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Nuklearmedizin 1999; 38(02): 72-74
DOI: 10.1055/s-0038-1632194
Originalarbeiten — Original Articles
Schattauer GmbH

Follow-up Findings in Regional Cerebral Blood Flow (r-CBF)-SPECT in a Case of idiopathic Childhood Hemidystonia

Functional Neuroiniaging and Pathophysiological ImplicationsSPECT der regionalen Hirndurchblutung zur Verlaufskontrolle bei kindlicher idiopathischer HemidystonieFunktionelle Bildgebung und pathophysiologische Implikationen
A. Fiedler
1   Department of Pediatrics (Head: Priv.-Doz. Dr. med. H. Segerer), St. Hedwig Hospital, Regensburg, Germany
,
J. Marienhagen
*   Department of Nuclear Medicine (Head: Prof. Dr. Chr. Eilles), University Hospital, Regensburg, Germany
,
Judith Aderbauer
1   Department of Pediatrics (Head: Priv.-Doz. Dr. med. H. Segerer), St. Hedwig Hospital, Regensburg, Germany
,
Elke Bock
*   Department of Nuclear Medicine (Head: Prof. Dr. Chr. Eilles), University Hospital, Regensburg, Germany
,
H. Segerer
1   Department of Pediatrics (Head: Priv.-Doz. Dr. med. H. Segerer), St. Hedwig Hospital, Regensburg, Germany
,
Chr. Eilles
*   Department of Nuclear Medicine (Head: Prof. Dr. Chr. Eilles), University Hospital, Regensburg, Germany
› Author Affiliations
Further Information

Publication History

Received: 14 September 1998

accepted: 11 November 1998

Publication Date:
03 February 2018 (online)

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Summary

A 9½-year-old girl suffered from intermitting tremor and jitteriness of her left hand and oral muscles every 4 to 6 weeks with long lasting episodes. Clinically myoclonias and dystonic positioning of the left arm, hand and facial muscles were seen. No evidence of trauma, infection or inborn errors of metabolism was found. Successful therapy with carbamazepine was initiated while L-DOPA failed. An ictal 99m-Tc-HMPAO-SPECT showed severe asymmetry with focal hyperperfusion of the contralateral right thalamus and basal ganglia as well as of the bifrontal cortex, whereas no anatomical lesions were found by MRI. In contrast, an interictally performed 99m-Tc-HMPAO SPECT showed hypoperfusion of the right thalamus and normalisation of the frontal perfusion under medical treatment. These 99m-Tc-HMPAO-SPECT findings may provide new insights into the localisation and pathophysiological pathways of idiopathic childhood dystonia.

Zusammenfassung:

Ein 9½jähriges Mädchen litt an rezidivierenden, langdauernden Schüben von Tremor und Zittern der linken Hand und der perioralen Muskulatur links. Klinisch fanden sich eine dystone Haltung des linken Armes und unerschöpfliche Myoklonien des Armes, der Hand und der Gesichtsmuskulatur links. Trauma, Infektion oder ein Stoffwechseldefekt als Ursache lagen nicht vor. Ein Therapieversuch mit L-DOPA war erfolglos. Unter Gabe von Carbamazepin wurde Beschwerdefreiheit bleibend erreicht. Ein iktuales 99m-Tc-HMPAO-SPECT zeigte eine fokale Hyperperfusion des rechtsseitigen Thalamus und der Basalganglien, sowie des bifrontalen Kortex. Ein interiktuales 99m-Tc-HMPAOSPECT ergab dann eine deutliche Hypoperfusion des rechtsseitigen Thalamus bei normalisierter Perfusion des bifrontalen Kortex. Eine kranielle Magnetresonanztomographie (MRI) ergab einen unauffälligen Befund. Der Vergleich der iktualen und interiktualen Perfusionsverhältnisse weist auf neue pathophysiologische Zusammenhänge bei idiopathischer kindlicher Dystonie hin.

Key words

Hemidystonia - thalamus - SPECT

Schlüsselwörter

Hemidystonie - Thalamus - SPECT
 

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