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S Defoort-Dhellemmes

Publications and source records attributed to S Defoort-Dhellemmes.

17 recordsLinked to original sources
PubMed2006

Unusual ocular motor findings in multiple sclerosis.

In multiple sclerosis (MS), nystagmus or internuclear ophthalmoplegia (INO) are the usual ocular motor dysfunctions. However, in patients with focal brainstem lesions, other rare manifestations may be observed, such as an isolated ocular motor nerve palsy or complex ocular motor disturbances. We report five MS patients with unusual ocular motor disturbances (bilateral third nerve palsy [n = 2], opsoclonus, Horner's syndrome and one-and-a-half syndrome). We discuss possible correlations between clinical disturbances and MRI abnormalities. Patients were seen in two MS centres. They had a confirmed diagnosis of MS and underwent a brain MRI and a complete neuro-ophthalmological work-up. In one case (opsoclonus), ocular motor manifestations were the first manifestation of MS. In the other four cases they occurred 3 months (Horner syndrome), 6 years and 12 years (bilateral third nerve palsy) and 2 years (one-and-a-half syndrome) after the disease onset, respectively. Four out of five patients were still in a relapsing-remitting form of MS. In the opsoclonus case, there was no evidence of a brainstem lesion. A gadolinium-enhanced lesion (2 cases) or a new T2-weighted lesion located in the brainstem correlated with the clinical presentation. All patients completely or partially recovered after corticosteroid infusions. Our study shows some rare or previously undescribed complex ocular motor symptoms in MS.

Adjuvants, Immunologic↗
PubMed2005

ERG and anatomical abnormalities suggesting retinopathy in dementia with Lewy bodies.

The authors used flash electroretinography to demonstrate dysfunction of the photopic and scotopic retina in patients with dementia with Lewy bodies and visual hallucinations (VHs) compared with patients with Parkinson disease, patients without VHs, and controls. The retinal dysfunction may be related to slight alteration of the photoreceptors and numerous pale inclusions in the outer plexiform layer found at the post mortem examination, suggesting a specific retinopathy.

Aged↗
PubMed2005

[Bilateral third cranial nerve palsy triggered by an acute relapse of multiple sclerosis].

INTRODUCTION: We report a case of bilateral third cranial nerve palsy in a patient with a secondary progressive multiple sclerosis. OBSERVATION: MRI revealed a large hyperintense lesion in T2-weighted images in the mesencephalic area. The clinical and radiological outcome was good after intravenous methylprednisolone. The oculomotor signs were probably caused by this mesencephalic lesion. CONCLUSION: This case of bilateral third cranial nerve palsy is, to our knowledge, the first one occurring in a patient with multiple sclerosis.

Anti-Inflammatory Agents↗
PubMed2005

[Tonic pupils in Sjögren's syndrome].

INTRODUCTION: Adie's syndrome is usually a disease of unknown origin. We report two cases secondary to Sjögren syndrome. CASE REPORTS: A 26-year-old man developed in few months a sensitive neuropathy with a bilateral tonic pupil. A 50-year-old woman complained of sensitive signs probably related to a ganglionopathy and dysautonomic disorders affecting sudomotor and vasomotor functions. Adie syndrome had been diagnosed three years earlier. In both patients, the systemic signs and the results of the complementary tests led to the diagnosis of Sjögren's syndrome. Corticosteroids had limited effects on the sensitive signs and no influence on the tonic pupils. CONCLUSION: Adie syndrome, isolated or accompanied by other dysautonomic disorders, may reveal or precede the diagnosis of Sjögren's syndrome.

Adult↗
PubMed2005

[Quadruple sectoranopia].

Quadruple sectoranopia is a rare campimetric syndrome involving upper and lower, homonymous, congruent field blind sectors sparing a horizontal zone. Ischemia or infarction of the lateral parts of the lateral geniculate body, supplied by the distal part of the anterior choroidal artery, accounts for the visual field defect. Ganglionic nerve fiber atrophy matched to the visual field defect may be found if the lateral geniculate body dysfunction involves infarction. The four cases reported so far involve the following etiologies: a case of surgical ligation of the distal part of the anterior choroidal artery during cerebral meningioma removal, two cases of stroke with anterior choroidal artery infarction, and a case of vascular steal with anterior choroidal artery blood flow being shunted away from the lateral geniculate body by an arteriovenous malformation. If lateral geniculate body infarction is not solely involved, partial recovery may occur, ischemic quiescent neuronal areas being able to resume their activity following ischemia resolution.

Geniculate Bodies↗
PubMed2004

[Pure subacute pandysautonomia: an assessment of treatment with intravenous polyvalent immunoglobulins].

INTRODUCTION: Acute or sub-acute pure dysautonomia is uncommon. We report a case of sub-acute pure pandysautonomia with favorable outcome after intravenous immunoglobulin therapy. CASE REPORT: A 29-year-old right-handed student, with an uneventful medical history presented, for one month, bilateral loss of visual acuity and digestive disorders, associating diarrhea, vomiting and anorexia. Physical examination revealed bilateral intrinsec oculomotor nerve palsy, a dryness syndrome and severe orthostatic hypotension. Ophthalmologic examination showed bilateral diffuse parasympathic impairment associating an Argyll Robertson pupil and full pupil light reflex abolition. Elevated protein level (0.93g/l) was the only cerebrospinal fluid anomaly. Serum tests were negative for anti-gangliosides antibodies. The patient improved slowly after two series of intravenous immunoglobulin infusions. CONCLUSION: Clinical course and laboratory findings suggest that acute or sub-acute pure pandysautonomia events are likely to be related to acute polyradiculoneuritis. Therefore intravenous polyvalent immunoglobulin infusions should be attempted, even if their efficacy needs to be confirmed.

Acute Disease↗
PubMed2004

[Congenital achromatopsia: electroretinogram in early diagnosis].

PURPOSE: Achromatopsia is a hereditary disease responsible for congenital low vision. Patients present with nystagmus, abnormal visual behavior or photophobia. Only the electroretinogram (ERG) can confirm the diagnosis in infants. PATIENTS AND METHODS: Thirty children referred for nystagmus or low vision were included in this retrospective study. A complete ophthalmological examination, an ERG and when possible a color vision test (Ishihara, Farnsworth 15 Hue test) was done. A Ganzfeld ERG was performed in accordance with ISCEV standards in patients more than 6 years of age. In younger patients, a simplified method using electroluminescent diode stimulation was used and a comparative ERG in accordance with ISCEV standards was performed when the patients were old enough. RESULTS: The ERG response was identical in children and adults. It confirmed the diagnosis of achromatopsia: the scotopic components obtained in dark adapted conditions were normal, (scotopic a-wave, b2 wave). The photopic components, recorded in light-adapted conditions, in order to inhibit the scotopic response (photopic wave, b1 wave), were not recordable. The color vision tests confirmed color blindness; however, in some patients color denomination was correct. CONCLUSION: The simplified ERG procedures performed in our series were reliable in detecting achromatopsia. However, it may not be sufficient to discriminate complete from incomplete achromatopsia.

Adolescent↗
PubMed2000

[Quantitive evaluation of metamorphopsia in macular disease].

INTRODUCTION: Visual acuity loss and scotoma are not the only functional consequence of macular disease. Frequently, patients also complain of metamorphopsia. Such visual distortion should be taken into account when evaluating the potential benefit of surgical procedures in macular disease. The Amsler grid does not allow any quantified analysis. In addition, a precise confrontation of the macular lesion with its functional consequences is not possible by this test. We present a new method for analyzing metamorphopsia in macular disease. METHODS: Opto-acustic modulation controlled laser emission allowed to generate a retinal image of 256 squares with an angular size of 1 degrees each. The subject was instructed to fixate the center of the grid and to push a handheld button every time he perceived abnormalities within the presented pattern. At the end of the procedure, the responses were represented on graph superimposed on the fundus image. 15 eyes of 15 successive patients complaining of metamorphopsia were included in this preliminary study. Informed consent was obtained prior to inclusion. All patients reported abnormalities on standard Amsler testing. The test-retest reliability was evaluated by repeating the same procedure between 2 hours and 7 days after the first procedure. RESULTS: The evaluation of 3 patients did not provide reliable information on metamorphopsia because of instable fixation. In 12 patients, a coherent response was obtained. In 9 eyes, the result was unchanged when repeating the procedure, concerning both the number of abnormal elements and their spatial distribution. CONCLUSION: The preliminary results appear to be encouraging, indicating a good reproducibility of the results of this method. They should be confirmed on a larger scale. Further work is necessary to evaluate the interest of this method in assessing functional results of macular surgery.

Adult↗
PubMed1999

Outer retinal dysfunction in patients treated with vigabatrin.

OBJECTIVE: To assess early visual impairment related to vigabatrin prospectively in patients with and without visual symptoms. BACKGROUND: Vigabatrin acts as an inhibitor of gamma-aminobutyric acid (GABA) transaminase. GABA-induced ion transport changes in the retinal pigment epithelium have been described. The electro-oculogram (EOG) is a clinical test that reflects photoreceptor and pigment epithelium function. PATIENTS AND METHODS: Of the 22 consecutive patients presenting with a history of partial seizures currently treated with vigabatrin, 20 were included in the study. A complete clinical ophthalmologic and neurologic examination was performed, including static 100-point perimetry, EOG, and electroretinogram (ERG). RESULTS: In 14 of 20 patients, the light/dark ratio (Arden ratio) of the standard EOG was reduced in at least one eye. The a- and b-wave amplitudes and implicit time of the ERG were within the normal range in all patients; however, ERG oscillatory potentials could not be recorded in 10 patients. Twelve patients had visual field constriction; five complained of visual symptoms. The most severe visual impairment was observed in patients treated with both vigabatrin and valproate. CONCLUSIONS: There is some evidence of outer retinal dysfunction in the patients treated with vigabatrin. EOG, a more sensitive diagnostic tool than ERG for screening vigabatrin-treated patients, also appears to be more specific.

Adolescent↗
PubMed1998

[Retinal pigment anomalies associated with Fabry's disease].

INTRODUCTION: Fabry's disease is an X linked disease with ocular, skin and kidney lesions. We report a case which presented all the ophthalmologic signs and particular retinal manifestations. CASE REPORT: Fabry's disease was suspected in a 18 year old young man, with angiokeratomes, attacks of pain located in fingers, toes and also in the abdomen. Typical ocular morphological findings, namely whorl-like corneal opacities, posterior spoke-like cataracts and tortuosity of conjunctival vessels were found. His mother had the same ocular signs. The patient was also examined with fluorescein fundus angiography. DISCUSSION: The diagnostic value of the ocular abnormalities can be significant and are very indicative of the disease in hemizygous and heterozygous patients. Except the retinal vascular tortuosity, we describe peripheric abnormalities of the retinal pigment epithelium suggesting another disease finding.

Adolescent↗
PubMed1997

[Eye disorders in children and adolescents].

PURPOSE: We tried to better characterize non organic ocular disorders in children and adolescents: particularly clinical, perimetric and electrophysiological features. METHODS: We performed a prospective study in 25 cases during a period of 16 months. RESULTS: Non organic ocular disorders were seen among young girls between 9 and 11 years, mostly with bilateral, symmetrical and rapid visual loss. The symptoms were variable in time and responded favorably to suggestion. The ocular examination was normal. Visual field was abnormal in 52% of cases and very often showed a spiral (or snail) picture or a tubular constriction. 7' pattern visual evoked potentials were present in 88% of cases. The acuity was 20/20 in 92% of cases after the consultation and 100% later. CONCLUSION: The ophthalmic and electrophysiologic examination confirmed our diagnosis and eliminated more severe pathology.

Adolescent↗
PubMed1997

[Management of congenital microphthalmos and anophthalmos].

PURPOSE: To better characterize congenital anophthalmos and microphthalmos in order to distinguish which patients need surgical treatment. MATERIALS AND METHODS: A retrospective study of 42 cases with congenital anophthalmos and microphthalmos over a 16 years period was performed. Seven anophthalmos, 20 microphthalmic globes with no associated colobomatous orbital cyst and 15 microphthalmic globes associated with colobomatous orbital cyst were observed. Complete history, pediatrical and ophthalmological examination, electrophysiological feature, oculo-cerebral imagery and karyotype on each of the patients were reviewed. RESULTS: Among all patients, lack of development of the lids was observed in 45% of cases. In our group of anophthalmos, 100% had micro-orbit. In our group of microphthalmic globes with no associated colobomatous orbital cyst, 30% had micro-orbit and in our group of microphthalmic globes associated with colobomatous orbital cyst, 6% had micro-orbit. 75% of patients had ocular anomalies and 39% had systemic anomalies, mostly on the face. Aetiology were found in 36% of cases. Visual evoked potentials and retinal electric feature were useful to better determine visual function. CONCLUSION: Expandable orbital prosthesis would appear to be the most effective therapy for certain cases of anophthalmos and microphthalmos with micro-orbit.

Abnormalities, Multiple↗
PubMed1991

[Thalamocapsular metastasis of muco-epidermoid adenocarcinoma of the parotid gland].

A 58 years woman with muco-epidermitis carcinoma of the left parotid gland treated by parotidectomy and external radiation developed seven years later a left hemianopsia and moria related to thalamo-capsulo-lenticular lesions. Three stereotactic biopsies were performed. Neuropathological examination confirmed a secondary lesion of carcinoma with the same histological features than primary lesion of the parotid. Due to the metastasis location treatment consisted in external radiotherapy guided by stereotactic coordinates.

Adenocarcinoma↗
PubMed1989

[Jadassohn's sebaceous nevus].

We report the case of a child's Jadassohn sebaceous noevus, localised on the vertex cranii, the trunk and the limb, associated with the presence of ophthalmological and neurological abnormalities. The diagnosis of this syndrome has been difficult because all the signs were not present at birth. The surgical treatment of the vertex cranii lesions will be realised after expansion with sub cutaneous prothesis, allowing a large exeresis and an adequate reconstruction. Only the anatomo-pathological examination allows any malignant degeneration of the lesion.

Central Nervous System Diseases↗
PubMed1989

[Jaffe's syndrome and anomalies of capillary permeability].

The parallelism between ophthalmoscopy and angiography in the different grade classifications of idiopathic epiretinal membranes is not compulsory: spotty and irregular fluorescein leakage is apparent in many cases, even early. This leakage, who might bring biochemical factors stimulating cellular migration and proliferation could therefore be usually associated with more progression of the membrane.

Adult↗