[MRI in the diagnostic and evolutive control of subacute combined degeneration. A case report]. / RM en el diagnóstico y control evolutivo de la degeneración combinada subaguda. A propósito de un caso.

A deficit of vitamin B12, generally resulting from pernicious anaemia, can give rise to disorders of the spinal cord, brain, optic and peripheral nerves. The principal neurological syndrome is subacute combined degeneration of the spinal cord (SCD), which can cause progressive motor and/or sensitive alterations, instability and incontinency, due to the demyelination of the posterior horn of the spinal cord. The identification by magnetic resonance (MR) of signal hyperintensity in T2 weighted sequences at the level of the posterior horns of the spinal and/or cervical cord can be of great use in diagnosising the patient with SCD, above all when the symptoms are mild or nonspecific, and the patient does not have haematological or gastrointestinal alterations. Besides, the evolution of the altered signal of the posterior horns in MR can be of use in evaluating the efficacy of treatment, since their normalization is related to clinical improvement.

RM en el diagnóstico y control evolutivo de la degeneración combinada subaguda. A propósito de un caso / MRI in the diagnostic and evolutive control of subacute combined degeneration. A case report

El déficit de vitamina B12, consecuencia generalmente de la anemia perniciosa, puede dar lugar a trastornos de la médula espinal, cerebro, nervios ópticos y periféricos. El principal síndrome neurológico es la degeneración combinada subaguda de la médula (DCS), que puede causar alteraciones motoras y/o sensitivas progresivas, inestabilidad e incontinencia, debido a la desmielinización de los cordones posteriores de la médula. La identificación por resonancia magnética (RM)de una hiperintensidad de señal en secuencias potenciadas en T2 a nivel de los cordones posteriores de la médula cervical y/o dorsal, puede ser de gran utilidad en la orientación diagnóstica del paciente con DCS, sobre todo cuando los síntomas son leves o inespecíficos, y el paciente no tiene alteraciones hematológicas o gastrointestinales. Además, la evolución de la alteración de la señal del cordonal posterior en RM puede ser de utilidad para valorar la eficacia del tratamiento, ya que su normalización se relaciona con la mejoría clínica(AU)

A deficit of vitamin B12, generally resulting from pernicious anaemia, can give rise to disorders of the spinal cord, brain, optic and peripheral nerves. The principal neurological syndrome is subacute combined degeneration of the spinal cord (SCD), which can cause progressive motor and/or sensitive alterations, instability and incontinency, due to the demyelination of the posterior horn of the spinal cord. The identification by magnetic resonance (MR) of signal hyperintensity in T2weighted sequences at the level of the posterior horns of the spinal and/or cervical cord can be of great use in diagnosising the patient with SCD, above all when the symptoms are mild or nonspecific, and the patient does not have haematological or gastrointestinal alterations. Besides, the evolution of the altered signal of the posterior horns in MR can be of use in evaluating the efficacy of treatment, since their normalization is related to clinical improvement(AU)

The impact of silent vascular brain burden in cognitive impairment in Parkinson's disease.

BACKGROUND AND PURPOSE: White matter hyperintensities (WMHs) detected by magnetic resonance imaging (MRI) of the brain are associated with dementia and cognitive impairment in the general population and in Alzheimer's disease. Their effect in cognitive decline and dementia associated with Parkinson's disease (PD) is still unclear. METHODS: We studied the relationship between WMHs and cognitive state in 111 patients with PD classified as cognitively normal (n = 39), with a mild cognitive impairment (MCI) (n = 46) or dementia (n = 26), in a cross-sectional and follow-up study. Cognitive state was evaluated with a comprehensive neuropsychological battery, and WMHs were identified in FLAIR and T2-weighted MRI. The burden of WMHs was rated using the Scheltens scale. RESULTS: No differences in WMHs were found between the three groups in the cross-sectional study. A negative correlation was observed between semantic fluency and the subscore for WMHs in the frontal lobe. Of the 36 non-demented patients re-evaluated after a mean follow-up of 30 months, three patients converted into MCI and 5 into dementia. Progression of periventricular WMHs was associated with an increased conversion to dementia. A marginal association between the increase in total WMHs burden and worsening in the Mini Mental State Examination was encountered. CONCLUSIONS: White matter hyperintensities do not influence the cognitive status of patients with PD. Frontal WMHs have a negative impact on semantic fluency. Brain vascular burden may have an effect on cognitive impairment in patients with PD as WMHs increase overtime might increase the risk of conversion to dementia. This finding needs further confirmation in larger prospective studies.

Neuroimagen de los tumores cerebrales / Neuroimaging in brain tumors

Aunque el estudio neuropatológico es insustituible para conseguir el mejor control posible de los tumores craneales, el diagnóstico, el tratamiento y el seguimiento posterior ha ido progresando en buena medida conforme han avanzado las técnicas de neuroimagen. Tanto la cirugía como la radioterapia son técnicas dependientes de la imagen para su planificación. En el momento actual, la RM es un estándar que permite conocer de forma muy precisa la localización de la lesión y su relación anatómica con las estructuras vecinas. En gliomas de alto grado, se acepta que la parte de tumor captante de contraste en la TC o en las secuencias T1 de RM tras la administración de gadolinio, corresponde a tumor sólido. Sin embargo, la relación entre la imagen y la parte invasiva del tumor queda mucho menos definida. Por tanto, se acepta que la RM convencional no es suficiente para discriminar la extensión de la parte invasiva del tumor. En los últimos años las técnicas de PET con FDG o radiofármacos aminoácidos (metionina, FDOPA, FET), SPECT con talio, así como secuencias avanzadas de RM como la perfusión, la difusión, el tensor de difusión y la tractografía, y la RM funcional están aportando una información complementaria de gran importancia en la caracterización, planificación terapéutica y diagnóstico diferencial de la recidiva de los tumores cerebrales. En este artículo de formación continuada sobre tumores cerebrales se tratan desde un punto de vista multidisciplinar los diferentes aspectos técnicos y aplicaciones clínicas de las principales técnicas de neuroimagen(AU)

Advances in neuroimaging have modified diagnosis, treatment and clinical management of brain tumors. However, neuropathological study remains necessary in order to get the best clinical management. Surgery and radiotherapy planning are imaging-dependent procedures, and MRI is the standard imaging modality for determining precisely tumor location and its anatomical relationship with surrounding brain structures. In high-grade tumors it has been accepted that tumoral areas with contrast uptake in CT, or T1-weighted MRI contrast enhancement corresponds to solid tumor. However, relationship between MRI and invasive tumor areas remains less defined. Therefore, it is generally accepted that conventional MRI is not sufficient to delineate the real extension of brain tumors. In recent years, PET using 18FDG and amino acid radiotracers (11C-Methionine, 18FDOPA, 18FET) and SPECT with 201-Thallium, as well as advanced MRI sequences (Perfusion, Diffusion-weighted, Diffusion tensor imaging and tractography), and functional MRI, have added important complementary information in the characterization, therapy planning and recurrence differential diagnosis of brain tumors. In this continuing education review of neuroimaging in brain tumors, technical aspects and clinical applications of different imaging modalities are approached in a multidisciplinary way(AU)

[Neuroimaging in brain tumors]. / Neuroimagen de los tumores cerebrales.

Advances in neuroimaging have modified diagnosis, treatment and clinical management of brain tumors. However, neuropathological study remains necessary in order to get the best clinical management. Surgery and radiotherapy planning are imaging-dependent procedures, and MRI is the standard imaging modality for determining precisely tumor location and its anatomical relationship with surrounding brain structures. In high-grade tumors it has been accepted that tumoral areas with contrast uptake in CT, or T1-weighted MRI contrast enhancement corresponds to solid tumor. However, relationship between MRI and invasive tumor areas remains less defined. Therefore, it is generally accepted that conventional MRI is not sufficient to delineate the real extension of brain tumors. In recent years, PET using 18FDG and amino acid radiotracers ((11)C-Methionine, (18)FDOPA, (18)FET) and SPECT with (201-)Thallium, as well as advanced MRI sequences (Perfusion, Diffusion-weighted, Diffusion tensor imaging and tractography), and functional MRI, have added important complementary information in the characterization, therapy planning and recurrence differential diagnosis of brain tumors. In this continuing education review of neuroimaging in brain tumors, technical aspects and clinical applications of different imaging modalities are approached in a multidisciplinary way.

Hallazgos neurorradiológicos de la Acidosis Glutárica tipo I / No disponible

La acidosis glutárica tipo I (AGTI) es un trastorno poco frecuente del metabolismo de los ácidos orgánicos consecuencia de un défi cit congénito de la enzima mitocondrial glutaril-CoA deshidrogenasa. Existe una alteración del metabolismo de los aminoácidos triptófano, lisina e hidroxilisina y se produce un aumento de los niveles de ácido glutárico que clínicamente se manifi esta con crisis distónicas agudas en niños de corta edad. La acumulación de ácido glutárico produce neurotoxicidad en los ganglios de la base y corteza fronto-temporal lo que puede causar distonía progresiva, hipotonía, disartria y crisis epilépticas. El diagnóstico precoz de esta enfermedad es crucial ya que la dieta y el tratamiento pueden alterar su historia natural. Presentamos los hallazgos neuroradiológicos de una adolescente de 16 años con AGTI que presentaba un síndrome distónico crónico. La RM demostró afectación del putamen de forma bilateral y de la sustancia blanca periventricular, además de atrofi a temporal en ambos hemisferios y ensanchamiento de ambas cisuras de Silvio

Glutaric aciduria type I is a rare disorder of organic acid metabolism caused by defi ciency of glutaryl-CoA dehydrogenase, a mitochondrial enzyme. Improper degeneration of amino acids: tryptophan, lysine, and hydroxylysine, results in increased levels of glutaric acid, which typically becomes clinically manifest as an acute dystonic crisis in young children. Accumulation of glutaric acid causes neurotoxicity in the basal ganglia and fronto-temporal cortex which can lead to progressive dystonia, hypotonia, permanently impaired speech and seizures. Because dietary and drug therapy may alter the natural history of the disease, early diagnosis of such patients is critical. We report the magnetic resonance (MR) imaging fi ndings in a 16 year-old girl with this disorder who presented with a chronic dystonic syndrome and previously diagnosed of brain paralysis. MR imaging demonstrated bilateral involvement of the putamina and periventricular white matter, and bilateral temporal atrophy and widened Silvian fi ssures

[Neuroradiologic findings of glutaric aciduria type I]. / Hallazgos neurorradiológicos de la Acidosis Glutárica tipo I.

Glutaric aciduria type I is a rare disorder of organic acid metabolism caused by deficiency of glutaryl-CoA dehydrogenase, a mitochondrial enzyme. Improper degeneration of amino acids: tryptophan, lysine, and hydroxylysine, results in increased levels of glutaric acid, which typically becomes clinically manifest as an acute dystonic crisis in young children. Accumulation of glutaric acid causes neurotoxicity in the basal ganglia and fronto-temporal cortex which can lead to progressive dystonia, hypotonia, permanently impaired speech and seizures. Because dietary and drug therapy may alter the natural history of the disease, early diagnosis of such patients is critical. We report the magnetic resonance (MR) imaging findings in a 16 year-old girl with this disorder who presented with a chronic dystonic syndrome and previously diagnosed of brain paralysis. MR imaging demonstrated bilateral involvement of the putamina and periventricular white matter, and bilateral temporal atrophy and widened Silvian fissures.