We report the results of pathologic and biochemical studies in a patient with 6q-linked autosomal recessive juvenile parkinsonism (AR-JP). Neuronal loss and gliosis were restricted to the substantia nigra and the locus ceruleus. No Lewy bodies were found, but neurofibrillary tangles and argyrophilic astrocytes were seen in the cerebral cortex and brainstem nuclei. The later findings, which have not been reported previously in AR-JP, suggest the pathologic heterogeneity of 6q-linked AR-JP.
In the embryonic period, several developmental anomalies of the cerebral arteries occur. The knowledge of these anatomic variations of the cerebral artery is important to avoid the unnecessary surgery and to undergo surgery or interventional radiology with safety. We reviewed 3000 MR angiographies and 700 cerebral angiographies of the previous 5 years to assess cerebral arterial system, and to illustrate the embryological development, imaging findings, occurrence, and clinical significance of the anatomic variation of the cerebral arteries. The normal development and variations of the cerebral arteries are depicted. Knowledge of the anatomic variations is important since it can influence surgical and interventional procedure.
Brachial plexus injury (BPI) is a severe neurologic injury that causes functional impairment of the affected upper limb. Imaging studies play an essential role in differentiating between preganglionic and postganglionic injuries, a distinction that is crucial for optimal treatment planning. Findings at standard myelography, computed tomographic (CT) myelography, and conventional magnetic resonance (MR) imaging help determine the location and severity of injuries. MR imaging sometimes demonstrates signal intensity changes in the spinal cord, and enhancement of nerve roots and paraspinal muscles at MR imaging indicates the presence of root avulsion injuries. New techniques including MR myelography, diffusion-weighted neurography, and Bezier surface reformation can also be useful in the evaluation and management of BPI. MR myelography with state-of-the-art technology yields remarkably high-quality images, although it cannot replace CT myelography entirely. Diffusion-weighted neurography is a cutting-edge technique for visualizing postganglionic nerve roots. Bezier surface reformation allows the depiction of entire intradural nerve roots on a single image. CT myelography appears to be the preferred initial imaging modality, with standard myelography and contrast material-enhanced MR imaging being recommended as additional studies. Work-up will vary depending on the equipment used, the management policy of peripheral nerve surgeons, and, most important, the individual patient.
Fifty-nine acute flaccid myelitis cases were reported nationwide, coincident with enterovirus D68 (EV-D68) outbreak from August to December 2015. Strong temporal association was noted, and EV-D68 was detected from some cerebrospinal fluid and blood specimens. Antiganglioside antibodies were identified in some patients, and prognostic factors were analyzed.
Since its introduction in the mid-1990s, balloon-occluded retrograde transvenous obliteration (BRTO) has become widely accepted in Japan as a minimally invasive, highly effective treatment for gastric varices. Sufficient filling and stagnation of the sclerosing agent in the entire variceal complex is essential for successful BRTO of gastric varices. However, the success of BRTO in this context also requires familiarity with the hemodynamic features of the varices, including the patterns of their afferent and draining veins, which affect the degree of difficulty in performing BRTO. Thus, accurate assessment of the hemodynamic pattern before and during each procedure is essential for successful treatment. Sixty cases of gastric varices that were successfully treated with transcatheter techniques over the past 5 years were reviewed and analyzed. From this study, a classification system for gastric varices was developed that is based on the hemodynamic pattern of the varices.
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