World Neurosurg
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Acrodysostosis is a rare syndrome characterized by peripheral dysostosis, nasal hypoplasia, and frequently mental retardation. Only two adult cases of acrodysostosis have been reported to have neurological symptoms. ⋯ Special attention should be given to these patients to detect signs of spinal stenosis, as early decompression can lead to neurological recovery.
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The Rho pathway has been shown to have a role in the pathophysiology of spinal cord injury (SCI). Upregulation of the Rho signaling pathway occurs as a result of SCI. Activation of Rho and its downstream effector kinases triggers growth cone collapse and represents a significant barrier to axon regeneration. Furthermore, there is evidence that Rho-ROCK signaling mediates the inhibitory effects of chondroitin sulfate proteoglycans on neurons, and that inhibition of Rho and ROCK can reverse chondroitin sulfate proteoglycan-mediated inhibition of neurite outgrowth. Work building on these findings suggests that inhibition of this pathway may boost neuroprotection and axonal regeneration after SCI. ⋯ The Rho pathway has been shown to have a role in the pathophysiology of SCI and preclinical and clinical work and is currently a promising target for the treatment of patients with SCI.
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To present a case of a cavernous malformation presenting with a subdural hematoma. ⋯ The clinical course, radiologic, and intraoperative findings suggest that the subdural hemorrhage was caused by extralesional hemorrhage of the cavernous malformation.
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Lipomatosis of nerve (LN) is a condition of massive peripheral nerve enlargement due to proliferation of fibrous and adipose tissue within the nerve, the natural history of which is currently unknown. We measured the pattern of growth in individuals with long-term radiologic follow-up. ⋯ Serial MRI reveals progressive enlargement of LN. The rate of growth was more profound in youth, but also occurred in early adulthood.
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Neurenteric cysts (NECs) are uncommonly encountered lesions of the central nervous system with heterogeneous imaging characteristics. The object of this study was to review the preoperative imaging findings represented among a cohort of surgically treated posterior fossa NECs. These findings are considered in the context of surgical technique, and inform an understanding of aberrant neuroembryological development associated with NECs. ⋯ Posterior fossa NECs display variable patterns of MRI signal and are commonly considered as part of a broad differential of cystic posterior fossa masses. We identified tumor insinuation between the ventral brainstem and vertebrobasilar system as a highly sensitive and specific radiographic sign for NECs. This finding was not observed among a large cohort of posterior fossa masses representative of other multiple pathologies.