Occlusive Disease of Intracranial Main Arteries With Collateral Networks (Moyamoya Disease) in Adults

Abstract

Three adult cases of occlusive disease of the intracranial main arteries with collateral networks (moyamoya disease) are reported. Each patient had a characteristic angiographic picture: occlusion of the internal carotid artery at the level of the siphon just above the origin of the posterior communicating artery, combined with typical collateral networks in the base of the brain and in the convexity. Transitory ischaemic attacks occurred in the initial phase of the disease. One female patient had her initial transitory symptoms during the use of contraseptive pills. Her first objective neurological deficits, spastic hemiparesis and dysphasia, occurred half a year later, some days after discontinuation of the pills. Later she had a spontaneous intracerebral and subdural haematoma; she was then treated conservatively. The other female patient also exhibited severe dysphasia combined with a slight hemisyndrome. Biopsy of the temporal artery showed marked intimal proliferation in this female case only. The male patient suffered mainly from neurotic and depressive symptoms. Brain scanning suggested cerebral infarction, which disappeared later. EEG findings varied from slight asymmetry to focal changes. At follow‐up examination the male patient was able to work, but both females were unable to care for themselves. Several aetiological factors possibly operate in the background of the disease, which does not appear to be confined to any particular race.

Aspirin use and platelet aggregation in ischemic onset-type pediatric moyamoya patients with intractable headaches (moya-ache)

“…Although at this stage its clinical characteristics cannot be defined strictly, moya-ache is used to designate a pain symptom which is characteristic for pediatric moyamoya disease and the pathological headache associated with it and does not appear related to improvement in CBFD by surgery. Another trait which is characteristic of moya-ache is that this symptom gradually becomes less severe around the second half of the school-age period (around age [13][14] and in most cases, administration of aspirin can be discontinued before adulthood (around age 18), as was done in our series. With respect to pediatric moyamoya disease, the observable phenomenon that the value of the PAT (collagen) is related to the pathology of pediatric moyamoya disease itself leaves room for further study.…”

“…In the cases of pediatric moyamoya disease, it has been reported that fibrous connective tissue proliferates histologically inside the basal membrane which suggests that it has a multilayer structure and that it is formed after a certain period of time [13][14][15]. The growth of fibrous tissue among vascular endothelial cells means that collagen could be more easily exposed, and the present research results show that platelet aggregation in patients with ischemic onset-type pediatric moyamoya disease shows higher reactivity to collagen [16][17][18].…”

A psychiatric clinical picture revealing moyamoya disease

“…Impaired basal ganglia function due to abnormal vessel collateralization or ischemia may account for this tenderness, which is similar in dementia with lewy bodies. Antipsychotics, however, are effective in reducing hallucinations and paranoia [4,13]. Secondgeneration agents, such as quetiapine, are preferred given the lower risk of extrapyramidal complications.…”

“…Dosage reduction after prolonged resolution of symptoms seems reasonable, particularly if symptoms occurred before surgery. Antipsychotic treatment may also help with motor tics if present [4].…”

“…The clinical expression of this disease is highly variable, particularly in adults. It can be noisy and characterized by the occurrence of infarction and/or cerebral hemorrhage or paucisymptomatic or even asymptomatic [4]. A typical or atypical psychiatric clinical picture may be an expression of this rare disease [5].…”