen Mary Univ. of London (United Kingdom))

2014-12-01

257

Bilateral Kimura's disease of the eyelids.  

PubMed Central

A case of Kimura's disease affecting the eyelids bilaterally is reported in a 5-year-old boy of Afro-Caribbean extraction who has been followed for 12 years with repeat biopsies. He initially presented at 5 years of age with swelling of the left upper eyelid, left cervical lymphadenopathy, and eosinophilia. One year later he developed swelling of the right upper eyelid. There has been no change in the clinical appearance over the next 12 years. Repeated biopsies of the eyelids showed a diffuse inflammatory infiltrate with many eosinophils and lymphocytes. A lymph node biopsy showed reactive lymphoid hyperplasia. Immunohistochemistry using lymphoid markers showed a polyclonal pattern. Kimura's disease is a rare cause of eyelid swelling, particularly at such a young age and with bilateral involvement. This case demonstrates that bilateral orbital lymphoid lesions with cervical node involvement do not always imply lymphoma, but may have a benign pathogenesis. The unusually long follow up in this case confirms an excellent prognosis for Kimura's disease with conservative management. Accurate diagnosis in small orbital biopsies may spare the patient unnecessary radical surgery. Images PMID:1486082

Kennedy, S. M.; Pitts, J. F.; Lee, W. R.; Gibbons, D. C.

1992-01-01

258

High Performance, Three-Dimensional Bilateral Filtering  

SciTech Connect

Image smoothing is a fundamental operation in computer vision and image processing. This work has two main thrusts: (1) implementation of a bilateral filter suitable for use in smoothing, or denoising, 3D volumetric data; (2) implementation of the 3D bilateral filter in three different parallelization models, along with parallel performance studies on two modern HPC architectures. Our bilateral filter formulation is based upon the work of Tomasi [11], but extended to 3D for use on volumetric data. Our three parallel implementations use POSIX threads, the Message Passing Interface (MPI), and Unified Parallel C (UPC), a Partitioned Global Address Space (PGAS) language. Our parallel performance studies, which were conducted on a Cray XT4 supercomputer and aquad-socket, quad-core Opteron workstation, show our algorithm to have near-perfect scalability up to 120 processors. Parallel algorithms, such as the one we present here, will have an increasingly important role for use in production visual analysis systems as the underlying computational platforms transition from single- to multi-core architectures in the future.

Bethel, E. Wes

2008-06-05

259

Bilateral elastofibroma dorsi: A case report  

PubMed Central

INTRODUCTION Elastofibroma dorsi(EFD) is slow-growing, benign, soft tissue tumor of unclear pathogenesis, typically located at the subscapular region of elderly people. It may be unilateral or bilateral. As it exhibits benign behavior, it should be surgically removed only in symptomatic patients. Magnetic resonance imaging (MRI) is a useful tool for assessment of EFD and can potentially help avoid the need for unnecessary biopsy and surgery. PRESENTATION OF CASE A 62-year-old woman presented with 2 years complaint of back pain, particularly aggravated with shoulder movements and swelling with bilateral elastofibroma dorsi. Both masses were totally excised with bilateral posterolateral subscapular incision. Symptoms were completely controlled and significant discomfort from the surgical procedure was completely resolved with in a few weeks interval from the operation. DISCUSSION The pathogenesis of the EFD still remains unclear. Elastofibroma dorsi has an unspecific clinical presentation and can be confused with other tumors of the periscapular region Imaging studies are useful for diagnosis. CONCLUSION Elastofibroma dorsi is benign soft tissue tumor mostly observed in the subscapular regions of elderly female patients. The surgery is indicated in symptomatic cases and must be confined to simple excision of the lesion. PMID:25437657

Sarici, Inanc Samil; Basbay, Elif; Mustu, Mehdi; Eskut, Burak; Kala, Ferhat; Agcaoglu, Orhan; Akici, Murat; Ozkurt, Enver

2014-01-01

260

The ovarian remnant syndrome and ureteral obstruction: medical management.  

PubMed

The ovarian remnant syndrome represents the development of symptoms due to residual ovarian tissue after bilateral salpingo-oophorectomy. Treatment generally consists of surgical resection but recurrence after resection is common. A case is reported in which a postoperative recurrent ovarian remnant was successfully managed by using a luteinizing hormone-releasing hormone agonist. In addition, this report includes a survey of gynecologists to determine the frequency of this syndrome and of ureteral involvement. PMID:8201651

Koch, M O; Coussens, D; Burnett, L

1994-07-01

261

Ovarian remnant syndrome.  

PubMed

Ovarian remnant syndrome (ORS) refers to a condition occurring in women who have had a bilateral salpingo-oophorectomy (BSO), with or without a hysterectomy, that leaves behind ovarian tissue. This residual ovarian tissue then results in pelvic pain or a pelvic mass. Risk factors associated with incomplete removal of an ovary and subsequent development of ORS include a history of endometriosis, pelvic inflammatory disease, multiple previous surgeries, and pelvic adhesive disease. Patients most frequently present with chronic pelvic pain, pelvic pain associated with a pelvic mass, or an asymptomatic pelvic mass. Definitive criteria for diagnosis of ORS include a history of BSO with histologic documentation of ovarian tissue obtained during subsequent surgical excision. The recommended treatment for ORS is surgical excision by laparotomy or, more recently, laparoscopy. We present the presentation and management of patients with ORS and a review of the published literature. PMID:16885659

Magtibay, Paul M; Magrina, Javier F

2006-09-01

262

Cushing's syndrome in medullary thyroid carcinoma.  

PubMed

Ectopic ACTH secretion is a rare cause of hypercortisolism. Induced metabolic disturbances are often serious, and the management of such patients may be difficult. We report here our experience with four medullary thyroid carcinoma (MTC) patients with distant metastases in whom an ectopic ACTH syndrome occurred. The clinical presentation was significant by the severity and the rapidity of the hypercortisolism. Diagnosis and follow-up were realized by measurements of plasma cortisol, urinary free cortisol, urinary 17-hydroxycorticosteroid, plasma ACTH, plasma LPH, serum calcitonin and carcino-embryonic antigen. Initial treatment with adrenalytic medical therapy failed to control the disease. Only bilateral adrenalectomy cured the excessive cortisol production, and for a long time despite tumor progression. In conclusion, bilateral adrenalectomy should be considered in MTC patients with Cushing's syndrome even at the stage of distant spread. In fact, with regard to the slow growth rate of most MTC's, they may survive for years. PMID:7615903

Mure, A; Gicquel, C; Abdelmoumene, N; Tenenbaum, F; Francese, C; Travagli, J P; Gardet, P; Schlumberger, M

1995-03-01

263

Exfoliation Syndrome in Nigeria  

PubMed Central

Purpose: The purpose of this study was to estimate the prevalence of exfoliation syndrome (XFS) and its association with ocular disease in patients attending the eye clinic of the University College Hospital (UCH) in Ibadan, Nigeria. Materials and Methods: A total of 448 consecutive new patients, aged 30-90 years who presented to the eye clinic of UCH between December 2009 and November 2010 were evaluated. Each patient had a complete ophthalmic examination. Patients with exfoliative material on the anterior lens surface and/or pupillary margin in either or both eyes were considered to have XFS. Means, standard deviation, and 95% confidence intervals were calculated. Results: All the patients examined were from the southern part of Nigeria. Majority (94.2%) were of the Yoruba tribe from southwestern Nigeria, while 5.8% were from southeastern Nigeria. The mean age of the study cohort was 58.5 ± 13.8, 54.8% were males, 12 (2.7%) had XFS. All patients with XFS were of the Yoruba tribe, with a mean age 65.6 ± 5.6 years. There was a male predilection (66.7%). All eyes with XFS had lenticular opacities. XFS was bilateral in eight patients (66.7%) of whom seven patients (87.5%) had glaucoma and lenticular opacities bilaterally. Conclusion: This is the first report of the existence of XFS in Nigeria. Larger studies are necessary in this population to further investigate the disease. PMID:23248543

Olawoye, Olusola O.; Ashaye, Adeyinka O.; Teng, Christopher C; Liebmann, Jeffrey M.; Ritch, Robert; Ajayi, Benedict G.

2012-01-01

264

Learning about Down Syndrome  

MedlinePLUS

... genetic terms used on this page Learning About Down Syndrome What is Down syndrome? What are the symptoms ... syndrome Additional Resources for Down Syndrome What is Down syndrome? Down syndrome is a chromosomal condition related to ...

265

An Unusual Case of Neuralgic Amyotrophy Presenting with Bilateral Phrenic Nerve and Vocal Cord Paresis  

PubMed Central

Background Neuralgic amyotrophy (brachial plexus neuropathy, brachial plexus neuritis, or Parsonage-Turner syndrome) is an uncommon inflammatory condition typically characterized by acute and severe shoulder pain followed by paresis with muscle weakness and atrophy of the upper limb or shoulder girdle. We report an unusual clinical manifestation of neuralgic amyotrophy, namely bilateral phrenic nerve palsy with concomitant laryngeal paresis. Case Report A 55-year-old male presented with orthopnea and aphonia after an episode of bilateral shoulder pain preceded by an upper respiratory tract infection. Spirometry, chest X-ray and videolaryngoscopy revealed bilateral and simultaneous paresis of the diaphragm and the vocal cords. Clinical examination at admission and at the 2-month follow-up did not show upper limb weakness or atrophy, except for a mild atrophy of the right supraspinatus muscle. An electromyography of the upper limb muscles and nerve conduction studies did not reveal signs of denervation. Analysis of the cerebrospinal fluid and an MRI of the neuraxis were unremarkable. After treatment with prednisolone, vocal cord function markedly improved within 8 weeks, whereas paresis of the diaphragm persisted. Conclusion Shoulder pain followed by diaphragmatic paralysis with dyspnea and hoarseness may be a manifestation of neuralgic amyotrophy even if upper limb or shoulder girdle palsies are absent. PMID:21490716

Holtbernd, F.; Zehnhoff-Dinnesen, A. am; Duning, T.; Kemmling, A.; Ringelstein, E.B.

2011-01-01

266

Aicardi Syndrome  

MedlinePLUS

... males normally have an X and a Y chromosome.) The precise gene or genetic mechanism causing Aicardi syndrome is not yet known. Originally, Aicardi syndrome was characterized by three main features: 1) partial or complete absence of ...

267

Tourette Syndrome  

MedlinePLUS

If you have Tourette syndrome, you make unusual movements or sounds, called tics. You have little or no control over them. Common tics ... words, spin, or, rarely, blurt out swear words. Tourette syndrome is a disorder of the nervous system. ...

268

Marfan Syndrome  

MedlinePLUS

... syndrome is a condition in which your body's connective tissue is abnormal. Connective tissue helps support all parts of your body. It ... and develops. Marfan syndrome most often affects the connective tissue of the heart and blood vessels, eyes, bones, ...

269

Down Syndrome  

MedlinePLUS

... Body Works Main Page The Pink Locker Society Down Syndrome KidsHealth > Kids > Health Problems > Birth Defects & Genetic Problems > ... skills. Continue Do a Lot of People Have Down Syndrome? About 1 out of every 800 babies born ...

270

Turner Syndrome  

MedlinePLUS

Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

271

Klinefelter syndrome  

MedlinePLUS

47 X-X-Y syndrome ... have two XX chromosomes. Boys normally have an X and a Y chromosome. Klinefelter syndrome is when ... boy is born with at least one extra X chromosome. Usually, this occurs due to one extra ...

272

[Styloid syndrome; Sluder's syndrome; Charlin's syndrome].  

PubMed

The styloid syndrome is caused by an irritation of the glossopharyngeal nerve from an excessive development of the styloid apophysis. Treatment consists of the resection of the same. The Sluder syndrome represents a nevralgy with origin in the sphenopalatine ganglion and a dysfunction of the parasympathetic system. The Charlin syndrome, much less frequent, manifests itself by pains in the territory of the nasociliar nerve and the ciliar ganglion. PMID:293019

Strupler, W

1979-02-01

273

Unilateral hyperhydrosis in Pourfour du Petit syndrome.  

PubMed

Upper limp hyperhydrosis is an idiopathic disease with bilateral involvement. However, Pourfour du Petit syndrome, the opposite of Horner syndrome, may result in unilateral upper limb hyperhydrosis. It occurs following hyperactivity of the sympathetic cervical chain as a consequence of irritation secondary to trauma. We report herein two cases with Pourfour du Petit syndrome showing unilateral upper limb hyperhydrosis. The patients presented with right-sided mydriasis and ipsilateral hemifacial hyperhydrosis. The onset of disease was followed by a trauma in both patients. They underwent upper thoracic sympathectomy with favorable outcome. A history of an antecedent trauma in patients with unilateral upper limb hyperhydrosis and anisocoria may imply a possible diagnosis of Pourfour du Petit syndrome. PMID:15296919

Kara, Murat; Dikmen, Erkan; Akarsu, Cengiz; Birol, Ahu

2004-08-01

274

Bilateral adrenal hemorrhage: The unrecognized cause of hemodynamic collapse associated with heparin-induced thrombocytopenia  

PubMed Central

Objective Heparin-induced thrombocytopenia is a common adverse effect of treatment with heparin resulting in paradoxical thromboses. An immunoglobulin G class “heparin-induced thrombocytopenia antibody” attaches to a heparin—platelet factor 4 protein complex. The antibody then binds to the Fc?IIa receptor on the surface of a platelet, resulting in activation, consumption, and thrombocytopenia in the clinical syndrome of heparin-induced thrombocytopenia. In contradistinction to other drug-induced thrombocytopenias that lead to a risk of hemorrhage, the state of thrombocytopenia in heparin-induced thrombocytopenia leads to an acquired hypercoagulability syndrome. Bilateral adrenal hemorrhage associated with heparin-induced thrombocytopenia has become an increasingly documented association. The adrenal gland has a vascular construction that lends itself to venous thrombus in the setting of heparin-induced thrombocytopenia and subsequent arterial hemorrhage. A literature search revealed 17 reported cases of bilateral adrenal hemorrhage in the setting of heparin-induced thrombocytopenia uniformly presenting with complete hemodynamic collapse. Data Sources An Ovid MEDLINE search of the English-language medical literature was conducted, identifying articles describing cases of bilateral adrenal hemorrhage in the setting of heparin-induced thrombocytopenia. Study Selection All cases with this association were included in the review. Data Extraction and Data Synthesis A total of 14 articles were identified, describing 17 individual case reports of bilateral adrenal hemorrhage associated with heparin-induced thrombocytopenia. All cases confirmed known characteristics of heparin-induced thrombocytopenia and uniformly revealed hypotension due to adrenal insufficiency. There were five deaths, resulting in an overall mortality rate of 27.8%, and 100% mortality in the three cases where adrenal insufficiency went unrecognized. Conclusions The secondary complication of adrenal vein thrombosis leading to bilateral adrenal hemorrhage remains insufficiently recognized and undertreated. The nonspecific presentation of adrenal hemorrhage and insufficiency as a complication of heparin-induced thrombocytopenia, coupled with the catastrophic clinical course of untreated adrenal collapse, requires a high index of suspicion to achieve rapid diagnosis and provide life-saving therapy. PMID:21242799

Rosenberger, Laura H.; Smith, Philip W.; Sawyer, Robert G.; Hanks, John B.; Adams, Reid B.; Hedrick, Traci L.

2011-01-01

275

[Ovarian remnant syndrome. Apropos of 4 cases].  

PubMed

Four observations of "residual ovarian syndrome" are presented. This syndrome is to be discriminated from the one of "the remaining ovary". These observations are discussed and compared with literature data. After bilateral ovariectomy--or adnexectomy--generally difficult, appear with a variable reaction time, different unrelated or coupled symptoms: signs of renewal of ovarian hormonal activity after a phase of surgical menopause, pelvic pains, dyspareunia, et cetera.... Clinical, biology, echography and even tomodensitometry lead to the diagnosis. The treatment can be: the return of surgery, the pelvic irradiation, antigonadotropic progestogens or abstention. PMID:3764293

Bouquet de Jolinière, J; de Brux, J; Senèze, J; Ulman, D

1986-01-01

276

Nephrotic syndrome.  

PubMed

Nephrotic syndrome refers to excessive proteinuria, with associated hypoalbuminemia, edema, and hyperlipidemia. A diverse spectrum of disorders has been associated with nephrotic syndrome and related neurologic complications, although the relative infrequency of these cases limits conclusive associations. Neurologic manifestations of nephrotic syndrome may result from hypoproteinemia, hypercoagulability, hyperlipidemia, hypertension, amyloid deposition, hormonal changes, or electrolyte disorders. Neurologic diagnosis hinges on prompt recognition of this syndrome and rational therapeutic strategies are aimed at the underlying systemic disorder. PMID:24365309

Liebeskind, David S

2014-01-01

277

Imaging findings of Gorlin-Goltz syndrome  

PubMed Central

A 15-year-old girl was referred to a dentist complaining of parageusia, bad taste in the mouth, which started 9 months ago. Panoramic X-ray and non-enhanced computed tomography scan revealed multiple bilateral unilocular cysts in the mandible and maxilla, along with calcification of anterior part of the falx cerebri. She was eventually diagnosed with Gorlin-Goltz syndrome based on imaging and histopathologic finding of keratocystic odontogenic tumor. PMID:25610614

Hajalioghli, Parisa; Ghadirpour, Ali; Ataie-Oskuie, Reza; Kontzialis, Marinos

2015-01-01

278

Globus Pallidus Interna Deep Brain Stimulation in a Patient with Medically Intractable Meige Syndrome  

PubMed Central

Medical therapies in patients with Meige syndrome, including botulinum toxin injection, have been limited because of incomplete response or adverse side effects. We evaluated a patient with Meige syndrome who was successfully treated with deep brain stimulation (DBS) in the globus pallidus interna (GPi). This case report and other previous reports suggest that bilateral GPi DBS may be an effective treatment for medically refractory Meige syndrome, without significant adverse effects. PMID:25360233

Bae, Dae-Woong; Son, Byung-chul; Kim, Joong-Seok

2014-01-01

279

HELLP syndrome  

MedlinePLUS

HELLP syndrome is a group of symptoms that occur in pregnant women who have: H -- hemolysis (the breakdown ... The cause of HELLP syndrome has not been found. HELLP syndrome occurs in about 1 to 2 out of 1,000 pregnancies, and in ...

280

Down syndrome  

MedlinePLUS

Down syndrome is a genetic condition in which a person has 47 chromosomes instead of the usual 46. ... In most cases, Down syndrome occurs when there is an extra copy of chromosome 21. This form of Down syndrome is called Trisomy 21. ...

281

Usher Syndrome  

MedlinePLUS

Home Health Info Hearing, Ear Infections, and Deafness Usher Syndrome Usher Syndrome On this page What is Usher syndrome? Who is affected by Usher ... legally blind. Type 1 Type 2 Type 3 Hearing Profound deafness in both ears from birth Moderate to severe hearing loss from ...

282

Aase syndrome  

MedlinePLUS

Aase-Smith syndrome; Hypoplastic anemia/Triphalangeal thumb syndrome ... Jones KL, ed. Aase syndrome. In: Smith's Recognizable Patterns Of Human Malformation. 6th ed. Saunders. 2005. Clinton C, Gazda HT. Diamond-Blackfan Anemia. 2009 Jun 25 [Updated 2013 Jul ...

283

Rowell syndrome  

PubMed Central

Rowell syndrome is a rare disease consisting of erythema multiforme-like lesions associated with lupus erythematosus. The syndrome occurs mostly in middle-aged women. The authors describe the syndrome in a 15-year-old boy who responded well to systemic steroids and hydroxychloroquine. PMID:25506561

Bhat, Ramesh Y; Varma, Chaitanya; Bhatt, Sonia; Balachandran, C

2014-01-01

284

Metabolic Syndrome  

MedlinePLUS

... to occur together. You must have at least three metabolic risk factors to be diagnosed with metabolic syndrome. A large ... syndrome may overtake smoking as the leading risk factor for heart disease. It is possible to prevent or ... Metabolic Syndrome Clinical Trials Clinical trials are ...

285

Velocardiofacial Syndrome  

ERIC Educational Resources Information Center

Velocardiofacial syndrome (VCFS), also known as DiGeorge, conotruncal anomaly face, and Cayler syndromes, is caused by a microdeletion in the long arm of Chromosome 22. We review the history of the syndrome from the first clinical reports almost half a century ago to the current intriguing molecular findings associating genes from the…

Gothelf, Doron; Frisch, Amos; Michaelovsky, Elena; Weizman, Abraham; Shprintzen, Robert J.

2009-01-01

286

Rowell syndrome.  

PubMed

Rowell syndrome is a rare disease consisting of erythema multiforme-like lesions associated with lupus erythematosus. The syndrome occurs mostly in middle-aged women. The authors describe the syndrome in a 15-year-old boy who responded well to systemic steroids and hydroxychloroquine. PMID:25506561

Bhat, Ramesh Y; Varma, Chaitanya; Bhatt, Sonia; Balachandran, C

2014-11-01

287

Prenatal Diagnosis of Bilateral Ectrodactyly and Radial Agenesis Associated with Trisomy 10 Mosaicism  

PubMed Central

Ectrodactyly or split hand and foot malformations (SHFMs) are rare malformations of the limbs, characterized by median clefts of the hands and feet, syndactyly, and aplasia and/or hypoplasia of the phalanges. They represent a clinically and genetically heterogeneous disorder, with both sporadic and familial cases. Most of the genomic rearrangements identified to date in some forms of SHFM are autosomal dominant traits, involving various chromosome regions. Bilateral radial ray defects comprise also a large heterogenous group of disorders, including trisomy 18, Fanconi anemia, and thrombocytopenia-absent-radius syndrome, not commonly associated with ectrodactyly. The present paper describes a case of ectrodactyly associated with bilateral radial ray defects, diagnosed in the first trimester of pregnancy, in a fetus affected by trisomy 10. Only four cases of sporadic and isolated ectrodactyly, diagnosed by ultrasonography between 14 and 22 weeks' gestation, have been reported. To our knowledge, the present case is the first report of mosaic trisomy 10 associated with SHFM and radial aplasia. Trisomy 10 is a rare lethal chromosomal abnormality, most frequently found in abortion products. Only six liveborn mosaic trisomy 10 infants, with severe malformations, dead in early infancy, have been reported. A severe clinical syndrome can be defined, comprising ear abnormalities, cleft lip/palate, malformations of eyes, heart, and kidneys, and deformity of hands and feet and most often associated with death neonatally or in early infancy. PMID:23401811

Lévy, Jonathan; Jouannic, Jean-Marie; Saada, Julien; Dhombres, Ferdinand; Siffroi, Jean-Pierre; Portnoï, Marie-France

2013-01-01

288

Status epilepticus in a child with Sanjad Sakati syndrome.  

PubMed

We report a 6-year-old boy who presented with status epilepticus, who had facial dysmorphism, growth and mental retardation. On investigation, he had hypocalcaemia, hypoparathyroidism and bilateral calcification of basal ganglia in cranial tomographs; features consistent with Sanjad Sakati syndrome. He was treated with intravenous calcium gluconate initially followed by oral calcium and calcitriol and recovered completely. PMID:23378547

Prasad, Rajniti; Kumari, Chhaya; Mishra, Om Prakash; Singh, Utpal Kant

2013-01-01

289

Dance Therapy with Physical Therapy for Children with Down Syndrome.  

ERIC Educational Resources Information Center

This study sought to investigate effects of a dance program on bilateral toe-standing balance and single-point static balance skills of a group of children with Down Syndrome. Thirteen experimental and 10 control group students between the ages of 3 and 13 years were assessed on toe-standing balance and single-point standing balance on the right…

Dupont, Blanche Burt; Schulmann, Diana

290

Status epilepticus in a child with Sanjad Sakati syndrome  

PubMed Central

We report a 6-year-old boy who presented with status epilepticus, who had facial dysmorphism, growth and mental retardation. On investigation, he had hypocalcaemia, hypoparathyroidism and bilateral calcification of basal ganglia in cranial tomographs; features consistent with Sanjad Sakati syndrome. He was treated with intravenous calcium gluconate initially followed by oral calcium and calcitriol and recovered completely. PMID:23378547

Prasad, Rajniti; Kumari, Chhaya; Mishra, Om Prakash; Singh, Utpal Kant

2013-01-01

291

A case of Ross syndrome presented with Horner and chronic cough  

PubMed Central

Ross syndrome is a rare sweating disorder associated with Adie's tonic pupil, decreased or diminished tendon reflex and unknown etiology. Although autonomic disturbances affecting sudomotor and vasomotor functions are seen commonly, they are rarely symptomatic. While Ross syndrome is typically characterized with dilated tonic pupil, it may be rarely manifested with miotic pupils (little old Adie's pupil), which can make diagnosis difficult. In this article, we aim to specify the atypical clinical manifestations of syndrome by means of Ross syndrome manifested by autonomic symptoms, Horner syndrome, chronic cough together with bilateral little old Adie's pupil. PMID:25288844

Baran, Aslihan; Balbaba, Mehmet; Demir, Caner F.; Özdemir, Hasan H.

2014-01-01

292

A case of Ross syndrome presented with Horner and chronic cough.  

PubMed

Ross syndrome is a rare sweating disorder associated with Adie's tonic pupil, decreased or diminished tendon reflex and unknown etiology. Although autonomic disturbances affecting sudomotor and vasomotor functions are seen commonly, they are rarely symptomatic. While Ross syndrome is typically characterized with dilated tonic pupil, it may be rarely manifested with miotic pupils (little old Adie's pupil), which can make diagnosis difficult. In this article, we aim to specify the atypical clinical manifestations of syndrome by means of Ross syndrome manifested by autonomic symptoms, Horner syndrome, chronic cough together with bilateral little old Adie's pupil. PMID:25288844

Baran, Aslihan; Balbaba, Mehmet; Demir, Caner F; Ozdemir, Hasan H

2014-10-01

293

Wolfram syndrome maps to distal human chromosome 4p  

SciTech Connect

Wolfram syndrome (MIM 222300) is an autosomal recessive disorder defined by the occurrence of diabetes mellitus and progressive bilateral optic atrophy. Wolfram syndrome homozygotes develop widespread nervous system abnormalities; in particular, they exhibit severe behavioral difficulties that often lead to suicide attempts or psychiatric hospitalizations. The Wolfram syndrome gene also predisposes heterozygous carriers to psychiatric disorders. Since these heterozygotes are common in the general population, the Wolfram syndrome gene may contribute significantly to the overall burden of psychiatric illness. Based on a linkage analysis of 11 families segregating for this syndrome, using microsatellite repeat polymorphisms throughout the human genome, we found the Wolfram syndrome gene to be linked to markers on the short arm of human chromosome 4, with Zmax=6.46 at {theta}=0.02 for marker D4S431.

Polymeropoulos, M.H. [National Center for Human genome Research, Bethesda, MD (United States); Swift, R.; Swift, M. [New York Medical College, Hawthorne, NY (United States)

1994-09-01

294

Refeeding syndrome.  

PubMed

Refeeding syndrome is a complex syndrome that occurs as a result of reintroducing nutrition (oral, enteral or parenteral) to patients who are starved or malnourished. Patients can develop fluid-balance abnormalities, electrolyte disorders (hypophosphataemia, hypokalaemia and hypomagnesaemia), abnormal glucose metabolism and certain vitamin deficiencies. Refeeding syndrome encompasses abnormalities affecting multiple organ systems, including neurological, pulmonary, cardiac, neuromuscular and haematological functions. Pathogenic mechanisms involved in the refeeding syndrome and clinical manifestations have been reviewed. We provide suggestions for the prevention and treatment of refeeding syndrome. The most important steps are to identify patients at risk, reintroduce nutrition cautiously and correct electrolyte and vitamin deficiencies properly. PMID:19712606

Fernández López, M T; López Otero, M J; Alvarez Vázquez, P; Arias Delgado, J; Varela Correa, J J

2009-01-01

295

[Bartter's syndrome].  

PubMed

Bartter syndrome is an uncommon tubular disorder inherited as an autosomal recessive entity. It is associated with hypokalemic metabolic alkalosis with high renin and aldosterone plasma concentration with low or normal blood pressure. Recent studies have demonstrated genetic heterogeneity in Bartter syndrome. Mutations of two genes encoding the Na/K/2Cl cotransporter and potassium channel ROMK are responsible for clinical features of neonatal Bartter syndrome. Mutations of gen encoding the chloride channel ClC-Kb is identified as being causative for the classic Bartter syndrome. And dysfunction of Na/Cl cotransporter in the distal convoluted renal tubule is described as Gitelman syndrome. PMID:15518434

Daniluk, Urszula; Kaczmarski, Maciej; Wasilewska, Jolanta; Matuszewska, Elzbieta; Semeniuk, Janusz; Sidor, Katarzyna; Krasnow, Aleksander

2004-05-01

296

A Neonate with CLOVES Syndrome  

PubMed Central

Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE) syndrome is a recently delineated disorder that comprises vascular malformations (typically truncal), dysregulated adipose tissue, scoliosis, enlarged bony structures (typically of the legs) without progression, or distorting bony overgrowth. The name CLOVE was subsequently extended to CLOVES to emphasize the association with scoliosis/skeletal and spinal anomalies and seizures/central nervous system malformations. We herein report a very rare case of CLOVES syndrome with the findings of lipomatous overgrowth in the cheek (facial asymmetry), vascular malformation (hemangiomas), epidermal nevi (large port wine stains), and skeletal abnormalities (widened first interdigital space, dystrophia in the nail of the first digit of the right foot, and bilateral hypertrophy of the first digits of the feet). PMID:25400966

Akin, Mustafa Ali; Kurtoglu, Selim; Tubas, Filiz; Sarici, Serdar Umit

2014-01-01

297

A Neonate with CLOVES Syndrome.  

PubMed

Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE) syndrome is a recently delineated disorder that comprises vascular malformations (typically truncal), dysregulated adipose tissue, scoliosis, enlarged bony structures (typically of the legs) without progression, or distorting bony overgrowth. The name CLOVE was subsequently extended to CLOVES to emphasize the association with scoliosis/skeletal and spinal anomalies and seizures/central nervous system malformations. We herein report a very rare case of CLOVES syndrome with the findings of lipomatous overgrowth in the cheek (facial asymmetry), vascular malformation (hemangiomas), epidermal nevi (large port wine stains), and skeletal abnormalities (widened first interdigital space, dystrophia in the nail of the first digit of the right foot, and bilateral hypertrophy of the first digits of the feet). PMID:25400966

Sarici, Dilek; Akin, Mustafa Ali; Kurtoglu, Selim; Tubas, Filiz; Sarici, Serdar Umit

2014-01-01

298

Mechanism of Highly Synchronized Bilateral Hippocampal Activity  

PubMed Central

In vivo studies of epileptiform discharges in the hippocampi of rodents have shown that bilateral seizure activity can sometimes be synchronized with very small delays (< 2 ms). This observed small time delay of epileptiform activity between the left and right CA3 regions is unexpected given the physiological propagation time across the hemispheres (> 6 ms). The goal of this study is to determine the mechanisms of this tight synchronization with in-vitro electrophysiology techniques and computer simulations. The hypothesis of a common source was first eliminated by using an in-vitro preparation containing both hippocampi with a functional ventral hippocampal commissure (VHC) and no other tissue. Next, the hypothesis that a noisy baseline could mask the underlying synchronous activity between the two hemispheres was ruled out by low noise in-vivo recordings and computer simulation of the noisy environment. Then we built a novel bilateral CA3 model to test the hypothesis that the phenomenon of very small left-to-right propagation delay of seizure activity is a product of epileptic cell network dynamics. We found that the commissural tract connectivity could decrease the delay between seizure events recorded from two sides while the activity propagated longitudinally along the CA3 layer thereby yielding delays much smaller than the propagation time between the two sides. The modeling results indicate that both recurrent and feedforward inhibition were required for shortening the bilateral propagation delay and depended critically on the length of the commissural fiber tract as well as the number of cells involved in seizure generation. These combined modeling/experimental studies indicate that it is possible to explain near perfect synchronization between the two hemispheres by taking into account the structure of the hippocampal network. PMID:24262205

Wang, Y.; Toprani, S.; Tang, Y.; Vrabec, T.; Durand, D.M.

2014-01-01

299

Mechanism of highly synchronized bilateral hippocampal activity.  

PubMed

In vivo studies of epileptiform discharges in the hippocampi of rodents have shown that bilateral seizure activity can sometimes be synchronized with very small delays (<2 ms). This observed small time delay of epileptiform activity between the left and right CA3 regions is unexpected given the physiological propagation time across the hemispheres (>6 ms). The goal of this study is to determine the mechanisms of this tight synchronization with in-vitro electrophysiology techniques and computer simulations. The hypothesis of a common source was first eliminated by using an in-vitro preparation containing both hippocampi with a functional ventral hippocampal commissure (VHC) and no other tissue. Next, the hypothesis that a noisy baseline could mask the underlying synchronous activity between the two hemispheres was ruled out by low noise in-vivo recordings and computer simulation of the noisy environment. Then we built a novel bilateral CA3 model to test the hypothesis that the phenomenon of very small left-to-right propagation delay of seizure activity is a product of epileptic cell network dynamics. We found that the commissural tract connectivity could decrease the delay between seizure events recorded from two sides while the activity propagated longitudinally along the CA3 layer thereby yielding delays much smaller than the propagation time between the two sides. The modeling results indicate that both recurrent and feedforward inhibition were required for shortening the bilateral propagation delay and depended critically on the length of the commissural fiber tract as well as the number of cells involved in seizure generation. These combined modeling/experimental studies indicate that it is possible to explain near perfect synchronization between the two hemispheres by taking into account the structure of the hippocampal network. PMID:24262205

Wang, Y; Toprani, S; Tang, Y; Vrabec, T; Durand, D M

2014-01-01

300

Bilateral TMJ Involvement in Rheumatoid Arthritis  

PubMed Central

Rheumatoid arthritis (RA) is a systemic inflammatory, slowly progressive disease that results in cartilage and bone destruction. Temporomandibular joint (TMJ) involvement is not uncommon in RA, and it is present in about more than 50% of patients; however, TMJ is usually among the last joints to be involved and is associated with many varied clinical signs and symptoms. Hence, RA of TMJ presents to the dentist with great diagnostic challenges. This report presents a case of RA with bilateral TMJ involvement with its classical radiographic findings and review literature. PMID:24804125

Ruparelia, Pritesh B.; Shah, Deep S.; Ruparelia, Kosha; Sutaria, Shreyansh P.; Pathak, Deep

2014-01-01

301

Topiramate induced bilateral angle-closure glaucoma.  

PubMed

Topiramate is a sulfamate-substituted monosaccharide and is used as an antiepileptic medication. It can also be used as a prophylactic agent for headache from migraine. The main purpose of this article is to create awareness among medical colleagues regarding severe ophthalmic side effect of topiramate. This is the first reported case of topiramate induced bilateral angle-closure glaucoma in Nepal. This case was managed successfully by discontinuing topiramate and by starting anti-glaucoma medication along with atropine eye drops which were tapered and eventually discontinued. PMID:18604026

Singh, S K; Thapa, S S; Badhu, B P

2007-01-01

302

Progressive bilateral thinning of the parietal bones  

SciTech Connect

Observation of a case of progressive bilateral parietal thinning within a period of 14 years induced us to study skull films of 3 636 consecutive patients. Parietal thinning was found in 86 patients (2.37%). It was more common in women, with a sex ratio of 1:1.9. The mean age of the females was 72 years, and that of the males 63 years. Previous skull films of 25 of these patients were available and showed progression in 10. It is concluded that parietal thinning is a slowly progressive disease of middle-aged and old patients and is not an anatomical variant or congenital dysplasia of the dipole.

Cederlund, C.G.; Andren, L.; Olivecrona, H.

1982-03-01

303

Bilateral Hydronephrosis and Cystitis Resulting from  

PubMed Central

Ketamine associated urinary dysfunction has become increasingly more common worldwide. Point-of-care ultrasound (POCUS) is an established modality for diagnosing hydronephrosis in the emergency department. We describe a case of a young male ketamine abuser with severe urinary urgency and frequency in which POCUS performed by the emergency physician demonstrated bilateral hydronephrosis and a focally thickened irregular shaped bladder. Emergency physicians should consider using POCUS evaluate for hydronephrosis and bladder changes in ketamine abusers with lower urinary tract symptoms. The mainstay of treatment is discontinuing ketamine abuse. PMID:25035738

Abuse, Chronic Ketamine; Tran, Vu Huy; Nelson, Mathew; Nogar, Joshua; Bramante, Robert M.

2014-01-01

304

Bilateral hydronephrosis and cystitis resulting from.  

PubMed

Ketamine associated urinary dysfunction has become increasingly more common worldwide. Point-of-care ultrasound (POCUS) is an established modality for diagnosing hydronephrosis in the emergency department. We describe a case of a young male ketamine abuser with severe urinary urgency and frequency in which POCUS performed by the emergency physician demonstrated bilateral hydronephrosis and a focally thickened irregular shaped bladder. Emergency physicians should consider using POCUS evaluate for hydronephrosis and bladder changes in ketamine abusers with lower urinary tract symptoms. The mainstay of treatment is discontinuing ketamine abuse. PMID:25035738

Abuse, Chronic Ketamine; Tran, Vu Huy; Nelson, Mathew; Nogar, Joshua; Bramante, Robert M

2014-07-01

305

[Congenital bilateral dacryocystitis and craniofacial dysraphia].  

PubMed

It is presented the observation of a child of 8 years old having a bilateral congenital dacryocyst by the absence of the lacrimonasal duct and multiple facial malformations are: telorbitismus, frontal meningocele, some lesions determined by a craniofacial dehiscence (fissura 2-12) with paramedian dysraphia. The affection has been produced by a disturbance of embryogenesis that occurred during the V-VI intrauterine development weeks, by metrorrhagias and repeated hypoxia that determined a disturbance at the level of the welding frontal buds with meningocele, of the olfactive guttier and the mass of the ethmoid with telorbitism and arthresia of the lacrimal duct. PMID:1525141

Cernea, P; Talea, L

1992-01-01

306

Bilateral vertebral artery stenosis present with vertigo  

PubMed Central

Of ischaemic stroke patients, about 25% rise from the posterior or vertebrobasilar system. The ischaemia of vertebral arteries may emerge for different vascular pathological reasons, at different localisations and with different clinical findings. Despite its low morbidity and mortality risk, early diagnosis and treatment is of importance. Vertebrobasilar ischaemia symptoms can be observed clinically such as vertigo, tinnitus, double vision, headache, hypokinesis and hearing disorders, etc. In this article, a 42-year-old stroke patient case is presented, who applied to the emergency service with vertigo and then, was diagnosed with bilateral vertebral artery stenosis by means of cranial MR angiography. PMID:23376658

Kotan, Dilcan; Sayan, Saadet; Acar, Bilgehan Atilgan; Polat, Pinar

2013-01-01

307

Bilateral ossiculoplasty in 1 case of achondroplasia.  

PubMed

Achondroplasia is the most common skeletal dysplasia and it combines various complications with normal longevity. Hearing disturbance due to otitis media or an ossicular anomaly is one of the most common complications. Conductive hearing loss is suggested as the most common form of hearing loss. Temporal bone and middle ear structures are distorted in achondroplasia because of rotational change of the skull base. Authors experienced a case of an achondroplastic patient with bilateral hearing disturbance. We faced making a potential mistake during the previous operation but a favorable postoperative result occurred. Our experience could be helpful to other clinicians who face achondroplastic patients. PMID:24653923

Jung, Jongyoon; Yang, Chulwon; Lee, Sunkyu; Choi, June

2013-12-01

308

Bilateral Ossiculoplasty in 1 Case of Achondroplasia  

PubMed Central

Achondroplasia is the most common skeletal dysplasia and it combines various complications with normal longevity. Hearing disturbance due to otitis media or an ossicular anomaly is one of the most common complications. Conductive hearing loss is suggested as the most common form of hearing loss. Temporal bone and middle ear structures are distorted in achondroplasia because of rotational change of the skull base. Authors experienced a case of an achondroplastic patient with bilateral hearing disturbance. We faced making a potential mistake during the previous operation but a favorable postoperative result occurred. Our experience could be helpful to other clinicians who face achondroplastic patients. PMID:24653923

Jung, Jongyoon; Yang, Chulwon; Lee, Sunkyu

2013-01-01

309

Restoration algorithm for image noise removal using double bilateral filtering  

NASA Astrophysics Data System (ADS)

We propose a double bilateral (DoBi) filter that consists of a classical bilateral filter and a new bilateral filter for image restoration. Bilateral filtering is a simple, noniterative, and effective denoising filter that smooths images while preserving edges by means of a nonlinear combination of adjacent pixel values. A median-metric weighting function is introduced by incorporating a switching median filter into the similarity function. This median-metric component associated with a spatial function constitute the second bilateral filter, which compensates the classical bilateral filter. Moreover, a parameter automation mechanism is proposed to facilitate the restoration procedure. A wide variety of images contaminated by various degrees of Gaussian, impulse, and mixed noise were used to assess the performance of this new restoration algorithm. Experimental results indicated that the DoBi filter outperformed several state-of-the-art methods in both visual image quality and restored signal quantity.

Chang, Herng-Hua; Chu, Woei-Chyn

2012-04-01

310

Multispectral image denoising with optimized vector bilateral filter.  

PubMed

Vector bilateral filtering has been shown to provide good tradeoff between noise removal and edge degradation when applied to multispectral/hyperspectral image denoising. It has also been demonstrated to provide dynamic range enhancement of bands that have impaired signal to noise ratios (SNRs). Typical vector bilateral filtering described in the literature does not use parameters satisfying optimality criteria. We introduce an approach for selection of the parameters of a vector bilateral filter through an optimization procedure rather than by ad hoc means. The approach is based on posing the filtering problem as one of nonlinear estimation and minimization of the Stein's unbiased risk estimate of this nonlinear estimator. Along the way, we provide a plausibility argument through an analytical example as to why vector bilateral filtering outperforms bandwise 2D bilateral filtering in enhancing SNR. Experimental results show that the optimized vector bilateral filter provides improved denoising performance on multispectral images when compared with several other approaches. PMID:24184727

Peng, Honghong; Rao, Raghuveer; Dianat, Sohail A

2014-01-01

311

The effects of X monosomy on brain development: monozygotic twins discordant for Turner's syndrome.  

PubMed

Monosomy for the X chromosome is the most frequent cause of Turner's syndrome, a common clinical syndrome associated with particular physical and neurobehavioral features. The results from comprehensive assessment of prepubertal monozygotic female twins discordant for X monosomy are presented. Zygosity was established with DNA Fingerprinting and no evidence of chromosomal mosaicism was seen in either child. Physical features in the affected twin were relatively mild with respect to the full spectrum of physical malformations and disabilities associated with Turner's syndrome. The neurobehavioral phenotypes of the twins were compared. Although both sisters scored in the superior range of intelligence, the affected twin's Performance IQ was 18 points less than her sister, whereas Verbal IQ showed only a 3-point difference between the sisters. Other relative differences were noted within the executive, visuospatial, and visuomotor domains of function. Behavioral evaluation indicated greater problems with attention, hyperactivity, and anxiety in the affected twin. Quantitative analysis of brain anatomy revealed evidence of both general and regional effects of X monosomy on neurodevelopment. Cerebrospinal fluid volume was increased by 25% in the affected twin compared with her sister with a corresponding decrease in gray matter volume. The right frontal, right parietal-occipital, and left parietal-perisylvian regions showed the greatest discrepancy between the sisters with respect to increased cerebrospinal fluid and decreased gray matter volumes in twin with X monosomy. Differences in the posterior fossa were also noted with a 50% relative increase in the volumes of the fourth ventricle and cisterna magna and a 10 to 15% relative reduction in size of the cerebellar vermis, pons, and medulla in the affected twin. The association between the neurobehavioral and neuroanatomical findings in the affected twin is discussed. The unique nature of the naturally occurring genetic phenomenon seen in this twin pair provides an opportunity to more fully elucidate the neurobehavioral phenotype associated with X monosomy and Turner's syndrome. PMID:8517687

Reiss, A L; Freund, L; Plotnick, L; Baumgardner, T; Green, K; Sozer, A C; Reader, M; Boehm, C; Denckla, M B

1993-07-01

312

Bilateral luxatio erecta with greater tuberosity fracture: a case report  

PubMed Central

Bilateral shoulder dislocation with greater tuberosity fracture and luxatio erecta, both are rare by themselves, with only few reports of each. We report an unusual case of posttraumatic bilateral symmetrical shoulder dislocation involving luxatio erecta with greater tuberosity fracture in a young male. To our knowledge, this is the first case of symmetrical bilateral shoulder dislocation with greater tuberosity fracture involving luxatio erecta dislocation from Indian subcontinent.

Saxena, Vikas; Pradhan, Pavan

2013-01-01

313

Pediatric bilateral large concurrent thalamic glioblastoma: An unusual case report  

PubMed Central

Bilateral thalamic tumors are rare. Pediatric bilateral thalamic glioblastomas are even rarer, only five cases reported in the English literature till date. The clinical presentation, natural history, and prognosis of pediatric thalamic tumors are still relatively obscure. In this article, we report an 8-year-old patient with large bilateral thalamic glioblastomas and briefly discuss its clinical presentation, possible modalities of management, and prognosis, in the light of available literature. PMID:24891914

Pandey, Nityanand; Singh, Pankaj Kumar; Mahapatra, Ashok K.; Kakkar, Aanchal; Sharma, Bhawani Shankar

2014-01-01

314

Bilateral angiosarcoma of breast in a young lady.  

PubMed

We report a young lady with bilateral angiosarcoma of breast because of its rarity. A 29 year old unmarried female presented with bilateral breast lump. She underwent bilateral mastectomy with axillary dissection histopathology of which showed low grade angiosarcoma of left breast and high grade angiosarcoma of right breast. CT Thorax revealed right hilar and right mediastenal lymph nodes. She was treated with radiotherapy and chemotherapy postoperatively. PMID:22557784

Roy, Sanjoy; Devleena; Maji, Tapas; Choudhuri, Prabir; Biswas, Jaydip; Lahiri, Debarshi

2011-07-01

315

Bilateral chylothorax: an unusual complication of cervical rib resection.  

PubMed

Bilateral chylothorax is a rare cause of pleural effusions. Here we report an unusual acute presentation of bilateral chylothorax following thoracic outlet surgery. Unique to this case was the disparate characteristics of pleural fluid analyses with an exudate on the left and a transudate on the right. This report describes the recognition and management of bilateral chylothoraces, an uncommon but potentially serious complication of this frequently performed surgical procedure. PMID:25553240

Morjaria, Jaymin B; Aslam, Imran; Johnson, Brian; Greenstone, Michael A; Kastelik, Jack A

2015-01-01

316

A rare case of bilateral sequential spermatocytic seminoma  

PubMed Central

Spermatocytic seminoma (SS) is a rare testicular neoplasm characterized by a palpable, painless, slowly enlarging mass in the testis. Even more rare is a synchronous bilateral presentation. Only eight cases of bilateral SS have been reported in the literature, of which three cases were present with synchronous testis enlargement, and five were sequential. Here, we report an additional case of synchronous bilateral SS and present a comprehensive relevant literature review concerning clinical features, histopathology, and treatment. PMID:23915031

2013-01-01

317

Bilateral chylothorax: an unusual complication of cervical rib resection  

PubMed Central

Bilateral chylothorax is a rare cause of pleural effusions. Here we report an unusual acute presentation of bilateral chylothorax following thoracic outlet surgery. Unique to this case was the disparate characteristics of pleural fluid analyses with an exudate on the left and a transudate on the right. This report describes the recognition and management of bilateral chylothoraces, an uncommon but potentially serious complication of this frequently performed surgical procedure. PMID:25553240

Aslam, Imran; Johnson, Brian; Greenstone, Michael A.; Kastelik, Jack A.

2015-01-01

318

Multiple osteochondroses of bilateral knee joints.  

PubMed

We experienced a patient with a combination of multiple osteochondroses: Blount's disease, bipartite patella, and Sinding-Larsen-Johansson disease in the left knee, and a combination of bipartite patella and Osgood-Schlatter disease in the right knee. The patient was a healthy, active 12-year-old boy with bilateral knee pain. He had been diagnosed with Blount's disease of the left tibia at 2 years of age, and had been treated with open wedge osteotomy. He was diagnosed with bilateral bipartite patellae at the age of 9 years, and was diagnosed with Osgood-Schlatter disease in the right knee and Sinding-Larsen-Johansson disease in the left knee at 10 years of age. The second growth spurt was observed during this period. At 11 years of age, he was diagnosed with an osteochondral fracture of the left lateral femoral condyle and was observed without surgery. This patient showed the sequential appearance of an ossification disorder, probably due to the abnormal response of enchondral ossification to mechanical stress. Overuse in this growth period may have played a role in the development of these osteochondroses. The osteochondral fracture was probably caused by a disruption at one of the weakest parts of the developing skeleton, between the ossification center and the overlying cartilage in the background of an ossification disorder. PMID:11484125

Segawa, H; Omori, G; Koga, Y

2001-01-01

319

Bilateral clavicle osteomyelitis: A case report  

PubMed Central

INTRODUCTION Osteomyelitis of the clavicle is rare. Infection occurs from hematogenous spread or trauma. In adults infection is usually secondary due to an exogenous cause such as open fractures, surgery (iatrogenic) or spread from local tissue with infection. PRESENTATION OF CASE The case is presented here of a 50-year old female with bilateral clavicular fractures, who was operated on with open reduction and internal fixation. At the 6-month follow-up, she had complaints of bilateral osteomyelitis which was successfully treated with resection of the infected segment of the bone, and antibiotic impregnated collagen. DISCUSSION Predisposing factors include diabetes, intravenous drug abuse, tuberculosis or immune suppression. Management involves the removal of bone fixation, debridement of the bone and if there is a defect, coverage with a muscle flap is applied. CONCLUSION In cases of clavicular osteomyelitis where infection continues despite debridement and antibiotic therapy, excision of the sequestered clavicular section is a successful treatment approach and has been seen to improve quality of life without any functional loss. PMID:25460439

Saglam, Fevzi; Saglam, Seymanur; Gulabi, Deniz; Eceviz, Engin; Elmali, Nurzat; Yilmaz, Mustafa

2014-01-01

320

Idiopathic bilateral vestibulopathy: an autoimmune disease?  

PubMed

Bilateral vestibulopathy (BV) is the loss of function of both peripheral labyrinths or of the eighth nerves. Its etiology remains obscure in approximately 20% to 50% of cases (so-called idiopathic bilateral vestibulopathy, IBV). Alternatively, the cause could be viral or vascular; to date, causative gene mutations have not been identified. Other potential disease mechanisms include autoimmune disorders. Antibodies have been detected against inner ear tissue (primarily against vestibular membranous labyrinth). The data suggest that the bulk of anti-labyrinthine autoantibodies may be an epiphenomenon, but a small subgroup of organ-specific autoantibodies may synergize with a cellular response to develop vestibular lesions. The two key symptoms of BV are the following: 1. unsteadiness of gait, particularly in the dark or on uneven ground, and 2. oscillopsia associated with head movements. Episodes of vertigo are reported by patients with IBV, particularly early in the development of vestibular loss. Associated hearing loss seldom occurs in the idiopathic type of this condition. Post-mortem examinations revealed a remarkably selective loss of vestibular hair cells in the vestibular end organs but normal hair cells in the cochlea. The diagnosis is made with a simple bedside test for defective vestibular function. The diagnosis can be confirmed by bithermal caloric testing and pendular body rotation. The therapy is based on steroid treatment, and the early initiation of immunosuppression appears to be essential for therapeutic success. PMID:25173622

Greco, Antonio; De Virgilio, Armando; Gallo, Andrea; Fusconi, Massimo; Ruoppolo, Giovanni; Turchetta, Rosaria; Pagliuca, Giulio; de Vincentiis, Marco

2014-10-01

321

"Mirror-Image" Bilateral Giants: Intracavernous Carotid Artery Aneurysms  

PubMed Central

Summary The literature on the incidence of "mirror image" bilateral giant intracavernous aneurysms, their symptoms and their association with other entities is reviewed, with a brief comment on their evolution and treatment. A case of "mirror image" bilateral giant intracavernous aneurysms in a 76-year-old man who presented a sudden diplopia with pupillary sparing is reported. A CT scan showed parasellar images and dolichomega circle of Willis arteries that enhanced with endovenous contrast. MRI and angiography disclose bilateral aneurysms in detail, associated with an anomalous origin of the left common carotid artery and bilateral renal artery stenosis. PMID:20569579

Díaz, M.B.; Mercado, F.C.; Lemme Plaghos, L.A.

2006-01-01

322

Bilateral aniridia lenticular coloboma and snowflake retinal degeneration.  

PubMed

A 6-year-old boy presented with bilateral aniridia associated with lens coloboma and snowflake retinal degeneration. Ophthalmologic examination revealed bilateral corneal peripheral epithelial thickening and aniridia. Additionally, the patient had lenticular coloboma and snowflake retinal degeneration in both eyes. Intraocular pressure was 22 mm Hg bilaterally. The patient also had pendular nystagmus. Uncorrected visual acuity was counting fingers at 2 meters for both eyes, but improved to 0.2 and 0.05, respectively, with correction. Congenital aniridia has been reported with various ophthalmic pathologies, but this is the first case to display bilateral lenticular coloboma and snowflake retinal degeneration associated with aniridia. PMID:19205498

Doganay, Selim; Emre, Sinan; Firat, Penpegül

2009-01-01

323

Simultaneous bilateral hernia repair. A case against conventional wisdom.  

PubMed Central

The timing of bilateral hernia repair remains controversial. Because of reported high recurrence rates after simultaneous bilateral repair, staged procedures have been suggested. This study determined recurrence and complication rates of unilateral versus simultaneous bilateral repair. Of 659 patients undergoing hernia repair between 1974 and 1980, 333 underwent unilateral repair and 329 had simultaneous bilateral repair. More than 90% of patients were followed until death or a minimum of 60 months (median, 104 months). Perioperative complications were associated with 18% of repairs. More morbidity occurred in the bilateral group. However complication rates for specific events were not significantly different, except for urinary retention, which occurred in 20 patients (6.1%) of the unilateral group and 49 (15%) of the bilateral group (p less than 0.001). Overall 25 recurrences occurred in the unilateral group and 31 in the bilateral group. Recurrence rates at 5 and 9 years were, respectively, 4.8% and 8.8% in the unilateral group and 5.0% and 9.1% in the bilateral group (p = 0.861). These data suggest that simultaneous bilateral inguinal herniorrhaphy does not result in increased rates of most postoperative complications or recurrence when compared with unilateral repair. PMID:1998409

Miller, A R; van Heerden, J A; Naessens, J M; O'Brien, P C

1991-01-01

324

Secondary pseudoainhum in a patient with Turner syndrome.  

PubMed

Secondary pseudoainhum is an autoamputation that develops in individuals aged approximately 20 to 50 years and is caused by diseases such as keratodermas, trauma, or congenital factors. The authors report a novel case of secondary pseudoainhum in a patient with Turner syndrome (45,X) who presented with bandlike constrictions in the toes bilaterally. To the authors' knowledge, secondary pseudoainhum has not been reported to be associated with Turner syndrome. However, physicians should be aware of this potentially deforming disease in patients with Turner syndrome. PMID:25288716

Davis, Brady S; Harris, Scott; Forman, Mitchell D

2014-10-01

325

Ellis-van Creveld syndrome with facial hemiatrophy.  

PubMed

Ellis-van Creveld (EVC) syndrome is a rare autosomal recessive congenital disorder characterized by chondrodysplasia and polydactyly, ectodermal dysplasia and congenital defects of the heart. We present here a case of a 16-year-old short-limbed dwarf with skeletal deformities and bilateral postaxial polydactyly, dysplastic nails and teeth, also having left-sided facial hemiatrophy. The diagnosis of EVC syndrome was made on the basis of clinical and radiological features. To the best of our knowledge, this is the first report of EVC syndrome with facial hemiatrophy in the medical literature from India. PMID:20445298

Bhat, Yasmeen J; Baba, Asif Nazir; Manzoor, Sheikh; Qayoom, Seema; Javed, Sheikh; Ajaz, Hakeem

2010-01-01

326

Laparoscopic Adrenalectomy in a Patient of Von Hippel Lindau Syndrome with Ventriculo-Peritoneal Shunt-Anaesthetic management  

PubMed Central

Von Hippel Lindau (VHL) syndrome has a predilection to manifest multiple haemangioblastomas in the retina and central nervous system. We report a rare case of raised intracranial pressure during bilateral laparoscopic adrenalectomy for pheochromocytoma in a patient of VHL syndrome who had a ventriculoperitoneal shunt. PMID:25386452

Mungasuvalli, Nagaraj Channappa; Chinnappa, Jithendra; Iyer, Sadasivan Shanker; Thammanna, Prathima Padavarahalli

2014-01-01

327

Long-term low-dose ketoconazole treatment in bilateral macronodular adrenal hyperplasia  

PubMed Central

Summary Medical therapy for Cushing's syndrome due to bilateral macronodular adrenal hyperplasia (BMAH) is generally administered for a limited time before surgery. Aberrant receptors antagonists show inconsistent efficacy in the long run to prevent adrenalectomy. We present a patient with BMAH, treated for 10 years with low doses of ketoconazole to control cortisol secretion. A 48-year-old woman presented with headaches and hypertension. Investigations showed the following: no clinical signs of Cushing's syndrome; enlarged lobulated adrenals; normal creatinine, potassium, and aldosterone; normal urinary aldosterone and metanephrines; elevated urinary free cortisol and steroid metabolites; and suppressed plasma renin activity and ACTH. A screening protocol for aberrant adrenal receptors failed to show any illegitimate hormone dependence. Ketoconazole caused rapid normalisation of cortisol and ACTH that persists over 10 years on treatment, while adrenals show no change in shape or size. Ketoconazole decreases cortisol in patients with Cushing's syndrome, and may prevent adrenal overgrowth. Steroid secretion in BMAH is inefficient as compared with normal adrenals or secreting tumours and can be controlled with low, well-tolerated doses of ketoconazole, as an alternative to surgery. Learning points Enlarged, macronodular adrenals are often incidentally found during the investigation of hypertension in patients harboring BMAH. Although laboratory findings include low ACTH and elevated cortisol, the majority of patients do not display cushingoid features.Bilateral adrenalectomy, followed by life-long steroid replacement, is the usual treatment of this benign condition, and alternative medical therapy is sought. Therapy based on aberrant adrenal receptors gives disappointing results, and inhibitors of steroidogenesis are not always well tolerated.However, ketoconazole at low, well-tolerated doses appeared appropriate to control adrenal steroid secretion indefinitely, while preventing adrenal overgrowth. This treatment probably constitutes the most convenient long-term alternative to surgery. PMID:25535576

Comte-Perret, Sophie; Zanchi, Anne

2014-01-01

328

Intracranial Volume in 15 Children with Bilateral Coronal Craniosynostosis  

PubMed Central

Background: Intracranial volume (ICV) growth in patients with bilateral coronal craniosynostosis (BCS) is not well described. It is therefore important to evaluate the consequences of cranial surgery in children with this condition. The aim of the present study was to evaluate ICVs in patients operated on for BCS. Methods: A consecutive series of patients with BCS were operated on using spring-assisted cranioplasty, with computed tomography scans in 0.6-mm slices, were included. A MATLAB-based computer program capable of measuring ICV was used. Patients were compared with an age- and gender-matched control group of healthy children. Student’s t test was used for statistical analysis. Results: Fifteen patients (7 girls and 8 boys) with 43 computed tomography scans were identified. The diagnoses were 13 syndromic BCS (3 Apert, 1 Crouzon, 6 Muenke, and 3 Saethre-Chotzen) and 2 nonsyndromic BCS. The mean preoperative volume at the age of 5 months (n = 15) was 887?mL (range, 687–1082). Mean volume at follow-up at the age of 3 years (n = 13) was 1369?mL (range, 1196–1616). In comparison, the mean ICVs for controls at the ages of 5 months (n = 30) and 3 years (n = 26) were 854?mL and 1358?mL, respectively. The differences were not statistically significant (P > 0.05). Conclusions: Patients with BCS were operated on with spring-assisted cranioplasty seem to maintain their age-related ICV at 3 years of age when compared to normal children. PMID:25506526

Maltese, Giovanni; Wikberg, Emma; Bernhardt, Peter; Kölby, Lars; Tarnow, Peter E. W.

2014-01-01

329

Exercise Induced Rhabdomyolysis with Compartment Syndrome and Renal Failure  

PubMed Central

Exertional rhabdomyolysis is sequela that is occasionally seen after strenuous exercise. The progression to compartment syndrome or renal failure is a rare complication that requires prompt recognition and treatment to prevent morbidity (Giannoglou et al. 2007). We present a case of a 22-year-old college football player who presented to the emergency department (ED) after a typical leg workout as part of his weight conditioning. He was found to have rhabdomyolysis with evidence of renal insufficiency. His condition progressed to bilateral compartment syndrome and renal failure requiring dialysis. After bilateral fasciotomies were performed he had resolution of his compartment syndrome. He continued to be dialysis dependent and had no return of his renal function at discharge 12 days after admission. PMID:25105034

Bhalla, Mary Colleen; Dick-Perez, Ryan

2014-01-01

330

Gorlin Syndrome  

PubMed Central

Gorlin Syndrome, a rare genodermatosis, otherwise known as Nevoid basal cell carcinoma syndrome (NBCCS) is a multisystem disease affecting skin, nervous system, eyes, endocrine glands, and bones. It is characterized by multiple basal cell carcinomas, palmoplantar pits, jaw cysts, and bony deformities like kyphoscoliosis and frontal bossing. We would like to report a case of Gorlin syndrome with classical features, as this is a rare genodermatosis. PMID:23723494

Devi, Basanti; Behera, Binodini; Patro, Sibasish; Pattnaik, Subhransu S; Puhan, Manas R

2013-01-01

331

First case of X-linked dystonia-parkinsonism ("Lubag") to demonstrate a response to bilateral pallidal stimulation.  

PubMed

"Lubag" or X-linked dystonia-parkinsonism (XDP) is a genetic syndrome afflicting Filipino men. Intracranial surgical procedures for Lubag have been unsuccessful. We report a 45-year-old Filipino male with genetically confirmed XDP who underwent bilateral pallidal deep brain stimulation (DBS) surgery. The patient started to exhibit improvement on initial programming, most notably of his severe jaw-opening dystonia. At 1-year follow-up, his Burke-Fahn-Marsden dystonia score and motor Unified Parkinson's Disease Rating Scale score were improved by 71% and 62%, respectively, with the stimulators on compared to stimulators off state. Bilateral pallidal DBS may be a viable option for Lubag patients with medically refractory symptoms. PMID:17579361

Evidente, Virgilio Gerald H; Lyons, Mark K; Wheeler, Mark; Hillman, Renee; Helepolelei, Luann; Beynen, Froukje; Nolte, Dagmar; Müller, Ulrich; Starr, Philip A

2007-09-15

332

Perlman syndrome: report, prenatal findings and review.  

PubMed

Perlman syndrome is a rare overgrowth syndrome characterized by polyhydramnios with neonatal macrosomia, nephromegaly, distinctive facial appearance, renal dysplasia, nephro-blastomatosis, and predisposition to Wilms tumor (WT). We report on a newborn with prenatal sonographic signs of Perlman syndrome, large fetal ascites, nephromegaly and macrosomia. The clinical course was marked by neonatal distress, renal failure and refractory hypoxemia leading to death at 2 days of life. Renal histologic examination showed bilateral nephroblastomatosis. Genetic or epigenetic alterations of the 11p15 region (involved in the BWS), including mutation of the CDKN1C gene were excluded. No mutation of the GPC3 gene was identified. We review the 28 patients who have been reported with Perlman syndrome. The prognosis of Perlman syndrome is poor with a high neonatal mortality rate. Among the infants who survived beyond the neonatal period, 64% developed a WT and all had a developmental delay. Fetal macrosomia, ascites and polyhydramnios are frequent manifestations. Clinical overlaps with other overgrowth syndromes particularly Beckwith-Wiedemann syndrome and Simpson-Golabi-Behmel syndrome have been emphasized. Perlman syndrome is considered as an autosomal recessive condition. We review 19 patients from seven sibships with parental consanguinity in two families only. The other cases were sporadic. The 28 reported patients had only 10 unaffected sibs. The low percentage of consanguinity among parents is also puzzling for a rare recessive condition. The molecular basis of Perlman syndrome is unknown. (Epi)genetic anomalies of 11p15 and mutations in GPC3 were not studied in most of the previous reports. PMID:18780370

Alessandri, Jean-Luc; Cuillier, Fabrice; Ramful, Duksha; Ernould, Sandrine; Robin, Stéphanie; de Napoli-Cocci, Stefan; Rivière, Jean-Pierre; Rossignol, Sylvie

2008-10-01

333

Interstitial Keratitis, Vertigo, and Vasculitis: Typical Cogan's Syndrome  

PubMed Central

Cogan's syndrome (CS) is a chronic inflammatory disorder of unknown etiology that most commonly affects young adults. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between CS and systemic vasculitis as well as aortitis also exists. The diagnosis of CS is based upon presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. We describe classic Cogan's syndrome in a 47-year-old female from Ardabil. The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally. Conjunctival hyperemia, bilateral cataract, and interstitial keratitis were detected with a slit lamp examination. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The other differential diagnosis of CS was studied and ruled out. Pulse i.v. methylprednisolone and cyclophosphamide were given and were followed by oral prednisolone and cyclophosphamide. Clinical follow-up showed partial improvement. PMID:24715922

Azami, Ahad; Kalantar Hormozi, Mohammadreza; Tavosi, Zahra

2014-01-01

334

Interstitial Keratitis, Vertigo, and Vasculitis: Typical Cogan's Syndrome.  

PubMed

Cogan's syndrome (CS) is a chronic inflammatory disorder of unknown etiology that most commonly affects young adults. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between CS and systemic vasculitis as well as aortitis also exists. The diagnosis of CS is based upon presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. We describe classic Cogan's syndrome in a 47-year-old female from Ardabil. The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally. Conjunctival hyperemia, bilateral cataract, and interstitial keratitis were detected with a slit lamp examination. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The other differential diagnosis of CS was studied and ruled out. Pulse i.v. methylprednisolone and cyclophosphamide were given and were followed by oral prednisolone and cyclophosphamide. Clinical follow-up showed partial improvement. PMID:24715922

Azami, Ahad; Maleki, Nasrollah; Kalantar Hormozi, Mohammadreza; Tavosi, Zahra

2014-01-01

335

A very rare bilateral adrenal tumor.  

PubMed

We report a case of very rare adrenal tumor. A 54-year-old patient was classified as affected by bilateral adrenal incidentaloma that surprisingly, on histology resulted solitary fibrous tumors. Solitary fibrous tumor (SFT) is an uncommon mesenchymal neoplasm. Only five cases of localization of SFT in adrenal gland are reported in the literature, while the frequency of retroperitoneum localization is more frequent, about 30 cases. Immunohistochemically, SFT can be positive for CD34 antigen, vimentin, CD99, and bcl-2 and usually negative for cytokeratins, chromogranin A, NSE, neurofilaments, synoptophysin, and S-100. Surgical excision remains the main treatment in fact the recurrence is locoregional and correlated with positive margins due to incomplete excision, while distant metastases are correlated with atypical or malignant features. PMID:24146413

Toniato, Antonio; Boschin, Isabella Merante; Pelizzo, Maria Rosa

2014-04-01

336

Bilateral mechanical rotational vertebral artery occlusion.  

PubMed

Rotational vertebral artery occlusion, or bow hunter's stroke, is reversible, positional symptomatic vertebrobasilar ischemia. The typical mechanism of action is obstruction of a dominant vertebral artery with contralateral head rotation in the setting of baseline ipsilateral vertebral artery stenosis or occlusion. Here we present a rare case of mechanical occlusion of bilateral patent vertebral arteries manifesting as near syncope with rightward head rotation. Diagnostic cerebral angiography showed dynamic right C5 vertebral occlusion and left C2 vertebral occlusion. The patient underwent right C4/5 transverse process decompression. Postoperative angiogram showed patent flow through the right vertebral artery in neutral position and with head turn with resultant resolution of symptoms. PMID:23465174

Dargon, Phong T; Liang, Conrad W; Kohal, Anmol; Dogan, Aclan; Barnwell, Stanley L; Landry, Gregory J

2013-10-01

337

Framing effect following bilateral amygdala lesion  

PubMed Central

A paradigmatic example of an emotional bias in decision making is the framing effect, where the manner in which a choice is posed – as a potential loss or a potential gain – systematically biases an ensuing decision. Two fMRI studies have shown that the activation in the amygdala is modulated by the framing effect. Here, contrary to an expectation based on these studies, we show that two patients with Urbach-Wiethe (UW) disease, a rare condition associated with congenital, complete bilateral amygdala degeneration, exhibit an intact framing effect. However, choice preference in these patients did show a qualitatively distinct pattern compared to controls evident in an increased propensity to gamble, indicating that loss of amygdala function does exert an overall influence on risk-taking. These findings suggest either that amygdala does contribute to decision making but does not play a causal role in framing, or that UW is not a pure lesion model of amygdala function. PMID:20227427

Talmi, Deborah; Hurlemann, René; Patin, Alexandra; Dolan, Raymond J.

2010-01-01

338

Down Syndrome What causes Down syndrome?  

E-print Network

04/13 Down Syndrome What causes Down syndrome? Individuals with Down syndrome usually have an extra chromosome 21. Down syndrome occurs in about 1 in every 700 to 1,000 births. The chance of giving birth to a baby with Down syndrome increases if the mother is over 35 years old. Down syndrome affects both males

Palmeri, Thomas

339

Bilateralism, multilateralism, and the quest for global free trade  

Microsoft Academic Search

We develop an equilibrium theory of trade agreements in which both the degree and the nature (bilateral or multilateral) of trade liberalization are endogenously determined. To determine whether and how bilateralism matters, we also analyze a scenario where countries pursue trade liberalization on only a multilateral basis. We find that when countries have asymmetric endowments or when governments value producer

Kamal Saggi; Halis Murat Yildiz

2009-01-01

340

Bilateralism, multilateralism, and the quest for global free trade  

Microsoft Academic Search

We develop an equilibrium theory of trade agreements in which both the degree and the nature (bilateral or multilateral) of trade liberalization are endogenously determined. To determine whether and how bilateralism matters, we also analyze a scenario where countries pursue trade liberalization on only a multilateral basis. We find that when countries have asymmetric endowments or when governments value producer

Kamal Saggi; Halis Murat Yildiz

2010-01-01

341

Limb bone bilateral asymmetry: variability and commonality among modern humans  

E-print Network

Limb bone bilateral asymmetry: variability and commonality among modern humans Benjamin M. Auerbach the greatest absolute and directional asymmetry among all populations, with lower asymmetry evident in maximum are unique among primates in the magnitude of di- rectional bilateral asymmetry exhibited in both use

Auerbach, Benjamin M.

342

Bilateral congenital ureteral strictures in a young cat.  

PubMed

An 8-month-old cat was presented with bilateral hydronephrosis. Bilateral ureteral obstructions were identified by diagnostic imaging and confirmed by necropsy. Histopathologic findings revealed polypoid transitional epithelial hyperplasia with chronic lymphoplasmacytic inflammation. This report documents congenital ureteral strictures as a cause of ureteral obstruction in a young cat. PMID:25183890

Lee, Namsoon; Choi, Mihyun; Keh, Seoyeon; Oh, Yein; Seo, Jimin; Choi, Heeyeon; Kim, Hyunwook; Yoon, Junghee

2014-09-01

343

Bilateral Cochlear Implantation in Children: Experiences and Considerations  

ERIC Educational Resources Information Center

Between 2000 and 2006, the University Clinic for Ear Nose and Throat and Communication Disorders in Mainz, Germany, performed 41 bilateral cochlear implantations in children. This article addresses some of the factors to be considered in a decision to bilaterally implant a child, including the age of the child at the first implant, the length of…

Bohnert, Andrea; Spitzlei, Vera; Lippert, Karl L.; Keilmann, Annerose

2006-01-01

344

Bilateral psoas and bilateral perinephric abscesses complicating acute pyelonephritis in pregnancy  

PubMed Central

Acute pyelonephritis complicates 1-2% of pregnancies and causes significant maternal and fetal morbidity and mortality. The diagnosis of renal tuberculosis (TB) is often delayed and commonly presents with sterile pyuria or along with other pyogenic organisms. We report a case where the diagnosis of renal TB was missed in a pregnant woman when she presented with acute pyelonephritis, septic shock, and acute renal failure. There was clinical recovery with antibiotics, but bilateral psoas and perinephric abscesses (TB, Enterococcus sp., and E. coli) were diagnosed when she presented with loin pain and palpable left renal angle swelling. Bilateral psoas abscess due to TB in the absence of skeletal TB and human immunodeficiency virus infection is rare. The presentation of renal TB in pregnancy, its complications, and its management are discussed. PMID:23580809

Veerappan, I.; Shanmugam, A.; Kumar, S.; Velayutham, P.

2013-01-01

345

Bilateral Tubal Gestation Associated with Schistosomiasis in an African Woman  

PubMed Central

Background. The incidence of tubal ectopic gestation caused by schistosomiasis induced tubal pathology is undocumented in this environment, which may be due to rarity of this pathology. Bilateral tubal gestation is common in patients that have undergone in vitro fertilization. We report a hitherto undocumented case of spontaneous bilateral ectopic gestation following tubal schistosomiasis. Case Report. Mrs. OB was a 32-year-old G4P3+0 (3 alive) woman who complained of abdominal pain and bleeding per vaginam of 4 and 2 days' duration respectively following 8 weeks of amenorrhea. A clinical impression of ruptured ectopic gestation was confirmed by ultrasound scanning. She had bilateral salpingectomy with histology of specimens showing bilateral ectopic gestation with Schistosoma haematobium induced salpingitis (findings of Schistosoma haematobium ova noted on slide). Conclusion. Schistosoma induced salpingitis is a rare but possible cause of bilateral tubal gestation. PMID:25580321

Odubamowo, K. H.; Akinpelu, O. M.; Lawal, O. O.; Okolo, C. A.; Odukogbe, A. A.; Adekunle, A. O.

2014-01-01

346

Misdiagnosis of bilateral tubal pregnancy: a case report  

PubMed Central

Introduction The incidence of bilateral tubal pregnancy is rising due to the increase of pelvic inflammatory disease and assisted reproductive techniques. Because the clinical manifestations of bilateral tubal pregnancy are not specific, we often ignore inspection of the other fallopian tube when focusing on the lesions, which may cause misdiagnosis. Case presentation A 33-year-old Chinese woman presented with vaginal bleeding after menopause and with an abnormality found by transvaginal ultrasound scan for which she underwent laparoscopy and salpingectomy. Unfortunately, she had to undergo a repetitive laparoscopic salpingotomy for the other tubal pregnancy due to misdiagnosis of her bilateral tubal pregnancy. Conclusions The incidence of unusual presentations of ectopic pregnancies has risen. Surgeons should always keep in mind the possibility of bilateral tubal pregnancy. An attentive examination of the pelvis, especially the two fallopian tubes, is necessary to avoid missing bilateral tubal pregnancy. PMID:25312677

2014-01-01

347

Intraocular Lens Subluxation in Marfan Syndrome  

PubMed Central

Purpose : Ectopia lentis (EL) is a major criteria for the diagnosis of Marfan syndrome, it may vary from an asymptomatic mild displacement to a significant subluxation that places the equator of the lens in the pupillary axis. The purpose of this work is to present the case of a patient with Marfan syndrome who received treatment for subluxation at our institution. Case Report : A 51-year-old female diagnosed with Marfan syndrome presented to the emergency department with bilateral eye redness, foreign body sensation and crusting around the eyes on awakening. She had the following history of cardiac and ophthalmologic complications, including: 1. Lens subluxation 2. High myopia 3. Aortic root dilation, 4. Mitral valve prolapse and 5. Tricuspid insufficiency. Conclusion : The ophthalmological management of Marfan patients is challenging and periodical follow-up is needed. Surgical versus conservative management is controversial, each case needs to be evaluated individually to analyze the risks and benefits of the procedures. PMID:25279020

Rodrigo, Bolaños-Jiménez; Paulina, López-Lizárraga E; Francesc, March de R; Eduardo, Telich-Tarriba J; Alejandro, Navas

2014-01-01

348

Unique phenotype in a patient with CHARGE syndrome.  

PubMed

CHARGE is a phenotypically heterogeneous autosomal dominant disorder recognized as a cohesive syndrome since the identification of CHD7 as a genetic etiology. Classic features include: Coloboma, Heart defects, Atresia choanae, Retarded growth and development, Genitourinary abnormalities, and Ear anomalies and/or deafness. With greater accessibility to genetic analysis, a wider spectrum of features are emerging, and overlap with disorders such as DiGeorge syndrome, Kallmann syndrome, and Hypoparathyroidism Sensorineural Deafness and Renal Disease syndrome, is increasingly evident. We present a patient with a unique manifestation of CHARGE syndrome, including primary hypoparathyroidism and a limb anomaly; to our knowledge, he is also the first CHARGE subject reported with bilateral multicystic dysplastic kidneys. Furthermore, with structural modeling and murine expression studies, we characterize a putative CHD7 G744S missense mutation. Our report continues to expand the CHARGE phenotype and highlights that stringent fulfillment of conventional criteria should not strictly guide genetic analysis. PMID:21995344

Jain, Shobhit; Kim, Hyung-Goo; Lacbawan, Felicitas; Meliciani, Irene; Wenzel, Wolfgang; Kurth, Ingo; Sharma, Josefina; Schoeneman, Morris; Ten, Svetlana; Layman, Lawrence C; Jacobson-Dickman, Elka

2011-01-01

349

Unique phenotype in a patient with CHARGE syndrome  

PubMed Central

CHARGE is a phenotypically heterogeneous autosomal dominant disorder recognized as a cohesive syndrome since the identification of CHD7 as a genetic etiology. Classic features include: Coloboma, Heart defects, Atresia choanae, Retarded growth and development, Genitourinary abnormalities, and Ear anomalies and/or deafness. With greater accessibility to genetic analysis, a wider spectrum of features are emerging, and overlap with disorders such as DiGeorge syndrome, Kallmann syndrome, and Hypoparathyroidism Sensorineural Deafness and Renal Disease syndrome, is increasingly evident. We present a patient with a unique manifestation of CHARGE syndrome, including primary hypoparathyroidism and a limb anomaly; to our knowledge, he is also the first CHARGE subject reported with bilateral multicystic dysplastic kidneys. Furthermore, with structural modeling and murine expression studies, we characterize a putative CHD7 G744S missense mutation. Our report continues to expand the CHARGE phenotype and highlights that stringent fulfillment of conventional criteria should not strictly guide genetic analysis. PMID:21995344

2011-01-01

350

Asperger's Syndrome  

Microsoft Academic Search

Asperger's Syndrome (AS), a Pervasive Developmental Disorder on the Autism Spectrum, is a burgeoning mental health concern faced by children, their families, schools, and mental health practitioners. Although it is a relatively new phenomenon, prevalence rates have increased 10 fold in the past decade. Whether this increase is a true increase in the prevalence of the syndrome has been the

Ernst O. Vanbergeijk; Oren Shtayermman

2005-01-01

351

Metabolic Syndrome  

MedlinePLUS

Having three or more risk factors is a sign that the body is resistant to insulin, an important hormone produced by the pancreas. ... diagnosed with the metabolic syndrome if you have three or more risk factors (see table below). FAcT SHeeT the metabolic syndrome ...

352

Angelman syndrome  

MedlinePLUS

... Genet. 2003;40(2):87-95. Dagli A, Williams CA. Angelman Syndrome. In: Pagon RA, Bird TD, Dolan CR, et ... Med Genet A. 2006;140(5):413-8. Williams CA, Driscoll DJ, Dagli AI. Clinical and genetic aspects of Angelman syndrome. Genet Med. 2010;12(7):385-95.

353

Eagle's syndrome.  

PubMed

Eagle's syndrome is a rare entity, which is not commonly suspected in clinical practice. The occurrence of similar signs in diseases other than Eagle's syndrome may make a precise diagnosis difficult and time-consuming for many clinicians. Radiological examinations are useful to make the accurate diagnosis. Three-dimensional volume-rendering CT scan is the most valuable diagnostic tool. PMID:24198961

Bouzaïdi, K; Daghfous, A; Fourati, E; Kechaou, I; Jabnoun, F; Chtioui, I

2013-01-01

354

Escobar Syndrome Mimicing Congenital Patellar Syndrome  

PubMed Central

Multiple pterygium syndrome (MPS) is a syndrome that is characterized abnormal face, short length and skin pterygiums on some body legions (servical, antecubital, popliteal, interdigital and on neck). It is also called as Pterygium Colli syndrome, Escobar syndrome or Pterygium syndrome. Escobar (multyple pterygium) syndrome is a rare syndrome. Intrauterin growth reterdation, abnormal face, wide-spead pterygiums that resulted in joint contractures, ptosis, chryptoorchidism, patellar dysplasia and foot deformities are seen on this syndrome. Primarly autosomal resesive crossing are observed; also autosomal dominant and X-linked crossing. This case were presented as it has components of Escobar syndrome and Isolated Patellar Aplasia syndrome in same time.

Ezirmik, Naci; Yildiz, Kadri; Can, Cahit Emre

2012-01-01

355

Periodic Syndromes  

Microsoft Academic Search

\\u000a \\u000a \\u000a \\u000a \\u000a  \\u000a \\u000a Hereditary periodic fever syndromes are autoinflammatory diseases characterized by episodes of fever with serosal, synovial,\\u000a and\\/or skin inflammation.\\u000a \\u000a \\u000a \\u000a \\u000a  \\u000a \\u000a Familial Mediterranean fever (FMF) and hyperimmunoglobulinemia D with periodic fever syndrome (HIDS) are inherited in an autosomal\\u000a recessive manner and tumor necrosis factor receptor-associated periodic syndrome (TRAPS), familial cold autoinflammatory syndrome\\u000a (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease

John G. Ryan; Daniel L. Kastner

356

Clinical effectiveness of unilateral deep brain stimulation in Tourette syndrome  

PubMed Central

Dysfunctional basal ganglia loops are thought to underlie the clinical picture of Tourette syndrome (TS). By altering dopaminergic activity in the affected neural structures, bilateral deep brain stimulation is assumed to have a modulatory effect on dopamine transmission resulting in an amelioration of tics. While the majority of published case reports deals with the application of bilateral stimulation, the present study aims at informing about the high effectiveness of unilateral stimulation of pallidal and nigral thalamic territories in TS. Potential implications and gains of the unilateral approach are discussed. PMID:22833207

Kuhn, J; Bartsch, C; Lenartz, D; Huys, D; Daumann, J; Woopen, C; Hunsche, S; Maarouf, M; Klosterkötter, J; Sturm, V

2011-01-01

357

Clinical effectiveness of unilateral deep brain stimulation in Tourette syndrome.  

PubMed

Dysfunctional basal ganglia loops are thought to underlie the clinical picture of Tourette syndrome (TS). By altering dopaminergic activity in the affected neural structures, bilateral deep brain stimulation is assumed to have a modulatory effect on dopamine transmission resulting in an amelioration of tics. While the majority of published case reports deals with the application of bilateral stimulation, the present study aims at informing about the high effectiveness of unilateral stimulation of pallidal and nigral thalamic territories in TS. Potential implications and gains of the unilateral approach are discussed. PMID:22833207

Kuhn, J; Bartsch, C; Lenartz, D; Huys, D; Daumann, J; Woopen, C; Hunsche, S; Maarouf, M; Klosterkötter, J; Sturm, V

2011-01-01

358

Connectivity-Based Parcellation of the Thalamus Explains Specific Cognitive and Behavioural Symptoms in Patients with Bilateral Thalamic Infarct  

PubMed Central

A novel approach based on diffusion tractography was used here to characterise the cortico-thalamic connectivity in two patients, both presenting with an isolated bilateral infarct in the thalamus, but exhibiting partially different cognitive and behavioural profiles. Both patients (G.P. and R.F.) had a pervasive deficit in episodic memory, but only one of them (R.F.) suffered also from a dysexecutive syndrome. Both patients had an MRI scan at 3T, including a T1-weighted volume. Their lesions were manually segmented. T1-volumes were normalised to standard space, and the same transformations were applied to the lesion masks. Nineteen healthy controls underwent a diffusion-tensor imaging (DTI) scan. Their DTI data were normalised to standard space and averaged. An atlas of Brodmann areas was used to parcellate the prefrontal cortex. Probabilistic tractography was used to assess the probability of connection between each voxel of the thalamus and a set of prefrontal areas. The resulting map of corticothalamic connections was superimposed onto the patients’ lesion masks, to assess whether the location of the thalamic lesions in R.F. (but not in G. P.) implied connections with prefrontal areas involved in dysexecutive syndromes. In G.P., the lesion fell within areas of the thalamus poorly connected with prefrontal areas, showing only a modest probability of connection with the anterior cingulate cortex (ACC). Conversely, R.F.’s lesion fell within thalamic areas extensively connected with the ACC bilaterally, with the right dorsolateral prefrontal cortex, and with the left supplementary motor area. Despite a similar, bilateral involvement of the thalamus, the use of connectivity-based segmentation clarified that R.F.’s lesions only were located within nuclei highly connected with the prefrontal cortical areas, thus explaining the patient’s frontal syndrome. This study confirms that DTI tractography is a useful tool to examine in vivo the effect of focal lesions on interconnectivity brain patterns. PMID:23755128

Serra, Laura; Cercignani, Mara; Carlesimo, Giovanni A.; Fadda, Lucia; Tini, Nadia; Giulietti, Giovanni; Caltagirone, Carlo; Bozzali, Marco

2013-01-01

359

Mutational analysis of steroidogenic factor 1 (NR5a1) in 24 boys with bilateral anorchia: a French collaborative study†  

PubMed Central

BACKGROUND Steroidogenic factor 1 (SF1/AdBP4/FTZF1, NR5A1) is a nuclear receptor transcription factor that plays a key role in regulating adrenal and gonadal development, steroidogenesis and reproduction. Recently, haploin-sufficiency of SF1 has been described in several 46,XY individuals with mild gonadal dysgenesis and impaired androgenization, but normal adrenal function, suggesting that dosage-sensitive or domain-specific effects of SF1 action are important in human testicular development and function. Our objective was to investigate whether partial defects in SF1 function might be associated with milder male reproductive phenotypes, such as bilateral anorchia (‘vanishing testis syndrome’) and micropenis. METHODS This study involved mutational analysis of NR5A1 in 24 individuals with bilateral anorchia and micropenis from the French Collaborative Anorchia study, as well as in vitro functional studies of SF1-dependent transcriptional activation and computer modeling. RESULTS A novel heterozygous missense mutation (V355M) in SF1 was found in one boy with a micropenis and testicular regression syndrome. This non-synonymous change was found to affect a highly conserved amino acid within helix 7 of the ligand-binding domain of SF1. This V355M mutation did not affect stability or nuclear localization, but did result in an ~50% reduction in SF1 activity in several different assay systems. CONCLUSIONS In conclusion, heterozygous partial loss of function mutations in SF1 may be associated with bilateral anorchia (‘vanishing testis syndrome’) and micropenis in humans. PMID:17940071

Philibert, Pascal; Zenaty, Delphine; Lin, Lin; Soskin, Sylvie; Audran, Françoise; Léger, Juliane; Achermann, John C.; Sultan, Charles

2010-01-01

360

Van Wyk and Grumbach syndrome: two case reports and review of the published work.  

PubMed

Van Wyk and Grumbach syndrome is characterized by a long-standing history of hypothyroidism, high levels of thyroid-stimulating hormone, uni- or bilateral ovarian cysts, isosexual precocity and a delayed bone age. All of the features can be reversed with treatment of the underlying hypothyroidism. Here, we describe two cases of Van Wyk and Grumbach syndrome presenting with bilateral multicystic ovaries and enlargement of the pituitary gland. One patient suffered from ovarian cyst and hypophysoma resection, the other patient promptly responded to L-thyroxine with complete regression of the ovarian cyst and other symptoms. PMID:24118179

Zhang, Huiying; Geng, Nv; Wang, Yingmei; Tian, Wenyan; Xue, Fengxia

2014-02-01

361

[Isolated bilateral Sternocostoclavicular Hyperostosis (SCCH) with a pathological clavicular fracture--a 10-year follow-up].  

PubMed

The presented case demonstrates the clinical and radiological course of a woman suffering from bilateral sternocostoclavicular hyperostosis (SCCH) from early changes to a spontaneous clavicula fracture after 10 years. SCCH is characterised by a chronic recurrent painful swelling of the sternoclavicular region due to an aseptic inflammation and hyperostosis of the clavicula, sternum, upper ribs and the adjacent soft tissues. Frequently the picture is accompanied by cutaneous and other skeletal symptoms. The disease can be part of the SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome. The chronic process is represented by the typical "bull horn sign" in a bone scan. NSAIDS should represent the first line treatment. PMID:16380903

Müller-Rath, R; Mumme, T; Zilkens, K W; Spüntrup, E

2005-01-01

362

Fetal Alcohol Syndrome  

MedlinePLUS

... Conditions Frequently Asked Questions Español Condiciones Chinese Conditions Fetal Alcohol Syndrome Read in Chinese What is Fetal Alcohol Syndrome (FAS)? Fetal Alcohol Syndrome (FAS) describes changes in ...

363

Does atherosclerosis contribute the development of Fahr’s Syndrome?  

PubMed Central

Fahr’s syndrome is a rare neurological condition in which calcification of vessels in the basal ganglia and cerebellum usually occurs. Impaired glucose tolerance (IGT) may be present, causing multiple organ complications by means of atherosclerosis. We report a case of Fahr’s syndrome associated with hypoparathyroidism and IGT. We present the case of a 52-year-old man with Fahr’s syndrome and IGT. The patient’s neurological examination was normal except for bilateral tremor. Laboratory investigations showed that the level of serum Ca (calcium) was 5.9 mg/dl, the parathormone (PTH) level was 1.95 pg/ml (normal level 15–65 pg/ml) and the phosphate (P) level was 5.45 mg/dl (normal level 2–6 mg/dl). Bilateral and symmetric calcifications in the basal ganglia, the centrum semiovale, and the cerebellum were demonstrated with a computerized scan of the head (CT) and magnetic resonance imaging (MRI). Therefore, the patient was diagnosed with Fahr’s syndrome. An endocrinologist was consulted, and treatment was started according to the suggestions of the endocrinologist. Here we report a case of Fahr’s syndrome associated with hypoparathyroidism and IGT. Atherosclerosis due to IGT may contribute to the calcification of brain vessels. Further investigations will be needed to clarify the pathogenesis of Fahr’s syndrome.

Varoglu, Asuman Orhan

2008-01-01

364

A new patient with LACHT syndrome (Mardini-Nyhan association).  

PubMed

LACHT syndrome, Lung Agenesis, Congenital Heart defects, and Thumb anomalies, (Mardini-Nyhan Association OMIM #601612) is a rare condition characterized by unilateral or bilateral lung agenesis, complex cardiac defects, especially anomalous pulmonary venous return, and thumb anomalies. Based on previous cases, its inheritance pattern seems to be autosomal recessive. In 1985, the syndrome was firstly described by Mardini and Nyhan in four patients from unrelated families. Until now, a total of eight patients have been reported in the literature. Molecular cause of the disease is still unknown. Here, we report on a patient with LACHT syndrome diagnosed by clinical findings. In this study, we present a 4.5-month-old female infant with right lung agenesis and inguinal hernia, in which ovaries are revealed on ultrasonography. The infant was born to consanguineous parents following a 38th week of gestation, with a birth weight of 2,800?g. Overall development was consistent with age; she had thumb abnormalities. Echocardiography showed peripheral pulmonary stenosis. The girl was diagnosed as LACHT syndrome based on the findings of unilateral lung agenesis, thumb anomalies, and peripheral pulmonary stenosis. LACHT syndrome should be considered in the differential diagnosis of patients with unilateral or bilateral lung agenesis. Here, we report on the 9th case in the literature. The consanguinity of the parents supports autosomal inheritance as the genetic basis of LACHT syndrome. © 2014 Wiley Periodicals, Inc. PMID:25487726

Atik, Tahir; Torun, Huseyin Ozan; Cogulu, Ozgur; Ozkinay, Ferda

2015-02-01

365

The role of bilateral adrenalectomy in the treatment of refractory Cushing's disease.  

PubMed

Cushing's syndrome (CS) results from sustained exposure to excessive levels of free glucocorticoids. One of the main causes of CS is excessive adrenocorticotropic hormone (ACTH) secretion by tumors in the pituitary gland (Cushing's disease [CD]). Cushing's disease and its associated hypercortisolism have a breadth of debilitating symptoms associated with an increased mortality rate, warranting urgent treatment. Currently, the first line of treatment for CD is transsphenoidal surgery (TSS), with excellent long-term results. Transsphenoidal resections performed by experienced surgeons have shown remission rates ranging from 70% to 90%. However, some patients do not achieve normalization of their hypercortisolemic state after TSS and continue to have persistent or recurrent CD. For these patients, various therapeutic options after failed TSS include repeat TSS, radiotherapy, medical therapy, and bilateral adrenalectomy (BLA). Bilateral adrenalectomy has been shown to be a safe and effective treatment modality for persistent or recurrent CD with an immediate and definitive cure of the hypercortisolemic state. BLA was traditionally performed through an open approach, but since the advent of laparoscopic adrenalectomy, the laparoscopic approach has become the surgical method of choice. Advances in technology, refinement in surgical skills, competency in adrenopathology, and emphasis on multidisciplinary collaborations have greatly reduced morbidity and mortality associated with adrenalectomy surgery in a high-risk patient population. In this article, the authors review the role of BLA in the treatment of refractory CD. The clinical indications, current surgical and endocrinological results reported in the literature, surgical technique (open vs laparoscopic), drawbacks, and complications of BLA are discussed. PMID:25639327

Wong, Anni; Eloy, Jean Anderson; Liu, James K

2015-02-01

366

Bilateral topographic symmetry patterns across Aphrodite Terra, Venus  

NASA Technical Reports Server (NTRS)

Topographic profiles have been obtained across Aphrodite Terra to test for bilateral symmetry of the type associated with thermal boundary layer topography at divergent plate boundaries on earth. In addition to a broad bilateral symmetry at a range of angles across Aphrodite Terra, detailed bilateral symmetry is noted within domains between linear discontinuities in directions parallel to the strike of the discontinuities. The results suggest that western Aphrodite Terra is similar to terrestrial oceanic divergent plate boundary environments, and that the cross-strike discontinuities are analogous to oceanic fracture zones rather than strike-slip faults.

Crumpler, L. S.; Head, James W.

1988-01-01

367

Bilateral Tibial Tubercle Sleeve Fractures in a Skeletally Immature Patient  

PubMed Central

Tibial tubercle sleeve fracture is a rare injury. In concept, it is similar to the patellar sleeve fracture in a skeletally immature patient. We describe a unique case of simultaneous bilateral tibial tubercle sleeve fractures in a 12-year-old boy. Radiographs and MRI confirmed the injury. The patient underwent open surgical repair of bilateral sleeve fractures with suture anchor fixation. At the final followup, 3 years after his initial injury, the patient demonstrated full knee function bilaterally without radiographic evidence of growth disturbances. PMID:23691395

Desai, Rasesh R.; Parikh, Shital N.

2013-01-01

368

[Simultaneous bilateral primary lymphomatous tumors of the testis].  

PubMed

Among the malignant tumours of the gonads, primary lymphoma of the testis represents a rare pathology. It is even more rare to observe this pathology in a bilateral localisation. After a careful evaluation of the cases described in the literature, the authors report a case or primary synchronous and bilateral lymphoma of the testis in a 68-year-old patient; they examine the clinical data and diagnostic methods and analyse the surgical treatment. In conclusion the authors affirm that primary synchronous bilateral lymphoma of the testis represents a rare form of tumour which is burdened by a high mortality rate. PMID:8322114

Vadalà, G; Basile, G; Altamore, S; Mauceri, M; Mangiameli, A; Roveccio, S; Bartoluccio, R

1993-03-01

369

Bilateral transient osteoporosis of the talus in pregnancy.  

PubMed

We present the case of bilateral transient osteoporosis of the talus in pregnancy, which is rarely reported in the medical literature, confirmed by pre- and postpartum MRI.A gravid 33-year-old white female G4P2A1 with bilateral transient osteoporosis of the talus confirmed by magnetic resonance imaging (MRI) is presented. She was conservatively managed and had clinical resolution several weeks postpartum. A follow-up MRI confirmed improvement in bilateral talus consistent with the natural course of transient osteoporosis. PMID:19343470

Daniel, R S; Farrar, E K; Norton, H R; Nussbaum, A I

2009-11-01

370

Reconstruction of bilateral tendoachilles with fascia lata graft.  

PubMed

A 19 year old male presented with progressive enlargement of both tendoachilles for 2 years and difficulty in walking for 3 months. The neurological history and examination revealed progressive mental deterioration and ataxia. The blood investigation revealed hypercholesterolemia. We report this rare case of cerebrotendinous xanthomatosis with bilateral tendoachilles enlargement, which was treated by excision of bilateral tendoachilles and reconstruction with fascia lata. The American Orthopedic Foot and Ankle Society hindfoot score was 93/100 bilaterally and the subjective evaluation of the patient showed very good results. PMID:24379473

Saxena, Vikas; Pradhan, Pavan; Yadav, Ashok; Nathani, Neeraj

2013-11-01

371

Case report: bilateral ankle pain in an aerobic dancer.  

PubMed

A 32-yr-old female presented to Sports Clinic with bilateral ankle pain and swelling following the initiation of an aerobic dance program 3 months earlier. Physical exam revealed bilateral ankle edema and the recent appearance of discrete tender nodules on her anterior tibias, consistent with erythema nodosum (EN). As part of her evaluation, chest radiographs were performed and demonstrated bilateral hilar adenopathy. This case discusses the evaluation of the patient with joint involvement, EN, and hilar adenopathy. The increased accessibility of sports medicine clinics makes it incumbent on the physician to be familiar with a wide range of differential diagnoses. PMID:1470012

Green, G A; Maltz, B A

1992-12-01

372

Fast bilateral filter with arbitrary range and domain kernels.  

PubMed

In this paper, we present a fast implementation of the bilateral filter with arbitrary range and domain kernels. It is based on the histogram-based fast bilateral filter approximation that uses uniform box as the domain kernel. Instead of using a single box kernel, multiple box kernels are used and optimally combined to approximate an arbitrary domain kernel. The method achieves better approximation of the bilateral filter compared to the single box kernel version with little increase in computational complexity. We also derive the optimal kernel size when a single box kernel is used. PMID:21411406

Gunturk, Bahadir K

2011-09-01

373

Olmsted Syndrome  

PubMed Central

Olmsted syndrome is a rare congenital, sharply circumscribed transgredient palmoplantar keratoderma. It was first described by Olmsted in 1927. The diagnosis of this rare disease depends on clinical features like symmetrical involvement of keratoderma of the palms and soles and the symmetrical hyperkeratotic plaques around the body orifices. It starts in the neonatal period or in childhood. The disease has a slow but progressive and extremely disabling course. Treatment of Olmsted syndrome is often based on topical therapy with retinoic acid, corticosteroid, emollients, and keratolytics. The present paper describes a case of Olmsted syndrome and its treatment. PMID:23320205

Elise Tonoli, Renata; De Villa, Damiê; Hübner Frainer, Renata; Pizzarro Meneghello, Luana; Ricachnevsky, Nelson; de Quadros, Maurício

2012-01-01

374

Catamenial pneumothorax due to bilateral pulmonary endometriosis.  

PubMed

Co-existence of catamenial pneumothorax and hemoptysis is rare. We present a case of catamenial pneumothorax due to bilateral pulmonary endometriosis in a 45-year-old woman. The patient presented with a 3-year history of intermittent productive cough with blood-tinged sputum, chronic anemia, loss of appetite, and general weakness associated with menstruation. Three years prior to this presentation the patient had undergone a sigmoidectomy as treatment for endometriosis of the sigmoid colon with bleeding. Chest radiographs and computed tomography (CT) scan revealed multiple nodules in both lung parenchyma and recurrent pneumothorax. CT-guided biopsy revealed chronic inflammation of those pulmonary nodules, and laboratory studies disclosed elevated serum levels of carbohydrate antigen 19-9 (CA 19-9) and CA 125. Thoracoscopic wedge resection of the pulmonary nodules was performed, and histopathological examination of the resected nodules revealed endometriosis. At one-year follow-up there was no evidence of recurrence of gastrointestinal bleeding or pneumothorax. PMID:22273501

Fang, Hsin-Yuan; Jan, Chia-Ing; Chen, Chien-Kuang; Chen, William Tzu-Liang

2012-07-01

375

Bilateral Chylhotorax after Falling from Height  

PubMed Central

Chylothorax is accumulation of chyle in the pleural cavity due to disruption of the thoracic duct. The causes can be classified as neoplastic, traumatic (iatrogenic or noniatrogenic), congenital, sporadic, spontaneous, and miscellaneous. A 22-year-old man with no feature in his history and family history was referred to emergency department with the case of falling from height. Abdominal computed tomogram (CT) revealed laceration of liver, grade 5 splenic laceration, fracture of the left acetabulum, and dislocation of the left hip. He was optimized for emergency splenectomy and close left hip reduction. On the 2nd day of the operation, bilateral chylotorax revealed. The treatment depends on its etiology, the amount of drainage, and the clinical picture. Treatment can be classified into 3 categories treatment of the underlying condition, conservative management (such as bed rest, nil by mouth or low fat medium chain triglycerides by mouth and total parenteral nutrition), and surgical management by ligation or clipping of the thoracic duct with open thoracotomy or video-assisted thoracoscopic surgery. The main purpose of surgical treatment is to stop the chylous leak. PMID:25089210

Akpinar, Vildan; Duran, Fulya Yilmaz; Duman, Elif; Özkalkanli, Murat Yasar; Duran, Özgür; Horsanali, Burcu

2014-01-01

376

From bilateral to multi-countries cooperation  

NASA Astrophysics Data System (ADS)

In 2010, the Office for Science and Technology of the French Embassy in Washington DC provided seed funding to enable a new scientific collaboration between the US and France. The work primarily focuses on the understanding of the interaction of particles with the DNA. The National Society of Black Physicists managed the funds. This so-called G4TRAP work is an extension of the ESA-funded G4DNA project that uses the Geant4 Monte Carlo toolkit and is spearheaded by the University of Bordeaux 1 in France. This initiative spawns to new areas (ultrafast lasers, nuclear/high energy physics, radiation biology and space science) and includes US industries, thanks to the interdisciplinary activities needed to advance this project. In addition, the bi-lateral collaboration was expanded to two other countries: Canada and Senegal. Subsequent funding requests were made to US agencies (NSF and DoE) as well as other French programs. More are anticipated that will also include NIH, NASA and the Canadian programs. A review of this multidisciplinary effort will be presented along with the present status on the various aspects of the G4TRAP project.

Gueye, Paul

2011-04-01

377

Fahr Syndrome Unknown Complication: Overactive Bladder  

PubMed Central

A 38-year-old male patient was admitted to our outpatient department because of frequency and urgency incontinence. During evaluation it was detected that the patient was suffering from frequency which was progressive for one year, feeling of incontinence, and urgency incontinence. There was no urologic pathology detected in patient's medical and family history. Neurologic consultation was requested due to his history of boredom, reluctance to do business, balance disorders, and recession for about 3 years. Brain computerized tomography (CT) scan revealed that amorphous calcifications were detected in the bilaterally centrum semiovale, basal ganglia, capsula interna, thalami, mesencephalon, pons and bulbus, and the bilateral cerebellar hemispheres. We have detected spontaneous neurogenic detrusor overactivity without sphincter dyssynergia after evaluating the voiding diary, cystometry, and pressure flow study. We consider the detrusor overactivity which occurred one year after the start of the neurological symptoms as the suprapontine inhibition and damage in the axonal pathways in the Fahr syndrome. PMID:25140274

Tuglu, Devrim; Yuvanç, Ercan; Bal, Fatih; Türkel, Yakup; Da?, Ersel; Y?lmaz, Erdal; Batislam, Ertan

2014-01-01

378

Eagle's syndrome: a case report  

PubMed Central

Eagle's syndrome is a disease caused by an elongated styloid process or calcified stylohyoid ligament. Eagle defined the disorder in 1937 by describing clinical findings related to an elongated styloid process, which is one of the numerous causes of pain in the craniofacial and cervical region. The prevalence of individuals with this anatomic abnormality in the adult population is estimated to be 4% with 0.16% of these individuals reported to be symptomatic. Eagle's syndrome is usually characterized by neck, throat, or ear pain; pharyngeal foreign body sensation; dysphagia; pain upon head movement; and headache. The diagnosis of Eagle's syndrome must be made in association with data from the clinical history, physical examination, and imaging studies. Patients with increased symptom severity require surgical excision of the styloid process, which can be performed through an intraoral or an extraoral approach. Here, we report a rare case of stylohyoid ligament bilaterally elongated to more than 60 mm in a 51-year-old female. We did a surgery by extraoral approach and patient's symptom was improved. PMID:24627843

Moon, Chang-Sig; Lee, Baek-Soo; Kwon, Yong-Dae; Choi, Byung-Jun; Lee, Jung-Woo; Lee, Hyun-Woo; Yun, Sun-Ung

2014-01-01

379

Eagle's syndrome: a case report.  

PubMed

Eagle's syndrome is a disease caused by an elongated styloid process or calcified stylohyoid ligament. Eagle defined the disorder in 1937 by describing clinical findings related to an elongated styloid process, which is one of the numerous causes of pain in the craniofacial and cervical region. The prevalence of individuals with this anatomic abnormality in the adult population is estimated to be 4% with 0.16% of these individuals reported to be symptomatic. Eagle's syndrome is usually characterized by neck, throat, or ear pain; pharyngeal foreign body sensation; dysphagia; pain upon head movement; and headache. The diagnosis of Eagle's syndrome must be made in association with data from the clinical history, physical examination, and imaging studies. Patients with increased symptom severity require surgical excision of the styloid process, which can be performed through an intraoral or an extraoral approach. Here, we report a rare case of stylohyoid ligament bilaterally elongated to more than 60 mm in a 51-year-old female. We did a surgery by extraoral approach and patient's symptom was improved. PMID:24627843

Moon, Chang-Sig; Lee, Baek-Soo; Kwon, Yong-Dae; Choi, Byung-Jun; Lee, Jung-Woo; Lee, Hyun-Woo; Yun, Sun-Ung; Ohe, Joo-Young

2014-02-01

380

Apert syndrome  

MedlinePLUS

... by ridging along sutures (craniosynostosis) Frequent ear infections Fusion or severe webbing of the 2nd, 3rd, and ... face Skeletal (limb) abnormalities Short height Webbing or fusion of the toes Several other syndromes can lead ...

381

Wellens' syndrome.  

PubMed

A 31-year-old male smoker with diabetes mellitus presented with intermittent, exertional chest pain. Electrocardiography revealed anterior T-wave inversions with biphasic lateral T waves, which raised concern about Wellens' syndrome. PMID:25551527

Kannan, Lakshmi; Figueredo, Vincent M

2015-01-01

382

Troyer Syndrome  

MedlinePLUS

... Funding Information Research Programs Training & Career Awards Enhancing Diversity Find People About NINDS NINDS Troyer Syndrome Information ... News From NINDS | Find People | Training | Research | Enhancing Diversity Careers@NINDS | FOIA | Accessibility Policy | Contact Us | Privacy ...

383

Marfan Syndrome  

MedlinePLUS

Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, blood vessels, and other organs. One of these proteins is fibrillin. A problem with the ...

384

Marfan syndrome  

MedlinePLUS

Marfan syndrome is a disorder of connective tissue. This is the tissue that strengthens the body's structures. Disorders of connective tissue affect the skeletal system, cardiovascular system, eyes, and skin.

385

Alagille Syndrome  

MedlinePLUS

... person with an enlarged spleen should avoid contact sports to protect the organ from injury. Advanced portal ... for people with Alagille syndrome depends on several factors, including the severity of liver damage and heart ...

386

Moebius Syndrome  

MedlinePLUS

... syndrome: Group I, characterized by small or absent brain stem nuclei that control the cranial nerves; Group II, ... areas of damage, and hardened tissue in the brainstem nuclei, and, Group IV, characterized by muscular symptoms ...

387

Alport Syndrome  

MedlinePLUS

... older the risk of kidney failure increases. All boys and girls with the autosomal recessive type of Alport Syndrome ... Register Now KEEP Healthy Event at Terre Haute Boys and Girls Club Mar 01, 2015 KEEP Healthy Event at ...

388

Reye syndrome  

MedlinePLUS

... may be used to diagnose Reye syndrome: Blood chemistry tests Head CT or head MRI scan Liver ... RM, Behrman RE,Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics . 19th ed. Philadelphia ,Pa: Saunders ...

389

Asperger syndrome  

MedlinePLUS

... is often considered a high functioning form of autism. It can lead to difficulty interacting socially, repeat ... syndrome is a pervasive developmental disorder (PDD) or autism spectrum disorder (ASD). The main difference between Asperger ...

390

Piriformis Syndrome  

MedlinePLUS

... and lifestyle changes. Failure to treat this condition can lead to permanent nerve damage, so be sure to follow your doctor’s instructions. Self-care tips for piriformis syndrome include the following: ...

391

Aortoduodenal syndrome.  

PubMed

Duodenal obstruction caused by abdominal aortic aneurysm (AAA), i.e. "aortoduodenal syndrome," first described by Osler in 1905 is a rare clinical entity, with only several dozens of cases reported in the literature. In the present paper, we systematically searched literature and reviewed them. Databases including MEDLINE and EMBASE were searched from January 1981 to April 2014 using Web--based search engines (PubMed and OVID). Eighteen papers reported 21 cases with aortoduodenal syndrome. Mean age of patients was 74.5±8.4 years, 71.4% of patients were men, and mean AAA diameter was 7.2±2.1 cm. We also discussed confusion between aortoduodenal syndrome and "superior mesenteric artery syndrome" associated with AAA. PMID:25216355

Takagi, H; Watanabe, T; Umemoto, T

2014-09-12

392

Cushing's Syndrome  

MedlinePLUS

... Common symptoms of Cushing's syndrome include upper body obesity, severe fatigue and muscle weakness, high blood pressure, backache, elevated blood sugar, easy bruising, and bluish-red stretch marks on ...

393

Cushing syndrome  

MedlinePLUS

... Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology. 12th ed. Philadelphia, Pa: Elsevier Saunders; 2011:chap 15. Susmeeta TS, Nieman LK. Cushing's syndrome: all variants, detection, and treatment. Endocrinol Metab Clin ...

394

Bartter syndrome  

MedlinePLUS

... syndrome include: High levels of potassium , calcium, and chloride in the urine High levels of the hormones renin and aldosterone in the blood Low blood chloride Metabolic alkalosis These same signs and symptoms can ...

395

Aicardi syndrome  

MedlinePLUS

... the two sides of the brain (called the corpus callosum) is partly or completely missing. Nearly all ... Aicardi syndrome if they meet the following criteria: Corpus callosum that is partly or completely missing Female ...

396

Marfan Syndrome  

MedlinePLUS

... Loeys-Dietz is more likely to cause fatal aortic aneurysms, and treatment for the two is different. A ... shown that the blood pressure medication losartan prevents aortic aneurysms in a mouse model of Marfan syndrome. Studies ...

397

Metabolic Syndrome  

MedlinePLUS

... to manage your condition. Medications can also treat risk factors such as high blood pressure or high blood glucose. If you think you have risk factors for the metabolic syndrome, talk with your doctor. ...

398

Gardner Syndrome  

MedlinePLUS

... syndrome also have a higher risk of developing colorectal cancer and other FAP-related cancers . Other features of ... person with multiple adenomatous colon polyps and/or colorectal cancer along with some of the benign tumors listed ...

399

[Ogilvie's syndrome].  

PubMed

"Ogilvie's syndrome" or the idiopathic dilatation of the colon is an infrequent pathology whose underlying physiopathology is not yet well known. On the basis of their experience and having reviewed the literature, the authors affirm that this syndrome is caused by the inhibition of gastrointestinal hormones which, under the control of the neurohypophysis, contribute to colon motility. This supposition is backed up by the fact that medical treatment with somatostatin or octreotide leads to the resolution of the disorder. PMID:9676186

Vadalà, G; Santonocito, G; Mangiameli, A; Castorina, R; Caragliano, L; Caragliano, P

1998-05-01

400

Cardiometabolic Syndrome  

Microsoft Academic Search

\\u000a Cardiometabolic syndrome is a common condition that is increasing in prevalence in the USA and developing nations. Epidemiological\\u000a studies indicate a strong association between cardiometabolic syndrome and subsequent risks for diabetes and cardiovascular\\u000a events. Accumulating evidence suggests it may also be a risk factor for incident chronic kidney disease (CKD) and cardiovascular\\u000a events in individuals with pre-existing CKD. In studies

Manjula Kurella Tamura; Tara I. Chang

401

Turner Syndrome  

Microsoft Academic Search

\\u000a Turner syndrome (TS) or monosomy X, is the most common cause of hypergonadotropic hypogonadism in girls and young women. This\\u000a chapter reviews the prevalence and different presentations of the syndrome and explains its chromosomal origins. The interpretation\\u000a of chromosomal studies in diagnosis of and prognosis for TS, including prenatal testing, is reviewed. The most recent data\\u000a on the TS phenotypic

Carolyn A. Bondy

402

[Edwards' syndrome].  

PubMed

Two cases of Edwards' syndrome were observed. One occurred in a girl aged 6 months, the other in a male neonate. The two children showed a highly characteristic appearance of both the face and the extremities, so that it arouse suspicion of 18-chromosomy. The latter was confirmed by a postmortem cytogenetic examination. The syndrome also includes malformations of inner organs and microscopical architectural changes of organs, which occurred in both the cases described, but different in type. PMID:455513

Zuntová, A; Goetz, P; Macek, M; Karpenko, A

1979-05-01

403

Noonan syndrome  

PubMed Central

Noonan syndrome is a genetic multisystem disorder characterised by distinctive facial features, developmental delay, learning difficulties, short stature, congenital heart disease, renal anomalies, lymphatic malformations, and bleeding difficulties. Mutations that cause Noonan syndrome alter genes encoding proteins with roles in the RAS–MAPK pathway, leading to pathway dysregulation. Management guidelines have been developed. Several clinically relevant genotype–phenotype correlations aid risk assessment and patient management. Increased understanding of the pathophysiology of the disease could help development of pharmacogenetic treatments. PMID:23312968

Roberts, Amy E; Allanson, Judith E; Tartaglia, Marco; Gelb, Bruce D

2014-01-01

404

Right Calf Claudication Revealing Leriche Syndrome Presenting as Right Sciatic Neuropathy  

PubMed Central

The syndrome of aortoiliac occlusive disease, also known as Leriche syndrome, is characterized by claudication, pain, and diminished femoral pulse. We highlight an unusual case of right sciatic neuropathy caused by Leriche syndrome, which was initially misdiagnosed. A 52-year-old male, with a past medical history of hypertension and bony fusion of the thoracolumbar spine, visited our hospital complaining of right leg pain and claudication, and was initially diagnosed with spinal stenosis. The following electrophysiologic findings showed right sciatic neuropathy; but his symptom was not relieved, despite medications for neuropathy. A computed tomography angiography of the lower extremities revealed the occlusion of the infrarenal abdominal aorta, and bilateral common iliac and right external iliac arteries. All these findings suggested omitted sciatic neuropathy associated with Leriche syndrome, and the patient underwent a bilateral axillo-femoral and femoro-femoral bypass graft. PMID:24639938

Yoon, Do Hyun; Cho, Hyungpil; Seol, Seung Jun

2014-01-01

405

ASSOCIATIONS BETWEEN FAMILY HISTORY OF CARPAL TUNNEL SYNDROME AND ABNORMAL MEDIAN NERVE CONDUCTION WITH AND WITHOUT SYMPTOMS OF CARPAL TUNNEL SYNDROME  

Microsoft Academic Search

Background: Few studies have examined the relationship between family history and carpal tunnel syndrome (CTS) on a population basis. Methods: Workers (N=841) completed a questionnaire, structured interview, two standardized physical examinations, and a bilateral nerve conduction study (NCS). Self-reported family history of CTS was recorded from the questionnaire. Results: 37.0 % of subjects with CTS by a case definition (abnormal

Hannah Edwards; Kurt T. Hegmann

406

Interactions between bilaterally paired components of the cockroach circadian system  

Microsoft Academic Search

The hypothesis that bilaterally redundant, and mutually coupled circadian pacemakers are located near the lobula of the optic lobes of the cockroach,Leucophaea maderae, was investigated in a series of lesion experiments.

Terry L. Page

1978-01-01

407

Bilateral Central Retinal Artery Occlusion in Behçet Disease.  

PubMed

Abstract Purpose: To report a case of bilateral central retinal artery occlusion as the primary ocular manifestation of Behçet disease. Methods: A 38-year-old patient, with no history of previous ocular complaints, admitted with bilateral sudden vision loss. His ophthalmologic examination revealed a visual acuity of no light perception in both eyes and the diagnosis of bilateral central retinal artery occlusion. A detailed systemic investigation was conducted. Results: With the history of recurrent oral and genital ulcers, and the presence of ocular and skin findings, the diagnosis of Behçet disease was made. Despite a potent immunosuppressive therapy the patient became absolutely blind. Conclusions: Even though retinal arteries are rarely affected in Behçet disease, in the cases of retinal artery occlusion, it should be considered among the differential diagnosis. To our knowledge this is the first reported case of bilateral central retinal artery occlusion as the primary ocular manifestation of Behçet disease. PMID:24654996

Esen, Ebru; Sizmaz, Selcuk; Sariyeva, Ayna; Demircan, Nihal

2014-03-21

408

Genetics Home Reference: Congenital bilateral absence of the vas deferens  

MedlinePLUS

... progressive damage to the respiratory system and chronic digestive system problems. Many men with congenital bilateral absence of ... lubricates and protects the linings of the airways, digestive system, reproductive system, and other organs and tissues. Mutations ...

409

Early Deprivation Impairs the Development of Balance and Bilateral Coordination  

PubMed Central

This study examined balance and bilateral coordination skills in a sample of internationally adopted, post-institutionalized (PI) children. We compared the performance of these PI children to two age-matched groups. One was a group of children who were internationally adopted from foster care (FC). The second group consisted of non-adopted children being raised in their birth families, who served as controls (Control). Both PI and FC children scored lower than control children on balance, while PI children scored lower than both FC and control children on bilateral coordination. These results suggest that aspects of institutional rearing impact the development of bilateral coordination, while factors common to internationally adopted children other than institutionalization impact the development of balance. Region of birth (Asia, Latin/South America, Russia/Eastern Europe) did not moderate associations between institutional duration and bilateral coordination. PMID:24014461

Roeber, Barbara J.; Gunnar, Megan R.; Pollak, Seth D.

2014-01-01

410

Cooled Radiofrequency Ablation for Bilateral Greater Occipital Neuralgia  

PubMed Central

This report describes a case of bilateral greater occipital neuralgia treated with cooled radiofrequency ablation. The case is considered in relation to a review of greater occipital neuralgia, continuous thermal and pulsed radiofrequency ablation, and current medical literature on cooled radiofrequency ablation. In this case, a 35-year-old female with a 2.5-year history of chronic suboccipital bilateral headaches, described as constant, burning, and pulsating pain that started at the suboccipital region and radiated into her vertex. She was diagnosed with bilateral greater occipital neuralgia. She underwent cooled radiofrequency ablation of bilateral greater occipital nerves with minimal side effects and 75% pain reduction. Cooled radiofrequency ablation of the greater occipital nerve in challenging cases is an alternative to pulsed and continuous RFA to alleviate pain with less side effects and potential for long-term efficacy. PMID:24716017

Chhatre, Akhil

2014-01-01

411

Central Pain Syndrome  

MedlinePLUS

NINDS Central Pain Syndrome Information Page Table of Contents (click to jump to sections) What is Central Pain Syndrome? Is there ... being done? Clinical Trials Organizations What is Central Pain Syndrome? Central pain syndrome is a neurological condition ...

412

Tics and Tourette Syndrome  

MedlinePLUS

MENU Return to Web version Tics and Tourette Syndrome Overview What is Tourette syndrome? Tourette syndrome is a type of tic disorder. Children who have Tourette syndrome will repeat both movements ...

413

Androgen insensitivity syndrome  

MedlinePLUS

... of the penis Reifenstein syndrome (also known as Gilbert-Dreyfus syndrome or Lubs syndrome) Infertile male syndrome ... HM, Schlomo M, Polansky KS, Larsen PR, eds. Williams Textbook of Endocrinology . 11th ed. St. Louis, Mo: ...

414

Hantavirus Pulmonary Syndrome  

MedlinePLUS

... for ENews Home > Lung Disease > Hantavirus Pulmonary Syndrome Hantavirus Pulmonary Syndrome Hantavirus pulmonary syndrome (HPS) is a disease that comes ... may improve a person's chances of recovery. Understanding Hantavirus Pulmonary Syndrome Symptoms, Diagnosis and Treatment Preventing Hantavirus ...

415

Bilateral thecoma presenting as premenopausal hirsutism: Laproscopic removal  

PubMed Central

Hyperandrogenism is a common disorder among women in the reproductive age group. One of the rare causes for androgen excess is sex cord- stromal tumors of the ovary. These are usually unilateral. Here we report case of a 48 year old woman who presented with hyperandrogenism due to bilateral ovarian thecoma. Androgen levels normalized following resection of the tumor. This, to the best of our knowledge, is the first case of bilateral thecoma presenting as hirsutism in a premenopausal woman. PMID:24910832

Ramkumar, S.; Jyotsna, V. P.; Mallick, S.; Kachhawa, Garima; Kandasamy, D.; Kriplani, A.; Ammini, A. C.

2013-01-01

416

Bilateral thoracoscopic thymectomy using a novel positioning system.  

PubMed

Several techniques of bilateral video-assisted thoracoscopic extended thymectomy have been proposed, and each has its own proponents. We summarize our experience in 20 patients who underwent bilateral video-assisted thoracoscopic extended thymectomy, using a new patient positioning that amplifies the thoracoscopic view of the cardiophrenic regions which are often difficult to visualize with standard techniques. In all cases, en-bloc thymectomy with complete dissection of the mediastinal fatty tissue was achieved without sternal retractors or additional incisions. PMID:24887902

Caronia, Francesco; Fiorelli, Alfonso; Monte, Attilio Lo

2014-11-01

417

Recurrent Bilateral Breast Abscess Due to Nontuberculous Mycobacterial Infection  

PubMed Central

Since recurrent bilateral breast infection due to nontuberculous mycobacterium (NTM) is rare, its diagnosis is easily overlooked; in addition, complete recovery is often difficult to achieve. We report a case of recurrent bilateral infection in a 35-year-old woman who had completed treatment for NTM. Although various infectious diseases show similar clinical conditions and imaging findings, recurrences should raise suspicion of NTM infection, and this possibility should be considered in differential diagnoses. PMID:25320630

Yoo, Hyunkyung; Choi, Sang Hyun; Kim, Sei Joong; Cho, Young Up; Choi, Suk Jin

2014-01-01

418

Application of bilateral slotlines to broadband microwave components  

Microsoft Academic Search

Novel design of doubly-balanced mixers\\/modulators and even order frequency multipliers employing broadband microstrip-balanced stripline-bilateral slotline-unilateral slotline-microstrip baluns is proposed. Circuit concept is presented first, followed with the design and evaluation of balun components which involved a transverse resonance approach to the analysis of asymmetrical bilateral slotlines. Examples of application are: an X-Ku band mixer, a Ku-K band frequency doubler and

J. Chramiec; H. Aubert; H. Baudrand

1993-01-01

419

[Bilateral periventricular nodular heterotopia in a couple mother-infant].  

PubMed

Bilateral periventricular nodular heterotopia in a couple mother-infant. Bilateral periventricular nodular heterotopia (BPNH) is a malformation of neuronal migration, recently recognized X-linked. We report a case of BPNH associated with mega-cisterna magna diagnosed by ultrasound imaging at 27 weeks' gestation and confirmed by MR at 11 days of life. Similar findings in the mother's MR underlines the recurrency of this malformation in the same family. PMID:16922057

Stival, G; Braga, M; Di Marco, A; Riva, E

2005-01-01

420

Bilateral adrenal non-Hodgkin's lymphoma with adrenal insufficiency  

PubMed Central

A 74 year old women presented with lethargy and weight loss and was found to have profound adrenal insufficiency and bilateral adrenal mass lesions. Histological examination revealed non-Hodgkin's lymphoma. There was no evidence of lymphoma outside the adrenal glands. Isolated bilateral adrenal masses may rarely be due to primary adrenal non-Hodgkin's lymphoma, which is often associated with adrenal insufficiency.???Keywords: lymphoma; adrenal insufficiency PMID:10908383

Ellis, R; Read, D

2000-01-01

421

Bilateral chondrodermatitis helicis: case presentation and literature review.  

PubMed

Chondrodermatitis helicis is a benign, painful lesion of the auricle affecting the elderly population, particularly men. These lesions usually affect the helix of the ear, although the antihelix and, less commonly, the antitragus may be involved. The lesions are most frequently unilateral, although bilateral involvement has been reported. An unusual case of bilateral involvement of the antitragus is presented. The etiologic and pathologic manifestations are reviewed and current treatment modalities outlined. PMID:3985132

Cannon, C R

1985-03-01

422

Churg-Strauss syndrome presented as paraneoplastic syndrome with thymic neuroendocrine carcinoma: a case report.  

PubMed

Churg-Strauss syndrome (CSS) is rarely presented as paraneoplastic syndrome. We reported here a 47-year-old man with refractory asthma for 3 years, and his CT scan showed an anterior mediastinal mass, pulmonary reticular opacities and bilateral maxillary sinusitis. He also presented with elevated peripheral blood eosinophils (EOS). Thoracoscopic resection of the mediastinal mass and a lung biopsy were performed, and thymic neuroendocrine carcinoma (TNC) and pulmonary eosinophilic vasculitis were pathologically diagnosed. The laboratory and clinical findings fulfilled the criteria of the diagnosis of CSS (Masi et al. in Arthritis Rheum 33(8):1094-1100, 1990). Asthma was improved, and elevated EOS was recovered to normal range after surgery and four courses of chemotherapy. This was the first report of CSS manifested as a paraneoplastic syndrome of TNC. PMID:21901349

Liu, Tao; Cai, Baiqiang; Feng, Ruie

2012-11-01

423

Synchronous bilateral neuroendocrine ductal carcinoma in situ.  

PubMed

Neuroendocrine ductal carcinoma in situ (NE-DCIS) is a breast malignancy that has characteristic clinicopathological features and can, therefore, be regarded as a distinct variant of DCIS. The patient was a 54-year-old premenopausal woman with hemorrhagic nipple discharge in her left breast. Magnetic resonance imaging and ultrasound (US) images of the left breast showed mass-like lesions, while concurrent images of the right breast showed non-mass-like lesions. These findings suggested the presence of both benign and malignant tumors. Pathological findings from US-guided core-needle biopsy of the left mass were highly suspicious of a malignant tumor. Excisional biopsy of both breasts was performed. We could define the diagnosis of breast cancer by the second opinion on pathological diagnosis. The tumor cells showed histological characteristics of NE-DCIS. Bilateral breast lesions had histopathological similarities and were composed of predominantly solid growth of carcinoma cells, frequently with well-developed vascular structures, in mammary ducts and ductules. Carcinoma cells were polygonal or occasionally spindle shaped and had fine-granular, relatively eosinophilic cytoplasm. The nuclei of these cells showed round to ovoid in shape and fine-granular chromatin pattern. There was not any invasive component, as confirmed by careful histological examination. Thus, additional immunohistochemical stainings for NE markers (chromogranin A and synaptophysin) were performed. Staining statuses of these markers were positive in almost all tumor cells from both breasts. Both tumors were therefore diagnosed as NE-DCIS. To our knowledge, this case is the first report of NE-DCIS diagnosed synchronously in both breasts. PMID:21735237

Honami, Hisae; Sotome, Keiichi; Sakamoto, Goi; Iri, Hisami; Tanaka, Yoichi; Fukamachi, Shigeru; Morozumi, Kyoei

2014-07-01

424

Bilateral Traumatic Globe Luxation with Optic Nerve Transection  

PubMed Central

Purpose The purpose of this study was to document clinical findings and management of a patient with bilateral globe luxation and optic nerve transection. Materials and Methods A 25-year-old female patient was admitted to the emergency department with bilateral traumatic globe luxation following a motor vehicle accident. Results Visual acuity testing showed no light perception. The right pupil was dilated and bilaterally did not react to light. The globes were bilaterally intact. A computed tomography scan revealed Le Fort type II fractures, bilateral optic nerve transection and disruption of all extraocular muscles. The globes of the patient were bilaterally reduced into the orbit. However, the patient developed phthisis bulbi in the right eye at month 3. Conclusion Globe luxation presents a dramatic clinical picture, and may lead to the development of severe complications due to the concomitance of complete optic nerve dissection and multiple traumas. Even if the luxated globe is repositioned into the orbit, there is still an increased risk of the development of phthisis due to ischemia. PMID:25606034

Tok, Levent; Tok, Ozlem Yalcin; Argun, Tugba Cakmak; Yilmaz, Omer; Gunes, Alime; Unlu, Elif Nisa; Sezer, Sezgin; Ibisoglu, Seda; Argun, Mehmet

2014-01-01

425

Goldenhar syndrome with right circumflex aortic arch, severe coarctation and vascular ring in a twin pregnancy  

PubMed Central

Goldenhar syndrome (GS) or oculo-auriculo-vertebral dysplasia (OAVD), involves a wide variety of organ systems. Cardiovascular anomalies are among the frequent malformations. The purpose of this report is to introduce a male case of a dizygotic twin pregnancy with GS and right circumflex aortic arch (RCAA), severe coarctation, hypoplastic aortic arch, aberrant right subclavian artery, vascular ring, bilateral renal artery stenosis, and mild Dandy-Walker syndrome. The embryology of RCAA and coarctation is revisited. PMID:25298700

Rad, Elaheh Malakan

2014-01-01

426

Seizure, deafness and renal agenesis: A rare case of barakat syndrome  

PubMed Central

Etiologic diagnosis of seizure requires proper consideration of apparently unrelated clinical features of the patient. Here, we report the case of a patient of status epilepticus with moderate-to-severe bilateral sensorineural deafness. Investigations showed extensive intracranial calcification, hypoparathyroidism and unilateral renal agenesis. The features were consistent with Barakat syndrome, a rare developmental disorder associated with mutations in the GATA3 gene. To the best of our knowledge, this is the first reported case of Barakat syndrome from India. PMID:23661972

Sau, Tanmayjyoti; Chatterjee, Atri; Ghosh, Kaushik; Dey, Sandip

2013-01-01

427

The red ear syndrome  

PubMed Central

Red Ear Syndrome (RES) is a very rare disorder, with approximately 100 published cases in the medical literature. Red ear (RE) episodes are characterised by unilateral or bilateral attacks of paroxysmal burning sensations and reddening of the external ear. The duration of these episodes ranges from a few seconds to several hours. The attacks occur with a frequency ranging from several a day to a few per year. Episodes can occur spontaneously or be triggered, most frequently by rubbing or touching the ear, heat or cold, chewing, brushing of the hair, neck movements or exertion. Early-onset idiopathic RES seems to be associated with migraine, whereas late-onset idiopathic forms have been reported in association with trigeminal autonomic cephalalgias (TACs). Secondary forms of RES occur with upper cervical spine disorders or temporo-mandibular joint dysfunction. RES is regarded refractory to medical treatments, although some migraine preventative treatments have shown moderate benefit mainly in patients with migraine-related attacks. The pathophysiology of RES is still unclear but several hypotheses involving peripheral or central nervous system mechanisms have been proposed. PMID:24093332

2013-01-01

428

Marfan syndrome masked by Down syndrome?  

Microsoft Academic Search

Down syndrome is the most common chromosomal abnormality. A simultaneous occurrence with Marfan syndrome is extremely rare.\\u000a We present a case of a 28-year-old female with Down syndrome and a mutation in the fibrillin-1 gene. The patient showed strikingly\\u000a few manifestations of Marfan syndrome. Although variable expression is known to be present in Marfan syndrome, phenotypic\\u000a expression of Marfan syndrome

J. C. Vis; K. van Engelen; J. Timmermans; B. C. J. Hamel; B. J. M. Mulder

2009-01-01

429

Adult presentation of Stickler syndrome type III.  

PubMed

Few clinical cases have been published on Stickler syndrome type III, and all describe the pediatric presentation. We describe an adult presentation of the syndrome in a 67-year-old woman and provide a report on the clinical and radiographic features supporting diagnosis. A chart review and updated investigations were performed to elucidate the presenting history and disease progression in the patient. Clinically, the patient was 42 years old when she presented with a 22-year history of bilateral knee pain and atypical osteoarthritis (OA) of an inflammatory but non-erosive character. The patient also reported increasing hearing loss for high-pitched frequencies. Radiography/X-rays at age 42 years showed severe, non-erosive tri-compartmental OA changes. Atypical destructive changes were identified in the small joints. The son of the patient developed left knee pain and bilateral knee swelling since the age of 9 years. He was diagnosed with early-onset OA and also underwent bilateral knee arthroplasties in his third decade, similar to his mother. The grandson presented at age 4 years, with recurrent, acute episodes of pain and swelling in his ankles and knees and was diagnosed with pediatric OA. A mutation of the COL11A2 gene was confirmed in the patient at aged 63 years. This is the first clinical case report on the adult presentation of Stickler syndrome type III. In particular, early-onset OA or an unrecognized skeletal dysplasia can be considered as rationale for genetic testing, screening, and surveillance of both past and present family members of an affected patient. PMID:20112039

Li, Kayi; Thorne, Carter

2010-07-01

430

EphA4-Mediated Ipsilateral Corticospinal Tract Misprojections Are Necessary for Bilateral Voluntary Movements But Not Bilateral Stereotypic Locomotion  

PubMed Central

In this study, we took advantage of the reported role of EphA4 in determining the contralateral spinal projection of the corticospinal tract (CST) to investigate the effects of ipsilateral misprojections on voluntary movements and stereotypic locomotion. Null EphA4 mutations produce robust ipsilateral CST misprojections, resulting in bilateral corticospinal tracts. We hypothesize that a unilateral voluntary limb movement, not a stereotypic locomotor movement, will become a bilateral movement in EphA4 knock-out mice with a bilateral CST. However, in EphA4 full knock-outs, spinal interneurons also develop bilateral misprojections. Aberrant bilateral spinal circuits could thus transform unilateral corticospinal control signals into bilateral movements. We therefore studied mice with conditional forebrain deletion of the EphA4 gene under control by Emx1, a gene expressed in the forebrain that affects the developing CST but spares brainstem motor pathways and spinal motor circuits. We examined two conditional knock-outs targeting forebrain EphA4 during performance of stereotypic locomotion and voluntary movement: adaptive locomotion over obstacles and exploratory reaching. We found that the conditional knock-outs used alternate stepping, not hopping, during overground locomotion, suggesting normal central pattern generator function and supporting our hypothesis of minimal CST involvement in the moment-to-moment control of stereotypic locomotion. In contrast, the conditional knock-outs showed bilateral voluntary movements under conditions when single limb movements are normally produced and, as a basis for this aberrant control, developed a bilateral motor map in motor cortex that is driven by the aberrant ipsilateral CST misprojections. Therefore, a specific change in CST connectivity is associated with and explains a change in voluntary movement. PMID:24719100

Serradj, Najet; Paixão, Sónia; Sobocki, Tomasz; Feinberg, Mitchell; Klein, Rüdiger; Kullander, Klas

2014-01-01

431

Ocular growth in infant aphakia. Bilateral versus unilateral congenital cataracts.  

PubMed

In a prospective study the changes in the ocular axial lengths and in the overall refractions were examined in cases of unilateral and bilateral congenital cataract requiring surgery during the first year of life. Measurements were taken on 18 children with unilateral and on 20 children with bilateral congenital cataract at the time of surgery and up to eight years postoperatively. Surgery was performed via a pars plana/plicata approach, and all infants were fitted with contact lenses. In cases of unilateral cataract, the ocular axial length tended to be superior to the age-matched values already prior to surgery. After four to eight years, one third of the eyes were clearly above normal. In cases of bilateral cataract, the axial lengths were reduced at the age of surgery in the majority of cases, and particularly in eyes that required surgery during the first six months of life. The curvatures of the contact lenses tended to remain unchanged in bilateral cataract, and decreased by about 0.7 mm in unilateral cases. This also reflects the high degree of microphthalmia in bilateral cases. After four to eight years, the degree of microphthalmia had usually increased. The overall refraction decreased significantly in unilateral and bilateral cataract during the first four years of life. The mean values were higher in bilateral than in unilateral cataract at all ages. The mean decrease was 15 diopters in unilateral cataract (SD +/- 5.5 dpt), and 10 diopters in bilateral cataract (SD +/- 6 dpt). When correlating the age-matched differences in the ocular axial lengths at the time of surgery with the overall refractions after four to eight years, a good correlation was found in the unilateral cases (eight eyes), and a poor correlation in the bilateral cases (24 eyes). The data indicate that intraocular implants should not be used in bilateral cataract requiring surgery during the first year of life as long as there is no possibility to change their refraction while in place. In unilateral cases, a relatively accurate prediction appears possible in a small number of eight eyes. However, an additional important correction with glasses would be needed before reaching the final refraction resulting in a high degree of aniseiconia, and eliminating the chances for binocular vision that are small anyway. Furthermore, data from other authors would indicate that the change in refraction may be much more important than expected when using intraocular implants in unilateral congenital cataract requiring surgery during the first year of life.(ABSTRACT TRUNCATED AT 400 WORDS) PMID:8015788

Lorenz, B; Wörle, J; Friedl, N; Hasenfratz, G

1993-12-01

432

Lateral geniculate lesions causing reversible blindness in a pre-eclamptic patient with a variant of posterior reversible encephalopathy syndrome.  

PubMed

Bilateral lateral geniculate nucleus (LGN) injury is a rare cause of vision loss. We describe a patient with pre-eclampsia who developed profound but reversible bilateral vision loss, bilateral serous retinal detachments, and magnetic resonance imaging signs of a variant of posterior reversible encephalopathy syndrome (PRES) that affected both LGNs and spared the retrogeniculate pathways. We provide evidence that the visual loss was not from the chorioretinal lesions but from the LGN lesions. The concurrence of PRES and lesions attributed to choroidal hypoperfusion provides support for the notion that vasoconstriction also underlies the pathogenesis of PRES. PMID:24739995

Stem, Maxwell S; Fahim, Abigail; Trobe, Jonathan D; Parmar, Hemant A; Ibrahim, Mohannad

2014-12-01

433

A Case of Prune Belly Syndrome.  

PubMed

Prune belly syndrome (PBS) is a rare congenital disorder characterized by deficient abdominal wall muscles, urinary tract malformation, and, in males, cryptorchidism. We present a case of PBS in China. The patient was a newborn baby boy who had wrinkled, "prune-like" abdominal skin, bilateral cryptorchidism, and urinary system malformation, complicated with hypoplasia of the lung and branch of the coronary artery-right ventricular fistula. His kidney function was inadequate. The patient subsequently died at age 28 days due to septicemia from a severe urinary tract infection. PMID:23639747

Xu, Wei; Wu, Hui; Wang, Dong-Xuan; Mu, Zhi-Hong

2013-04-29

434

[Munchausen syndrome by proxy, an unusual presentation].  

PubMed

We report a 12 year old girl that first consulted for fever with bilateral knee arthralgias. A neurological workout was started due to a progressive gait disturbance, but all results were incongruent with nerve or nerve root lesions, leading to the diagnosis of a functional paralysis. The patient worsened to the point of prostration. Due to the suspicion that the mother was inducing the symptoms, the patient was admitted to the hospital, where she improved notably. She was discharged walking. The improvement during hospital stay confirmed the diagnosis of a Münchausen by proxy syndrome, mimicking a disabling neurological condition. PMID:11680966

Maida, A M; Molina, M E; Erazo, R

2001-08-01

435

Mycobacterium abscessus Lung Disease in a Patient with Kartagener Syndrome  

PubMed Central

Primary ciliary dyskinesia (PCD) is characterized by the congenital impairment of mucociliary clearance. When accompanied by situs inversus, chronic sinusitis and bronchiectasis, PCD is known as Kartagener syndrome. The main consequence of impaired ciliary function is a reduced mucus clearance from the lungs, and susceptibility to chronic respiratory infections due to opportunistic pathogens, including nontuberculous mycobacteria (NTM). There has been no report of NTM lung disease combined with Kartagener syndrome in Korea. Here, we report an adult patient with Kartagener syndrome complicated with Mycobacterium abscessus lung disease. A 37-year-old female presented to our hospital with chronic cough and sputum. She was ultimately diagnosed with M. abscessus lung disease and Kartagener syndrome. M. abscessus was repeatedly isolated from sputum specimens collected from the patient, despite prolonged antibiotic treatment. The patient's condition improved and negative sputum culture conversion was achieved after sequential bilateral pulmonary resection. PMID:25309609

Kim, Jung Hoon; Jun, Ji Eun; Ryu, Duck Hyun; Lee, Ji Eun; Jeong, Ho Jung; Jeong, Suk Hyeon; Kang, Hyung Koo; Kim, Jung Soo; Lee, Hyun; Chon, Hae Ri; Jeon, Kyeongman; Kim, Dohun; Kim, Jhingook; Koh, Won-Jung

2014-01-01

436

Van der Woude Syndrome with Short Review of the Literature  

PubMed Central

Van der Woude syndrome (VWS) is a rare autosomal dominant condition with high penetrance and variable expression. Clinical manifestation of this autosomal dominant clefting syndrome includes bilateral midline lower lip pits, cleft lip, and cleft palate along with hypodontia. These congenital lip pits appear as a malformation in the vermilion border of the lip, with or without excretion. Discomfort caused by spontaneous or induced drainage of saliva/mucus when pressure is applied or during a meal as well as poor aesthetic match is one of the main complaints of patients with congenital lip fistula. The pits are treated by surgical resection. Dentists should be aware of the congenital lip pits as in Van der Woude syndrome because they have been reported to be associated with a variety of malformations or other congenital disorders. Here, the authors report a rare case of Van der Woude syndrome with short review of the literature. PMID:25050184

Deshmukh, Pallavi K.; Deshmukh, Kiran; Mangalgi, Anand; Patil, Subhash; Hugar, Deepa; Kodangal, Saraswathi Fakirappa

2014-01-01

437

Oral manifestations associated with systemic complications of prune belly syndrome.  

PubMed

Prune belly syndrome (PBS) is a rare congenital malformation of unknown etiology characterized by congenital abnormalities including abdominal wall flaccidity, urinary tract alterations, and bilateral cryptorchidism. The incidence of the syndrome is between 1/35000 and 1/50000 live births and there is little information about the oral findings. The present case describes the oral manifestations of a 15-year-old boy diagnosed with PBS. The findings include enamel hypoplasia associated with generalized hypocalcemic dental lines. In the radiographic exam, pronounced demineralization of the trabecular bone of the jaws, loss of lamina dura in all the teeth, and discontinuity of the mandibular cortical bone were observed. Prune belly syndrome is a rare disease, whose clinical dental aspects are not pathognomonic of the syndrome. The comprehension of the systemic mechanism of PBS and its comorbidities enable an understanding of the systemic etiologic factors associated with oral manifestations. PMID:22901648

Pessoa, Larissa; Galvão, Virgilio

2013-01-01

438

[Reiter's syndrome following intravesical instillation of Bacillus Calmette-Guerin].  

PubMed

A 54-year-old man who had received intravesical instillation of bacillus Calmette-Guerin(BCG) after transurethral resection for bladder cancer suffered from multiple arthritis, bilateral conjunctivitis, miction pain and high fever. Under the diagnosis of Reiter's syndrome, a nonsteroidal anti-inflammatory drug, histamine antagonists, anti-tubercular agent and corticosteroid were administered. The symptoms were improved within one month. It is important in early diagnosis and treatment of Reiter's syndrome to observe carefully the complications following intravesical instillation of BCG. PMID:20185998

Okamoto, Kohei; Hamano, Tatsuya; Kawaguchi, Takuya

2010-02-01

439

Recurrent Transient Ischemic Attacks in a Patient with POEMS Syndrome  

PubMed Central

A 47-year-old female with a prior history of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome was admitted with transient ischemic attacks complicated by dysarthria and right-sided hemiparesis. A blood survey indicated thrombocytosis and hyperfibrinogenemia while imaging of intracranial vasculature showed occlusion of the bilateral middle cerebral arteries. POEMS syndrome, of which arterial thromboses have been mentioned as a manifestation, is rarely accompanied by transient ischemic attacks. The pathophysiologic mechanism is yet unclear and needs further investigation. PMID:24716013

Akyol, A.; Nazliel, B.; Batur Caglayan, H. Z.; Oner, Y.; Sucak, G. Turkoz

2014-01-01

440

[Livedoid vasculitis in a patient with antiphospholipid syndrome].  

PubMed

The authors present a clinical case of a 30 year old male patient admitted to the hospital for recurrent cyanosis and feet pain lasting for one year. In addition he presented bilateral purpuric lesions in the lateral maleolar region, one of which with ulceration. The finding of anticardiolipin antibodies associated with intraluminal thrombosis in the dermal vessels, allowed to conclude for Antiphospholipid syndrome. The cutaneous changes identified are named livedoid vasculitis. PMID:20711098

Serra, Sara; Saavedra, M João; Salvador, M João; Reis, J Pedro; Malcata, A

2010-01-01

441

Polymicrogyria and deletion 22q11.2 syndrome: window to the etiology of a common cortical malformation.  

PubMed

Several brain malformations have been described in rare patients with the deletion 22q11.2 syndrome (DEL22q11) including agenesis of the corpus callosum, pachygyria or polymicrogyria (PMG), cerebellar anomalies and meningomyelocele, with PMG reported most frequently. In view of our interest in the causes of PMG, we reviewed clinical data including brain-imaging studies on 21 patients with PMG associated with deletion 22q11.2 and another 11 from the literature. We found that the cortical malformation consists of perisylvian PMG of variable severity and frequent asymmetry with a striking predisposition for the right hemisphere (P = 0.008). This and other observations suggest that the PMG may be a sequela of abnormal embryonic vascular development rather than a primary brain malformation. We also noted mild cerebellar hypoplasia or mega-cisterna magna in 8 of 24 patients. Although this was not the focus of the present study, mild cerebellar anomalies are probably the most common brain malformation associated with DEL22q11. PMID:17036343

Robin, Nathaniel H; Taylor, Clare J; McDonald-McGinn, Donna M; Zackai, Elaine H; Bingham, Peter; Collins, Kevin J; Earl, Dawn; Gill, Deepak; Granata, Tiziana; Guerrini, Renzo; Katz, Naomi; Kimonis, Virginia; Lin, Jean-Pierre; Lynch, David R; Mohammed, Shehla N; Massey, Roger F; McDonald, Marie; Rogers, R Curtis; Splitt, Miranda; Stevens, Cathy A; Tischkowitz, Marc D; Stoodley, Neil; Leventer, Richard J; Pilz, Daniela T; Dobyns, William B

2006-11-15

442

Single-Session Radiofrequency Ablation of Bilateral Lung Metastases  

SciTech Connect

Purpose: This retrospective study examined the feasibility and efficacy of bilateral lung radiofrequency ablation (RFA) performed in a single session. Methods: From 2002-2009, patients with bilateral lung metastases were treated by RFA, where possible in a single session under general anesthesia with CT guidance. The second lung was punctured only if no complications occurred after treatment of the first lung. Five lung metastases maximum per patient were treated by RFA and prospectively followed. The primary endpoint was the evaluation of acute and delayed complications. Secondary endpoints were calculation of hospitalization duration, local efficacy, median survival, and median time to tumor progression. Local efficacy was evaluated on CT or positron emission tomography (PET) CT. Results: Sixty-seven patients were treated for bilateral lung metastases with RFA (mean age, 62 years). Single-session treatment was not possible in 40 due to severe pneumothoraces (n = 24), bilateral pleural contact (n = 14), and operational exclusions (n = 2). Twenty-seven (41%) received single-session RFA of lesions in both lungs for 66 metastases overall. Fourteen unilateral and four bilateral pneumothoraces occurred (18 overall, 66.7%). Unilateral (n = 13) and bilateral (n = 2) chest tube drainage was required. Median hospitalization was 3 (range, 2-8) days. Median survival was 26 months (95% confidence interval (CI), 19-33). Four recurrences on RFA sites were observed (4 patients). Median time to tumor progression was 9.5 months (95% CI, 4.2-23.5). Conclusions: Although performing single-session bilateral lung RFA is not always possible due to pneumothoraces after RFA of first lung, when it is performed, this technique is safe and effective.

Palussiere, Jean, E-mail: palussiere@bergonie.org; Gomez, Fernando; Cannella, Matthieu; Ferron, Stephane; Descat, Edouard [Institut Bergonie, Department of Radiology, Regional Cancer Centre (France); Fonck, Marianne [Institut Bergonie, Department of Digestive Oncology (France); Brouste, Veronique [Institut Bergonie, Clinical and Epidemiological Research Unit (France); Avril, Antoine [Institut Bergonie, Department of Surgery (France)

2012-08-15

443

Proteus syndrome.  

PubMed

Proteus syndrome is a rare sporadic, hamartoneoplastic disorder of vascular, skeletal, and soft tissues that causes asymmetry of the skull, body, arms, and the legs. The name "Proteus" of the Greek god who had the ability to change his shape was coined to define the variety of deformities including partial gigantism of the hands or feet, asymmetry of the arms and legs, hypertrophy of long bones, plantar hyperplasia, haemangiomas, lipomas, varicosities, linear verrucous epidermal naevi, macrocephaly, and cranial hyperostoses. The basic defect seems to be the focal overgrowth of cellular elements in skin, bone, and other connective tissues. The variable features of the syndrome make differential diagnosis challenging for clinicians. The most important features are the hamartomatous disorders. The long-term prognosis is still not clear. As it is a hamartoneoplastic and incompletely delineated syndrome, the patients must be followed up because of the possible risk of neoplasms. PMID:14649692

Bilkay, Ufuk; Tokat, Cenk; Ozek, Cuneyt; Gundogan, Hakan; Erdem, Ozgur; Gurler, Tahir; Cagdas, Arman

2003-01-01

444

Compartment syndromes  

NASA Technical Reports Server (NTRS)

The compartment syndrome is defined as a condition in which high pressure within a closed fascial space (muscle compartment) reduces capillary blood perfusion below the level necessary for tissue viability'. This condition occurs in acute and chronic (exertional) forms, and may be secondary to a variety of causes. The end-result of an extended period of elevated intramuscular pressure may be the development of irreversible tissue injury and Volkmann's contracture. The goal of treatment of the compartment syndrome is the reduction of intracompartmental pressure thus facilitating reperfusion of ischaemic tissue and this goal may be achieved by decompressive fasciotomy. Controversy exists regarding the critical pressure-time thresholds for surgical decompression and the optimal diagnostic methods of measuring intracompartmental pressures. This paper will update and review some current knowledge regarding the pathophysiology, aetiology, diagnosis, and treatment of the acute compartment syndrome.

Mubarak, S. J.; Pedowitz, R. A.; Hargens, A. R.

1989-01-01

445

Apert's Syndrome  

PubMed Central

ABSTRACT Apert's syndrome (acrocephalosyndactyly) is a rare congenital disorder characterized by craniosynostosis, midfacial malforma­tion and symmetrical syndactyly of hands and feet. Craniofacial deformities include cone-shaped calvarium, fat forehead, prop-tosis, hypertelorism and short nose with a bulbous tip. Intraoral findings include high arched palate with pseudocleft, maxillary transverse and sagittal hypoplasia with concomitant dental crowding, skeletal and dental anterior open bite and several retained primary teeth. We report one such case of 14-year-old boy having all the classical features of Apert's syndrome with particular emphasis on brief review of genetic features. How to cite this article: Kumar GR, Jyothsna M, Ahmed SB, Lakshmi KRS. Apert's Syndrome. Int J Clin Pediatr Dent 2014;7(1):69-72. PMID:25206244

Jyothsna, Mandapati; Ahmed, Syed Basheer; Sree Lakshmi, Ketham Reddy

2014-01-01

446

Cardiorenal syndromes  

PubMed Central

Cardiorenal syndromes (CRS) have been subclassified as five defined entities which represent clinical circumstances in which both the heart and the kidney are involved in a bidirectional injury and dysfunction via a final common pathway of cell-to-cell death and accelerated apoptosis mediated by oxidative stress. Types 1 and 2 involve acute and chronic cardiovascular disease (CVD) scenarios leading to acute kidney injury or accelerated chronic kidney disease. Types 2 and 3 describe acute and chronic kidney disease leading primarily to heart failure, although it is possible that acute coronary syndromes, stroke, and arrhythmias could be CVD outcomes in these forms of CRS. Finally, CRS type 5 describes a simultaneous insult to both heart and kidneys, such as sepsis, where both organs are injured simultaneously. Both blood and urine biomarkers are reviewed in this paper and offer a considerable opportunity to enhance the understanding of the pathophysiology and known epidemiology of these recently defined syndromes. PMID:21286212

McCullough, Peter A; Ahmad, Aftab

2011-01-01

447

Large bilateral internal auditory meatus associated with bilateral superior semicircular canal dehiscence.  

PubMed

Superior semicircular canal dehiscence and an abnormally wide internal auditory meatus are clinical entities characterized by vestibular and cochlear symptoms. These symptoms are induced by hypersensitivity of labyrinthine receptors secondary to a bone defect of the otic capsule. We report the case of a 41-year-old man with congenital right-sided hearing loss who presented with bilateral superior semicircular canal dehiscence that was associated with wide, bulbous internal auditory meatus and a loss of the bony wall separating the lateral end of the meatus from the cochlea. The patient was experiencing vestibular and cochlear symptoms in the right ear and disabling tinnitus in the left ear. However, he refused all treatment and was lost to follow-up. PMID:23354888

Manzari, Leonardo; Scagnelli, Paola

2013-01-01

448

A different type of branchial fistula as part of a branchiootorenal syndrome.  

PubMed

We describe an extremely rare case of a complete fistula, a combination of the first 2 branchial arches as a component of branchiootorenal syndrome. A 13-year-old girl presented with the complaint of intermittent drainage from bilateral preauricular and right lower neck external openings. A contrast fistulogram revealed a complete fistula. Diagnostic features and surgical techniques are discussed in detail. PMID:22325402

Can, Ilknur Haberal; Do?an, Sedat; Dönmez, Melahat; Do?an, Meryem; Samim, Ethem Erdal

2012-02-01

449

Flammer syndrome  

PubMed Central

The new term Flammer syndrome describes a phenotype characterized by the presence of primary vascular dysregulation together with a cluster of symptoms and signs that may occur in healthy people as well as people with disease. Typically, the blood vessels of the subjects with Flammer syndrome react differently to a number of stimuli, such as cold and physical or emotional stress. Nearly all organs, particularly the eye, can be involved. Although the syndrome has some advantages, such as protection against the development of atherosclerosis, Flammer syndrome also contributes to certain diseases, such as normal tension glaucoma. The syndrome occurs more often in women than in men, in slender people than in obese subjects, in people with indoor rather than outdoor jobs, and in academics than in blue collar workers. Affected subjects tend to have cold extremities, low blood pressure, prolonged sleep onset time, shifted circadian rhythm, reduced feeling of thirst, altered drug sensitivity, and increased general sensitivity, including pain sensitivity. The plasma level of endothelin-1 is slightly increased, and the gene expression in lymphocytes is changed. In the eye, the retinal vessels are stiffer and their spatial variability larger; the autoregulation of ocular blood flow is decreased. Glaucoma patients with Flammer syndrome have an increased frequency of the following: optic disc hemorrhages, activated retinal astrocytes, elevated retinal venous pressure, optic nerve compartmentalization, fluctuating diffuse visual field defects, and elevated oxidative stress. Further research should lead to a more concise definition, a precise diagnosis, and tools for recognizing people at risk. This may ultimately lead to more efficient and more personalized treatment. PMID:25075228

2014-01-01

450

Flammer syndrome.  

PubMed

The new term Flammer syndrome describes a phenotype characterized by the presence of primary vascular dysregulation together with a cluster of symptoms and signs that may occur in healthy people as well as people with disease. Typically, the blood vessels of the subjects with Flammer syndrome react differently to a number of stimuli, such as cold and physical or emotional stress. Nearly all organs, particularly the eye, can be involved. Although the syndrome has some advantages, such as protection against the development of atherosclerosis, Flammer syndrome also contributes to certain diseases, such as normal tension glaucoma. The syndrome occurs more often in women than in men, in slender people than in obese subjects, in people with indoor rather than outdoor jobs, and in academics than in blue collar workers. Affected subjects tend to have cold extremities, low blood pressure, prolonged sleep onset time, shifted circadian rhythm, reduced feeling of thirst, altered drug sensitivity, and increased general sensitivity, including pain sensitivity. The plasma level of endothelin-1 is slightly increased, and the gene expression in lymphocytes is changed. In the eye, the retinal vessels are stiffer and their spatial variability larger; the autoregulation of ocular blood flow is decreased. Glaucoma patients with Flammer syndrome have an increased frequency of the following: optic disc hemorrhages, activated retinal astrocytes, elevated retinal venous pressure, optic nerve compartmentalization, fluctuating diffuse visual field defects, and elevated oxidative stress. Further research should lead to a more concise definition, a precise diagnosis, and tools for recognizing people at risk. This may ultimately lead to more efficient and more personalized treatment. PMID:25075228

Konieczka, Katarzyna; Ritch, Robert; Traverso, Carlo Enrico; Kim, Dong Myung; Kook, Michael Scott; Gallino, Augusto; Golubnitschaja, Olga; Erb, Carl; Reitsamer, Herbert A; Kida, Teruyo; Kurysheva, Natalia; Yao, Ke

2014-01-01

451

Cardiohepatic Syndrome.  

PubMed

Accumulating evidence shows that acute as well as chronic heart disease can directly contribute to an acute or chronic worsening of liver function and vice versa. Description and definition of cardiohepatic syndrome (CHS) in this review are based on the cardiorenal syndrome (CRS) concept. The eye-catching analogy between CHS and CRS is applied to facilitate an understanding of the pathophysiology and overall burden of disease for each of the proposed CHS subtypes, their natural course, and associated morbidity and mortality. PMID:25391350

Poelzl, Gerhard; Auer, Johann

2014-11-13

452

Piriformis syndrome.  

PubMed

The author presents a case of a female runner with left buttock pain aggravated by exercise. She underwent extensive testing by other physicians and received cortisone injections on two occasions. The patient was referred to the podiatry department because of continued discomfort and the inability to run. Examination revealed pain on hip flexion and abduction against resistance. Because of the possibility of piriformis syndrome, she started stretching exercises, reduced her activity, and the pain disappeared. The diagnosis of piriformis syndrome was confirmed by a general surgeon. PMID:2724111

Julsrud, M E

1989-03-01

453

Bite force and electromyograpy during maximum unilateral and bilateral clenching.  

PubMed

Maximum voluntary bite force has often been studied as an indicator of the functional state of the masticatory system. Bilateral, as well as unilateral, methods have been used to determine bite force. Only a few studies have compared the outcomes of both methods. The aim of this study was to measure bite force and jaw-muscle activity during bilateral as well as unilateral maximum clenching in a large number of healthy subjects, so that the results could be compared. In a group of 81 dentate subjects we observed an average bilateral bite force of 569 N. The average unilateral bite force was significantly lower, being 430 N (right) and 429 N (left). Masseter and anterior temporal muscle activities were also significantly lower during unilateral clenching as compared with bilateral clenching. The masseter muscles showed no difference in activity between the ipsilateral side and the contralateral side during unilateral clenching. In contrast, the activity of the anterior temporal muscle on the ipsilateral side was significantly higher than on the contralateral side. Thus, the change in the forces acting on the jaw during unilateral clenching compared with bilateral clenching leads to a different response in the temporal muscles than in the masseter muscles. PMID:18471239

van der Bilt, Andries; Tekamp, Anneke; van der Glas, Hilbert; Abbink, Jan

2008-06-01

454

Cryptococcal meningitis presenting with bilateral complete ophthalmoplegia: a case report  

PubMed Central

Background Cryptococcus neoformans is saprophytic encapsulated yeast. Infection is acquired by inhalation of the organism and could be asymptomatic or limited to the lungs, specially in the immunocompetent host. Cryptococcal meningitis is a serious opportunistic infection among post transplant recipients. Cranial nerve palsies and ophthalmoplegia are well known complications of this disease, but bilateral complete ophthalmoplegia is a very rare presentation. Case Presentation A Sri Lankan young male, who is a post kidney transplant recipient, presented with bilateral complete ophthalmoplegia and subsequently was diagnosed to have cryptococcal meningitis based on Indian ink stain and culture of cerebrospinal fluid (CSF). His magnetic resonance imaging (MRI) showed bilateral multiple nodular lesions in both basal ganglia and thalami. Brainstem imaging was normal. Conclusions Cryptococcal meningitis is a serious fungal infection in post transplant patients. It should be suspected in any immunocompromised patient with fever, headache and focal neurological signs. Bilateral thalamic lesions, inflammation and invasion of the cranial nerves and raised intracranial pressure were thought to be possible mechanisms resulting in bilateral complete ophthalmoplegia in this patient. PMID:24885277

2014-01-01

455

Bilateral iliopsoas hematoma: Case report and literature review  

PubMed Central

Background: Clinically significant spontaneous bilateral iliopsoas hematoma is a rare complication of anticoagulation therapy. Definitive treatment of spontaneous iliopsoas hematomas is not well-established and varies between observation and surgical intervention. The intramuscular hematoma causes severe pain, muscle dysfunction, and occasionally nerve palsy with the femoral nerve most commonly affected. Most patients are neurologically normal but when a significant neurological deficit is associated with iliopsoas hematoma, optimal treatment recommendations vary. We report a case of spontaneous bilateral iliopsoas hematomas causing significant bilateral femoral nerve dysfunction. Case Description: The authors present the case of a 63-year-old female who developed bilateral femoral nerve palsy due to anticoagulation bleeding complication. Magnetic resonance imaging demonstrated large bilateral intramuscular psoas hematomas causing femoral nerve compression. Surgical evacuation and decompression of the femoral nerves was performed with rapid neurological improvement. Conclusion: Management recommendations depend on the volume and cause of the hematoma, timing of diagnosis, and the degree of neurological impairment. A conservative approach with bed rest and correction of bleeding abnormalities to allow the hematoma to spontaneously resorb has been utilized for patients with small hematomas and little to no neurological symptoms. In contrast, more aggressive recommendations have been made for patients with large hematomas, severe motor function deficits, or hemodynamic instability. PMID:24232386

Basheer, Azam; Jain, Rajan; Anton, Toomas; Rock, Jack

2013-01-01

456

Hole filling using joint bilateral filtering for moving object segmentation  

NASA Astrophysics Data System (ADS)

Due to various reasons (e.g., poor quality of image, sudden changes in light conditions, occlusions, or objects with similar colors), holes may appear in the moving object region when performing moving object segmentation. How to remove these holes is a crucial problem in high-quality segmentation. A moving object segmentation approach combining symmetrical differencing and joint bilateral filtering is proposed. One important aspect of our approach is that joint bilateral filtering is used to eliminate/reduce the holes in the moving object region, which makes the extracted moving object more accurate and complete, in many cases in contrast to the reference temporal difference approaches. The approach first performs block-based symmetrical differencing to obtain coarse moving object regions. Then, a joint bilateral filter that uses the current gray image as a guide image is adopted to smooth the difference image. Next, edge detection is performed on the smoothed difference image and the current gray image, respectively, to get the edges of moving objects. Finally, the undesired background is distinguished from the moving object and is cut off by a postprocessing module. In addition, the fast joint bilateral filters that can be used as substitutes for the classical joint bilateral filter are discussed. Experimental results show that this approach can effectively fill the holes in the moving object region and provide an improvement in accuracy of moving object segmentation in many cases.

Liu, Ran; Li, Bole; Huang, Zhengwei; Cao, Donghua; Tan, Yingchun; Deng, Zekun; Xu, Miao; Jia, Ruishuang; Tan, Weimin

2014-11-01

457

Simultaneous repair of bilateral inguinal hernias under local anesthesia.  

PubMed Central

OBJECTIVE: The authors confirm the advantages of simultaneous repair of bilateral inguinal hernias, indicate that it is feasible to perform the procedure under local anesthesia, and suggest that when an open tension-free technique is used, the results are superior to those of laparoscopic repair of bilateral inguinal hernias. SUMMARY BACKGROUND DATA: Between 1971 and 1995, simultaneous repair of bilateral inguinal hernias were performed in 2953 men. Initially, between 1971 and 1984, patients with indirect hernias underwent the traditional tissue approximation repair. Those with direct hernias had the same procedure, with the repair additionally buttressed by a sheet of Marlex mesh (Davol, Inc., Cronston, RI). Between 1984 and 1995, both direct and indirect hernias were repaired using the open tension-free hernioplasty procedure. METHOD: The 2953 patients underwent simultaneous repair of bilateral inguinal hernias under local anesthesia in a private practice setting in general hospitals. RESULTS: In those cases in which the "tension free" technique was used, patients experienced minimal to mild postoperative pain and had a short recovery period, with a recurrence rate of 0.1%. CONCLUSIONS: Uncomplicated bilateral inguinal hernias in adults are best treated simultaneously. It is feasible to perform the operation under local anesthesia, and when an open tension-free repair is used, postoperative pain and recovery periods are equally comparable with those of laparoscopic repair, although the complication and the recurrence rates are significantly less. Images Figure 1. Figure 2. Figure 3. PMID:8604904

Amid, P K; Shulman, A G; Lichtenstein, I L

1996-01-01

458

Bilateral Patching in Retinal Detachment: Fluid Mechanics and Retinal “Settling”  

PubMed Central

Purpose. When a patient suffers a retinal detachment and surgery is delayed, it is known clinically that bilaterally patching the patient may allow the retina to partially reattach or “settle.” Although this procedure has been performed since the 1860s, there is still debate as to how such a maneuver facilitates the reattachment of the retina. Methods. Finite element calculations using commercially available analysis software are used to elucidate the influence of reduction in eye movement caused by bilateral patching on the flow of subretinal fluid in a physical model of retinal detachment. Results. It was found that by coupling fluid mechanics with structural mechanics, a physically consistent explanation of increased retinal detachment with eye movements can be found in the case of traction on the retinal hole. Large eye movements increase vitreous traction and detachment forces on the edge of the retinal hole, creating a subretinal vacuum and facilitating increased subretinal fluid. Alternative models, in which intraocular fluid flow is redirected into the subretinal space, are not consistent with these simulations. Conclusions. The results of these simulations explain the physical principles behind bilateral patching and provide insight that can be used clinically. In particular, as is known clinically, bilateral patching may facilitate a decrease in the height of a retinal detachment. The results described here provide a description of a physical mechanism underlying this technique. The findings of this study may aid in deciding whether to bilaterally patch patients and in counseling patients on pre- and postoperative care. PMID:21666245

2011-01-01

459

Kallmann syndrome--a case report.  

PubMed

Kallmann syndrome is a very rare hereditary disease. It is characterized by hypogonadotropic hypogonadism in association with anosmia ot hyposmia, both of which occur as a result of the failure of neuronal migration of the luteinizing hormone releasing hormone (LHRH)--secreting neurons and the neurons of the vemeronasal nerve. It can be autosomal dominant, autosomal recessive, or X-linked mode of inheritance. We report a case of Kallmann syndrome that presented with delay puberty, color blindness, gynecomastia, and absence of smell. Plasma levels of LH, FSH and testosterone were very low. The patient's adrenal and thyroid hormone levels were normal. Chromosome analysis showed 46, XY karyotype without deletion in KAL gene (Xp22.3) from FISH. After 9 months of treatment by HCG and HMG, the amount of pubic hair and the volume of bilateral testes, as well as the level of testosterone had increased. Most importantly, motile sperm count be found in semen. PMID:12380326

Li, Ching-Chia; Chao, Mei-Chyn; Huang, Shu-Pin; Chou, Yii-Her; Huang, Chun-Hsiung; Chen, A-Hsiung; Wu, Yi-Ping

2002-07-01

460

[West syndrome associated with epileptic negative myoclonus].  

PubMed

We report a 10-month-old girl who had brief epileptic negative myoclonus during the course of West syndrome. She began to have epileptic spasms in series at the age of 8 months. Video-electroencephalograph (EEG) monitoring revealed that she also had brief epileptic negative myoclonus when she was 10 months old. Brief atonia of limbs occurred in isolation or in a cluster during drowsiness or sleep. The ictal EEG exhibited diffuse polyspikes and waves or diffuse high-voltage slow waves that were overlapped by low-voltage fast waves. 3 to 4 hundred milliseconds of silent periods were observed in the bilateral deltoid electromyograms, which correspond to the EEG patterns. The occurrence of other types of seizures, partial seizures in particular, accompanied by epileptic spasms has been fully investigated. This is the first case report of a patient with West syndrome whose coexisting epileptic negative myoclonus was confirmed by a silent electromyogram pattern. PMID:25403066

Shibata, Takashi; Yoshinaga, Harumi; Oka, Makio; Kobayashi, Katsuhiro

2014-09-01

461

Asperger Syndrome  

MedlinePLUS

... Organizations Column1 Column2 MAAP Services for Autism, Asperger Syndrome, and PDD P.O. Box 524 Crown Point, IN 46308 info@aspergersyndrome.org http://www.aspergersyndrome.org/ Tel: 219-662-1311 Fax: 219-662-1315 Autism Science Foundation 29 West 39th Street Suite 502 New York, NY 10018 ...

462

Klinefelter Syndrome  

MedlinePLUS

... testosterone. That doesn't make a guy less male, but it can affect things like penis and testicle growth. Boys with Klinefelter syndrome may ... bigger muscles, a deeper voice, growth of the penis, and facial and body ... or reverse infertility. Physical therapy, occupational therapy, and ...

463

[SAPHO syndrome].  

PubMed

The SAPHO syndrome, an acronym for synovitis, acne, pustulosis, hyperostosis and osteitis, is a rare disease which affects bones, joints and the skin. The main osteoarticular features are hyperostosis and osteitis. Osteoarticular symptoms predominantly occur on the anterior chest wall but the spine and the peripheral skeleton can also be involved. The most important skin affections are palmoplantar pustulosis and severe acne. The etiology of this syndrome remains unclear but infectious, immunological and genetic factors are involved. The diagnostic features of SAPHO syndrome are clinical and radiological. The most important diagnostic procedure is Tc-99 m bone scintigraphy but conventional x-rays as well as computed tomography (CT) and magnetic resonance imaging (MRI) can also contribute to the final diagnosis. Bone histology and positron emission tomography CT (PET-CT) may help to differentiate SAPHO syndrome from malignancies and infectious osteomyelitis. Nonsteroidal anti-inflammatory drugs (NSAIDs) are the cornerstone of treatment. The results obtained using antibiotics and disease-modifying antirheumatic drugs (DMARDs), such as sulfasalazine and methotrexate are inconsistent. Bisphosphonates and anti-tumor necrosis factor (anti-TNF) drugs have shown promising results in small studies but further research is still necessary. PMID:25260820

Heldmann, F; Kiltz, U; Baraliakos, X; Braun, J

2014-10-01

464

Metabolic Syndrome  

MedlinePLUS

... Web version Metabolic Syndrome Overview What is insulin resistance? Your body changes most of the food you eat into glucose (a form of sugar). Insulin is a hormone produced by the pancreas that allows ... as insulin resistance. If you have insulin resistance, your body will ...

465

[Locomotive syndrome and metabolic syndrome].  

PubMed

The Japanese Orthopedic Association coined the term locomotive syndrome (LS) to designate a condition of elderly people in high risk groups of requiring nursing care because of problems with their musculoskeletal diseases. LS is a socioeconomic concept, and closely associated with osteoporosis, osteoarthritis, and sarcopenia. Recent studies have revealed that metabolic syndrome (MS), a clustering of cardiovascular risk factors, has been related with LS. For example, individuals with MS have a greater risk of osteoarthritis and sarcopenia. Secreted factors from adipose tissue and skeletal muscles, namely, adipokines and myokines, are involved in the association of LS and MS. PMID:25509811

Fukushi, Jun-ichi; Iwamoto, Yukihide

2014-10-01

466

Fibromuscular dysplasia presenting with asymptomatic bilateral renal infarctions.  

PubMed

Fibromuscular dysplasia (FMD) is a noninflammatory nonatherosclerotic vascular disease. It is the second cause of renovascular hypertension after atherosclerosis. Although FMD usually has a good prognosis, renal infarctions and artery dissections have been described. We present the case of a 38-year-old woman with hypertension and asymptomatic bilateral renal infarctions. Bilateral FMD of segmental branches of the renal arteries was diagnosed by digital subtraction angiography after an exhaustive study. Previous intake of nonsteroidal anti-inflammatory drugs may also have played a significant role in the development of renal infarctions. To our knowledge, bilateral renal infarctions complicating FMD have been reported in only four previous cases; only in one of those cases, renal infarctions were asymptomatic. PMID:23292597

González-Moreno, J; Campins, M A; Buades, J M

2014-01-01

467

Acute bilateral angle closure glaucoma induced by methazolamide  

PubMed Central

Purpose To report a case of bilateral acute myopia and angle closure glaucoma after ingestion of methazolamide. Methods An interventional case report of a 70-year-old male who developed bilateral, acute myopia and angle closure glaucoma after ingesting methazolamide tablets for the treatment of normal tension glaucoma. Results Bilateral anterior chamber shallowing associated with ciliary body edema, supraciliary effusions, and shallow posterior choroidal effusions were documented with slit-lamp photography and high-frequency ultrasonography. Near complete resolution of these signs after discontinuation of methazolamide were also documented. Conclusion Methazolamide may be associated with secondary myopia and angle closure glaucoma. Discontinuation of methazolamide leads to resolution of this process, as documented by slit-lamp photography and high-frequency ultrasonography. PMID:23430961

Aref, Ahmad A; Sayyad, Fouad E; Ayres, Bernadete; Lee, Richard K

2013-01-01

468

Bilateral vestibular hypofunction in neurosarcoidosis: a case report.  

PubMed

We describe the case of a 59-year-old woman who presented with progressive bilateral vestibular hypofunction and who was found to have bilateral granulomatous mass lesions of the mesial temporal lobe. Initially, her condition stabilized neurologically with corticosteroids, but a diagnosis of neurosarcoidosis was delayed because of the unusual presentation and persistently normal chest imaging results and serum angiotensin-converting enzyme (ACE) levels. Approximately 1 year after her initial presentation, the patient died of complications of a myocardial infarction and pulmonary embolism. Sarcoidosis should be considered in the differential diagnosis of idiopathic bilateral vestibular hypofunction even if the chest imaging and serum ACE levels are normal, particularly when there is evidence of a multisystem process. PMID:21229491

Smith, Jonathan H; Stovall, Kyndra C; Coons, Stephen; Fife, Terry Douglas

2011-01-01

469

Multiple Symmetric Lipomatosis (Madelung's Disease) Presenting as Bilateral Huge Gynecomastia  

PubMed Central

Multiple symmetric lipomatosis (MSL), or Madelung's disease, is a rare disease of unknown etiology. It is characterized by the presence of loose adipose tissue deposits localized in the cervical region and upper body. MSL presenting as bilateral huge gynecomastia is an extremely rare phenomenon. The present report describes a case of MSL in a 66-year-old man. The patients presented with bilateral breast bulging. He had a history of cigarette and alcohol use. His condition was treated with a bilateral nipple-sparing mastectomy. MSL can present as a form of gynecomastia, for its accurate diagnosis and proper treatment of MSL, increasing awareness of the clinical characteristics of the disease is required, especially amongst breast surgeons. Herein, we review the literature and discuss the clinical characteristics, pathology, and surgical treatment of MSL. PMID:25548590

Jang, Jae Hoon; Lee, Anbok; Han, Sang-Ah; Ryu, Jung-Kyu

2014-01-01

470

Bilateral Diffuse Tumorous Pseudoangiomatous Stromal Hyperplasia: A Case of Bilateral Mastectomy in a 29-Year-Old Woman  

PubMed Central

Pseudoangiomatous stromal hyperplasia (PASH) is a benign breast lesion commonly encountered as an incidental microscopic finding. However, it can also manifest as a mass-forming lesion (tumorous PASH) capable of recurrence after surgical excision. Most of the previously reported cases of tumorous PASH present as a single dominant mass. Here we reported a rare case of diffuse tumorous PASH involving bilateral breasts clinically mimicking malignancy. A 29-year-old African-American female presented with a one-year history of bilateral breast enlargement and asymmetry. Physical examination revealed multiple palpable nodules in bilateral breasts. Imaging studies demonstrated innumerable homogeneously enhancing masses throughout both breasts, greater on the left, with multiple cysts and edema. Biopsy of the breast nodules demonstrated histopathological changes consistent with PASH. Due to the extent of the lesions and progressive clinical symptoms, decision was made to perform bilateral mastectomy. Macroscopic examination of the bilateral mastectomy specimens revealed markedly enlarged breasts with marked edema and numerous well-defined firm nodules. Microscopic evaluation of the nodules confirmed the diagnosis of PASH. No evidence of malignancy was identified. Recognition of this rare form of PASH is essential for the proper clinical management. PMID:25544925

Dai, Hongyan; Connor, Carol; Cui, Wei; Gatewood, Jason; Fan, Fang

2014-01-01

471

The Source for Syndromes.  

ERIC Educational Resources Information Center

Designed for practicing speech-language pathologists, this book discusses different syndrome disabilities, pertinent speech-language characteristics, and goals and strategies to begin intervention efforts at a preschool level. Chapters address: (1) Angelman syndrome; (2) Asperger syndrome; (3) Down syndrome; (4) fetal alcohol syndrome; (5) fetal…

Richard, Gail J.; Hoge, Debra Reichert

472

Outpatient bilateral supracostal tubeless percutaneous nephrolithotomy for staghorn calculi  

PubMed Central

Percutaneous nephrolithotomy (PCNL) is the surgical procedure of choice to treat staghorn calculi. Most centres perform PCNL as the traditional inpatient procedure. However, outpatient PCNL has been successfully attempted and represents a feasible method of reducing hospital costs. We report the case of a 35-year-old female who underwent outpatient simultaneous PCNL for bilateral renal staghorn calculi. The patient was discharged in stable condition less than 3 hours following the procedure with minimal discomfort. To the best of our knowledge, this case report is the first to describe a successfully completed outpatient bilateral supracostal tubeless PCNL for staghorn calculi. PMID:24839499

Kokorovic, Andrea; Wilson, James W.L.; Beiko, Darren

2014-01-01

473

Bilateral choanal atresia-Management with hegar's dilator.  

PubMed

Here we report a case of bilateral choanal atresia which is managed by transnasal perforation of atretic bony laminae using Hegar's uterine dilators The baby's airway is patent even after one yearAdvantages of using Hegar's dilator for correction of choanal atresia soon after birth are it is a blunt instrument, it's curvature fits with the curvature of the nasal cavity, dilators of increasing sizes are available, and as such injuries to the nasopharynx and surrounding areas are avoidedWe recommend this procedure as it is simple and effective in an emergency situation like bilateral choanal atresia Moreover this procedure is cheap when compared with other procedures. PMID:23120066

John, Mathai; Prethapan, Nair

2004-04-01

474

Neoplastic meningitis presenting with dysphagia and bilateral vocal cord paralysis.  

PubMed

Neoplastic meningitis is the infiltration of the leptomeninges and subarachnoid space by tumor cells occurring in 3% to 5% of patients with systemic malignancies. Most cases present with multifocal neurological symptoms that vary according to the central nervous system territory involved. Here, we describe the first reported case to our knowledge of neoplastic meningitis causing bilateral vocal cord paralysis. Early diagnosis of this progressive disease process is essential to achieving a better treatment response and improved survival. This report demonstrates that neoplastic meningitis should be considered in the investigation of bilateral vocal cord paralysis, particularly in patients with a history of metastatic disease. PMID:24510714

Dixon, Peter R; Alsaffar, Hussain; Symons, Sean P; Enepekides, Danny; Higgins, Kevin M

2014-08-01

475

Bilateral carotid endarterectomy as treatment of vascular pulsatile tinnitus.  

PubMed

Atherosclerotic carotid artery disease (ACAD) is a rare but recognized cause of pulsatile tinnitus. Existing literature of reported cure for pulsatile tinnitus is reviewed. We found: (1) a male preponderance exists; (2) ipsilateral carotid endarterectomy (CEA) for tinnitus is 92% (12 of 13) effective; (3) proximal lesions lend themselves to CEA whereas distal lesions have been treated by stenting; (4) overall 68% (15 of 22) are cured by intervention; and (5) 89% (17 of 19) can expect immediate relief. We now present a case of bilateral pulsatile tinnitus relieved by bilateral carotid endarterectomy. PMID:19446986

Singh, Devinder P; Forte, Antonio J V; Brewer, Michael B; Nowygrod, Roman

2009-07-01

476

Bilateral retinal phototoxic injury during cataract surgery in a child.  

PubMed

The intense illumination of the operating microscope has been implicated in photic retinopathy in patients and in animal studies. We report a case of bilateral macular phototoxicity occurring in an eleven-year-old child who underwent bilateral cataract surgery for radiation cataracts. We are unaware of other reports of retinal toxicity occurring during pediatric cataract surgery. We hypothesize that this child may have been predisposed to macular injury because of previous chemotherapy and radiotherapy exposure. Ophthalmic surgeons should be aware that light toxicity from the operating microscope might also occur in the pediatric population. PMID:15226731

Long, Vernon W; Woodruff, Geoffrey H

2004-06-01

477

Horner'sy syndrome and its significance in the management of head and neck trauma.  

PubMed

The history, mechanism and aetiology of Horner'sy Syndrome is presented and the pharmacology of the pupil is discussed. The case reported is a rare combination of Horner's Syndrome in a patient who sustained bilateral fractures of the mandible and a chest injury. It is emphasised that the miotic changes in Horner's Syndrome, in combination with head injuries can lead to confusion in diagnosis and the potential anaesthetic hazards and their influence on the management of the facial injury are outlined. PMID:1070343

White, P R

1976-11-01

478

Lofgren's Syndrome-Acute Onset Sarcoidosis and Polyarthralgia: A Case Report  

PubMed Central

Lofgren's syndrome is an acute form of sarcoidosis characterized by erythema nodosum, bilateral hilar lymphadenopathy (BHL), and polyarthralgia or polyarthritis. This syndrome is common among Caucasians but rare in the Korean population. A 44-year-old woman was admitted to our hospital complaining of polyarthralgia. A chest radiograph revealed BHL and nodular shadows. Angiotensin-converting enzyme levels were within the normal range. Tissue biopsy from a mediastinum lymph node showed noncaseating granulomas. We diagnosed her with Lofgren's syndrome, an acute form of sarcoidosis. PMID:23705129

Byun, Chan Woo; Yoon, Joon Shik; Kim, Se Hwa

2013-01-01

479

Glioblastoma multiforme associated with klinefelter syndrome.  

PubMed

A 54-year-old man with Klinefelter syndrome presented with glioblastoma multiforme manifesting as a 2-week history of motor weakness of the bilateral extremities. Magnetic resonance imaging showed multiple heterogeneously enhanced tumors in the bilateral frontal lobes. Angiography showed no tumor stain or arteriovenous shunt. The tumor was partially removed through a right craniotomy. The histological diagnosis was glioblastoma. Immunohistochemical examination showed no O(6)-methylguanine-deoxyribonucleic acid methyltransferase protein expression. Postoperative local radiotherapy (60 Gy/30 fractions) combined with temozolomide (75 mg/m(2) x 42 days) and interferon-beta (3,000,000 U, 3 times/week) was performed. The patient's clinical status rapidly deteriorated during chemoradiotherapy, and he died of tumor progression 3.5 months after the surgery. Postmortem examination revealed widespread glioblastoma infiltrating the basal ganglia and thalamus. Klinefelter syndrome is associated with increased cancer predisposition, especially for male breast cancer and germ cell tumors, but glioma is extremely rare. The abnormal genetic constitution of this patient may have been directly responsible for the poor outcome. PMID:19940404

Sasayama, Takashi; Mizukawa, Katsu; Sakagami, Yoshio; Mizowaki, Takashi; Tanaka, Kazuhiro; Ohbayashi, Chiho; Mori, Kiyoshi; Kitazawa, Sohei; Kohmura, Eiji

2009-11-01

480

Sweet syndrome and differentiation syndrome in a patient with acute promyelocytic leukemia  

PubMed Central

The differentiation syndrome is an inflammatory reaction with increased capillary permeability that occurs in up to 25% of patients with acute promyelocytic leukemia treated with all-trans retinoic acid. A 50-year-old man with acute promyelocytic leukemia underwent chemotherapy with idarubicin and all-trans retinoic acid. On day +21 the patient developed pruritic prepatelar papules as well as several 10 mm subcutaneous nodules in both thighs accompanied by persistent fever. On the day +25 the patient presented with bilateral pulmonary crackles, infiltrates in the right lower lobe and severe hypotension which required dopamine infusion. Biopsy of one of the thighs nodules was performed. A Sweet syndrome associated to a differentiation syndrome was suspected. All-trans retinoic acid therapy was discontinued and dexamethasone was administered. In 48 h the patient showed remission of the fever and the infiltrates and the skin lesions acquired a residual aspect. It is debatable whether these two syndromes are distinct entities with common mechanisms or whether they are poles of the same spectrum. Dermatologists and hematologists must be aware of these two syndromes and its pathophysiologic association.

Solano-López, Guillermo; Llamas-Velasco, Mar; Concha-Garzón, Maria José; Daudén, Esteban

2015-01-01

481

Sweet syndrome and differentiation syndrome in a patient with acute promyelocytic leukemia.  

PubMed

The differentiation syndrome is an inflammatory reaction with increased capillary permeability that occurs in up to 25% of patients with acute promyelocytic leukemia treated with all-trans retinoic acid. A 50-year-old man with acute promyelocytic leukemia underwent chemotherapy with idarubicin and all-trans retinoic acid. On day +21 the patient developed pruritic prepatelar papules as well as several 10 mm subcutaneous nodules in both thighs accompanied by persistent fever. On the day +25 the patient presented with bilateral pulmonary crackles, infiltrates in the right lower lobe and severe hypotension which required dopamine infusion. Biopsy of one of the thighs nodules was performed. A Sweet syndrome associated to a differentiation syndrome was suspected. All-trans retinoic acid therapy was discontinued and dexamethasone was administered. In 48 h the patient showed remission of the fever and the infiltrates and the skin lesions acquired