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Corticobasal degeneration (CBD) is a rare neurological disease in which parts of the brain deteriorate or degenerate. CBD is also known as corticobasal ganglionic degeneration, or CBGD.

Several regions of the brain degenerate in CBD. The cortex, or outer layer of the brain, is severely affected, especially the fronto-parietal regions, located near the center-top of the head. Other, deeper brain regions are also affected, including parts of the basal ganglia, hence the name "corticobasal" degeneration. The combined loss of brain tissue in all these areas causes the symptoms and findings seen in people with CBD.

Causes of Corticobasal Degeneration
Unfortunately, the cause of CBD is entirely unknown. There is currently no strong evidence to suggest CBD is an inherited disease, and no other risk factors, such as toxins or infections, have been identified.

Studies of brain tissue of individuals with CBD show certain characteristic cell changes. Similar, although not identical, changes are observed in two other neurodegenerative diseases, Pick's disease and progressive supranuclear palsy. These changes, involving a brain protein called tau, have provided researchers some initial clues in their search for the causes of CBD.

Symptoms of Corticobasal Degeneration
Symptoms of CBD usually begin after age 60. The initial symptoms of CBD are often stiffness, shakiness, jerkiness, slowness, and clumsiness, in either the upper or lower extremities. Other initial symptoms may include dysphasia (difficulty with speech generation), dysarthria (difficulty with articulation), and difficulty controlling the muscles of the face and mouth, or walking and balance difficulties. Symptoms usually begin on one side of the body, and spread gradually to the other. Some patients (probably more than commonly recognized in the past) may have memory or behavioral problems as the earliest or presenting symptoms.

CBD is a progressive disease, meaning the symptoms worsen over time. Over the course of one to several years, most people with CBD gradually worsen, with symptoms progressing to involve upper and lower extremities and other body regions.
Symptoms of advanced CBD include:
• parkinsonism (rigidity, slow movements, postural instability)
• tremor
• myoclonus (sudden, brief jerky movements)
• dystonia, including blepharospasm
• speech difficulty
• mild-to-moderate cognitive impairment (memory loss, difficulty planning or executing unrehearsed movements, dementia)
• sensory loss
• "alien hand/limb" phenomenon (difficulty controlling the movements of a limb, which seems to undertake movements on its own, sometimes combined with a feeling that the limb is not one's own)

Diagnosis of Corticobasal Degeneration
Early in the disease course, it is often difficult to distinguish CBD from similar neurodegenerative diseases. Diagnosis of CBD involves a careful neurological exam, combined with one or more types of laboratory evaluations. Electrophysiological studies, including an EEG (electroencephalogram), may show changes in brain function over time that are consistent with the neurodegeneration. CT or MRI scans can also be used in this way, providing images of asymmetric atrophy of the fronto-parietal regions of the brain's cortex, the regions most frequently involved in the disease.

Approaches to Treatment
Unfortunately, there are no drugs or other therapies that can slow the progress of the disease, and very few that offer symptomatic relief. Tremor and myoclonus may be controlled somewhat with some allopathic drugs such as clonazepam. Baclofen may help reduce rigidity somewhat. Levodopa and other dopaminergic drugs used in Parkinson's disease are rarely beneficial, but may help some CBD patients.

Homoeopathy
In Homoeopathy there are some Medicines which can help to reduce the severity of disease, i.e Argentum Met, Aur.S, Camph mbr., Causticum, Hyos., Merc Sol. Phos, Plumb., Zinc.cy.
I have little better experience with Argent.met., Causticum, Phosphorus & Plumbum in CBD patients.

Physical therapy exercises may be useful to maintain range of motion of stiff joints. This may prevent pain and contracture (muscle shortening), and help maintain mobility. Occupational therapy may be used to design adaptive equipment that supports the activities of daily living, thus helping to maintain more functional independence. Speech therapy is used to improve articulation and volume.

A person with CBD will usually become immobile due to rigidity within five years of symptom onset, and may require a gastrostomy tube for feeding at some point before that. Most often, within ten years of onset, pneumonia or other bacterial infections may lead to life-threatening complications.

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Excellent article, thanks for sharing.

We read so many things during our education, but unfortunately forget many topics. Thanks for reminding this topic.

Does corticobasal degeneration exist? A clinicopathological re-evaluation

It appears diagnosis may take some time, including misdiagnosis of other similar diseases.

The pathological findings of corticobasal degeneration are associated with several distinct clinical syndromes, and the corticobasal syndrome has been linked with a number of diverse pathologies. We have reviewed all the archival cases in the Queen Square Brain Bank for Neurological Disorders over a 20-year period with either a clinical diagnosis of corticobasal syndrome or pathological diagnosis of corticobasal degeneration in an attempt to identify the main diagnostic pitfalls.

Of 19 pathologically confirmed corticobasal degeneration cases, only five had been diagnosed correctly in life (sensitivity = 26.3%) and four of these had received an alternative earlier diagnosis. All five of these had a unilateral presentation, clumsy useless limb, limb apraxia and myoclonus, four had cortical sensory impairment and focal limb dystonia and three had an alien limb.

Eight cases of corticobasal degeneration had been clinically diagnosed as progressive supranuclear palsy, all of whom had vertical supranuclear palsy and seven had falls within the first 2 years. On the other hand, of 21 cases with a clinical diagnosis of corticobasal syndrome, only five had corticobasal degeneration pathology, giving a positive predictive value of 23.8%; six others had progressive supranuclear palsy pathology, five had Alzheimer’s disease and the remaining five had other non-tau pathologies.

Corticobasal degeneration can present very commonly with a clinical picture closely resembling classical progressive supranuclear palsy or Richardson’s syndrome, and we propose the term corticobasal degeneration-Richardson’s syndrome for this subgroup. Cases of corticobasal degeneration-Richardson’s syndrome have delayed onset of vertical supranuclear gaze palsy (>3 years after onset of first symptom) and the infrequent occurrence of predominant downgaze abnormalities, both of which can be helpful pointers to their underlying corticobasal degeneration pathology.

Fourty-two per cent of corticobasal degeneration cases presented clinically with a progressive supranuclear palsy phenotype and 29% of cases with corticobasal syndrome had underlying progressive supranuclear palsy pathology. In contrast, in the Queen Square Brain Bank archival collection, corticobasal syndrome is a rare clinical presentation of progressive supranuclear palsy occurring in only 6 of the 179 pathologically diagnosed progressive supranuclear palsy cases (3%). Despite these diagnostic difficulties we conclude that corticobasal degeneration is a discrete clinicopathological entity but with a broader clinical spectrum than was originally proposed. ~ jaonnolz

Corticobasal degeneration is primarily a Syphilitic miasmatic condition. In this type of degeneration, there is a unique point that its initiation is due to Sycosis which may be in latent form initially. Due to Turmoil in life, at genetic level, the sycosis becomes active and starts fruiting. But due to fundamentally syphilitic trend, the syphilis is aroused due to partial interaction of Psora and both the miasms- Psora and Sycosis are SUPPRESSED while Syphilis becomes predominently active and manifests its full course, often ending in termination of life....

The treatment required is based on antimiasmatic ground. Firstly, we have to SUPPRESS Syphilis so that the degenerations could be immediately stopped. Then the Psora who triggered the Syphilis is to be treated, so that the Exciting cause of Syphilis might be removed. Now the syphilis is spontaneously towards regression. In last, we should treat Sycosis to establish perfact cure. But Remember- The permanent irriversible changes occured in body may not be cured and some deformity may persist even after a cure.

Thank you, Dr Rajneesh, for this explanation on miasms. Thus, this type of disease requires stages of healing and considerable amount of time to see improvement. Dr Arun, have you seen many cases of this nature?

You are welcome Debby.

Debby,

your Intervention will help us to discuss CBD logically.yes I have not much cases but I am dealing two cases at a time one is 65 yrs old lady & another is 57yrs old.I am not sure about compleate cure but my both pt. is responding & runing well with Homoeopathy. 

All the best Dr. Arun.

You are welcome Dr. Rajneesh kumar sharma,M.D(Hom).

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