Journal of neuroimaging : official journal of the American Society of Neuroimaging
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Hyperargininemia (HA) is a rare autosomal recessive metabolic disorder and the neuroimaging features of this disease have seldom been reported. Hyperammonemic encephalopathy is uncommon in HA, and the clinical presentation of HA is distinct from other urea cycle disorders. This paper describes the brain MRI findings and a magnetic resonance spectroscopy (MRS) study of a series of Brazilian HA patients. ⋯ We present the MRI and MRS findings of a large series of HA patients. Variable degrees of brain atrophy and mild cerebellar atrophy were observed, and these findings were not specific. No metabolic abnormality was observed using MRS in this series of patients.
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In patients with critical carotid stenosis and ischemic stroke, it is crucial to determine whether distal hypoperfusion or artery-to-artery embolism is the predominant mechanism. The role of transcranial doppler (TCD) in this setting is vital. We report a patient with fibromuscular dysplasia and recurrent orthostatic transient ischemic attacks where fall in cerebral perfusion was clearly demonstrated by TCD.
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Case Reports
Neuroblastoma with a solitary intraventricular brain metastasis visualized on I-123 MIBG scan.
Although metastatic skull lesions of neuroblastoma are not uncommon, brain involvement is infrequent and prompt diagnosis is of utmost importance in such cases. Previous studies have shown that Meta-Iodo-Benzyl-Guanidine (MIBG) scans were not always reliable in detecting central nervous system metastases, however most published reports referred to the Iodine-131 ((131)I)-MIBG scans. Herein, we report an intraventricular metastasis of neuroblastoma diagnosed using an Iodine-123 ((123)I)-MIBG scan, which is increasingly being used in clinical practice and reported as a more accurate method for detecting metastatic lesions. ⋯ Our case shows that (123) I-MIBG scintigraphy can be useful in detecting intraventricular brain metastases of neuroblastoma. Although the (123)I-MIBG scintigraphy has been reported to have a significantly superior sensitivity in monitoring asymptomatic patients with neuroblastoma compared with (131)I-MIBG scans, bone marrow histology, bone scan, CT, and urinary catecholamine levels, further studies may be necessary to evaluate its sensitivity in detecting brain lesions.
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Recurrence following endovascular treatment of intracranial aneurysm is attributed to either coil compaction or aneurysm growth but these processes have not been studied as distinct processes. ⋯ This is one of the first studies to differentiate coil compaction and aneurysm growth as distinct etiologies for aneurysm recurrence.