Heterogeneity of Baló's concentric sclerosis: a study of eight cases with different therapeutic concepts.

BACKGROUND: Baló's Concentric Sclerosis (BCS) is a rare heterogeneous demyelinating disease with a variety of phenotypes on Magnetic Resonance Imaging (MRI). Existing literature lacks data especially on the therapeutic approach of the disease which we intended to elucidate by means of suggesting a new possible BCS classification and introducing different therapeutic concepts based on each BCS-subgroup characteristics. METHODS: We present a retrospective study of eight treated patients with BCS-type lesions, emphasizing on MRI characteristics and differences on therapeutic maneuvers. RESULTS: Data analysis showed: at disease onset the BCS-type lesion was tumefactive (size ≥2 cm) in 6 patients, with a mean size of 2.7 cm (± 0.80 SD); a coexistence of MS-like plaques on brain MRI was identified in 7 patients of our cohort. The mean age was 26.3 years (±7.3 SD) at disease onset and the mean follow-up period was 56.8 months (range 9-132 months). According to radiological characteristics and response to therapies, we further categorized them into 3 subgroups: a) Group-1; BCS with or without coexisting nonspecific white matter lesions; poor response to intravenous methylprednisolone (IVMP); treated with high doses of immunosuppressive agents (4 patients), b) Group-2; BCS with typical MS lesions; good response to IVMP; treated with MS-disease modifying therapies (2 patients), c) Group-3; BCS with typical MS lesions; poor response to IVMP; treated with rituximab (2 patients). CONCLUSIONS: Our study introduces a new insight regarding the categorization of BCS into three subgroups depending on radiological features at onset and during the course of the disease, in combination with the response to different immunotherapies. Immunosuppressive agents such as cyclophosphamide are usually effective in BCS. However, therapeutic alternatives like anti-CD20 monoclonal antibodies or more classical disease-modifying MS therapies can be considered when BCS has also mixed lesions similar to MS. Future studies with a larger sample size are necessary to further establish these findings, thus leading to better treatment algorithms and improved clinical outcomes.

MRI Planimetry and Magnetic Resonance Parkinsonism Index in the Differential Diagnosis of Patients with Parkinsonism.

BACKGROUND AND PURPOSE: Differential diagnosis of multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration from Parkinson disease on clinical grounds is often difficult. MR imaging biomarkers could assist in a more accurate diagnosis. We examined the utility of MR imaging surface measurements (MR imaging planimetry) in the differential diagnosis of patients with parkinsonism. MATERIALS AND METHODS: Fifty-two patients with Parkinson-plus (progressive supranuclear palsy, n = 24; corticobasal degeneration, n = 9; multiple system atrophy, n = 19), 18 patients with Parkinson disease, and 15 healthy controls were included. Corpus callosum, midbrain, and pons surfaces; relevant indices; and the Magnetic Resonance Parkinsonism Index were calculated. Corpus callosum subsection analysis was performed, and the corpus callosum posteroanterior gradient was introduced. RESULTS: A Magnetic Resonance Parkinsonism Index value of >12.6 discriminated progressive supranuclear palsy from other causes of parkinsonism with a 91% sensitivity and 95% specificity. No planimetry measurement could accurately discriminate those with multiple system atrophy with parkinsonism from patients with Parkinson disease. A corpus callosum posteroanterior gradient value of ≤191 was highly specific (97%) and moderately sensitive (75%) for the diagnosis of corticobasal degeneration versus all other groups. A midbrain-to-corpus callosum posteroanterior gradient ratio of ≤0.45 was highly indicative of progressive supranuclear palsy over corticobasal degeneration (sensitivity 86%, specificity 88%). CONCLUSIONS: MR imaging planimetry measurements are potent imaging markers of progressive supranuclear palsy and promising markers of corticobasal degeneration but do not seem to assist in the diagnosis of multiple system atrophy with parkinsonism.

Diffusion tensor imaging (DTI) in the detection of white matter lesions in patients with mild cognitive impairment (MCI).

Mild cognitive impairment (MCI) is recognized as a precursor to dementia. The amnestic MCI progresses usually to Alzheimer disease. Amnestic MCI multiple domain (md-MCI) seems to progress more rapidly than amnestic MCI single domain (a-MCI). In an attempt to identify patients at risk, we examined white matter changes in MCI subtypes using diffusion tensor imaging (DTI). We also tried to correlate DTI findings to neuropsychological tests. Forty-four amnestic single domain (a-MCI) patients, 19 amnestic multi domain (md-MCI), and 25 cognitively normal (NC) controls were included in the present study. All participants were assessed clinically using a battery of cognitive tests. DTI was performed to measure fractional anisotropy (FA) and apparent diffusion coefficient (ADC). Areas studied were corpus callosum, posterior cingulum (PC), anterior cingulum (AC), and superior longitudinal fasciculus (SLF). ADC and FA of the above areas were related to the scores of certain neuropsychological tests that evaluate visual and verbal memory. No difference in DTI measurements was found between the two MCI subtypes. ADC in MCI cases was increased in comparison with NC in the genu, PC, right SLF, and left AC. FA was spared. Verbal memory was related to ADC of the genu, PC, right AC and right SLF, and to FA of the left SLF. Visual memory was related to ADC of the genu, PC, right AC, and SLF. The strongest correlation found was between the visual memory and the ADC of the right PC (Spearman ρ = 0.45, p < 0.001). DTI revealed that ADC was increased in certain brain areas in MCI patients. No difference in DTI measurements was found between the two MCI subtypes. DTI indices correlate with cognitive performance.

Radiological confirmation of brain death: digitised cerebral parenchymography. Preliminary report.

Rates of organ procurement from brain dead subjects have fallen substantially in recent years. In France, the legal definition of brain death is based on electroencephalographic criteria in patients with clinical evidence of irreversible coma. However, sedative drugs used in intensive care units usually render the electroencephalogram uninterpretable, and in our medicolegal framework, it is necessary that intracerebral circulatory arrest be demonstrated. We discuss the value of the various available techniques and report our experience with digitised intra-arterial cerebral parenchymography. This simple, fast technique does not alter physiological conditions and provides high-quality images, ensuring prompt diagnosis, which is a prerequisite for optimal organ harvesting.

In vivo proton MR spectroscopy: the diagnostic possibilities of lipid resonances in brain tumors.

The lipid resonances detected in proton MR spectra of brain tumors were evaluated from the diagnostic point of view. Seventy five non-radiated brain tumors were examined in vivo and lipids were detected in 29% of anaplastic astrocytomas grade III, 60% of glioblastomas multiforme and 50% of metastatic tumors. Lipids were also detected in all brain abscesses and an epidermoid cyst. No lipids were detected in benign tumors (low grade and pilocytic astrocytomas, oligodendrogliomas, meningiomas, pituitary adenomas etc.). Thus, the presence of lipids in the MR spectra is indicative of malignancy (with a few exceptions).

Local intraarterial thrombolysis in the carotid territory.

SUMMARY: A new series of 142 patients treated by local intraarterial thrombolysis is presented. After haemorrhage was ruled out by CT, all patients had an emergency angiogram. Patients with occlusion of the lenticulostriate arteries were not treated after the sixth hour (79 cases). Patients without involvement of these arteries were treated up to the 12th hour (61 cases). This selection led to a dramatic reduction of post-thrombolysis intraparenchymatous haemorrhage (0.7%) compared to other series. All deaths (7 cases) were related to vasogenic oedema due to incomplete reduction of infarcted cerebral volume, in most cases (4 cases) in intracerebral extension of a cervical internal carotid occlusion. Digitized parenchymography proved to be a reliable technique to document the exact location and extent of brain ischaemia before and after thrombolysis. It also allows a prognosis of spontaneous recovery without using thrombolysis. By reducing the infarcted cerebral volume, local intraarterial thrombolysis seems beneficial to the patient as long as a strict selection based on the angiographic location of the occlusion and the time delay is respected.