What are the signs and symptoms of corticobasal degeneration (CBD)?

Updated: Sep 24, 2018
  • Author: Stephen M Bloomfield, MD; Chief Editor: Selim R Benbadis, MD  more...
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Clinical onset of CBD occurs in the sixth decade of life. Rinne et al reported 5 initial presentations, including a "useless" arm (55%), gait disorder (27%), prominent sensory symptoms, isolated speech disturbance, and behavioral disturbance. [38]

Symptoms on long-term follow-up include focal or asymmetric rigidity, bradykinesia, postural and action tremor, and marked dystonia. These problems usually arise predominantly in one upper extremity. Limb apraxia may become a serious problem, with independent movements occasionally as severe as an alien limb. The incidence of limb apraxia is far higher in CBD than in PSP, with a similar level of cognitive impairment.

Current clinical criteria tend to be biased in terms of motor symptoms. However, they are shifting toward criteria that include the important cognitive element. The dissociation between pathological criteria and clinical presentations has led to the term corticobasal syndrome (CBS), which distinguishes CBD from the classic clinical picture just described.

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