Moderate Hydrocephalus Research Articles

Resolution of Tonsillar Herniation and Cervical Syringomyelia Following Resection of a Large Petrous Meningioma: Case Report and Review of Literature

We describe a case of a large, petrous meningioma associated with tonsillar herniation and cervical syringomyelia. The patient, a 53-year-old woman, had a 6-month history of a dull, aching pain in the occipital region associated with numbness in the right C2 dermatome and left gaze evoked nystagmus. Magnetic resonance imaging (MRI) revealed a large tumor in the right posterior fossa associated with moderate supratentorial hydrocephalus. Secondary tonsillar herniation and cervical syringomyelia extending from C2 to C6 were also identified. The tumor, later confirmed to be a meningioma originating from the petrous region, was resected completely via a retrosigmoid approach. Postoperative MRI demonstrated total resolution of the tonsillar herniation and cervical syringomyelia. The radiological features, potential pathophysiological mechanisms, and treatment strategies are discussed in relation to the recent literature.

Tumor-third ventricular relationships in supradiaphragmatic craniopharyngiomas: correlation of morphological, magnetic resonance imaging, and operative findings.

To disclose the relationships of primarily supradiaphragmatic craniopharyngiomas with the third ventricular floor (3rdVF) by means of preoperative magnetic resonance imaging (MRI) and thus to select the surgical approach avoiding the hypothalamic structures. MRI findings in 76 consecutive patients with craniopharyngiomas operated on between June 1991 and December 2002 were interpreted on the basis of the results of the authors' own previous microanatomic studies. The assumed tumor-3rdVF relationships were then correlated with the operative findings. MRI features characteristic for different topographical relationships were analyzed in 44 patients (18 children, 26 adults) with exclusively supradiaphragmatic tumors. In 14 of 15 patients with the tumor located below the 3rdVF (suprasellar extraventricular craniopharyngioma), the anterior communicating artery was displaced upward and indirectly indicated the position of the chiasm between the prechiasmatic and the retrochiasmatic tumor portions. Hydrocephalus was absent in 14 patients, including those with giant tumors. The anterior part of the third ventricular cavity was found in front of the level of the foramina of Monro in 6 patients. All 28 tumors growing partially inside and partially outside the third ventricular cavity (intraventricular and extraventricular craniopharyngioma) were retrochiasmatic. They caused severe or moderate hydrocephalus in 20 patients and mild hydrocephalus in 2. One purely intraventricular tumor caused severe hydrocephalus. The position of the optic chiasm and the size of the lateral ventricles on preoperative MRI enable us to determine the position of the 3rdVF or its remnants in relation to the supradiaphragmatic craniopharyngiomas and to select the proper surgical approach allowing exposure of the tumor while avoiding the hypothalamic structures.

Computed Tomographic Parameters Predicting Fatal Outcome in Large Middle Cerebral Artery Infarction

Background: Large middle cerebral artery (MCA) ischaemic stroke when associated with extensive mass effect can result in brain herniation and neurological death. As yet there are few guidelines to aid the selection of patients for aggressive interventional therapies, such as decompression hemicraniectomy and/or hypothermia. Methods: We studied a cohort of patients from seven centres with large MCA infarction requiring neurocritical care. The purpose of this analysis was to assess the use of early radiological signs on follow-up computed tomographic (CT) signs performed within 48 h of stroke onset for predicting mortality at 30 days. The CT parameters assessed included horizontal displacement of the septum pellucidum, pineal shift, complete or partial infarction of the temporal lobe, involvement of additional vascular territories, and the presence of hydrocephalus. The primary outcome measure was in-hospital death within 30 days. Results: One hundred and thirty-five patients who had follow-up CT scans within 48 h were identified from a total of 201 patients with large MCA infarction that received conventional medical therapy alone. The median age was 68 (range 29–99), 56% were female, and the median NIHSS category was 26–30 at 48 h. Among CT variables in univariable analysis, anteroseptal shift ≧5 mm, pineal shift ≧2 mm, complete temporal lobe infarction, involvement beyond the MCA territory, and moderate or severe hydrocephalus were equally predictive of death. Multivariable analysis adjusting for time to CT scan revealed the following predictors of fatal outcome: anteroseptal shift ≧5 mm (OR 10.9; 95% CI 3.2–37.6), NIHSS within 48 h >20 (OR 6.6; 95% CI 2.3–19.3), and infarction beyond the MCA territory (OR 4.9; 95% CI 1.6–15.0). Conclusions: We identified the role of early CT signs in predicting death following massive MCA infarction. The CT parameters anteroseptal shift (>5 versus ≤5 mm), pineal shift ≧2 mm, hydrocephalus, temporal lobe infarction, and other vascular territory infarction if present were predictive of fatal outcome. These CT parameters require prospective validation before they should be considered reliable markers for decision-making.

Amifostine Protects Against Cisplatin-Induced Nephrotoxicity in a Child with Medulloblastoma

Amifostine (WR-2721, Ethyol) (S-2-(3-aminopropylamino)-ethylphosphorothioic acid) protects normal tissue from the cytotoxic damage induced by radiation and by chemotherapy. Several studies in adults have shown that amifostine reduces the cumulative renal toxicities associated with cisplatin, with no reduction in its anti-tumor efficacy [1, 2]. In the pediatric age group, amifostine has also been shown to be well tolerated and to ameliorate the myelotoxicity from alkylating agents and from cisplatin, in a variety of solid tumors [3, 4]. We describe the first reported case of the use of amifostine to protect against cisplatin-induced nephrotoxicity in a child with medulloblastoma who had a solitary kidney. A 7-year-old boy was referred with complaints of headache, vomiting, and diplopia. Magnetic resonance imaging (MRI) scan of the brain showed an enhancing tumor 3 cm in diameter located in the cerebellar vermis, invading the right cerebellar hemisphere, and partially filling the fourth ventricle, with moderate internal hydrocephalus. MRI scan of the spine demonstrated gross nodular seedlings in the thoraco-lumbar subarachnoid space. The tumor was staged as T2M3 according to the classification by Chang et al. [5]. The patient underwent posterior fossa decompression and a partial resection of the tumor mass with less than 50% residual disease demonstrated postoperatively. Histopathological analysis confirmed the diagnosis of medulloblastoma. Within 28 days of surgery, he began radiation therapy and received 3780 cGy to the craniospinal axis with a 1980-cGy boost to the local tumor site (a total radiation dose to the tumor site of 5760 cGy). Daily fractions of

Timing of Shunt Surgery in Childhood Tuberculous Meningitis with Hydrocephalus

Hydrocephalus is a common complication of tuberculous meningitis (TBM) in children. The aims of this study are to review our experience of hydrocephalus in childhood TBM and to evaluate the effect of the timing of ventriculoperitoneal shunting (VPS) on the final outcome. In this study, 156 patients with TBM and hydrocephalus were reviewed retrospectively between 1990 and 2000. Patients’ ages ranged from 6 months to 15 years, with a mean age of 4.1 years. There were 85 boys, and the male-to-female ratio was 1.19:1.0. Sixty-two percent of the children were younger than 6 years old. VPS was performed 2 days after the diagnosis in 100 patients, and in the remaining 56 patients, 3 weeks after the diagnosis. The average follow-up period was 8.5 months. Good recovery or minor sequelae was seen in 82 patients (52.6%), and 51 died (12.3%). The timing of the VPS procedure and cerebral complications had an effect on the final outcome. Early VPS gave a better outcome in mild and moderate hydrocephalus (p = 0.040). This study has shown that early surgical procedure for mild/moderate hydrocephalus has a positive effect on the morbidity and mortality of hydrocephalus in childhood TBM (p = 0.014, p = 0.040, respectively). In severe hydrocephalus, there was a tendency for early shunting to have a positive effect on morbidity, although this did not reach statistical significance.

Post-traumatic intradiploic leptomeningeal fistula and cyst

A 59 year old female patient presented with ataxia and difficulty in walking. The neurological examination revealed right homonymous hemianopia and ataxia. Radiographic evaluation revealed a large occipital intradiploic cyst mainly in the left suboccipital area. There was also moderate hydrocephalus and encephalomalacia of the left occipital pole. Bone window studies also demonstrated a growing fracture extending from the upper pole of the cyst to the vertex. Both pathologies were attributed to child abuse the patient suffered when she was a child. At first surgery, decompression of the cerebellum was followed by duroplasty and acrylic cranioplasty to the posterior cranial fossa. A month later, a shunt had to be inserted for hydrocephalus. At 7 months postoperatively, the patient is well and free of any symptoms or recurrence.

A programmed ependymal denudation precedes congenital hydrocephalus in the hyh mutant mouse.

Hydrocephalic hyh mice are born with moderate hydrocephalus and a normal cerebral aqueduct. At about the fifth postnatal day the aqueduct becomes obliterated and severe hydrocephalus develops. The aim of the present investigation was to investigate the mechanism of this hydrocephalus, probably starting during fetal life when the cerebral aqueduct is still patent. By use of immunocytochemistry and scanning electron microscopy, mutant (n = 54) and normal (n = 61) hyh mouse embryos were studied at various developmental stages to trace the earliest microscopic changes occurring in the brains of embryos becoming hydrocephalic. The primary defect begins at an early developmental stage (E-12) and involves cells lining the brain cavities, which detach following a well-defined temporo-spatial pattern. This ependymal denudation mostly involves the ependyma of the basal plate derivatives. There is a relationship between ependymal denudation and ependymal differentiation evaluated by the expression of vimentin and glial fibrillary acidic protein. The ependymal cells had a normal appearance before and after detachment, suggesting that their separation from the ventricular wall might be due to abnormalities in cell adhesion molecules. The process of detachment of the ventral ependyma, clearly visualized under scanning electron microscope, is almost completed before the onset of hydrocephalus. Furthermore, this ependymal denudation does not lead to aqueductal stenosis during prenatal life. Thus, the rather massive ependymal denudation appears to be the trigger of hydrocephalus in this mutant mouse, raising the question about the mechanism responsible for this hydrocephalus. It seems likely that an uncontrolled bulk flow of brain fluid through the extended areas devoid of ependyma may be responsible for the hydrocephalus developed by the hyh mutant embryos. The defect in these embryos also includes loss of the hindbrain floor plate and a delayed in the expression of Reissner fiber glycoproteins by the subcommissural organ.

Neuron tolerance during hydrocephalus

Whether or not neuron death plays a major role in pathophysiology during hydrocephalus is not well known. The goals of this study were to determine if neural degeneration occurred during hydrocephalus, and to determine if neuron tolerance developed during this pathophysiologic procedure. Neural damage as visualized by a sensitive staining technique, silver impregnation, was observed in three experimental groups: (1) adult hydrocephalic rats induced by kaolin injection into the cisterna magna, (2) adult rats with chronic hydrocephalus for 10 weeks subjected to acute forebrain ischemia induced by four-vessel occlusion, and (3) adult rats without hydrocephalus subjected to acute forebrain ischemia. The magnitude of hydrocephalus was also evaluated during this time. In mild or moderate hydrocephalus, little cell death was found. In severe hydrocephalus, axon and neuropil degeneration was extensively distributed, but cell death was still rarely observed. Although some neuron degeneration was found after acute forebrain ischemia in hydrocephalic rats, the extensive cell death in cortical layers III and V, and in hippocampal areas CA1 and CA4 that is commonly observed in the ischemic brain without hydrocephalus, was not seen. This study suggests that neuron death was not a major pathological change in the brain during hydrocephalus, with cerebral ventricles being enlarged during the development of hydrocephalus. Less neuron death in hydrocephalic rats after acute forebrain ischemia suggests that neuronal tolerance to ischemia occurs during hydrocephalus.

Axonal damage associated with enlargement of ventricles during hydrocephalus: A silver impregnation study

Motor and cognitive deficits are commonly associated with hydrocephalus. Although the mechanisms responsible for these impairments have not been confirmed, neuronal cell death and axon degeneration may play an important role, and have long lasting consequences on neuronal connectivity. The goal of this study was to determine if neural degeneration occurred during hydrocephalus in structures anatomically related to cognitive motor functioning, namely, the sensorimotor cortex, neostriatum, hippocampus and corpus callosum. Neural damage, as visualized by silver staining, was examined in adult rats 2–10 weeks after obstructive hydrocephalus was induced by kaolin injection into the cisterna magna. In mild or moderate hydrocephalus, mostly occurring 2–6 weeks after kaolin injections, silver-labeled axons were scattered in the white matter of the sensorimotor cortex, corpus callosum, neostriatum, and hippocampus. In severe hydrocephalus, 10 weeks after kaolin injections, axon degeneration was more extensive in these areas, as well as in layers IV through VI of the sensorimotor cortex. Axons in the subiculum and the fimbria were heavily labeled, suggesting damage to hippocampal afferent and efferent fibers. In contrast, neuron cell death was rarely observed at any stage of hydrocephalus. The major pathological change of brain regions involved in motor and learning functions during hydrocephalus is axon degeneration, and this degeneration is correlated with an enlargement of the cerebral ventricles. [Neurol Res 2001; 23: 581-587]

A temporo-spatial programmed ependymal denudation leads to hydrocephalus in the hyh mutant mouse

Hyh is a lethal autosomal recessive mouse mutation located in chromosome 7. Newborn homozygous mutants presented moderate hydrocephalus, and then a severe hydrocephalus develops during the first postnatal days (Perez-Figares et al, 1998). Mutant embryos showed a well-defined pattern of ependymal cell denudation in floor and in basal plate derivatives and later in alar plate derivatives. A relationship between ependymal denudation and degree of ependymal differentiation can be observed. Ependymal denudation precedes hydrocephalus; an increased bulk flow of brain fluid may be causing the hydrocephalus developed in these embryos. The floor plate of hindbrain detached whereas that of the spinal cord did not, further supporting the functional zonation of the floor plate.

Subcommissural organ, cerebrospinal fluid circulation, and hydrocephalus.

Under normal physiological conditions the cerebrospinal fluid (CSF) is secreted continuously, although this secretion undergoes circadian variations. Mechanisms operating at the vascular side of the choroidal cells involve a sympathetic and a cholinergic innervation, with the former inhibiting and the latter stimulating CSF secretion. There are also regulatory mechanisms operating at the ventricular side of the choroidal cells, where receptors for monoamines such as dopamine, serotonin, and melatonin, and for neuropeptides such as vasopressin, atrial natriuretic hormone, and angiotensin II, have been identified. These compounds, that are normally present in the CSF, participate in the regulation of CSF secretion. Although the mechanisms responsible for the CSF circulation are not fully understood, several factors are known to play a role. There is evidence that the subcommissural organ (SCO)--Reissner's fiber (RF) complex is one of the factors involved in the CSF circulation. In mammals, the predominant route of escape of CSF into blood is through the arachnoid villi. In lower vertebrates, the dilatation of the distal end of the central canal, known as terminal ventricle or ampulla caudalis, represents the main site of CSF escape into blood. Both the function and the ultrastructural arrangement of the ampulla caudalis suggest that it may be the ancestor structure of the mammalian arachnoid villi. RF-glycoproteins reaching the ampulla caudalis might play a role in the formation and maintenance of the route communicating the CSF and blood compartments. The SCO-RF complex may participate, under physiological conditions, in the circulation and reabsorption of CSF. Under pathological conditions, the SCO appears to be involved in the pathogeneses of congenital hydrocephalus. Changes in the SCO have been described in all species developing congenital hydrocephalus. In these reports, the important question whether the changes occurring in the SCO precede hydrocephalus, or are a consequence of the hydrocephalic state, has not been clarified. Recently, evidence has been obtained indicating that a primary defect of the SCO-RF complex may lead to hydrocephalus. Thus, a primary and selective immunoneutralization of the SCO-RF complex during the fetal and early postnatal life leads to absence of RF, aqueductal stenosis, increased CSF concentration of monoamines, and a moderate but sustained hydrocephalus.

Endoscopic management of colloid cysts.

Several surgical approaches have been proposed for the treatment of colloid cysts, which still remains controversial. The most recent technique used is endoscopy. By its nature, endoscopy cannot offer complete removal, as compared to microsurgical techniques, but can do more than puncture. To evaluate the usefulness of endoscopy for colloid cyst surgery, a series of 15 patients who were operated on for colloid cysts under endoscopic control since 1994 was reviewed. The presenting symptoms of our patients (10 men and 5 women) were intermittent headache (10 patients), nausea (3 patients), short-term memory loss (4 patients), coma (2 patients), gait disturbance (3 patients), blurred vision (2 patients), and mental status changes (3 patients). The sizes of the cysts ranged from 4 to 50 mm (median, 22.93 mm). Depending on the radiological appearance, the procedure was performed via a right (10 patients) or left (5 patients) precoronal burr hole. A rigid neuroendoscope was used. Initial stereotactic placement of the neuroendoscope was used in two patients who had moderate hydrocephalus. In the other patients, hand-guided endoscopy was performed using an articulated arm. The cysts were perforated with a needle. The opening was enlarged with microscissors. The cyst material was aspirated, and the remaining capsule was coagulated. The average follow-up was 15.26 months (range, 1-28 mo). Total aspiration of the cysts was achieved in 12 patients, as revealed by normal postoperative magnetic resonance imaging. Control magnetic resonance imaging revealed residual cysts in three patients. One patient presented with an asymptomatic recurrence at 1 year. Resolution of the symptoms was obtained in all patients except for two of the four patients with preoperative memory deficit (improvement without complete recovery). There was no mortality or morbidity. These results show that endoscopy is a safe and promising percutaneous technique for the treatment of colloid cysts of the third ventricle. Longer follow-up is, however, still required.

MR imaging of symptomatic neurocutaneous melanosis in children.

Neurocutaneous melanosis is a syndrome consisting of cutaneous nevi and melanocytosis of the leptomeninges. Over a 5-year period (1989-1994) we evaluated with MR imaging the central nervous system of five children with a confirmed histologic diagnosis of neurocutaneous melanosis. The children ranged in age from 7 to 10 years and consisted of two girls and three boys. They all had multiple pigmented skin lesions (cutaneous nevi) and presented with seizures, signs of raised intracranial pressure, cranial nerve palsies and/or myelopathy. The MR studies were performed with T1-weighted, T2-weighted and T1-weighted post-gadolinium images of the brain in addition to T1-weighted post-gadolinium images of the entire spine. The MR findings in all the children consisted of marked, diffuse enhancement of thickened leptomeninges surrounding the brain and spinal cord which was only demonstrated on the post-gadolinium T1-weighted images and mild to moderate hydrocephalus. We present our MR findings and compare these findings with other imaging findings in the literature. Our findings represent part of a spectrum of imaging abnormalities seen in patients with neurocutaneous melanosis.

An Association Between Fetal Parvovirus B19 Infection and Fetal Anomalies: A Report of Two Cases

The association between fetal parvovirus B19 infection and hydrops was first reported in 1984. The virus has a predilection for the erythroid cell line, which in the fetus may produce anemia. Recent cases of parvovirus infection in other fetal cell lines have raised concern that the infection may induce fetal anomalies in rare cases. We report two pregnancies complicated by parvovirus B19 infection. In each instance the patient had normal second trimester ultrasounds but subsequently developed fetal abnormalities--disruptions of normal structure. One infant has myocardial infarction, splenic calcifications, and mild hydrocephalus. The other had moderate hydrocephalus with central nervous system scarring. There are two possible mechanisms in which parvovirus may induce fetal anomalies. Both direct infection of fetal organs and vascular inflammation have been documented in association with B19 parvovirus. Although fetal abnormalities associated with parvovirus are rare, continued study of this organism may indicate a greater pathologic potential than is now thought.

Infantile hydrocephalus: management using CT assessment

When determining a management plan for infantile hydrocephalus, the determining factor for or against the implantation of a shunt is the degree of ventricular dilatation. The author has devised a standardised method of estimating this, the use of which has been shown to achieve consistently successful results. Dilatation was determined using the ventricular/biparietal (V/BP) ratio from the axial CT scan at the mid-portion of the bodies of the lateral ventricles, showing the greatest ventricular dilatation. According to this method, hydrocephalus was classified into four grades. These were mild (V/BP ratio 0.26-0.40), moderate (V/BP ratio 0.41-0.60), severe (V/BP ratio 0.61-0.90) and extreme (V/BP ratio 0.91-1). A V/BP ratio of less than 0.26 was considered normal. This method appeared to be accurate and reproducible in infants with hydrocephalus including those with asymmetrical and multiloculated ventricular dilatation. In all the patients with mild hydrocephalus, spontaneous regression or stabilisation occurred and their developmental outcome was normal. Patients with moderate and severe hydrocephalus needed a ventricular shunt and the developmental outcome was satisfactory in 87% of the cases. They were functionally normal although 18 had some abnormal neurological signs. In patients with extreme hydrocephalus the developmental outcome following shunting was satisfactory in 31% of the cases. They were functionally normal although four had abnormal neurological signs. This plan of management was used in a total of 144 infants and it proved to be highly successful.(ABSTRACT TRUNCATED AT 250 WORDS)

Androctonus crassicauda (Olivier), a dangerous and unduly neglected scorpion—I. Pharmacological and clinical studies

Androctonus crassicauda venom has an i.v. LD50 in mice of 0.32±0.02 mg/kg, which makes the scorpion among the most toxic species in the world. Fifty-one non-fatal and one fatal cases of scorpion sting were presented. Pain and tenderness were very common following the sting. Generalized erythema occurred in 20–25% of all infants and children below the age of 5 years. Severe CNS manifestations including seizures, unconsciousness and marked irritability occurred mainly in infants and young children, while hypertension occurred in the majority of victims below the age of 11 years. Two pregnant victims were treated with antivenom with no bad consequences on mothers or foetuses. The fatal case described was inadequately treated with antivenom and presented a rare situation of intracranial coagulation in the basal cisterns or low in the cranial subarachnoid space. The victim developed moderate hydrocephalus of the communicating type with clear ventricular CSF and strongly xanthocromic fluid from lumbar puncture. The effects of A. crassicauda venom on isolated hearts, atria and anaesthetized rat blood pressure appeared to be mediated largely through stimulation of the autonomic nervous system with predominance of sympathetic stimulation and release of tissue catecholamines. Electrocardiograms recorded simultaneously with blood pressure changes showed evidence of ectopic foci during the hypertensive phase and ischaemia, inferior wall infarction and different degrees of heart block during the late hypotensive phase. Androctonus crassicauda venom was unique in following a three-compartment open model comprising a central compartment ‘blood’, a rapidly equilibrating ‘shallow’ tissue compartment and a slowly equilibrating ‘deep’ tissue compartment. The overall elimination half-life, t12β, was 24 hr, indicating that the venom has the slowest elimination among all known scorpion venoms. The long stay of the venom in the body might explain the increased risk of toxicity and the good potential for treatment with serotherapy even hours after the sting.

Small arterial granular degeneration in familial Binswanger's syndrome

A 55-year-old mildly hypertensive woman died after having developed a subcortical dementia during the past 9 years, with focal neurological signs. She presented at the age of 46 years with short episodes of dizziness and diplopia, suggesting that transient ischemic attacks involved the posterior fossa structures. Over the next 8 years, she developed difficulty in walking, urinary incontinence and seizures. On examination in 1989, she was severely demented. There was tetraparesis, bilateral arm and leg spasticity with hyperreflexia and bilateral Babinski signs. She showed epilepsia partialis continua involving the eyes, left hemiface and limbs. CT showed hypodensity of the white matter and lacunes in the basal ganglia and centrum semiovale, moderate hydrocephalus with cerebellar and cortical atrophy. Clinical and radiological features were similar to those of Binswanger's disease. Similar cases had occurred in the family affecting the patient's grandfather, father and two brothers, suggesting an autosomal dominant hereditary disease. Postmortem examination disclosed a Binswanger type of leukoencephalopathy caused by a peculiar microangiopathy characterized by a slightly basophilic small arterial granular degeneration of the medial sheath associated with the presence of ballooned smooth muscle cells with clear cytoplasm. Electron microscopic study revealed degenerative changes in the parietal vessels with notable increase of basal-membrane-type material and electron-dense granular deposits. These lesions could correspond to a specific familial pathology of the small arteries of the brain. They are identical to those reported in some patients with autosomal dominant inheritance. For other patients with similar clinical features and the same familial pattern, reported as "hereditary multi-infarct dementia'' and "chronic familial vascular encephalopathy'', there are no sufficient objective pathological facts to consider that they have the same disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Intracranial and Intraspinal Dissemination from Pineal Yolk Sac Tumor Treated by PVB Therapy

A 15-year-old male was admitted because of diplopia and persistent headache. Postcontrast computed tomography (CT) revealed a homogeneously enhanced large mass, 3 x 4 cm in size, in the pineal region and moderate obstructive hydrocephalus. A right ventriculoperitoneal shunt was installed. At that time, the serum alpha-fetoprotein (AFP) level increased to 23,036 ng/ml, but the level of serum beta-subunit of human chorionic gonadotropin was less than 0.2 ng/ml. These data indicated the tumor to be a pure yolk sac tumor. Following cisplatin-vinblastine-bleomycin (PVB) therapy and whole-brain irradiation (50 Gy), the tumor disappeared on CT, although the AFP level did not return to normal. Eight months after the completion of initial therapy, he had lumbago. Spinal magnetic resonance imaging revealed a metastatic mass at the L5-S2 levels, which was subtotally removed and histologically diagnosed as yolk sac tumor. Postoperative local irradiation (30 Gy) was performed. Seven months after the operation, spinal dissemination at the Th7 level occurred and, 1 month later, intracranial dissemination in the left cerebellopontine angle was detected. He died 25 months after the first admission. PVB therapy did not prevent spinal dissemination in this case.

Medical decisions and prognostications of pediatricians for infants with meningomyelocele

To determine how pediatricians' prognostications and decisions about the treatment of infants with meningomyelocele are affected by varying degrees of hydrocephalus, we randomly selected a sample of pediatricians to receive written surveys. This questionnaire assessed the relationship between the decisions and the prognostications, and compared their accuracy with that of outcome studies. Each pediatrician completed a questionnaire for two of three possible cases, which were identically described as infants with meningomyelocele and complete paraplegia varying only in the degree of hydrocephalus at birth from none to moderate to severe. Recommendations for treatment and prognostications (the Prognostics Belief Scale of Impressions of adult functional capabilities and likely residential and vocational placements) were compared. Significantly fewer pediatricians would present information to parents in an encouraging light, and significantly fewer would treat their own child, if the case involved severe hydrocephalus in comparison with no hydrocephalus. Pediatricians' prognostications were the least optimistic for the infant with severe hydrocephalus and most optimistic for the infant without hydrocephalus, and they were considerably less optimistic than seems justified on the basis of follow-up studies. Last, physicians' less-than-optimistic prognoses were significantly related to their decisions concerning surgery for the infants with moderate and severe hydrocephalus. The major import of the findings is that pediatricians' prognoses reflect, in part, a misconception of the impact of hydrocephalus on children born with meningomyelocele and that these prognoses then have and impact on the pediatricians' decisions concerning treatment.

Cerebellar Haemorrhage — Diagnosis and Treatment: A Study of 75 Consecutive Cases

A retrospective study of 75 consecutive cases of spontaneous cerebellar haemorrhage was undertaken in order to evaluate the clinical features and natural history of this condition. A wide spectrum of clinical findings contributed to the poor clinical diagnostic accuracy of 23 per cent, with common misdiagnoses including brainstem stroke and vestibular or labyrinthine disturbance. Presentation with, or the later development of stupor or coma strongly correlated with poor outcome (severe disability or death; p = 0.002). The characteristics of conscious patients who remained stable were compared with those who subsequently deteriorated. The initial conscious state (alert, drowsy or confused), severity of symptoms and ataxia, and the size of haemorrhage on CT scan were not reliable prognostic indicators. However, the presence of bilateral gaze paresis, anarthria (present in eight cases), limb weakness, a systolic blood pressure above 200 mmHg and moderate hydrocephalus significantly correlated with a poor outcome. The presence of these signs warrants consideration of urgent surgical intervention at the time of diagnosis. Since no clinical or radiological findings excluded the possibility of further deterioration, careful monitoring in an intensive care unit is necessary within the first 48 h in those conscious patients who are likely to remain stable. Guidelines for making the clinical diagnosis and for selecting those patients who will require transfer to adequately equipped centres are suggested.