Lisencefalia, hipoplasia cerebelar y atresia de vías biliares extrahepáticas: una asociación inusual / Lissencephaly, cerebellar hypoplasia, and extrahepatic biliary atresia: An unusual association

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[Ischemic stroke and auricular septal aneurysm: six cases]. / Ictus isquémico y aneurisma del septo auricular: estudio de seis casos.

INTRODUCTION: Aneurysms of the atrial septum (AAS) are uncommon. They have been considered to be related to embolic phenomena. They are usually associated with other cardiac anomalies, especially persistence of the formen ovale. PATIENTS AND METHODS: We studied six patients diagnosed during a period of 24 months as having ischemic ictus and AAS. They were investigated for vascular risk factors and possible causes of cardiac embolism by means of ECG, transthoracic and transoesophagic echography (ETE). Cases with the clinical characteristics of embolism were anticoagulated. RESULTS: Five patients were men with an average age of 47.6 years. Three had the clinical features of established ictus, one of RIND and two of AIT. Three patients had vascular risk factors. The TSA study showed pathology of the carotid arteries to be present in three patients. In all six cases the AAS was identified on ETE. There were no thrombi in the atria in any case, and in two there was left-right communication. In four patients the condition was considered to have a cardio-embolic origin. No patient has had further episodes of cerebral ischaemia. DISCUSSION: It seems there is a certain risk of cerebral embolus associated with AAS. The simultaneous presence of alterations in cardiac rhythm together with other structural cardiac pathology seems to have a synergic effect on this. Paradoxical embolism, arrhythmias and intra-aneurysmal thrombi appear to be the mechanisms involved in the appearance of emboli. The best therapeutic approach is still unknown. New studies are therefore necessary to establish whether or not it is necessary to anticoagulate these patients.

[Neuroimaging and efficacy of treatment in advanced African trypanosomiasis]. / Neuroimagen y eficacia del tratamiento en la tripanosomiasis africana avanzada.

INTRODUCTION: West African trypanosomiasis (WAT) is rare in Spain. Delay in its diagnosis and treatment leads to irreversible diffuse meningoencephalitis and finally death of the patients. CLINICAL CASE: We described a patient in whom the diagnosis of advance WAT had been delayed. He had headache, alteration of the level of consciousness and sleep pattern, psychiatric disorders, crises, extrapyramidal, sensitive and endocrinological clinical alterations. Biochemical investigation showed slight anemia with marked thrombocytopenia, and raised ESR and IgM. Initially serology was negative for trypanosomes, although examination of the CSF confirmed the diagnosis. MR showed extensive lesions of the basal nuclei, brainstem and white matter. Both the clinical abnormalities and the lesions shown on MR disappeared after treatment with intravenous difluoromethylornithine. The patient is now symptom-free and at work as usual. DISCUSSION: Find diagnosis of WAT requires isolation of the parasite from the blood, the bone marrow or CSF. When other complementary tests (such as serology) are negative, the diagnosis should not be ruled out. There are few neuroimaging studies of WAT, and only by CT. We have found no MR studies of patients with WAT, in the literature. We emphasize the close correlation between the clinical and radiological findings in this case, and the excellent result obtained after treatment.

[Biopercular lesion with inverse dissociation]. / Lesión biopercular con disociación inversa.

Foix-Chavany-Marie Syndrome or anterior opercular syndrome constitutes the cortical form of pseudobulbar palsy. Its most common etiology is stroke in the region of either operculum. Clinically it can be characterized by severe dysarthria and facio-pharyngo-glosso-masticatory dysplasia with automatic-voluntary dissociation. An inversion of this classical dissociation can be seen in patients with emotional paralysis in the face, in whom asymmetry of emotional expression but conservation of voluntary movement can be seen. The lesional topography of this inverse dissociation corresponds to the supplementary motor area. both processes support the existence of an anatomical base which is different for emotional movements and voluntary types. We describe the clinical case of a 65 year old woman with a history of breast cancer who presented a subacute and progressive clinical picture of dysphagia and severe dysarthria. Upon neurological examination slight velo-palato-pharyngea was noticed; most relevant was paralysis of the facial muscles for either emotional or automatic movements while voluntary innervation was maintained. Neuroradiological studies show the presence of brain metastasis located in both Roland opercula. Although the neuroanatomic bases for automatic-voluntary dissociation remain unclear, our case lends weight to the evidence that impulses for voluntary and emotional movements originate in different cortical areas or else take a different route through the brain. We have been unable to find any cases of biopercular lesion with inverse dissociation described in the literature we reviewed.