There was an interesting article published online last week by Italian neurologists about corticobasal degeneration (CBd). Here are the highlights:
* CBD has low diagnostic accuracy — 25–56% of cases.
* The only way to confirm a diagnosis is through brain donation. If it’s not CBD, what is it? Alzheimer’s disease (AD), progressive supranuclear palsy (PSP), Pick’s disease, dementia with Lewy bodies (DLB), Creutzfeldt-Jakob disease (CJD), etc.
Robin’s note: Of the many supposed CBD brain donation cases I’ve been involved with, we’ve seen all of these — AD, PSP, Pick’s, DLB, and CJD. Probably we’ve seen more AD than anything else.
* People with confirmed CBD can be grouped into four main “clinical types” —
- CBS (corticobasal syndrome)
- frontal behavioral-spatial syndrome
- nonfluent/agrammatic variant of primary progressive aphasia
- progressive supranuclear palsy syndrome
* There are few randomized clinical trials of pharmacological treatment of CBS symptoms. Physicians rely on expert opinion or anecdotal descriptions in prescribing medications.
* “Levodopa is…not particularly helpful in CBS.”
* “Amantadine has been occasionally reported to improve akinesia and rigidity in patients with AP, but evidence is anecdotal.” (AP = atypical parkinsonism)
* Botox “may be helpful for CBS-associated limb dystonia and may be used to alleviate abnormal posture, pain and for maintaining hand hygiene.”
* “Deficits in single cognitive domains have been described at disease onset in CBS, but dementia is not a classical presenting feature of CBS, and will occur in only about 25% of cases with advanced disease. Dementia is more frequent in CBD patients who do not present with classical CBS, occurring in the FTLD-tau phenotype in which the central presenting features are cognitive/behavioral symptoms.”
* “It is also important to consider that CBS patients who partially respond to AChEIs/memantine treatment may have underlying AD as well.”
* “For psychosis, aggression and agitation, antipsychotics (preferably atypical agents) are currently employed despite the possible adverse events that include extrapyramidal symptoms, cognitive deficits and also death; particular caution is needed when using neuroleptics in patients with overt parkinsonism.”
* “CBS patients also frequently report depressive symptoms. Selective serotonin reuptake inhibitors (SSRIs) provide effective treatment in these subjects.”
* “[R]ehabilitation therapies are an essential component of the multidisciplinary approach to CBS patients. The rehabilitation team may address mobility, safety, assistive devices, activities of daily living and communication.”
* Transcranial direct current stimulation “may represent a promising tool for cognitive enhancement and neuro-rehabilitation but further studies in a larger cohort are necessary.”
* “[A]dvanced CBS patients ideally require assistance in specialized palliative care units, with the specific aim of alleviating the burden of symptoms, and of preserving patients’ autonomy and supporting their families.”
* “In particular, aspiration pneumonia and urinary infections are the two main causes of death in CBD patients. All these problems are common in advanced stages of neurodegenerative disorders and clinicians need to recognize and discuss their implications with the patient and the family. For example, patients with dysphagia should be immediately referred for swallowing evaluations with fibrolaryngoscopy. As the disease progresses, patients, relatives and caregivers have to be informed about the opportunity to place a percutaneous endoscopic gastrostomy (PEG) tube, especially if all other aspects of quality of life are preserved. Moreover education and support of caregivers and families by the multidisciplinary team members is recommended where these services exist.”
I think this is adequate for most of you (and maybe even too much already!). For those who want to read more, I’ve copied the abstract below. You can link from the abstract to the full article; there may be a fee to purchase the full article.
Parkinsonism & Related Disorders. 2015 Sep 9. [Epub ahead of print]
Therapeutic interventions in parkinsonism: Corticobasal degeneration.
Marsili L, Suppa A, Berardelli A, Colosimo C.
Corticobasal degeneration (CBD) is a progressive neurodegenerative disorder resulting from pathological accumulation of tau protein and is included in the spectrum of Atypical Parkinsonism. The typical clinical phenotype of CBD is characterized by the Corticobasal syndrome (CBS). In recent years it has become clear that the clinical picture of CBS may be caused by different pathological conditions, resulting in frequent misdiagnosis. CBD has high morbidity and poor prognosis with no effective therapies. In this review, we will discuss the symptomatic treatment, the palliative care and the disease modifying strategies currently in use. Symptomatic treatment in patients with CBD may sometimes be useful for improving motor (parkinsonism, dystonia and myoclonus) and non-motor (cognitive-behavioral) symptoms, but the effects are often unsatisfactory. In addition, non-pharmacological strategies and palliative care are useful integrating components of the multidisciplinary therapeutic approach for patients with CBD. Despite many efforts, a disease-modifying treatment is still unavailable for CBD.
Copyright © 2015 Elsevier Ltd.
PubMed ID#: 26382843