Susana Arias-Rivas

High pro-BNP levels predict the occurrence of atrial fibrillation after cryptogenic stroke

Objective: Antiplatelets are recommended for secondary prevention in patients with cryptogenic stroke; however, some patients may present with a cardioembolic source that has not been detected, which may modify the treatment. Because high pro–brain natriuretic peptide (BNP) levels are associated with cardioembolic stroke, our objective was to determine whether pro-BNP levels in the acute phase of stroke predict the development of atrial fibrillation (AF) in patients with cryptogenic stroke. Methods: A prospective study including patients with cryptogenic stroke was conducted. Demographic data, medical history, and stroke characteristics were assessed at admission. A blood sample was obtained within the first 24 hours from stroke onset to determine pro-BNP levels. Patients were followed by a neurologist at 3 and 6 months and later by a primary care physician for 2 years to evaluate the development of AF. Results: One thousand fifty patients with ischemic stroke were evaluated. Three hundred seventy-two patients (35%) had cryptogenic stroke. One hundred eight patients were excluded from the study, so 264 patients were valid for the analysis. AF was detected in 15 patients (5.6%) during the follow-up. Patients who developed AF were older, had hypertension more frequently, and showed higher levels of pro-BNP. In the logistic regression model, we found that pro-BNP ≥360 pg/mL was the only variable independently associated with the risk of developing AF (odds ratio 5.70, 95% confidence interval 1.11–29.29, p = 0.037). Conclusions: Pro-BNP ≥360 pg/mL increases by 5-fold the possibility of detecting AF during follow-up in patients with cryptogenic stroke.

'Costa da Morte' ataxia is spinocerebellar ataxia 36: clinical and genetic characterization

Spinocerebellar ataxia 36 has been recently described in Japanese families as a new type of spinocerebellar ataxia with motor neuron signs. It is caused by a GGCCTG repeat expansion in intron 1 of NOP56. Family interview and document research allowed us to reconstruct two extensive, multigenerational kindreds stemming from the same village (Costa da Morte in Galicia, Spain), in the 17th century. We found the presence of the spinocerebellar ataxia 36 mutation co-segregating with disease in these families in whom we had previously identified an ∼0.8 Mb linkage region to chromosome 20 p. Subsequent screening revealed the NOP56 expansion in eight additional Galician ataxia kindreds. While normal alleles contain 5–14 hexanucleotide repeats, expanded alleles range from ∼650 to 2500 repeats, within a shared haplotype. Further expansion of repeat size was frequent, especially upon paternal transmission, while instances of allele contraction were observed in maternal transmissions. We found a total of 63 individuals carrying the mutation, 44 of whom were confirmed to be clinically affected; over 400 people are at risk. We describe here the detailed clinical picture, consisting of a late-onset, slowly progressive cerebellar syndrome with variable eye movement abnormalities and sensorineural hearing loss. There were signs of denervation in the tongue, as well as mild pyramidal signs, but otherwise no signs of classical amyotrophic lateral sclerosis. Magnetic resonance imaging findings were consistent with the clinical course, showing atrophy of the cerebellar vermis in initial stages, later evolving to a pattern of olivo-ponto-cerebellar atrophy. We estimated the origin of the founder mutation in Galicia to have occurred ∼1275 years ago. Out of 160 Galician families with spinocerebellar ataxia, 10 (6.3%) were found to have spinocerebellar ataxia 36, while 15 (9.4%) showed other of the routinely tested dominant spinocerebellar ataxia types. Spinocerebellar ataxia 36 is thus, so far, the most frequent dominant spinocerebellar ataxia in this region, which may have implications for American countries associated with traditional Spanish emigration.

Using Leo Plus stent as flow diverter and endoluminal remodeling in endovascular treatment of intracranial fusiform aneurysms

Background and purpose Treatment of intracranial fusiform aneurysms is complex and controversial, and is associated with high morbidity and mortality rates. The goal of this study was to evaluate the strategy of stent only therapy. Materials and methods A retrospective analysis of 20 patients with 20 intracranial fusiform aneurysms undergoing endovascular treatment using stent only therapy was undertaken. Feasibility, safety, and angiographic follow-up were evaluated. Results 28 Leo Plus stents were successfully deployed in 20 patients. No technical difficulties in relation to stent navigation, placement, or detachment from the delivery system were observed. A 6 month, 1 year, and 2 year follow-up angiograms were performed in all patients. 15 aneurysms showed complete occlusion and five partial stable occlusion. During the follow-up period, there was no evidence of in-stent stenoses or occlusion of the incorporated branching vessels. In this study, no patient presented with subarachnoid hemorrhage or died during follow-up. Conclusions Stent only therapy in this study proved very useful as a flow diverter for the treatment of intracranial fusiform aneurysms in which parent artery occlusion or stenting–coiling was not feasible.

Rasmussen encephalitis in the sixth decade: magnetic resonance image evolution and immunoglobulin response.

Case Report A 51-year-old male, with no relevant background, was admitted to the neurology ward after suffering two generalized motor seizures. In the previous days, he had symptoms suggesting auditory epileptic seizures, including some intermittent metallic tinnitus, like a bell ringing. Neuropsychological and neurological examination was unremarkable without any auditory and vestibular dysfunction signs. Laboratory studies, electrocardiography and plain-film chest X-ray were also normal. A cranial computed tomography (CT) scan displayed a hypodense left lateral temporal lesion ( fig. 1 ). CSF had 30 lymphocytes per cubic millimeter, and glucose and proteins were normal. CSF Gram stain and culture were negative. Borrelia, syphilis, HIV tests and herpes simplex virus polymerase chain reaction were negative as well. Angiotensin-converting enzyme and adenosine deaminase were within the normal range. The patient was put on phenytoin and intravenous acyclovir; seizures subsided and the patient became symptom free. Magnetic resonance imaging (MRI) displayed left lateral temporal hyperintense lesion on T 2 -weighted images, fluid attenuation inversion recovery (FLAIR) and diffusion-weighted images (DWI) ( fig. 1 ), and no gadolinium enhancement Dear Sir, Rasmussen encephalitis (RE) is a rare neurological condition, usually suffered by children and adolescents. Refractory epileptic seizures with progressive neurological deterioration and brain atrophy are the main clinical manifestations. In most cases, this disorder involves only one cerebral hemisphere [1–4] . The first three cases of RE were reported by Theodore Rasmussen in 1958 at the Montreal Neurological Institute [1] . RE etiology remains elusive. It is widely accepted as a sort of focal autoimmune encephalitis, resulting in a particular epileptic encephalopathy. This hypothesis is based on several facts: (1) clinical resemblance to Russian spring-summer encephalitis of viral origin, which causes epilepsia partialis continua; (2) presence of lymphocyte inflammation and glial nodules in brain biopsy; (3) inconstant finding in serum and cerebrospinal fluid (CSF) of antibodies against subunit 3 of ionotropic glutamate receptor; (4) in some cases, good response to steroids, immunoglobulins and plasmapheresis [2–11] . We present a patient whose RE apparently began at age 51. We have focused on clinical findings (posterior hemispheric involvement with absence of epilepsia partialis continua), neuroimaging evolution, brain biopsy and good response to intravenous inmunoglobulins. Received: March 1, 2006 Accepted: July 26, 2006 Published online: October 20, 2006

The autoscopic phenomena in neurological clinic: A study of two cases

Autoscopic phenomena are part of the reduplicative misidentification syndromes. These disorders may be a manifestation of both neurological and psychiatric conditions and consist of a double perception of the own body. These phenomena have been attributed to a dysfunction of the nondominant gyrus angularis. Two cases with this symptom are described. Case 1: A female with previous history of multiple sclerosis presented with episodes in which she saw herself in her extrapersonal space. Holter EEG was normal. An MRI scan showed multiple T2 hyperintensities. She was treated with carbamazepine and the symptom subsided. Case 2: A man presented with visual loss in the left field and episodic visualization of his own image. Examination confirmed left homonymous hemianopia. Serum glucose was 417 mg/dL. MRI was normal. Glycemia correction made the autoscopia disappear. In case 1, ephaptic transmission is proposed as the pathogenic mechanism and in the case 2, transient glucose toxicity is likely to explain the autoscopic phenomena.

Recurrent leukoencephalopathy with microhemorrhages: gradient-echo MRI study diagnostic value in CNS primary angiitis

Diagnosis of primary angiitis of the central nervous system (PACNS) is difficult in relation to variability in its clinical manifestations and absence of specific signs in neuroimaging. A young patient presented with a recurrent encephalopathic clinical course. T2 and fluid-attenuated inversion recovery-magnetic resonance imaging (FLAIR-MRI) showed hyperintense lesions in the cerebral white matter suggesting demyelination. Those lesions decreased or even disappeared after treatment with steroids and immunoglobulins. In echo gradient MRI (T2*-MRI), there were permanent cortical-subcortical petechial hypointense lesions (microhemorrhages). Definite diagnosis was established after cerebral biopsy. Intravenous cyclophosphamide was administrated with no new relapses in more than 18 months of follow-up. In a compatible clinical course, the finding of petechial hemorrhages in T2*-WI could play an important role in early diagnosis of PACNS.

Substraction acetazolamide SPECT co-registered to MRI in moyamoya disease: quantitative cerebrovascular reserve map.

A 48-year-old-woman suffering from recurrent left hemipalsy was referred to the hospital. MRI showed hyperintense lesions in periventricular white matter. Cerebral angiography revealed stenosis in the bilateral internal carotid and hypertrophic collateral vessels at the brain base, compatible with moyamoya syndrome. A visual comparison between perfusion SPECT with and without acetazolamide is employed in evaluation of these patients. Nevertheless, this traditional evaluation has some drawbacks associated with the variability between the 2 SPECT images and the lack of anatomical information. We propose a quantitative method based on the realigment, normalization, substraction, and co-registration of the 2 perfusion SPECT with MRI.

Late onset multiple sclerosis.

Abstract Introduction Late onset multiple sclerosis (LOMS) is an unusual entity, poorly characterized and difficult to diagnose. Objective To study a series of patients with LOMS (presentation of the first symptom of disease after the age of 50 years). Patients and methods In this retrospective study we review demographic characteristics, first onset symptom, diagnostic delay, disability at the time of diagnosis (EDSS), disease course and findings in CSF, VEP and MRI studies. Results We included 18 patients (12 F and 6 M) with LOMS (4.8% of the total). The most frequent first symptoms were motor deficits (33%), multisystem deficits (33%) and cerebellum disorder (16%). Clinical course (all the cases with a minimal follow-up of 5 years after the diagnosis): primary progressive-MS (62%), secondary progressive MS (22%), relapsing-remitting MS (16%). The initial EDSS score was higher than 4 points in one third of patients and diagnosis delay was over 5 years in two thirds of cases. The cerebral MRI study was abnormal and compatible with MS in all patients and fulfilled the Barkhof criteria in 12 cases (67%). IgG oligoclonal bands were positive in 64% of patients in the CSF study and VEP were abnormal in 73%. The most frequent wrong diagnoses were cerebrovascular disorders and spondyloarthritic cervical myelopathy. Conclusions LOMS course is often primary, progressive and motor and multisystem symptoms are the most frequent. The diagnosis is usually delayed and when it is made patients have a high disability score. The findings of cerebral and spinal MRI, CSF and VEP studies are of high diagnostic yield. Cerebrovascular disorders and spondyloarthritic cervical myelopathy are the most important entities in the differential diagnosis of LOMS.

Numb ears in resurrection: Great auricular nerve injury in hanging attempt

Hanging is described as incomplete (near-hanging) when some part of the body (usually the feet) is still in contact with the ground.1 The possible causes of death due to hanging are cardiac arrest (pressure on the carotid bodies), mechanical obstruction of the airways with cerebral hypoxia, intracranial hypertension (pressure on jugular veins), and spinal cord injury.1,2 We report two cases of near-hanging with a peculiar clinical manifestation derived …

Familial hemiplegic migraine with prolonged global aura: Follow-up findings of subtraction ictal SPECT co-registered to MRI (SISCOM)

All authors contributed equally to this work. Susana Arias-Rivas wrote the main paper. Manuel Rodriguez-Yáñez investigated the supplementary data. Julia Cortés and Pablo Aguiar performed the nuclear image studies and analysed and described the neuroimaging results. María Pardo, Rogelio Leira and Jose Castillo jointly conceived the study, followed the patient and prepared the manuscript. Miguel Blanco discussed the results. All authors discussed the results and implications and commented on the manuscript at all stages.