The utility of susceptibility-weighted imaging to evaluate the extent of iron accumulation in the choroid plexus of patients with ß-thalassaemia major.

AIM: To assess iron accumulation in the choroid plexus of ß-thalassaemia patients using fast spin echo (FSE) T2-weighted, gradient echo (GRE) T2*-weighted, susceptibility-weighted imaging (SWI) and compare the results. MATERIALS AND METHODS: Eighteen patients with transfusion-dependent ß-thalassaemia and the control group underwent magnetic resonance imaging (MRI) examinations. Signal intensities were separately evaluated using a "number of hypointensity in the choroid plexus" (NHICP) grading system on axial FSE T2-weighted, GRE T2*-weighted, and SWI images. The NHICP grading system scores were compared using the chi-squared test. Spearman's correlation analysis was used to explore relationships between the variables and NHICP grading system scores. RESULTS: The sensitivity of each technique was calculated: FSE T2-weighted imaging=0.17, GRE T2*-weighted imaging=0.48, and SWI=0.81. Three-sample test for equality of proportions showed that chi-squared=74.85, df=2, p<0.0001. All of the FSE T2-weighted, GRE T2*-weighted, and SWI images differed significantly in terms of their capacity to reveal iron accumulation in the choroid plexus. Of the three methods, SWI was the most sensitive. CONCLUSIONS: SWI is useful for revealing iron deposition in the brains of ß-thalassaemia patients, especially those in the early stages of disease, and it can be used to predict disease prognosis. The present study contributes to an understanding of the important role played by the choroid plexus in brain iron metabolism.

Evaluation of parenchymal neuro-behçet disease by using susceptibility-weighted imaging.

BACKGROUND AND PURPOSE: Neurologic involvement in Behçet disease, also known as NBD, is one of the most devastating manifestations of the disease. The precise pathologic mechanism of parenchymal NBD lesions has not been established. We evaluated lesion characteristics and probable venous hemorrhage in parenchymal NBD by using SWI, and we compared the imaging results with conventional MR imaging sequences. MATERIALS AND METHODS: We performed cranial MR imaging by using a 1.5T scanner in 23 patients with a definitive diagnosis of parenchymal NBD. We compared the proportion of lesion detection and the performance of hemorrhagic detection with the T2 FSE, T2*GE, and SWI magnitude, and SWI mIP by using the χ(2) test. RESULTS: The proportion of lesion detection with both SWI magnitude and SWI MinMIP was significantly larger than that with T2*GE. The proportions of lesion detection among all other pairs of methods were not significantly different according to the corresponding P value (χ(2) = 17.4929, df = 3, P = .0006). Proportions of hypointense hemorrhagic lesions with T2 FSE and T2*GE were not significantly different, and likewise for the proportions of hypointense hemorrhagic lesions with SWI magnitude and SWI mIP. In contrast, the proportions of hypointense hemorrhagic lesions with SWI magnitude and SWI mIP were significantly larger than that with T2 FSE and T2*GE (χ(2) = 108.5396, df = 3, P < .0001). CONCLUSIONS: Most of the lesions in parenchymal NBD were found to be hemorrhagic with SWI, supporting the proposed venous theory in pathology. In addition, compared with T2 FSE and T2*GE sequences, SWI was more successful in the determination of widespread involvement of the disease, particularly in nonchronic cases.

Cerebral microhemorrhages detected by susceptibility-weighted imaging in amateur boxers.

BACKGROUND AND PURPOSE: SWI is a new technique for evaluating diffuse axonal injury associated with punctate hemorrhages. The aim of our study was to determine the prevalence of cerebral microhemorrhages in amateur boxers compared with nonboxers by using SWI and to evaluate the sensitivity of SWI compared with T2 FSE and T2*GE sequences. MATERIALS AND METHODS: We performed cranial MR imaging with a 1.5T scanner in 21 amateur boxers and 21 control subjects. The study protocol included conventional MR images, T2 FSE, T2*GE, and SWI sequences. The proportions of boxers and controls having CSP, DPVS, cerebral atrophy, cerebellar atrophy, ventricular dilation, PSWMD, and microhemorrhages were computed and were compared by using the χ(2) test of proportions. The relationship between microhemorrhages and boxing-related covariates was assessed by using the Wilcoxon rank sum test. The association between the categories was tested by using the Fisher exact test. RESULTS: Using SWI, microhemorrhages were found in 2 (9.52%) of 21 boxers. The microhemorrhages were not visible on T2 FSE or T2*GE images. The proportion of subjects with microhemorrhages did not differ significantly between the boxers and control subjects (χ(2) = 0.525, df = 1, P = .4688). The prevalence of CSP and DPVS was significantly higher in the boxers than in the control subjects. CONCLUSIONS: More microhemorrhages were detected in amateur boxers than in controls, but this difference was not statistically significant.

Cerebrospinal fluid flow dynamics in patients with multiple sclerosis: a phase contrast magnetic resonance study.

Cerebrospinal fluid (CSF) flow dynamics, which supposedly have a strong relationship with chronic cerebrospinal venous insufficiency (CCSVI), might be expected to be affected in multiple sclerosis (MS) patients. In this study, CSF flow at the level of the cerebral aqueduct was evaluated quantitatively by phase contrast magnetic resonance imaging (PC-MRI) to determine whether CSF flow dynamics are affected in MS patients. We studied 40 MS patients and 40 healthy controls using PC-MRI. We found significantly higher caudocranial (p=0.010) and craniocaudal CSF flow volumes (p=0.015) and stroke volume (p=0.010) in the MS patients compared with the controls. These findings may support the venous occlusion theory, but may also be explained by atrophy-dependent ventricular dilatation independent of the venous theory in MS patients.

Intrathecal gadolinium-enhanced MR cisternography in the evaluation of CSF leakage.

BACKGROUND AND PURPOSE: Radiologic identification of the location of the CSF leakage is important for proper surgical planning and increases the chance of dural repair. This article describes our experience in analyzing clinically suspected cranial CSF fistulas by using MR imaging combined with the intrathecal administration of a gadolinium-based contrast agent. MATERIALS AND METHODS: A total of 85 consecutive patients with suspected CSF fistulas who presented with persistent or intermittent rhinorrhea or otorrhea lasting for more than 1 month between 2003 and 2007 were included in this study. RESULTS: We observed objective CSF leakage in 64 of 85 patients (75%). The CSF leak was located in the ethmoidal region in 37 patients (58%), in the superior wall of the sphenoid sinus in 8 patients (13%), in the posterior wall of the frontal sinus in 10 patients (15%), in the superior wall of the mastoid air cells in 6 patients (9%), and from the skull base into the infratemporal fossa in 1 patient (2%). Two patients (3%) showed leakage into >1 paranasal sinus. CONCLUSIONS: MR cisternography after the intrathecal administration of gadopentate dimeglumine represents an effective and minimally invasive method for evaluating suspected CSF fistulas along the skull base. It provides multiplanar capabilities without risk of radiation exposure and is an excellent approach to depict the anatomy of CSF spaces and CSF fistulas.

A cerebellar demyelinating lesion following treatment of acne with isotretinoin.

We report the case of a demyelinating lesion located in the left cerebellar region that developed 3 months after the onset of oral isotretinoin treatment. In April 2001, 1 year before admission, the patient underwent cranial magnetic resonance imaging (MRI) because of endocrinological problems. This was found to be completely normal. In January 2002, oral isotretinoin treatment was started to treat severe acne. Three months after the onset of therapy, the patient reported lack of appetite, faintness and tinnitus. Her second cranial MRI scan showed a cerebellar lesion, and oral isotretinoin treatment was stopped (April 2002). One month after the cessation of oral isotretinoin treatment, the lesion became less prominent on the MRI scan, and after 3 months, it had disappeared. Although it is difficult to determine the causal association between the demyelinating cerebellar lesion and isotretinoin treatment, we would like to alert physicians to this possibility, because of the common usage of this drug in daily practice.

Spontaneous intracranial hypotension after labor without spinal intervention.

We present a 29-year-old woman admitted with severe postural headache after spontaneous term labor. Lactation ceased for the duration of headache. Magnetic resonance imaging (MRI) revealed dural thickening that is suggestive of spontaneous intracranial hypotension. CT-cisternography disclosed cervicodorsal dural leak. She was treated with a high-volume epidural blood patch (EBP) and her symptoms were relieved. Lactation returned to normal after EBP. She had normal findings on follow-up MRI examination at 6 months.

Gadolinium-enhanced MR cisternography to evaluate dural leaks in intracranial hypotension syndrome.

BACKGROUND AND PURPOSE: We evaluated the use of MR cisternography after intrathecal administration of gadopentetate dimeglumine to detect the presence and localization of CSF leaks in 19 patients diagnosed with spontaneous intracranial hypotension syndrome according to the criteria of International Headache Society. MATERIALS AND METHODS: Lumbar puncture with an injection of 0.5 mL of gadopentetate dimeglumine into the subarachnoid space in the lumbar area was performed. MR images of the cervical, thoracic, and lumbar regions in axial, coronal, and sagittal planes with fat-saturated T1-weighted images were acquired. RESULTS: We observed objective CSF leakage in 17 (89%) of 19 patients. In 14 of these 17 patients, the site of dural tear was demonstrated accurately. In 3 of these 17 patients, the contrast leakage was diffuse, and site of the leak could not be located accurately. No leakage was observed in 2 patients. No complications were detected in any of the patients during the first 24 hours after the procedure or during the 6- to 12-month follow-up. CONCLUSION: The current results demonstrate the relative safety, accuracy, and feasibility of intrathecal gadolinium-enhanced MR cisternography to evaluate dural leaks.

Persistent hypoglossal artery associated with a ruptured ipsilateral posterior inferior cerebellar artery aneurysm. A case report.

A rare case of persistent hypoglossal artery in conjuction with a ruptured aneurysm at the left proximal posterior inferior cerebellar artery (PICA) is presented. A ruptured PICA aneurysm was successfully treated by endovascular occlusion of the aneurym with coiling.

Reversible reduction of apparent diffusion coefficient values in bilateral internal capsules in transient hypoglycemia-induced hemiparesis.

A woman aged 68 years who experienced recurrent right hemiparesis caused by hypoglycemia was admitted to our hospital. When she was experiencing a low level of glucose, diffusion-weighted MR imaging showed the presence of hyperintensity lesions in the bilateral internal capsule. Diffusion-weighted MR imaging has been infrequently performed in patients with hypoglycemia. We report the reversible hyperintensity lesions on diffusion-weighted MR imaging in a hypoglycemic period in a patient with reversible hemiparesis. A reduction of apparent diffusion coefficient in a hypoglycemic period was clearly shown.

The R110C mutation in Notch3 causes variable clinical features in two Turkish families with CADASIL syndrome.

Mutations in Notch3 gene are responsible for the cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). It is a late onset neurological disorder recognized by recurrent strokes and dementia. We describe here the clinical and molecular findings of three unrelated Turkish families with CADASIL syndrome. Two of the families were identified to have the same mutation, p.R110C (c.C328T), located in exon 3 of the Notch3 gene. Interestingly, the phenotypic expression of the disease in these two families was markedly different in severity and age of onset implicating additional genetic and/or non-genetic modulating factors involved in the pathogenesis. In addition, we identified the novel p.C201R (c.T601C) mutation in exon 4 of the Notch3 gene in a proband of the third family with two consecutive stroke-like episodes and typical MRI findings. Mutations described here cause an odd number of cysteines in the N-terminal of the EGF domain of Notch3 protein, which seems to have an important functional effect in the pathophysiology of CADASIL. The phenotypic variability in families carrying the same molecular defect as presented here makes the prediction of prognosis inconceivable. Although DNA analysis is effective and valuable in diagnosing approximately 90% of the CADASIL patients, lack of genotype-phenotype correlation and prognostic parameters makes the presymptomatic genetic counseling very difficult.

Adult intramedullary mature teratoma of the spinal cord: review of the literature illustrated with an unusual example.

Intramedullary mature teratomas particularly in adults are rarely encountered. In this manuscript the authors have reviewed the adult intramedullary lesions of the spinal cord published in the literature that are harbouring the characteristics of a mature teratoma and analysed the results with respect to histopathology, epidemiology, diagnostic methods and treatment. An illustrative case of an extremely unusual localization is also presented.

Vasculopathic changes in the cerebral arterial system with neurobrucellosis.

Brucellosis is a zoonotic disease characterized by multisystem involvement. Nervous system involvement is rare, with a reported incidence of 3%-13%. Brucellosis can also be manifested in the form of vasculopathy. Cerebral vasculopathy due to brucellosis is a very rare entity, with only a few cases reported in the literature. We present a patient with neurobrucellosis who had involvement of cerebral vasculature demonstrated by angiography.

Thalamic proton magnetic resonance spectroscopy in vegetative state induced by traumatic brain injury.

OBJECTIVES: To determine whether proton magnetic resonance spectroscopy (MRS), a newer radiographic technology, would be useful in the evaluation of the thalamus of patients in vegetative states resulting from traumatic brain injury. METHODS: 14 victims of severe traumatic brain injury who were in the vegetative state and whose magnetic resonance images of the thalamus were normal underwent bilateral thalamic proton (MRS) studies. The N-acetyl aspartate to creatine (NAA:Cr) and choline to creatine (Cho:Cr) ratios were obtained for each patient. The proton thalamic MRS findings of patients who were in a persistent vegetative state (n = 8) and in patients who had regained awareness after being in the vegetative state (n = 6) were compared with proton thalamic MRS findings in five healthy volunteers. RESULTS: While conventional magnetic resonance imaging suggested that each patient had a normal thalamus, proton MRS indicated that the thalamus of each patient in the series was damaged. The NAA:Cr ratio was significantly lower in the thalami of both the patients who remained in a persistent vegetative state for the duration of the study and in those who regained awareness after being in the vegetative state (p < 0.001). In addition, NAA:Cr ratios were lower in the group of patients who remained in a persistent vegetative state than in the group of patients who regained awareness after being in the vegetative state (p < 0.001). CONCLUSIONS: Results suggest that the NAA:Cr ratio within the thalamus is significant and that thalamic MRS may be helpful when attempting to determine the degree of severity of neuronal and axonal injury in patients in the vegetative state.