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Dopaminergic Dysfunction in Midbrain Dystonia
Anatomoclinical Study Using 3-Dimensional Magnetic Resonance Imaging and Fluorodopa F 18 Positron Emission Tomography
Marie Vidailhet, MD;
Corinne Dupel, MD;
Stéphane Lehéricy, MD, PhD;
Philippe Remy, MD, PhD;
Didier Dormont, MD;
Michel Serdaru, MD;
Pierre Jedynak, MD;
Hugues Veber, MD;
Yves Samson, MD;
Claude Marsault, MD;
Yves Agid, MD, PhD
Arch Neurol. 1999;56:982-989.
Objective To determine the role of damage to neuronal systems, especially the dopaminergic system, in patients with symptomatic dystonia and mesencephalic lesions.
Design Stereotaxic magnetic resonance imaging analysis and positron emission tomography after the administration of fluorodopa F 18.
Patients Of a group of 48 patients with unilateral dystonia following a stroke, 7 patients with a well-defined midbrain lesion were selected.
Results All patients had unilateral dystonic posture of an upper extremity and cerebellar dysmetria or hypotonia. Cerebellar tremor was present in 1 patient. Two patients had resting and postural tremor, which showed a marked improvement with treatment with levodopa. In patients with dystonia only, dopaminergic lesions were mostly confined to the ventromesial mesencephalon and red nucleus area, including the substantia nigra and nigrostriatal and cerebellothalamic fibers. Dystonia was severe and did not resolve with time in patients with lesions involving the nigrostriatal pathway, and the degree of dopaminergic denervation revealed by positron emission tomography was correlated with the severity of dystonia. In patients with resting and postural tremor, lesions of the dopaminergic structures were larger and located more laterally and dorsally in the pars compacta, the perirubral and retrorubral areas, and extending to the central tegmental tract.
Conclusions Dopaminergic dysfunction plays a role in the occurrence and severity of midbrain dystonia, and additional lesions to dopaminergic neurons in the perirubral and retrorubral areas result in tremor that responds to levodopa treatment.
From The French Institute of Health and Medical Research INSERM U289 (Drs Vidailhet, Lehéricy, and Agid) and the Departments of Neurology (Drs Vidailhet, Serdaru, Jedynak, Veber, and Agid) and Neuroradiology (Drs Lehéricy, Dormont, and Marsault), Hôpital de la Pitié-Salpêtrière, Paris; and the Service Hospitalier Frédéric Joliot (Drs Dupel, Remy, and Samson), Commissariat à l'Energie Atomique, Orsay, France. Dr Vidailhet is now with the Department of Neurology, Hôpital Saint Antoine, Paris.
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