Elisa Scola

MRI abnormalities found 1 year prior to symptom onset in a case of Creutzfeldt-Jakob disease.

Creutzfeldt–Jakob disease (CJD) is a rare disease causing rapidly progressive dementia, with an annual incidence of 1/1,000,000/year. It is caused by conformational change and accumulation of the prion protein in the brain, resulting in spongiform degeneration and producing the typical MRI appearance of increased DWI signal in cerebral cortex and/ or basal ganglia [1, 2]. We describe a patient in whom cortical DWI hyperintensity was evident 1 year prior to symptom onset. A 65-year-old woman with a history of hypertension and thyroid disease underwent a neurological evaluation for chronic tension-type headache in June 2013. Neurological examination, including mental state evaluation, was normal. However, brain MRI showed DWI hyperintensity of the right basal temporo-occipital cortex, with decreased ADC signal (Fig. 1a, b). A diagnosis of cortical stroke was made and antiplatelet therapy was recommended; other conditions that could be suggested by the MRI findings, namely encephalitis, vasculitis, sarcoidosis, posterior reversible encephalopathy syndrome, seizures, and hypoxic-ischemic encephalopathy were excluded as inconsistent with history and clinical findings [3]. After 12 months in good health, in June 2014 the patient was diagnosed with invasive ductal breast cancer (stage IIA, pT1N1M0), which was surgically removed in July. Almost concomitantly with the diagnosis of cancer, she developed rapidly progressive mental deterioration, consisting of forgetfulness, apathy, and difficulties with orientation and in performing daily activities; removal of the neoplasm did not influence progression of cognitive decline. She was therefore admitted to our department in September 2014. Neurological examination showed markedly reduced motor and verbal initiative, severe ideomotor apraxia, visual agnosia with simultanagnosia, and impairment of episodic memory, attention, and abstract thinking. The remainder of the examination was normal. Neuropsychological evaluation demonstrated severe multidomain deterioration. Routine blood tests were unremarkable. Brain MRI showed extension of the previously documented DWI abnormalities, involving the basal temporo-occipital and parietal cortex of both hemispheres (Fig. 1c, d). The absence of contrast enhancement ruled out neoplastic infiltration of the brain or meninges. Repeated EEG recordings displayed worsening delta-wave slowing on the right hemisphere. CSF analysis demonstrated normal protein and cells without intrathecal IgG synthesis, and no autoantibodies (antiHu, -Ri, -Ma1, -Ma2, -CV2, -amphiphysin, -NMDAR, VGKC, -thyroglobulin, or -thyroid peroxidase) were detected in CSF or serum, arguing against inflammatory, infectious, or paraneoplastic encephalitis. Conversely, CSF tau protein was markedly elevated (4216 pg/mL, normal range \400), and 14-3-3 protein was detected. Real-time quaking-induced conversion (RT-QuIC) assay detected the & Federico Verde frdc.verde@gmail.com

MR Imaging in Sudden Sensorineural Hearing Loss. Time to Talk

SUMMARY: Sudden sensorineural hearing loss is defined as acute hearing loss of the sensorineural type of at least 30 dB over 3 contiguous frequencies that occurs within a 72-hour period. Although many different causative factors have been proposed, sudden sensorineural hearing loss is still considered “idiopathic” in 71%–85% of cases, and treatments are empiric, not based on etiology. MR imaging implemented with a 3D FLAIR sequence has provided new insights into the etiology of sudden sensorineural hearing loss. Herein, we review the current management trends for patients with sudden sensorineural hearing loss, from the initial clinical diagnosis to therapeutic strategies and diagnostic work-up. We focused primarily on MR imaging assessment and discuss the relevance that MR imaging findings might have for patient management, pointing out different perspectives for future clinical research.

Paroxysmal Nocturnal Hemoglobinuria (Pnh): Brain Mri Ischemic Lesions In Neurologically Asymtomatic Patients

This study investigated for the first time brain ischemic involvement in 19 consecutive neurologically asymptomatic PNH patients by non-enhanced cerebral MRI, and by intracranial arterial and venous angio-MRI. Eleven cases (58%, 7 aged <65) showed pathological findings: 9 white matter (WM) abnormalities related to chronic ischemic small vessel disease, 2 a focal abnormality >5 mm, and 5 cases a score >4 by the age-related white matter changes (ARWMC) scale. Compared with age and sex-matched controls (1:2 ratio), patients showed an increased frequency of periventricular WM vascular degeneration (32% versus 5.2%, p = 0.04) and of severe lesions (ARWMC scale score >4) (26% versus 2.6%, p = 0.05), and a higher overall ARWMC scale score (3.5 ± 1.07 versus 2.0 ± 0.8, mean ± SD, p < 0.0001). Notably, vascular abnormalities suspected for prior partial venous thrombosis, were observed in PNH cases only. MRI lesions were not related to blood counts, hemolytic markers, clone size, disease duration, and therapy with eculizumab. Neurological examination was unremarkable in all patients but one (Parkinson disease). Psychiatric assessment revealed a case of generalized anxiety disorder, 1 bipolar disorder type 2, and 1 adjustment disorder. In conclusion, brain MRI may be useful at diagnosis and during the course of the disease to explore subclinical neurological involvement.

Word and Picture Version of the Free and Cued Selective Reminding Test (FCSRT): Is There Any Difference?

The Free and Cued Selective Reminding Test (FCSRT) is the most commonly used neuropsychological test to evaluate episodic memory. Two variants of FCSRT exist, using the recall of words (FCSRT-w) or pictures (FCSRT-p). Fourteen patients with mild cognitive impairment underwent neuropsychological evaluation and brain magnetic resonance. We found differences in FCSRT-w and FCSRT-p variants scores. FCSRT-p was correlated with atrophy in areas involved in visual stimuli processing while FCSRT-w was correlated to hippocampal atrophy. Our study suggests that FCSRT-w and FCSRT-p scores are not equivalent, but a larger cohort of patients is needed to validate these results.

Flat Panel Angiography in the Cross-Sectional Imaging of the Temporal Bone: Assessment of Image Quality and Radiation Dose Compared with a 64-Section Multisection CT Scanner

BACKGROUND AND PURPOSE: Cross-sectional imaging of the temporal bone is challenging because of the complexity and small dimensions of the anatomic structures. We evaluated the role of flat panel angiography in the cross-sectional imaging of the temporal bone by comparing its image quality and radiation dose with a 64-section multisection CT scanner. MATERIALS AND METHODS: We retrospectively collected 29 multisection CT and 29 flat panel angiography images of normal whole-head temporal bones. Image quality was assessed by 2 neuroradiologists, who rated the visualization of 30 anatomic structures with a 3-point ordinal scale. The radiation dose was assessed with an anthropomorphic phantom. RESULTS: Flat panel angiography showed better image quality than multisection CT in depicting the anterior and posterior crura of the stapes, the footplate of the stapes, the stapedius muscle, and the anterior ligament of the malleus (P < .05). In contrast, multisection CT showed better image quality than flat panel angiography in assessing the tympanic membrane, the bone marrow of the malleus and incus, the tendon of the tensor tympani, the interscalar septum, and the modiolus of the cochlea (P < .05). Flat panel angiography had a significantly higher overall image quality rating than multisection CT (P = .035). A reduction of the effective dose of approximately 40% was demonstrated for flat panel angiography compared with multisection CT. CONCLUSIONS: Flat panel angiography shows strengths and weaknesses compared with multisection CT. It is more susceptible to artifacts, but due to the higher spatial resolution, it shows equal or higher image quality in assessing some bony structures of diagnostic interest. The lower radiation dose is an additional advantage of flat panel angiography.

Is cutis verticis gyrata-intellectual disability syndrome an underdiagnosed condition? A case report and review of 62 cases

Cutis Verticis Gyrata‐Intellectual Disability (CVG‐ID) syndrome is a rare neurocutaneous syndrome characterized by intellectual disability and scalp folds and furrows that are typically absent at birth and are first noticed after puberty. First reported in 1893, the syndrome was mainly identified in subjects living in psychiatric institutions, where it was found to have a prevalence of up to 11.4%. Most patients were reported in the literature during the first half of the 20th century. CVG‐ID is now a less reported and possibly under‐recognized syndrome. Here, we report a patient with CVG‐ID that was diagnosed using the novel approach of magnetic resonance imaging and we conduct a systematic review of all patients reported in the last 60 years, discussing the core clinical features of this syndrome.

Auditory functional magnetic resonance in awake (nonsedated) and propofol-sedated children.

Functional Magnetic Resonance Imaging (fMRI) is often used in preoperative assessment before epilepsy surgery, tumor or cavernous malformation resection, or cochlear implantation. As it requires complete immobility, sedation is needed for uncooperative patients.

Optic Radiation Diffusion Tensor Imaging Tractography: An Alternative and Simple Technique for the Accurate Detection of Meyer's Loop

BACKGROUND The optic radiation (OR) is a white matter bundle with a very complex anatomy. Its anterior component bends sharply around the tip of the temporal horn, forming the Meyers loop (ML), the sparing of which during surgery is crucial to preserve visual function. Defining its exact anatomy and accurately identifying its position remain challenging, even with diffusion tensor imaging (DTI) tractography and the most refined tracking procedure. We have developed an alternative tracking technique to detect the ML position. METHODS We performed DTI studies in 26 patients undergoing resection of a temporo-parieto-occipital lesion. We then reconstructed the ORs of each patient using 2 techniques (the first developed by our team, the other taken from the literature), using the same tracking software and parameters. We evaluated the accuracy of each technique measuring 3 distances that define the ML position. We created 5 data groups and compared the 2 techniques. Finally, we compared our results with the results from 8 anatomic dissection studies and other tractographic studies. RESULTS Our findings show that our technique allows a more accurate definition of the ML position. We found a statistically significant (P < 0.05) difference for all the distances between the 2 techniques; our results resemble those obtained in dissection studies. Our technique is also easy to perform and repeatable. CONCLUSIONS Our tracking technique may be of marked interest for the evaluation and anatomic definition of the ML position, particularly for neurosurgeons approaching the anterior temporal region.

Flat-panel CT versus 128-slice CT in temporal bone imaging: Assessment of image quality and radiation dose

OBJECTIVE We compared the image quality and radiation dose of flat-panel CT (FPCT) and multi-slice CT (MSCT) performed respectively with an angiographic unit and a 128-slice CT scanner. We investigated whether the higher spatial resolution of FPCT translated into higher image quality and we sought to eliminate inter-subject variability by scanning temporal bone specimens with both techniques. MATERIALS AND METHODS Fifteen temporal bone specimens were imaged with FPCT and MSCT. Two neuroradiologists experienced in otoradiology evaluated 30 anatomical structures with a 0-2 score; 18 structures important from a clinical perspective were assigned a twofold value in calculation of the overall score. The radiation dose was calculated through the use of an anthropomorphic phantom. RESULTS The image quality was significantly higher for FPCT than MSCT for 10 of the 30 anatomical structures; the overall score was also significantly higher for FPCT (p = 0.001). The equivalent dose of the two techniques was very similar, but with different effective doses to the organs. CONCLUSION FPCT performed on an angiographic unit provides higher image quality in temporal bone assessment compared to MSCT performed on a 128-slice CT scanner thanks to its higher spatial resolution, with comparable equivalent doses but different effective doses to the organs.

Three-Tesla Magnetic Resonance Imaging of the vestibular endolymphatic space: a systematic qualitative description in healthy ears

BACKGROUND AND PURPOSE A detailed knowledge of the normal Magnetic Resonance (MR) anatomy of the vestibular endolymphatic space (ES) could be useful to understand the linkage between endolymphatic hydrops (EH) and Ménières disease (MD). Our aim was to describe the MR anatomy of the vestibular ES as depicted by MR imaging in healthy ears. METHODS This report describes a single-center retrospective study. Three readers analyzed the healthy ears of 22 consecutive patients who had undergone MRI for unilateral sudden hearing loss. The readers described the vestibular ES based on a delayed post-contrast 3D-FLAIR sequence according to six well-defined planes, three oblique sagittal (lateral, intermediate and medial) planes and three axial (superior, intermediate and inferior) planes. RESULTS On sagittal lateral and intermediate planes, we identified the SSC ampulla combined with the utricle in 22/22 ears. On the sagittal medial plane, the saccule was detectable in 15/22 (68%) ears, having a club shape with the long axis oriented cranio-caudally; in 7/22 (32%) ears, the saccule presented an oval/round shape that appeared more conspicuously on the axial intermediate plane. The ES occupied the half superior portion of the vestibule in 22/22 ears, never contacting the round and oval windows. On the axial plane, in 17/22 cases, the ES showed a Y-shaped arrangement, while in 5/22 ears (23%), the ES presented a more globular shape. CONCLUSION MR imaging represents a valid tool to explore the in vivo anatomy of the vestibular ES and to highlight its variability in normal ears.