Intracranial hemorrhage in congenital factor II deficiency

A case of congenital defect of factor II is reported. It concerns a newborn with a not traumatic haematoma due to congenital hypoprothrombinaemia, which is rarely described in scientific literature.     

Intracranial hemorrhage: diagnosis and emergency management

Intracranial hemorrhages are an important cause of acute neurologic disease presenting in the emergency setting. To optimize outcome, it is important that the physician quickly recognize intracranial hemorrhages. To minimize mortality and neurologic morbidity, it is often necessary to initiate urgent therapy in the emergency rooms and to obtain neurosurgical consultation in order to pursue early surgical therapy. This article discusses the recognition and early treatment of the various types of intracranial hemorrhages.     

Intracranial hemorrhage following thrombolytic therapy in acute myocardial infarct

The course of the psychopathology and social functioning in an experimental day-treatment group referred for inpatient psychiatric treatment is compared with that of a control group receiving standard inpatient care. During a follow-up period of 2 years subjects were interviewed three times. The interview comprised information about psychiatric symptoms, psychological functions, psychiatric diagnosis and social-role functioning. Apart from these discrete assessments an effort was made to map episodes of illness throughout the follow-up period. Upon entry the groups did not differ in terms of psychopathology or social functioning. At follow-up both groups had improved significantly with respect to symptomatology, psychological and social functioning. The extent to which the groups improved did not differ significantly regarding pathology, but self-care improved more in the experimental group. The average duration of episodes of illness was similar for the experimental and control group. During the 2-year follow-up patients suffered from a well-defined disorder during an average of 11 months. The fact that approximately 40% of them were still a psychiatric case after 2 years further underscores the severity of their pathology.     

Intracranial hemorrhage due to rupture of an arteriovenous malformation in a full-term neonate

A case of neonatal intracerebral hemorrhage (ICH) secondary to rupture of an arteriovenous malformation (AVM) is reported. The baby began to vomit frequently 9 h after birth. Computed tomographic scan revealed the presence of an ICH. Intravenous digital subtraction angiography (IVDSA) demonstrated an AVM, which was successfully excised on the 2nd day after birth. It is stressed that IVDSA is safe and noninvasive in comparison with conventional angiography and is useful for diagnosis of cerebral vascular disease in neonates.     

Standard weight for height curves in achondroplasia

Standard curves developed for the general population cannot be used to assess the growth of an individual who has a condition that results in disproportionate short stature. For this reason, efforts have been made to develop growth curves specific for several of the chondrodysplasias. However, data concerning weight for height have been largely lacking, although they may be of particular importance for conditions such as achondroplasia, where there is some consensus that an increased prevalence of obesity is a particular problem. In this paper we provide standard weight for height curves for males and females with achondroplasia, and discuss the use of several indices which have been applied to the assessment of body fat in the general population.     

Genotype phenotype correlation in achondroplasia and hypochondroplasia

Recent studies of the fibroblast growth factor receptor 3 (FGFR3) gene have established that achondroplasia and hypochondroplasia are allelic disorders of different mutations. To determine whether the genotype could be distinguished on the basis of the phenotype, we analysed height, arm span, and skeletal radiographs from 23 patients with achondroplasia and the G380R mutation of FGFR3 and eight with hypochondroplasia and the N540K mutation. Both conditions share the classical pathological features of micromelic short stature, reduced or unchanged interpedicular distances in the lumbar spine, disproportionately long fibulae, and squared and shortened pelvic ilia. These were significantly more severe in the G380R patients than in the N540K patients. Our findings have shown a firm statistical correlation between the genotype and the phenotype, although there were a few exceptional cases in which there was phenotypic overlap between the two conditions.     

Intracranial hemorrhage associated with idiopathic thrombocytopenic purpura: report of seven patients and a meta-analysis

We present the results of a meta-analysis using clinical data obtained from seven of our patients and 24 previously reported patients with idiopathic thrombocytopenic purpura complicated by intracranial hemorrhage. Twenty-four had an intracerebral hemorrhage (ICH) and seven had a subdural hematoma (SDH). Mean age of the patients with ICH was significantly younger than those with SDH. The mortality rate of ICH associated with ITP was similar to that of spontaneous ICH. All seven patients with SDH improved without sequelae.     

Intracranial hemorrhage observed in the cases of cervical internal carotid artery occlusion associated with moyamoya disease-like vessels]

We report on two peculiar cases of intracranial hemorrhage due to the rupture of moyamoya disease-like vessels associated with unilateral internal carotid occlusion at its origin. The first case is 44-year-old male showing intraventricular hemorrhage associated with right internal carotid occlusion. The second case is 58-year-old female presenting sub-arachnoid hemorrhage associated with left internal carotid occlusion. Although both cases showed the unique appearance of collateral flow resembling moyamoya disease, they were not classified as moyamoya disease nor unilateral Moyamoya-like state. Vascular abnormality such as cerebral aneurysm and arteriovenous malformation were not at all detected. The etiology of hemorrhage for both cases is presumed as the rupture of moyamoya disease-like vessels, however, it is unable for us to determine why the one case showed IVH, yet the other showed SAH. The two cases showed hypoperfusion of ipsilateral cerebral hemisphere on SPECT that followed by the external-internal carotid revascularization surgery in the chronic stage. Post operative study proved the improvement of the CBF reserve. Nevertheless, whether the reconstructive vascularization prevent an occurrence of rebleeding needs the longer observation and the accumulation of the resembling cases.     

Cerebrospinal fluid rhinorrhea in achondroplasia with hydrocephalus (author''s transl)

Intradermal (ID) administration of 0.1 ml of a bivalent influenza vaccine containing 40 CCA units each of influenza A/New Jersey (Hswine 1N1) and A/Victoria (H3N2) virus antigens and of a monovalent vaccine containing 100 CCA units of influenza B/Hong Kong virus to 70 adult volunteers produced no serious reactions and only 7% bothersome side effects. Excluding persons with high (1:64 or greater) initial antibody titers, then 90% and 85% of persons had fourfold or greater rises in HAI antibodies to A/New Jersey and B/Hong Kong antigens, whereas 53% had rises to A/Victoria. The authors feel the ID route deserves further consideration for giving killed influenza vaccines to adults. However, an influenza virus type that was prevalent for many years may fail to give sufficient rise in HAI to consider the patient protected.     

Medical complications of achondroplasia: a multicentre patient review

Achondroplasia is the most prevalent chondrodysplasia and numerous authors have documented the varied social and medical complications that may compromise a full and productive life. Complications include cervicomedullary compression, spinal stenosis, restrictive and obstructive lung disease, otitis media, and tibial bowing, among others. These known complications have led to recommendations for the anticipatory management of such patients. There are relatively few data on the actual rates and timing of these problems. This paper reports data on the rates and age of occurrence of several of these complications based on a review of recorded chart information of 193 patients ascertained from several well established genetic centres with a known interest in the chondrodysplasias. The length of follow up varied and the rates of occurrence at specific age intervals were used to estimate the cumulative percentage affected for each complication. The report includes information on otitis media, ventilation tubes, hearing loss, tonsillectomy, speech problems, tibial bowing and osteotomy, ventricular shunting, apnoea, cervicomedullary decompression, and neurological signs attributable to spinal stenosis.     

Intracranial aneurysms

MR angiography provides a rapid, accurate, and extremely flexible noninvasive evaluation of intracranial aneurysms without the cost and risk of conventional angiography. TOF and phase contrast techniques each have specific advantages and disadvantages that can be selectively exploited to optimize aneurysm evaluation. Present indications for MR angiography in aneurysm evaluation include: (1) the presence of incidental findings on a CT or MR examination that suggest the possibility of aneurysm (Figs. 7 and 8), (2) when angiography is contraindicated or when the risk is too high, (3) non-invasive follow-up of patients with known aneurysms, (4) patient refusal of contrast angiography, and (5) evaluation of patients with specific clinical symptoms (i.e., third cranial nerve palsy) or patients with non-specific subacute symptoms in whom an aneurysm might explain the clinical presentation. Although MR angiography certainly can detect aneurysms with a high rate of sensitivity and specificity, detailed decision analyses generally have not supported the overall benefit of this type of screening. Future technical advances as well as advances in the overall understanding of aneurysms may one day prove unequivocally the benefit of MR angiography in screening high-risk patient groups. MR angiography has not yet been clinically evaluated as a tool in the evaluation of acute subarachnoid hemorrhage. Potential obstacles to such an evaluation include the clinical instability of SAH patients, limited spatial resolution of the MR angiography acquisitions, the potential for subarachnoid blood or focal intraparenchymal hematomas to obscure or mimic small aneurysms, and the unreliability of MR angiography in demonstrating vasospasm. Currently these factors continue to provide an integral role for contrast angiography in aneurysm evaluation.     

Intracranial subependymoma

Subependymomas most commonly appear as a mass in the fourth ventricle of adults; these tumors are rare but must be considered in the differential diagnosis of a lesion arising in this location. We report a case of a fourth ventricule subependymoma.     

Intracranial tuberculoma

One hundred-and-two 13- to 18-day blastocysts were collected from 98 Scottish Blackface ewes mated to one of four New Zealand Romney rams heterozygous for the Massey I (t1) translocation. The karyotype of 75 blastocysts was established, of which 22 were 54,XY, 18 were 54,XX, 20 were 53,XY,t1, and 15 were 53,XX,t1. It is suggested that these results indicated that there was no increase in embryonic loss in ewes mated to rams heterozygous for the Massey I (t1) translocation and that the unbalanced secondary spermatocytes in heterozygous males probably did not mature to spermatozoa.     

Intracranial chondromas

Biochemical and hormonal effects of oral calcium supplementation in premature and asphyxiated neonates during the first few days of life are described. Eight pairs of infants were matched for gestational age and one-minute Apgar score. One member of each pair served as a control and the other was given supplemental oral calcium (75 mg/kg/24 hr) beginning at 12 and ending at 72 hours of age. The supplemental infants had significantly higher serum calcium values both during the time of supplementation and for 36 hours after supplementation was stopped. The oral calcium supplements had no significant effect on serum concentrations of phosphate, magnesium, 25-hydroxy-vitamin D3, or parathyroid hormone, The incidence of hypocalcemia after 12 hours of age was 0 in eight supplemented infants and three in eight control infants. In patients at risk for hypocalcemia, prospective use of oral calcium supplements during the period when there is inadequate calcium intake from feedings may prevent hypocalcemia, appears to be without deleterious effect on measurable chemical and hormonal factors important in calcium homeostasis, and results in maintenance of higher serum calcium levels after supplementation has been discontinued.     

Intracranial hypotension

A CLINICAL DIAGNOSIS: Headache which appears in the upright position and subsides in the reclining position is suggestive of intracranial hypotension Brain magnetic resonance imaging can eliminate an intracranial tumoral process. In most cases, contrast images after gadolinium injection show a diffuse hypersignal of all the meninges including the falx cerebri and the tentorium cerebelli. SPONTANEOUS OR SECONDARY: This clinical presentation is sufficient for diagnosis. Occurring after a recent neurosurgical procedure, the intracranial hypotension is termed secondary. Inversely, is no known cause can be identified, the condition is termed spontaneous. CEREBROSPINAL FLUID: If the clinical manifestations are somewhat doubtful, a spinal tap can reveal the very low pressure of the cerebrospinal fluid (CSF) although it is advisable to avoid further loss of fluid. CSF LEAK: It is generally accepted that intracranial hypotension results from leakages of CSF through a breach in the dura mater caused by untreated trauma to a particularly fragile area due to a meningocele or an arachnoid cyst for example. Occasionally, the CSF leak can be evidenced with isotopic labeling used to visualize the breach and guide therapy. TREATMENT: By analogy with the spinal tap syndrome, a blood-patch with autologous blood is usually proposed as first line medical treatment. Prognosis is generally good.     

Intracranial ependymomas

Clinically significant spinal implants develop in intracranial ependymomas. Spinal subarachnoid implants developed in 11 of 32 patients who received local irradiation at the University of Michigan between 1955 and 1972 for intracranial ependymoma. Seven of these patients received spinal axis irradiation for neurological disturbances resulting from the implants, and implants were found in the other 4 patients postmortem. Ten patients had infratentorial tumors, and one had a supratentorial tumor; nine tumors were poorly differentiated, two were well differentiated. The authors conclude that spinal irradiation is required if the tumor is poorly differentiated, or located in the infratentorial fossa.     

Intracranial pressure in single-suture craniosynostosis

To assess changing patterns of treatment for chronic anal fissure, a retrospective analysis of treatment for chronic anal fissure within one hospital between January 1990 and December 1996 was undertaken. A total of 221 patients received treatment for a chronic anal fissure in this period, of whom 209 had a surgical procedure. Manual dilatation of the anus was performed in 21 patients (10%) and has not been performed since 1995. Lateral internal sphincterotomy was performed in 183 patients (88%) and continues to be the mainstay of treatment. Five female patients (2%) were identified as having a sphincter defect by anal manometry combined with endoanal ultrasound and were treated by an anal advancement flap. From 1996 onwards, 15 patients (7%) were treated by topical glyceryl trinitrate (GTN) paste as the first line of treatment. Of these patients, nine have experienced healing of their fissure, and three have had relief of pain without healing of the fissure. Three have gone on to have a lateral internal sphincterotomy. Lateral internal sphincterotomy remains the primary form of treatment for chronic anal fissure. GTN cream has increasingly been offered as preliminary treatment over the last 12 months. Perioperative use of endoanal ultrasound allowed identification of patients who may be at high risk of postoperative incontinence from a sphincterotomy. An anal advancement flap has been used as an alternative surgical approach for these patients.