Hemiparkinsonism and hemiatrophy syndrome: a rare observation.
Hemiparkinsonism with hemiatrophy syndrome is defined by the occurrence of a body hemiatrophy with features of an early onset, slowly progressive, asymmetric parkinsonism more prominent on the side of the hemiatrophy. The underlying pathogenesis is not well understood but perinatal cerebral insults seem to play a crucial role. We describe the case of a 52-year-old woman who presented with a two year history of slowness and stiffness of left arm and leg with dystonic posturing of the ipsilateral hand. When examined, she had a left body hemiatrophy which was present since early childhood. Clinical and imaging findings supported the diagnosis of hemiparkinsonism with hemiatrophy syndrome with a good response to dopamine agonist therapy. This case report further characterizes this heterogeneous form of parkinsonism which deserves attention for clinical management and prognostic evaluation.
Tessitore
Alessandro
A
Second Division of Neurology, Second University of Naples, 80138, Naples, Italy. alessandro.tessitore@unina2.it
Russo
Antonio
A
Cirillo
Mario
M
Giordano
Alfonso
A
Marcuccio
Laura
L
Tedeschi
Gioacchino
G
D001284:Atrophy; D001923:Brain Chemistry / Q000502:physiology; D050483:Dopamine Plasma Membrane Transport Proteins / Q000378:metabolism; D004421:Dystonia / Q000150:complications; D005260:Female; D019788:Fluorodeoxyglucose F18; D007839:Functional Laterality / Q000502:physiology*; D020233:Gait Disorders, Neurologic / Q000150:complications; D006801:Humans; D008875:Middle Aged; D018908:Muscle Weakness / Q000150:complications; D009460:Neurologic Examination; D010300:Parkinson Disease / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D019275:Radiopharmaceuticals; D015899:Tomography, Emission-Computed, Single-Photon
D050483:Dopamine Plasma Membrane Transport Proteins; D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18