Gorham-Stout disease affecting the spine with cerebrospinal fluid leakage and Chiari-like tonsillar herniation: a rare case report and review of literature.

BACKGROUND: Gorham-Stout disease (GSD) is a very rare disorder characterized by massive osteolysis of poorly understood aetiology. The association between GSD involving the skull base and cerebrospinal fluid (CSF) leakage has been reported in the literature. However, few cases of CSF leakage and Chiari-like tonsillar herniation in GSD involving the spine have been reported. CASE PRESENTATION: We present the case of a 20-year-old man with GSD involving the thoracic and lumbar spine, which caused CSF leakage and Chiari-like tonsillar herniation. The patient underwent four spinal surgeries for osteolytic lesions of the spine over a 10-year period. Here, we discuss the possible aetiology of the development of CSF leakage. Epidural blood patch (EBP) was performed at the T11-T12 level to repair the CSF leakage. After EBP treatment, rebound intracranial hypertension (RIH) developed, and tonsillar herniation disappeared 2 months later. CONCLUSIONS: GSD involving the spine with CSF leakage and Chiari-like tonsillar herniation is relatively rare. For patients who have undergone multiple spinal surgeries, minimally invasive treatment is an alternative treatment for CSF leakage. EBP can repair CSF leakage secondary to GSD and improve chronic brain sagging, with reversibility of Chiari-like malformations.

Targeted Epidural Blood Patch Treatment for Refractory Spontaneous Intracranial Hypotension in China.

Objective An epidural blood patch (EBP) is the mainstay of treatment for refractory spontaneous intracranial hypotension (SIH). We evaluated the treatment efficacy of targeted EBP in refractory SIH. Methods All patients underwent brain magnetic resonance imaging (MRI) with contrast and heavily T2-weighted spine MRI. Whole spine computed tomography (CT) myelography with non-ionic contrast was performed in 46 patients, and whole spine MR myelography with intrathecal gadolinium was performed in 119 patients. Targeted EBPs were placed in the prone position one or two vertebral levels below the cerebrospinal fluid (CSF) leaks. Repeat EBPs were offered at 1-week intervals to patients with persistent symptoms, continued CSF leakage, or with multiple leakage sites. Results Brain MRIs showed pachymeningeal enhancement in 127 patients and subdural hematomas in 32 patients. One hundred fifty-two patients had CSF leakages on heavily T2-weighted spine MRIs. CSF leaks were also detected on CT and MR myelography in 43 and 111 patients, respectively. Good recovery was achieved in all patients after targeted EBP. No serious complications occurred in patients treated with targeted EBP during the 1 to 7 years of follow-up. Conclusions Targeted and repeat EBPs are rational choices for treatment of refractory SIH caused by CSF leakage.

Large-diameter compression arteries as a possible facilitating factor for trigeminal neuralgia: analysis of axial and radial diffusivity.

BACKGROUND: Neurovascular compression (NVC) of the trigeminal nerve is associated with trigeminal neuralgia (TN). Some arteries that compress the trigeminal nerve are large, while others are small. This study evaluated the influence of diameter of compression arteries (DCA) on NVC with and without TN using axial diffusivity (AD) and radial diffusivity (RD) of magnetic resonance (MR) imaging. METHODS: Fifty TN patients with unilateral NVC, 50 asymptomatic patients with unilateral NVC, and 50 healthy controls (HC) were divided into three groups (NVC with TN, NVC without TN, and HC). The three groups were imaged with a 3.0-T MR system using three-dimensional fast imaging employing steady-state acquisition (3D FIESTA) and diffusion tensor imaging (DTI). We compared the mean size of DCA between NVC with and without TN. The mean values of AD and RD at the site of NVC were compared between the three groups. Correlation analyses were performed between the DCA and the diffusion metrics (AD and RD) in NVC patients with and without TN. RESULTS: The mean DCA in NVC patients with TN (1.58 ± 0.34 mm) was larger than that without TN (0.89 ± 0.29 mm). Compared with NVC without TN and HC, the mean values of RD at the site of NVC with TN were significantly increased; however, no significant changes of AD were found between the groups. Correlation analysis showed that DCA positively correlated with RD in NVC patients with and without TN (r = 0.830, p = 0.000). No significant correlation was found between DCA and AD (r = 0.178, p = 0.077). CONCLUSIONS: Larger-diameter compression arteries may increase the chances of TN, and may be a possible facilitating factor for TN.

[Clinical characteristics of 42 cases of spontaneous intracranial hypotension with cerebrospinal leakage].

OBJECTIVE: To review the clinical characteristics of spontaneous intracranial hypotension (SIH) with cerebrospinal fluid (CSF) leakage. METHODS: Clinical data of 42 SIH patients with cerebrospinal leakage, whose diagnosis met the criteria of the International Headache Classification, were retrospectively reviewed. The patients were divided into short (n=27) and long (n=15) course groups. The clinical data and imaging features were compared between two groups. RESULTS: Thirty-nine patients (92.9%) had orthostatic headache. Compared with the short course group, the frequency of headache were significantly lower in patients with long disease duration (80% vs 100%, P =0.040); the ratio of high CSF opening pressure (>=60.0 mm H2O), the average CSF opening pressure, and the frequencies of subdural hematoma were higher in long course group than those in short course group [60.0% vs 20.8%, (64.7±42.1) vs (40.0±33.8)mm H2O, and 50.0% vs 11.6%; P=0.019, 0.038 and 0.018, respectively]. Forty-two patients underwent CT myelography;definite focal CSF leakage sites were found in all patients and multiple sites of CSF leakage in 38 patients. CONCLUSION: All SIH do not necessarily show the typical clinical manifestations, and cranial MRI and CT myelography are helpful in the diagnosis. Because of higher risk of subdural hemorrhage, patients with long disease duration require active intervention.

Vascular compression of the trigeminal nerve in asymptomatic individuals: a voxel-wise analysis of axial and radial diffusivity.

BACKGROUND: Neurovascular compression (NVC) of the trigeminal nerve is associated with trigeminal neuralgia (TN), but also occurs in many asymptomatic individuals. The purpose of this study was to investigate the possible microstructural tissue changes of trigeminal nerves (TGN) in asymptomatic individuals with NVC by using axial diffusivity (AD) and radial diffusivity (RD) of MR imaging and to discuss its underlying mechanisms. METHODS: Twenty asymptomatic individuals with unilateral NVC and 18 healthy controls (HCs) were divided into three groups (compressed, uncompressed side in asymptomatic individuals and HCs). Three groups were imaged with a 3.0-T MR system using three-dimensional fast imaging employing steady-state acquisition (3D FIESTA) and diffusion tensor imaging (DTI). We placed a region of interest over the root entry zone of the TGN and measured fractional anisotropy (FA), AD and RD. The mean values of FA, AD and RD were compared among the three groups. RESULTS: No significant changes in any of the diffusion metrics (FA, RD and AD) were found among the three groups (compressed, uncompressed side in asymptomatic individuals and HCs). CONCLUSIONS: Our study demonstrated that neither demyelination nor axonal injury is found in asymptomatic individuals with NVC.

Somatotopic reorganization of hand representation in bilateral arm amputees with or without special foot movement skill.

Bilateral arm amputees usually are excellent foot users. To explore the plasticity of the primary motor cortex in upper-extremities amputees and to determine if the acquisition of special foot movement skill is related with the bilateral hand amputation, we studied the primary motor cortex by using combined task and resting state functional magnetic resonance imaging (fMRI). We investigated 6 bilateral arm amputees with or without special foot movement skill. In the task fMRI study, we found that toe tapping of all the amputees activated the bilateral hand area, including cases without special foot skill. In addition, cases without special foot skill mainly activated the precentral gyrus, which differed from those with more adept foot motor skill who activated both the precentral and postcentral gyri. To further understand the plasticity of the hand area, the resting state functional connectivity was investigated between the foot and hand regions. One-tailed two-sample t-test suggested that the connections between two areas became significantly stronger in the amputee group. Our study demonstrates that hand region of the cortex does not remain 'silent' after bilateral arm amputation, but rather is recruited by other modalities such as adjacent or nonadjacent cortexes to process motor information in a functionally relevant manner. From the data presented, it seems that the bilateral arm amputees have a strong potential to develop new skills in their remaining extremities and practice may further enhance this potential.

Image quality and dose performance of 80 kV low dose scan protocol in high-pitch spiral coronary CT angiography: feasibility study.

To investigate the image quality and dose performance of 80 kV high-pitch spiral (HPS) coronary CT angiography (CCTA). 106 patients consecutively enrolled into prospectively ECG-triggering HPS CCTA (pitch = 3.4) exam using kV/ref. mAs = 80/400, 100/370, and 120/370 when patient BMI was ≤22.5 (n = 40), between 22.5 and 27.5 (n = 53) and >27.5 kg/m² (n = 13). Image quality was assessed per-segment by two observers independently using a 4-point scale (1-excellent, 4-non-diagnosable). Image noise and signal-to-noise ratio (SNR), contrast-to-noise ratio were measured. Diagnostic image quality was obtained in 503 of 507, 687 of 693, 164 of 167 coronary segments in 80, 100, 120 kV groups without significant difference (P = 0.482). The proportions of segments with score 1-4 were not significantly different among three kV groups (all P > 0.05). Image noise were significantly higher in 80 kV group than 100 and 120 groups (P < 0.001), while SNR was not (P = 0.097). The effective dose of 80 kV group (0.36 ± 0.03 mSv) was significantly lower than that of 100 kV group (0.86 ± 0.08 mSv) and 120 kV group (1.77 ± 0.18 mSv). The mean ± SD of HR in all patients was 54.8 ± 5.1 bpm. 80 kV HPS CCTA is feasible for patient with BMI ≤ 22.5 kg/m² which can save 58% dose than 100 kV group, while maintain diagnosable image quality.

[Researching the character and stenosis of coronary calcified plaques by using 16-slice spiral CT].

OBJECTIVE: To explore the characters of coronary calcified plaques by using 16-slice spiral CT and determine their stenosis degree according to the results of catheter coronary angiography. METHODS: Twenty patients who had received 16-slice spiral CT coronary angiography and conventional coronary angiography (CAG) were found to be with calcified plaques. The characters of these plaques, including the diameter of calcified plaques and lumen diameter of the exact artery segment, were retrospectively analyzed. The stenosis degree of the corresponding segment was judged in accordance with the results of CAG. RESULTS: Totally 84 calcified plaques were observed in 16-slice spiral CT images in these 20 patients. Among them there were 16 small nodules (diameter: < 0.15 cm), 56 purely calcified plaques (diameter: > or = 0. 15 cm), and 12 complex plaques with calcify component. There was no obvious stenosis in artery segments with little calcified nodules. The stenosis degree of most segments with purely calcified plaques (75%) was less than 50%. The stenosis degree had no significant correlation with the size of plaques (P > 0.05). However, the stenosis degree of complex plaques had much closer relationship with the characters of mixed plaques. CONCLUSIONS: Most coronary segments with calcified plaques have slight stenosis. Their stenosis degree is not related with the size of plaques. The stenosis degree of complex plaques has a closer relationship with the characters of mixed plaques.

[Functional MRI of human brain in musicians and non-musicians].

OBJECTIVE: To explore the differences in brain activation between musicians and non-musicians by use of functional MRI. METHODS: Twelve right-handed musicians and twelve right-handed non-musicians were recruited in the study. During a listening task, they were scanned on the Sigma 1.5T scanner (GE) while they were passively listening to several segments of music of "the Butterfly Love" and the white noise with same physical energy. RESULT: Both musicians and non-musicians demonstrated bilateral transverse gyrus weak activated while listening to the white noise. But when listening to music, they showed bilateral temporal areas strongly activated including superior temporal gyrus, transverse gyrus and some middle temporal areas. Moreover, musicians showed relative left dominance (10/12), whereas non-musicians demonstrated right dominance(11/12). Furthermore,besides bilateral temporal areas, more and stronger activated areas were found in musicians such as cuneus, precuneus,medial frontal and left middle occipital gyrus. CONCLUSION: There are different neuro-patterns between musicians and non-musicians.

Multi-detector CT enterography with iso-osmotic mannitol as oral contrast for detecting small bowel disease.

AIM: To assess the feasibility and usefulness of multi-detector CT enterography with orally administered iso-osmotic mannitol as negative contrast in demonstrating small bowel disease. METHODS: Thirteen volunteers and 38 patients with various kinds of small bowel disease were examined. We administered about 1 500 mL iso-osmotic mannitol as negative contrast agent and then proceeded with helical CT scanning on a Siemens Sensation 16 scanner. All volunteers and patients were interviewed about their tolerance of the procedure. Two radiologists post-processed imaging data with MPR, thin MIP, VRT and INSPACE when necessary and then interpreted the scans, and adequacy of luminal distention was evaluated on a four-point scale. Demonstration of features of various kinds of small bowel disease was analyzed. RESULTS: The taste of iso-osmotic mannitol is good (slightly sweet) and acceptable by all. Small bowel distention was excellent and moderate in most volunteers and patients. CT features of many kinds of diseases such as tumors, Crohn's disease,and small bowel obstruction, etc. were clearly displayed. CONCLUSION: Multi-detector CT enterography with iso-osmotic mannitol as negative contrast to distend the small bowel is a simple, rapid, noninvasive and effective method of evaluating small bowel disease.

Evaluation of computed tomography coronary angiography in patients with a high heart rate using 16-slice spiral computed tomography with 0.37-s gantry rotation time.

The aim of our study is to evaluate computed tomography (CT) coronary angiography in patients with a high heart rate using 16-slice spiral CT with 0.37-s gantry rotation time. We compare the image quality of patients whose heart rates were over 70 beats per minute (bpm) with that of patients whose heart rates were 70 bpm or less. Sixty patients with various heart rates underwent retrospectively ECG-gated multislice spiral CT (MSCT) coronary angiography. Two experienced observers who were blind to the heart rates of the patients evaluated all the MSCT coronary angiographic images and calculated the assessable segments. A total of 620 out of 891 (69.6%) segments were satisfactorily visualized. On average, 10.3 coronary artery segments per patient could be evaluated. In 36 patients whose heart rates were below 70 bpm [mean 62.2 bpm+/-5.32 (standard deviation, SD)], the number of assessable segments was 10.72+/-2.02 (SD). In the other 24 patients whose heart rates were above 70 bpm [mean 78.6 bpm+/-8.24 (SD)], the corresponding number was 9.75+/-1.74 (SD). No statistically significant difference was found in these two subgroups' t test, P>0.05. The new generation of 16-slice spiral CT with 0.37-s rotation time can satisfactorily evaluate the coronary arteries of patients with high heart rates (above 70 bpm, up to 102 bpm).