Spectrum of MR imaging findings in Wernicke encephalopathy: are atypical areas of involvement only present in nonalcoholic patients?

BACKGROUND AND PURPOSE: Although MR imaging is considered the most effective method to confirm a diagnosis of WE, MR imaging studies designed to distinguish WE between NA and AL patients have yielded controversial results. The purpose of this study was to determine potential differences in MR imaging features between AL and NA patients with WE and to compare neurologic symptoms with MR imaging findings. MATERIALS AND METHODS: This retrospective study included 24 consecutive patients (male/female, 15:9; mean age, 54 years) diagnosed with WE in a university hospital (AL = 13, NA = 11). Clinical manifestations and MR imaging findings between AL and NA patients were evaluated. Classic WE symptom triad and consciousness level and MR imaging findings were scored and compared with each other. Statistical analyses were performed with χ(2), Fisher exact, and Spearman tests. RESULTS: No differences were observed regarding the areas of hyperintense signal intensity on FLAIR imaging and enhancement of the mammillary bodies between AL and NA patients (P > .05). Frequent sites of involvement were the medial thalami (86%), dorsal medulla (82%), tectal plate (77%), and the periaqueductal gray matter (75%). A positive association was found between the consciousness levels of the patients and the involvement of atypical sites (P = .01). Only 4 of the 24 patients (17%) had all 3 symptoms of the classic WE symptom triad. CONCLUSIONS: MR imaging features of WE may not be different between AL and NA patients. The medulla is 1 of the most frequently involved sites, and consciousness level is also associated with atypical site involvement.

Optimal diffusion-weighted imaging protocol for lesion detection in transient global amnesia.

BACKGROUND AND PURPOSE: Diffusion-weighted imaging (DWI) can depict small punctate hyperintense lesions in the hippocampus in transient global amnesia (TGA). The purpose of this study was to find an optimal DWI protocol for lesion detection in TGA by investigating various imaging parameters and imaging timing after symptom onset. MATERIALS AND METHODS: Sixteen patients with TGA diagnosed during 14 months underwent DWI within 24 hours and again at follow-up 3 days after onset. Each DWI session included 4 different sequences using different b-values (seconds per square millimeter) and section thicknesses (millimeter): 1000/5, 1000/3, 2000/3, and 3000/3. The presence or absence of hyperintense lesions on the 8 DWIs was determined visually, and the number of lesions detected was compared. RESULTS: Thirteen of the 16 patients (81%) had either single or multiple punctate hyperintense lesions, totaling 24 lesions, and the remaining 3 patients had no lesions. All lesions detected were in the hippocampus except 1. The number of lesions detected on initial DWIs at a b-value/section thickness of 1000/5, 1000/3, 2000/3, and 3000/3 was 3, 9, 13, and 13, respectively, whereas that of follow-up DWIs was 17, 22, 24, and 24, respectively. CONCLUSION: On the basis of these preliminary results, the highest lesion detection was achieved for DWI with b = 2000/3 mm or b = 3000/3 mm at 3 days postonset. When no lesion is detected by DWI within 24 hours after onset, follow-up DWI is recommended several days later.

Ophthalmic rete mirabile: the first angiographic documentation of embryonic ophthalmic collaterals in a patient with moyamoya disease. A case report.

SUMMARY: Plexiform vascular networks of the rete mirabile in humans are rare but can be observed in some vertebrates such as cobaye, cat, cow and sheep. Each set of embryonic arteries can be a potential source of these vascular networks which reconstitute the distal flows in segmental agenesis of internal carotid or vertebral arteries especially in patients with PHACE (posterior fossa malformation of the brain, facial hemangiomas, arterial anomalies, cardiac anomalies with coarctation of aorta, and eye abnormalities) syndrome. Ophthalmic rete mirabile has been found in some mammals such as camel, deer and avian species of pigeon, Hawaiian seabirds and deep-water fish of the cod other than human. We describe the first human case of ophthalmic rete mirabile confirmed on angiography in a patient with moyamoya disease. These findings are the first description in the English literature as far as we know. The clinical implications with moyamoya disease will be briefly discussed according to the embryologic development of cranial arteries.

Rhabdoid meningioma: clinical features and MR imaging findings in 15 patients.

BACKGROUND AND PURPOSE: Rhabdoid meningioma (RM) is a recently described variant of malignant meningioma, with radiologic features currently not well characterized in the medical literature. The purpose of this study was to describe and characterize clinical features and imaging findings associated with RM. MATERIALS AND METHODS: CT (n = 8) and MR (n = 15) images of 15 patients (4 men and 11 women; mean age, 52 years; range, 22-75 years) with 16 pathologically proved RMs along with associated clinical records were retrospectively reviewed. All of the patients underwent surgical resection and had additional radiation therapy except for 1 patient. After surgery, the patients had follow-up brain MR imaging to evaluate for tumor recurrence. RESULTS: Nine lesions (56%) were located in the cerebral convexity, and 4 lesions (25%) were located in the parasagittal areas. The tumors were isointense (n = 15) to gray matter on T1-weighted images, whereas they were hyperintense (n = 14) on T2-weighted images. On gadolinium-enhanced T1-weighted images, homogeneous enhancement was seen in 10 lesions, and heterogeneous enhancement was seen in 6 lesions that had cysts. Cystic components were noted in 6 lesions (38%). Severe peritumoral edema was seen in 12 lesions (75%). Nine lesions (56%) had hyperostosis, and 5 of them also had bone destruction. Among the 8 cases with initial CT scans, only 1 had amorphous calcifications (13%). There was only 1 recurrence of RM found during the follow-up period after surgical resection. CONCLUSION: RMs tend to have prominent peritumoral edema, cystic components, and bone involvement.

Intracranial solitary fibrous tumors: imaging findings in 6 consecutive patients.

BACKGROUND AND PURPOSE: Intracranial solitary fibrous tumors (ISFTs) are rare mesenchymal neoplasms originating in the meninges. The aim of this study was to describe the CT, MR imaging, and angiographic features of the solitary fibrous tumor and to identify imaging characteristics. MATERIALS AND METHODS: We retrospectively reviewed CT, MR, and angiographic findings in 6 cases of ISFT. We evaluated the size, shape, and location of the tumor; the internal content and margin of the lesion; the pattern of enhancement; and the change of the adjacent structures. Density on noncontrast CT scans, signal intensity on MR images, and angiographic features were also documented. RESULTS: Each lesion appeared as a discrete extra-axial mass (size, 3-7 cm; mean, 5 cm). Five lesions were entirely solid, and 1 had peritumoral cyst. All 5 of the noncontrast CT scans showed hyperattenuated masses, and the tumors exhibited marked heterogeneous enhancement. No lesion contained calcification, and 2 cases showed bone invasions. On the MR images, 4 lesions showed mixed signal intensity on T2-weighted imaging. All of the lesions revealed marked heterogeneous enhancement. All of the tumors had thickening of the meninges adjacent to the tumor. Angiography showed delayed tumor blushing in all, and 3 of them had dysplastic dilation of the tumor vessels. CONCLUSION: Although there are no pathognomonic imaging findings, some imaging features, such as the "black-and-white mixed" pattern on T2-weighted images and marked heterogeneous enhancement, might be helpful in the diagnosis of intracranial solitary fibrous tumor.

MR features of intraocular ectopic lacrimal tissue.

A 2-year-old girl who had a 3-day history of swelling in her right eye presented with a case of intraocular ectopic lacrimal tissue. MR imaging findings and possible causes of the ectopic lacrimal tissue in the globe, including embryology, are reviewed. The differential diagnoses of other intraocular masses and the imaging features that can be of help in making a diagnosis are also discussed.

Combined spinal intramedullary arteriovenous malformation and lipomyelomeningocele.

Combined spinal arteriovenous malformation and lipomyelomeningocele are extremely rare. We present a rare combined case of a lipomyelomeningocele with an intramedullary arteriovenous malformation (AVM) occurred at the L3-L4 level in a 30-year-old man who suffered from low back pain radiating to the lower extremities, dysuria, and frequency for 5 years. The MR studies showed an intradural mass with high-signal intensity on both T1-weighted and T2-weighted images, intermingled with multiple signal-void structures. The mass extended extradurally toward a subcutaneously forming fatty mass on the patient's back. Spinal angiography showed an AVM supplied by the radiculopial branches of the lumbar arteries and drained by tortuous, dilated, perimedullary veins. Endovascular embolization and surgical resection were performed.

Supratentorial cerebral arteriovenous fistulas (AVFs) in children: review of 41 cases with 63 non choroidal single-hole AVFs.

PURPOSE: In this article we explore the various aspects of the supratentorial Single-Hole AVFs (ST AVFs) in children, focusing on their clinical features, angio-architecture, treatment indications and the role and results of endovascular management. MATERIALS AND METHODS: Among 1565 cases of brain AVMs seen at our neurovascular center, 620 cases were seen in the pediatric age group (

Anatomic variations of the superficial middle cerebral vein: embryologic aspects of the regressed embryonic tentorial sinus.

SUMMARY: The embryonic tentorial sinus usually regressses during postnatal development, but its typical prenatal drainage patterns and intradural anastomoses can be depicted as various developmental phenotypic representations. Here, we tried to clarify the variant types of the superficial middle cerebral vein (SMCV) associated with the embryonic tentorial sinus. Total 41 patients and 82 hemispheres were included in this study. CT angiography was performed in all patients as screening for cerebrovascular disease or other intracranial disorders. A separate workstation and 3D software were used to evaluate the cranial venous systems with 3D volume rendering techniques, thin-slice MIP images, and MPR techniques for the analysis of its complicated angioarchitecture. Variations of the SMCV were classified according to the developmental alterations of the embryonic tentorial sinus, including sphenoparietal sinus (cranial remnant of tentorial sinus), basal sinus (floor of middle cranial fossa), petrosal and caudal remnant of the tentorial sinus. Secondary intradural anastomoses of cavernous and superior petrosal sinuses were also evaluated for the efferent pathways. The most frequent type of remnant tentorial sinus, sphenoparietal sinus was present in 49% (40/82) of hemispheres examined. Other regressed patterns of embryonic tentorial sinus were also identified in 38% (31/82): nine caudal remnant type around the transverse sinus, 12 petrosal type, one basal type, five unclassified cases, and mixed type were found in four cases. Secondary intradural cavernous sinus anastomosis was seen in 44% (36/82), however the most prevalent pattern was no anastomosis (46/82) with cavernous sinus. Only one case of superior petrosal sinus anastomosis was found in this series associated with basal sinus type. Anatomic variations of SMCV can be clearly demonstrated with embryologic aspects of the tentorial sinus according to its developmental regression and postnatal secondary adaptations of cerebral venous drainage.

Anatomic variations of the deep cerebral veins,tributaries of Basal vein of rosenthal: embryologic aspects of the regressed embryonic tentorial sinus.

SUMMARY: The embryonic tentorial sinus regresses at the 60-80 mm embryologic stage and most of the deep venous channels constitute the basal vein of Rosenthal (BVR). Persisting remnants of the embryonic tentorial sinus can be seen in the adult configuration of the BVR.We tried to explain the anatomic representations of the BVR associated with the remnant embryonic tentorial sinus. A total 41 patients and 82 hemispheres were included in this study. CT angiography was performed in all patients as screening for cerebrovascular disease or other intracranial disorders. A separate workstation and 3D software were used to evaluate the cranial deep venous systems with 3D volume rendering techniques, thin-slice MIP images, and MPR techniques for the analysis of complicated angioarchitecture. Variations of the BVR were classified according to the developmental alterations of efferent pathways into four groups: telencephalic group (A) including tributaries of the uncal vein, inferior frontal vein, anterior communicating vein, and inferior striatal vein; diencephalic group (B) of the interior ventricular vein and peduncular vein; tegmental bridging group (C) of the longitudinal LMV anastomosis; tectal group (D) of the superior vermian vein and internal occipital vein in relation to the Galenic connection. The BVR constituted from the embryonic tentorial sinus was also assessed and the developmental aspects reviewed. Remnant embryonic tentorial sinus was visualized in 12% (10/82) of hemispheres, all of them invariably connected with the telencephalic (A) and diencephalic (B) groups. Most of those connections (9/10) to basal venous tributaries originated from the medial tentorial sinus except one case from the lateral tentorial sinus. No Galenic connections of the BVR were identified in 10% (8/82). Various tributaries of the BVR were classified as: Telencephalic group (A) 43% (35/82), Diencephalic group (B) 35% (29/82), Bridging group (C) 11% (9/82), and Tectal group (D) 6% (5/82). Four cases (5%) were unclassified and revealed only small basal tributaries of the BVR without connection to the great vein of Galen. Anatomic variations of the BVR connected with persistent embryonic tentorial sinus could often be demonstrated in adult configurations considering the embryologic aspects of developmental regression and secondary cerebral venous adaptations.

Posterior cranial fossa single-hole arteriovenous fistulae in children: 14 consecutive cases.

We report 14 consecutive children with 23 posterior cranial fossa arteriovenous fistula (AVF); six had multifocal lesions, involving the supratentorial brain in three and the spinal cord in one. There were two boys and four girls with a family history compatible with hereditary haemorrhagic telangiectasia. The diagnosis was made in infancy in eight cases and in a further six before the age of 12 years; mean age at diagnosis was 3.5 years. The male-to-female ratio was 1.8:1. Presenting features were macrocrania in four cases, haemorrhage or headache in three and nonhaemorrhagic neurological deficits or and cardiac overload in two. Dominant supply to the symptomatic fistula arose from the posterior inferior cerebellar artery in five cases, anterior inferior cerebellar artery in two and the upper basilar artery system in seven. All children were primarily treated by transarterial embolisation. We treated thirteen children (93%) by transarterial embolisation alone; one older child with a history of haemorrhage also underwent radiosurgery. We obtained 100% exclusion of the fistula(e) in six children, 95-80% in five, 80-50% in one and <50% in one. Of the incompletely treated cases, three had conservative management, and two with 80% and one with 60% reduction of their lesion are scheduled for elective treatment; two partially treated case died. There was no morbidity due to the endovascular procedures. Follow-up since referral is 6 months-10 years (mean 4.5 years). Ten children are neurologically normal, two have persistent (pre-existing) neurological deficits and two are dead.

Hereditary Haemorrhagic Telangiectasia Cerebrospinal Localization in Adults and Children. Review of 39 cases.

SUMMARY: Cerebral arteriovenous malformations (CAVM) can be associated with Hereditary Haemorrhagic Telangiectasia (HHT), a dominantly inherited vascular disorder with variable penetrance and expressivity. The presentation and angiographic features were analysed retrospectively. The purpose is to point to special groups of AVM patients within the overall CAVMs and to discuss the issue of screening. We reviewed 34 cases of HHT-related CAVM from the data bank in Bicêtre from 1985-2003. In Spinal cord AVM (SCAVM) there were 194 patients with 5 HHT. HHT was diagnosed when at least two criteria were met; cutaneous telangiectasia, epistaxis, visceral AVMs, angiographic findings of AVF and first degree family history. Intracranial haemorrhage was the presenting symptom in 8.8% and the risk of haemorrhage in the natural history was 0.7% per year. The commonest angiographic features in adults are nidus(81.8%) and multiplicity(45.5%), while in the paediatric group venous ectasia and giant pouches(91.3%), AVF(69.6%) and multiplicity( 52.2%). In spinal cord lesions macrofistulas are demonstrated in 83% of HHT with no multiplicity. HHT-related CAVMs present as multiple lesions, cortical in location, micro AVMs or AVF. HHT in SCAVM is expressed as single macro AVF, especially in the paediatric group. AVF in children are highly suggestive of HHT. We do not recommend screening in HHT adult patients for CAVM, while in the paediatric population, screening could be recommended at six months of age for cerebrospinal localization. These patients should be screened for Pulmonary AVF, which needs to be treated in priority.

MR Perfusion Imaging in a Case of a Vein of Galen Malformation with Secondary Capillary Angioectasia.

SUMMARY: Vein of Galen Aneurysmal Malformations (VAGMs) are uncommon vascular malformations associated with dilatation of the vein of Galen embryonic forerunner with single or multiple direct arteriovenous fistulas within its wall without direct reflux into normal cerebral veins. We describe a patient with a late neurological onset presenting a classic VGAM complicated by secondary thalamic capillary angioectasia imaged with MR perfusion. In our patient, abnormal MR perfusion parameters were not seen on conventional MRI; they probably reflect underlying venous hypertension. They were located in areas involved in motor neurological deficit.

Dural Sinus Malformations (DSM) with Giant Lakes, in Neonates and Infants. Review of 30 Consecutive Cases.

UNLABELLED: Abstract: Background and Purpose. Dural Arteriovenous Shunt (DAVS) in children include Dural sinus malformation (DSM), infantile and adult types. They are rare and seldom reported. Our purpose was to highlight the angiographic features of the DSM sub group for prognosis of clinical evolution and outcome and to lay guidelines for management. METHODS: From a dedicated neurovascular data bank, there were 52 cases of arteriovenous dural shunts in children from 1985 to 2003. Of these, there were 30 patients with DSM, which we analysed the various angioarchitecture, presentation and neurological outcome. Children clinical status was evaluated and scored at admission and follow up. Results. There was an overall male dominance of 2:1. Antenatal diagnosis was obtained in 8/30 (26.7%) cases. Mean age of diagnosis was 5 months. Mean age at first consultation was 8.7 months. No patient was diagnosed during childhood. The most common clinical presentations were macrocrania 76.7%, seizures 23.3% and mental retardation 23.3%. In 14/30 (35.7%) of the patients, the therapeutic decision was to manage conservatively; in 5/14 (30.7%) with predictable favourable evolution and in 9/14 (64.3%) with irreversible poor neurological outcome. In the remaining 16/30 (53.3%) patients, endovascular treatment was performed. In 12/16 (75.0%) patients the neurological outcome was good, 3/16 (18.8%) patients had unfavourable evolution despite embolization. There was no morbidity mortality related to the procedures themselves. 1/16 (6.3%) patient was lost to follow-up. Overall 12/29 (45.8%) patients had an unfavourable neurological outcome with 11 patients dead and 1 with severe neurological deficit. In the surviving group of children, 17/18 (94.4%) have a good neurological outcome; in 10/18 (55.5%) the lesion is morphologically excluded. Conclusion. DSM is rare disease with high mortality. They usually proceed to either total or partial spontaneous thrombosis before the age of 2 thus compromising normal cerebral venous drainage. DSM away from the torcular, good cavernous sinus, cavernous capture of sylvian veins, absence of pial veins, straight sinus or superior sagital sinus (SSS) reflux and absence of jugular bulb dysmaturation represent factors of good prognosis. Such patients will highly benefit for endovascular treatment. In partial endovascular approach the aim being is to separate the brain drainage from DSM drainage. This will be achieved by the transarterial approach to the associated mural arterio-venous shunts (AVS) and by disconnecting the pial reflux by transvenous route.

Use of Tc-99m HMPAO leukocyte scans to evaluate bone infection: incremental value of additional SPECT images.

PURPOSE: In this study, the diagnostic value of Tc-99m hexamethylpropylene amine oxime (HMPAO) leukocyte scans and the role of additional SPECT in the diagnosis of bone infection were evaluated. MATERIALS AND METHODS: The Tc-99m HMPAO leukocyte scans of 37 patients with clinically suspected bone infection were reviewed. The patients were divided into two groups according to the presence of orthopedic implants. Early (4 to 6 hours) and delayed (18 to 20 hours) planar images and early SPECT images were obtained. The final diagnosis of infection was made based on the pathologic, bacteriologic, and surgical data and clinical follow-up. RESULTS: Group 1 (25 patients with orthopedic implants) included 15 true-positive, 1 false-negative, 7 true-negative, and 2 false-positive results. Group 2 (12 patients without orthopedic implants) included 7 true-positive, 1 false-negative, and 4 true-negative results. The overall sensitivity of the Tc-99m HMPAO leukocyte scan with SPECT to detect bone infection was 92%, with a specificity rate of 85%. (Group 1: sensitivity, 94%; specificity, 78%. Group 2: 88% and 100% sensitivity and specificity, respectively.) CONCLUSION: The Tc-99m HMPAO leukocyte scan is useful in the diagnosis of bone infection, regardless of the presence of orthopedic implants. The additional SPECT images may be helpful to localize the site of infection more accurately.