*Neuropsychology of Moyamoya Disease DOI: http://dx.doi.org/10.5772/intechopen.96558*

been explained by the chronic cerebral hypoperfusion caused by bilateral internal carotid artery involvement. The neurosurgical procedure had relieved the patient of headaches, but attention deficits and behavioural problemsremained after the operation and required specialized intervention. The decline in the patient's ADHD scores after the neurosurgical procedure might have been due to the persisting cognitive dysfunction caused by the early onset, bilateral arterial involvement, and chronic cerebral hypoperfusion [49].

In rare cases, Moyamoya syndrome is associated with clinical features of movement disorders, like Tourette's syndrome. In this context the first reported clinical case was that of a 5-year-and-9-month-old boy who developed repetitive episodic involuntary winking of the right eye along with ipsilateral shoulder-shrugging movements associated with paroxysmal shouts and loud laughs and punctuated with abusive verbal expressions (coprolalia), progressive regression of verbal and cognitive milestones, emotional lability and aspects of attention deficit hyperkinetic disorder [50]. The child was evaluated by MRI, which showed characteristic ischaemic areas involving the basal ganglia and fronto-parietal cortical regions and the middle cerebral artery territory, predominantly on the left side. Subsequent cerebral angiography revealed extensive stenosis of bilateral (predominantly left-sided) internal cerebral arteries and middle cerebral arteries, with evidence of diffuse leptomeningeal collaterals. The patient was eventually diagnosed with Moyamoya disease with associated Tourette's syndrome. Subsequently, he underwent left-sided superficial temporal artery to middle cerebral artery anastomosis along with encephalo-duro-arterio-myo-synangiosis. Significant clinical-radiological improvement was noted after three months, at which point, the clinical deficiencies had dramatically resolved. There was evidence of an excellent development of direct and indirect surgical collaterals and the left middle cerebral artery territory. The incidence of Moyamoya syndrome associated with intracranial aneurysms ranges from 3% to 14% in adult patients, whereas it is complication rarely reported in children. Noureldine et al. recently reported the first case of an infrequent subarachnoid haemorrhage a child with a ruptured anterior cerebral artery-dissecting aneurysm secondary to a newly discovered, unilateral Moyamoya-like pathology. These authors argued that prompt intervention is essential to exclude the risk of the ruptured aneurysmrebleeding due to persistent hemodynamic stress [51]. Reports on patients with pMMD who present cerebral ischemic complications after intraventricular haemorrhage (IVH) and/or intracerebral bleeding (ICB) are minimal. In this sense, Inoue et al. reported a case of a 7-year-old girl with moyamoya disease with severe cerebral vasospasm and delayed cerebral infarction following an IVH. The authors stressed that, though such cases are rare, the potential for vasospasminduced cerebral infarction should be considered and, intensive treatment initiated immediately if suspected [52].

We can conclude, based on published evidence, that less neurological insult will lead to better cognitive outcomes. However, the impact of MMD on cognition remains unclear. Even though surgical treatment generally results in positive neurological outcomes, the relationship between the two needs to be investigated. Further clinical studies should focus on a wide range of neuropsychological tests and measurements ofcerebral blood flow and metabolism in large series.
