Moyamoya Disease Associated with Hyperhomocysteinemia: A Rare Cause of Stroke in Sub-Saharan Africa

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.

Spinal Cord Compression, a Rare Neurofibromatosis Complication

Objective: The objective of this study is to report a case of spinal cord compression, which is a rare complication of neurofibromatosis type 1. Observation: We report the case of a 45-year-old man, which presented a syndrome of thoracic spinal cord compression at the stage of spastic paraparesis. Its installation was gradually over 6 months associated with the inaugural back pain. He had a clinical history of neurofibromatosis type 1 with “Café-au-lait” spots. There were multiple painless nodules under the skin of different size on the chest, forearms and legs. A large isolated nodule, purplish was observed on the chest. The neuro-imaging showed a para-spinal anterior mass expansion inside the spinal canal causing spinal compression at the level of the second and third thoracic vertebra. It extends into the intervertebral foramen of the third and fourth thoracic vertebra leading to a scalloping. A second large heterogeneous left intra-abdominal mass containing cyst areas and calcifications was discovered in imaging. After a spinal decompression with laminectomy of the second and third thoracic vertebra, the reduction of pain and motor recovery was gradual. The large nodule excision was performed. Histology found a plexiform neurofibroma. Excision of the left intra-abdominal mass could not be performed because the patient’s consent had not been obtained. Conclusion: The spinal cord compression is a rare complication of neurofibromatosis type 1. However, it is essential to think about it in front of any spinal cord symptoms or any atypical long term spinal pain.

Symptomatic Extra-Dural Arachnoid Cyst

Objective: The aim of this study is to report a case of arachnoid cyst due to mass effect on the thoracic marrow. Observation: A 15-year-old patient was admitted to our institution and complaining of high back pain. Three months before he developed progressive and occasional back pain with thoracic irradiation in hemi-belt, increased by the dorsal decubitus. Neurological examination revealed a spasmodic paraplegia. The muscular strength was quoted as 2 to the left and 3 to the right on a scale of 5. Magnetic resonance imaging (MRI) revealed and extradural cyst located to the dorsal spinal cord. The ablation of a voluminous translucent cyst was achieved after a vast decompressive laminectomy from the sixth to the eighth thoracic vertebra. The dural communication with the cyst was stitched. After surgery, the pain has disappeared and the neurological recovery was progressive over a period of 21 days. The diagnosis of arachnoid cyst was confirmed by histological examination. Conclusion: The extradural thoracic arachnoid cyst is a rare affection of good forecast. In the symptomatic form, the surgery as soon as possible remains the solution. The MRI keeps all its interest for the diagnostic orientation and the therapeutic strategy.