[Hypoplasia of the pons and neocerebellum with deformation of the dentate nucleus].
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Studies with MRI and non-invasive vascular imaging have modified previous conceptions on clinical spectrum and causes of different types of brain stem infarcts. Wallenberg's syndrome caused by lateral medullary infarction (LMI) often presents with patterns of sensory loss different from the "classical" crossed type. LMI carries a risk for respiratory and cardiovascular complications in the acute phase, warranting close patient monitoring. Medial medullary infarcts often present with a lacunar syndrome mimicking capsular or pontine small vessel disease. Cerebellar infarcts are most often caused by cardiac embolism. Isolated vertigo may be the only presenting symptom. Neurosurgical intervention of expansive cerebellar infarcts may be life-saving. Clinical features of progressive multifocal brain-stem symptoms are often suggestive of basilar artery occlusion. CT-angiography is a useful initial diagnostic tool. Based on observational studies, intraarterial thrombolysis is used in selected patients with basilar artery occlusion, but further studies are needed to define treatment criteria more precisely.
INTRODUCTION: Friedreich's ataxia (FA) is the most frequent of the recessive hereditary ataxias. AIMS. Our aim was to analyse the findings from electrooculography studies in subjects with FA attended in our Service over a 30-year period. PATIENTS AND METHODS: Between the years 1970 and 1999, 51 patients with FA diagnosed in the Neurology Service of our hospital were examined. All of them were submitted to an electronystagmography study and an examination of the oculomotor system using electrooculography (EOG). In the EOG study, saccades, (square-wave) saccadic intrusions, spontaneous, triggered, positional and optokinetic nystagmus (OKN), visual suppression of the vestibulo-ocular reflex (VOR) and following were all evaluated. RESULTS: The disorders that were found most often were ataxic following (72.5%), abnormalities in rotational testing (56.8%) and dysmetria in the saccades (52.9%). The presence of square waves was observed in just over half the traces (52.9%). CONCLUSIONS: In FA the predominant EOG findings are those suggesting a cerebellar disease.
Nonacoustic tumors of the CPA include meningioma, epidermoid, arachnoid cyst, lipoma, facial nerve schwannoma, and metastatic tumor, among others. Like AN, these lesions present with audiovestibular symptoms, facial nerve dysfunction, and other cranial nerve neuropathies. Preoperative differentiation among these lesions based on clinical history, physical examination, and audiovestibular testing is difficult. CT and gadolinium-enhanced MRI reveal the characteristic appearance of these tumors and make accurate diagnosis possible.
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Central vertigo is most often expressed by a feeling of dizziness, non or badly systematized, but it can also appear, more seldom, like an isolated acute vertigo or associated to other neurological signs. A precise clinical exam can lead to evidence essential clinical informations (significant ataxia, neurological signs, gaze nystagmus, pursuit anomaly,...). Almost all acute lesions of central vestibular pathways, as for the peripheral ones, lead to a harmonious vestibular syndrome. The vascular lesion of the vertebro-basilar territory and multiple sclerosis are two main causes to it. The pseudo-labyrinthine forms are essentially described in occlusion infarcts of the AICA and PICA, but a hematoma can lead to the same picture; the diagnostic of multi- or monosymptomatic forms with a peripheral lesion is often very difficult, the classical classification of the central and peripheral vestibular syndromes has become obsolete and should be abandoned.
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The author describes a conical sub-dural space above the vermis limited in front by Galien's ampulla, behind by the right venous sinuses and, below by the superior cerebellar vein. This space, which is not filled with air injected by the cisternal route, is only shown in lateral views thanks to its venous outlines. In cases of raised intracranial pressure, the superior cerebellar vermis may occupy this space so that a sort of impaction occurs at this level, which the author proposes calling smaller than superior vermial impaction greater than. Among forms of cerebral herniation, vermial impaction may not occur when the CSF obstruction occurs at another level.
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The AA. present a review of the expansive processes of the cerebellopontine angle (CPA) diagnosed at their Department, during the six last years. Emphasis is made upon the clinic findings gained through neuro-otological explorations and imaging, both helpful for preoperative diagnosis of the nature of the tumor.
The condition of a patient with Wilson's disease was evaluated by magnetic resonance imaging (MRI) on initial presentation of the illness. The examination revealed mild atrophy of the superior vermis. Symmetric areas of increased signal intensity on T2-weighted images were detected in the anterior thalami, mesencephalic tectum and tegmentum. Marked symmetric hypointensities appeared in the head of caudate, pallida, substantia nigra and red nuclei. The histopathology of Wilson's disease suggests that these hypointensities may be secondary to the presence of protein-bound copper.
The examinations were performed in a group of 53 cerebellar lesion patients. In pendular test the symmetric reaction were in 43, and asymmetrical in 10 patients. There were no symmetrical reactions giving evidence of vestibular hyper-reaction.