Volume 8: Variant CJD
6. Diagnosis, treatment and care of vCJD patients
Symptoms of vCJD

6.3 On 21 March 1996, the CJDSU circulated to all neurologists in the UK a description of the clinical and pathological features of the new phenotype. ¹ The clinical features of the new phenotype of CJD described in the circular were as follows:

1. An early age of onset or death (average 27.6 years, range 18-41 years).
2. A prolonged duration of illness (average 13.1 months, range 7.5-24 months).
3. A predominantly psychiatric presentation including anxiety, depression, withdrawal and progressive behavioural changes.
   • The first evidence of neurological involvement in four patients was dysaesthesiae ² in the limbs and/or face.
   • Development of a cerebellar syndrome with problems with gait and limb muscle coordination after a period of weeks or months.
   • Development of forgetfulness and memory disturbance, often late in the clinical course, which progressed to severe cognitive impairment and a state of akinetic mutism ³ in the majority of cases.
   • Development of muscle twitching or spasms in the majority of patients (myoclonus), preceded by purposeless involuntary movements in some (chorea), with EEG appearances typical of sporadic CJD absent.

6.4 The circular noted that there was likely to be an increase in psychiatric referrals as the early symptoms of vCJD were 'relatively non-specific'. It also mentioned that early identification might depend on a history of evolving psychiatric disturbance followed by clear evidence of a cerebellar syndrome and/or memory disturbance. ⁴