A rare form of secondary parkinsonism has been reported as hemiparkinsonism-hemiatrophy syndrome (HPHA), first described by Klawans in 1981.1 This condition is defined by the occurrence of a body hemiatrophy with features of an ipsilateral, early onset, slowly progressive, levodopa-responsive hemiparkinsonism.1,2 The underlying pathogenesis is not well understood, but perinatal cerebral insults seem to play a crucial role. Magnetic resonance imaging (MRI) may reveal no abnormality or show cortical or subcortical herniatrophy (or both) contralateral to the side of HPHA with ventricle asimmetry.3 Functional neuroimaging studies have provided evidence of pre- and postsynaptic nigrostriatal dopaminergic dysfunction in patients with HPHA syndrome.4
Midbrain hemiatrophy and nigral rarefaction in a patient with hemiparkinsonism-hemiatrophy syndrome.
Arabia G;Sabatini U;
2016-01-01
Abstract
A rare form of secondary parkinsonism has been reported as hemiparkinsonism-hemiatrophy syndrome (HPHA), first described by Klawans in 1981.1 This condition is defined by the occurrence of a body hemiatrophy with features of an ipsilateral, early onset, slowly progressive, levodopa-responsive hemiparkinsonism.1,2 The underlying pathogenesis is not well understood, but perinatal cerebral insults seem to play a crucial role. Magnetic resonance imaging (MRI) may reveal no abnormality or show cortical or subcortical herniatrophy (or both) contralateral to the side of HPHA with ventricle asimmetry.3 Functional neuroimaging studies have provided evidence of pre- and postsynaptic nigrostriatal dopaminergic dysfunction in patients with HPHA syndrome.4I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
