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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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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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder affecting upper and lower motor neurons. Some ALS patients exhibit concomitant nonmotor signs, and thus ALS is considered a multisystem disorder. The aim of this study is to investigate autonomous nervous system involvement in ALS. ⋯ This study confirms the presence of autonomic dysfunction in ALS patients and provides evidence of VN atrophy that correlates with disease severity and duration and is independent of bulbar involvement. Degeneration of dorsal nucleus neurons of the VN is hypothesized.
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This study aims to investigate the feasibility of a "real-time" estimate of the optimal CT perfusion (CTP) acquisition time (Top ) in ischemic stroke patients. ⋯ The linear correlation between Top and the VOF time to peak is well suited to implement a new technique to automatically customize the patient's CTP acquisition time. The method does not require an additional dose of contrast medium and does not increase the overall study time, so its use would be desirable to decrease the average radiation dose.
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Magnetic resonance elastography to study the effect of amyloid plaque accumulation in a mouse model.
Biomechanical changes in the brain have not been fully elucidated in Alzheimer's disease (AD). We aimed to investigate the effect of β-amyloid accumulation on mouse brain viscoelasticity. ⋯ Aging had region-specific effects on cerebral viscoelasticity, namely, WB softening and hippocampal stiffening. Amyloid plaque deposition may have sex-specific effects, which require further scrutiny.