Neurological complications of childhood malignancies.

UNLABELLED: Between Jan 1982 to Jun 1994, 154 children with malignant non-central nervous system tumors, excluding leukemias and lymphomas, were admitted and treated at the UKMC. Fifty-one (33%) of these cases suffered with 64 neurological complications during the course of their diseases. Nine cases suffered with multiple neurological complications. Nervous system metastasis was the most common neurological complication (n = 24; 15.6%), which was followed by nervous system infection (n = 17; 11%). Twelve (7.7%) cases had treatment related peripheral or cranial neuropathies. Seven (4.5%) cases had new onset of grand-mall seizures. One case had paraneoplastic syndrome, one case had panhypopituitarism secondary to whole brain radiation, and one case had Horner's syndrome secondary to tumor removal. Ten cases suffered with neurological sequelae secondary to neurological complications. Three of these cases suffered with developmental delay and mental retardation. Fifty-one patients with neurological complications were followed for 9 to 102 months. While 30 (19.7%) patients were alive, 20 (13%) patients died and one case was lost during the analysis of the results. Neuroblastoma/ganglioneuroblastoma has the highest rate for causing neurological complication. IN CONCLUSION: neurological complications were seen on 33% of childhood solid malignant tumors. Nervous system metastasis had the worst prognosis and the most frequent neurological complication. Neurological complications did not increase the mortality rate, but one-third of surviving patients with neurological complications suffered with neurological sequelae.

Extracranial neuroblastomas and neurological complications.

Between October 1989 and March 1997, 25 pediatric inpatients were treated for primary extracranial neuroblastoma (NB; n=20) or ganglioneuroblastoma (GNB; n=5) at the University of Istanbul, Institute of Pediatric Oncology, and these children were the subjects of this retrospective study. Seventeen (68%) of these patients experienced 19 neurological complications during the course of their disease. Fourteen had nervous system metastases or invasion. Nonmetastatic complications, including CNS infections (n=3) and new onset of seizures (n=2) secondary to metabolic encephalopathy were seen in 5 cases. By the time of the final analysis of the results, 8 of the 17 patients with neurological complications had died, 7 had either been lost to follow-up (n=4) or were in the terminal stage of their disease (n=3), and 2 were in remission. Both of the patients who were in remission had dumbbell neuroblastoma (DNB), and 1 of them, with congenital DNB, also had neurological sequelae, characterized by paraplegia and neurogenic bladder. Neurological complications occurred in 68% of NB and GNB cases. Metastatic complications were more common than nonmetastatic complications and had a poor prognosis. Neurological complications were the primary cause of mortality in this study, mortality being related to neurological complications in 63% of cases, and the final outcome was worse than expected. However, regardless of any differences in social, economic and geographic factors and different treatment protocols for NB in different pediatric oncology institutions, neurological complication rates in pediatric NB are similar in all.

Neurologic complications of the primary pediatric extracranial neuroblastomas.

Between January 1982 and June 1994, 32 children with primary extracranial neuroblastomas (n = 24) and ganglioneuroblastomas (n = 8) were admitted and treated at the UKMC. Twenty-two (68.7%) of these patients suffered with 29 neurological complications (up to 3 complications per case) during the course of their disease. Fourteen cases had nervous system metastases or invasion. Nonmetastatic complications, such as nervous system infections (n = 4) and new onset of seizures secondary to brain metastasis, hypertensive and metabolic encephalopathies (n = 3) were seen on 7 cases. Five cases had treatment related complications, one case first presented with opsoclonus-polymyoclonus syndrome. Eight of these 22 patients died, due to relapse or progression of the disease. The mean follow-up period of 14 surviving patients was 44.9 months (range, 12-110 months). Five of these 14 patients suffered with neurologic sequelae. The incidence of neurologic complications was 68.7%. Nervous system metastasis was the most common neurologic complication. Although 5 patients suffered with neurologic sequelae, outcome was not influenced by presence of neurologic complication.

Sarcoidosis en-plaque. Report of a case and review of the literature.

A 31-year-old black male with sarcoidosis en-plaque of the dura mater, which is a rare morphological variant of neurosarcoidosis (NS), presented at our clinic. Magnetic resonance imaging (MRI) of the head with gadolinium showed non-specific enhancement of both tentorial leaves extending to the floor of right middle cranial fossa and cavernous sinus. The laboratory results were normal except for slightly increased serum angiotensin converting enzyme (SACE) (68 U/ml n = 4-56 U/ml) and cerebrospinal fluid (CSF) IgG index (0.57, n = 0.46). Biopsy of the intracranial dural lesion was consistent with sarcoidosis. Oral steroid therapy (Methylprednisolone 4 mg QID) was started and the patient became asymptomatic. However, MRI of the brain with gadolinium 2 months after biopsy showed progression and extension of the enhanced dural lesion. His SACE level was unchanged. We concluded that progression of the enhanced lesion seen in MRI could be recently formed scar tissue, new lesion or both. MRI findings should always be correlated with clinical findings for evaluation of NS during follow-up.

Cerebral metastases in childhood malignancies.

Between January 1982 to June 1994, 154 children with non-CNS malignant tumours excluding leukaemias and lymphomas were admitted and treated at the UKMC. Symptomatic (10 cases; 6.5%) and non-symptomatic (2 cases; 1.2%) cranial metastases (calvarial, dural and/or parenchymal) were seen in 12 (7.8%) cases. Among these 12 cases, 7 had intracranial parenchymal metastases (4.5%). Three cases had multiple intracranial parenchymal metastases. Only one case had infratentorial (cerebellar) metastasis. The patients' ages ranged from 1 to 18 years (mean age was 7.3 years). The male:female ratio was 5:2. While six patients' brain metastases diagnosed during subsequent relapses, one patient first presented with brain metastasis. Time elapsed between the diagnosis of the primary disease and intracranial metastasis ranged from 0 to 755 days (mean 327 days). Histopathological diagnoses were confirmed in 4 cases who had craniotomy and tumour removal. Mean survival following the diagnosis of intracranial lesion was 157 days (ranged from 0 to 412 days). Despite the aggressive treatment, including surgery, chemotherapy and radiotherapy, 6 cases died with progression or relapse of the intracranial disease. In conclusion, the incidence of paediatric intracranial parenchymal metastasis is 4.5%. Prognosis is poor and intracranial disease is the primary cause of death.

Primary spinal column sarcomas.

Five cases of primary spinal column sarcomas are presented. Sarcomas primarily originating from paravertebral soft tissues were excluded. Patients' age ranged from 1 to 14 years (mean 8.4 years). The male:female ration was 2:3. Two patients had Ewing's sarcoma (ES) originating from L5-S1 and L4-5 pedicles, respectively; two patients had mesenchymal chondrosarcoma (MCS) originating from L1-2 pedicles and L5 body, respectively; and one patient had osteogenic sarcoma (OS) of C4 body. All patients clinically presented with pain and progressive weakness of the extremities. The time that elapsed between the onset of symptoms and diagnoses ranged from one to five months. All cases were treated with chemotherapy, radiotherapy and subtotal tumour resection with spinal canal decompression. Two cases received posterior spinal fusion operations. Three patients were alive 10 to 98 months following diagnosis. Only the case with ES of L5-S1 pedicles was in complete remission and off therapy at the 98th postoperative month. The two MCS cases were in partial remission, and were receiving chemotherapy at the time of analysis. These tumours caused similar clinical findings and prognoses, and required combined treatment, which consisted of surgery, radiotherapy and chemotherapy; histologically three different types of malignant tumours are presented in the same category. We preferred surgical decompression and stabilization procedures especially for neurologically symptomatic patients, even if they had extensive tumours with high grades. By spinal canal decompression and stabilisation, we did not intend to cure the disease; however, we intended to provide neurological improvement, spinal stabilisation, improved quality of life, early mobilisation of the patient, and cytoreduction by means of surgical tumour ablation, which could render the chemotherapy more effective.

MRI of cerebellopontine angle choroid plexus papilloma.

We present a cerebellopontine angle choroid plexus papilloma that originated from the tuft of choroid plexus of the fourth ventricle protruding from the foramen of Luschka. MRI and CT did not establish the diagnosis, but the tumor was shown histopathologically to be a choroid plexus papilloma. Distinct features of the tumor on MRI are described and the differential diagnosis of other cerebellopontine angle tumors is discussed.

Mild hypothermia fails to protect late hippocampal neuronal loss following forebrain cerebral ischaemia in rats.

Anaesthetized male rats (n = 86) from both Long-Evans strain (LES) (n = 43) and Wistar strain (WS) (n = 43) were utilized for the experiments. While three animals from each strain were used as control, 40 rats from each strain underwent up to 10 minutes forebrain ischaemia by bilateral common carotid artery (CCA) occlusion combined with systemic hypotension [Mean Arterial Blood Pressure (MABP) = 50 mm/Hg]. The animals from each strain were divided into four (n = 10) groups. In both strains, groups (n = 10) 1 and 2, temporalis muscle (TM) and body temperatures of the animals were kept at 36-37 degrees C during the experiments. The groups 1 and 2 were killed in 3 and 7 days after the ischaemic insult, respectively. The groups 3 and 4 were also killed 3 and 7 days after the ischaemic insult, but the forebrain ischaemia was carried out under mild cerebral hypothermia (TM temperature = 33 degrees C). Pyramidal neurons of the hippocampal CA1 region from each group was evaluated semiquantitatively. In WS, groups 1 and 2 showed moderate and severe neuronal loss in the CA1 region, respectively. However, in LES while the group 1 (3 days survival) did not show any neuronal loss, group 2 showed moderate neuronal loss of the CA1 region. While in group 3 (3 days survival, hypothermia) WS and LES, hypothermia protected the CA1 region, group 4 of LES showed mild neuronal loss. However WS, group 4 (7 days survival, hypothermia) showed severe neuronal loss of the CA1 region. It was concluded that mild hypothermia during ischaemic insults did not prevent the delayed postischaemic neuronal damage of the hippocampal CA1 region of both strains, and following 10 minutes forebrain ischaemia, male LES rats were found more resistant than male WS rats to neuronal loss of the CA1 region.

Long-term follow-up results of thoracolumbar fractures after posterior instrumentation.

STUDY DESIGN: This retrospective study examined the hospital records of 60 patients with thoracolumbar fractures treated with posterior fusion and spinal instrumentation. The mean follow-up period was 66 months. OBJECTIVES: The goal of this study was to evaluate and analyze the long-term outcome and socioeconomic conditions of patients who had sustained a thoracolumbar fracture. SUMMARY OF BACKGROUND DATA: In four patients, additional spinal injuries were detected. Three of the patients evaluated with magnetic resonance imaging showed cord contusion and edema. In six patients, ruptured disc fragments were detected by preoperative magnetic resonance imaging or during surgery. METHODS: Long-term follow-up results in 60 patients with unstable thoracolumbar fractures treated with posterior fusion and spinal instrumentation were analyzed. Neurologic outcomes and independence in function and daily living activities were reviewed. Age, sex, mechanism of injury, associated injuries to the spinal cord, and associated injuries to the spinal cord and other systems were analyzed. Fractures were classified according to the system of Ferguson and Allen. RESULTS: The patients with incomplete spinal cord injury showed significant functional improvement. During the follow-up period, 28 patients showed neurologic improvement. Postoperative complications occurred in 11 patients. Five patients required late rod removal because of rod dislocation. CONCLUSION: Regardless of neurologic recovery, most patients reported some disability, usually caused by pain. Inability to return to alternative jobs resulted from insufficient educational background rather than neurologic dysfunction. Advanced academic achievement was the single most important predictive factor of ability to return to work.

Reversible hypoperfusion of the cerebral cortex in normal-pressure hydrocephalus on technetium-99m-HMPAO brain SPECT images after shunt operation.

A man with dementia underwent radionuclide cisternography to establish the diagnosis of communicating hydrocephalus. Technetium-99m-HMPAO brain SPECT images showed marked hypoperfusion of both posterior cerebral cortices and three-dimensional displays that demonstrated perfusion defects at both of the posterior parietotemporal regions. A successful ventriculoperitoneal shunt operation was performed. Five and one-half months later, repeat three-dimensional display showed that the perfusion defects had resolved and a repeat brain SPECT image showed marked improvement of the hypoperfusion. This concurred with postoperative clinical improvement. Technetium-99m-HMPAO brain SPECT, which provides objective documentation of clinical recovery after surgery, could be routinely used to evaluate patients with normal-pressure hydrocephalus.

Oligodendroglial hamartoma of the right temporal lobe: a case report and discussion of possible histogenesis.

A case of oligodendroglial hamartoma is reported in a 39-year-old man with a 20-year history of petit mal seizures. Magnetic Resonance Imaging of the brain showed a small focal area of abnormal decreased signal on T1-weighted images of the right temporal lobe. The patient became seizure free after the removal of the lesion and treatment with carbamazepine. Pathologic examination revealed several aggregates of oligodendroglial cells with small, dark, regularly stained nuclei and a clear, well-defined perinuclear halo; there was no mixture of neurons or astrocytes. Although these cells were negative, the background was strongly positive for glial fibrillary acidic protein, and myelin basic protein. The histopathologic diagnosis of the temporal lobe lesion was oligodendroglial hamartoma. This report documents an additional subtype of temporal lobe hamartoma associated with seizure disorder.

CT appearance of an unusual type of comminuted fracture/dislocation of C1 and C2.

An unusual type of traumatic comminuted fracture/dislocation of C1 and C2 is presented. The fracture differs significantly from previously described types of C1-2 complex fracture. Anatomically contoured titanium loops were used for stabilization of the occipitocervical junction. To the best of the author's knowledge this is the first description of comminuted fracture/dislocation of the bodies and laminae of C1 and C2 in the English literature.

Effects of antioxidants on the blood-brain barrier and postischemic hyperemia.

The role of free oxygen radicals in blood-brain barrier (BBB) disruption and postischemic hyperemia was evaluated in the rabbit model of focal cerebral ischemia-reperfusion. Six groups of rabbits underwent clipping of the anterior cerebral, middle cerebral, and intracranial internal carotid arteries. Cerebral blood flow (CBF) was measured by using radiolabeled microspheres, before, during, and 15 minutes after 1-hour occlusion of these arteries. After 50 minutes of ischemia, Group 1 animals (control) received a placebo. Animals in Groups 2-4 received one of three drugs: catalase at 10 mg/kg, methimazole at 5 mg/kg, or indomethacin at 10 mg/kg. A fifth group received a tungsten-supplemented diet for 14 days before ischemia was induced, and a sixth group was sham operated. Microvascular integrity within the brain was determined by the presence or absence of Evan's Blue (EB)-albumin dye leakage across the BBB and was measured by microspectrofluorometry. In the control group during ischemia, CBF dropped to 14%, 7%, and 11% of preischemic levels in rostral, middle, and caudal sections of the brain, respectively, as characterized by extensive EB-albumin dye leakage through the BBB into the ischemic hemisphere. During early reperfusion, postischemic hyperemia was associated with an increase in CBF of 128%, 123%, and 129% of control in the rostral, middle, and caudal sections of the brain, respectively. In all treated groups and in the group receiving a tungsten-supplemented diet, BBB integrity was protected during reperfusion without inhibition of postischemic hyperemia. This study suggests that early disruption of the BBB to large molecules is mediated by free oxygen radicals, which inhibit rather than cause postischemic hyperemia.

Measurement of cerebrovascular changes in cats after transient ischemia using dynamic magnetic resonance imaging.

BACKGROUND AND PURPOSE: Hemodynamic changes associated with acute ischemia cannot be measured with conventional nuclear magnetic resonance imaging. In this study, we used dynamic susceptibility-contrast magnetic resonance imaging to measure the changes in vascular transit time and relative cerebral blood volume in a feline occlusion-reperfusion model. METHODS: Dynamic susceptibility-contrast measurements were obtained before and during 10 minutes of global cerebral ischemia and for up to 3 hours after the onset of reperfusion. A cerebral blood flow index was calculated from the vascular transit time and relative cerebral blood volume measurements. Functional maps were constructed to demonstrate the regional hemodynamic differences resulting from the induced ischemia. RESULTS: During the early phase after reperfusion, both the relative cerebral blood volume and blood flow index rose sharply, followed by a fall to near-basal levels at 45 minutes (1 x control and 1.3 x control, respectively). Thereafter, the volume rose slowly, whereas the flow index continued to drop. At 3 hours, cerebral blood volume had reached 1.6 times its control value, whereas the flow index had returned to its base value. CONCLUSIONS: The hemodynamic behavior we observed in our model reflects the independent responses of the cerebral blood volume and flow index to ischemic insult. Measurements acquired by our method were consistent with the temporal behavior reported in previous radionuclide studies. Susceptibility-contrast nuclear magnetic resonance tomographic imaging proved to be valuable in detecting and quantifying both immediate and subsequent changes in the hemodynamic state of the ischemic and hyperemic feline brain.

Encephalo-myo synangiosis (EMS) in the vertebrobasilar occlusive disease. Case reports.

Two cases of vertebro-basilar insufficiency treated with infratentorial (IT) Encephalo-Myo-Synangiosis (EMS) using the pedicled occipitalis muscle flap, are presented. IT EMS was carried out for revascularization of the vertebro-basilar system. None of these cases has suffered from either carotid or vertebro-basilar insufficiency during seven years follow-up period. As far as we know this is the first report of successful IT EMS for vertebro-basilar insufficiency.

Recovery of vision after transcranial decompression of pituitary apoplexy characterized by third ventricular hemorrhage.

The authors present a case of pituitary apoplexy characterized by massive third ventricular hemorrhage. This rare complication, which caused total blindness in the patient, a 67-year-old man, was treated with transcranial decompression of the pituitary tumor and removal of a blood clot from the third ventricle. After surgery, the patient totally recovered his vision. To the best of our knowledge, this is the first case of a patient with pituitary apoplexy that ruptured into the third ventricle (diagnosed by imaging studies) who was treated by emergency transcranial decompression.

Effect of superoxide dismutase on acute reperfusion injury of the rabbit brain.

To study the involvement of free oxygen radicals of the blood-brain barrier (BBB) disruption during early reperfusion, we isolated the distal internal carotid artery, and the middle and anterior cerebral arteries via the transorbital approach in anesthetized rabbits. Using radiolabeled microspheres, regional cerebral blood flow (rCBF) was measured before, during and after 1-hour unilateral occlusion of these vessels. Fifty-five minutes after ischemia, animals received intravenous saline placebo (control), superoxide dismutase (SOD) at 8 mg/kg = 30,000 U/kg, or weakened superoxide dismutase (wSOD) at 8 mg/kg = 30,000 U/kg. Integrity of the BBB was assessed by leakage of Evan's Blue-albumin dye (EB-albumin dye), which was given at 15 minutes of reperfusion and allowed to circulate for an additional hour. In the control and wSOD-treated groups, rCBF decreased (26% and 40% of control, respectively) within the blue-tinted tissue of the occluded hemisphere during ischemia; hyperemia was observed during early reperfusion. In the control and wSOD-treated groups, EB-albumin dye leakage across the BBB increased 49% within the occluded hemisphere. However, within the SOD-treated group, the BBB showed minimal dye leakage even though rCBF of the occluded hemisphere (so-called blue-tinted tissue) decreased by 38% during ischemia. We conclude that 1-hour focal cerebral ischemia and reperfusion produce a vascular endothelial injury at the BBB. Since SOD administration showed significant protection, free-oxygen-radical production during early reperfusion is associated with breakdown of the BBB to large molecules.