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Changes in perfusion pattern using ECD‐SPECT indicate frontal lobe and cerebellar involvement in exercise‐induced paroxysmal dystonia
Author(s) -
Kluge Andreas,
Kettner Beatrice,
Zschenderlein Rolf,
Sandrock Dirk,
Munz Dieter L.,
Hesse Stefan,
Meierkord Hartmut
Publication year - 1998
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.870130124
Subject(s) - dystonia , ictal , paroxysmal dyskinesia , epilepsy , frontal lobe , neuroscience , medicine , basal ganglia , ictal interictal spect analysis by spm , pathophysiology , perfusion , cardiology , psychology , dyskinesia , parkinson's disease , central nervous system , disease
The clinical features of exercise‐induced paroxysmal dystonia (EPD) are delineated in a pedigree including two affected members (both male) showing an autosomal‐dominant inheritance trait. Gait analysis using kinematic electromyography during the motor attacks revealed coactivation of antagonistic calf muscles characteristic of dystonia. In the interval, impaired muscular alternation was observed. To characterize further the pathophysiological basis of the condition, ictal and interictal cerebral perfusion SPECT studies using technetium 99m‐ethyl cysteinate dimer (ECD) were performed to establish whether cortical hyperactivity indicative of epilepsy is present during the motor attacks and to identify regional changes in the ictal perfusion pattern that could indicate an anatomic structure relevant to the disease. During the motor attacks, decreased ictal perfusion of the frontal cortex was found in both patients. In contrast, increased cerebellar perfusion was observed. The perfusion of the basal ganglia also decreased. No cortical hyperfusion indicative of an epileptic nature was seen. Cerebellar hyperactivity in connection with prominent frontal hypoactivity has also been described in both the idiopathic and the symptomatic forms of dystonia. Our findings therefore suggest that EPD represents a paroxysmal movment disorder rather than epilepsy. It is concluded that changes in frontal and in cerebellar function are relevant to the pathophysiology of EPD.