[Linear scleroderma en coup de sabre: neurological symptoms, images and review]. / Esclerodermia lineal en coup de sabre. Manifestaciones neurologicas, imagenes y revision

INTRODUCTION: 'Sword stroke' linear scleroderma, which is better known as linear scleroderma en coup de sabre (LSCS), is a rare disease with an uncertain causation that is characterised by progressive craniofacial focal atrophy and is, at least in part, different from Parry-Romberg syndrome (PRS). CASE REPORTS: Here, we report on the cases of 3 patients with LSCS (2 females and 1 male, with a mean age of 40 years). The main neurological symptoms were headache and seizures. Although different alterations were observed in the X-ray images, they were all ipsilateral to the coup de sabre. Histopathological evidence for gliosis and mixed perivascular inflammatory infiltrate was found in the study of a biopsy specimen taken from one female. Cerebrovascular involvement was seen in another patient, as highlighted by the observation of an earlier subclinical cerebellar infarct and occlusion of the superior cerebellar artery in the absence of any other possible causation. CONCLUSIONS: When it affects the central nervous system, the clinical and radiological presentation of LSCS is heterogeneous. Both the imaging studies carried out during the clinical control and the histopathological findings suggest a focal inflammatory process that can be progressive. The arterial involvement is probably due to a non-atherosclerotic, occlusive and chronic inflammatory disease of the peripheral vessels.

[Intracerebral haemorrhage in a referral hospital in the central-western region of Mexico]. / Hemorragia intracerebral en un hospital de referencia de la region centro-occidente de Mexico.

INTRODUCTION: Intracerebral haemorrhage (ICH) has received little attention in studies in Mexico. Isolated reports talk of high frequency, its importance as a disorder among young people, its being mainly located in the lobar regions and a high rate of recurrence. AIMS: The objective of this study was to characterise the clinical, radiological, therapeutic and prognostic spectrum of ICH in a general hospital in the central-western region of Mexico. PATIENTS AND METHODS: The study involved 270 consecutive patients over the age of 15 years with spontaneous ICH who were hospitalised in the Neurology and Neurosurgical Service in the Antiguo Hospital Civil de Guadalajara between the years 2000 and 2002. Their clinical history and progression was known at least on discharge from the hospital. RESULTS: The mean age was 63 years (12% under 40 years old) with no predominance according to gender (53% males). Arterial hypertension was the main risk factor in 69%, followed by obesity in 38%. There were no differences in the Glasgow administered on admission in three pre-established subgroups. The ICH was ganglionic in 64% of cases and lobar in 24%. Arterial hypertension was the principal cause of ICH in 76%. Ventricular aperture was noted in 53%. All the patients were treated in a general ward. Mortality in the acute phase occurred in 49%, although a poor progression was observed in 83%. Overall recurrence was 13%. Outpatient follow-up was poor. CONCLUSIONS: ICH shares most of the features reported in Anglo-Saxon series including aetiology and location. In our population, mortality and recurrence are high with important sequelae. The high frequency of ICH (40%) may represent a bias in the selection of hospitals.

Transitory cognitive impairment in epileptic children during a CPT task.

EEGs and behavioral responses were studied in two sex matched groups of 58 epileptic and 20 healthy children between 8 and 12 years of age, during the execution of a go-no go CPT (X; A-X) task to determine transitory cognitive impairment (TCI) incidence. Paroxysmal discharges were found on 87.9% and 5% of the EEGs in the epileptic and control groups respectively, with no differences related to sex. The predominant EEG findings with respect to paroxysmal discharges were the association of two or more types of paroxysms with frequency higher than 5/minute, an average duration less than 0.5 second and topographical distribution over temporal-parietal-occipital areas without significant interhemispheric differences. TCI was detected in 36.2% of epileptic children. The epileptic group showed significantly higher numbers of behavioral errors and longer reaction times (RTs) in relation to the control group. Analyzing RTs on the two blocks of the task, linear discriminant analysis showed an acceptable classification of TCI incidence between groups.

Granulomatous amebic encephalitis due to Balamuthia mandrillaris (Leptomyxiidae): report of four cases from Mexico.

In this report, we describe four cases of granulomatous amebic encephalitis caused by Balamuthia (Leptomyxid ameba) in four previously healthy Mexican patients. All four cases were characterized by focal neurologic signs, increased intracranial pressure, and cerebral hyperdense lesions in computed tomography scans of the head. These patients underwent craniotomies for evaluation of mass lesions for possible brain tumors. Granulomatous chronic inflammatory reaction and amebic trophozoites were found in brain biopsies. At autopsy, areas of hemorrhagic encephalomalacia were located in both basal frontal lobes, right parieto-occipital lobes, and, less often, in the brainstem and cerebellum. Angiitis, necrotizing granulomatous encephalitis, and large numbers of amebic trophozoites in perivascular spaces were present. Amebic trophozoites were seen in the left adrenal gland in one of the cases. The amebas in all four cases were identified as Balamuthia mandrillaris (Leptomyxiidae) based on their reactivity with the anti-Balamuthia (Leptomyxiidae) serum in an immunofluorescence test.

Brucellosis and polyneuroradiculomyeloencephalitis. A case report.

Reports on simultaneous central and peripheral nervous system involvement in a patient with brucellosis are very rare. We report of one young female patient with a long history of consumption of non-pasteurized dairy products in which clinical and laboratory findings confirmed the existence of an active brucellosis with nervous system impairment. Cerebrospinal fluid (CSF) analyses were negative. Electrophysiology and positive findings on sural nerve biopsy complemented the diagnosis of polyneuroradiculomyeloencephalitis. Treatment with a combination of doxycycline and rifampin for 2 months was successfully applied. No relapse or sequelae occurred in the patient after 12 months of follow up.