Sergio Eiji Ono

Adult cerebellar medulloblastoma: CT and MRI findings in eight cases

Medulloblastoma is a brain tumor of neuroepithelial origin, which represents 15 to 30% of all pediatric brain tumors, and less than 1% of CNS adult neoplasms. We report the imaging findings of 8 adult patients with medulloblastoma. The mean age was 35 years, ranging from 20 to 65 years, and the male:female rate was 3:5. The tumors were predominantly lateral (63%), hyperdense on CT scans (83%), and on the MRI, hypointense on T1 (100%) and hyperintense on T2 (80%) weighted images. It was seen intratumoral necrosis and cysts in six cases and calcifications in three. Hydrocephalus was observed in 5 cases and brain stem invasion in four. The imaging findings of medulloblastomas in adults are different of those in child, and also nonspecific. Although these tumors are uncommon in adults, they must be considered in the differential diagnosis of cerebellar masses in the posterior fossa of this age group.

Adrenoleucodistrofia ligada ao X: correlação entre o escore de Loes e parâmetros do tensor de difusão

Objective The present study was aimed at evaluating the correlation between diffusion tensor imaging parameters and Loes score as well as whether those parameters could indicate early structural alterations. Materials and Methods Diffusion tensor imaging measurements were obtained in 30 studies of 14 patients with X-linked adrenoleukodystrophy and were correlated with Loes scores. A control group including 28 male patients was created to establish agematched diffusion tensor imaging measurements. Inter- and intraobserver statistical analyses were undertaken. Results Diffusion tensor imaging measurements presented strong Pearson correlation coefficients (r) of –0.86, 0.89, 0.89 and 0.84 for fractional anisotropy and mean, radial and axial diffusivities (p < 0.01). Analysis of changes in diffusion tensor measurements at early stage of the disease indicates that mean and radial diffusivities might be useful to predict the disease progression. Conclusion Measurements of diffusion tensor parameters can be used as an adjunct to the Loes score, aiding in the monitoring of the disease and alerting for possible Loes score progression in the range of interest for therapeutic decisions.Objective: The present study was aimed at evaluating the correlation between diffusion tensor imaging parameters and Loes score as well as whether those parameters could indicate early structural alterations. Materials and Methods: Diffusion tensor imaging measurements were obtained in 30 studies of 14 patients with X-linked adrenoleukodystrophy and were correlated with Loes scores. A control group including 28 male patients was created to establish agematched diffusion tensor imaging measurements. Inter- and intraobserver statistical analyses were undertaken. Results: Diffusion tensor imaging measurements presented strong Pearson correlation coefficients (r) of –0.86, 0.89, 0.89 and 0.84 for fractional anisotropy and mean, radial and axial diffusivities (p < 0.01). Analysis of changes in diffusion tensor measurements at early stage of the disease indicates that mean and radial diffusivities might be useful to predict the disease progression. Conclusion: Measurements of diffusion tensor parameters can be used as an adjunct to the Loes score, aiding in the monitoring of the disease and alerting for possible Loes score progression in the range of interest for therapeutic decisions.

Esclerose sistêmica progressiva: aspectos na tomografia computadorizada de alta resolução

OBJECTIVE: To describe the high-resolution computed tomography findings in the lung of patients with systemic sclerosis, independently of the respiratory symptoms. MATERIALS AND METHODS: Seventy-three high-resolution computed tomography scans of 44 patients with clinical diagnosis of systemic sclerosis were reviewed and defined by the consensus of two radiologists. RESULTS: Abnormalities were seen in 91.8% (n = 67) of the scans. The most frequent findings were reticular pattern (90.4%), ground-glass opacities (63%), traction bronchiectasis and bronchiolectasis (56.2%), esophageal dilatation (46.6%), honeycombing pattern (28.8%) and signs of pulmonary hypertension (15.6%). In most cases the lesions were bilateral (89%) and symmetrical (58.5%). The lesions were predominantly located in the basal (91.2%) and peripheral (92.2%) regions. CONCLUSION: In the majority of the patients, progressive systemic sclerosis can cause pulmonary fibrosis mainly characterized by reticular pattern with basal and peripheral distribution on high-resolution computed tomography.

Oculocerebrorenal syndrome of Lowe: magnetic resonance imaging findings in the first six years of life

Dr. Sergio Eiji Ono – Rua Brigadeiro Franco 122 80430-120 Curitiba PR Brasil. E-mail: ono.sergio@gmail.com The oculocerebrorenal syndrome of Lowe (OCRL), was first recognized as a distinct disease in 1952 by Drs. Lowe, Terrey and MacLachlan at Massachusetts General Hospital, in Boston, USA, describing three male children with organic aciduria, decreased renal ammonia production, hydrophtalmos and mental retardation. The X-linked recessive inheritance pattern was recognized first by LeFebvre. It is present in all races, with a predominance in those of Caucasian and Asian ancestries. Rarely females are affected. It is a very rare disease, with estimated prevalence in the general population of 1 in 500,000. In USA the Lowe Syndrome Association (LSA) documented 190 living patients in the year 2000 (0.67 × million inhabitants). It is caused by a mutation in the gene encoding oculocerebrorenal-Lowe protein (OCRL1), isolated in 1992, linked to the Xq24–q26 region of the X chromosome. Approximately 60% of OCRL patients demonstrate a loss of OCRL1 gene expression, and the definitive laboratory test, that can be used for prenatal diagnosis, is the biochemical assay for deficiency of phosphatidylinositol 4,5biphosphate 5-phosphate in cultured fibroblasts. The classic triad of eye, central nervous system, and kidney involvement are required for the diagnosis of Lowe’s syndrome. Cataract is present at birth in all patients and glaucoma is detected within the first year of life. Hypotonia compromises suction and causes serious respiratory problems in the first period of life. Motor development is retarded and mental retardation is moderate or severe in almost all cases. Obsessivecompulsive behavior is typical. Seizure is seen in approximately 50% of the patients over 18 years old. Renal disease is primarily characterized by renal Fanconi syndrome but many children are asymptomatic at birth. Renal involvement is initially related to bicarbonate, salt and water wasting, causing failure to thrive. Later, a significant number of patients develop chronic renal failure. The treatment includes cataract extraction, glaucoma control, physical and speech therapy, drugs addressed to behavioral problems and correction of the renal (tubular acidosis) and consequent bone diseases (rickets). Life span rarely exceeds 40 years. Brain magnetic resonance imaging (MRI) may show two patterns of lesions: hyperintensities on T2-weighted images, and periventricular cystic lesions. The present case reports these findings in a time interval of 4 years, showing that initially the hyperintensities are seen, and afterwards, the cystic images. This pattern would help the radiologist and pediatric neurologist to reinforce the clinical diagnosis, as these patterns of images can be seen in other conditions.

Bilateral narrow duplication of the internal auditory canal.

AIM To demonstrate diagnostic imaging of an extremely rare presentation of bilateral narrow duplication of the internal auditory canal. CASE REPORT An adolescent boy with profound sensorineural hearing loss presented for hearing rehabilitation. Imaging studies (i.e. multidetector computed tomography and magnetic resonance imaging) clearly demonstrated bilateral duplication of the internal auditory canals, with narrowing of the lower canals, unilateral cochlear and vestibular dysplasia, bilateral superior semicircular canal malformation, and bilateral absence of the posterior semicircular canals. CONCLUSION To our knowledge, this is only the third such case described in the literature. Considering that the vestibulocochlear nerve has been unable to be demonstrated in almost all cases of duplicated internal auditory canal (unilateral and bilateral), our case supports the hypothesis that vestibulocochlear nerve aplasia or hypoplasia leads to internal auditory canal stenosis. We consider this rare presentation of bilateral narrow duplication of the internal auditory canal to represent a contraindication for cochlear implantation.

Tomografia computadorizada de alta resolução nas complicações pulmonares pós-transplante de medula óssea: ensaio iconográfico

O transplante de medula ossea (TMO) tem sido utilizado como tratamento de escolha para diversas doencas hematologicas. Entretanto, as complicacoes pulmonares, que podem ocorrer em ate 60% dos pacientes, sao o principal motivo de falha no tratamento. As complicacoes pulmonares pos-TMO podem ser divididas em tres fases, de acordo com a imunidade do paciente. Na primeira fase, ate 30 dias apos o procedimento, predominam as complicacoes nao infecciosas e as pneumonias fungicas. Na fase precoce, que vai ate o 100o dia pos-TMO, as infeccoes virais, principalmente por citomegalovirus, sao mais comuns. Finalmente, na fase tardia pos-TMO, complicacoes nao infecciosas como bronquiolite obliterante com pneumonia em organizacao e doenca do enxerto contra o hospedeiro sao mais comumente observadas. Os autores apresentam um ensaio iconografico, enfatizando os aspectos de tomografia de alta resolucao em pacientes com complicacoes pulmonares pos-TMO.

MRI hydrographic 3D sequences in CADASIL

Two patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) (figure), evolving with cognitive deterioration, had MRI. Hydrographic 3D high-resolution turbo spin-echo (TSE) with variable flip angle sequence (SPACE) was performed to demonstrate the subcortical lacunar lesions (SLLs) (figure, A and B) considered specific in CADASIL.1

Calcificações intracranianas maciças em um paciente com lúpus eritematoso sistêmico

Central nervous system involvement is frequently reported in patients with systemic lupus erythematosus. Computed tomography and magnetic resonance imaging studies usually show brain atrophy, cerebral infarction and/or intracranial bleeding. Extensive intracranial calcification in patients with systemic lupus erythematosus is rare. We report a case of a patient with systemic lupus erythematosus who presented with seizures and massive basal ganglia calcification and mild calcifications in the frontal lobes, seen on the brain computed tomography scan. Magnetic resonance imaging showed hyperintensity on FLAIR images and hypointense signals on T2* gradient echo images in the basal ganglia.

Malformation of the brainstem accompanied by cortical dysplasia

http://dx.doi.org/10.1590/0100-3984.2016.0222 Patricia Pitta de Abreu1, Bernardo Carvalho Muniz1, Nina Ventura1, Emerson Gasparetto1, Edson Marchiori2 1. Instituto Estadual do Cérebro Paulo Niemeyer, Rio de Janeiro, RJ, Brazil. 2. Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil. Mailing address: Dra. Patricia Pitta de Abreu. Instituto Estadual do Cérebro Paulo Niemeyer. Rua do Rezende, 156, Centro. Rio de Janeiro, RJ, Brazil, 20230-024. E-mail: papitta@gmail.com. intraventricular gangliogliomas can originate in the lateral ventricles, in the third ventricle, and fourth ventricles—some even originating in the choroid plexus—and should always be included in the differential diagnosis of intraventricular lesions. The case presented here was one of an intraventricular ganglioglioma apparently originating in the third ventricle, extending to the lateral ventricles and the fourth ventricle, the histopathological diagnosis being WHO grade I ganglioglioma with signs of CSF dissemination during subsequent examinations. In conclusion, a diagnosis of ganglioglioma should be considered in the presence of intraventricular lesions. In addition, imaging of the neuroaxis is recommended, regardless of the histopathological grade of the lesion, because CSF dissemination has been reported in the monitoring of other low-grade tumors, including gangliogliomas.

Treatment of West syndrome with vigabatrin: Reversible MRI signal changes

1. Dracopoulos A, Widjaja E, Raybaud C, Westall CA, Snead OC. Vigabatrin-associated reversible MRI signal changes in patients with infantile spasms. Epilepsia 2010;51:1297-1304.2. Pearl PL, Vezina LG, Saneto RP, et al. Cerebral MRI abnormalities associated with vigabatrin therapy. Epilepsia 2009; 50:184-194.3. Thelle T, Gammelgaard L, Hansen JK, Ostergaard JR. Reversible magnetic resonance imaging and spectroscopy abnormalities in the course of vigabatrin treatment for West syndrome. Eur J Paediatric Neurology 2011; 15:260-264. 4. Simao GN, Zarei Mahmoodabadi S, Snead OC, Go C, Widjaja E. Abnormal axial diffusivity in the deep gray nuclei and dorsal brain stem in infantile spasm treated with vigabatrin. AJNR Am J Neuroradiol 2011;32:199-203.