I have nothing to sell you but hope, and that I give you for free.

[I am in the process of condensing the information in the following articles to one comprehensible document]

It is important to keep in mind that "corticobasal syndrome" (CBS) is a set symptoms.  The physical or medical cause of the symptoms can not be deterimined without a brain autopsy.  (Perhaps new neuroimaging techniques or tests of the cerebral spinal fluid or blood tests will some day be able to make diagnosing the cause without a brain autopsy or biopsy possible.)  Apparently, between 25 and 40% of clinically diagnosed CBS is due to Alzheimer's disease (AD) pathology.  Therefore, it is reasonable to try treatments known or suspected to help AD victims. 

NINDS Corticobasal Degeneration Information Page

What is Corticobasal Degeneration?
Corticobasal degeneration is a progressive neurological disorder characterized by nerve cell loss and atrophy (shrinkage) of multiple areas of the brain including the cerebral cortex and the basal ganglia. Corticobasal degeneration progresses gradually. Initial symptoms, which typically begin at or around age 60, may first appear on one side of the body (unilateral), but eventually affect both sides as the disease progresses. Symptoms are similar to those found in Parkinson disease, such as poor coordination, akinesia (an absence of movements), rigidity (a resistance to imposed movement), disequilibrium (impaired balance); and limb dystonia (abnormal muscle postures). Other symptoms such as cognitive and visual-spatial impairments, apraxia (loss of the ability to make familiar, purposeful movements), hesitant and halting speech, myoclonus (muscular jerks), and dysphagia (difficulty swallowing) may also occur. An individual with corticobasal degeneration eventually becomes unable to walk.

Is there any treatment?

There is no treatment available to slow the course of corticobasal degeneration [there are new ideas that haven't been tried yet -ed.], and the symptoms of the disease are generally resistant to therapy. Drugs used to treat Parkinson disease-type symptoms do not produce any significant or sustained improvement. Clonazepam may help the myoclonus. Occupational, physical, and speech therapy can help in managing disability.

What is the prognosis?

Corticobasal degeneration usually progresses slowly over the course of 6 to 8 years. Death is generally caused by pneumonia or other complications of severe debility such as sepsis or pulmonary embolism.

What research is being done?

The NINDS supports and conducts research studies on degenerative disorders such as corticobasal degeneration. The goals of these studies are to increase scientific understanding of these disorders and to find ways to prevent, treat, and cure them.

NIH Patient Recruitment for Corticobasal Degeneration Clinical Trials
At NIH Clinical Center
Throughout the U.S. and Worldwide

Organizations

National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT   06813-1968
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291

 WE MOVE (Worldwide Education & Awareness for Movement Disorders)
204 West 84th Street
New York, NY   10024
wemove@wemove.org
http://www.wemove.org
Tel: 212-875-8312
Fax: 212-875-8389

 
CUREPSP (Foundation for PSP|CBD and Related Brain Diseases)
Executive Plaza III
11350 McCormick Road, Ste. 906
Hunt Valley, MD   21031
info@curepsp.org
http://www.curepsp.org
Tel: 410-785-7004 800-457-4777
Fax: 410-785-7009
 
http://www.ninds.nih.gov/disorders/corticobasal_degeneration/corticobasal_degeneration.htm


Corticobasal degeneration, sometimes referred to as corticobasal ganglionic degeneration (CBGD), is a heterogeneous disease which clinically, genetically and pathologically is similar to, or overlaps with frontotemporal dementia (FTD). For this reason, CBD is considered to be part of the ‘Pick complex’ of neurodegenerative diseases (see FTD description).

CBD was first described in 1968 by Rebeiz and colleagues, who immediately recognized its potential relationship to FTD based on macroscopic and microscopic analyses of CBD brains. Historically, CBD patients have been diagnosed on the basis of movement problems which sometimes appear similar to Parkinson’’s disease (PD). Unlike PD, however, CBD patients typically do not respond significantly to PD medicines, such as levodopa/carbidopa (Sinemet). Also, many symptoms of CBD are not found in PD patients. For this reason CBD is often referred to as a ‘Parkinson’s-plus’ syndrome.

When a diagnosis of CBD is suspected, it is important to refer the patient to a neurologist who is experienced with this disorder. This is because the constellation of symptoms and problems experienced by affected individuals and their caregivers is unique. There have been significant advances in the understanding of CBD over the past 10 years, and as a result, improved counseling, support and symptomatic treatments are now available. We are actively involved in research to better understand the pathophysiology of CBD.

Demographics
CBD typically occurs in patients between 45 and 70. In our experience, women are affected more commonly than men. Rarely, there is a family history of dementia, psychiatric problems or a movement disorder.

Symptoms
Patients with CBD present with either a movement disorder or cognitive deficits. As the disease progresses, most patients will eventually develop both types of symptoms, often with a delay of 2-3 years.

Movement
A characteristic feature of movement symptoms in CBD is striking asymmetry of involvement. Most frequently symptoms begin insidiously in one hand or arm, less commonly in one leg. Rarely, symptoms may involve the mouth and facial muscles.

Many patients will complain initially of a subtle change in sensation or an inability to make the affected limb follow commands. This latter deficit is called apraxia and may be confused for clumsiness or weakness. There may be difficulties in completing specific tasks, such as opening a door or brushing one’s teeth or using tools, such as a can opener. When a leg is affected initially, a patient may have problems with complex movements such as dancing; or when more severe, a patient may begin to trip and fall. Some patients will experience an involuntary stiffening, twisting or contraction of the affected limb called dystonia. There may be uncontrolled jumping of the limb when it is tapped gently or when the patient is startled, called myoclonus.

Finally, CBD patients often complain that the affected limb feels like it is not a part of their body, a sensation called alien limb. Sometimes an alien limb will move on its own, in an uncontrollable way. For example, an alien hand will rise to touch the patient’s face. Alien limb phenomenon was dramatized by the actor Peter Sellers in the film Dr. Strangelove.

Movement symptoms tend to progress slowly from one side of the body to the other or from leg to arm on the same side of the body.

Cognition
Patients with CBD who present with cognitive difficulties are usually initially diagnosed with frontotemporal dementia or Alzheimer's disease. It is only after they develop movement symptoms that the diagnosis of CBD is entertained. Occasionally, a diagnosis of CBD is not apparent until a patient’s brain is examined at autopsy.

Progressive difficulty with language is a common cognitive complaint in CBD. This most commonly involves difficulty with expression of language, such as word finding difficulty or naming problems. Reading, writing and simple mathematical calculations may also be impaired.

Personality changes, inappropriate behavior, repetitive and/or compulsive activities similar to those seen in FTD (see FTD description) are also common in CBD. Short-term memory problems, such as repeating questions or misplacing objects are also common.

Many patients with the movement difficulties of CBD will also have mild cognitive problems when they are evaluated in a specialized dementia clinic.

Treatment
At this time, there is no specific treatment for CBD. Instead individual symptoms are targeted with specific medications. For example, rigidity and difficulty walking may partially respond to treatments for Parkinson’s disease. Dystonia and myoclonus may respond to muscle relaxants or anti-seizure medications. Memory and behavior problems may respond to treatments for Alzheimer's disease and/or depression.
http://memory.ucsf.edu/Education/Disease/cbd.html

Cortico-basal ganglionic degeneration (CBGD) is a complex neurobehavioural disorder characterised by insidious onset and gradually progressive cerebrocortical and extrapyramidal dysfunction.
A 52 years old male presented in May 1998 with behavioural abnormalities since November 1996 and abnormal movements since June 1997. Initially the patient had difficulty in dressing himself. Soon afterwards he had difficulty in finding his way while inside the house. Sometimes he spent the whole night outside the house, to be discovered next morning by his family members in the nearby field. In April 1997 he also developed speech abnormality. His speech gradually became incomprehensible and he would laugh or cry without any reason. He also became forgetful. He developed twisting movements of right sided fingers, wrist and forearm and neck and lower limbs. He also developed parkinsonian features and was unable to perform activities of daily living on his own. There was no history of myoclonus, seizures, motor weakness, sensory or bladder and bowel symptoms.
On examination he was restless, laughing or crying without reasons. He could follow some verbal commands but could not do so when written commands were provided. He could neither utter a comprehensible word nor write a legible letter. His cranial nerves were normal. Examination of motor system was normal except the presence of akinesia, rigidity and dystonic posturing of limbs. These were more profound on the right side. There was no tremor or ataxia. His deep tendon reflexes as well as the plantars were normal. There was gross postural instability. The rigidity and akinesia did not respond to levodopa.
Investigations revealed normal haemogram, blood glucose, renal function and liver function tests. Serum calcium, phosphorous and alkaline phosphatase were 9.6 mg/dl,3.5 mg/dl and 11KAU/L, respectively. The serum copper was 90 mgm/dl and ceruloplasmin was 30 mg/dl. Serum VDRL was nonreactive and ELISA for HIV 1 and 2 were negative. CSF examination revealed a protein content of 20 mg/dl and sugar of 68 mg/dl and was acellular. The T2 weighted images on MRI [Figure. 1] showed prominent sylvian cisterns and prominence of cortical sulci particularly in frontal and both parietal lobes, suggesting symmetrical atrophy of frontal and parietal lobes. There was no KF ring on eye examination.
The typical features of CBGD can be categorised into movement disorders (akinesia, rigidity, postural instability, limb dystonia, cortical myoclonus and postural/intention tremor) and cortical signs, such as cortical sensory loss, apraxias and the 'alien limb' phenomenon.[1],[2] The most striking features of CBGD is asymmetry of involvement which differentiates it from most other neurodegenerative disorders. Rinne et al[2] reviewed 36 patients, with mean age at onset of 60.9+9.7 years. (range : 40-76 years). In the patients reported by Riley et al, the mean age at onset was 60 years (range: 51-71 years) and men were more commonly affected than women. Riley et al[1] reported apraxia in 71% of cases of CBGD. Although they found ideational and ideomotor apraxias to occur early and were sometimes the presenting symptoms, a variety of other apraxias have also been reported in CBGD. Alien limb phenomenon is an unusual sign in neurology and its presence, in the absence of a known callosal lesion, is highly suggestive of the diagnosis of CBGD. Speech abnormalities and aphasia has been reported to occur in 21% of patients with CBGD and are considered to reflect left hemisphere cortical pathology in this disorder.[1] Involvement of right parietal cortex in CBGD gives rise to visuospatial and constructional disturbances. Personality change, impaired attention, acalculia, impaired recall and learning, concrete thinking and left-right confusion have been noted in a number of patients. Oculomotility disturbances particularly manifested by impaired convergence and vertical and horizontal gaze palsy has been noted in CBGD. This feature sometime confuse CBGD with progressive supranuclear palsy (PSP). Dementia is a late feature of CBGD and was found in 43% of patients by Riley et al.[1] The full spectrum of clinical features typically seen in CBGD can also be present in patients with Pick's disease, but the latter disorder is usually dominated by cognitive, behavioural, and language disturbances such as primary progressive aphasia. Moreover, apraxia and parkinsonism, if present, are usually late finding in Pick's disease. Pathological features in CBGD include neuronal degeneration in pre-and post-central cortical areas, degeneration of basal ganglia, including substantia nigra (SN), and presence of achromatic neural inclusion seen not only in the cortex but also in the thalamus, subthalamus nucleus, red nucleus, and SN.[4] CT scans were abnormal in 14 of the 15 patients in one series, 8 had asymmetrical parietal lobe atrophy corresponding to the most affected side, and 6 had bilateral parietal atrophy.[1] PET scans shows reduced 18(F) fluorodopa uptake in the caudate and putamen, and markedly asymmetrical cortical hypometabolism in the superior temporal and inferior parietal lobe.[5]
Our patient presented with dressing and situational apraxias as the initial symptoms. Gradually, he developed abnormalities of speech, emotional lability, loss of social inhibition etc., the features of diffuse cortical dysfunction. These features were combined with asymmetric involvement of right side with extrapyramidal features like limb dystonia, akinesia, rigidity and postural instability. Our patients did not have 'alien limb' phenomenon which is a very interesting features of CBGD. However, its absence does not exclude the diagnosis, as it was observed only in 50% of patients by Riley et al.[1] The presence of features of cortical dysfunction in the early phase of illness compounded by extrapyramidal features and the absence of oculomotility disturbances and ataxia, clearly suggest the diagnosis of CBGD in our patients. These clinical findings were corroborated by symmetrical atrophy of frontal and parietal lobes in neuroimaging studies.

1. Riley DE, Lang AE, Lewis A et al : Cortico-basal ganglionic degeneration. Neurology 1990; 40 : 1203-1212.       
2. Rinne JO, Lee MS, Thompson PD et al : Cortico-basal degeneration: a clinical study of 36 cases. Brain 1994; 117 : 1183-1196.       
3. Bogen JE : Split-brain syndrome. In: Handbook of clinical neurology. Vol. 1(45): Clinical Neuropsychology. Friederiks JAM, Ed. Amsterdam: Elselvier. 1985; 99-106.       
4. Lippa CF, Cohen R, Smith TW et al : Primary progressive aphasia with focal neuronal achromasia. Neurology1991; 42 : 882-886.       
5. Eidelberg D, Dhawan V, Moller JR et al : The metabolic landscape of cortico-basal ganglionic degeneration, regional asymmetries studies with positron emission tomography. J Neurol Neurosurg Psychiatry 1991; 54 : 856-862.       
http://www.neurologyindia.com/article.asp?issn=0028-3886;year=2000;volume=48;issue=4;spage=405;epage=6;aulast=Anand

Corticobasal ganglionic degeneration (which we will call CBD) is a rare progressive neurological disorder characterized by a combination of Parkinsonism and cortical dysfunction. It is a rare sporadic progressive disorder first reported in 1968. CBD appears to be closely related to another, less rare, sporadic extrapyramidal degenerative disorder named Progresive Supranuclear Palsy (PSP) . In CBD, cognitive symptoms dominate, while in PSP, eye movement symptoms dominate the picture.

The Parkinsonism is generally an asymetric akinetic rigid syndrome, unresponsive to levodopa, similar to that of multiple system atrophy and PSP. Eye movement abnormalities are common, as in PSP, and a supranuclear gaze palsy can be seen as in PSP. Given the genetic similarities between CBD and PSP, it seems possible that they are simply two "faces" of the same disease.

Neuroradiological imaging studies in CBD demonstrate cortical atrophy, which may be symmetrical or asymmetrical. Other cortical signs include

Alien limb phenomenon
Apraxia
Dysphasia
Cortical sensory loss
Pyramidal signs
Proposed diagnostic criteria include at least three of the following:

bradykinesia and rigidity that does not respond to levodopa
alien limb phenomena
cortical sensory signs
focal limb dystonia
action tremor
myoclonus
The "alien limb" symptom is highly specific but it is not necessary for the diagnosis. Arm levitation resembling alien limb phenomena has been reported in PSP (Barclay et al, 1999), which certainly can also show focal limb dystonia and bradykinesia. Other aspects of this picture could easily be mistaken for other neurodegenerative disease such as Alzheimer's or Picks disease, and in fact, even experienced clinicians are correct 50% of the time or less when judged by pathological criteria. Onset in the sixth or seventh decade is typical. Disease progression is quicker than in Parkinsonism but similar to that of PSP. Recently language disturbance has been documented to be frequent (Frattali et al, 2000).

Pathology.
There is neuronal loss and gliosis and swollen achromatic neurons (ballooned neurons) are found in all cortical layers, but especially so in superior frontal and parietal gyri. There is extensive loss of myelinated axons in the white matter. Scattered neuronal inclusions may be seen similar to Pick bodies. Ballooned neurons are strongly reactive for phosphorylated neurofilaments and may include the tau protein (see below)(Dickson et al, 1986). Neuronal loss and gliosis are also observed in the nuclei of the basal ganglia. Lewy bodys and neurofibrillary tangles are absent. The substantia nigra shows neuronal loss with extraneuronal melanin, gliosis and neurofibrillary inclusions, called "corticobasal bodies".

Differential Diagnosis:

CBD is difficult to diagnose in early stages, and experienced examiners typically diagnose it correctly less than 50% or the time (Litvan et al, 1997). CBD and may also be impossible to differentiate from PSP or a striato-niagral type of MSA. As more cortical signs develop in later stages, the disorders below may be possible to separate. As diagnostic sensitivity is poor, neuropathological confirmation remains the gold standard. Even here, one wonders if this disorder can be defined.

Parkinsonism
PSP (progressive supranuclear palsy, related by tau)
MSA (multiple system atrophy)
Picks disease
While CBD patients have normal saccadic velocity, this may be an artifact of case definition. If PSP and CBD share the same pathologic mechanism (see below), they may simply be two different presentations of the same disease.

The cause of CBD is presently unknown but because the tau protein accumulates in this disorder, it may be related to a mutation in the tau gene. (Higgins et al, 1999). Tau is a microtubule-binding protein that is normally abundant in neurons. Other "tauopathies" include Alzheimer's disease, Picks disease, frontotemporal dementia and parkinsonism, ALS-parkinson dementia complex of Guam, and progressive supranuclear palsy (PSP) (Higgins et al, 1999). According to Di Maria et al (2000) and Houlden et al (2001), CBD shares the same tau haplotype as do PSP patients (see above), suggesting that both CBD and PSP share the same genetic background, and possibly the same pathoogic mechanism.

Conventional Treatment
CBD patients do not respond to levodopa treatment (the standard treatment for Parkinsonism). Management is based on appropriate use of appliances, prevention of medical complications, and appropriate use of nursing. Patients with CBD and caregivers should establish early on the plan regarding invasive care -- intubation, feeding tubes, as these issues are almost certain to come up in the course of the disease.

References:
Barclay CL, Bergeron C, Lang AE. Arm levitation in progressive supranuclear palsy. Neurology 1999:52:879-882
Di Maria et al. Corticobasal degeneration shares a common genetic background with progressive supranuclear palsy. Ann Neurol 2000:47:374-377
Dickson DW and others. Ballooned neurons in select neurodegenerative disease contain phosphorylated neurofilament epitopes. Acta Neuropathol 71:216-223, 1986)
Frattali CM and others. Language disturbances in corticobasal degeneration. Neurology 2000:54:990-992
Higgins JJ, Litvan I, Nee LE, Loveless BS. A lack of the R406W tau mutation in progressive supranuclear palsy and corticobasal degeneration. Neurology 1999:52:404-406
Houlden H and others. Corticobasal degeneration and progressive supranuclear palsy share a common tau haplotype. Neurology 2001:56:1702-6.
Koller WC, Montgomery EB. Issues in the early diagnosis of Parkinson's disease. Neurology 1997:49 (Suppl 1), S10-25.
Litvan I, and others. Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study. Neurology 1997:48:119-125
Riley DE, Lange AE, Lewis A, et al. Cortico-basal ganglionic degeneration. Neurology 1990;40:1203-1212
****
http://www.dizziness-and-balance.com/disorders/central/movement/corticobasal.html

Corticobasal Degeneration Overview
What is corticobasal degeneration?
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
What causes the degeneration of brain tissue in CBD?
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
What are the symptoms of CBD?
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), 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
How is CBD diagnosed?
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
How is CBD treated?
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 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.

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.

What is the usual course of CBD?
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.

http://www.wemove.org/cbd/cbd.html

Cortical-basal ganglionic degeneration (CBGD), or corticobasal degeneration typically begins from 50 – 70 years of age. Mean survival is about 8 years. Its distinctive features are an asymmetric levodopa-resistant akinetic-rigid syndrome associated with "cortical" features such as apraxia, cortical sensory loss, and alien limb phenomenon. General cognitive function had been thought to be preserved.

This "classical" description emphasizing a parietal/perceptual-motor presentation may be biased because the cases mainly originate from movement disorder centers. Features of speech disturbances or dementia had been thought to represent the minority of cases. In a recent review by Grimes et al. only 4 of 13 pathologically proven patients had a prior clinical diagnosis of CBGD. It appears now that dementia can be a prominent feature of advanced disease and may be the most common feature. Aphasia can be seen in over 50% of patients. Depression is common. Apathy, social withdrawal, bizarre behavior, hypersexuality irritability, and anarthria have been described.

Parkinsonian signs including unilateral limb rigidity (79%), bradykinesia (71%), postural instability (45%) and apraxia are found in almost all patients. Dystonic posturing of the arm and hand is common (43%). Tremor when present is typically an action tremor that improves at rest. It frequently has a myoclonic (jerky) component. Stimulus sensitive myoclonus can be seen. The rigidity may be extreme and associated pain is common.

With progression cortical sensory deficits, pyramidal tract dysfunction, dysarthria, dysphagia and other symptoms emerge or worsen. Alien limb phenomena develop in 50% of cases. It may be as simple as levitation of a limb. Magnetic apraxia (approach behavior with groping and manipulation) is a sign of CBGD. Described eye movement abnormalities include saccadic pursuit, difficulty initiating saccades, and rarely supranuclear palsy.

CBGD, like PSP is a disorder of the tau protein (a tauopathy). It seems now that it has significant overlap with the frontotemporal dementias (FTD), parkinsonism associated with chromosome 17 (FTDP-17), primary progressive aphasia (PPA), Pick's disease, and PSP.

General References On Clinical Features of Parkinsonisms

*Handbook of Clinical Neurology Vol 49 Extrapyramidal disorders: Vinken, Bruyn, Klawans eds.   Elsevier Science publishers 1996

Movement Disorders a Comprehensive Survey;: Weiner, Lang A. eds. Futura publishing company 1989

Neurodegenerative Diseases, Calne, D., eds. W.B. Saunders Company 1994

Parkinson's Disease and Movement Disorders, Jankovic, Tolosa ,eds. Urban & Scharzenberg 1988

References for criteria in the diagnosis of Parkinsonism

CBGD

D. A. Grimes, A. E. Lang MD, FRCPC, C. B. Bergeron Dementia as the most common presentation of cortical-basal ganglionic degeneration Neurology Volume 53  Number 9  December 10, 1999
http://www.cmdg.org/Movement_/Parkinsons_Plus/CBGD/cbgd.htm



CBS stands for "corticobasal syndrome." It's a clinical diagnosis given while someone is alive. CBD stands for "corticobasal degeneration." Many clinicians (including those at Mayo Rochester) are trying to only use the term CBD for a pathological diagnosis made on a post-mortem basis. Upon brain autopsy, someone with a clinical diagnosis of CBS can actually show evidence of something other than CBD. The diagnostic accuracy for CBD is rather poor.

Mayo Rochester researchers looked at the clinical records of 11 patients diagnosed during life with CBS but upon brain autopsy 5 had Alzheimer's Disease-- that's almost 50% folks!-- (called CBS-AD in this paper) and 6 had CBD (called CBS-CBD in this paper). Part of the clinical records includes MRI scans taken while the patients were alive. A technique called voxel-based morphometry was used to compare patterns of gray matter atrophy.

The researchers found that "On direct comparisons between the two subject groups, CBS-AD showed greater loss in both temporal and inferior parietal cortices than CBS-CBD. ... In subjects presenting with CBS, prominent temporoparietal, especially posterior temporal and inferior parietal, atrophy may be a clue to the presence of underlying AD pathology."

Because this is a technique used with standard MRI scans, this approach could be utilized by all clinicians with access to a top radiologist.

In light of this, it seems reasonable to pursue treatments aimed at Alzheimer's disease since someone diagnosed with CBS may actually be suffering from AD.

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Updated: July 30, 2010
Inception: November 4, 2009