Neurophysiologie de la dégénérescence corticobasale

Abstract

Corticobasal degeneration (CBD) is a neurodegenerative disorder of mid- to late-adult life. From a clinical standpoint, CBD is characterized by (i) an insidious onset and a slowly progressing, unilateral, levodopa-unresponsive parkinsonian syndrome with dystonia or myoclonus and (ii) cerebral features such as apraxia, alien limb phenomena and cortical sensory loss. Decisive clinical diagnostic criteria are not available and thus a neuropathological study remains essential for accurate CBD diagnosis. Consequently, additional non-clinical criteria must be identified in order to improve diagnosis while patients are still alive. Electrophysiological exploration can yield functional information on a number of brain structures (both cortical and sub-cortical) involved in CBD. The disorder features a specific cortical (frontoparietal) alteration which could help with differential diagnoses for other extrapyramidal syndromes. Hence, exploration of a patient's myoclonus can provide some specific arguments for CBD. Indeed, myoclonus displays a number of clinical and electromyographical characteristics which are consistent with a cortical origin (a shorter latency of the cortical C response, for example). However, some typical cortical features are missing (giant somesthesic evoked potentials, and cortical potentials preceding myoclonus in jerk-locked back-averaging studies). Some authors explain these abnormalities in terms of a sub-cortical origin for the myoclonus. The frontoparietal alteration in CBD has also been explored in studies of oculomotor movement. Indeed, asymmetric lengthening of the lateral ocular saccade latency argues more in favour of CBD than progressive supranuclear palsy. Moreover, cognitive function is also compromised in the early stages of CBD, although it is sometimes difficult to distinguish between CBD, PSP and frontotemporal dementia. Studying cognitive potentials enables one to confirm subcorticofrontal abnormalities and to dissociate CBD patterns from PSP patterns. Other electrophysiological tests (such as the exploration of dysautonomia, the palmomental reflex and the blink reflex) produce results which overlap with those seen in extrapyramidal syndromes and synucleinopathies (polysomnography), prompting discussion of the physiopathological mechanisms of these various diseases. Electrophysiological exploration is of value for diagnosing CBD in general and for studying specific, frontoparietal dysfunctions in particular. These techniques could also significantly contribute to our understanding of the physiopathology of CBD.