<Superscript>68</Superscript>Ga-PSMA PET-CT Imaging of Metastatic Adenoid Cystic Carcinoma

A patient with a history of adenoid cystic carcinoma of the nasal cavity presented himself with bone pain and an elevated PSA level. On suspicion of metastatic prostate cancer a ⁶⁸Ga-PSMA PET-CT was performed. The PET-CT showed numerous lung and non-sclerotic bone metastasis. Biopsy of a bone metastasis was performed and pathology showed adenoid cystic carcinoma instead of prostate cancer. Immunohistochemical PSMA staining of the primary tumour showed intense PSMA expression in adenoid cystic carcinoma tumour cells. Because of the high PSMA expression of adenoid cystic carcinoma, ⁶⁸Ga-PSMA PET-CT might be a promising imaging modality for this malignancy.     

Atypical Antipsychotic Adjustments and Side-Effects over Time in Adults with Intellectual Disability,Tardive Dyskinesia,and Akathisia

Atypical antipsychotic use is prevalent in ID, and can result in serious side effects, particularly long-term. The purpose of this paper was to examine side effects over time and adjustments in atypicals in ID, tardive dyskinesia (TD), and akathisia. Side effects of 84 ID adults were assessed across four administrations of the Matson Evaluation of Drug Side-Effects (MEDS). Persons with TD or akathisia who experienced changes in atypicals were more likely to evince side effects. These trends were evident across total MEDS scores and subscales for cardiovascular and hematology, CNS for parkinsonism/dyskinesia and behavioral/akathisia. Atypical antipsychotic use poses a risk for side effects in individuals with ID, particularly in relation to medication adjustments over time and the presence of neuroleptic-induced movement disorders.     

Missense and splice site mutations in SPG4 suggest loss-of-function in dominant spastic paraplegia

We studied nine Italian families with a pure form of autosomal dominant spastic paraplegia (ADHSP) to assess the frequency of mutations in the SPG4 gene. We observed marked intrafamilial variability in both age-at-onset and clinical severity, ranging from severe congenital presentation to mild involvement after age 55 years to healthy carriers of the mutation after age 70. Four of nine probands harboured SPG4 mutations, We identified three new SPG4 mutations, all predicting a loss-of-function with apparently important consequences for spastin function. RT-PCR studies predict loss-of-function as a possible mechanism leading to spastin-related HSP. The current study expands the spectrum of allelic variants in SPG4, confirming their pathological significance in pure AD-HSP and suggesting implications for the presumed function of spastin. Received: 15 December 2000, Received in revised form: 29 May 2001, Accepted: 18 June 2001     

Whole brain apparent diffusion coefficient histogram: a new tool for evaluation of leukoaraiosis

PURPOSE: To test whole brain apparent diffusion coefficient histogram analysis as an alternative approach to visual score for the assessment of leukoaraiosis (LA). MATERIALS and METHODS: T2 and diffusion weighted images were obtained in 15 elderly patients. LA extension was assessed on T2 weighted images by two observers using a semiquantitative visual score. Apparent diffusion coefficient (ADC) maps of the entire brain were generated and, after exclusion of the skull with manual tracing and of the cerebrospinal fluid (CSF) by application of a threshold value, whole brain (WB)-ADC histogram was obtained. Moreover, a brain volume index (BVI) was calculated on ADC maps as (intracranial volume - CSF volume) /intracranial volume. RESULTS: The kappa inter-observer agreement for LA scoring was 0.69. Manual segmentation of the skull showed a mean inter-operator coefficient of variation below 3%. The median value of whole brain ADC histogram directly correlated with LA extension (P = 0.013). Moreover a significant inverse correlation (P = 0.002) was found between WB-ADC median value and BVI. CONCLUSION: WB-ADC histogram is a reproducible alternative tool for assessing LA extension and severity.     

Diffusion weighted MR: principles and clinical use in selected brain diseases

PURPOSE: To define the principles and technical bases of diffusion weighted MR imaging of the brain and report our experience in the evaluation of selected brain disorders including age-related ischemic white matter changes (leukoaraiosis), neoplastic and infective cysts and wallerian degeneration. MATERIAL AND METHODS: Between May 1999 and June 2000 we examined seventeen patients: 10 patients with leukoaraiosis and deterioration of cognitive and motor function, 5 patients with focal cystic lesions (one anaplastic astrocytoma, one glioblastoma, one metastasis from squamous cell lung carcinoma, one pyogenic abscess and one case with cerebral tubercolosis) and 2 patients with wallerian degeneration (one with post-hemorrhagic degeneration of right corticospinal tract and one with post-traumatic degeneration of left optic tract). All patients underwent a standard cranial MR examination including SE T1-, proton density, T2-weighted, FLAIR and diffusion weighted images. Post-contrast T1-weighted sequences were also obtained in the patients with cystic lesions. Diffusion weighted images were acquired with double shot echoplanar sequences. Diffusion sensitizing gradient along the x, y and z axes and b values ranging 800 to 1200 s/mm2 were used. For each slice a set of three orthogonal diffusion "anisotropic" images, an "isotropic" image and a standard T2-weighted image were reconstructed. Postprocessing included generation of the apparent diffusion coefficient maps and of the "trace" image that reflects pixel by pixel the diffusional properties of water particles only. Values of mean diffusivity within regions of interest were computed in the "trace" image and compared with those obtained in contralateral brain areas. In patients with leukoaraiosis the diffusivity in posterior periventricular white matter was compared with that measured in 10 age-matched control subjects without leukoaraiosis. RESULTS: In patients with leukoaraiosis the areas of increased periventricular signal intensity on T2-weighted images showed a significantly higher (p < 0.001) diffusivity (mean values 124.7 +/- 21.3 x 10(-5) mm2/s) as compared to control subjects (mean values 85 +/- 7 x 10(-5) mm2/s). Diffusion weighted images in 2 patients revealed the presence of a small focal area of increased signal and reduced diffusivity in "trace" images consistent with recent ischemic lesion. In neoplastic cystic lesions the central necrotic/cystic content was always hypointense on diffusion weighted images and showed increased diffusivity on "trace" images. On the other hand the central necrotic content of the pyogenic brain abscess was hyperintense and showed low diffusivity. In patients with wallerian degeneration diffusion weighted images and "trace" images demonstrated loss of anisotropy and increased diffusivity in the affected white matter tract relative to the contralateral. DISCUSSION: The increased diffusivity observed in areas of leukoaraiosis and the identification of subclinical acute ischemic lesions by diffusion weighted images might be more useful than standard MR sequences for monitoring the disease progression. Diffusion weighted images allow differentiation of the different parts of focal cystic lesions (edema, solid and cystic/necrotic portion) and are useful to differentiate pyogenic brain abscess from necrotic tumors. In patients with wallerian degeneration the loss of anisotropy and the increase of diffusivity values in the affected tract are probably related to myelin breakdown and allow better recognition of the affected tract relative to standard MR images. CONCLUSIONS: Diffusion weighted MR imaging can be performed during a standard cranial MR examination and add useful clinical information in several brain disorders besides acute ischemic stroke.