- Department of Neurosurgery, University of Toyama, Toyama, Japan.
Satoshi Kuroda, Department of Neurosurgery, University of Toyama, Toyama, Japan.
DOI:10.25259/SNI_661_2021Copyright: © 2021 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Yuichiro Koga, Daina Kashiwazaki, Emiko Hori, Naoki Akioka, Satoshi Kuroda. Oro-mandibular dystonia in pediatric moyamoya disease: Two cases report. 06-Sep-2021;12:449
How to cite this URL: Yuichiro Koga, Daina Kashiwazaki, Emiko Hori, Naoki Akioka, Satoshi Kuroda. Oro-mandibular dystonia in pediatric moyamoya disease: Two cases report. 06-Sep-2021;12:449. Available from: https://surgicalneurologyint.com/surgicalint-articles/11092/
Background: In this report, we describe rare two pediatric cases that developed oro-mandibular dystonia due to moyamoya disease.
Case Description: A 7-year-old boy presented with oro-mandibular dystonia and transient weakness of the left extremities, and was diagnosed as moyamoya disease. Another 7-year-old boy developed oro-mandibular dystonia alone and was diagnosed as moyamoya disease. In both, cerebral blood flow (CBF) was markedly decreased in the involved hemispheres, including the basal ganglia and cerebral cortex. They successfully underwent combined bypass surgery and experienced no further attacks of oromandibular dystonia during follow-up periods. CBF almost normalized through surgical collaterals through direct and indirect bypass.
Conclusion: When treating patients with oro-mandibular dystonia, moyamoya disease should be listed as one of the differential diseases. The underlying mechanism of oro-mandibular dystonia in moyamoya disease is still unclear, but persistent cerebral ischemia in the basal ganglia and/or parietal lobe may play a key role to induce this rare symptom.
Keywords: Bypass surgery, Cerebral blood flow, Involuntary movement, Moyamoya disease, Oromandibular dystonia
Moyamoya disease causes various presentations, but most of the pediatric patients develop transient ischemic attack (TIA) and ischemic stroke.[
This report first describes two pediatric cases of moyamoya disease that manifested involuntary movements in the oro-mandibular area.
A 7-year-old boy suddenly experienced transient loss of the ability to close his mouth while he was eating. This symptom of jaw opening lasted for about 5 min, followed by transient weakness of the left extremities for a few minutes. Neurological examination on admission revealed no abnormalities. No parenchymal lesions were observed on FLAIR image. MRA and cerebral angiography showed the narrowing of the carotid forks and their branches on both sides. SPECT demonstrated a marked decrease in cerebral blood flow (CBF), including the striatum and cortex, in the right cerebral hemisphere [
Radiological findings of a 7-year-old boy who developed oro-mandibular dystonia and transient ischemic attack (Case 1). (a) No parenchymal lesions were observed on FLAIR image (b) Magnetic resonance angiography showed the narrowing of the carotid forks and their branches on both sides. Moyamoya vessels were more apparent in the right side. (c) 123I-IMP SPECT demonstrated a marked decrease in cerebral blood flow (CBF) on the right side, including the striatum and cortex. (d) On follow-up SPECT at 4 months after superficial temporal artery to middle cerebral artery anastomosis and encephalo-duro-myo-arteriopericranial synang onto the right side, CBF markedly improved on the operated hemisphere.
A 7-year-old boy suddenly experienced involuntary movements of the jaw and tongue. Characteristically, dystonic and dyskinetic motions were seen, including jaw opening and the jaw and throat making clicking sounds. Neurological examination on admission showed no abnormalities. No parenchymal lesions were observed on FLAIR image. MRA and cerebral angiography demonstrated severe stenosis of the carotid forks on both sides. SPECT showed a decreased in CBF in the left hemisphere, including the striatum and cortex [
Radiological findings of a 7-year-old boy who developed oro-mandibular dystonia (Case 2). (a) No parenchymal lesions were observed on FLAIR image (b) Magnetic resonance angiography showed the stenosis of the carotid forks and their branches on both sides. (c) 123I-IMP SPECT demonstrated a significant decrease in cerebral blood flow (CBF) on the left side, including the striatum and cortex. (d) On follow-up SPECT at 6 months after superficial temporal artery to middle cerebral artery anastomosis and encephalo-duro-myo-arterio-pericranial synang on both sides, CBF markedly improved on the operated hemispheres.
In the present cases, unique movement disorder around their jaw is considered consistent to oromandibular dystonia. Dystonia is defined as involuntary lasting severe muscle contractions and is defined as oromandibular dystonia when the oral cavity such as the mouth, face, and jaw is involved.[
The mechanisms of involuntary movements in moyamoya disease are still unclear, but it is most likely that the basal ganglia may be largely involved in the occurrence, where the dilated moyamoya vessels are often found.[
Second, the hypertrophied moyamoya vessels in the basal ganglia may physically compress the neurons and/or the extrapyramidal tract in the basal ganglia, causing involuntary movements.[
Finally, involuntary movement may develop through hypermetabolic state in the striatum due to the dilated moyamoya vessels. Thus, glucose metabolism was reported to increase in the striatum responsible for unilateral chorea in two pediatric patients who developed involuntary movements “after” bypass surgery, although they did not experience it before surgery.[
Alternatively, recent studies have shown that dystonia is not only caused by an abnormality of the basal ganglia alone, but also by a functional impairment of other brain regions such as the cerebellum, thalamus, midbrain/brainstem, and cerebral cortex. For example, some investigators have largely focused on the cerebello-thalamo-cortical network.[
We report rare two cases that developed oro-mandibular dystonia due to moyamoya disease. The underlying mechanism of oromandibular dystonia in moyamoya disease is still unclear, but persistent cerebral ischemia in the basal ganglia and/or parietal lobe may play a key role in this rare symptom.
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