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- Title
Moyamoya syndrome in a patient with D-2-hydroxyglutaric aciduria type II: a rare association.
- Authors
Kühnl, Tobias; Januschek, Elke; Offenbach, Sana Klinikum
- Abstract
Purpose: Several underlying conditions of moyamoya syndrome (MMS) are well established, but so far, D-2-hydroxyglutaric aciduria (D-2-HGA) has not been mentioned. We are the first to describe a case of a patient suffering from D-2-HGA developing MMS. Methods: The co-occurrence of D-2-HGA and MMS in a patient is reported. Furthermore, we describe the neurosurgical revascularization procedure performed and report on the follow-up. Results: A 7-year-old girl suffering from D-2-HGA developed two transient ischemic attacks (TIAs). Using MRI/MRA and invasive angiography MMS was diagnosed. We performed an encephalo-duro-arterio-myo-synangiosis (EDAMS) as an indirect revascularization procedure first on the right and 2 months later on the left hemisphere. We have followed her up until the age of 10. Since the second surgery, she has not suffered further TIAs and is in a better general medical condition. Conclusion: Even though children with D-2-HGA often suffer epileptic attacks, every new (transient) neurological deficit should be followed up by an MRI/MRA so as not to oversee a possible underlying MMS. After diagnosis, EDAMS in combination with acetylsalicylic acid (ASA) is recommended to prevent further ischemic events.
- Subjects
ASPIRIN; TRANSIENT ischemic attack; SYNDROMES
- Publication
Child's Nervous System, 2024, Vol 40, Issue 7, p2241
- ISSN
0256-7040
- Publication type
Case Study
- DOI
10.1007/s00381-024-06340-9