Leukoencephalopathy with calcifications and cysts (LCC) is an uncommon condition of unknown etiology occurring in children and adults. Pathological findings include obliterative hyalinosis of the ...small vessels, myelin loss, intense gliosis, Rosenthal fiber formation, microcalcifications, and hemosiderin deposits. Herein we report a 55-year-old man with LCC documented 10 years ago, in whom we examined brain perfusion by pseudocontinuous arterial spin labeling technique. We demonstrated diffused hypoperfusion of the affected white matter (WM) and of the subcortical gray matter (GM) and cortical GM in the patient in comparison to a group of healthy control subjects, using both qualitative evaluation and region of interest analysis. WM and subcortical GM hypoperfusion reflects the known distribution of LCC microangiopathy. We speculate that cortical hypoperfusion may be related to cerebral atrophy or may reflect deafferentation secondary to severe leukoencephalopathy, and may possibly contribute to severe motor and cognitive impairment. Further studies addressing cerebral blood flow in LCC are necessary.
Unexplained focal neurologic episodes (FNEs) can occur in patients with transthyretin-related familial amyloidotic polyneuropathy (TTR-FAP) after orthotopic liver transplantation (OLT). A patient ...with Val30Met FAP underwent OLT at age 34 years. Twelve years after transplantation, she presented with recurrent FNEs lasting from 10 minutes to 8 hours each, with nonuniform deficitary clinical features and variably associated with headache. Magnetic resonance imaging showed multiple brain microbleeds and diffuse contrast enhancement of the craniospinal leptomeninges consistent with amyloid deposits. Our observation suggests that microbleeds associated with meningovascular amyloidosis can underlie FNEs in TTR-FAP. Moreover, it confirms that OLT does not halt progression of leptomeningeal and vascular amyloid deposition due to TTR production in the choroid plexuses. Such a progression might compromise the good long-term prognosis of patients with TTR-FAP due to increased risk of intracranial hemorrhages. Pharmacologic therapies targeting brain TTR production may modify this scenario.
The opened or closed status of the glottis might influence tracheal size changes in inspiratory and expiratory computed tomography (CT) scans. We investigated if the glottis status makes the tracheal ...collapse differently correlate with lung volume difference between inspiratory and expiratory CT scans.
Forty patients with chronic obstructive pulmonary disease whose glottis was included in the acquired scanned volume for lung CT were divided into two groups: 16 patients with the glottis closed in both inspiratory and expiratory CT, and 24 patients with the glottis open in at least one CT acquisition. Lung inspiratory (Vinsp) and expiratory (Vexp) volumes were automatically computed and lung ΔV was calculated using the following formula: (Vinsp - Vexp)/Vinsp × 100. Two radiologists manually measured the anteroposterior diameter and cross-sectional area of the trachea 1 cm above the aortic arch and 1 cm above the carina. Tracheal collapse was then calculated and correlated with lung ΔV.
In the 40 patients, the correlations between tracheal Δanteroposterior diameter and Δcross-sectional area at each level and lung ΔV ranged between 0.68 and 0.74 (ρ) at Spearman rank correlation test. However, in the closed glottis group, the correlations were higher for all measures at the two levels (ρ range: 0.84-0.90), whereas in the open glottis group, correlations were low and not statistically significant (ρ range: 0.29-0.34) at the upper level, and moderate at the lower level (ρ range: 0.51-0.55).
A closed or open glottis influences the tracheal size change in inspiratory and expiratory CT scans. With closed glottis, the tracheal collapse shows a stronger correlation with the lung volume difference between inspiratory and expiratory CT scans.
To define a statistically based variation of individual whole-lung densitometry above which a real increase of pulmonary extent can be suspected in lung cancer screening trials.
Baseline and 3-month ...follow-up low-dose computed tomography (LDCT) examinations of 131 smokers or former smokers recruited in the ITALUNG (32 subjects) and MILD (99 subjects) trials were compared using for each data set two different image processing tools for whole-lung densitometry. Both trials were approved by institutional review boards, and written informed consent was obtained from all participants. Assuming that no change of emphysema extent can occur in a 3-month interval, the Bland and Altman method was used to assess the agreement between baseline and follow-up LDCT examinations for lung volume, 15th percentile (Perc15) of lung density and Perc15 corrected for lung volume by application of a linear detrend on log-transformed data.
Similar results were obtained in each data set using two different image processing tools. In the ITALUNG cohort the 95% limits of agreement (LoA) interval of volume corrected Perc15 was -9.7 to 10.7% using image processing method 1 and -10.3 to 11.5% using image processing method 2. In the MILD cohort, the 95% LoA interval of volume corrected Perc15 was -14.7 to 17.3% with both image processing methods.
In the two considered lung cancer screening settings a range of 9.7-14.7% decrease of volume corrected Perc15 represents a statistically defined threshold to suspect a real increase of emphysema extent in serial LDCT examinations.
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