WorldWideScience

Sample records for angiomatosis

  1. Bacillary angiomatosis.

    Science.gov (United States)

    Lange, Danica; Oeder, Caroline; Waltermann, Katharina; Mueller, Anke; Oehme, Albrecht; Rohrberg, Robert; Marsch, Wolfgang; Fischer, Matthias

    2009-09-01

    An infection with Bartonella henselae transmitted from domestic cats to humans by scratching normally leads to cat-scratch disease. When the human host has severe immunosuppression or HIV infection, the potentially life-threatening disease bacillary angiomatosis can develop. A 79-year-old man presented with livid-erythematous, angioma-like skin lesions. We considered a cutaneous infiltrate from his known chronic lymphocytic leukemia, Merkel cell carcinoma, cutaneous metastases of internal tumors, cutaneous sarcoidosis, mycobacterial infection and even atypical herpes simplex infection. The correct diagnosis was proven histologically and by PCR. Because of increasing numbers of immunosuppressed and HIV-positive patients, as well as an infection rate of 13% for B. henselae in domestic cats in Germany, one must be alert to the presence of bacillary angiomatosis. PMID:19298547

  2. Encephalotrigeminal angiomatosis

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    Kotyal B Mahendrappa

    2013-01-01

    Full Text Available Encephalotrigeminal angiomatosis, more commonly known as Sturge-Weber syndrome (SWS, is a rare disorder occurring sporadically with a frequency of 1:50,000. It is characterized by facial nevus, seizures, hemiparesis, intracranial calcification, and mental retardation. We report here a young infant presenting with port-wine stain of one side of the face and transient hemiparesis (stroke-like episode of the opposite side, seizures, and intracranial calcification, suggesting the diagnosis of SWS. The child reported here presented with a stroke-like event at a relatively young age, which is unusual. The baby was given supportive therapy.

  3. Bacillary angiomatosis in an immunosuppressed dog.

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    Yager, Julie A; Best, Susan J; Maggi, Ricardo G; Varanat, Mrudula; Znajda, Nadine; Breitschwerdt, Edward B

    2010-08-01

    A dog being treated with immunosuppressive doses of prednisone and azathioprine for pancytopenia of unknown origin, developed, over a 2-week period, multiple erythematous nodular lesions in the skin including footpads. Skin samples revealed lesions identical to those of human bacillary angiomatosis (BA). The nodules were composed of multifocal proliferations of capillaries, each lined by protuberant endothelial cells. The capillary clusters were separated by an oedematous connective tissue, lightly infiltrated with degenerate inflammatory cells, including neutrophils and macrophages. Tissue sections stained with Warthin-Starry silver stain revealed large numbers of positively stained bacilli in the stromal tissue, most heavily concentrated around the proliferating capillaries. Lesions of vascular degeneration and inflammation were evident. Bartonella vinsonii subsp. berkhoffii genotype 1 was independently amplified and sequenced from the blood and the skin tissue. The pathognomonic nature of the histological lesions, demonstration of compatible silver-stained bacilli in the tissue, and identification of B. vinsonii subsp. berkhoffii in the blood and tissue indicates that this is most likely the aetiologic agent responsible for the lesions. Antibiotic therapy was successful in resolving the nodules. It would appear that B. vinsonii subsp berkhoffii, like Bartonella henselae and Bartonella quintana, has the rare ability to induce angioproliferative lesions, most likely in association with immunosuppression. The demonstration of lesions identical to those of human BA in this dog is further evidence that the full range of clinical manifestations of human Bartonella infection occurs also in canines. PMID:20374571

  4. Cystic angiomatosis with splenic involvement: unusual MRI findings

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    Vanhoenacker, F.M. [Dept. of Radiology, Univ. Hospital Antwerp, Edegem (Belgium); Dept. of Radiology, AZ St-Maarten, Campus Duffel, Duffel (Belgium); Schepper, A.M. [Dept. of Radiology, Univ. Hospital Antwerp, Edegem (Belgium); Raeve, H. [Dept. of Pathology, Univ. Hospital Antwerp, Edegem (Belgium); Berneman, Z. [Dept. of Hematology, Univ. Hospital Antwerp, Edegem (Belgium)

    2003-12-01

    Cystic angiomatosis is a rare disorder with a poor prognosis. We describe a case of a 33-year-old woman who presented with longstanding bone pain, hemolytic anemia, and an enlarged spleen. Radiologically, multiple osseous lesions with a mixed pattern of lytic and sclerotic areas were seen within the shoulders, spine, and pelvis. On CT and MRI of the abdomen, the spleen was markedly enlarged, with internal hyperdense foci on non-contrast CT scan, corresponding to low signal intensity areas on all MR pulse sequences. After administration of contrast, a mottled enhancement pattern throughout the entire spleen was seen both on CT and MRI. Cystic angiomatosis was proven by histological analysis of a biopsy specimen of an involved vertebra and histopathological examination of the spleen after subsequent splenectomy. This is the first report of a patient with disseminated cystic angiomatosis with splenic involvement in which the MRI features differ from the previous reports. Instead of the usual pattern consisting of multiple well-defined cystic lesions, a diffuse involvement replacing the entire spleen, with heterogeneous signal intensities on T2-weighted images and heterogeneous enhancement pattern, was seen in our patient. (orig.)

  5. Cystic angiomatosis with splenic involvement: unusual MRI findings

    International Nuclear Information System (INIS)

    Cystic angiomatosis is a rare disorder with a poor prognosis. We describe a case of a 33-year-old woman who presented with longstanding bone pain, hemolytic anemia, and an enlarged spleen. Radiologically, multiple osseous lesions with a mixed pattern of lytic and sclerotic areas were seen within the shoulders, spine, and pelvis. On CT and MRI of the abdomen, the spleen was markedly enlarged, with internal hyperdense foci on non-contrast CT scan, corresponding to low signal intensity areas on all MR pulse sequences. After administration of contrast, a mottled enhancement pattern throughout the entire spleen was seen both on CT and MRI. Cystic angiomatosis was proven by histological analysis of a biopsy specimen of an involved vertebra and histopathological examination of the spleen after subsequent splenectomy. This is the first report of a patient with disseminated cystic angiomatosis with splenic involvement in which the MRI features differ from the previous reports. Instead of the usual pattern consisting of multiple well-defined cystic lesions, a diffuse involvement replacing the entire spleen, with heterogeneous signal intensities on T2-weighted images and heterogeneous enhancement pattern, was seen in our patient. (orig.)

  6. [Successful treatment of angiomatosis with thalidomide and interferon alpha. A description of five cases and overview of treatment of angiomatosis and proliferating hemangiomas].

    Science.gov (United States)

    Adam, Z; Pour, L; Krejcí, M; Pourová, E; Synek, O; Zahradová, L; Navrátil, M; Mechl, M; Nebeský, T; Neubauer, J; Feit, J; Vokurková, J; Král, Z; Bednarík, O; Slampa, P; Dolezalová, H; Hájek, R; Mayer, J

    2010-08-01

    Our paper describes 5 patients with a vascular malformation - angiomatosis. In the first patient, a young man, angiomatosis affected the stomach, intestine, the area of mesenterium and retroperitoneum as well as mediastinum. Angiomatous mass had invaded pelvic bones and vertebrae. Treatment was initiated with interferon alpha in a maximum tolerated dose of 3 million units 3 times a week. Because of low efficacy of interferon alpha, thalidomide was added at a dose of 100 mg per day. Bone pain disappeared following a few applications of zoledronate administered in regular monthly intervals. After 3 years of concomitant administration of interferon alpha and thalidomide, we changed the regimen due to adverse effects and are administering thalidomide and interferon alternatively in 4-monthly intervals. Treatment has resulted in 50% reduction, according to imaging, of angiomatous mass, reduced intensity of disseminated intravascular coagulation and disappearance of clinical signs. The second was a case of multiple angiomatosis affecting the intestine only (multiple intestinal angiodysplasias) where we used thalidomide monotherapy. This treatment reduced blood losses and haemoglobin concentrations rose to normal levels. This male patient had consumed 120 transfusion units per year before the initiation of thalidomide. The third case was a slowly progressing vascular malformation of the face. This vascular malformation troubled its sufferer by spontaneous shortening that could not be resolved surgically because of its fragility. Two years of combined treatment with interferon a 6 million unites 3 times a week and thalidomide 100 mg daily led to a reduction and flattening of the malformation, paling of its colour and ceasing of spontaneous bleeding. This development enabled minor surgery--partial excision of this large vascular malformation. Histology examination confirmed that there was no evidence of new capillary formation. Histological examination thus confirmed

  7. Unraveling mysteries associated with cat-scratch disease, bacillary angiomatosis, and related syndromes.

    OpenAIRE

    Regnery, R; Tappero, J.

    1995-01-01

    The search for the infectious agents responsible for cat-scratch disease, bacillary angiomatosis, and related syndromes has a long and often circuitous history. Recognition of the etiologic agents and a new understanding of the fundamental features of the epidemiology and natural history of modern day Bartonella (formerly Rochalimaea)-associated diseases culminate a multipartite story that combines clinical medicine, traditional microbiology, and novel technological approaches to solve a long...

  8. Leptomeningeal angiomatosis of the left occipital surface detected by CT scan

    International Nuclear Information System (INIS)

    A case of left occipital leptomeningeal angiomatosis was reported. The patient was a 12-year-old boy who had episodes of severe vascular type headache accompanied by transient right homonymous hemianopsia. CT scan showed localized superficial high density area in the left occipital pole. Remarkable enhancement of the lower and inner surface of the left occipital lobe was demonstrated. Angiography showed poor filling of the distal portion of the left posterior cerebral artery. Skull tomograms showed linear calcifications in the left occipital region. Brain scan showed increased RI uptake in the left occipital region. During operation, the surface of the left occipital lobe was covered by excessive, fine, vascular networks which extended over the arachnoid membrane. The abnormal vessels were cauterized by a CO2 laser as throughly as possible. The occipital pole, felt gritty. Histologically, the abnormal vessels had spread into the subarachnoid space and were predominantly veins with thin and enlarged walls. The abnormal vessels followed the leptomeninges in the sulci of the cerebral cortex. Underneath the abnormal vessels, in the external layers of the cerebral cortex, calcium deposits were scattered and gliosis and degeneration of the ganglion cells were observed. The lesion was comparable with leptomeningeal angiomatosis. Though the pathological findings of the specimen, CT findings, and brain scan findings were extremely similar to those of Sturge-Weber disease, in this case, the typical clinical and roentgenographic findings of Sturge-Weber disease were all absent. (author)

  9. Can retinal microtrauma by internal limiting membrane peeling cause retinal angiomatosis proliferans?

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    Pukhraj Rishi

    2011-01-01

    Full Text Available A 32-year-old male presented with decreased vision in right eye since 1 month following trauma with plastic ball. Best-corrected visual acuity (BCVA was 20/160 in right eye and 20/20 in left. Right eye examination revealed angle recession, choroidal rupture, and macular hole. He underwent vitrectomy, internal limiting membrane (ILM peeling, and 14% C3F8 gas injection. After 6 weeks, BCVA was 20/30; fundus showed macular hole closure. Six months after surgery, fundus revealed retinal vascular lesions suggestive of stage I RAP-like lesions; vision was maintained. Clinical findings were confirmed on Video ICGA, FFA, and OCT. The patient was periodically reviewed and lesions were nonprogressive until last follow-up, 13 months after surgery. It seems quite probable that ILM peeling may have caused retinal microtrauma leading to the formation of RAP-like lesions. What factors lead to such an event is as yet not clearly understood. Hence, larger studies with a longer follow-up are warranted to better understand these findings.

  10. Mediastinal nodal angiomatosis - an unusual mass lesion in the superior mediastinum

    International Nuclear Information System (INIS)

    Full text: A 54-year-old male, lifelong non-smoker, presented to the authors' hospital with a 5-day history of fever and productive cough, not improving on oral amoxicillin. His past medical history was unremarkable. On examina tion, he was febrile, tachycardic and tachypneic, and had right basal crackles on auscultation. Initial blood tests revealed a leucocytosis with increased neutrophils and normal lymphocytes. C-reactive protein was elevated at 312 mg/L 5). The coagulation profile was unremarkable. The initial chest X-ray (CXR) showed areas of opacifi cation in the right lower lobe and a right paratracheal mediastinal bulging (Fig, 1). A previous CXR from 6 years before, unrelated to this recent presentation, was normal. Further evaluation by contrast-enhanced chest CT (SIEMENS Sensation 16 (Siemens, Erlangen, Germany); slice thickness 3 mm) showed a well-defined heteroge neous 4.0 x 4.8 cm mass lesion in the superior mediast num with densities of 60-70 HU, separate from vessels, the esophagus or the trachea (Fig. 2a) along with bilateral lower lobe patchy confluent pulmonary infiltrates (not shown), more conspicuous on the right. The patient's hospital stay was complicated by a non-ST elevation myocardial infarction, which precluded him from having a transbronchial biopsy of the mediastinal mass. The patient's serum tested positive for mycoplasma pneumo niae antibodies with a titre of 640 40); the pneumonia was successfully treated with a course of oral doxycycline administered over 14 days, the symptoms fully resolving. He was eventually discharged, having recovered from the pneumonia and from the myocardial infarction. A subs quent CT scan performed 3 months later showed persis tence of the mediastinal mass with no significant changes in size or density (Fig. 2b); the bilateral pneumonic infil trates have fully resolved. The ensuing endobronchial ultrasound (EBUS)-guided needle aspiration with biopsy of the mediastinal mass produced a sample of macrophages and erythrocytes, but no malignant cells. Still without a clear diagnosis, MRI (SIEMENS Avanto, 1.5 T, MAGNEVIST contrast, 10 mL) was performed. The mass appeared as a hyper intense lesion on T2 weighted imaging (Fig. 3a) and as a solid well-defined heterogenous enhancing tumour with prolonged pooling of contrast material on dynamic T1 acquisition (Fig, 3b-e). Finally, the lesion was thoracoscopically excised, given the presence of an almost 5-cm mediastinal mass with vascular supply. Histology showed florid.

  11. Blue rubber bleb nevus syndrome: a report of one case associated with recurrent epistaxis

    Institute of Scientific and Technical Information of China (English)

    LIU Qiong; CHEN Yi-peng; LI You-ming

    2007-01-01

    @@ Blue rubber bleb nevus syndrome (BRBNS), or Bean's syndrome, is a rare angiomatosis characterized by distinctive cutaneous and gastrointestinal venous malformations that usually cause massive or occult gastrointestinal hemorrhage and iron deficiency anemia secondary to the bleeding episodes.

  12. Sturge-Weber syndrome

    Directory of Open Access Journals (Sweden)

    Natarajan Manivannan

    2012-01-01

    Full Text Available Encephalotrigeminal angiomatosis (Sturge-Weber syndrome is a rather uncommon congenital condition characterized by the combination of venous angioma of the leptomeninges over the cerebral cortex with ipsilateral angiomatous lesions of the face, and sometimes the skull, jaws, and oral soft tissues. A case of portwine stain with intraoral gingival hemangioma is presented. There were no other systemic manifestations. Patient reported with a complaint of localized tumor-like swelling in gums. Based on the presence of sharply demarcated vascular lesion unilaterally on the face and with ipsilateral intraoral vascular hyperplasia in the lip and gingiva, a variant of encephalotrigeminal angiomatosis was diagnosed. Ultrasound Doppler flowmetry was used to determine the blood flow. Dental management included plaque control instructions, scaling, root planning, and excision of the lesion done under general anesthesia. Close follow-up and meticulous plaque control have kept the oral condition under fairly good control.

  13. Medical imaging findings in Cobb syndrome: two case reports

    Institute of Scientific and Technical Information of China (English)

    WANG Guang-bin; XU Lei; ZHAO Bin; CAI Shi-feng; SHI Hao; LI Hui-hua; QU Lei

    2005-01-01

    @@ Cobb syndrome, also known as cutaneomeningospinal angiomatosis, is a rare clinical entity characterized by the combination of a vascular skin nevus and an angioma in the spinal canal at the same metamere.1 It was first described by Berenbruch in 1890 and did not receive recognition until Cobb's description2 in 1915. Only few documents about this disease had been reported.1 We present two cases here and review the other reported cases.

  14. Sturge-Weber syndrome: a case report

    OpenAIRE

    Luiz Felipe G. dos Santos; Joanna G. da Conceição; Thaís Pimentel de Sá Bahia; Vanessa de A. S. Silva; Maria Eliza Barbosa Ramos; Mônica Israel

    2011-01-01

    Introduction: The Sturge-Weber Syndrome, also known as encephalotrigeminal angiomatosis, is a rare vascular neurocutaneous alteration. The main clinical features of this syndrome are facial vascular cutaneous naevus, usually unilateral, which often follows the outline distribution of trigeminal nerve. Objective: To report a clinical case of Sturge-Weber Syndrome in a 29-yeral-old male patient who presented oral manifestations related to the syndrome. Case report and conclusion: The patient re...

  15. Does a Feline Leukemia Virus Infection Pave the Way for Bartonella henselae Infection in Cats? ▿

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    Buchmann, Alexandra U.; Kershaw, Olivia; Kempf, Volkhard A. J.; Gruber, Achim D.

    2010-01-01

    Domestic cats serve as the reservoir hosts of Bartonella henselae and may develop mild clinical symptoms or none after experimental infection. In humans, B. henselae infection can result in self-limiting cat scratch disease. However, immunocompromised patients may suffer from more-severe courses of infection or may even develop the potentially lethal disease bacillary angiomatosis. It was reasoned that cats with immunocompromising viral infections may react similarly to B. henselae infection....

  16. Divry-Van Bogaert syndrome in a female: relationship to Sneddon's syndrome and radiographic appearances

    International Nuclear Information System (INIS)

    A 28-year-old woman presented with generalised livedo reticularis, dementia, epilepsy, and pyramidal and extrapyramidal signs. Multiple focal infarcts were seen on MRI. Angiography demonstrated widespread cerebromeningeal angiomatosis with multiple small and medium size arterial occlusions. A lifelong personal and family history of mental handicap in the absence of anticardiolipin antibodies suggests Divry-Van Bogaert syndrome, not previously been reported in a female. Similarities to Sneddon's syndrome are discussed. (orig.)

  17. Pestilence, persistence and pathogenicity: infection strategies of Bartonella

    OpenAIRE

    Minnick, Michael F.; Battisti, James M.

    2009-01-01

    It has been nearly two decades since the discovery of Bartonella as an agent of bacillary angiomatosis in AIDS patients and persistent bacteremia and ‘nonculturable’ endocarditis in homeless people. Since that time, the number of Bartonella species identified has increased from one to 24, and 10 of these bacteria are associated with human disease. Although Bartonella is the only genus that infects human erythrocytes and triggers pathological angiogenesis in the vascular bed, the group remains...

  18. Sturge–Weber syndrome

    OpenAIRE

    Manivannan, Natarajan; Gokulanathan, Subramanium; Ahathya, Ramakrishnan Swamy; Gubernath,; Daniel, Rajkumar; Shanmugasundaram

    2012-01-01

    Encephalotrigeminal angiomatosis (Sturge–Weber syndrome) is a rather uncommon congenital condition characterized by the combination of venous angioma of the leptomeninges over the cerebral cortex with ipsilateral angiomatous lesions of the face, and sometimes the skull, jaws, and oral soft tissues. A case of portwine stain with intraoral gingival hemangioma is presented. There were no other systemic manifestations. Patient reported with a complaint of localized tumor-like swelling in gums. Ba...

  19. Sturge-Weber syndrome

    OpenAIRE

    Natarajan Manivannan; Subramanium Gokulanathan; Ramakrishnan Swamy Ahathya; Gubernath,; Rajkumar Daniel; Shanmugasundaram

    2012-01-01

    Encephalotrigeminal angiomatosis (Sturge-Weber syndrome) is a rather uncommon congenital condition characterized by the combination of venous angioma of the leptomeninges over the cerebral cortex with ipsilateral angiomatous lesions of the face, and sometimes the skull, jaws, and oral soft tissues. A case of portwine stain with intraoral gingival hemangioma is presented. There were no other systemic manifestations. Patient reported with a complaint of localized tumor-like swelling in gums. Ba...

  20. Anaesthetic Management of Cataract Surgery in a Patient with Sturge-Weber Syndrome

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    Vasantha Kumar

    2015-05-01

    Full Text Available Sturge-Weber Syndrome (SWS, also called as encephalo trigeminal angiomatosis, is a rare congenital syndrome, characterized by lepto meningeal haemangioma, a facial port-wine stains distributed over the trigeminal nerve area, (usually involving one side and glaucoma. During cataract surgery, there may be rupture of choroid haemangioma, leading to excessive bleeding, or of haemangioma involving the airway, leading to difficult mask ventilation, laryngoscopy and intubation. We discuss the anaesthetic management of the patient with SWS for cataract surgery.

  1. Prävalenz und molekulare Epidemiologie der Bartonella henselae-Infektion bei Katzen in Berlin

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    Klose, Alexander

    2010-01-01

    Bartonella henselae is a world-wide distributed bacterial pathogen, which was first described in the 1990s. Its best known manifestations are Cat Scratch disease and Bacillary angiomatosis. In this study we determined a prevalence of 9,8% in Berlin composed of two populations prevalences: 1% in the group of cats which lived mainly indoors and 18,7% in the group of stray cats. Riskfactors for Bacteremia in cats are flee-infestation and age. We could confirm the flee-infestation as a riskfactor...

  2. [Massive Gorham-Stout osteolysis of the temporal bone and the craniocervical transition].

    Science.gov (United States)

    Plontke, S; Koitschev, A; Ernemann, U; Pressler, H; Zimmermann, R; Plasswilm, L

    2002-04-01

    Massive osteolysis Gorham-Stout is a rare, benign but locally aggressive angiomatosis which results in destruction and resorption of bone. The etiology and pathogenesis are undefined. The occurrence of the disease in the skull base is uncommon. A 54-year-old female presented with isolated, one-sided surditas. Eight years before the patient underwent surgery and radiation therapy for treatment of hypopharyngeal cancer. A transtemporal biopsy was taken and a highly vascularized, cystic lesion with destruction of the right occipital and temporal bone and the atlas was found. Histopathology showed thin-walled capillaries with flattened endothelial lining cells. After exclusion of malignant and infectious components the diagnosis of Gorham's disease was established. Review of the literature suggests radiation therapy as the method of choice for stopping the disease's progress. The aim of this case report is to emphasize the Gorham-Stout-Syndrome as a rare differential diagnosis for skull base lesions. PMID:12063694

  3. Sturge-Weber syndrome: a case report

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    Luiz Felipe G. dos Santos

    2011-10-01

    Full Text Available Introduction: The Sturge-Weber Syndrome, also known as encephalotrigeminal angiomatosis, is a rare vascular neurocutaneous alteration. The main clinical features of this syndrome are facial vascular cutaneous naevus, usually unilateral, which often follows the outline distribution of trigeminal nerve. Objective: To report a clinical case of Sturge-Weber Syndrome in a 29-yeral-old male patient who presented oral manifestations related to the syndrome. Case report and conclusion: The patient reported that he had presented a cutaneous vascular nevus on the face during childhood as well as epileptic crisis episodes. However, he had no ophthalmic alterations. Sturge-Weber syndrome is a systemic condition commonly affecting oral cavity through vascular lesions, therefore, dentists’ knowledge is extremely important to provide an adequate dental treatment without complications.

  4. Hemodynamics in the Sturge-Weber syndrome utilizing stable Xe-CT

    International Nuclear Information System (INIS)

    In 4 Sturge-Weber-syndrome child patients with calcified lesions mainly in the occipital lobe, regional cerebral blood flow (rCBF) was determined with stable Xe-CT in the resting state and after iv injection of acetazolamide (AA) and megimide (M), with the purpose of examining factors affecting deterioration of neurologic manifestations and the influence of lobectomy. In the resting state, rCBF of the temporal and occipital areas was significantly lower on the affected than unaffected sides. The side-to-side asymmetry of rCBF in the affected and unaffected sides decreased from the frontal to temporal and occipital areas. It was indistinct after AA injection because cerebral vasoreactivity became higher on the affected side. A low rCBF in association with a high cerebral vasoreactivity on the lesion side suggested that the low rCBF matched the low cerebral metabolism of the brain area affected by leptomeningeal angiomatosis. However, cerebral vasoreactivity to AA depended on clinical presentations. Two patients presented with progressive mental retardation. The other two patients were in clinically stable condition. Cerebral vasoreactivity to AA in the former two cases was poorer on both the affected and unaffected sides than that in the latter two cases. M administration was associated with a significantly decreased rCBF in the area of leptomeningeal angiomatosis and cerebral calcification and with a significantly increased rCBF of the 'pericalcified' area. In one patient undergoing extended occipital lobectomy on the affected side, no decrease in rCBF was noted in the resting state in either affected or unaffected side, and cerebral vasoreactivity to AM on the unaffected side was apparently increased. Both circulatory disturbance and seizure appear to play a role in clinical presentations, as well as their deterioration in Sturge-Weber-Syndrome patients. (N.K.)

  5. Inter- and intraspecies identification of Bartonella (Rochalimaea) species.

    Science.gov (United States)

    Roux, V; Raoult, D

    1995-06-01

    Species of the genus Rochalimaea, recently renamed Bartonella, are of a growing medical interest. Bartonella quintana was reported as the cause of trench fever, endocarditis, and bacillary angiomatosis. B. henselae has been implicated in symptoms and infections of human immunodeficiency virus-infected patients, such as fever, endocarditis, and bacillary angiomatosis, and is involved in the etiology of cat scratch disease. Such a wide spectrum of infections makes it necessary to obtain an intraspecies identification tool in order to perform epidemiological studies. B. vinsonii, B. elizabethae, seven isolates of B. quintana, and four isolates of B. henselae were studied by pulsed-field gel electrophoresis (PFGE) after restriction with the infrequently cutting endonucleases NotI, EagI, and SmaI. Specific profiles were obtained for each of the four Bartonella species. Comparison of genomic fingerprints of isolates of the same species showed polymorphism in DNA restriction patterns, and a specific profile was obtained for each isolate. A phylogenetic analysis of the B. quintana isolates was obtained by using the Dice coefficient, UPGMA (unweighted pair-group method of arithmetic averages), and Package Philip programming. Amplification by PCR and subsequent sequencing using an automated laser fluorescent DNA sequencer (Pharmacia) was performed on the intergenic spacer region (ITS) between the 16 and 23S rRNA genes. It was found that each B. henselae isolate had a specific sequence, while the B. quintana isolates fell into only two groups. When endonuclease restriction analysis of the ITS PCR product was done, three enzymes, TaqI, HindIII, and HaeIII, allowed species identification of Bartonella spp. Restriction fragment length polymorphism after PCR amplification of the 16S-23S rRNA gene ITS may be useful for rapid species identification, and PFGE could be an efficient method for isolate identification. PMID:7650189

  6. Musculoskeletal disorders associated with HIV infection and AIDS. Part I: Infectious musculoskeletal conditions

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    Tehranzadeh, Jamshid [Department of Radiological Sciences, University of California, Irvine, Irvine, California (United States); Department of Radiological Sciences, Rt. 140, 101 The City Drive ZC 5005, CA 92868-3298, Orange (United States); Ter-Oganesyan, Ramon R. [College of Medicine, University of California, Irvine, Irvine, California (United States); Steinbach, Lynne S. [Department of Radiological Sciences, University of California, San Francisco, San Francisco, California (United States)

    2004-05-01

    The musculoskeletal system can be affected by a variety of abnormalities in association with human immunodeficiency virus (HIV) infection. Although not as common as complications involving other organ systems, such as the pulmonary and the central nervous systems, HIV-associated musculoskeletal disorders are sometimes the initial presentation of the viral illness. Knowledge of the existence and the characteristic appearance of the conditions affecting bone, joint, and muscle in HIV-infected patients is valuable to radiologists for diagnosis and to clinicians for detection and appropriate treatment. We reviewed recent literature to provide a comprehensive assessment of the HIV-associated musculoskeletal disorders, and present radiologic examples from our own collection. This article is divided into two parts. In the first part we review the infectious musculoskeletal disorders associated with HIV illness and AIDS, including cellulitis, abscesses, pyomyositis, septic bursitis, septic arthritis, osteomyelitis, and bacillary angiomatosis. We also present a comprehensive spectrum of mycobacterial infections, consisting of tuberculous spondylitis and spondylodiskitis, arthritis, osteomyelitis, and tenosynovitis, as well as infections caused by atypical mycobacteria. Part II of this review will concentrate on non-infectious musculoskeletal conditions, including rheumatic disorders and neoplasms. (orig.)

  7. Orofacial manifestation of Sturge-Weber syndrome: A case report with review

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    Mandeep Kaur

    2012-01-01

    Full Text Available Sturge-Weber syndrome (SWS, sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. It is one of the phakomatoses which is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma. It is characterized by proliferation of arteries of the brain, resulting in multiple angiomas that occur on the same side due to arteriovenous malformations. Normally, only one side of the head is affected and mainly results from the errors in both the mesodermal and ectodermal development. Here, we report a rare case of SWS having oral and facial manifestations, where an 8-year-old boy had actually come for treatment of swollen gums, and on examination, we found that he had typical signs and symptoms of this rare developmental anomaly. The main purpose of this publication is to make the clinician aware of the oral manifestations that are seen in the patients with SWS and also to lay stress on the importance of radiographs in the diagnosis of this syndrome.

  8. Imaging of Sturge-Weber syndrome. Cranial CT and MR findings

    International Nuclear Information System (INIS)

    Sturge-Weber syndrome (SWS) is a phakomatosis characterized by vascular nevus flammeus, leptomeningeal venous angiomatosis, seizures, dementia, hemiplegia, hemianopsia, and glaucoma. Various imaging findings (gyriform calcification, atrophy of the ipsilateral hemisphere, leptomeningeal enhancement, ipsilateral choroid plexus enlargement, thickened calvarium, enlargement of paranasal sinuses and mastoid air cells, enlargement of deep veins, and white matter change adjacent to leptomeningeal enhancement) are seen in SWS. We examined the efficacy of CT and MR imaging in making the diagnosis in 14 patients. All patients underwent CT and MRI, and 11 of 14 patients underwent contrast-enhanced MRI. The most specific finding was leptomeningeal enhancement. Gyriform calcification, atrophy of the ipsilateral hemisphere, and ipsilateral choroid plexus enlargement were seen at high frequencies. Thickened calvarium was more frequent in adult patients. Enlargement of paranasal sinuses and mastoid air cells, enlargement of deep veins, and white matter change adjacent to leptomeningeal enhancement were seen in some (3-5) patients. A combination of findings of plain CT and MRI (including postcontrast MRI and MR venography) are useful for diagnosing SWS. (author)

  9. Musculoskeletal disorders associated with HIV infection and AIDS. Part I: Infectious musculoskeletal conditions

    International Nuclear Information System (INIS)

    The musculoskeletal system can be affected by a variety of abnormalities in association with human immunodeficiency virus (HIV) infection. Although not as common as complications involving other organ systems, such as the pulmonary and the central nervous systems, HIV-associated musculoskeletal disorders are sometimes the initial presentation of the viral illness. Knowledge of the existence and the characteristic appearance of the conditions affecting bone, joint, and muscle in HIV-infected patients is valuable to radiologists for diagnosis and to clinicians for detection and appropriate treatment. We reviewed recent literature to provide a comprehensive assessment of the HIV-associated musculoskeletal disorders, and present radiologic examples from our own collection. This article is divided into two parts. In the first part we review the infectious musculoskeletal disorders associated with HIV illness and AIDS, including cellulitis, abscesses, pyomyositis, septic bursitis, septic arthritis, osteomyelitis, and bacillary angiomatosis. We also present a comprehensive spectrum of mycobacterial infections, consisting of tuberculous spondylitis and spondylodiskitis, arthritis, osteomyelitis, and tenosynovitis, as well as infections caused by atypical mycobacteria. Part II of this review will concentrate on non-infectious musculoskeletal conditions, including rheumatic disorders and neoplasms. (orig.)

  10. Adhesion and host cell modulation: critical pathogenicity determinants of Bartonella henselae

    Directory of Open Access Journals (Sweden)

    Kempf Volkhard AJ

    2011-04-01

    Full Text Available Abstract Bartonella henselae, the agent of cat scratch disease and the vasculoproliferative disorders bacillary angiomatosis and peliosis hepatis, contains to date two groups of described pathogenicity factors: adhesins and type IV secretion systems. Bartonella adhesin A (BadA, the Trw system and possibly filamentous hemagglutinin act as promiscous or specific adhesins, whereas the virulence locus (VirB/VirD4 type IV secretion system modulates a variety of host cell functions. BadA mediates bacterial adherence to endothelial cells and extracellular matrix proteins and triggers the induction of angiogenic gene programming. The VirB/VirD4 type IV secretion system is responsible for, e.g., inhibition of host cell apoptosis, bacterial persistence in erythrocytes, and endothelial sprouting. The Trw-conjugation system of Bartonella spp. mediates host-specific adherence to erythrocytes. Filamentous hemagglutinins represent additional potential pathogenicity factors which are not yet characterized. The exact molecular functions of these pathogenicity factors and their contribution to an orchestral interplay need to be analyzed to understand B. henselae pathogenicity in detail.

  11. Adhesins of Bartonella spp.

    Science.gov (United States)

    O'Rourke, Fiona; Schmidgen, Thomas; Kaiser, Patrick O; Linke, Dirk; Kempf, Volkhard A J

    2011-01-01

    Adhesion to host cells represents the first step in the infection process and one of the decisive features in the pathogenicity of Bartonella spp. B. henselae and B. quintana are considered to be the most important human pathogenic species, responsible for cat scratch disease, bacillary angiomatosis, trench fever and other diseases. The ability to cause vasculoproliferative disorders and intraerythrocytic bacteraemia are unique features of the genus Bartonella. Consequently, the interaction with endothelial cells and erythrocytes is a focus in Bartonella research. The genus harbours a variety of trimeric autotransporter adhesins (TAAs) such as the Bartonella adhesin A (BadA) of B. henselae and the variably expressed outer-membrane proteins (Vomps) of B. quintana, which display remarkable variations in length and modular construction. These adhesins mediate many of the biologically-important properties of Bartonella spp. such as adherence to endothelial cells and extracellular matrix proteins and induction of angiogenic gene programming. There is also significant evidence that the laterally acquired Trw-conjugation systems of Bartonella spp. mediate host-specific adherence to erythrocytes. Other potential adhesins are the filamentous haemagglutinins and several outer membrane proteins. The exact molecular functions of these adhesins and their interplay with other pathogenicity factors (e.g., the VirB/D4 type 4 secretion system) need to be analysed in detail to understand how these pathogens adapt to their mammalian hosts. PMID:21557057

  12. Adhesion and host cell modulation: critical pathogenicity determinants of Bartonella henselae.

    Science.gov (United States)

    Franz, Bettina; Kempf, Volkhard A J

    2011-01-01

    Bartonella henselae, the agent of cat scratch disease and the vasculoproliferative disorders bacillary angiomatosis and peliosis hepatis, contains to date two groups of described pathogenicity factors: adhesins and type IV secretion systems. Bartonella adhesin A (BadA), the Trw system and possibly filamentous hemagglutinin act as promiscous or specific adhesins, whereas the virulence locus (Vir)B/VirD4 type IV secretion system modulates a variety of host cell functions. BadA mediates bacterial adherence to endothelial cells and extracellular matrix proteins and triggers the induction of angiogenic gene programming. The VirB/VirD4 type IV secretion system is responsible for, e.g., inhibition of host cell apoptosis, bacterial persistence in erythrocytes, and endothelial sprouting. The Trw-conjugation system of Bartonella spp. mediates host-specific adherence to erythrocytes. Filamentous hemagglutinins represent additional potential pathogenicity factors which are not yet characterized. The exact molecular functions of these pathogenicity factors and their contribution to an orchestral interplay need to be analyzed to understand B. henselae pathogenicity in detail. PMID:21489243

  13. Whole-body magnetic resonance imaging: a useful additional sequence in paediatric imaging

    Energy Technology Data Exchange (ETDEWEB)

    Laffan, Eoghan E.; O' Connor, Rachael; Ryan, Stephanie P.; Donoghue, Veronica B. [Department of Radiology, Children' s University Hospital, Temple Street, 1, Dublin (Ireland)

    2004-06-01

    Background: Whole-body MR (WBMR) imaging allows the acquisition of images of the entire body in a matter of minutes. Its use has primarily been in the evaluation of possible metastases in the setting of a known primary tumour. Objective: To document the value of WBMR imaging in ten children in whom this was added as an additional sequence when the primary diagnosis had not yet been made. Materials and methods: Ten children, age range 4 months-15 years (mean 7 years 4 months) had WBMR imaging after initial MR showed an abnormality that raised the possibility of systemic disease. Initial scanning was of the brain (n=1), spine (n=2), retroperitoneum (n=4), hips (n=1), femur (n=1) and wrist (n=1). Results: Abnormalities were detected in eight patients. Two patients had acute lymphoblastic leukaemia, and another had an anaplastic lymphoma, unsuspected prior to the WBMR. Two patients had a previously undiagnosed neuroblastoma with bone marrow metastases. Two patients had Langerhans' cell histiocytosis. Another had multiple bone lesions due to cystic angiomatosis. Conclusions: WBMR imaging may be a useful additional sequence in children in whom a systemic and especially a bone marrow abnormality is suspected. (orig.)

  14. BASED TO CLINICAL CASE. VON HIPLEA-LINDAU SYNDROME

    Directory of Open Access Journals (Sweden)

    Brzeziński Piotr

    2011-01-01

    Full Text Available von Hippel-Lindau syndrome (VHL is a rare, genetic multi-system disorder characterized by the abnormal growth of tumors in certain parts of the body (angiomatosis. The tumors of the central nervous system (CNS are benign and are comprised of a nest of blood vessels and are called hemangioblastomas. Hemangioblastomas may develop in the brain, the retina of the eyes, and other areas of the nervous system. Other types of tumors develop in the adrenal glands, the kidneys, or the pancreas. Symptoms of VHL vary among patients and depend on the size and location of the tumors. Symptoms may include headaches, problems with balance and walking, dizziness, weakness of the limbs, vision problems, and high blood pressure. Cysts (fluid-filled sacs and/or tumors (benign or cancerous may develop around the hemangioblastomas and cause the symptoms listed above. Individuals with VHL are also at a higher risk than normal for certain types of cancer, especially kidney cancer. Based on the case of 30-year old patient with characteristics of von Hippel-Lindau syndrome as phakomatosis.

  15. The centenary of the discovery of trench fever, an emerging infectious disease of World War 1.

    Science.gov (United States)

    Anstead, Gregory M

    2016-08-01

    In 1915, a British medical officer on the Western Front reported on a soldier with relapsing fever, headache, dizziness, lumbago, and shin pain. Within months, additional cases were described, mostly in frontline troops, and the new disease was called trench fever. More than 1 million troops were infected with trench fever during World War 1, with each affected soldier unfit for duty for more than 60 days. Diagnosis was challenging, because there were no pathognomonic signs and symptoms and the causative organism could not be cultured. For 3 years, the transmission and cause of trench fever were hotly debated. In 1918, two commissions identified that the disease was louse-borne. The bacterium Rickettsia quintana was consistently found in the gut and faeces of lice that had fed on patients with trench fever and its causative role was accepted in the 1920s. The organism was cultured in the 1960s and reclassified as Bartonella quintana; it was also found to cause endocarditis, peliosis hepatis, and bacillary angiomatosis. Subsequently, B quintana infection has been identified in new populations in the Andes, in homeless people in urban areas, and in individuals with HIV. The story of trench fever shows how war can lead to the recrudescence of an infectious disease and how medicine approached an emerging infection a century ago. PMID:27375211

  16. Seroprevalence of Bartonella spp. infection in HIV patients in Catalonia, Spain

    Science.gov (United States)

    Pons, Immaculada; Sanfeliu, Isabel; Nogueras, María Mercedes; Sala, Montserrat; Cervantes, Manuel; Amengual, M José; Segura, Ferran

    2008-01-01

    Background Although the first clinical descriptions of Bartonella infection were associated with immunocompromised patient with bacillary angiomatosis, we currently know that this organism is directly involved in diseases affecting a large number of patients, regardless of their immune status. Cat scratch disease, hepatic peliosis, and some cases of bacteraemia and endocarditis, are directly caused by some species of the genus Bartonella. The purpose of this study was to determinate the prevalence of IgG antibodies against Bartonella henselae and B. quintana in HIV patients and to identify the epidemiological factors involved. Methods Serum samples were collected from HIV patients treated at Hospital de Sabadell. Antibodies to B. henselae and B. quintana from 340 patients were examined by indirect immunofluorescence assay (IFA). Significance levels for univariate statistical test were determined by the Mann-Whitney U test and χ2 test. Results Of 340 patients, 82 were women and 258 men, with a median age of 42.21 ± 10.35 years (range 16–86 years). Seventy-six (22.3%) patients reacted with one or more Bartonella antigens. Of all the factors concerning the seroprevalence rate being studied (age, sex, intravenous drugs use, alcohol consumption, CD4 levels, AIDS, HCV, HBV, residential area), only age was statistically significant. Conclusion A high percentage of HIV patients presents antibodies to Bartonella and is increasing with age. PMID:18452613

  17. Risk Factors for Bartonella species Infection in Blood Donors from Southeast Brazil.

    Science.gov (United States)

    Diniz, Pedro Paulo Vissotto de Paiva; Velho, Paulo Eduardo Neves Ferreira; Pitassi, Luiza Helena Urso; Drummond, Marina Rovani; Lania, Bruno Grosselli; Barjas-Castro, Maria Lourdes; Sowy, Stanley; Breitschwerdt, Edward B; Scorpio, Diana Gerardi

    2016-03-01

    Bacteria from the genus Bartonella are emerging blood-borne bacteria, capable of causing long-lasting infection in marine and terrestrial mammals, including humans. Bartonella are generally well adapted to their main host, causing persistent infection without clinical manifestation. However, these organisms may cause severe disease in natural or accidental hosts. In humans, Bartonella species have been detected from sick patients presented with diverse disease manifestations, including cat scratch disease, trench fever, bacillary angiomatosis, endocarditis, polyarthritis, or granulomatous inflammatory disease. However, with the advances in diagnostic methods, subclinical bloodstream infection in humans has been reported, with the potential for transmission through blood transfusion been recently investigated by our group. The objective of this study was to determine the risk factors associated with Bartonella species infection in asymptomatic blood donors presented at a major blood bank in Southeastern Brazil. Five hundred blood donors were randomly enrolled and tested for Bartonella species infection by specialized blood cultured coupled with high-sensitive PCR assays. Epidemiological questionnaires were designed to cover major potential risk factors, such as age, gender, ethnicity, contact with companion animals, livestock, or wild animals, bites from insects or animal, economical status, among other factors. Based on multivariate logistic regression, bloodstream infection with B. henselae or B. clarridgeiae was associated with cat contact (adjusted OR: 3.4, 95% CI: 1.1-9.6) or history of tick bite (adjusted OR: 3.7, 95% CI: 1.3-13.4). These risk factors should be considered during donor screening, as bacteremia by these Bartonella species may not be detected by traditional laboratory screening methods, and it may be transmitted by blood transfusion. PMID:26999057

  18. [Medullary venous malformation with azygos anterior cerebral artery aneurysm: a case report].

    Science.gov (United States)

    Harada, K; Kobayashi, S; Sigemori, M; Watanabe, M; Kuramoto, S

    1987-03-01

    A case of medullary venous malformation with azygos anterior cerebral artery aneurysm is reported, which was associated with the leptomenigeal angiomatosis on the cortex of the right frontal lobe. A 62-year-old female was admitted to the Kurume University Hospital on June 21, 1985, because of a convulsive seizure. On admission, neurological and general examinations, except for slightly liver function disorder, was no abnormality. Plain CT scan showed the cortical atrophy and two small high-density areas in the right frontal lobe. Enhanced CT scan showed a high-density area in the genu of the corpus callosum. MRI (magnetic resonance imaging), on T2 weighted image, showed a high signal intensity area in the right paraventricular deep white matter and the right frontal region. A right internal carotid angiogram in the venous phase showed numerous enlarged medullary veins in the deep frontal region, converging into a single large draining vein that empties into the basal vein of Rosenthal. An azygos anterior cerebral artery was visualized on the right and left carotid angiograms. The aneurysm was situated at the distal end of the azygos artery. Twenty eight days after admission, a right frontal cranioplastic craniotomy was performed. During operation, the surface of the right frontal lobe was covered by fine, vascular networks, which was reddish brown. A right frontal lobectomy, including venous malformation and vascular networks, was performed. The aneurysm was clipped via an interhemispheric approach. Histologically, the malformation vessels had spread into the subarachnoid space.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:3600991

  19. Pityriasis rosea, Gianotti-Crosti syndrome, asymmetric periflexural exanthem, papular-purpuric gloves and socks syndrome, eruptive pseudoangiomatosis, and eruptive hypomelanosis: do their epidemiological data substantiate infectious etiologies?

    Directory of Open Access Journals (Sweden)

    Antonio Chuh

    2016-03-01

    Full Text Available Many clinical and laboratory-based studies have been reported for skin rashes which may be due to viral infections, namely pityriasis rosea (PR, Gianotti-Crosti syndrome (GCS, asymmetric periflexural exanthem/unilateral laterothoracic exanthem (APE/ULE, papularpurpuric gloves and socks syndrome (PPGSS, and eruptive pseudo-angiomatosis (EP. Eruptive hypomelanosis (EH is a newly discovered paraviral rash. Novel tools are now available to investigate the epidemiology of these rashes. To retrieve epidemiological data of these exanthema and analyze whether such substantiates or refutes infectious etiologies. We searched for articles published over the last 60 years and indexed by PubMed database. We then analyzed them for universality, demography, concurrent patients, temporal and spatial-temporal clustering, mini-epidemics, epidemics, and other clinical and geographical associations. Based on our criteria, we selected 55, 60, 29, 36, 20, and 4 articles for PR, GCS, APE/ULE, PPGSS, EP, and EH respectively. Universality or multiple-continental reports are found for all exanthema except EH. The ages of patients are compatible with infectious causes for PR, GCS, APE/ULE, and EH. Concurrent patients are reported for all. Significant patient clustering is demonstrated for PR and GCS. Mini-epidemics and epidemics have been reported for GCS, EP, and EH. The current epidemiological data supports, to a moderate extent, that PR, GCS, and APE could be caused by infectious agents. Support for PPGSS is marginal. Epidemiological evidences for infectious origins for EP and EH are inadequate. There might be growing epidemiological evidence to substantiate or to refute our findings in the future.

  20. [Intraspinal hemangioblastomas].

    Science.gov (United States)

    Hurth, M

    1975-06-01

    138 cases of spinal haemangioblastomas have been reviewed (30 original cases). The first part of this study is devoted to a general survey concerning history, pathological anatomy, genetics and nosology of haemangioblastomas of the central nervous system. Microscopic anatomy of these tumors has been a much debated question and is responsible for the numerous denominations proposed: angiomatosis, capillary angiomas, angioreticulomas, haemangiomas, haemangioendotheliomas, haemangiopericytomas, angioblastic meningiomas; haemangioblastoma seems the most correct. Recent studies in optical and mainly electron microscopy do not allow exact typing of tumoral cells and histogenesis remains uncertain. It is generally accepted that the tumor arises in vascular nesenchyme as a result of dygenetic abnormality. This theory provides a rational explanation for visceral localizations and classification of Lindau disease among phakomatoses. An hereditary factor is present in 25 percent of cases, mainly in multifocal localizations. In the second part, interest is focused on spinal haemangioblastomas, 17 extradural, 121 subdural haemangioblastomas are studied and listed. Extradural tumors are always solitary, mostly in the dorsal area. Subdural tumors may be out of the cord (10 tumors), on the posterior roots (28 tumors), or within the cord. The lesions are situated mainly in the cervicodorsal and dorsolumbar regions. Intramedullary haemangioblastomas are situated in nearly all cases in the dorsal part of the cord behind the central canal. They are close to the dorsal surface of the cord (60 percent) or partly out of the cord (6 percent) or totally intramedullary (30 percent). Cysts close to the tumor or at a distance are present in nearly 50 percent of cases and their origin is still questionnable. Clinical patterns have no special peculiarities, syrinomyelic symptomatology occurs in only 25 percent of cases. Neuroradiology and mainly spinal angiography are a very important step

  1. Sturge-Weber综合征临床分析及影像诊断%Clinical and imaging features of Sturge-Weber syndrome

    Institute of Scientific and Technical Information of China (English)

    黄晓星; 王志龙; 肖学红; 杨昂

    2015-01-01

    目的:探讨 Sturge-Weber 综合征的临床特点及 CT、MRI 表现及诊断。方法归纳8例 Sturge-Weber 综合征患者的临床表现、影像学特点,进行回顾性分析,并复习相关文献。结果8例均有脑内软脑膜血管瘤,其中3例伴有颜面部血管瘤,均位于三叉神经眼支分布区(均累及单侧);7例表现为癫痫发作,其中5例伴有头痛,1例伴有头晕及一侧肢体乏力;1例表现为单纯反复头痛;均无智力低下或青光眼。影像学检查可清晰显示脑萎缩、脑回样钙化、软脑膜广泛强化伴静脉畸形。结论 Sturge-Weber综合征是一种少见疾病,根据典型临床特点及CT、MRI等影像学检查有助于确诊。%Objective To determine the clinical and imaging features of Sturge-Weber syndrome (SWS). Methods The clinical and imaging features of 8 patients with Sturge-Weber syndrome were reviewed. Results Leptomeningeal angiomatosis was found in all patients,accompanied by unilateral facial hemangioma (3) in the distribution of the ophthalmic division of the trigeminal nerve. Of 7 patients with epilepsy , 5 were accompanied by headache , 1 by dizziness and unilateral muscle weakness , 1 by recurring simple headache. There was no mental retardation or glaucoma in any of the patients...CT and MRI clearly showed brain atrophy, gyriform calcification, diffuse enhancement of pia mater accompanied by cerebral venous malformation. Conclusion Sturge-Weber syndrome has typical clinical and imaging features on CT and MRI for diagnosis.