TITLE:
Moyamoya Disease Associated with Hyperhomocysteinemia: A Rare Cause of Stroke in Sub-Saharan Africa
AUTHORS:
Raphael Marie Kabore, Habib A. K. Ouiminga, Jean Kabore, Jean-Michel Vallat
KEYWORDS:
Moyamoya, Headache, Silent Infarcts, Hyperhomocysteinemia, Diagnosis, Treatment
JOURNAL NAME:
World Journal of Neuroscience,
Vol.12 No.1,
February
9,
2022
ABSTRACT: Introduction: Moyamoya disease (MMD) is a chronic cerebrovascular disorder
characterized by progressive bilateral occlusion of the supraclinoid internal
carotid artery (ICA) and its main branches, associated with the development of
fine collateral networks, especially adjacent to the site of occlusion in the
deep areas of the brain. MMD frequently occurs in East Asian populations, including pediatric and adult patients, and
may lead to ischemic or hemorrhagic
stroke, headache, epilepsy or transient ischemic attack. The majority is
presumed to be of genetic origin and few cases of thrombophilia have been reported in MMD. We report a
case of MMD in a young sub-Saharan African
woman associated with Hyperhomocysteninemia (HHCys). Observation: A
33-year-old female was seen for aphasia, which had suddenly appeared
fourteen days before, and was associated with memory impairment. She mentioned chronic headaches during the 10
previous years, of frontal seat, without migraine criteria. She had no
history of hypertension, no obesity, no known dyslipidemia, and there was no
family history of stroke. Neurological examination noted Wernicke’s aphasia,
with discreet right central facial palsy, associated with memory impairment.
The brain MRI revealed semi recent infarct
in the left parietal lobe, sequelae in the left temporo-occipital and right
frontal with lacunar ischemic lesions, associated with several foci of sub- cortical demyelination. MRA showed stenosis of the supraclinoid carotid
arteries and their division branches. In addition, there was a stenosis of the
posterior cerebral arteries, with a network of anastomosis at the level of the
base of the skull. An investigation of thrombophilia showed
hyperhomocysteinemia (HHCys). The diagnosis of moyamoya disease associated with
hyperhomocysteinemia was mentioned. She was treated by Aspirin combined
to folic acid. After a 3-year setback the patient was asymptomatic. Conclusion: MMD is a rare cause of stroke in sub-Saharan Africa. MRA should be performed
whenever possible, as well as investigation for thrombophilia when it’s
available. Combined antiplatelet agent with folic acid for ischemic stroke prevention is
effective.