Slowly progressive hemiparesis in childhood as a consequence of Rasmussen encephalitis without or with delayed-onset seizures

Five young children developed slowly progressive hemiparesis as the initial manifestation of Rasmussen encephalitis (RE). Three have remained seizure free over an observational period of 1.3–1.9 years. In the remaining two patients, seizures occurred after 0.5 and 0.6 years respectively. We suggest that RE might be presently underdiagnosed and should be suspected in cases of new onset hemiparesis. In this series, three out of five patients showed oligoclonal bands on examination of cerebrospinal fluid (CSF) which represented additional diagnostic hints towards an immune-mediated condition. According to recently published formal diagnostic criteria, evidence of progressive cerebral hemiatrophy or bioptic identification of RE-typical inflammation confirms the diagnosis in such cases. Long-term immunotherapy is recommended in order to prevent further tissue loss and functional decline.     

Familial Sneddon's syndrome

We report the familial occurrence and apparent autosomal dominant inheritance of Sneddon's syndrome with variable clinical expression. The proband, a 40-year-old woman, presented with livedo reticularis and progressive neurological deterioration following a stroke. The diagnosis was confirmed by cerebral angiogram and skin biopsy, both showing the characteristic findings. Two of the patient's sisters were reported to have been similarly affected in the past. Her mother, two additional siblings and five of her seven children exhibited various vasospastic skin phenomena. Familial aggregation of this disorder may be common and a genetic basis may be involved in its pathogenesis.     

MR relaxation times and iron content of thalassemic spleens: an in vitro study

To determine the relationship between MR relaxation times and the iron content of the spleens in patients with thalassemia, we measured these parameters at 0.19 and 1.18 T in 19 thalassemic spleen specimens in vitro. The correlation was best between iron content and the dependence between the interecho interval and the 1/T2 (T2 relaxation rate) at 1.18 T(r = .9361, p less than .001). No statistically significant correlation was found between T1 and iron content at either field strength. The variation of the 1/T2 with interecho interval may be useful for measuring iron content in vivo. It supports the theory that the T2 relaxation of iron deposits occurs via cellular field gradients produced by intralysosomal granules of hemosiderin.     

Antagonism of central melanin-concentrating hormone 1 receptor alleviates steatohepatitis in mice

Blockade of brain melanin-concentrating hormone 1 receptor (MCH1R) significantly ameliorates fatty liver as well as obesity. However, the mode of action of this effect is unknown. This study examined the effect of a MCH1R antagonist in murine steatohepatitis models with and without obesity and clarified whether these pharmacological effects were attributed to anti-obesity effects. Steatohepatitis with concomitant obese phenotypes was developed after 52-week exposure to a high-fat diet, and steatohepatitis with reduced body weight was developed by exposure to a methionine- and choline-deficient diet for 10 days. Chronic intracerebroventricular infusion of a peptidic MCH1R antagonist reduced hepatic triglyceride contents and ameliorated steatohepatitis on histological observations in both mice models. Improvement of steatohepatitis was concomitant with amelioration of obese phenotypes such as hyperinsulinemia and hyperleptinemia in the case of the obese model, whereas body weight reduction was not associated with amelioration of steatohepatitis by the antagonist in the lean model. Reduction of hepatic gene expressions encoding cytochromes P450 4A was identified by treatment with the antagonist in both the obese and lean models. These results suggest that brain blockade of MCH1R could alleviate steatohepatitis independently from anti-obesity effects. In conclusion, MCH1R antagonist could have a new therapeutic potential for the treatment of human nonalcoholic steatohepatitis.     

Association of intracerebral venous angioma and true arteriovenous malformation: a rare, distinct entity

We report a mixed cerebrovascular malformation in which a true arteriovenous malformation drained into associated venous angiomas. We describe the MRI and angiographic appearances and review the literature on mixed vascular malformations.     

Occipital condyle fracture and ligament injury: Imaging by CT

The true incidence of fracture of the occipital condyles is unknown. It may be associated with instability at the craniocervical joint. CT is the modality of choice for the demonstration of these fractures, but its use for imaging of the associated ligament injury has not been reported. In order to demonstrate normal anatomy, occipital condyle fracture and ligament injury, and to estimate the incidence of this lesion, 21 children and young adults with high-energy blunt craniocervical injury were examined prospectively. Thin-slice, axial, contiguous, CT was performed from the base of C2 to above the foramen magnum. Bone and soft tissue windows and coronal, sagittal, and curvilinear 2D reconstructions were performed. Five occipital condyle fractures were identified in four patients (19 %), with demonstration of alar ligament injury in two cases and local hematoma in one. In four, artifacts or rotation precluded assessment of ligaments. In all remaining cases normal bone and ligament anatomy was demonstrated. Fracture of the occipital condyles following craniocervical injury is not uncommon in children and young adults. Normal bone and ligament anatomy and pathology can be safely and clearly demonstrated in seriously injured patients and others using this CT technique. Increased awareness of this entity and a low threshold for performing CT should avoid the potentially serious consequences of a missed diagnosis.     

Wall of infundibular recess: a CT and MR study

A ring of enhancement immediately posterior to the optic chiasm has been observed on postcontrast, thin section, axial CT. This ring represents enhancement of the infundibular recess' wall and does not have any pathologic significance. Magnetic resonance confirmed this anatomic interpretation.     

Endovascular management of exsanguinating vertebral artery transection

BACKGROUND: Vertebral artery (VA) transection is a rarely described entity that may present dramatically with a life-threatening external hemorrhage. CASE DESCRIPTION: A 25-year-old man that was victim of a terrorist attack presented a gunshot wound to the mouth with massive bleeding. The bullet perforated the oropharynx and injured the right VA. Combined antegrade and retrograde endovascular embolization by means of detachable coils allowed rapid control of the bleeding. CONCLUSION: Bleeding related to VA transection can be managed safely by means of endovascular combined approaches.