Moyamoya syndrome in a patient with D-2-hydroxyglutaric aciduria type II: a rare association.
Childs Nerv Syst
; 40(7): 2241-2244, 2024 Jul.
Article
in En
| MEDLINE
| ID: mdl-38668906
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.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Moyamoya Disease
Limits:
Child
/
Female
/
Humans
Language:
En
Journal:
Childs Nerv Syst
Journal subject:
NEUROLOGIA
/
PEDIATRIA
Year:
2024
Document type:
Article
Affiliation country:
Alemania
Country of publication:
Alemania