Case of moyamoya disease in a patient with advanced acquired immunodeficiency syndrome.

BACKGROUND: Moyamoya disease is an occlusion of the terminal portion of internal carotid arteries and proximal portion of middle and anterior cerebral arteries of unknown origin. Moyamoya syndrome is associated with meningitis, tuberculosis, syphilis, head trauma, head irradiation, brain tumor, von Recklinghausen's disease, tuberous sclerosis, Marfan syndrome, sickle cell anemia, arteriosclerosis, hypertension, and oral contraceptive use. To our knowledge, acquired immunodeficiency syndrome (AIDS) as a cause of moyamoya syndrome has not been reported in an adult population. OBJECTIVE: We report a case of moyamoya syndrome in a patient with AIDS and without other conditions associated with occlusion of the circle of Willis and formation of collateral network at the base of the brain and basal ganglia. METHODS: We present a case report. RESULTS: A 29-year-old woman with an 8-year history of AIDS on multiple antiretroviral medications presented with recurrent tingling of the left extremities which 1 month later progressed to mild hemiparesis and dysarthria. During the next few months the patient developed progressive cognitive decline and on-and-off fluctuations in the degree of hemiparesis. Brain magnetic resonance imaging showed multiple small subcortical infarct's in both parietal lobes. Magnetic resonance angiography showed occlusion of middle cerebral arteries distal internal carotid arteries, with prominent collateral network. Cerebral angiography confirmed moyamoya pattern. Lumbar puncture showed: white blood cell count 1, red blood cell count 418, protein 56, glucose 53, negative bacterial and acid-fast bacilli smear and culture, negative VDRL test, India ink, cryptococcal antigen, cytology and negative polymerase chain reaction for cytomegalovirus, Epstein-Barr virus, varicella-zoster virus, and herpes simplex virus type 1 and 2. Electroencephalography showed diffuse background slowing. CONCLUSIONS: We hypothesize that human immunodeficiency virus (HIV) caused central nervous system vasculitis, which eventually led to formation of moyamoya pattern. No other definite causes of central nervous system vasculitis were found in our patient. Cerebrovascular disorders should be considered in patients with HIV/AIDS with focal neurologic deficit. Moyamoya syndrome as a cause of stroke should be considered in patients with HIV/AIDS, especially as survival improves.

Cerebral aneurysmal arteriopathy in an adult patient with acquired immunodeficiency syndrome.

In adult patients with acquired immunodeficiency syndrome (AIDS), cerebral arteritis usually takes the form of arterial wall thickening, stenosis, and occlusion, leading to cerebral ischemia and infarction. Aneurysms and intracranial hemorrhage are much less commonly associated with cerebral vasculitis. For reasons not entirely clear, this form is seen more often in pediatric patients infected with human immunodeficiency virus. We report an adult patient with cerebral aneurysmal arteriopathy who presented shortly after his AIDS-defining illness in a setting of severe immune suppression and high viral load.

Reduction in phosphorylated heavy neurofilament in the cerebellum in HIV disease.

In the absence of significant neuronal infection HIV induces neuronal damage and death. The pathogenesis of this process is not clear and can only be assessed in the HIV infected brain by examining surviving neuronal populations. Cerebellar Purkinje cells are a model population. We have already demonstrated glutamate receptor alterations in these neurons in AIDS, and in the current study we have investigated the phosphorylation status of heavy neurofilament (NF-H), which is under the control of various intracellular kinases. While the number of Purkinje cells expressing non-phosphorylated NF-H was unchanged, the number of Purkinje cells expressing phosphorylated NF-H was decreased by 36% in the HIV group. This may be a marker of neuronal damage, and possibly indicate alteration in the activity of various intracellular signalling kinase pathways in the HIV infected brain.

Short echo-time proton magnetic resonance spectroscopy in regions of severe HIV-related diffuse white matter abnormality on MRI.

The purpose of this study was to determine whether short echo-time proton magnetic resonance spectroscopy (H-MRS) could detect mobile lipid resonances attributable to myelin breakdown products in the deep cerebral white matter of patients with AIDS who have severe diffuse/patchy white matter hyperintensity on T2-weighted magnetic resonance imaging (MRI). Seven patients with AIDS and clinical HIV-associated dementia complex (HADC) and 12 male controls were studied at 1.5T using a single 8 ml voxel, gradient localised, stimulated echo acquisition mode (STEAM) spectroscopy sequence. Spectra were acquired at an echo time of 20 ms with a repetition time of 5000 ms. No spectroscopic peaks were identified at 0.9 ppm and 1.3 ppm (corresponding to lipid resonances) in 6 of the 7 patients with AIDS or in any of the controls. Lipid resonances were identified in 1 patient who had been taking anti-retroviral therapy for 8 weeks. Follow up MRI/H-MRS, performed after a further 14 weeks of anti-retroviral therapy, showed partial resolution of white matter hyperintensity and lipid resonances were not detectable. These data suggest that mobile lipids are only rarely detected by H-MRS in patients with HADC and abnormalities on MRI and that their presence may be transitory.