Progressive multifocal leukoencephalopathy: a challenging diagnosis established at autopsy

Progressive multifocal leukoencephalopathy (PML) is a feared entity that occurs most frequently in conditions of extreme immunodeficiency. The diagnosis is often made long after the onset of symptoms due to the physicians' unfamiliarity, and the unavailability of diagnostic tests in some medical centers. Although the incidence of PML is decreasing among HIV patients with the advent of highly active antiretroviral therapy (HAART), in Brazil this entity is the fourth highest neurological complication among these patients. The authors present the case of a middle-aged man who tested positive for HIV concomitantly with the presentation of hyposensitivity in the face and the right side of the body, accompanied by mild weakness in the left upper limb. The clinical features worsened rapidly within a couple of weeks. The diagnostic work-up pointed to the working diagnosis of PML after brain magnetic resonance imaging; however, the detection of the John Cunningham virus (JCV) in the cerebral spinal fluid was negative. HAART was started but the patient died after 7 weeks of hospitalization. The autopsy revealed extensive multifocal patchy areas of demyelination in the white matter where the microscopy depicted demyelination, oligodendrocytes alterations, bizarre atypical astrocytes, and perivascular lymphocytic infiltration. The immunohistochemistry was positive for anti-SV40, and the polymerase chain reaction of the brain paraffin-embedded tissue was positive for JCV. The authors highlight the challenges for diagnosing PML, as well as the devastating outcome of PML among HIV patients.

Traumatic injury to the internal carotid artery by the hyoid bone: a rare cause of ischemic stroke

Central nervous system (CNS) ischemic events, besides being a common and devastating disease, are accompanied by severe disability and other morbidities. The cause of such events is not always that simple to diagnose, and among the young, a broad spectrum of possibilities should be considered. We present the case of a young man who presented two episodes of CNS ischemia with a 1 year gap between them, which occurred in the same situation while he was walking and carrying a heavy backpack. The second event first presented as a transient ischemic attack followed by a stroke the day after. The diagnostic work-up showed an indentation of the greater cornu of the hyoid bone over the internal carotid artery, which injured the media and intimal layers. At the arterial injury site, a micro thrombus was found, which explained the source of the embolic event to the CNS. The patient was operated on, and the procedure included the resection of the posterior horn of the hyoid bone, the resection of the injured segment of the internal carotid artery followed by carotid­carotid bypass with the great saphenous vein. The postoperative period and the recovery were uneventful as was the 5-month follow-up. We call attention to this unusual cause of stroke and present other cases reported in the literature.

Carotid intima-media thickness in the Brazilian Longitudinal Study of Adult Health (ELSA-Brasil): a narrative review

ABSTRACT BACKGROUND: Carotid intima-media thickness (CIMT), as measured by ultrasound, has been used in large studies as a non-invasive marker for subclinical atherosclerosis. The Brazilian Longitudinal Study of Adult Health (ELSA-Brasil) is a cohort of 15,105 civil servants in six Brazilian cities that included CIMT evaluation in its baseline assessment. The aim of the present narrative review was to provide an overview of ELSA-Brasil CIMT articles published up to July 31, 2017. DESIGN AND SETTING: Narrative review of ELSA-Brasil CIMT studies using baseline assessment data. METHODS: We searched PubMed for the terms "ELSA-Brasil" and "intima-media". This search yielded 21 published articles using CIMT data from the ELSA-Brasil baseline assessment, which were included in this review. We also present information about intima-media thickness assessment from ongoing onsite reevaluations of the study participants. RESULTS: Most published studies focused on the association with traditional and novel cardiovascular risk factors. Studies also presented information about the ELSA-Brasil CIMT protocol at baseline and CIMT value distribution in this large sample. CONCLUSIONS: Analyses on the ELSA-Brasil data led to important insights on CIMT interpretation and physiology. Besides the highlighted contributions which have already been made in this field, new data gathered during the ongoing third onsite assessment will enable investigation of substantially new research questions.

Migrainous infarction: a rare and often overlooked diagnosis

Migraine is a neurological entity and a well-known independent risk factor for cerebral infarction, which mostly afflicts the young female population. Researching focal neurological signs in this subset of the population with the diagnosis of a neurological ischemic event should always take into account the migraine as the etiology or as an associated factor. The etiology of central nervous system (CNS) ischemia is considerable. Migraine, although rare, also may be included in this vast etiological range, which is called migrainous infarction. In this setting, the diagnostic criteria required for this diagnosis is extensive. Herein, we present the case of a female adolescent who submitted to the emergency facility complaining of diplopia, dysarthria, and imbalance, which started concomitantly with a migrainous crisis with aura­a challenging clinical case that required extensive research to address all possible differential diagnoses.