[Carcinomatous cerebellar degeneration with cortical atrophy and inflammatory reaction involving the dentate nucleus: case report (author's transl)].
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Altered metabolism of neuroexcitatory amino acids has been described in patients with a form of olivopontocerebellar atrophy (OPCA) associated with glutamate dehydrogenase (GDH) deficiency. To further investigate the specificity of these results, oral glutamate loading tests were performed in healthy controls, patients with GDH deficient OPCA as well as patients with non-GDH deficient degenerative disorders affecting primarily the function of the cerebellum and/or the basal ganglia. Following oral intake of monosodium glutamate, plasma levels of glutamate, aspartate and taurine increased significantly in controls and similar increases also occurred in patients with non-GDH deficient disorders. However, patients with GDH-deficient OPCA showed much greater elevations in plasma glutamate and aspartate and a rather flat taurine curve.
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A case of tuberculous abscess of the cerebellum is reported by the authors. A check-up of concerned literature of ten-past years is drawn up. Diagnosis is seldom raised before surgery in spite of former or evolved tuberculosis. Histology and bacteriology are essential for a supportive therapy. The prognosis is favorable in seventy three per cent of the cases. This pathology must not be forgotten in patients who imigrate from countries of high endemic level of tuberculosis.
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Four patients with bleeding venous angiomas in the cerebellar hemispheres are reported. All of the hemorrhages were subacute; three were recurrent. A literature review substantiated venous angioma as a recognized source of spontaneous cerebellar hemorrhage. Cerebellar venous angiomas may be more prone to bleed than venous angiomas in other locations. Their natural history seems to be similar to that of arteriovenous malformations. Though a minority bleed, the hemorrhagic event can be acute or subacute, recurrent, or catastrophic. Thus the clinical course is not always innocuous.
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Two post-operative cases of ocular flutter with electro-oculographic recordings are reported. In both cases, the lesion was unique and restricted, suggesting that a cerebellar damage was responsible for this abnormal eye movement. In both cases, ocular flutter was directed toward the healthy side. Therefore it is suggested that flutter may result from a focal cerebellar lesion and in that case be an oriented eye movement.
Dendritic plasticity in man is analysed on the example of the Purkinje cell. The conspicuous and easily demonstrable dendrites of this cell lend themselves particularly to such a study. Abnormalities of the dendritic tree have been observed in a variety of conditions, some of them originating in early infancy, others acquired in later life, all of them associated with a substantial loss of granule cells. While differences between the various diseases undoubtedly exist, the basic pattern remains remarkably constant. Three types of dendritic abnormality may be present: (i) persistence or neoformation of somal dendrites; (ii) expansion and malorientation of primary apical dendrites (the staghorn deformity); and (iii) bizarre ramification of secondary and tertiary dendrites, sometimes associated with expansions known as cactus, stellate or sunray figures. It is suggested that all these features should be interpreted as a response of the Purkinje cell to partial deafferentation and not as congenital malformations. In addition, dislocation of the Purkinje cell into the molecular layer is frequently seen in this group of cases. This may be ascribed to loss of tethering of the cell by its afferent fibres.
The contribution of the scanner, among the different radiological examinations performed for suspected cerebellopontine angle syndromes, was assessed in 101 cases, of which 76 were neurinomas. Direct signs, with evaluation of the different characteristics of the tumors, were studied together with indirect signs. Results demonstrated that computed tomography was a simple, reliable, and low-risk examination, and that it could avoid more traumatic radiological investigations in the majority of cases. Furthermore, when associated with spinal canal air injections it provides confirmation of diagnosis of suspected intraspinal tumors. As a complementary clinical or paraclinical examination (audiometry, E.N.G., acoustic reflexes, evoked potentials, conventional radiographs, tomography, among others) it can establish the precise diagnosis of a cerebellopontine angle tumor and should be performed in all suspected cases.
After a short description of the pathogenesis and morphology of intracranial epidermoids, the clinic symptoms and the diagnosis are dealt with. Therapeutically, it is exclusively a total extirpation with the use of a surgical microscope that can lead to curing. A report is given on nine patients, eight of whom were successfully operated on. It is hardly possible to establish a founded preoperative diagnosis of the kind of the epidermoid. Hypodense space occupying processes in the computer tomogram should, however, give rise to consider differentialdiagnostically also the presence of epidermoids.
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Diagnostic criteria of intracranial epidermoid cysts were established from the observation of 15 cases, confirmed by pathology, seen during the last 5 years. Tumors could be divided into four groups: posterior fossa, supratentorial spaces, transtentorial and extradural. Regions of hypodensity associated with the irregular nature of the outlines of the lesion are generally sufficient to suggest the diagnosis in a pre-operative scan, but certain tumors may be isodense or, more exceptionally, spontaneously hyperdense. Contrast images are extremely rare, and are seen only at the periphery of the lesions. The "mass-effect" is remarkably discrete when compared with the tumor size, sometimes of very large proportions.
Two cases of cerebellar haemorrhage with hydrocephalus are presented which were successfully treated with isolated ventricular drainage. Both occurred in high risk patients, 77 and 68 years old. The first observation confirms the value of this operative procedure when the volume of the haemorrhage is relatively restricted, whatever may be the importance of the neurologic symptoms and brainstem disfunction. But the second observation also shows the risk of misappreciating the causative lesion (here a single metastasis). The importance of an extended C.T. follow up must be underlined.
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