Neuronal loss is an early component of subacute sclerosing panencephalitis.

OBJECTIVE: We performed diffusion tensor imaging (DTI) and magnetic resonance spectroscopy (MRS) studies in a group of patients with subacute sclerosing panencephalitis (SSPE) in order to estimate the pathologic process underlying the phenotypic variability. METHODS: Patients with SSPE who had MRI including DTI and MRS examinations were evaluated according to their clinical status as determined by the SSPE Scoring System and their mental age as determined by tests appropriate for age and developmental level. Comparisons of fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values and metabolite ratios of frontal periventricular white matter, parieto-occipital periventricular white matter, and globus pallidus in both hemispheres were made between control and SSPE groups, and between SSPE subgroups. RESULTS: Control (n = 18) and SSPE (n = 39) groups differed in all DTI and MRS parameters except FA, choline (Cho), and Cho/creatine (Cr). SSPE cases had higher ADC and lower N-acetylaspartate (NAA), NAA/Cho, and NAA/Cr in all regions of interest, suggesting cell loss. Disease progression rate and neurologic deficit appeared to be associated with the degree of ADC elevation and NAA reduction: the group with severe global deterioration had the lowest NAA (230.75 ± 197.97 in forceps minor), and rapid progression was associated with acute reduction in NAA. CONCLUSIONS: The combination of MRS and diffusion MRI findings suggests neuronal loss can be a primary target in rapidly or subacutely progressing SSPE, and preservation or regeneration of axonal structure may be beneficial in chronic cases.

Effect of cervical spondylosis on vertebral arterial flow and its association with vertigo.

The aim in this study was to evaluate the effect of cervical spondylosis on vertebral arterial flow. The flow changes on Doppler measurements in patients with vertigo were also assessed. Ninety-one patients with different grades of degeneration on disks and apophysis were evaluated. The severity of degeneration was determined by two observers, and the effect of cervical degeneration on vertebral arterial flow was assessed. The relation between vertigo and changes in Doppler measurements of vertebral artery in neck and cranium was analyzed. As total degeneration increases, the flow volume decreases in the right vertebral artery but increases in the left. No other correlation between degeneration scores, cervical curve measurements, and Doppler measurements was noted. According to the total degeneration scores, no differences were noted between the patients with and without vertigo. In patients with vertigo, the resistive index was higher in the neck before the vertebral foramina. In correlations of the ratios of the intra/extracranial vertebral artery with Doppler measurements, the only change was observed in the left flow volume. The severity of degeneration in disks and apophysis does not cause a considerable change in vertebral arterial flow, and also no significant finding on Doppler measurements were noted in patients with vertigo.

Bifid median nerve in patients with carpal tunnel syndrome.

OBJECTIVE: The aim of this study was to investigate the frequency of the anatomic variation of a bifid median nerve in patients with carpal tunnel syndrome (CTS) and to determine the size criteria for sonography. METHODS: On axial sonograms of 320 hands of 170 patients with CTS and 240 hands of 120 unaffected individuals, the median nerve was evaluated morphologically for a bifid median nerve, and the cross-sectional area was measured at 3 levels (radial-ulnar junction, pisiform, and hook of the hamate). Electrophysiologic studies were performed in addition to clinical and sonographic evaluations in all patients, controls with a bifid median nerve, and controls with a cross-sectional area of greater than 0.09 cm(2). RESULTS: A bifid median nerve was seen in 32 (19%) of 170 patients and 11 (9%) of 120 controls. It occurred relatively frequently in patients with CTS (P < .01). The cross-sectional area of the bifid median nerve was relatively higher than that of the nonbifid median nerve in controls at 2 of the 3 levels (P < .001; P = .226; P < .01). The cutoff values for the cross-sectional area at the level of the pisiform were 0.11 cm(2) (sensitivity, 90%; specificity, 99%; P < .001) for patients with a bifid median nerve and 0.10 cm(2) (sensitivity, 98%; specificity, 81%; P < .001) for patients with a nonbifid median nerve. CONCLUSIONS: A bifid median nerve occurs relatively frequently in patients with CTS. It may facilitate compression of the median nerve in the carpal tunnel because of its relatively higher cross-sectional area compared with a nonbifid median nerve. The size criterion for CTS in patients with a bifid median nerve is slightly higher than in those with a nonbifid median nerve.

A comparison of low-dose and normal-dose gadobutrol in MR renography and renal angiography.

OBJECTIVE: It has been advocated that a reduced injection volume with highly concentrated (1 M) contrast material can produce a sharper bolus peak and an increased intravascular first-pass gadolinium concentration when compared with the use of a lower concentration (0.5 M). A higher concentration would also cause a reduction in dose. The purpose of our study was to test the use of a low dose (0.05 mmol/kg) of gadobutrol in magnetic resonance renography and angiography and compare the findings with a dose of 0.1 mmol/kg. MATERIALS AND METHODS: One-hundred-thirty-four patients referred for magnetic resonance angiography for suspected renovascular disease participated in the study. Contrast enhanced MR renography and angiography were performed after administration of a bolus of 0.1 mmol/kg or 0.05 mmol/kg gadobutrol in randomized patients. The relative signal intensity-time curves of the aorta, peripheral cortex and parenchyma, were obtained. Two radiologists evaluated the angiographic images and evaluated the quality of angiography. RESULTS: The signal intensity with a low dose of gadobutrol was significantly lower in early phases, in the peripheral cortex (for 36, 54, 72 and 90 seconds), the parenchyma (for 36, 54, 72 seconds) and the aorta (for 18, 36, 54, 72 seconds). The decreases in the early phase obtained with a low dose of gadobutrol caused blunter time intensity curves. The difference in the quality scores of the readers for the angiographic images for the use of the two different doses was not statistically significant (p > 0.05). CONCLUSION: A lower dose of gadobutrol can be used for MR renal angiography, but for MR renography the normal dose should be used.

Physical function, muscle strength and muscle mass in children on peritoneal dialysis.

The aim of this study was to examine the physical function and muscle strength of children on peritoneal dialysis (PD) and to assess whether the muscle structure alterations influence physical function and muscle strength in these children. Twenty-two children on PD and 16 healthy children were enrolled into the study. A 6-min walk distance and gait speed tests were used to evaluate physical performance. Quadriceps muscle strength (QMS) was measured with a hand-held dynamometer. Magnetic resonance imaging was used to determine the cross-sectional area (CSA) and T2 signal intensity of the quadriceps muscle. Significant differences in the performance of these functional tests were found between PD patients and controls. Quadriceps muscle strength was significantly lower in PD patients than in controls. The CSA corrected for the body mass index (CSA/BMI) was not different between groups, whereas T2 signal intensity was significantly higher in PD patients than in the controls. Physical functioning tests and QMS had a close relationship with muscle CSA/BMI and with T2 signal intensity. In conclusion, along with the other previously documented mechanisms, increased fat in muscles may contribute to the decreased physical functioning and muscle strength in PD patients.

Early spondyloarthropathy: scintigraphic, biological, and clinical findings in MRI-positive patients.

There are no specific diagnostic tests or a gold standard method for measuring disease activity and outcome in spondyloarthropathies (SpA). Many different methods have been developed to assess the signs and symptoms in SpA. The aim of this study was to evaluate the value of scintigraphy, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and Bath Ankilosing Spondylitis Disease Activity Index (BASDAI) in the evaluation of disease activity in early axial SpA diagnosed with magnetic resonance imaging (MRI). Thirty early MRI-positive axial SpA patients (23 males, 7 females) with a median age of 35 (18-55) years and a median duration of inflammatory low back pain of 24 (8-60) months were included in the study. In the patients with sacroiliitis, the sensitivity, specificity, and positive and negative predictive values of disease activity parameters were determined regarding MRI as the gold standard method. The sensitivities of quantitative scintigraphy, visual scintigraphy, ESR, CRP, and BASDAI were 32, 82, 35, 71, and 60%, respectively. The specificities of quantitative scintigraphy, ESR, CRP, and BASDAI were 100, 100, 50, and 100%, respectively. The positive predictive values of quantitative scintigraphy, visual scintigraphy, ESR, CRP, and BASDAI were 100, 92, 100, 95, and 100%, respectively. The negative predictive values of quantitative scintigraphy, ESR, CRP, and BASDAI were 9, 10, 11, and 15%, respectively. Regarding MRI as the gold standard in the evaluation of disease activity, combined visual and quantitative bone scintigraphy can be valuable in patients with MRI-incompatible implants. Additionally, use of cheaper, simple, and readily reproducible tests such as CRP and BASDAI together could be valuable and practical in detecting disease activity in long-term follow-up of these patients.

Joubert syndrome with atrial septal defect and persistent left superior vena cava.

Joubert syndrome is a rare disorder characterized by hypotonia, ataxia, episodic hyperpnoea, psychomotor delay, abnormal ocular movements, and molar tooth sign on magnetic resonance imaging (MRI). This syndrome is inherited as an autosomal recessive trait, but the molecular basis and specific chromosomal locus have not yet been identified. MRI features are the most important diagnostic criteria. Molar tooth sign was previously described in Joubert syndrome and was found in 85% of patients with Joubert syndrome. Many authors now claim that this finding can be present in other syndromes, including Dekaban-Arima, Senior-Löken, COACH, and Varadi-Papp. We present a 7-month-old girl with Joubert syndrome in whom MRI showed the typical features of this condition. She also had polydactyly, atrial septal defect, and persistent left superior vena cava.

Bilateral adrenal cystic neuroblastoma with superior vena cava syndrome and massive intracystic haemorrhage.

Bilateral cystic adrenal tumours are a rare presentation of neuroblastoma. Intratumoural haemorrhage is a frequent finding in neuroblastoma, but is rarely symptomatic. We present an 11-month-old girl with predominantly cystic bilateral neuroblastomas and distant lymph-node metastasis. Massive intracystic haemorrhage and superior vena cava (SVC) syndrome were ominous prognostic factors, leading to death. Large tumours with intracystic haemorrhage might require a conservative approach.

An unusual supracondylar process syndrome.

We report a case of a supracondylar process that was located beneath the neurovascular bundle and caused tenting of the bundle with elbow extension and supination.