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Corticobasal degeneration/syndrome
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Corticobasal degeneration/syndrome (CBD/S)

Corticobasal degeneration/syndrome (CBD/S)

 

Investigators

Principal investigator:

Prof Huw R Morris1

Co-Investigators:

Dr Ruth Lamb1, Dr Jonathan D Rohrer2, Prof Andrew J Lees3, Dr John Woodside1, Marcia Darvell1

 

1 Department of Clinical Neuroscience, UCL Institute of Neurology, UCL, London, UK

2 Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, UCL, London, UK

3 Department of Molecular neuroscience, UCL Institute of Neurology, UCL, London, UK

 

Case Definition

Aged 16 years or over

Affected by clinically defined corticobasal syndrome or corticobasal degeneration

 

Background

Corticobasal degeneration (CBD) is a pathologically defined rare progressive neurodegenerative disease characterised by progressive asymmetrical rigidity and apraxia. The diagnosis of CBD is difficult due to the absence of specific biomarkers and the fact that many of the clinical features of CBD overlap with the other neurodegenerative diseases. Corticobasal syndrome (CBS) is the classical clinical presentation of CBD but may also be caused by Alzheimer, motor neuron disease, prion, fronto-temporal dementia pathology and other pathology.  Probable CBS is defined under current criteria an asymmetric presentation involving more than two of limb rigidity/akinesia, dystonia or myoclonus as well as two of orobuccal or limb apraxia, corticosensory deficit and alien limb phenomemon, whereas possible CBS is defined as symmetric or asymmetric onset of one of limb rigidity/akinesia, limb dystonia or limb myoclonus plus one of orobuccal apraxia or limb apraxia, cortical sensory deficit and alien limb phenomemnon (1).

 

Study Aims

The primary aim of this study is to understand how clinical and biological features can help us define and explain the varying pathology that underlies corticobasal syndrome, and to define the markers of pathological corticobasal degeneration.  The secondary aim of this study is to establish a cohort of patients with clinically well defined CBS that will be available for future therapeutic and clinical studies. We intend to identify CBS and CBD patients in the UK who are willing to participate in translational research related to CBS its aetiology, biology, progression and future treatment. 

 

Clinical involvement and case ascertainment

Reporting consultants will receive a permission to contact form to send out to patients with a clinical diagnosis of CBS and/or CBD, that is a probable or possible diagnosis of CBS as per the Armstrong criteria. Presence of any exclusion criteria will not prohibit entry to the study. (1). Should they wish to participate the patient will return a consent form directly to the study team.  A member of the study team at UCL will then contact the patient to discuss the study and respond to any questions that they might have.

 

Patients who consent to take part will either be recruited to the cross sectional arm or the longitudinal arm of the PROSPECT study.  Patients who join the cross sectional arm of the study will be invited to complete questionnaires and donate blood samples, this can be undertaken remotely. The study team will contact the local GP to see if they are willing to take the blood samples for the study.

 

Patients who consent to join the longitudinal arm of the study will be invited to have a study assessment at a local study centre (currently 7 centres in the UK).  This may include  neurological assessment, cognitive assessments, neuro-psychometry, donating blood samples, cerebro-spinal fluid samples,  eye movement assessments, smell tests and MRI scans.

 

All study investigations will be co-ordinated by the main study team. Neurologists will not be asked to carry out any formal assessment of inclusion/exclusion criteria or formal study procedures.  Inclusion in the study will be fed back to the referring consultant with the patient’s consent.  The patient will be able to have feedback on the development or availability of new NHS tests based on their samples if they choose and this will be communicated to the referring consultant with the patients’ permission.  If the patient consents, we may approach the clinical team to obtain copies of clinical assessments and investigations.  We will provide general study updates through presentations and posters at the ABN annual meeting.

 

References

(1). Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, Borroni B, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013;80(5):496–503

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