Celi Santos Andrade

Toxic Leukoencephalopathies, Including Drug, Medication, Environmental, and Radiation-Induced Encephalopathic Syndromes

Toxic leukoencephalopathies can be secondary to the exposure to a wide variety of exogenous agents, including cranial irradiation, chemotherapy, antiepileptic agents, drugs of abuse, and environmental toxins. There is no typical clinical picture, and patients can present with a wide array of signs and symptoms. Involvement of white matter is a key finding in this scenario, although in some circumstances other high metabolic areas of the central nervous system can also be affected. Magnetic resonance (MR) imaging usually discloses bilateral and symmetric white matter areas of hyperintense signal on T2-weighted and fluid-attenuated inversion recovery images, and signs of restricted diffusion are associated in the acute stage. In most cases, the changes are reversible, especially with prompt recognition of the disease and discontinuation of the noxious agent. Either the MR or clinical features may be similar to several nontoxic entities, such as demyelinating diseases, leukodystrophies, hepatic encephalopathy, vascular disease, hypoxic-ischemic states, and others. A high index of suspicion should be maintained whenever a patient presents recent onset of neurologic deficit, searching the risk of exposure to a neurotoxic agent. Getting to know the most frequent MR appearances and mechanisms of action of causative agents may help to make an early diagnosis and begin therapy, improving outcome. In this review, some of the most important causes of leukoencephalopathies are presented; as well as other 2 related conditions: strokelike migraine attacks after radiation therapy syndrome and reversible splenial lesions.

Malformations of cortical development: current concepts and advanced neuroimaging review

Malformations of cortical development (MCD) result from disruptions in the complex process of the human brain cortex formation and are highly associated to severe epilepsy, neurodevelopmental delay and motor dysfunction. Nowadays, magnetic resonance imaging (MRI) is the cornerstone of the work-up of patients with epilepsy and modern advanced imaging techniques have improved not only our ability to detect and characterize cortical malformations, but also in identifying associated functional abnormalities that are far beyond the structural visualized lesions. Herein, we address the most currently used classifications of MCD and make a concise review of the embryological process of cortical development. Our main goal is to summarize recent advances and new trends in diagnostic imaging techniques concerning MCD. Thereafter, follows a brief discussion of specific disorders and their radiological features.

Phosphorus magnetic resonance spectroscopy in malformations of cortical development.

Purpose:  The aim of this study was to evaluate phospholipid metabolism in patients with malformations of cortical development (MCDs).

Widespread pH abnormalities in patients with malformations of cortical development and epilepsy: a phosphorus-31 brain MR spectroscopy study.

INTRODUCTION Neuroimaging studies demonstrate that not only the lesions of malformations of cortical development (MCD) but also the normal-appearing parenchyma (NAP) present metabolic impairments, as revealed with (1)H-MRS. We have previously detected biochemical disturbances in MCD lesions with phosphorus-31 magnetic resonance spectroscopy (31P-MRS). Our hypothesis is that pH abnormalities extend beyond the visible lesions. METHODS Three-dimensional 31P-MRS at 3.0 T was performed in 37 patients with epilepsy and MCD, and in 31 matched-control subjects. The patients were assigned into three main MCD subgroups: cortical dysplasia (n=10); heterotopia (n=14); schizencephaly/polymicrogyria (n=13). Voxels (12.5 cm3) were selected in five homologous regions containing NAP: right putamen; left putamen; frontoparietal parasagittal cortex; right centrum semiovale; and left centrum semiovale. Robust methods of quantification were applied, and the intracellular pH was calculated with the chemical shifts of inorganic phosphate (Pi) relative to phosphocreatine (PCr). RESULTS In comparison to controls and considering a Bonferroni adjusted p-value <0.01, MCD patients presented significant reduction in intracellular pH in the frontoparietal parasagittal cortex (6.985±0.022), right centrum semiovale (7.004±0.029), and left centrum semiovale (6.995±0.030), compared to controls (mean values±standard deviations of 7.087±0.048, 7.096±0.042, 7.088±0.045, respectively). Dunnet and Dunn tests demonstrated that the differences in pH values remained statistically significant in all MCD subgroups. No significant differences were found for the putamina. CONCLUSION The present study demonstrates widespread acidosis in the NAP, and reinforces the idea that MCD visible lesions are only the tip of the iceberg.

Características de imagem na ressonância magnética de gossipiboma intracraniano: relato de caso e revisão da literatura

Many reports in the literature have described findings of gossypibomas at conventional radiography and computed tomography. However, magnetic resonance imaging findings are still to be completely known. The purpose of the present study was to describe imaging findings of gossypibomas in a patient with previous history of brain surgery, presenting fever and mental confusion.

Extratemporal abnormalities in phosphorus magnetic resonance spectroscopy of patients with mesial temporal sclerosis

OBJECTIVE We evaluated extratemporal metabolic changes with phosphorus magnetic resonance spectroscopy (31P-MRS) in patients with unilateral mesial temporal sclerosis (MTS). METHOD 31P-MRS of 33 patients with unilateral MTS was compared with 31 controls. The voxels were selected in the anterior, posterior insula-basal ganglia (AIBG, PIBG) and frontal lobes (FL). Relative values of phosphodiesters- PDE, phosphomonoesters-PME, inorganic phosphate - Pi, phosphocreatine- PCr, total adenosine triphosphate [ATPt = γ- + a- + b-ATP] and the ratios PCr/ATPt, PCr/γ-ATP, PCr/Pi and PME/PDE were obtained. RESULTS We found energetic abnormalities in the MTS patients compared to the controls with Pi reduction bilaterally in the AIBG and ipsilaterally in the PIBG and the contralateral FL; there was also decreased PCr/γ-ATP in the ipsilateral AIBG and PIBG. Increased ATPT in the contralateral AIBG and increased γ-ATP in the ipsilateral PIBG were detected. CONCLUSION Widespread energy dysfunction was detected in patients with unilateral MTS.

DTI-based tractography of the arcuate fasciculus in patients with polymicrogyria and language disorders

OBJECTIVES To assess the integrity of the arcuate fasciculus (AF) with diffusion tensor imaging (DTI) and tractography in patients with congenital polymicrogyria (PMG) and language disorders. METHODS Twelve patients with PMG and 12 matched controls were prospectively evaluated with DTI (32 gradient encoding directions, b-value=1000 s/mm(2)) at 3.0T. The AF was virtually dissected with a deterministic streamline approach. DTI metrics included FA (fractional anisotropy), mean diffusivity (MD), axial diffusivity (AD) and radial diffusivity (RD). A subset of patients (n=4) was evaluated to assess cognitive performance and language skills. RESULTS Qualitative evaluation revealed several abnormalities in tracts size and architecture in nearly all PMG patients. Remarkably, in 3 patients with bilateral PMG, the AF was not delineated on both hemispheres. In comparison to controls, patients exhibited significant decrease of FA (p=0.003) in addition to increase of RD (p=0.03) in the right AF, whereas there was significant increase of MD in the left AF (p=0.04). All 4 patients with language evaluation had suboptimal performance on lexical fluency and prosodic linguistic. CONCLUSIONS DTI and tractography suggest that the AF is severely disrupted in patients with PMG, providing an anatomical in vivo substrate for the language disorders commonly associated with these cortical malformations.

Corpus callosum diffusion abnormalities in refractory epilepsy associated with hippocampal sclerosis

OBJECTIVES To detect by diffusion tensor imaging (DTI) the extent of microstructural integrity changes of the corpus callosum (CC) in patients with hippocampal sclerosis (HS) and to evaluate possible association with clinical characteristics. METHODS Fourty-two patients with temporal lobe epilepsy (TLE) and HS and 30 control subjects were studied with DTI. We grouped patients according to lesion side (left or right) HS. Mean diffusivity (MD), fractional anisotropy (FA), radial (RD) and axial diffusivity (AD) were extracted from five segments in CC midsagittal section obtained by automatic segmentation. CC DTI findings were compared between groups. We also evaluated association of DTI changes and clinical characteristics. RESULTS HS patients displayed decreased FA and increased MD and RD in the anterior, mid-posterior and posterior CC segments, compared to controls. No differences were observed in AD. Patients reporting febrile seizure as the initial precipitating event presented more intense diffusion changes. No differences were seen comparing left and right HS. Age at epilepsy onset, disease duration and seizure frequency were not associated with DTI findings. CONCLUSIONS This is one of the largest series of TLE-HS patients evaluating CC white matter fiber integrity by DTI, which allowed us to study how some clinical characteristics, such as seizure frequency, disease duration and lesion side, are related to CC integrity. Occurrence of febrile seizure was the only factor that had significant impact on tract integrity. Diffusion changes were not restricted to the posterior part of the CC; we observed the same changes for the anterior part of the CC. Diffusion changes were characterized by an increase in RD, while the AD remained intact for all regions of the CC.

Dural amyloidoma An unusual presentation of CNS amyloidosis

A 46-year-old previously healthy woman presented with recurring left frontal and occipital headache. A brain MRI demonstrated diffuse dural thickening (figure 1, top). CSF analysis showed no abnormalities. A meningeal biopsy had pathology typical of an amyloid tumor (figure 2). Further investigations did not reveal evidence of plasma-cell tumor, multiple myeloma, systemic amyloidosis, underlying inflammatory disorder, or malignancy. Follow-up brain MRIs revealed regression of the pachymeningeal disease after treatment with corticosteroids and radiotherapy (figure 1, bottom).

Massive fatal cerebral air embolism as a negative contrast angiogram

Cerebral gas embolism is a rare and potentially fatal event that may occur as a complication of medical procedures and trauma. Radiologic diagnosis is remarkable and favours immediate treatment. We present an unusual case of massive fatal cerebral air embolism, diagnosed with multidetector-row computed tomography (MDCT), where final appearance resembled a negative contrast angiogram. A female patient born with a meningomyelocele developed chronic renal insufficiency. She underwent kidney transplantation from a cadaver donor when she was 20 years old. The patient presented with persistent haematuria and haemoptysis 6 months after the procedure. A chest CT scan demonstrated multiple lung …