Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0025362 (mental retardation)
 15,878 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Authors review 53 children, aged 0 to 14 years, affected with cerebrovascular ischemic strokes. Largest aetiological groups were: a) congenital heart disease, 16 patients; b) arteritis of unknown cause, 11; c) idiopathic arterial occlusion without arteritis images on angiography, 7; d) moyamoya disease, 6; and d) local or systemic infections, 5. The mode of onset was as completed stroke in 72% and stroke in evolution in 24%. After acute stage 17.6% of patients presented other definitive strokes, 11.7% suffered only transient ischemic strokes (TIA), and 4% reversible ischemic neurologic deficits (RIND). Mean follow-up was 4.36 years, 9.8% of patients died, 11.8% recovered completely and 52.9% improved after initial stroke. Poor global evolution was associated with heart disease (p less than 0.05) and with onset of strokes before age 2 (p less than 0.05). Most important sequelae, besides motor impairment, were epilepsy (49%) and mental retardation (50% got less than IQ 80). Late epilepsy was associated with seizures at onset (p less than 0.05). Clinical factors of adverse mental development were: a) seizures at onset, b) late epilepsy and c) stroke before age 2. 66% of cases had two or more arterial lesions in the same or in different arterial trees. Therefore, embolic and arteritic factors probably play an important role in infancy and childhood stroke.
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PMID:[Ischemic cerebrovascular accidents in childhood]. 340 Sep 36

We have reported the characteristic dynamic changes at the base of the brain in childhood Moyamoya disease in terms of follow-up angiography. So we have classified the angiographical findings of Moyamoya disease into six stages. However, there have been no report that proved these findings in long-term follow-up. On the other hand, these angiographical findings are mainly observed in children, we can seldom to find out any changes in adults. We performed long-term follow up angiography in adult cases whose onset was in childhood to clarify the natural course of this disease and to understand the difference between the cases of children and adults. Eleven cases (4 males and 7 females) of Moyamoya disease were investigated by angiography. Average onset was 5.1 years old. All of them were diagnosed by initial angiography in childhood (average age were 6.5 years old) and they grew up into adolescence (average age were 18.6 years old) at the time of follow-up study. Initial symptoms of them were all ischemic (TIA 7, RIND 2 or completed stroke 2). The symptoms at the time of follow-up were mental retardation in 5 patients, slight neurological deficits in 1, TIA in 2 and normal in 3. Among them, one patient had suffered from intraventricular hemorrhage. The results were as follows: the progression of angiographical stages was observed in 95% of sides. In 71% of sides, angiographical stage was in 5 or 6 which is thought to be an end stage of this disease.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Long-term follow-up angiography of moyamoya disease--cases followed from childhood to adolescence]. 395 60

Familial occurrence of moyamoya disease is described in the mother and four daughters, including identical twins. Physical examination findings on admission were all normal and no mental retardation was observed. The third daughter had suffered from a ventricular septal defect when aged 6 years, but the others all had unremarkable past histories. Four of the five patients presented with transient ischemic attack as the initial symptoms, but one patient remains asymptomatic. Two patients had had repeated transient ischemic attacks. Cerebral angiography revealed either stenosis or occlusion of the intracranial portion of the bilateral internal carotid arteries associated with moyamoya vessels in all patients. The findings of moyamoya disease in a parent and four siblings including identical twins suggest that genetic factors are important in the pathogenesis of moyamoya disease.
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PMID:Familial occurrence of moyamoya disease in the mother and four daughters including identical twins. 968 18