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Bruch's membrane thickens with age, slowing the transport of metabolites. This may lead to the formation of drusen in age-related macular degeneration. [4] There is also a buildup of deposits (Basal Linear Deposits or BLinD and Basal Lamellar Deposits BLamD) on and within the membrane, primarily consisting of phospholipids.
Corticobasal degeneration (CBD) is a rare neurodegenerative disease involving the cerebral cortex and the basal ganglia. [1] CBD symptoms typically begin in people from 50 to 70 years of age, and typical survival before death is eight years.
Drusen, from the German word for node or geode (singular, "Druse"), are tiny yellow or white accumulations of extracellular material that build up between Bruch's membrane and the retinal pigment epithelium of the eye. The presence of a few small ("hard") drusen is normal with advancing age, and most people over 40 have some hard drusen. [1]
In fact, the majority of people over age 60 have drusen with no adverse effects. The risk of developing symptoms is higher when the drusen are large and numerous, and associated with the disturbance in the pigmented cell layer under the macula. Large and soft drusen are thought to be related to elevated cholesterol deposits. [citation needed]
The terms “basal lamina” and “basement membrane” were often used interchangeably, until it was realised that all three layers seen with the electron microscope constituted the single layer seen with the light microscope. This has led to considerable terminological confusion; if used, the term “basal lamina” should be confined to its ...
Symptoms may be symmetric or asymmetric, with one or more of the following: [citation needed] limb rigidity or akinesia; limb dystonia; limb myoclonus, plus one of: orobuccal or limb apraxia; cortical sensory deficit; alien limb phenomena (more than simple levitation)
Choroidal nevus with drusen can be considered as a sign of chronicity since drusen take years to develop and appear. [14] Drusen are composed of lipids and can actually be an indicator that a tumour is a benign nevus as opposed to a cancerous melanoma. [15] In nevi imaged by OCT, about 41% are found to have drusen. [16]
Primary familial brain calcification [1] (PFBC), also known as familial idiopathic basal ganglia calcification (FIBGC) and Fahr's disease, [1] is a rare, [2] genetically dominant or recessive, inherited neurological disorder characterized by abnormal deposits of calcium in areas of the brain that control movement.