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Case presentation A 29 year-old woman presented to the emergency with gait imbalance and dysarthria. At admission, neurologic examination revealed normal cognition, ataxia, dysarthria, dysmetria on both sides of the body, bilateral vertical nystagmus and loss of the lateral eye movement. Blood examination was notable for a increase in WBC count and demonstrated erythrocyte sedimentation rate of 18 mm/h. Examination of cerebral spinal fluid (CSF) revealed a protein concentration of 166 mg/ dL, a glucose concentration of 56 mg/dL, and pleocytosis. Serum glucose concentration was 126 mg/dL. The patient had no history of immunosuppression or another comorbidity and anti-HIV test was negative. Neurological evaluation included a head computed tomography (CT) scan which revealed normal findings. An MRI of the brain revealed bilateral increased signal intensity in the cerebellum on fluid-attenuated inversion recovery images (FLAIR)/T2, without contrast enhancement, suggesting an inflammatory process confined to the cerebellum (Figure 1 and 2). Furthermore, the cerebellar cortex appeared swollen, a finding consistent with diffuse cerebellitis. There were no alterations in the brainstem. Initially, the possibility of bacterial rhomboencephalitis caused by Listeria monocytoges was considered, since it is the most commom cause of rhomboencephalitis. After a few days with antibiotic therapy (ceftriaxone and ampicillin), polymerase chain reaction (PCR) test of the CSF was positive for Herpes Simplex Virus 1/2 (HSV). Bacterial culture of CSF samples showed no growth, and the results of Gram staining of CSF were negative. Anti-Listeria antibody was also negative and ampicillin discontinued. CSF PCR analysis for other herpesviruses (varicella-zoster virus, Epstein-Barr virus, cytomegalovirus, and human herpesvirus types 6–8) and enteroviruses were also negative. Upon treatment with acyclovir (50 mg/kg/day) during 21 days, symptoms improved. One month later after the first MRI, a significantly reduce of imaging abnormalities was detected (Figure 3).

Mucopolysaccharidosis (MPS) is an inherited metabolic disease and a member of the group of lysosomal storage disorders. Its hallmark is a deficiency of lysosomal enzymes involved in the degradation of mucopolysaccharides, also known as glycosaminoglycans (GAGs). The products of GAG degradation accumulate within lysosomes and in the extracellular space, thereby interfering with the degradation of other macromolecules. This process leads to chronic degeneration of cells, which in turn affects multiple organs and systems. There are seven distinct types of MPS (I, II, III, IV, VI, VII, and IX), which are divided into subtypes according to the deficient enzyme and the severity of the clinical picture. Although clinical manifestations vary considerably among the different types of MPS, the central nervous system (CNS) is characteristically affected, and magnetic resonance (MR) imaging is the method of choice to evaluate brain and spinal cord abnormalities. Enlarged perivascular spaces, white matter lesions, hydrocephalus, brain atrophy, cervical spinal canal stenosis with or without spinal cord compression and myelopathy, and bone abnormalities in the skull and spine (dysostosis multiplex) are typical imaging findings described in the literature and reviewed in this article. The differential diagnosis of MPS is limited because the constellation of imaging findings is highly suggestive. Thus, radiologists should be aware of its typical neuroimaging findings so they can recognize cases not yet diagnosed, exclude other metabolic diseases, monitor CNS findings over time, and assess treatment response. (©)RSNA, 2016.

The term rhombencephalitis refers to inflammatory diseases affecting the hindbrain (brainstem and cerebellum). Rhombencephalitis has a wide variety of etiologies, including infections, autoimmune diseases, and paraneoplastic syndromes. Infection with bacteria of the genus Listeria is the most common cause of rhombencephalitis. Primary rhombencephalitis caused by infection with Listeria spp. occurs in healthy young adults. It usually has a biphasic time course with a flu-like syndrome, followed by brainstem dysfunction; 75% of patients have cerebrospinal fluid pleocytosis, and nearly 100% have an abnormal brain magnetic resonance imaging scan. However, other possible causes of rhombencephalitis must be borne in mind. In addition to the clinical aspects, the patterns seen in magnetic resonance imaging can be helpful in defining the possible cause. Some of the reported causes of rhombencephalitis are potentially severe and life threatening; therefore, an accurate initial diagnostic approach is important to establishing a proper early treatment regimen. This pictorial essay reviews the various causes of rhombencephalitis and the corresponding magnetic resonance imaging findings, by describing illustrative confirmed cases.

Hematopoietic stem cell transplantation (HSCT) is the only available treatment for the neurological involvement of disorders such as late-onset metachromatic leukodystrophy (MLD), mucopolysaccharidosis type I-Hurler (MPS-IH), and X-linked cerebral adrenoleukodystrophy (CALD). Objective To describe survival and neurological outcomes after HSCT for these disorders. Methods Seven CALD, 2 MLD and 2 MPS-IH patients underwent HSCT between 2007 and 2014. Neurological examinations, magnetic resonance imaging, molecular and biochemical studies were obtained at baseline and repeated when appropriated. Results Favorable outcomes were obtained with 4/5 related and 3/6 unrelated donors. Two patients died from procedure-related complications. Nine transplanted patients were alive after a median of 3.7 years: neurological stabilization was obtained in 5/6 CALD, 1/2 MLD, and one MPS-IH patient. Brain lesions of the MPS-IH patient were reduced four years after HSCT. Conclusion Good outcomes were obtained when HSCT was performed before adulthood, early in the clinical course, and/or from a related donor. RESUMO O transplante de células tronco hematopoiéticas (TCTH) é o único tratamento disponível para o envolvimento neurológico de doenças como a leucodistrofia metacromática (MLD), a mucopolissacaridose tipo I-Hurler (MPS-IH) e a adrenoleucodistrofia (CALD). Objetivos Descrever a sobrevida e os desfechos neurológicos após o TCTH nessas doenças. Métodos Sete pacientes CALD, 2 MLD e 2 MPS-IH realizaram TCTH entre 2007 e 2014. Avaliações neurológicas, ressonância nuclear magnética e estudos bioquímicos e moleculares foram feitos no baseline e repetidos quando apropriado. Resultados Desfechos favoráveis foram obtidos em 4/5 TCTH de doadores relacionados e em 3/6 não relacionados. Dois pacientes faleceram de complicações do procedimento. Nove transplantados sobreviveram após uma mediana de 3,7 anos: estabilização neurológica foi obtida em 5/6 CALD, ½ MLD e em um caso MPS-IH. As lesões encefálicas de um caso MPS-IH reduziram-se quatro anos após o TCTH. Conclusão Bons desfechos foram obtidos quando o TCTH foi feito antes da vida adulta, cedo no curso clínico e/ou a partir de um doador relacionado.

Hydrocephalus can be defined as a process in which the cerebrospinal fluid compartments are actively enlarged at the expense of brain tissue. The clinical presentation depends on the age of the patient. Hydrocephalus is classified as either obstructive, when the drainage pathways is occluded, or communicating, when no clear obstruction can be demonstrated. It can be evaluated using ultrasound, computed tomography, and magnetic resonance imaging. The latter is the best imaging tool to characterize the cause and extension of hydrocephalus and treatment complications. Treatment depends on cause, patient age, and rapidity of onset of the symptoms.

The clinical diagnosis of acute stroke is inaccurate approximately 10%-30% of the time, which can lead to unnecessary administration of thrombolytic therapy or delays in appropriate therapy. Rapid and accurate neuroimaging triage is essential to guide therapy and exclude mimics. Although many conditions that mimic stroke clinically have imaging appearances that can overlap acute stroke, these conditions can be differentiated in most cases by using a careful pattern-based approach. We describe a case of 67 yo male patient who had a clinic of wakeup stroke and at the first magnetic resonance image (MRI) it was found that was an acute stroke of middle cerebral artery.The patient did not improve and a second MRI revelead a two times growth of the lesion, and the MRI findings were compatible with tumor. At the surgery they found a infiltrative lesion and the anatomopathological exam showed that it was a high grade glioma.The diagnosis of ischemic stroke is often straight forward; however, the clinical diagnosis of acute stroke is inaccurate in many cases. Furthermore, many of these conditions, such as encephalitis, mass lesions, seizures, hypoglycemia, transient global amnesia (TGA),demyelinating disease, drug toxicity, and metabolic disturbances, have imaging appearances that can mimic acute or subacute infarction; however, an accurate diagnosis can often be made by using a pattern-based approach.

Editors' Note: Kaiwar questions the relevance of an article cited by Fernandes et al. on the lentiform fork sign, which was initially reported to be associated with metabolic acidosis. Fernandes et al. clarify their point, noting that the sign occurred in their metformin-treated patient who did not have metabolic acidosis and resolved following discontinuation of metformin. Akula disagrees with the conclusion of the Vigabatrin Retinal Toxicity Observational Study, doubting that ERG could effectively monitor vigabatrin-induced retinal damage. Authors Westall and Wright clarify their …

A 63-year-old woman with type 2 diabetes mellitus on hemodialysis presented with subacute onset of slurred speech and difficulty walking after 1 week of inadvertent metformin use.