Table 4.

Features supporting and contravening diagnosis of Perry syndrome

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.