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NEUROLOGICAL SOCIETY OF PARIS

1934; American Medical Association; Volume: 32; Issue: 3 Linguagem: Inglês

10.1001/archneurpsyc.1934.02250090186013

2330-9628

Marine Vincent,

Neurological and metabolic disorders

ed by Walter Freeman, M.D., Washington, D. C. Alterations of the Cerebellum and of the Spinal Ganglia in Friedreich's Disease. J. Lhermitte. A girl presented rather typical signs of Friedreich's ataxia, with diminution in strength, loss of reflexes, diminution in deep sensibility and a positive plantar reflex. The upper limbs were involved, and there were kyphoscoliosis, tremor of the head, drooping of the eyelids, nystagmus and monotonous speech. The patient was irritable and required commitment. Pathologic examination revealed marked degeneration of the spinal cord in both the lateral and dorsal funiculi, of the type usually found. In the spinal ganglia there was diminution in the capsular cells, with marked lymphocyte and plasma cell infiltrations and reduction in nerve fibers. The cerebellar cortex showed few fibers and Purkinje cells, the basket fibers often enveloping clear spaces. The Purkinje dendrites often showed swellings and serpentine twistings. These findings are new proof that Friedreich's disease is associated with lesions of the cerebellum. Palatal Myoclonus : Pathologic Examination. Georges Guillain, R. Thurel and I. Bertrand. This case was previously presented from the clinical standpoint. The myoclonia involved not only the muscles of the palate and pharynx but those of the diaphragm, the lip and the eyes, the orbicularis palpebrarum and even the muscles of the neck and those of the upper and lower limbs. In spite of its extent, the myoclonia was practically limited to the left side. It was absent during complete repose and, depending on the posture of the patient, could be brought out with considerable force. The movements were constant in rhythm at 130 per minute and entirely synchronous. Necropsy showed a few small lacunae in the white matter and putamen on each side. In the pons there were several of these, and there was an infarct destroying practically half of the right tegmentum and involving the upper end of the right superior cerebellar peduncle. The degenerations were striking ; the left red nucleus was extremely pallid, the right median fillet practically destroyed, the right central tegmental fasciculus degenerated and the right olivary body greatly enlarged and pale. Areas of a similar type of hypertrophie degeneration were found in the left dentate nucleus. The median longitudinal fasciculus was unaffected. The destruction of the central tegmental fasciculus at any point provokes degeneration as far down as the homolateral olive, and this is followed by pseudohypertrophic sclerosis of the olive and even sometimes by transsynaptic degenera¬ tion of the olivary cells and of the olivocerebellar fibers. Lesion of the red nucleus provokes retrograde degeneration of the superior cerebellar peduncle and occa¬ sionally severe atrophy of the corresponding cerebellar hemisphere and restiform body. Lesion of the dentate nucleus or of the restiform body causes a degenDownloaded From: http://archneurpsyc.jamanetwork.com/ by a Microsoft Bing User on 05/07/2016 eration of the arcuate fibers and of the contralateral olivary body, with pallor of the fibrillary meshwork, but without transsynaptic degeneration of the central tegmental fasciculus. Degeneration of one olivary body is accompanied by degen¬ eration of the olivocerebellar fibers and pallor of the cerebellar hemisphere of the opposite side and some atrophy of the opposite olive ; in such cases the central tegmental fasciculus is entirely normal. This is not simple atrophy ; it is accom¬ panied by excessive proliferation of the neuroglia and even by hypertrophie degen¬ eration of the olivary cells. It is probably a lesion of this type that conditions the myoclonic syndrome. The extension of the myoclonia from one side to the opposite side is apparently caused by progressive cellular degeneration. This hypothesis explains the slow development of the myoclonia after the original injury. There seems to be some relation between this type of olivary involvement and the tremors of pontocerebellar atrophy in the advanced stage. The olivary lesions as well as those elsewhere are similar. The one feature that seems constant in this condition is the olivary degeneration on the side opposite the myoclonia. Parinaud's Syndrome and Palatal Myoclonus : An Anatomic Study. Raymond Garcin, Ivan Bertrand and Pierre Frumusan. The anatomic foundation of conjugate paralysis of the eyes and of palatal myoclonia is incompletely understood ; so a case combining the two is of unusual interest. The multiplicity of lesions in such cases makes the problem more difficult, since no one lesion can be blamed for the symptoms exhibited. The patient was a woman, aged 65, who presented severe right hemiplegia with contracture and anarthria. There were spasmodic weeping and trouble in swallowing. The left side of the body was normal. Voluntary vertical movements of the eyes were practically abolished, and likewise the automatic vertical reflexes on lowering and raising the head. Convergence was abolished, but the lateral movements and the pupils were normal. The palate showed myoclonic twitchings at the rate of 150 per minute, sometimes skipping a beat, as in cardiac extrasystoles. The palatal reflex was abolished, but the pharyngeal reflex was preserved. The palate was partially paralyzed ; nevertheless, there was no reflux of liquids through the nose. There were moderate hypertension and profound asthenia. At autopsy the left hemisphere showed several small lacunae in the lenticular nucleus and thalamus ; there was marked atrophy but no focal lesion in Wernicke's region ; anterior to the left ganglion habenuiae was a small softening in the wall of the third ventricle, just rostral to the posterior commissure. In the right hemisphere several small foci were found in the basal ganglia as well as a minute lesion in the knee of the internal capsule. Microscopically, it could be seen that many fibers of the posterior commissure were destroyed by the lesion, which also involved the capsule of the red nucleus and Meynert's fasciculus. The degeneration of the left pyramidal tract was profound; in the pons many foci were found ventrally, while the tegmentum was absolutely intact. The olivary bodies showed hypertrophie degeneration in the dorsomesial aspects, with overgrowth of neuroglia. The extraciliary fibers of both dentate nuclei were degenerated, but the hilus was preserved. The Parinaud syndrome was believed to be due to the lesion in the vicinity of the habenula. The integrity of the central tegmental fasciculus is important, since in most of the cases of palatal myoclonus previously reported this structure has been incriminated. Severe degeneration of the dentate nuclei seemed to be of prime importance in this case, particularly since the dentate nucleus is tied up with the olivary bodies and the red nucleus in a functional triangle, as indicated by Guillain and Mollaret.