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Biomedical subjects

B Pelosse

Publications and source records attributed to B Pelosse.

At least 19 recordsLinked to original sources
PubMed2006

[Kikuchi's disease and ocular manifestation].

PURPOSE: Report a case of oculomotor palsy with papillary edema in a child showing signs of Kikuchi's disease and review the literature. MATERIALS AND METHODS: A 10-year-old girl presented with diplopia with right ptosis, right exophthalmia, headache, generalized weakness, and fever. The assessment consisted of an ocular and neurological examination, imagery by cerebral magnetic resonance, a biological and immunological assessment, and a biopsy. RESULTS: The ophthalmologic examination found right exophthalmia with absence of right abduction and adduction, and right ptosis. The ocular fundus showed a bilateral papillary edema. Magnetic resonance revealed a bilateral intraconic attack of the orbital apex. The biopsy of cervical adenopathy showed a histiocytic necrotizing lymphadenitis. Infection assessment was negative and immunologic tests were normal. DISCUSSION: Kikuchi's disease, or histiocytic necrotizing lymphadenitis, is a rare disease in young patients that is characterized by adenopathy, fever, and neutropenia. The diagnosis was confirmed histologically. The etiology of Kikuchi's disease remains unknown, although a viral or autoimmune hypothesis has been suggested. The course of the disease is spontaneously favorable and recurrence is rare. Association with systemic lupus erythematous had been described. The differential diagnosis of Kikuchi's disease includes infectious necrotizing lymphadenitis, systemic lupus erythematous, and lymphoma. Ocular manifestation is exceptionally described (uveitis). CONCLUSION: Kikuchi's disease should be considered in children revealing ocular manifestations with lymphadenopathy and fever of unknown origin.

Child↗
PubMed2005

RPGR is mutated in patients with a complex X linked phenotype combining primary ciliary dyskinesia and retinitis pigmentosa.

INTRODUCTION: Primary ciliary dyskinesia (PCD) is a rare disease classically transmitted as an autosomal recessive trait and characterised by recurrent airway infections due to abnormal ciliary structure and function. To date, only two autosomal genes, DNAI1 and DNAH5 encoding axonemal dynein chains, have been shown to cause PCD with defective outer dynein arms. Here, we investigated one non-consanguineous family in which a woman with retinitis pigmentosa (RP) gave birth to two boys with a complex phenotype combining PCD, discovered in early childhood and characterised by partial dynein arm defects, and RP that occurred secondarily. The family history prompted us to search for an X linked gene that could account for both conditions. RESULTS: We found perfect segregation of the disease phenotype with RP3 associated markers (Xp21.1). Analysis of the retinitis pigmentosa GTPase regulator gene (RPGR) located at this locus revealed a mutation (631_IVS6+9del) in the two boys and their mother. As shown by study of RPGR transcripts expressed in nasal epithelial cells, this intragenic deletion, which leads to activation of a cryptic donor splice site, predicts a severely truncated protein. CONCLUSION: These data provide the first clear demonstration of X linked transmission of PCD. This unusual mode of inheritance of PCD in patients with particular phenotypic features (that is, partial dynein arm defects and association with RP), which should modify the current management of families affected by PCD or RP, unveils the importance of RPGR in the proper development of both respiratory ciliary structures and connecting cilia of photoreceptors.

Adolescent↗
PubMed2004

[Management of acute visual loss in children].

Acute or rapidly progressive visual loss in children needs urgent attention and treatment. It may be unilateral orbilateral. Etiology depends upon the involved areas: eye ball, optic nerve, retro-chiasmatic pathways. Psychogenic origin is quite common in school-age children, however, it has to be considered last. Unilateral visual loss may be overlooked. Acute total transitory visual loss may be due to epilepsy or to migraine. Rapidly progressive visual loss may be due to retinal disease, optic neuritis or cortical blindness. Management of visual loss depends on clinical features, associated symptoms, and aspect of the optic disc. It needs collaboration between ophthalmologist,pediatrician and neuropediatrician. Retinal hemorrhages first call to mind a traumatic origin. Swelling of the optic disc may be due to increased intracranial pressure or due to optic neuritis. When the optic disc is normal it is necessary to rule out organic diseases before establishing the diagnosis of a psychogenic vision disturbance. In emergency, brain neuroimaging is the best way to diagnose intracranial mass and visualize optic pathways.

Acute Disease↗
PubMed2004

[Amblyopia and peri-ocular capillary hemangioma of infancy: screening and clinical course before and after surgery].

PURPOSE: The analysis of visual complications provoked by infantile periocular hemangiomas and the understanding of their evolution before and after surgery. MATERIAL: and methods: A retrospective study including 30 children (31 eyes) who were operated for a periocular hemangioma with a surgical technique with the aid of ultrasonic scalpel Cavitron. Ophthalmological evaluation before and after surgery included: appreciation of the palpebral occlusion, strabismus, ocular motility, objective refraction and amblyopia. RESULTS: Surgical operation was performed within an average age of 9.5 months. The excision of periocular hemangiomas was effective on the release of the visual axis and on the amblyopia: 51.7% (16 eyes) of palpebral occlusion before surgery against 6.4% (2 eyes) after surgery and 77.4% (24 eyes) of amblyopia before surgery against 38.7% (12 eyes) after surgery. The anisometropia decreased after surgery. The ocular motility and the strabismus also improved. CONCLUSION: Surgical excision of periocular hemangiomas with the aid of ultrasonic scalpel is an effective technique presenting few complications. This surgery can be suggested early in infancy. It has a very good result in the release of the visual axis and the astigmatism which aided the reeducation of the amblyopia.

Amblyopia↗
PubMed2001

[The Wiedemann-Beckwith syndrome and a congenital cataract].

Wiedemann-Beckwith syndrome is a congenital syndrome with characteristic abnormalities: omphalocele, macroglossia, neonatal gigantism, visceromegaly, hemihypertrophy and a predisposition to embryonic tumors. Ophthalmologic abnormalities have not been described with Wiedemann-Beckwith syndrome. The authors report one case of Wiedemann-Beckwith syndrome associated with bilateral congenital cataract. Family studies indicate linkage of the Wiedemann-Beckwith syndrome locus to the marker 11p15,5. The genetics of cataract is heterogenic. Several mutations responsible for congenital cataract have been described. The association of the Wiedemann-Beckwith syndrome and cataract may contribute to the understanding of the genetics of congenital cataract.

Beckwith-Wiedemann Syndrome↗
PubMed2001

[Study of visual field and vigabatrin treatment in children].

INTRODUCTION: Concentric visual field defects have been described in association with vigabatrin, a GABA mimetic antiepileptic agent. Few cases have been reported in children. METHODS: A systematic ophthalmological examination was performed in 14 children treated with vigabatrin for seizures. A manual kinetic perimetry test (Goldmann) was done in 11 cases. The ERG was recorded in the 3 cases where perimetry could not be done. RESULTS: All children were asymptomatic. The mean age was 9.6 years. The mean duration of vigabatrin treatment was 41 months. The visual field was abnormal when central and peripheral fields were constricted. A visual field defect was discovered in 6 cases: 4 were severe, 2 were mild. When vigabatrin treatment was stopped, 1 case became worse, 1 case was slightly better, and 1 case remained stationary. A disturbed ERG was found in 3 children (depressed b-wave, raised a/b ratio). CONCLUSION: The visual field defects discovered in children treated with vigabatrin are similar to those described in adults. The incidence and progression of visual field constriction in children with and after vigabatrin treatment are not yet well known. Children treated with vigabatrin should therefore have systematic and regular ophthalmological perimetry, and ERG examinations.

Adolescent↗
PubMed2000

[Congenital corneal anesthesia in children: diagnostic and therapeutic problems].

Congenital corneal anesthesia (CCA) is an uncommon condition difficult to diagnose. We report the case of a 9-month-old girl who presented bilateral congenital corneal anesthesia. The child had a corneal ulcer which had been unresponsive to adapted local treatment. Self-inflicted corneal injuries were present. Local treatment and arm splints led to quick healing. CCA occurs either alone or in association with neurological diseases (familial dysautonomia) or systemic congenital abnormalities (Goldenhar Gorlin syndrome). It is important to search for corneal anesthesia in children with chronic ulcerations of the cornea and self-inflicted injuries. Early diagnosis is important due to the risk for the visual prognosis. Short and longterm prevention of self-inflicted corneal injuries should be associated with a local treatment to assure rapid healing and a relapse free outcome.

Corneal Diseases↗
PubMed1997

Cerebral infarction associated with vasculitis due to varicella zoster virus in patients infected with the human immunodeficiency virus.

Cases of herpes zoster ophtalmicus (HZO) with delayed contralateral hemiparesis caused by hemispheric stroke secondary to granulomatous angiitis have been reported and are a well-recognized complication of herpes zoster. Similar cases have been reported more recently during infection with human immunodeficiency virus (HIV). We describe two HIV+ patients without any clinical history of zoster dermatitis who developed a sudden hemiparesis followed 2 weeks later for one by an acute retinal necrosis. Computerized tomography (CT) scan, magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), and digital subtraction angiography (DSA) were performed and showed a hemispheric stroke with evidence of a segmental arteritis of the carotid syphon. Varicella zoster virus (VZV) was found in the cerebro spinal fluid (CSF) in the two patients and after puncture of the vitreous fluid of the patient with the acute retinal necrosis. These two cases exemplify the difficulty of diagnosis of stroke in HIV+ patients, which seems to be more frequent than in similarly aged non-infected patients and demonstrates that VZV needs to be taken in consideration and identified even without any past history of zoster dermatitis.

AIDS-Related Opportunistic Infections↗
PubMed1990

[Ocular toxoplasmosis in AIDS].

Since 1983, we have seen 11 cases of toxoplasmic retinochoroiditis in patients with AIDS. Ocular toxoplasmosis appears to be a growing concern in AIDS: 3.3% in 1983, 6.1% in 1988 and 5.9% during the first trimester of 1989. Anti-Toxoplasma gondii antibody titers in the aqueous humour were not conclusive, therefore the diagnosis was based on ophthalmoscopic criteria and on a rapid response to specific therapy. As for brain toxoplasmosis, treatment must be continued indefinitely to prevent relapse. Seven out of 11 patients had cerebral toxoplasmosis, which was diagnosed by computerized tomography (CT) scan. This suggests that a neurological evaluation and a brain CT scan should be performed in all AIDS patients with toxoplasmic retinochoroiditis.

Acquired Immunodeficiency Syndrome↗
PubMed1990

[Bouchut's tubercles. A clinical and angiographic study].

Four patients (3 young adults and 1 child) with disseminated tuberculosis had choroidal tubercles; none of them was immunodepressed. The clinical and angiographic aspects of these nodules and their evolution are described. Their clinical interest is discussed: fundus examination is always indicated in cases of disseminated tuberculosis and, more generally, in cases of systemic granulomatosis.

Adolescent↗
PubMed1989

[Long-term follow-up and etiologic factors in edematous forms of central retinal vein obstruction].

We report a study of 61 cases of retinal vein occlusion, followed for 3-10 years. The oedematous form only were studied; 39 were branch vein occlusion (BVO) and 22 central retinal vein occlusion (CRVO). Of the CRVO's 5 were female, 17 were male, ages ranged from 5 to 75 years, averaging 58 years of the BVO's there were 19 females and 20 males with ages ranging from 27 to 79 years, averaging 61 years. High blood pressure and atherosclerosis were the most frequent causes. High intraocular pressures were mostly associated with CRVO (25%). Study of serum lipids and blood coagulation are most useful in CRVO in young patients. Platelet agreggation anomalies, protein C deficiency were seen in some cases. Visual prognosis is relatively good; bilateralisation was seen in 5% of BVO and in 9% of CRVO. Visual acuity better than 20/200 was observed in 87% of BVO and in 75% of CRVO.

Adult↗
PubMed1989

[Homonymous lateral quadranopsia disclosing cerebral toxoplasmosis in a patient with AIDS].

A case of Homonymous lateral quadranopsia revealing cerebral toxoplasmosis is reported in AIDS patient. Neuro-ophthalmologic manifestations must be explored by neuro-radiologic examination. Nuclear magnetic resonance would rather be performed than CT scan. Frequencies of cerebral and ocular toxoplasmosis localisations are compared. Severity of toxoplasmosis localisation justifies an early diagnosis based on immunological assay detecting the infection. Prophylactic treatment has to be instored.

Acquired Immunodeficiency Syndrome↗
PubMed1988

[Natural history of malignant melanoma of the choroid].

Study of five patients with uveal melanomas, followed 3 to 11 years without treatment. In all cases the tumor progressed, involving orbital tissue in 2 cases. One patient died of metastasis after 4 years of evolution. The authors emphasize the variability of the growth rate in individual cases which explains the various clinical aspects. Metastasis can be seen in treated or untreated melanomas, often after a prolonged period. In case four, pulmonary metastasis was documented 20 years after enucleation with a very low growth rate. Natural history of uveal melanoma shows a low but regular growth which ends in local and general extension.

Aged↗