[Susac syndrome: study of five cases]. / Syndrome de Susac: étude de cinq cas.

INTRODUCTION: Susac syndrome is a rare microangiopathy, responsible for small cerebral, retinal and cochlear infarcts. The classic clinical triad includes multiple neurologic signs (from headaches to coma), retinal branch occlusions and sensorineural hearing loss. METHODS: We report a series of five patients with Susac syndrome followed in our department from 1997 to 2007. RESULTS: There were four women and one man (mean age at onset: 35.2 years). Clinical symptoms at onset were neurological (n=1), ophthalmological (n=1), auditory (n=1) and clinical triad (n=2). Neurologic symptoms included encephalopathy (n=2), headache (n=5), transient ischemic attacks (n=1). Brain MRI showed T2 lesions in the white and grey matter, corpus callosum and gadolinium-enhanced punctiform lesions. Cerebrospinal fluid contained an elevated protein level in three cases. Immunologic treatments (steroids [n=4], cylophosphamid [n=3], intravenous immunoglobulins [n=5]) associated with aspirin and/or oral anticoagulants, despite early relapses (n=2), led to dramatic clinical improvement (n=5). CONCLUSION: Due to its polymorphism the SS is difficult to diagnose when the clinical triad is lacking. In the absence of clinical trial and consensus treatment is empiric and based on supposed pathogenesis.

[Bilateral papilledema: prospective study of fifty patients]. / OEdèmes papillaires bilatéraux : étude prospective de 50 patients.

In this prospective study, we report fifty consecutive cases of bilateral papilledema without neurosurgical or obvious ophthalmologic etiology, referred to our institution between January 2005 and March 2007. Lumbar puncture with opening CSF pressure measurement distinguished two groups of patients: Group 1 (n=39) with and Group 2 (n=11) without intracranial hypertension. In Group 1, 9/39 patients presented secondary intracranial hypertension mainly due to cerebral venous thrombosis. In 30 patients, after complete investigations, a diagnosis of idiopathic intracranial hypertension was made: as commonly reported, patients were predominantly overweight (96.7% with body mass index>25kg/m2) young (mean age=27.6 years) and women (96.7%). Eleven patients with intracranial hypertension had no headaches. In Group 2, the most common diagnosis was bilateral non-arteritic anterior ischemic optic neuropathy, but rare causes have been identified.

[Recognizing the pitfalls. Pseudoglaucomatous visual fields]. / Reconnaître les pièges. Les champs visuels pseudo-glaucomateux.

Pseudo-glaucomatous visual fields pose two major problems: diagnosing optic nerve compression; diagnosing congenital optic nerve pathology. Looking for the deviation of each point on the schema is not enough: every total deviation value must be compared on both sides of the vertical median. Often, optic disc examination and clinical context can rectify a diagnosis. However, since 2002, it has been clear that normal-tension glaucoma diagnosis requires magnetic resonance imaging (MRI) to rule out intracranial compressive lesions involving the anterior visual pathway (of course, before imaging, other types of glaucoma will be ruled out).

[Neuro-ophthalmologic initial presentation of sarcoidosis]. / Manifestations neuro-ophtalmologiques révélatrices d'une neuro-sarcoïdose.

PURPOSE: To describe different forms of neuro-ophthalmologic onset of sarcoidosis: clinical signs, means of diagnosis, treatment, and progression. PATIENTS AND METHODS: Retrospective study of 13 patients with neuro-ophthalmologic initial onset of sarcoidosis diagnosed in three departments between 1997 and 2003. RESULTS: There were ten women and three men, with a mean age of 36 years. Six patients suffered from diplopia. In three cases, the cavernous sinus was involved; the three other patients with diplopia had meningoradiculitis. Nine patients had infiltration of the anterior visual pathway: the optic nerve was involved in five cases, the chiasm in two cases, and two patients had papilledema. Two patients also had both symptoms. The dosage of the angiotensin-converting enzyme level was evaluated in 11 patients and was elevated in six cases. Nine patients underwent a lumbar puncture; the cerebrospinal fluid protein was high in seven cases. Chest radiography and CT were abnormal in nine cases of 11. Ten patients had histological proof of sarcoidosis; the three others had enough evidence to support this diagnosis. All of them were treated with systemic corticosteroids. The diplopia improved for the six patients. Among the seven patients with optic nerve or chiasmal infiltration, one recovered completely, two were partially improved, and four remained stable. CONCLUSIONS: Diplopia and anterior visual pathway abnormalities can be the manifestation of initial onset of sarcoidosis; therefore this diagnosis must be kept in mind when these frequent neuro-ophthalmologic signs are encountered. Complementary exams, mainly biopsy of the involved areas with histological analysis, are needed to confirm this diagnosis. Corticosteroid treatment is generally followed by improvement, but relapses may occur.

[Papilledema and intracranial hypertension]. / Oedème papillaire de l'hypertension intracrânienne.

Isolated bilateral papilloedema require magnetic resonance imaging (MRI), looking for tumor, hydrocephalus, cerebral venous thrombosis. If MRI is normal and doesn't show any risk for tonsillar herniation, lumbar puncture has to be done with cerebrospinal fluid (CSF) pressure evaluation. Intracranial hypertension without any intracranial lesion (mass lesion, arteriovenous shunt, venous thrombosis) is Pseudotumour Cerebri Syndrome. Risk of such Pseudotumour Cerebri is progressive blindness. Treatment cures intracranial hypertension and stops visual defect.

[Susac's syndrome: improvement with combined cyclophosphamide and intravenous immunoglobulin therapy]. / Syndrome de Susac: un cas d'évolution favorable sous cyclophosphamide et immunoglobulines intraveineuses.

We report a case of Susac's syndrome characterized by subacute encephalopathy, bilateral hearing loss and multiple bilateral branch retinal artery occlusions in a forty-year-old-white-woman. Brain Magnetic Resonance Imaging showed on T2-weighted images multiple, punctate areas of increased signal intensity in periventricular white matter, gray matter and brainstem most of them being enhanced by gadolinium. Cerebrospinal fluid was acellular but with an increased protein level (1.66 g/l). Treatment with cyclophosphamid and intravenous immmunoglobulin resulted in dramatic improvement of the clinical status over the following months and CSF normalization.

[Persistent value of intra-arterial fibrinolysis 8 hours or more following central retinal artery occlusion or of its branches]. / Intérêt persistant de la fibrinolyse intra-artérielle après 8 h ou plus d'occlusion de l'artère centrale de la rétine ou de ses branches.

PURPOSE: To establish that intra-arterial fibrinolysis of the ophthalmic artery can still be efficient 8 hours or more after a central retinal artery occlusion. MATERIAL AND METHODS: Nine to twenty hours (average 12.5 H) after a loss of vision due to a central retinal artery occlusion or the occlusion of its branches, 7 patients were treated by intra-arterial fibrinolysis. The vision of the affected eye was limited to light perception for four patients, to hand movement at 1 meter for two patients, and to 4/10 P2 for one patient. Under radiological guidance, 300.000 IU of urokinase was injected through a microcatheter placed in the ophthalmic artery (6 patients), or in the facial artery (1 patient); in this case the angiogram showed an occlusion of the internal carotid artery and a retrograde filling of the ophthalmic artery through the facial artery. RESULTS: We did not observed any complication. The 3 patients who had a vision better than light perception recovered a vision of 10/10 P2. For the others: two patients recovered a vision of 9/10 P2 and 5/10 P2, two did not recover. CONCLUSION: Fibrinolysis of the ophthalmic artery must ideally be performed as soon as possible, but a recovery is still possible more than 8 hours after the onset of the loss of vision. The time limit is not yet defined but after 24 hours, the chance of improvement seems to be very low.

Sex differences in the left and right hemifields of normal subjects with computerised static perimetry.

Results of routine perimetric testing of the left and right hemifields in normal subjects have been assumed to be symmetric although asymmetry due to hemispheric dominance has been established for other psychophysical tests. These asymmetries have sometimes been related to sex. With the advent of computerised static perimetry, subtle differences between the left and the right hemifields might be found that were not obvious before. This study investigated differences in retinal sensitivity between the hemifields and the role of sex and eye dominance. Forty three unequivocally right handed and right eye dominant normal adult volunteers, 24 females and 19 males, underwent Humphrey 24-2 testing, half beginning with the left eye, the other half with the right eye. The Peridata program was used to calculate decibel totals per hemifield. Four subjects were excluded because of poor cooperation or test artefacts. In females, the total of the left hemifield was significantly less than the right (p < 0.01) by a mean 18.2 (SD 24) dB equivalent to a difference of 0.34 dB per tested point. No significant difference in hemifields was found for males, between the sexes for both eyes combined, or between the two eyes for either sex. It was concluded that asymmetries in retinal sensitivity with respect to the vertical axis may be physiological and found in females, but not in males.