An 81 year old right handed woman developed a left alien hand syndrome characterised by involuntary movements of choking and hitting the face, neck, and shoulder. The patient showed multiple disorders of primary sensation, sensory processing, hemispatial attention, and visual association, as well as a combination of sensory, optic, and cerebellar ataxia (triple ataxia) of the left arm in the ab...

In monkeys and humans, two functionally specialized cortical streams of visual processing emanating from V1 have been proposed: a dorsal, action-related system and a ventral, perception-related pathway. Traditionally, a separate organization of the two streams is assumed; the extent of functional interaction is unknown. After lesions of the dorsal stream in patients with optic ataxia, it has re...

IN a previous paper (Luria, 1959), one of the present authors communicated a case in which marked disorders of "simultaneous perception" (simuhanagnosia) resulted from bilateral occipital brain injury. As in earlier cases of the same type (Balint, 1909; Holmes and Horrax, 1919; Paterson and Zangwill, 1944; H6caen and Ajuriaguerra, 1954) the patient was able to perceive only one object at a time...

Patients with optic ataxia, a deficit in visually guided action, paradoxically improve when pantomiming an action towards memorized stimuli. Visual form agnosic patient D.F. shows the exact opposite pattern of results: although being able to grasp objects in real-time she loses grip scaling when grasping an object from memory. Here we explored the dissociation between immediate and delayed gras...

When reaching towards a visual stimulus, spatial information about the target must be transformed into an appropriate motor command. Visual information is coded initially in retinotopic coordinates, while the reaching movement ultimately requires the specification of the target position in limb-centred coordinates. It is well established that the posterior parietal cortex (PPC) plays an importa...

Friedreich ataxia (FRDA) is an autosomal recessive neurodegenerative disorder usually characterized by progressive early-onset ataxia. The most common ophthalmic manifestation of FRDA is optic neuropathy, which is usually late in onset, is slowly progressive, and rarely causes severe visual loss. The genetic basis of FRDA in most patients is the homozygous expansion of a GAA trinucleotide repea...

Patients with bilateral optic ataxia fail to show rapid perturbation-induced corrections during manual aiming movements. Based on this, it has been proposed that this pathology results from a disruption of processes of on-line motor control in the posterior parietal cortex (PPC). Here, we show that on-line motor control performance in a patient with unilateral optic ataxia is similar to that of...

The mitochondrial protein SLC25A46 has been recently identified as a novel pathogenic cause in a wide spectrum of neurological diseases, including inherited optic atrophy, Charcot-Marie-Tooth type 2, Leigh syndrome, progressive myoclonic ataxia and lethal congenital pontocerebellar hypoplasia. SLC25A46 is an outer membrane protein, member of the Solute Carrier 25 (SLC25) family of nuclear genes...

Optic neuropathy is common in mitochondrial disorders, but poorly characterized in Friedreich's ataxia (FRDA), a recessive condition caused by lack of the mitochondrial protein frataxin. We investigated 26 molecularly confirmed FRDA patients by studying both anterior and posterior sections of the visual pathway using a new, integrated approach. This included visual field testing and optical coh...