Corticobasal syndrome

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Corticobasal syndrome (CBS) is a rare, progressive atypical Parkinsonism syndrome and is a tauopathy related to frontotemporal dementia. [1] [2] CBS is typically caused by the deposit of tau proteins forming in different areas of the brain. [1] [3]

Contents

Classification

CBS is the most common type of corticobasal degeneration (CBD) although the terms CBD and CBS have been used interchangeably in the past. [2] The other three phenotypes of CBD are: [1]

Symptoms and signs

Symptoms of CBS include apraxia, alien limb phenomenon, frontal deficits, and extrapyramidal motor symptoms such as myoclonus or rigidity. [5] Movement deficits often begin on one side and progress to the other. [1]

Pathophysiology

CBD is the pathology underlying approximately 50% of CBS cases. [6]

Diagnosis

The Armstrong criteria were proposed in 2013; the accuracy of these is limited and further research is needed. [7] Symptoms may be symmetric or asymmetric, with one or more of the following:[ citation needed ]

  1. limb rigidity or akinesia
  2. limb dystonia
  3. limb myoclonus, plus one of:
  4. orobuccal or limb apraxia
  5. cortical sensory deficit
  6. alien limb phenomena (more than simple levitation)

The onset is insidious with gradual progression, lasting one year or more, with no exclusion criteria present. The diagnosis is more likely if onset is after age 50, there is no family history (2 or more relatives),[ clarification needed ] and there is no genetic mutation affecting T[ clarification needed ] (e.g., MAPT). [8]

Probably sporadic CBS is more likely if there are two of:

  1. limb rigidity or akinesia
  2. limb dystonia
  3. limb myoclonus
  1. orobuccal or limb apraxia,
  2. cortical sensory deficit
  3. alien limb phenomena (more than simple levitation) [8]

The diagnosis is excluded if there is evidence of:

The diagnostic criteria for clinical use may result in a misdiagnosis of other tau-based diseases. [7]

The probable criteria are proposed for clinical research. [7]

Differential

Other degenerative pathologies that can cause corticobasal syndrome include:

The symptoms of classic CBS differ from CBD in that CBD also includes cognitive deficits in the executive functions. [10]

Prognosis

The average survival time after disease onset is estimated at 6.5  years. [2]

Related Research Articles

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<span class="mw-page-title-main">Dementia with Lewy bodies</span> Type of progressive dementia

Dementia with Lewy bodies (DLB) is a type of dementia characterized by changes in sleep, behavior, cognition, movement, and regulation of automatic bodily functions. Memory loss is not always an early symptom. The disease worsens over time and is usually diagnosed when cognitive impairment interferes with normal daily functioning. Together with Parkinson's disease dementia, DLB is one of the two Lewy body dementias. It is a common form of dementia, but the prevalence is not known accurately and many diagnoses are missed. The disease was first described by Kenji Kosaka in 1976.

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<span class="mw-page-title-main">Corticobasal degeneration</span> Rare neurodegenerative disease

Corticobasal degeneration (CBD) is a rare neurodegenerative disease involving the cerebral cortex and the basal ganglia. CBD symptoms typically begin in people from 50 to 70 years of age, and the average disease duration is six years. It is characterized by marked disorders in movement and cognition, and is classified as one of the Parkinson plus syndromes. Diagnosis is difficult, as symptoms are often similar to those of other disorders, such as Parkinson's disease, progressive supranuclear palsy, and dementia with Lewy bodies, and a definitive diagnosis of CBD can only be made upon neuropathologic examination.

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References

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  10. Fredericks CA, Lee SE (2016). "The cognitive neurology of corticobasal degeneration and progressive supranuclear palsy". In Miller, Bruce L.; Boeve, Bradley F. (eds.). The Behavioral Neurology of Dementia (Second ed.). Cambridge, United Kingdom: Cambridge University Press. pp. 203–6. ISBN   9781107077201. OCLC   934020279. [CBD is] reminiscent of classic CBS but with executive function deficits