[Leber "plus" disease: optic neuropathy, parkinsonian syndrome and supranuclear ophthalmoplegia]. / Maladie de Leber "plus": neuropathie optique, syndrome parkinsonien et ophtalmoplégie supranucléaire.

A 34-year-old right handed man presented with a bilateral subacute optical neuropathy associated with cervical dystonia, parkinsonism and supranuclear ophtalmoplegia. Magnetic resonance imaging showed in T2 increased intensity of signal in the dorsal mesencephalum and pons as well as in dorsal part of striata. The 3,460 mutation of mitochondrial DNA was found in a blood sample. This observation adds to the variability of presentation of Leber's "plus".

Room tilt illusion. A central otolith dysfunction.

BACKGROUND: We report a sudden 90 degrees room tilt illusion (RTI) following vestibular stimulation in 3 patients with persistent skew deviation caused by a brain stem lesion. Room tilt illusion is a transient tilt perception of the visual surrounding, on its side or even upside down, that is often reported with brain stem lesions. Although its pathophysiologic cause is not well known, the RTI suggests an impairment of otolith pathways, as reported in skew deviation. METHODS: The 3 patients with brain stem lesions were reexamined as part of a follow-up of patients with signs of otolith dysfunction. A registration of vestibular function was performed with a rotatory chair, including earth-vertical axis rotation for canal stimulation and off-vertical axis rotation (OVAR) for otolith stimulation. Measurement of the subjective visual vertical (SVV) was also performed. RESULTS: The otolith-ocular reflex registered by OVAR was impaired in the 3 patients with skew deviation and the SVV in 2 patients. After each direction of OVAR stimulation, the 3 patients reported an RTI as the room was illuminated. CONCLUSIONS: The coexistence of otolith oculomotor (skew deviation and impaired otolith-ocular reflex) and perceptual (tilt of SVV and RTI) disorders suggests a common otolith dysfunction. However, an RTI occurred specifically after vestibular stimulation and when the room was illuminated. We thus suggest that RTI reflects a dynamic visuo-otolith mismatch.

Ophthalmic results in patients with macroprolactinomas treated with a new prolactin inhibitor CV 205-502.

Macroprolactinomas are pituitary tumours which have been effectively treated medically since the introduction of bromocriptine. The visual function of 13 patients treated with a new prolactin (PRL) inhibitor CV 205-502 (Sandoz Basle), a potent and selective dopamine D2 receptor agonist, was evaluated. This is the first detailed ophthalmic report of the use of this drug in macroprolactinomas. Patients were enrolled from June 1988 to July 1990 (mean follow up 30 months). Visual function including visual acuity, ocular pressure, and visual fields was regularly controlled. Visual fields (VF) were tested with Goldmann and automatic static perimetry (Vision Monitor). Treatment was globally effective. No modifications of the visual function were observed in nine patients (six normal, three previous VF losses after surgery). In four other patients, visual function dramatically improved (regression of a III paresis, one case; disappearance of a chiasmatic syndrome, three cases). A pituitary necrosis was observed in one case and successfully cured. CV 205-502 seems to be an effective and well tolerated treatment of macroprolactinomas.

Idiopathic intracranial hypertension after ofloxacin treatment.

We report the occurrence of idiopathic intracranial hypertension in a patient treated with ofloxacin, a fluoroquinolone antimicrobial agent, for 16 months. The withdrawal of ofloxacin and acetazolamide therapy were followed by a complete recovery of visual function.

Homonymous horizontal sectoranopia: report of four cases.

Four cases of right homonymous horizontal sectoranopia are described. They were investigated with automatic static perimetry matched in three cases with Goldmann perimetry. In two cases--a wound of lateral sinus, a temporal surgically excised oligo-dendroglioma--neuroradiological investigations localised the visual pathways lesion to the optic radiations. In two other cases the lesions of vascular origin were less precise. In both of these there were ischaemic phenomena. In one of them the left anterior superficial sylvian territory was involved and in the other the left anterior choroidal artery territory was in question. This visual field pattern, less rare than was previously supposed, seems in some cases to correspond to retrogeniculate and varied topographic lesions.

Pituitary adenomas: automatic static perimetry and Goldmann perimetry. A comparative study of 345 visual field charts.

In a series of 81 cases of pituitary adenoma 345 charts of visual field performed with static automatic perimetry (AP) on the Vision Monitor and Goldmann perimetry (GP) were compared. Generally both methods were equivalent in the detection of chiasmal compression. The charts were divided into two groups: (A) 208 charts and (B) 137 charts according to the number of isoptres investigated: two (V4, 112) or three (V4, 112, 12). In group A the AP was more often altered than the GP, and the difference was statistically significant (p less than 0.001). In group B the GP seemed more effective, but the number of questionable cases was greater and the difference was not statistically significant. When the most internal isoptre of GP was outside the central 30 degrees, AP was more often abnormal (29%) than GP (2.2%), and the difference was statistically significant (chi 2, p less than 0.001). Within the central 30 degrees the GP seemed more often to be altered (23% of cases) than the AP (19.4%), but one-third of the cases were questionable. Within the central 30 degrees both techniques gave identical results and there was no statistically significant difference. The discrepancies between both static and kinetic techniques are an argument for their complementary use.

Effects of a new prolactin inhibitor, CV 205-502, in the treatment of human macroprolactinomas.

The effects of a new PRL inhibitor, CV 205-502 (CV), on human macroprolactinomas were studied in nine patients according to a prospective protocol. Five patients had undergone surgery leaving tumor remnants and persistent hyperprolactinemia. The four others were de novo patients, two of whom had received short term treatment with Parlodel. Plasma PRL levels ranged from 235-6050 micrograms/L before treatment. The doses of CV used in this trial ranged from 0.075-0.600 mg. Plasma PRL normalized in eight of the nine patients during treatment with CV. The time to normalize varied from 2 weeks to 9 months, and the doses from 0.075-0.450 mg. A tumor volume reduction of more than 50% was obtained in all four patients who had not been operated on before CV treatment. Only one of the five patients with postoperative tumor remnants had no reduction in tumor size. The drug was generally well tolerated, and no patient interrupted the treatment. Slight and short-lasting gastrointestinal symptoms were noted in several patients, and a single episode of fainting occurred in one patient when the drug was not taken at bedtime as instructed. A noticeable and persistent weight loss with anorexia was noted in two patients. Since CV 205-502, administered in a single daily dose, has tolerable side-effects and is effective in reducing PRL secretion and tumor size, it can be considered to be a useful treatment for macroprolactinomas.

Contrast sensitivity function and pituitary adenoma: a study of 40 cases.

The authors studied the contrast sensitivity function in 40 patients with pituitary adenoma. It seems to be a good index of compression of the visual pathway in this disease, but it lacks specificity and it cannot be used alone.

[Value of contrast sensitivity in neuro-ophthalmology. Main indications]. / Intérêt de la sensibilité au contraste en neuro-ophthalmologie. Principales indications.

Contrast visual testing (CVT) is a psychovisual test newly used in the clinical study of neurovisual disorders. Using a micro E (Luer-Essilor) the authors present their technique and discuss the principal indications of CVT in neuro-ophthalmology.

[Spatial contrast sensitivity in multiple sclerosis]. / La sensibilité au contraste spatial dans la sclérose en plaques.

Spatial contrast sensitivity was measured in 110 patients with multiple sclerosis (definite = 72, probable = 22, possible = 16) as part of a routine evaluation in a neuro-ophthalmological clinic. Results were compared with those of 37 normal controls matched for age. The test was abnormal in 71 p. 100 of patients. Contrast sensitivity was attenuated for 97 p. 100 of the eyes with optic neuritis and visual acuity drop, for 60 p. 100 of the eyes with recovered optic neuritis and for 36 p. 100 of the non affected eyes in the cases of unilateral optic neuritis. Among the 57 patients with normal visual acuity and no history of optic neuritis, 62 p. 100 had abnormal findings. Globally, contrast sensitivity was reduced on the whole spatial frequency range in cases of current optic neuritis, and mostly on the high or high and medium frequencies in the other cases. Our study confirms that spatial contrast sensitivity is the most sensitive of psychophysical methods to detect subclinical visual impairement in multiple sclerosis. Comparison with VEP's was performed in 66 patients. Both tests were roughly equally sensitive, but findings were concordant in only 63 p. 100 of the cases. The use of both VEP's and spatial contrast sensitivity increases the detection of latent optic neuritis.

[Evaluation of the central visual field by the Friedmann Mark I analyzer and color vision in 85 patients with multiple sclerosis. Correlation with visual evoked potentials in 50 cases]. / Etude du champ visuel central par l'analyseur de Friedmann Mark I et de la vision des couleurs chez 85 patients atteints de sclérose en plaques (S.E.P.). Corrélations avec les P.E.V. dans 50 cas..

Analysis of the visual field using Friedmann's analyser Mark I and color study in 85 multiple sclerosis patients. Static perimetry of the central visual field and test batteries (Ishihara plates, 15 Hue Standard, 15 Hue of Lanthony) for acquired color vision defects were performed in 85 multiple sclerosis patients (61 definite, 12 probable, 12 possible cases). Results in patients were compared to data obtained in 53 control subjects matched for age. 64% of the 85 patients and 52% of 48 patients with no history of optic nevritis showed visual field abnormalities and/or color vision defects. Comparison with VEP was available in 50 patients. While 10 patients had abnormal VEP and normal static perimetry and coloration tests, 5 patients had the reverse findings.

[Reese's conjunctival melanosis. Two anatomicoclinical observations with ultrastructural study in one case]. / Mélanose conjonctivale de Reese. A propos de 2 observations anatomocliniques avec étude ultra-structurale dans un cas.

Two cases of Reese's conjunctival melanosis are reported. Inflammatory signs revealed the presence of the lesion in a 58-year-old woman, examination demonstrating diffuse brown conjunctival, tarsal, and bulbar pigmentation in the right eye, raised above the surface at the sclerocorneal junction. Histology after exenteration demonstrated coexisting diffuse atypical melanocytic hyperplasia (precancerous melanosis), an intraepithelial melanoma (cancerous melanosis), and an invasive melanoma which was the object of an ultrastructural study. The second case, a 57-year-old woman, had a 20-year history of stable, diffuse black pigmentation of the bulbar conjunctiva of the right eye. Four months after biopsy, a pedunculated tumor of the internal canthus developed, corresponding to a poorly differentiated epithelioid cell malignant melanoma. These two cases are compared with similar observations reported in the literature.