Nephrotic syndrome complicated by renal and cerebral infarctions in a 14‐year‐old girl

Venous thrombosis is a well‐known complication of nephrotic syndrome (NS), while arterial thrombosis is rare. We know of no reports of children with this complication. Here we report a case of 14‐year‐old girl with NS, who complicated with renal and cerebral infarctions resulting from arterial thrombosis. Urinary examination showed heavy proteinuria. She had intravascular dehydration. Serum albumin was 0.9 g/dL. Contrast‐enhanced computed tomography (CT) showed a low‐attenuation area in the right kidney. Decreased blood flow in the right middle cerebral artery was observed on MRA and also on multi‐detector‐row head CT. Urokinase and heparin were given. Cerebral infarction was treated neuroprotectively by i.v. infusion of edaravone. Comprehensive assessment of intravascular dehydration and the coagulation–fibrinolysis system is needed to guide decisions concerning prophylactic anticoagulation therapy. Better understanding of NS and its risks, as well as the necessity of drug therapy, may help teenagers to accept and cooperate with treatment.     

Intracerebral malignant peripheral nerve sheath tumor in a child with neurofibromatosis Type 1 and middle cerebral artery aneurysm treated with endovascular coil embolization

Among the neoplastic conditions that affect patients with neurofibromatosis Type 1 (NF1) are malignant peripheral nerve sheath tumors (MPNSTs), which typically arise from peripheral nerves of the limbs, trunk, and lumbar and brachial plexuses. Ionizing radiation is an established risk factor for MPNST development, especially in susceptible patients such as those with NF1. Patients with NF1 are also at risk for intracranial aneurysms, which are increasingly being successfully managed with endovascular therapies. The authors describe the case of a 9-year-old, previously healthy girl who presented in extremis with a right frontal intracerebral hemorrhage resulting from a ruptured right middle cerebral artery (MCA) trifurcation aneurysm. Following urgent decompressive craniectomy, the patient underwent endovascular coil embolization of the MCA aneurysm without complication. Given her mother's history of NF1, the child underwent genetic testing, which disclosed signs positive for NF1. The patient recovered well, but follow-up MR imaging and MR angiography performed at 14 months demonstrated a large frontotemporal mass encasing the right MCA trifurcation. The patient underwent frontotemporal craniotomy and subtotal resection of the mass, which was histologically found to be an intracranial MPNST. The patient received chemotherapy and focal radiation therapy and remains alive at 6 months postresection. To the authors' knowledge, this represents the only known case of intracranial neoplasm arising in the region of an intracranial aneurysm repaired by endovascular coil embolization. While patients with NF1 represent a population with genetic susceptibility to radiation-induced tumors, the pathogenesis of intracerebral MPNSTs remains poorly understood.     

Pseudotumoral acute hemicerebellitis in a child

Acute cerebellitis is an inflammatory disorder usually involving both sides of the cerebellum and presenting with localized signs such as ataxia. Hemicerebellitis is extremely rare in children and may clinically and radiologically resemble a tumor. There are very few reports of hemicerebellitis needing decompressive surgery. We report a case of hemicerebellitis in a 15 year old child presenting with severe headache but no cerebellar symptoms whose brain CT revealed an ill-defined mass compressing the fourth ventricle (pseudotumoral). MRI of the posterior fossa revealed a swollen left cerebellar hemisphere, supra-tentorial hydrocephalus and tonsil herniation, without any cerebral or brain stem lesions. Due to worsening symptoms she required a decompressive craniectomy, and the biopsy revealed an inflammatory process, possibly viral. At follow-up she was asymptomatic and the MRI revealed only slight cerebellar atrophy. This case had an atypical clinical presentation and illustrates that though a self-limited evolution is expected, surgical intervention may be needed in acute cerebellitis.     

Unilateral decompressive craniectomy for children with severe brain injury. Report of seven cases and review of the relevant literature

Severe head injuries in children (under 15 years of age) have many features that differentiate them from head injuries in adults. In such cases, non-surgical treatment cannot always prevent fatal herniation. We report on seven cases of children with severe head injury, presenting with decorticate posturing and treated by unilateral decompressive craniectomy. The aim of the unilateral decompressive craniectomy was to decompress the midbrain and the brainstem. Post-operatively, all patients showed good recovery within 5 weeks (Glasgow Coma Scale score 15). The alternative treatment modalities are discussed critically in an attempt to determine the value of the unilateral decompressive craniectomy procedure and to define the clinical parameters that might identify those children most likely to benefit from this technique. CONCLUSION: the unilateral decompressive craniectomy has an advantage over non-surgical treatment of children with severe brain injury and should be considered in their management.     

Vasculopathy after intracystic bleomycin administration for a recurrent cystic craniopharyngioma: case report

Intracystic bleomycin (ICB) administration is known to be effective in the treatment of cystic craniopharyngiomas (CRPs) and rarely causes serious complications. The authors report a case of vasculopathy after ICB injection for a recurrent cystic CRP. A 5-year-old boy presented with the cystic recurrence of a CRP. A catheter and Ommaya system were inserted into the cyst, followed by a leakage test 2 weeks later. Bleomycin was delivered at 3 mg per week for 14 weeks (total dose 42 mg). Four months later, the patient's activity decreased and right hemiparesis occurred. Magnetic resonance imaging and cerebral angiography demonstrated a recurrent multicystic mass and infarction at the left middle cerebral artery territory with stenosis/occlusion of the left distal internal carotid artery and posterior cerebral artery. During the operation to remove the recurrent cystic mass, fibrotic stenoocclusion of the left intracranial arteries was identified. The cyst was totally removed. However, additional multiple border zone infarctions occurred in the left hemisphere, and the patient became wheelchair bound. Leakage of ICB was considered as the most probable cause of the cerebral vasculopathy and corresponding infarction. A leakage test may not always guarantee a leakage-free status even if the test is negative. Direct bypass surgery should have been considered to prevent a high risk of postoperative infarction in this case of a recurrent CRP with confirmed vasculopathy.     

Perinatal cortical infarction within middle cerebral artery trunks

AIM: To define neonatal pial middle cerebral artery infarction. METHODS: A retrospective study was made of neonates in whom focal arterial infarction had been detected ultrasonographically. A detailed study was made of cortical middle cerebral artery infarction subtypes. RESULTS: Forty infarctions, with the exception of those in a posterior cerebral artery, were detected ultrasonographically over a period of 10 years. Most were confirmed by computed tomography or magnetic resonance imaging. Factor V Leiden heterozygosity was documented in three. The onset was probably antepartum in three, and associated with fetal distress before labour in one. There were 19 cases of cortical middle cerebral artery stroke. The truncal type (n=13) was more common than complete (n = 5) middle cerebral artery infarction. Of six infarcts in the anterior trunk, four were in term infants and five affected the right hemisphere. Clinical seizures were part of the anterior truncal presentation in three. One of these infants, with involvement of the primary motor area, developed a severe motor hemisyndrome. The Bayley Mental Developmental Index was above 80 in all of three infants tested with anterior truncal infarction. Of seven patients with posterior truncal infarction, six were at or near term. Six of these lesions were left sided. Clinical seizures were observed in three. A mild motor hemisyndrome developed in at least three of these infants due to involvement of parieto-temporal non-primary cortex. CONCLUSIONS: Inability to differentiate between truncal and complete middle cerebral artery stroke is one of the explanations for the reported different outcomes. Severe motor hemisyndrome can be predicted from neonatal ultrasonography on the basis of primary motor cortex involvement. Clinical seizures were recognised in less than half of the patients with truncal infarction; left sided presentation was present in the posterior, but not the anterior truncal type of infarction. Asphyxia is a rare cause of focal arterial infarction.     

Brain and arterial abnormalities following prenatal X‐ray irradiation in mice assessed by magnetic resonance imaging and angiography

The present study aimed to quantitatively characterize changes in the whole brain and arterial morphology in response to prenatal ionizing irradiation. Magnetic resonance imaging (MRI) and angiography (MRA) were used to evaluate brain and arterial abnormalities in 8‐week‐old male mice prenatally exposed to X‐ray radiation at a dose of 0.5 or 1.0 Gy on embryonic day (E) 13. Irradiated mice demonstrated decreased brain volume, increased ventricular volume, and arterial malformation. Additionally, MRA signal intensity and arterial thickness in the anterior cerebral artery, middle cerebral artery, and basilar artery were lower in radiation‐exposed mice than in control mice. MRI and MRA are useful tools for assessing brain and arterial abnormalities after prenatal exposure to radiation.     

Does cranial ultrasound imaging identify arterial cerebral infarction in term neonates?

OBJECTIVE: To evaluate the diagnostic accuracy of cranial ultrasound (CUS) for detection of neonatal arterial territory cerebral infarction in term infants. METHODS: CUS scans from term infants with neonatal magnetic resonance imaging (MRI) evidence of neonatal infarction were reviewed. The scans were grouped by acquisition time after birth: 1-3 days (early) or 4-14 days (late). RESULTS: Brain MRI showed infarction in the territory of the middle cerebral artery in 43 of 47 infants, anterior cerebral artery in one, and posterior cerebral artery in three. Twelve of the 47 had minor changes on MRI in the white matter in the contralateral hemisphere, and four infants had bilateral infarctions. The early CUS scans were abnormal in 68% of the infants; the late CUS scans were abnormal in 87%. The late CUS scans were correct for laterality and site of lesion in 25/47 (53%) infants. In six infants with smaller lesions of the cortical middle cerebral artery branch or lesions in the posterior cerebral artery territory, the CUS scans were persistently normal. CONCLUSION: Normal early CUS scans do not exclude a diagnosis of neonatal stroke, although most scans are abnormal. CUS scans performed after day 3 were abnormal in 87% of infants. CUS scan findings were accurate for lesion laterality and site in 53%, and, in 34%, the scans showed abnormality strongly suggestive of infarction but not always site specific. For optimal prognostic information, infants with clinical histories or CUS scan findings suggestive of infarction should have a neonatal brain MRI scan.     

新生婴儿脑动脉梗塞的B超、CT和MRI表现

目的　探讨新生儿及小婴儿脑动脉梗塞的B超、CT和MRI影像诊断特点。方法　1998年 12月至2000年 2月期间,对入住上海新华医院新生儿病区的新生儿及小婴儿同时进行头颅B超、CT和MRI检查,并酌情复查。结果　5例足月小婴儿被诊断为脑动脉梗塞,其中重度窒息 2例,轻度窒息 1例,均在新生儿期后 0. 5~3个月因惊厥收住院。脑动脉梗塞部位涉及大脑前、中、后动脉所分布区域,以左侧中动脉梗塞较为常见。B超可清晰诊断脑动脉梗塞的早期(强回声)和晚期(低回声),但未能诊断大脑后动脉梗塞;CT和MRI对整体颅脑病变的显示优于B超,但对脑动脉梗塞的早、晚期则均呈现相同的低密度或T1 低信号。结论　B超、CT和MRI均可诊断脑动脉梗塞,其中B超可床边检查,对颅脑中央部位的病变分辨力高,应为新生儿颅内病变的首选影像诊断方法。     

新生儿出血性及梗死性脑血管病诊治探讨

目的：提高对新生儿脑血管病的认识,探讨其临床发病特点及诊断方法。方法：选用不同的影像学方法对9例由于脑血管畸形所致的新生儿脑实质出血, 17例不同部位脑梗死患儿进行了检查、确诊,并依据神经系统症状,分析临床发病特点。结果：9例脑实质出血发生于额叶、颞叶和枕叶,其中7例在生后72h内发病; 10例因脑血管发育异常致大脑前动脉、中动脉、后动脉供血区梗死; 7例继发于不同疾病,为分支血管供血障碍所致的继发性脑梗死。典型的临床特征是频繁惊厥。结论：新生儿脑血管病最显著的临床症状是频繁惊厥,影像学检查为确诊提供了科学依据,早期治疗对预后有改善作用。     

Supratentorial arterial ischemic stroke following cerebellar tumor resection in two children

We describe two children who developed ischemic strokes in the territory of the middle cerebral artery, one 7 days and one 11 days after resection of a cerebellar tumor. In the first child, another infarction occurred in the territory of the contralateral middle cerebral artery 5 days after the first stroke. No specific cause or underlying risk factor other than the surgical procedure was found. The subacute clinical course at stroke onset resembled that of the 'posterior fossa syndrome', suggesting a common underlying mechanism.     

Spastic hemiparesis and presumed prenatal embolisation.

An infant with clinical evidence of prenatal right subclavian arterial occlusion subsequently developed left hemiparesis with cystic infarction of the territory of the right middle cerebral artery. Perinatal strokes have not been reported with signs of limb ischaemia at birth; this finding may be a warning of possible simultaneous cerebral infarction.     

Neonatal focal seizures and transitory inappropriate ADH secretion syndrome due to an infarction in the region of the middle cerebral artery]

Cerebral arterial infarction is a very rare condition during the neonatal period. In recent times, cerebral infarctions with an atypical bland course are reported in healthy, unsuspected newborns. For the first time, we saw focal seizures and a transient syndrome of inappropriate ADH secretion in a full-term, male newborn with an infarction of the middle cerebral artery as the first sign of this disorder. On the basis of these rare clinical manifestations, the literature about neonatal cerebral infarction is reviewed. The diagnostic possibilities and prognostic expectations are discussed. It is the aim of this case report to call attention to this rare disease in order to avoid a delay in diagnosis and therapy, even if the initial manifestations are atypical.     

Open-wound treatment for gunshot to the brain

The authors report a case of a gunshot wound to the brain in a 2.5-year-old girl. To treat the uncontrollably elevated intracranial pressure, the patient underwent bilateral decompressive craniectomy and experimental open-wound treatment. She recovered to a good functional level.     

A study of cerebral perfusion using single photon emission computed tomography in neonates with brain lesions

In this study we used a single photon emission computed tomography technique (SPECT) with radiolabelled ^99mTc HMPAO to assess cerebral perfusion in newborn infants with documented cerebral lesions and to determine to what extent brain SPECT might be useful in the neonatal period. A total of 15 newborn infants with the following cerebral pathologies were enrolled: severe parietal bilateral periventricular leucomalacia (PVL, n = 6); moderate parietal bilateral PVL ( n = 2); intraventricular haemorrhage grade II with unilateral parietal parenchymal extension (IHV + PE, n = 3); cerebral infarction (CI, n = 2) in the zone of middle cerebral artery; and post-haemorrhagic hydrocephalus ( n = 2). Follow-up was available in all infants. Alterations in cerebral perfusion were seen in only 12 of 15 infants and at the location of severe PVL, PE and CI. We have noted that the regions of diminished perfusion extended, beyond the apparent extent of cerebral pathology delineated by ultrasound or magnetic resonance imaging. Markedly diminished perfusion was seen in 1 infant with hydrocephalus, which recovered following placement of ventriculo-peritoneal shunt. Regarding outcome, SPECT data failed to provide additional information than that of neuroradiological investigations. We conclude that the use of SPECT, under these conditions, to assess alteration of cerebral perfusion in the neonatal period will not provide any additional information than that of neuroradiological investigations.     

Fungal intracranial aneurysm in a child with familial chronic mucocutaneous candidiasis

We report on a patient who presented at 5 years of age with a hemiparesis due to a middle cerebral artery infarction. An embolism had originated from a mycotic aneurysm located in the internal carotid artery. For several months prior to admission he had been suffering from therapeutically resistant candidiasis of the mouth and nails. Family history revealed chronic mycotic infections of the skin, hair, nails and mouth in the father and paternal grandmother suggestive of chronic mucocutaneous candidiasis with autosomal dominant mode of inheritance. Clipping of the aneurysm, after 3 months of anti-mycotic treatment, followed by sustained treatment with itraconazole and fluconazole, led to a favourable outcome. Conclusion Chronic mucocutaneous candidiasis can be associated with an intracranial aneurysm and complicated by cerebral infarction. Received: 8 October 1998 / Accepted in revised form: 11 February 1999     

Ischemia of the internal capsule due to mild head injury in a child

We encountered an instructive case of repetitive reversible severe neurological deficit due to ischemia of the internal capsule after mild head injury. A 1-year-old boy fell and hit his head on the floor without losing consciousness. Intermittent episodes of left hemiparesis lasting from 30 s to 30 min developed 4 h later. Magnetic resonance (MR) imaging revealed acute infarction in the left internal capsule and corona radiata on diffusion-weighted imaging, and no microbleeding on susceptibility-weighted imaging. MR angiography of the intracranial and cervical vessels showed no obstruction of the large cerebral arteries. Motor impairment began to improve the next day with conservative therapy. Neurological deficit gradually resolved over the course of 1 month. This tiny lesion of the internal capsule and corona radiata may have represented a small infarction caused by mechanical vasospasm of the perforating vessels branching from the middle cerebral artery after minor injury. Even mild head injuries may cause infarction of the internal capsule, although minor head injuries are common accidents in childhood and usually do not result in severe complications.     

Malignant hypertension and cerebral haemorrhage in Seckel syndrome

Seckel syndrome is an autosomal recessive condition with severe short stature and facial and neurological anomalies. Intracranial haemorrhage, due to rupture of a cerebral aneurysm, is a very rare complication of this syndrome. Malignant hypertension may play an important role in the aetiology of the aneurysm and early detection is essential in order to prevent organ damage. Conclusion:we report a new case of Seckel syndrome associated with malignant hypertension and cerebral haemorrhage.Abbreviations BA basilar artery - MCA middle cerebral artery - PICA posterior internal cerebral artery - PICU paediatric intensive care unit - SS Seckel syndrome     

Multiple Idiopathic Emboli in a Full Term Neonate

ABSTRACT. We report a case of multiple idiopathic emboli leading to cerebral infarction and to limb ischaemia in a full term neonate. The increased frequency of diagnosis of cerebral infarction is highlighted. Treatment of cerebral and limb emboli is discussed and the need for further research into the causes and treatment of cerebral infarction is stressed.     

Neonatal cerebral infarction and visual function at school age

OBJECTIVE: To assess various aspects of visual function at school age in children with neonatal cerebral infarction. PATIENTS AND METHODS: Sixteen children born at term, who had cerebral infarction of perinatal onset on neonatal magnetic resonance imaging (MRI) were assessed using a battery of visual tests. This included measures of crowding acuity (Cambridge Crowding Cards), stereopsis (TNO test), and visual fields. The results of the visual assessment were compared with the type and the extent of the lesion observed on neonatal MRI. RESULTS: Only six of the 16 children (28%) had some abnormalities of visual function on these tests. Visual abnormalities were more common in children with more extensive lesions involving the main branch of the middle cerebral artery and were less often associated with lesions in the territory of one of the cortical branches of the middle cerebral artery. The presence of visual abnormalities was not always associated with the involvement of optic radiations or occipital primary visual cortex. Abnormal visual fields were only found in children who also developed hemiplegia. CONCLUSIONS: Abnormality of visual function is not common in children who had neonatal infarction and, when present, tends to be associated with hemiplegia and more extensive lesions.