SPECT abnormalities in generalized dystonia

• Published in: Pediatric neurology 1995-07, Vol.13 (1), p.5-10
• Main Authors: LeDoux, Mark S., Lane Rutledge, S., Mountz, James M., Darji, Jaykrishna T.
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; Brain - blood supply; Brain Mapping; Cerebellar Nuclei - blood supply; Child; Contrast Media; Dominance, Cerebral - physiology; Dystonia - congenital; Dystonia - diagnostic imaging; Dystonia - physiopathology; Female; Follow-Up Studies; Humans; Medical sciences; Nervous system (semeiology, syndromes); Nervous system as a whole; Neurologic Examination; Neurology; Organotechnetium Compounds; Oximes; Regional Blood Flow - physiology; Technetium Tc 99m Exametazime; Tomography, Emission-Computed, Single-Photon
• ISSN: 0887-8994; 1873-5150
• DOI: 10.1016/0887-8994(95)00109-S

A patient with severe, generalized dystonia and 6 age range-matched controls were studied with the regional cerebral blood flow tracer technetium-99m hexamethylpropyleneamine oxime by single-photon emission computed tomography to test the hypothesis that cerebellar function is abnormal in dystonia. Analysis was performed by drawing regions of interest around the caudate head nuclei, hemithalami, deep cerebellar nuclei, and cerebellar hemicortices. The counts in each region of interest were normalized to whole brain cerebral blood flow in an identical manner for each subject. The dystonic patient had a difference in regional cerebral blood flow between the right and left deep cerebellar nuclei, increased regional cerebral blood flow in subcortical motor structures, and an abnormal relationship between right cerebellar cortical and right deep cerebellar nuclear regional cerebral blood flow. The findings in this patient provide evidence that the cerebellum may play a role in the pathophysiology of motor signs in some patients with dystonia.