Table 4

Features supporting and contravening diagnosis of Perry syndrome

Clinical featuresLaboratory features
Supporting featuresNon-supporting featuresSupporting featuresNon-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.