A rare case of a secondary pituitary abscess arising in a craniopharyngioma with atypical presentation and clinical course / Un caso raro de absceso hipofisario secundario derivado de un craneofaringioma con presentación y evolución clínica atípicas

Pituitary abscesses are very uncommon. They are divided into primary, arising within a healthy gland, and secondary, observed with an underlying pre-existing lesion. Here we present the eighth case reported of a secondary abscess within a craniopharyngioma. A 59-year-old-woman presented with a 3-week history of headache, and fever. Physical examination was unremarkable. An Magnetic Resonance Imaging (MRI) showed a pituitary lesion suggestive of a chronic inflammatory process. She was diagnosed with lymphocytic meningitis with hypophysitis and she was treated with corticosteroids. Two months later she presented with headache and fever again. Control MRI showed enlargement of the pituitary lesion. Therefore, a transsphenoidal biopsy was performed. During the procedure, purulent material was released. Histological study demonstrated a craniopharyngioma and meningeal inflammation. Empiric antibiotics were started. Three months post-operatively, a follow-up MRI showed a suspect minimal residual mass. Secondary pituitary abscesses are rare. The key to successful management is a high index of suspicion. Transsphenoidal surgical evacuation plus antibiotics is the mainstay of treatment. Although most symptoms resolve, endocrinopathies improve only rarely (AU)

Los abscesos hipofisarios son infrecuentes. Se pueden dividir en primarios o secundarios, si se producen sobre una lesión previa. Presentamos el octavo caso de un absceso asentado sobre un craneofaringioma. Una mujer de 59 años consultó por fiebre y cefalea de tres semanas de evolución. La exploración física era anodina. Una resonancia magnética (RMN) evidenció una lesión hipofisaria sugestiva de un proceso inflamatorio crónico. Finalmente, se diagnosticó de una meningitis linfocítica e hipofisitis y se trató con corticoides. Dos meses después reconsultó por los mismos síntomas. En la RMN se evidenció crecimiento de la lesión, por lo que se biopsia endoscópicamente. Durante el procedimiento salió pus. En el examen histológico se evidenció un craneofaringioma y una inflamación meníngea. Se iniciaron antibióticos empíricamente. En el seguimiento a tres meses, la RMN evidenciaba un dudoso resto. Los abscesos hipofisarios secundarios son raros y hay que tener un alto índice de sospecha para diagnosticarlos. El tratamiento se basa en antibioterapia y evacuación transesfenoidal. Aunque los síntomas se suelen resolver, las endocrinopatías no (AU)

Atypical bacterial infections of the central nervous system transmitted by ticks: An unknown threat.

Infections of the central nervous system caused by atypical bacteria are becoming more common. Borrelia burgdorferi and Rickettsia conorii are microorganisms transmitted by ticks; infection with these bacteria result in a wide spectrum of manifestations on imaging. In areas where these tick-borne microorganisms are endemic, including Spain, these infections must be included in the differential diagnosis of patients with a variety of systemic and neurologic symptoms. The clinical presentation of these infections is nonspecific, and CT is normally the initial imaging technique, although MRI is more sensitive to early changes. On MRI, these infections can manifest as small lesions in the deep supratentorial white matter that are hyperintense on T2-weighted/FLAIR sequences. It is fundamental to know the imaging characteristics of the different atypical bacterial infections and their differential diagnoses. Good history taking combined with complementary tests (blood tests and CSF analysis) and the neuroimaging findings can help reach the right diagnosis and enable appropriate treatment, thereby preventing possible neurological sequelae.

Late vascular complications after cranial radiotherapy: A report of two illustrative cases.

Cranial radiotherapy (CRT) is used to treat a large variety of benign and malignant disorders. We present two cases of late neurological complications after CRT and briefly discuss its diagnosis and their shared pathophysiological aspects. The first case is a patient with cognitive impairment associated to mineralizing microangiopathy ten years after CRT for nasopharyngeal carcinoma and the second one is a woman with Stroke-like Migraine Attacks after Radiation Therapy (SMART) syndrome two years after CRT for anaplastic meningioma. Nowadays, higher survival rates might cause an increase in appearance of late neurological complications after CTR. These reported cases show that late complications can mimic a wide variety of neurological conditions and the importance of magnetic resonance image (MRI) to get a diagnosis.

Septo-optic dysplasia caused by a novel FLNA splice site mutation: a case report.

BACKGROUND: Septo-optic dysplasia (SOD), also known as de-Morsier syndrome, is a rare disorder characterized by any combination of optic nerve hypoplasia, pituitary gland hypoplasia, and midline abnormalities of the brain including absence of the septum pellucidum and corpus callosum dysgenesis. The variable presentation of SOD includes visual, neurologic, and/or hypothalamic-pituitary endocrine defects. The unclear aetiology of a large proportion of SOD cases underscores the importance of identifying novel SOD-associated genes. CASE PRESENTATION: To identify the disease-causing gene in a male infant with neonatal hypoglycaemia, dysmorphic features, and hypoplasia of the optic nerve and corpus callosum, we designed a targeted next-generation sequencing panel for brain morphogenesis defects. We identified a novel hemizygous deletion, c.6355 + 4_6355 + 5delAG, in intron 38 of the FLNA gene that the patient had inherited from his mother. cDNA studies showed that this variant results in the production of 3 aberrant FLNA transcripts, the most abundant of which results in retention of intron 38 of FLNA. CONCLUSIONS: We report for the first time a case of early-onset SOD associated with a mutation in the FLNA gene. This finding broadens the spectrum of genetic causes of this rare disorder and expands the phenotypic spectrum of the FLNA gene.

Actualización en la fisiopatología de la hidrocefalia crónica del adulto idiopática: ¿nos enfrentamos a otra enfermedad neurodegenerativa? / Current concepts on the pathophysiology of idiopathic chronic adult hydrocephalus: Are we facing another neurodegenerative disease?

INTRODUCCIÓN: Desde la descripción hace 5 décadas de la hidrocefalia crónica del adulto idiopática (HCAi), su fisiopatología ha sido considerada básicamente relacionada con el efecto que la dilatación ventricular ejerce sobre las estructuras adyacentes al sistema ventricular. Sin embargo, las alteraciones en el flujo sanguíneo cerebral (FSC) y, sobre todo, la reducción en el recambio licuoral parecen emerger como componentes fisiopatológicos principales de esta enfermedad. DESARROLLO: En la HCAi se observa una compresión del tracto piramidal, de los circuitos cortico-subcorticales fronto-estriatales y fronto-reticulares, y de las fibras profundas del fascículo longitudinal superior. En el cuerpo calloso se objetiva un descenso en el número de fibras comisurales, que son reemplazadas por gliosis. El FSC se encuentra alterado, con un patrón de última pradera en la región subcortical adyacente a los ventrículos, correspondiente a la intersección entre las arterias subependimarias y las arterias perforantes dependientes de los grandes troncos arteriales de la circulación anterior. El recambio diario del LCR se ve disminuido en un 75%, lo que conlleva una reducción del aclaramiento de neurotóxicos y la interrupción de las señalizaciones neuroendocrinas y paracrinas que ocurren a través del LCR. CONCLUSIONES: La HCAi emerge como una entidad nosológica compleja, en la que los efectos de la microangiopatía subcortical y la disminución del recambio de LCR desempeñan un papel fundamental. Esta base fisiopatológica aleja la HCAi del concepto clásico de hidrocefalia y la acerca al perfil de otras enfermedades neurodegenerativas, como la enfermedad de Alzheimer o la enfermedad de Binswanger

INTRODUCTION: Since its description five decades ago, the pathophysiology of idiopathic chronic adult hydrocephalus (iCAH) has been traditionally related to the effect that ventricular dilatation exerts on the structures surrounding the ventricular system. However, altered cerebral blood flow, especially a reduction in the CSF turnover rate, are starting to be considered the main pathophysiological elements of this disease. DEVELOPMENT: Compression of the pyramidal tract, the frontostriatal and frontoreticular circuits, and the paraventricular fibres of the superior longitudinal fasciculus have all been reported in iCAH. At the level of the corpus callosum, gliosis replaces a number of commissural tracts. Cerebral blood flow is also altered, showing a periventricular watershed region limited by the subependymal arteries and the perforating branches of the major arteries of the anterior cerebral circulation. The CSF turnover rate is decreased by 75%, leading to the reduced clearance of neurotoxins and the interruption of neuroendocrine and paracrine signalling in the CSF. CONCLUSIONS: iCAH presents as a complex nosological entity, in which the effects of subcortical microangiopathy and reduced CSF turnover play a key role. According to its pathophysiology, it is simpler to think of iCAH more as a neurodegenerative disease, such as Alzheimer disease or Binswanger disease than as the classical concept of hydrocephalus

Current concepts on the pathophysiology of idiopathic chronic adult hydrocephalus: Are we facing another neurodegenerative disease? / Actualización en la fisiopatología de la hidrocefalia crónica del adulto idiopática: ¿nos enfrentamos a otra enfermedad neurodegenerativa?

INTRODUCTION: Since its description five decades ago, the pathophysiology of idiopathic chronic adult hydrocephalus (iCAH) has been traditionally related to the effect that ventricular dilatation exerts on the structures surrounding the ventricular system. However, altered cerebral blood flow, especially a reduction in the CSF turnover rate, are starting to be considered the main pathophysiological elements of this disease. DEVELOPMENT: Compression of the pyramidal tract, the frontostriatal and frontoreticular circuits, and the paraventricular fibres of the superior longitudinal fasciculus have all been reported in iCAH. At the level of the corpus callosum, gliosis replaces a number of commissural tracts. Cerebral blood flow is also altered, showing a periventricular watershed region limited by the subependymal arteries and the perforating branches of the major arteries of the anterior cerebral circulation. The CSF turnover rate is decreased by 75%, leading to the reduced clearance of neurotoxins and the interruption of neuroendocrine and paracrine signalling in the CSF. CONCLUSIONS: iCAH presents as a complex nosological entity, in which the effects of subcortical microangiopathy and reduced CSF turnover play a key role. According to its pathophysiology, it is simpler to think of iCAH more as a neurodegenerative disease, such as Alzheimer disease or Binswanger disease than as the classical concept of hydrocephalus.

Diplejía facial y afagia de aparición súbita / Sudden onset of facial diplegia and aphagia

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Nonconvulsive status epilepticus causing prolonged stupor after intraventricular hemorrhage: report of a case.

We describe the case of an octogenarian woman who experienced a severe alteration of mental state due to non-convulsive status epilepticus (NCSE) complicating an intraventricular hemorrhage. Our report emphasizes that NCSE may be the cause of unexplained neurological deterioration in elderly patients with acute brain injury.

Dolor abdominal en paciente tratado con acenocumarol. Un caso de hematoma intramural espontáneo de intestino delgado / Abdominal ain in a patient using acenocoumarol. A case of spontaneous intramural samll-bowel hematoma

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Computed tomography perfusion usefulness in early imaging diagnosis of herpes simplex virus encephalitis.

An early diagnosis is crucial in herpes simplex virus encephalitis patients in order to institute acyclovir therapy and reduce mortality rates. Magnetic resonance imaging (MRI) is considered the gold standard for evaluation of these patients, but is frequently not available in the emergency setting. We report the first case of a computed tomography (CT) perfusion study that helped to establish a prompt diagnosis revealing abnormal increase of blood flow in the affected temporoparietal cortex at an early stage.

Unusual perforated appendicitis within umbilical hernia: CT findings.

We present the first imaging report of perforated appendicitis in an umbilical hernia. Computed tomography demonstrated a gas-forming abscess within an umbilical hernia and the cecum was found inside the hernial sac, with an inner relation to the abscess. Computed tomographic findings suggested appendicitis as possible diagnosis, which was confirmed at surgery. Physicians must consider appendicitis within the differential diagnosis of an abdominal abscess located near to the cecum, even at an unexpected location.

Value of helical computed tomography in the management of upper esophageal foreign bodies.

PURPOSE: To analyze the utility of helical computed tomography (CT) in the diagnosis of suspected upper esophageal foreign bodies. MATERIAL AND METHODS: A prospective study was performed on 36 patients (26 F, 10 M, mean age 70 years) with a history of foreign body impaction. All had negative findings at indirect laryngoscopy. Radiologic assessment included unenhanced helical CT and a barium contrast study. Patients with positive findings were taken to esophagoscopy. All patients had a posterior clinical surveillance. RESULTS: Twenty patients had both normal CT and barium study and satisfactory clinical outcome. In 12 patients a foreign body was noted in the cervical esophagus by CT, barium study, and endoscopy. In one patient a fish bone was detected by CT (and not by barium) confirmed with esophagoscopy. Another patient had a fish bone esophageal perforation which was observed only by CT and confirmed at surgery. Two patients with normal barium and endoscopy presented a false-positive CT result. CONCLUSION: Barium swallow is currently the first radiologic study, but may involve a risk of aspiration and can impede a subsequent esophagoscopy. Esophagoscopy is an invasive technique with a certain risk of serious complications that can be avoided with a satisfactory radiologic assessment. CT is easy, fast, has 100% sensitivity and is therefore the first choice technique for diagnosing suspected upper esophageal foreign bodies not expected to be visible on plain radiographs.