Idiopathic normal pressure hydrocephalus (iNPH) is a syndrome that comprises a triad of gait disturbance, dementia and urinary incontinence, associated with ventriculomegaly in the absence of elevated intraventricular cerebrospinal fluid (CSF) pressure. It is important to identify patients with iNPH because some of its clinical features may be reversed by the insertion of a CSF shunt. The diagnosis is based on clinical history, physical examination and brain imaging, especially magnetic resonance imaging (MRI). Recently, some papers have investigated the role of diffusion tensor imaging (DTI) in evaluating white matter alterations in patients with iNPH. DTI analysis in specific anatomical regions seems to be a promising MR biomarker of iNPH and could also be used in the differential diagnosis from other dementias. However, there is a substantial lack of structured reviews on this topic. Thus, we performed a literature search and analyzed the most recent and pivotal articles that investigated the role of DTI in iNPH in order to provide an up-to-date overview of the application of DTI in this setting. We reviewed studies published between January 2000 and June 2020. Thirty-eight studies and four reviews were included. Despite heterogeneity in analysis approaches, the majority of studies reported significant correlations between DTI and clinical symptoms in iNPH patients, as well as different DTI patterns in patients with iNPH compared to those with Alzheimer or Parkinson diseases. It remains to be determined whether DTI could predict the success after CSF shunting.
Purpose Diffusion tensor imaging is a magnetic resonance technique that provides information about the orientation and anisotropy of the white matter tracts. The aim of this study was to analyse diffusion tensor imaging quantitative parameters in idiopathic normal pressure hydrocephalus patients, in order to determine whether this method could correlate to clinical scores and cerebrospinal fluid flowmetry data. Methods and materials Fifteen consecutive patients with idiopathic normal pressure hydrocephalus and 15 age-matched controls underwent cerebrospinal fluid flowmetry and diffusion tensor imaging using a 1.5 Tesla system. Fractional anisotropy, mean diffusivity, axial diffusivity and radial diffusivity values were calculated using region of interest atlas-based tract-mapping in nine cerebral areas and compared among the two groups. In addition, for idiopathic normal pressure hydrocephalus patients, diffusion tensor imaging parameters were correlated to clinical scores (mini mental state examination and frontal assessment battery) and cerebrospinal fluid flowmetry data. Results Mean fractional anisotropy was significantly lower for the idiopathic normal pressure hydrocephalus group than for the control group in the forceps minor and motor cortex; the idiopathic normal pressure hydrocephalus group had significantly higher mean axial diffusivity for the genu of the corpus callosum and forceps minor. We did not find significant correlation between diffusion tensor imaging parameters and cerebrospinal fluid flowmetry and mini mental state examination, while we observed a correlation between forceps minor fractional anisotropy and frontal assessment battery; no correlation between flowmetry and clinical scores was found. Conclusion Our findings suggest that diffusion tensor imaging provides a non-invasive biomarker of white matter changes in idiopathic normal pressure hydrocephalus patients. Forceps minor is the best site to analyse. As diffusion tensor imaging offers a better correlation to clinical status than cerebrospinal fluid flowmetry, it should be included in the routine idiopathic normal pressure hydrocephalus protocol.
Bickerstaff brainstem encephalitis (BBE) is a rare neurological disease that generally has a good prognosis. We describe an atypical case of a patient with severe BBE; the presentation was uncommon because of the lack of ophthalmoplegia and because of evidence of both peripheral neuropathy and brainstem encephalitis. The article reports clinical and biochemical evaluation and focuses on magnetic resonance imaging (MRI) findings in diagnosis and management of the patient. Notably, we found a previously unreported dramatic spinal cord involvement on MRI. We believe these findings could add to diagnostic tools, and that this case may represent a new variant of BBE with more aggressive behavior.
The primary aim of the study was to evaluate the long-term effectiveness of adalimumab (ADA) in a cohort of non-radiographic axial spondyloarthritis (nr-axSpA), and the secondary aims were to identify predictive factors of response and evaluate radiological progression.We evaluated 37 patients (male/female: 12/25; mean age 49 ± 14; mean disease duration: 6.3 ± 5.8) with active nr-axSpA (Assessment of SpondyloArthritis International Society criteria), despite the treatment with ≥1 nonsteroidal anti-inflammatory drug for at least 3 months, initiating the treatment with ADA 40 mg every other week. Patients were treated for 24 months, and evaluated at baseline, 6, 12, and 24 months. Outcome measures included Ankylosing Spondylitis Disease Activity Score, Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), and Bath Ankylosing Spondylitis Functional Index. Radiograph of the spine and sacroiliac joints and magnetic resonance of the sacroiliac joints were performed at baseline and according to the standard of assessment for the disease.The proportion of patients that achieved a BASDAI50 response at 6, 12 and 24 months was 51.3%, 70.3%, and 76.8%, respectively. Treatment was well tolerated with no unexpected adverse events and/or serious adverse events. All patients remained on treatment for 2 years, with a good compliance. We did not identify any predictive factor of response to therapy. Moreover, modified Stoke Ankylosing Spondylitis Spine Score and Spondyloarthritis Research Consortium of Canada scores showed a trend of improvement during the study period.ADA was effective on clinical and radiological outcomes at 2-year follow-up; thus, early treatment with ADA may prevent radiographic damage and be associated with low disease activity or remission. Moreover, data from this cohort study have confirmed safety and tolerability profile of ADA in nr-axSpA in the long term.
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