Lhermitte-Duclos disease

in Acta Neuropathologica (1983), 59(2), 88-94

A new case of Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) was recognized in an adult man after neurosurgery. Conventional, Golgi, and electron-microscopic investigations support ... [more ▼]

A new case of Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) was recognized in an adult man after neurosurgery. Conventional, Golgi, and electron-microscopic investigations support the view that this rare lesion results from a progressive hypertrophy of granular cell neurons with excess of myelination of their axons in the molecular layer while inducing the local disappearance of Purkinje cells and the central core of the cerebellar folia. After a follow-up of 4 years, the patient now leads a normal life; no recurrence has occurred and no other neuropathologic alteration have been detected. [less ▲]

The dendritic organization of the human spinal cord: the motoneurons.

in Journal of Comparative Neurology (The) (1982), 211(3), 226-47

The dendritic organization of motoneurons was analyzed with the Golgi stain and a morphometric method in the immature and adult human spinal cord. Each motoneuronal column was found to be characterized by ... [more ▼]

The dendritic organization of motoneurons was analyzed with the Golgi stain and a morphometric method in the immature and adult human spinal cord. Each motoneuronal column was found to be characterized by a specific orientation of dendritic trees and by a distinct pattern of dendritic bundling. Ventromedial motoneurons have a pyramidal dendritic tree with numerous, short longitudinal branches and elongated dorsal branches. The latter form thick bundles oriented toward the ventral gray commissure. Longitudinal dendrites form a narrow-meshed dendritic plexus, containing abundant microbundles. Motoneurons of the ventromedial column have fewer primary dendrites and a lower ramification index than other motoneurons. Central motoneurons are predominantly oriented longitudinally. The meshes of the rostrocaudal dendritic plexus are looser and the microbundles are finer. Most transverse dendrites run laterally and participate in dendritic bundles which penetrate into the ventrolateral funiculus. The rostrocaudal dendritic domain of ventrolateral motoneurons is the largest dendritic domain of all spinal neurons. The longitudinal dendritic network contains fine microbundles and appears wide-meshed. Transverse dendrites form lateral or medial dendritic bundles depending upon the position of their perikaryon. Dorsolateral motoneurons differ from other motoneurons by their multipolar organization with a slight preponderance of dorsoventral dendritic spread. Rudimentary lateral dendrite bundles are restricted to marginal neurons. The longitudinal plexuses of motoneuronal dendrites and the verticotransverse dendrite bundles of the ventromedial column are well developed in the 26-28-week-old fetus. In contrast, the horizontotransverse dendrite bundles of central and ventrolateral motoneurons can only be recognized from 36 weeks on. The possible specific functions of the various types of dendrites bundles are examined and a laminar dendroarchitectonic schema of the human cord is proposed. [less ▲]

L'organisation neuronale de la moelle épinière de l'homme

Book published by Editions Sciences et Lettres (1981)

Les Chorées

in Encyclopédie Médico-Chirurgicale (1980)

Motoneuropathie hereditaire: la forme proximale de l'adulte liee au sexe (ou maladie de Kennedy). Observations cliniques et neuroendocrinologiques.

in Journal of the Neurological Sciences (1979), 41(3), 343-57

Clinical, electrophysiological, histological and neuroendocrinological findings in a peculiar form of progressive spinal and bulbar muscular atrophy affecting 4 members in two different families are ... [more ▼]

Clinical, electrophysiological, histological and neuroendocrinological findings in a peculiar form of progressive spinal and bulbar muscular atrophy affecting 4 members in two different families are reported. The clinical hallmarks, which characterize this entity among the group of degenerative motor neuron diseases are: sex-linked recessive inheritance; onset in the third decade; slow progression; involvement of facial and bulbar muscles in addition to wasting of the proximal and, in some cases, the distal musculature; asymmetry of clinical signs; consistent and abundant fasciculations predominantly in the peri-oral muscles; intention tremor and a well-developed gynaecomastia, which is the first clinical sign. Electrophysiological and histological findings confirm the neurogenic origin of the muscular atrophy; in addition, muscle biopsy shows "myopathic-like" changes in one case and serum muscle enzyme levels are elevated in all neurologically affected patients. It is believed that the clinical entity corresponds to the rare type III proximal hereditary motor neuropathy (or "Kennedy's disease"), of which 25 published cases are reviewed. Neuroendocrinological data in two patients demonstrate an androgenic insufficiency of hypothalamo-hypophyseal origin and high levels of circulating oestrogens, which probably have induced gynaecomastia. Dynamic neuroendocrinological tests suggest that lesions of certain hypothalamic nuclei may exist, which should be looked for in the forthcoming anatomical studies. [less ▲]

Hyperthyroidism as a cause of persistent choreic movements.

in Acta Neurologica Scandinavica (1978), 58(5), 309-12

A patient is described who had thyrotoxicosis more than 30 years ago and suffers since that time from presistent choreic movements. This case suggests for the first time that chorea induced by ... [more ▼]

A patient is described who had thyrotoxicosis more than 30 years ago and suffers since that time from presistent choreic movements. This case suggests for the first time that chorea induced by hyperthyroidism may outlast the endocrine disorder and become irreversible. [less ▲]

Histoenzymology of the developing rat spinal cord.

in Neuropathology & Applied Neurobiology (1978), 4(1), 37-46

Using histochemical methods the activity of acetycholinesterase, acid phosphatase and various dehydrogenases was investigated in the developing rat spinal cord. At the ninth embryonic day (E9) only the ... [more ▼]

Using histochemical methods the activity of acetycholinesterase, acid phosphatase and various dehydrogenases was investigated in the developing rat spinal cord. At the ninth embryonic day (E9) only the activity of the lactate dehydrogenase slow-moving isoenzymes was prominent in spinal neurons. Between E13 and E15 an increase was observed in the activity of most of the dehydrogenases and of acid phosphatase in motoneurons and posterior root ganglion cells. Between E15 and E17 acetylcholinesterase activity increased markedly. On E17 and E20, this enzyme was also detectable in anterior and posterior roots and in neurons of the intermediate grey matter. On E20, although all grey matter neurons were cytologically fully differentiated, their enzymatic content was found to be still incomplete. The prominent acid phosphatase reaction within laminae I and II, which is characteristic of the adult rat, was absent in the fetal spinal cord. These findings indicate that the spinal cord metabolism is predominantly anaerobic during the first two-thirds of gestation. The histoenzymological maturation of grey-matter neurons is delayed in comparison to their cytological differentiation. Furthermore, the ontogenesis of motoneuronal acetylcholinesterase activity correlates well with the development of motor activities in the rat fetus. [less ▲]

Non-hypertrophic familial neuropathy associated with intention tremor. A variety of Charcot-Marie-Tooth disease?

in Journal of the Neurological Sciences (1976), 27(1), 59-69

A family with an association of sensorimotor neuropathy and intention tremor is reported. Clinical examination of 3 affected family members showed in varying degrees areflexia, muscle wasting, impairment ... [more ▼]

A family with an association of sensorimotor neuropathy and intention tremor is reported. Clinical examination of 3 affected family members showed in varying degrees areflexia, muscle wasting, impairment of deep sensation with an ataxic gait, pes cavus and disabling intention tremor. Motor nerve conduction velocities were moderately slowed. A superficial peroneal nerve biopsy showed axonal degeneration without segmental demyelination or onion bulb formation. Our observation seems to indicate an association of intention tremor with the non-hypertrophic variety of Charcot-Marie-Tooth disease. It can therefore be suggested that the two classical types of Charcot-Marie-Tooth syndrome possess variants which are associated with intention tremor. This association is well-known for the hypertrophic type; our report gives an example of the non-hypertrophic type. [less ▲]

Etude histoenzymologique des couches cytoarchitectoniques de la moelle de différents mammifères et de l'homme

in Bulletin de l'Association des Anatomistes (1974), 58

Localisation histochimique et propriétés d'un isoenzyme fluororésistant de la phosphatase acide de la moelle épinière du rat et de la souris

in Bulletin de l'Association des Anatomistes (1970), 145