. 2013 Jun 16;22(21):4349–4356. doi: 10.1093/hmg/ddt284

Table 3.

Patient selection criteria

Patients falling in one of the following categories
A	Psychosis^a combined with ≥1 pre-defined neurological^{^b} or visceral^{^c} symptom/syndrome
B	Early-onset progressive cognitive decline^{^d} combined with ≥1 pre-defined neurological^{^b} or visceral^{^c} symptom/syndrome
C	Early-onset progressive cognitive decline^{^d} combined with psychosis^a

^aPsychosis defined according to ICD-10 and DSM-IV criteria, with onset at <50 years of age.

^bIncludes focal dystonia, cerebellar ataxia, dysarthria, vertical supranuclear gaze palsy, cataplexy, epilepsy.

^cHepatomegaly or splenomegaly.

^dEarly-onset progressive cognitive decline (at <50 years of age) includes dysexecutive syndrome, attention deficits, aphasia, apraxia, agnosia, memory impairment, dementia.