[Cognitive function of the temporal cortex].

In a brain composed of localized but connected specialized areas, disconnection leads to dysfunction. This simple formulation underlay a range of 19th century neurological disorders, referred to collectively as disconnection syndromes. Although disconnectionism fell out of favour with the move against localized brain theories in the early 20th century, in 1965, an American neurologist brought disconnection to the fore once more in a paper entitled, 'Disconnexion syndromes in animals and man'. In what was to become the manifesto of behavioural neurology, Norman Geschwind outlined a pure disconnectionist framework which revolutionized both clinical neurology and the neurosciences in general. For him, disconnection syndromes were higher function deficits that resulted from white matter lesions or lesions of the association cortices, the latter acting as relay stations between primary motor, sensory and limbic areas. From a clinical perspective, the work reawakened interest in single case studies by providing a useful framework for correlating lesion locations with clinical deficits. In the neurosciences, it helped develop contemporary distributed network and connectionist theories of brain function. Geschwind's general disconnectionist paradigm ruled clinical neurology for 20 years but in the late 1980s, with the re-emergence of specialized functional roles for association cortex, the orbit of its remit began to diminish and it became incorporated into more general models of higher dysfunction. By the 1990s, textbooks of neurology were devoting only a few pages to classical disconnection theory. Today, new techniques to study connections in the living human brain allow us, for the first time, to test the classical formulation directly and broaden it beyond disconnections to include disorders of hyperconnectivity. In this review, on the 40th anniversary of Geschwind's publication, we describe the changing fortunes of disconnection theory and adapt the general framework that evolved from it to encompass the entire spectrum of higher function disorders in neurology and psychiatry.

Agnosia↗

PubMed2005

Acquiring long-term representations of visual classes following extensive extrastriate damage.

Different areas of human visual cortex are thought to play different roles in the learning of visual information: whereas in low/intermediate cortical areas, plasticity may be manifested by enhanced selectivity to learned visual features, in higher-level areas, plasticity may result in generalization and development of tolerance to degraded versions of the learned stimuli. The most effective tolerance to degraded information is presumably achieved in the case of cooperation between the different forms of plasticity. Whether this tolerance to degraded information also applies when the visual input is degraded as a result of a lesion to lower levels of the visual system remains an open question. To address this, we studied visual classification learning in a patient with an extensive bilateral lesion affecting intermediate/low-level visual areas but sparing higher-level areas. Despite difficulty in perceiving the stimuli, the patient learned to classify them, albeit not as quickly as control participants. Moreover, the patient's learning was maintained over the long term and was accompanied by improved discrimination of individual stimuli. These findings demonstrate that degraded output from lesioned, lower areas can be exploited in the service of a new visual task and the results likely implicate a combination of bottom-up and top-down processing during visual learning.

Agnosia↗

PubMed2005

Frontotemporal dementia with ubiquitinated neuronal inclusions and visuospatial impairment.

Dementia with ubiquitinated neuronal inclusions has been described only with frontotemporal dementia (FTD). The authors report a patient with progressive FTD accompanied by prominent impairments in visuospatial cognitive functions. Pathology was characterized by ubiquitin-positive intranuclear and cytoplasmic neuronal inclusions. Cortical pathology was widespread and posteriorly accentuated but spared the hippocampal dentate gyrus.

Agnosia↗