Diagnosis of patients with advanced disease

After 10 years of illness, dementia is more severe, with poor performance on all aspects of the cognitive examination except naming. Speech is dysfluent with long lapses between the examiner' question and the patient' reply, rather like Brocca' aphasia. Some patients will be virtually unable to speak, although language is relatively preserved. Patients (if they are co-operative) can carry out commands and will recognize relatives and nursing staff. Often irritability and depression have improved, but some patients continue to make suicide attempts or be physically aggressive. Physical disabilities are much worse. Patients often need to be fed, toileted, and helped with most daily needs. They have difficulty in walking and are at risk for falls that can cause further disability through broken limbs or subdural haematomas. Chorea often stabilizes and may even subside,(9) but the ability to carry out voluntary movements is more severely impaired. If they survive long enough, patients become unable to initiate speech, swallow with great difficulty, are unable to walk, and have such severely rigid muscle tone that they may be nearly unable to move their bodies. Clonus and positive Babinski signs are present. Patients in this sort of 'persistent vegetative state' (19 are difficult to distinguish from individuals with other movement disorders or dementias; as in early disease, eliciting a positive family history may be the only way to make a confident diagnosis without genetic testing.

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