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Canavan's disease. An astrocytary mitochondropathy?
150 voluntary postural hyperkinesia with hypertrophic neuropathy closely resembling the first case was reported by Salisachs and Lapresle (1973, the corresponding film was shown at the X th neurological congress in Barcelona). The differences of the nerve biopsy specimens of these two patients and of the second case are striking. The nosological situation of these cases is discussed in relation to Pierre-Marie-Boveri disease, Roussey-Levy disease, Charcot-Marie-Tooth disease. Stress is laid on the changing conceptions of hypertrophic neuropathy and changing descriptions of tremor and dyskinesia in the literature. So it seems erroneous to state that the distinguishing feature between CharcotMarie-Tooth disease and Roussy-Levy disease is the presence of tremor in the latter disease. The authors conclude that the 3 cases cited above and the cases of Pierre-Marie-Boveri seem to be identical on clinical grounds; that the anatomical basis of the nerve lesion may apparently vary from the onion bulb type to axonal degeneration; that these facts tend to identify the neuropathy in these cases with the hypertrophic type of Charcot-Marie-Tooth, following the classification of Dyck; that nothing can be said for the moment as to the mode of heridity in these cases. To be published in extenso: Rev. Neurol. (Paris).
LOSS OF V O L U N T A R Y C O N T R O L W I T H R E T E N T I O N O F A U T O M A T I C A N D R E F L E X ACTIVITY O F MUSCLES I N N E R V A T E D BY C R A N I A L NERVES IN TWO CASES O F A M Y O T R O P H I C LATERAL SCLEROSIS. J. LAPaESLE and P. SAUSACHS (Neurological Clinic, H6pital Bic6tre, Le Kremlin Bic~.tre). The authors present two clinical observations of amyotrophic lateral sclerosis (A.L,S.) in which a loss of voluntary control was associated with retention of automatic and reflex activity of muscles innervated by cranial nerves. In the first case the face and swallowing were affected, in the second the movements of the eyes. These phenomena are exceptional in A.L.S. To be published in extenso in: Rev. Neurol. (Paris).
C A N A V A N ' S DISEASE. AN A S T R O C Y T A R Y M I T O C H O N D R O P A T H Y ? R. LE COULTRE and E. J. EBELS (Neurological Clinic and Laboratory of Neuropathology, University of Groningen). Three cases of Canavan's disease are reported. Microscopic examination of brain biopsies showed the classical subcortical spongy aspect. The interest of these observations was given by the electron microscope studies: structures encountered in the astrocytes were thought to be pathologically changed mitochondria. The primary vs. secondary nature of these mitochondrial changes is discussed. To be published in extenso in: Clin. Neurol. Neurosurg.
SLEEP D I S O R D E R S IN S T E E L E - R I C H A R D S O N DISEASE. A P O L Y G R A P H I C STUDY O F FIVE CASES. Mrs. F. LEYGONIE, J. THOMAS, J. D. DEGOS, A. BOUCHAREINE and J. BARBIZET (Neurological Clinic, H6pital Henri Mondor, Cr6teil). 27 recordings of night-time sleep in five patients suffering from progressive supranuclear paralysis were carried out. There was observed: a tendency to insomnia, disappearance of spindles, an invasion of slow sleep immediately followed by the muscular and ocular criteria of paradoxical sleep, a considerable reduction in paradoxical sleep in the strict sense of the term, the latency of which in making its appearance was, however, maintained; progressive invasion of the paradoxical phases by slow waves. These abnormalities differ from those found in Parkinson's disease. To be published in extenso in: Rev. Neurol. (Paris).
LOSS OF V O L U N T A R Y C O N T R O L W I T H R E T E N T I O N O F A U T O M A T I C A N D R E F L E X ACTIVITY O F MUSCLES I N N E R V A T E D BY C R A N I A L NERVES IN TWO CASES O F A M Y O T R O P H I C LATERAL SCLEROSIS. J. LAPaESLE and P. SAUSACHS (Neurological Clinic, H6pital Bic6tre, Le Kremlin Bic~.tre). The authors present two clinical observations of amyotrophic lateral sclerosis (A.L,S.) in which a loss of voluntary control was associated with retention of automatic and reflex activity of muscles innervated by cranial nerves. In the first case the face and swallowing were affected, in the second the movements of the eyes. These phenomena are exceptional in A.L.S. To be published in extenso in: Rev. Neurol. (Paris).
C A N A V A N ' S DISEASE. AN A S T R O C Y T A R Y M I T O C H O N D R O P A T H Y ? R. LE COULTRE and E. J. EBELS (Neurological Clinic and Laboratory of Neuropathology, University of Groningen). Three cases of Canavan's disease are reported. Microscopic examination of brain biopsies showed the classical subcortical spongy aspect. The interest of these observations was given by the electron microscope studies: structures encountered in the astrocytes were thought to be pathologically changed mitochondria. The primary vs. secondary nature of these mitochondrial changes is discussed. To be published in extenso in: Clin. Neurol. Neurosurg.
SLEEP D I S O R D E R S IN S T E E L E - R I C H A R D S O N DISEASE. A P O L Y G R A P H I C STUDY O F FIVE CASES. Mrs. F. LEYGONIE, J. THOMAS, J. D. DEGOS, A. BOUCHAREINE and J. BARBIZET (Neurological Clinic, H6pital Henri Mondor, Cr6teil). 27 recordings of night-time sleep in five patients suffering from progressive supranuclear paralysis were carried out. There was observed: a tendency to insomnia, disappearance of spindles, an invasion of slow sleep immediately followed by the muscular and ocular criteria of paradoxical sleep, a considerable reduction in paradoxical sleep in the strict sense of the term, the latency of which in making its appearance was, however, maintained; progressive invasion of the paradoxical phases by slow waves. These abnormalities differ from those found in Parkinson's disease. To be published in extenso in: Rev. Neurol. (Paris).