Corticobasal degeneration

Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.

Corticobasal degeneration (CBD) is a rare progressive neurodegenerative disease characterized by marked disorders in movement and cognitive dysfunction. Clinical diagnosis is difficult, as symptoms of CBD are often similar to those of other diseases, such as Parkinson’s disease (PD) and progressive supranuclear palsy (PSP). Furthermore, a definitive diagnosis of CBD is only possible after death, as the only absolute determinant of the disease requires the application of neuropathology and histopathology.

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.”[1] 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.[2] Additional distinguishing neurological features of those diagnosed with CBD consist of asymmetric atrophy of the frontal and parietal cortical regions of the brain.[1] Postmortem studies of patients diagnosed with CBD indicate that histological attributes often involve ballooning of neurons, gliosis, and tauopathy.[2] Much of the pioneering advancements and research performed on CBD has been completed within the past decade or so, due to the relevantly recent formal discovery of the disease.

Clinical presentation of CBD usually does not occur until age 60, with the earliest recorded diagnosis and subsequent postmortem verification being age 28.[3] Although men and women present with the disease, some analysis has shown a predominant appearance of CBD in women. Current calculations suggest that the prevalence of CBD is approximately 4.9 to 7.3 per 100,000 people. The prognosis for an individual diagnosed with CBD is death within approximately eight years.[4]

Because CBD is progressive (it gradually worsens), a set of standard diagnostic criteria can be used, which is centered on the disease’s evolution. Included in these fundamental features are problems with cortical processing, dysfunction of the basal ganglia, and a sudden and detrimental onset.[1] Psychiatric and cognitive dysfunctions, although present in CBD, are much less prevalent and lack establishment as common indicators of the presence of the disease.[4]

Some of the most prevalent symptom types in people exhibiting CBD pertain to identifiable movement disorders and problems with cortical processing. These symptoms are initial indicators of the presence of the disease. Each of the associated movement complications typically is seen in an asymmetric manner, in which the symptom is not observed uniformly throughout the body. For example, a person exhibiting an alien hand syndrome (explained later) in one hand, will not correspondingly display the same symptom in the contralateral limb. Predominant movement disorders and cortical dysfunctions associated with CBD include:

[tubepress mode='tag', tagValue='Corticobasal degeneration']