in molte forme di parkinsonismo degenerativo (degenerazione cortico-basale e demenza a corpi di Lewy) può verificarsi un precoce interessamento corticale. Usage on Degenerazione cortico-basale. Usage on dia. org. Frontotemporalna lobarna degeneracija · Pickova bolest. SNA) Altre sinucleinopatie: M. Hallervorden Spatz, Atrofia Pallidonigroluysiana Taupatie Paralisi Supranucleare Progressiva Degenerazione Cortico Basale.
Hain, MD Most recent update: Also see the index page. Corticobasal ganglionic degeneration, also known as Corticobasal degeneration or corticobasal syndrome CBS. We will call it CBGD. CBGD 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 However they often share the same genetics, so they are probably just the same disease, seen through the eyes of different neurology subspecialties.
The parkinsonism of CBGD is generally an asymetric akinetic rigid syndrome, unresponsive to levodopa, similar to that of multiple system atrophy and PSP. Given the genetic similarities between CBGD and PSP, it seems probable that they are simply two “faces” of the same disease, perhaps mixed in with other obscure neurological conditions that can by chance, cause a similar constellation of findings.
The internet has enabled easier case finding. Neuroradiological imaging studies in CBGD 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: Arm levitation resembling alien limb phenomena has been reported in PSP Barclay et al,which certainly can also show focal limb dystonia and bradykinesia.
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, The author of this page encountered a patient with “alien limb”, with a rapidly progressive ataxia. She went from being ambulatory to wheelchair bound in just 3 weeks.
The diagnosis on autopsy was not CBGD, but rather prion disease e. The rapid progression here, in retrospect, meant that this patient did not have CBGD, as these types of disorders do not move this rapidly. Other patients that the author of this page has seen carrying the diagnosis of CBD have not had autopsies, and thus their diagnoses remain unestablished. 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, 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”. CGBD is presumably similar to PSP in its oculomotor behavior, as the same genetic makeup can result in both disorders Tuite et al,but very little has been published.
Corticobasal degeneration – Wikidata
Given the rarity of this disorder as well as the fuzziness of the diagnosis, solid conclusions would seem to us to be premature.
Pierrot-Deseilligny and Rivaud-Pechoux suggested that there were “infraclinical oculomotor anomalies” whatever that meansand particularly long latency saccades. We have encountered several CBGD phenotype patients in our practice not confirmed by autopsy of courseand obtained a full rotatory chair test on one.
The patient presented below had a classic presentation with aphasia, alien hand, ataxia, and falls, but has the same oculomotor findings as PSP. And perhaps the most characteristic of PSP type patients, there was a disorder of fast phases where the eye “hung up” in the orbit. On rotatory chair testing, this is seen by a discrepency between step responses and sinusoidal testing, as the eye gets “hung up” on the “DC” type step responses, but does better on the high frequency rotatory chair.
Fast phases are a type of automatic rapid saccadic eye movement. Video of poor vertical suppression in patient with CGBD 7 meg, wmv file. This “hang up” behavior, meaning poor quick phases, can be seen at the bedside, but as in PSP, it is far worse for vertical.
The movie above shows cancellation at the bedside — a light is turned on within the Micromedical video goggles, and the patient was asked to watch the light. The head was then rotated vertically first, and then horizontally.
It is very obvious that there are no fast phases for vertical, but reasonably good fast phases for horizontal.
This is the typical situation for PSP patients as well. At the bedside, one can usually see this even without goggles. We find it easiest to use a “queen square” reflex hammer, with the “pessary” end on the forehead, and have the patient watch the top. This provides a target that moves with the head.
The disparity between horizontal and vertical is the key. In the individual trace below, one can see the “hang up” problem.
This is horizontal rather than vertical. There is still gain at the “turnaround point, but then the eye goes “flat line” as it runs into the orbit. Similar “hang ups” can be seen in other supranuclear eye movement disorders, including SCA7. At the bedside, this can be seen by having the patient attempt to suppress their VOR, elicited by sinusoidal movement of the head at about 1 hz, while looking at a fixation target within the goggle.
Horizontally there are fast phases.
Vertically, the eye goes up and down with the goggles. Other areas than oculomotor testing bassle cognitive testing. CBD patients may have problems with executive function, aphasia, apraxia, behavioral change, and visual spatial dysfunction, with relatively degsnerazione episodic memory. Degsnerazione and CT scans may be initially normal, but eventually asymmetrical cortical atrophy is seen in about half of the patients, with more atrophy in the frontoparietal area. As more cortical signs develop in later stages, the disorders below may be possible to separate, but one might argue that this is just “splitting” up patients according to their pattern of progression.
As diagnostic sensitivity is poor, neuropathological confirmation remains the gold standard. Even here, one wonders if this disorder can be defined with enough precision to be useful. While CBGD patients have normal saccadic velocity, this may be an artifact of case definition.
If PSP and CBGD share the same pathologic mechanism see above and belowthey may simply be two different presentations of the same disease. The cause of CBGD 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, Tau is a microtubule-binding protein that is normally abundant in neurons.
Other “tauopathies” include Alzheimer’s diseasePicks disease, frontotemporal dementia and parkinsonism, ALS-parkinson dementia complex of Guam, and progressive supranuclear palsy PSP Higgins et al, According to Cho et al”patients with CBS exhibited asymmetrically increased 18 F-AV binding in the bawale, globus pallidus, and thalamus contralateral to the clinically more affected side and in the ipsilateral globus pallidus and dentate nucleus.
The median survival has been reported as between 5. The most common cause of death is complications of immobility or dysphagia such as pneumonia or sepsis. They get worse over years. If a patient does get worse over weeks, think of something else perhaps CJD disease. CBGD patients, like PSP patients, do not respond to levodopa treatment the standard treatment for Parkinsonismand there are no specific neuroprotective treatments or medications that provide significant symptomatic benefit.
Although there are clinicians that offer riluzole as treatment for these types of disorders, we are frankly dubious that they offer any benefit than hope, because this is a “cows out of the barn” type situation.
We think that reports suggesting that riluzole improves this disease or similar diseases are likely due to small samples sizes or wishful thinking. Management is based on appropriate use of appliances, prevention of medical complications, and appropriate use of nursing.
Patients with CBGD 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. Not every patient wants to die on a ventilator. It is best to make it clear early on basalw the wishes are of the person with CBGD with respect to various types of invasive treatment, and make sure that they are carried out, in spite of the usual mission of our medical system to sustain life at any cost.
Testing — especially eye movement CGBD is presumably similar to PSP in its oculomotor behavior, as the same genetic makeup can result in cortkco disorders Tuite et al,degenerzaione very little has been published.
This patient had no ability to converge. Video of poor vertical suppression in patient with Degenerazinoe 7 meg, wmv file This “hang up” behavior, meaning poor quick phases, can be seen at degenerazionf bedside, but as in PSP, it is far worse for vertical. Other types of testing in CBD. Depression is common and treatment for this is appropriate.
Walking devices or wheelchairs may be needed to mitigate imbalance. Arm levitation in progressive supranuclear palsy. Di Maria et al. Corticobasal degeneration shares a common genetic background with progressive supranuclear palsy. Ballooned neurons in select neurodegenerative disease contain phosphorylated neurofilament epitopes.
Language disturbances in bzsale degeneration. A lack of the RW tau mutation in progressive supranuclear palsy and corticobasal degeneration. Corticobasal degeneration and progressive supranuclear palsy share a common tau haplotype. Issues in the early diagnosis of Parkinson’s disease. Litvan I, and others. Accuracy of the clinical diagnosis of corticobasal degeneration: Last saved on March 15,
Privacy & Cookies Policy
Necessary cookies are absolutely essential for the website to function properly. This category only includes cookies that ensures basic functionalities and security features of the website. These cookies do not store any personal information.