Marchiafava-Bignami disease with widespread extracallosal lesions and favourable course.

We report a 61-year-old alcoholic man who presented with subacute physical deterioration and severe dysarthria. MRI, suggestive of corpus callosum demyelination with associated white matter involvement in both cerebral hemispheres, indicated the diagnosis of Marchiafava-Bignami disease. During his stay in hospital the patient showed remarkable improvement, and was discharged 22 days after admission. On MRI 2 months later, the extracallosal lesions had disappeared. This case raises questions about some previous ideas on this disease, such as the prognosis of its acute forms and the significance of the extracallosal lesions seen on neuroimaging.

['Rubral' tremor after vascular thalamic lesions]. / Temblor 'rúbrico' tras lesiones vasculares talámicas.

INTRODUCTION: The appearance of tremor after thalamic lesions is well-known but infrequent. Amongst the semiological varieties of thalamic tremors, a particularly uncommon type--which is extremely incapacitating owing to its great amplitude, appearance during action and poor therapeutic response--is the so-called rubric or mesencephalic tremor. CLINICAL CASES: We present four cases, of tremor with the semiological characteristics of rubric tremors after thalamic lesions of ischaemic or haemorrhagic origin. We review the relevant literature. DISCUSSION: The rubric tremor has been said to have its physiopathological origin in a lesion of the nigro-striate via and the efferent cerebellar vias at some point of the mesencephalic or subthalamic path, often without direct involvement of the red nucleus. CONCLUSIONS: The presentation of this type of tremor due to lesions which do not effect the red nucleus and the mesencephalum show the unsuitability of the name.

[Spontaneous intravascular rupture of a giant neuroepithelial cyst]. / Rotura espontánea intraventricular de quiste neuroepitelial gigante.

We report the case of a woman presenting with a gait disorder. Magnetic resonance the brain showed a large parieto-neuroepithelial cyst. Surgery was ruled out and a new image taken nearly two years later showed the cyst cavity had collapsed spontaneously. The benign course of this type of lesion and the possibility of spontaneous recurrence suggest that a wait-and-see attitude can be chosen over surgery in patients with few clinical manifestations.

[Clinical variability of bilateral paramedian thalamic infarcts]. / Variabilidad clínica de los infartos talámicos paramedianos bilaterales.

INTRODUCTION: Thalamic infarcts in paramedian artery territory are seen fairly frequently owing to certain peculiarities of the vascularization of the thalamus. However, clinical diagnosis is usually difficult because of the many varieties and peculiarities of the symptomatology. MATERIAL AND METHODS: We present a review of twelve cases of bilateral paramedian infarcts of the thalamus seen in our Department of Neurology and in a private surgery. We analyze the symptoms and their relationship to the neuro-radiological findings. Finally we compare our observations with the descriptions published by other authors and seek and anatomo-functional relationship for each of the symptoms and signs observed. RESULTS: The usual clinical outline in our patients included disorders of consciousness, different types of oculomotor disorders and cerebellar symptoms, mainly of gait. Other less common findings were memory disorders and abnormal movements. In no case were there sensory changes and pyramidal signs were rare in the absence of significant extra-thalamic lesions. CONCLUSIONS: Our findings, although generally comparable to those described in the literature consulted, were somewhat different with regard to cerebellar symptoms and the absence of sensory and pyramidal signs. We also emphasize the marked differences seen between the individual patients in our series. A good knowledge of the possible clinical variants of these lesions is necessary for a correct initial diagnostic approach in the study of these patients.

[The gastrointestinal changes in dystrophia myotonica]. / Alteraciones gastrointestinales en la distrofia miotónica.

Myotonic dystrophy is a multisystemic disorder inherited as an autosomal dominant trait. The secondary gastrointestinal disturbances are not well recognized. Two cases of myotonic dystrophy in a 51-year-old and 39-year-old woman with gastrointestinal symptoms disturbances are reported. One patient presented motor dysfunction in the colon and esophagus, organoaxial gastric volvulus and cholestasis. The second patient developed an acute colonic pseudo-obstruction. The patient improved with cisapride. The digestive manifestations of myotonic dystrophy and the treatment are reviewed.