Visual vertigo in children- adaptation and validation of the visual vertigo analogue scale to European Portuguese.

BACKGROUND: Visual vertigo occurs after vestibular and non-vestibular pathology and can be present in children and adolescents. It can be assessed by "the Visual Vertigo Analogue Scale" (VVAS), a questionnaire with a Portuguese version for adults. OBJECTIVES: To perform the adaptation to pediatric age and validation of VVAS in European Portuguese. METHODS: This prospective study involved the pediatric adaptation of the Portuguese VVAS, according to recognized guidelines. It was then completed by 30 healthy controls and 18 children with vestibulopathy. Patient caregivers also completed the Dizziness Handicap Inventory - Patient Caregivers (DHI-PC) to further explore the link between questionnaires. Groups were compared for severity of visual vertigo and VVAS test-retest reliability was tested. RESULTS: The VVAS score was significantly higher in vestibular group (p <  0.001). No statistically significant differences were found between VVAS initial and re-test scores (p = 0.33). VVAS severity scores showed a positive correlation with DHI-PC (r = 0.598, p = 0.009). CONCLUSION: The present Pediatric adaptation of VVAS in European Portuguese shows good psychometric properties for the assessment of visual vertigo. A positive correlation with the DHI-PC was showed, establishing the potential use of both questionnaires in the evaluation of vertigo children.

Visual and ocular motor function in the atypical form of neurodegeneration with brain iron accumulation type I.

BACKGROUND/AIMS: Neurodegeneration with brain iron accumulation (NBIA) type I is a rare disease that can be divided into a classical or atypical variant, according to age of onset and clinical pattern. Neuro-ophthalmological involvement has been documented in the classical variant but only anecdotically in the atypical variant. We sought to describe the visual and ocular motor function in patients with atypical form of NBIA type I. METHODS: Cross-sectional study, including patients with genetically confirmed NBIA type I and classified as atypical variant, who underwent ophthalmological examination with best corrected visual acuity (BCVA), optical coherence tomography (OCT), fundus autofluorescence (FAF), electroretinography (ERG), visual evoked potentials (VEP) and video-oculography. RESULTS: Seven patients with a mean BCVA of 0.12±0.14 logMAR were included. Only two patients showed structural evidence of advanced retinopathy in OCT and FAF, and there were no cases of optic atrophy. ERG data, however, showed abnormal scotopic and/or photopic responses in all patients. VEP were normal in all three patients. Ocular fixation was markedly unstable (eg, increased rate of saccadic pulses) in the majority of patients (5). Additional mild ocular motor disturbances included low gain pursuit (2), hypermetric saccades (1), low gain optokinetic (2) and caloric and rotatory responses (3). CONCLUSION: Functional retinal changes associated with marked instability of ocular fixation should be included in the clinical spectrum of NBIA, particularly in the atypical form.

Downbeat nystagmus elicited by eyelid closure.

We describe a patient with downbeat nystagmus (DBN) evoked only by eye closure. Brain and spinal cord magnetic resonance imaging revealed a T2 paramedian lesion in the left lower basis pontis and other white matter lesions consistent with multiple sclerosis. One potential mechanism for DBN in this case involves transverse ephaptic spread of excitation from areas that subserve coordinated lid closure to the decussating ventral tegmental tract.