Clinical features | Laboratory features | ||
Supporting features | Non-supporting features | Supporting features | Non-supporting features |
(1) Frontal signs | (1) Hypoxia and hypercapnia due to cardiac and pulmonary diseases | (1) MRI/CT: normal or frontotemporal atrophy | (1) Genetic test: mutation in the MAPT gene |
(2) Oculomotor disorders | (2) Seizures | (2) Functional imaging: reduction of striatal tracer uptake | (2) Pathology: evidence of other neurodegenerative diseases |
(3) Cognitive impairment | (3) Myoclonus | (3) Cardiac MIBG scintigraphy: decreased uptake | |
(4) Autonomic dysfunction | (4) Cerebellar ataxia | (4) Functional imaging: frontotemporal hypometabolism | |
(5) Sleep disturbances | (5) Sensory impairment | ||
(6) Amyotrophy |
MIBG, [123I]-metaiodobenzylguanidine.