Journal of neuroimaging : official journal of the American Society of Neuroimaging
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Review Case Reports
Hirayama disease: Nosological classification and neuroimaging clues for diagnosis.
Hirayama disease (HD) is a rare, benign, and nonprogressive motor neuron disease (MND) affecting the upper limbs. It usually presents with weakness and amyotrophy in a single upper extremity with an insidious onset between adolescence and the third decade of life. Since its description in 1959, HD has been known under several names and eponyms in Europe and in Asian countries probably due to its heterogeneous clinical features. ⋯ Also, we report a case of a 18-year-old boy who presented to our Clinic complaining of muscle weakness of the left distal upper limb without other neurological signs. The cervical MRI, in the neutral position, revealed a high T2 signal intensity in the C5-C7 cervical myelomeres as well as the loss of cervical lordosis, whereas, during neck flexion, it showed the anterior displacement of the posterior dura ad the post-gadolinium T1-weighted imaging enhancement of the posterior epidural plexus. These findings are typical for HD allowing the diagnosis as well as the differential diagnosis from other neuromuscular diseases.
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The tauopathies are a heterogeneous group of neurodegenerative disorders in which the prevailing underlying disease process is intracellular deposition of abnormal misfolded tau protein. Diseases often categorized as tauopathies include progressive supranuclear palsy, chronic traumatic encephalopathy, corticobasal degeneration, and frontotemporal lobar degeneration. Tauopathies can be classified through clinical assessment, imaging findings, histologic validation, or molecular biomarkers tied to the underlying disease mechanism. ⋯ The imaging characteristics will be outlined with select examples of emerging imaging techniques. Finally, current treatment options and emerging therapies will be discussed. This is by no means a comprehensive review of the literature but is instead intended for the practicing radiologist as an overview of a rapidly evolving topic.
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Recent studies indicate disrupted functional mechanisms of salience network (SN) regions-right anterior insula, left anterior insula, and anterior cingulate cortex-in mild cognitive impairment (MCI). However, the underlying anatomical and molecular mechanisms in these regions are not clearly understood yet. It is also unknown whether integration of multimodal-anatomical and molecular-markers could predict cognitive impairment better in MCI. ⋯ These findings shed light on the underlying anatomical volumetric and molecular amyloid alterations in SN regions and show the significance of multimodal markers integration approach in better predicting cognitive impairment in MCI.
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In patients with dolichoectasia, it is uncertain how dilatation and/or elongation relate to each other. We aimed to examine the correlation between arterial diameter and length within arteries and across the circle of Willis (COW). ⋯ COW configuration should be considered when using arterial diameter cutoffs to define dolichoectasia. Further studies are needed to discern whether arterial diameter or length best identify individuals at risk of vascular events attributable to dolichoectasia.
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To evaluate the accuracy of transcranial duplex sonography (TCS) for measuring the diameter of the third ventricle (DTV) and the brain midline shift (MLS), as compared to cerebral CT. ⋯ TCS seems to be a reliable and accurate bedside technique for measuring both DTV and MLS, which might allow detection of acute hydrocephalus among NICU patients.