Putaminal hypointensity on T2*-weighted MR imaging is the most practically useful sign in diagnosing multiple system atrophy: A preliminary study.

Objective: To identify useful MRI abnormalities in the putamen for diagnosing multiple system atrophy.

Methods: Patients with multiple system atrophy (n=15), Parkinson's disease (n=16), or progressive supranuclear palsy (n=9) and healthy controls (n=10) were enrolled. Using a visual analog scale, 4 examiners independently rated high-intensity signals along the lateral putamen on T2-weighted and T2*-weighted images, low-intensity signals within the putamen on T2-weighted and T2*-weighted images, and putaminal atrophy.

Effects of a polymorphism in the GFAP promoter on the age of onset and ambulatory disability in late-onset Alexander disease.

Alexander disease (AxD) is a rare neurodegenerative disorder. Most patients with AxD have a de novo dominant missense mutation in the glial fibrillary acidic protein (GFAP) gene. Patients with late-onset AxD exhibit a more variable onset and severity than patients with early-onset AxD, suggesting the existence of factors that modify the clinical phenotype of late-onset AxD.

"Bright spotty lesions" on spinal magnetic resonance imaging differentiate neuromyelitis optica from multiple sclerosis.

Background: Spinal magnetic resonance imaging (MRI) finding of longitudinally extensive spinal cord lesions (LESCL) extending over three vertebral segments and involvements of spinal central gray matter have been reported in patients with neuromyelitis optica (NMO).

Objectives: We aimed to review spinal MRI findings in NMO and multiple sclerosis (MS), and to determine whether the "bright spotty lesions" (BSLs) are a discriminative finding of NMO.

Methods: For this study, 24 consecutive patients with NMO and 34 patients with MS were enrolled.

A case of adult-onset alexander disease featuring severe atrophy of the medulla oblongata and upper cervical cord on magnetic resonance imaging.

Adult-onset Alexander disease (AOAD) has been increasingly recognized since the identification of the glial fibrillary acidic protein gene mutation in 2001. We report on a 56-year-old man who was genetically confirmed as AOAD with the glial fibrillary acidic protein mutation of p.M74T.

Diffuse and heterogeneous T2-hyperintense lesions in the splenium are characteristic of neuromyelitis optica.

Background: Callosal lesions in multiple sclerosis (MS) are usually focal, involving the inferior aspect of the corpus callosum on brain magnetic resonance imaging (MRI), but little is known about callosal lesions in neuromyelitis optica (NMO).

Objective: To clarify MRI abnormalities in callosal lesions of NMO.

Methods: Japanese patients with NMO (n=28) or MS (n=22) were assessed.

Truncal polyradiculopathy due to sarcoidosis.

We report the case of a 43-year-old woman who developed multiple cranial nerve palsy, the symptoms of which included hyposmia, visual loss, facial hypoesthesia, facial weakness, dysphagia, gustatory disturbance, and sensory disturbance of the trunk and ulnar side of the bilateral arms. The clinical features included swelling of the bilateral hilar lymph nodes, uveitis, an elevated serum angiotensin-converting enzyme level, and negative tuberculin reactions, which led to a diagnosis of neurosarcoidosis. Her symptoms improved after administration of steroids.