Acute infantile hemiplegia in a patient with propionic acidaemia

A 10-month-old girl with mild developmental delay became hemiplegic after seizures. Cranial CT scan and magnetic resonance imaging (MRI) revealed no lesions related to vascular diseases, but brain atrophy on the right side was remarkable. Digital subtraction angiography showed slightly decreased visualization of peripheral branches of the right medial cerebral artery. Propionic acidaemia was diagnosed on the basis of high plasma levels of propionic acid and its metabolites and the elevated urinary excretion of these acids. With therapy, the levels of these acids fell, and her left hemiplegia disappeared 3 months later.     

Acute infantile hemiplegia caused by cerebral ischemic infarction. Etiology, clinical features and investigations

In 19 children with acute infantile hemiplegia an ischemic cerebral infarct was found clinically and by serial computertomography. In 11 patients an angiography has been performed in addition. 9 of the children had chronic diseases which are known as predisposing factors for cerebrovascular disease (congenital heart disease in 7 and chronic renal failure with hypertension in 2). One child had a severe hypernatremic dehydration due to infantile diarrhea and in 1 child thrombosis of the internal carotid artery occurred 3 days after a perforating trauma of the soft palate. No obvious reason for the ischemic stroke could be evaluated in 8 children. The onset of symptoms was either acute or slowly progressive. An altered state of consciousness was present in 11 children. Hemiparesis was found in 18 patients (13 right, 5 left) accompanied by facial palsy in 12 and aphasia in 6. Seizures occurred in 6 patients. One patient with incomplete occlusion of a vertebral artery showed acute cerebellar ataxia. In children without predisposing factors the prevalence of girls was higher (2 : 6) and there was a history of a preceding acute febrile illness in 5 of 8 patients. Laboratory investigations showed polycythemia in 4 children with cyanotic heart disease and additional hypochromia in two. Blood sedimentation rate was increased in 6 out of 8 patients without a known predisposing factor. Cerebrospinal fluid (CSF) showed a slight increase of erythrocytes (36-88/cmm) in 4 children, in two others purulent CSF was obtained after the infarct had developed into a brain abscess. The etiology of ischemic stroke in childhood and the possibility of an inflammatory vascular disease are discussed.     

Acute hemiplegia in infancy

Conclusion Although acute infantile hemiplegia has been under scrutiny for more than a century, our knowledge of the pathogenesis of this disabling condition remains limited. Previously healthy infants and small children become acutely ill and hemiplegic, often after having had prolonged focal seizures. What is the mechanism of the cere-brovascular compromise? In the neonate, do hemodynamic factors account for prenatal and perinatal vascular occlusions due to placental emboli? In the older infant, is a hypercoagulable state induced by the fever or a circulating toxin, or is autoregulation immature? At this time, intensive rehabilitation is the most appropriate way of remediating the problem. More effective means of preventing the morbidity of this disease may be devised when we obtain a better understanding of the patho-physiologic processes that are involved.     

Acute Werdnig-Hoffmann disease: Acute infantile spinal muscular atrophy

76 cases of acute Werdnig-Hoffmann disease (acute infantile spinal muscular atrophy) have been reviewed. The cases comprise an unselected consecutive series in which rigid diagnostic criteria have been applied. The natural history of the disease has been investigated. In at least one-third of cases the disease is manifest before or at delivery. All cases have shown delayed milestones by 5 months of age: 95% are dead by 18 months. Cumulative frequency curves for age at onset and age at death figures are presented for use both as prognostic guidelines and as aids in the management of sibs of index cases. Diagnosis, management, and genetic implications are discussed.     

婴儿急性白血病24例分析

目的：探讨婴儿急性白血病(IAL)的临床特征、细胞遗传学及分子生物学特征和治疗情况。方法：对24例IAL患儿临床及实验室检查资料进行分析。结果：ALL 11例,其中L1 6例,L2 5例。ANLL 13例,其中M4 3例,M5 5例。临床表现多样化,髓外浸润及高白细胞性白血病多见,IAL中ALL以CD10 -的早前B细胞型多见。结论：上述特征均提示急性白血病预后不良。     

Acute infantile gastroenteritis caused by rotavirus in Japan

We had an outbreak of acute infantile gastroenteritis accompanied by milky-white stool (called Hakuri in Japanese) during the winters of 1976 and 1977. Stool specimens collected from 72 cases of Hakuri were studied by negative-staining electron microscopy. Rotavirus was detected with a very high frequency (89%).Rotavirus obtained from one of the patients was isolated and passaged in cultures of primary human embryonic kidney cells. Viral antigens could be detected in the cytoplasm of the cells by indirect immuno-fluorescence. The fluorescence-positive cells increased in number with repeated passage.Serum anti-viral activities in 11 patients were titrated by indirect immuno-fluorescence, using the cells infected with the passaged rotavirus. All 11 patients developed IgM responses in the convalescent phase. However, in 4 of the 11 patients, no IgG responses were detected even 2–3 weeks after the onset of illness. The reinfection which has occasionally be seen in our country may be related to these poor IgG responses.     

Acute infantile haemorrhagic oedema associated with hepatitis A

Acute infantile haemorrhagic oedema (AIHO) is characterised by purpura, ecchymosis and inflammatory oedema of the face and extremities. It is seen in children aged 4-24 months. The atiology is not known. We report a case of AIHO diagnosed by skin biopsy demonstrating leukocyte-elastic vasculitis. Laboratory studies showed positive hepatitis A IgM and IgG antibodies. The liver function tests were normal, indicating subclinical hepatitis. Cryoglobulinaemia was detected, suggesting that the disease was related to hepatitis A.