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corticobasal degeneration
Corticobasal degeneration (CBD) or Corticobasal ganglionic degeneration (CBGD) is a rare, progressive neurodegenerative disease involving the cerebral cortex and the basal ganglia.〔(【引用サイトリンク】title=Corticobasal Degeneration Information Page: National Institute of Neurological Disorders and Stroke (NINDS) )〕 CBD symptoms typically begin in people from 50–70 years of age,and the average disease duration is six years. It is characterized by marked disorders in movement and cognitive dysfunction, and is classified as one of the Parkinson plus syndromes. Clinical diagnosis is difficult, as symptoms of CBD are often similar to those of other disorders, such as Parkinson's disease (PD), progressive supranuclear palsy (PSP), and Dementia with Lewy bodies (DLB).
Due to the various clinical presentations associated with CBD, a final diagnosis can only be made upon neuropathologic examination. Patients who are suffering from this disorder can learn more about CBD and reach out to others with the same condition for support, through The Foundation for PSP | CBD and Related Brain Diseases, also known as CurePSP.
==Background and History==
CBD was first identified by Rebeiz and his associates in 1968, as they observed three individuals who exhibited characteristic symptoms of the unique and previously unknown disorder. They initially referred to the neurodegenerative disease as “corticodentatonigral degeneration with neuronal achromasia,” after which various other names were used, including “corticonigral degeneration with nuclear achromasia” and “cortical basal ganglionic degeneration.” Although the underlying cause of CBD is unknown, the disease occurs as a result of damage to the basal ganglia, specifically marked by neuronal degeneration or depigmentation (loss of melanin in a neuron) in the substantia nigra. Additional distinguishing neurological features of those diagnosed with CBD consist of asymmetric atrophy of the frontal and parietal cortical regions of the brain.〔 Postmortem studies of patients diagnosed with CBD indicate that histological attributes often involve ballooning of neurons, gliosis, and tauopathy.〔 Much of the pioneering advancements and research performed on CBD has been completed within the past decade or so, due to the relatively recent formal recognition of the disease.
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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