Abducens (sixth) nerve palsy presenting as a rare case of isolated brainstem metastasis from a primary breast carcinoma.

Most isolated abducens (sixth) nerve palsies are ischaemic in nature. However, there are other causes that can mimic an abducens nerve palsy, which requires aggressive diagnostic management. A 56-year-old hypertensive woman presented with a right abduction deficit. Her past history revealed that she had undergone a mastectomy and completed a course of chemo- and radiation therapy for breast carcinoma. She was well until she develped binocular diplopia five months later. Magnetic resonance imaging showed a right pontine mass. Stereotactic biopsy was performed, and histopathology revealed a metastatic carcinoma that was compatible with an origin from the breast primary. We conclude that identifying and managing patients with metastatic lesions involves a multidisciplinary approach. Thorough history-taking and neuro-ophthalmologic evaluation would help physicians in establishing the primary differentials, which could not only be sight-saving but life-saving as well.

Breast and ovarian recurrence of acute lymphoblastic leukaemia after allogeneic peripheral blood haematopoietic stem cell transplantation.

Breast recurrence of acute lymphoblastic leukaemia (ALL) after stem cell transplant is uncommon, with less than 20 reported cases in the literature. In the majority of cases, the lesions developed without simultaneous involvement of other sites or graft-versus-host disease (GvHD). We describe the first case of simultaneous bilateral breast and ovarian relapses after allografting in ALL, occurring in an 18-year-old female Chinese patient while she was having oral and hepatic chronic GvHD, persistent haematological remission and donor haematopoiesis. She received radiotherapy and chemotherapy, which resulted in resolution of the breast and ovarian lesions, and remained disease free ten months after the onset of the relapse. This case suggests that there may be different mechanisms for bone marrow vs. extramedullary relapses and a complex relationship between GvHD and graft-versus-leukaemia.

Isolation of purified autologous peripheral blood CD34+ cells with low T cell content using CliniMACS device--a local experience.

INTRODUCTION: Peripheral blood stem cells (PBSC) mobilised with growth factor with or without chemotherapeutic regimens, are used increasingly in both autologous and allogeneic transplantation. Previously, many PBSC harvests are used directly without ex vivo manipulation, and these PBSC have been shown to be contaminated with tumour cells, which may contribute to subsequent relapses post transplantation. Therefore, requirement for purging of malignant cells from the harvest has initiated the use of various methods to reduce tumour cell contamination of the graft by the positive selection of CD34+ progenitor cells or negative selection of tumour cells using other cell-specific antigens. We report here our local experience with the CliniMACS (magnetic-activated cell separation system) in eight adult patients with haematologic malignancies. OBJECTIVE: To evaluate the purity, recovery and viability of CD34+ cells selected from harvested peripheral blood stem cells using the CliniMACS device, as well as to evaluate the T and B cell contents of these products. METHOD: Eight adult patients with malignant haematological diseases (5 non-Hodgkin's lymphomas in 2nd complete remission (CR) and 3 acute myeloid leukaemias in 1st CR) were mobilised with granulocyte colony-stimulating factor (G-CSF) with or without chemotherapeutic regimens. A total of nine leukaphereses for peripheral blood stem cell harvest using the Cobe Spectra cell separator (Cobe BCT Lakewood, CO) were performed. The harvested PBSC were then positively selected for CD34+ cells using the CliniMACS device (Milteny Biotech, Germany). RESULTS: A total of nine leukapheresis products from eight adults with a median pre-selection total CD34+ cell count of 282.2 x 10(6) (range 103.7 - 738.2 x 10(6)) were positively selected with CliniMACS. The median post-selection total CD34+ cell count was 99.5 x 10(6) (range 7.7 - 443.9 x 10(6)) with the median recovery was 66.0% (range 2 - 94%) and median purity of products of 79% (range 18 - 86%). The median total T cell count was reduced dramatically from 3.1 x 10(9) pre-selection to 7.9 x 10(6) post-selection. The selection did not affect the viability of selected cells that was tested with trypan blue exclusion method with a median pre and post selection viabilities of 98% (range 95 - 98%). CONCLUSION: We conclude that positive selection of CD34+ cells using magnetic separation technology by CliniMACS device results in low T-cell content stem cell with acceptable purity and recovery for autologous peripheral blood stem cell transplantation.

Optic neuritis in Singapore.

INTRODUCTION: Optic neuritis (ON) is the commonest optic neuropathy encountered in the younger group of patients from the Singapore Neuro-Ophthalmology Study Group. Previous surveys in Asia and our study suggest that ON in Singapore differs from that in Western populations where the majority of cases are associated with multiple sclerosis (MS). METHODS: Patients satisfying our entry criteria for ON seen between September 2002 and June 2004 were enrolled in the study. The data collected was recorded in a central database and analysed two years later. RESULTS: The majority (60 percent) of our patients had anterior ON with optic disc swelling (papillitis), were idiopathic in 49.1 percent, and associated with MS in only 25.5 percent. Bilateral cases comprised 16.4 percent of our series and were usually of the anterior variety and mostly idiopathic, although it is still essential to rule out secondary causes. Recurrent ON is indicative of an underlying disease process. CONCLUSION: The pattern of ON as seen in Singapore differs from that reported in Caucasian studies and from the seminal Optic Neuritis Treatment Trial. We found a higher incidence of optic disc swelling, i.e. anterior ON (papillitis), and a lesser association with MS; visual recovery is similarly good but our recurrence rate is lower.

The use of mycophenolate mofetil in treating patients with non responding aplastic anemia.

Aplastic anemia is a relatively uncommon disease and conventional management options include immunosuppressive drugs and/or haematopoeitic stem cell transplantation. It is now known that the pathogenesis of aplastic anemia is immune mediated. Mycophenolate mofetil is a common immunosuppressive drug now used mainly in prophylaxis of graft rejection in organ transplant and also for prevention/treatment for graft versus host disease in haemtopoeitic stem cell transplantation. It is thought that mycophenolate mofetil may be useful in this group of patients. In this short report, mycophenolate mofetil was tried in 6 patients who had severe aplastic anemia with variable doses for a minimum duration of 9 months. The result has however not been encouraging.

Incidence of upper limb venous thrombosis associated with peripherally inserted central catheters (PICC).

The objective of this study was to prospectively determine the incidence of venous thrombosis (VT) in the upper limbs in patients with peripherally inserted central catheters (PICC). We prospectively investigated the incidence of VT in the upper limbs of 26 patients who had PICC inserted. The inclusion criteria were all patients who had a PICC inserted, whilst the exclusion criterion was the inability to perform a venogram (allergies, previous contrast medium reaction and inability of gaining venous access). Both valved and non-valved catheters were evaluated. Prior to removal of the PICC, an upper limb venogram was performed. The number of segments involved with VT were determined. The duration of central venous catheterization was classified as; less than 6 days, between 6 days and 14 days and more than 14 days. VT was confirmed in 38.5% (10/26) of the patients. The majority 85.7% (12/14) were complete occlusive thrombi and the majority of VT only involved one segment. There was no statistical correlation between the site of insertion of the PICC and the location of VT. Neither was there any observed correlation between the occurrence of VT with the patient's history of hypertension, hypercholesterolaemia, coronary artery disease, diabetes mellitus, cardiac insufficiency, smoking or cancer. There was also no statistical correlation with the size of the catheter. In conclusion, PICCs are associated with a significant risk of upper extremity deep vein thrombosis (UEVT).

A case of T-cell acute lymphoblastic leukemia after treatment of acute promyelocytic leukemia.

We diagnosed T-cell acute lymphoblastic leukemia (T-ALL) with multiple cytogenetic abnormalities in a 17-year-old girl a year after she had received a diagnosis of acute promyelocytic leukemia (APML). After the diagnosis of APML in June 2001, the patient was treated with idarubicin and all-trans-retinoic acid. In September 1999, her younger sister also received a diagnosis of APML and to date has remained well. T-ALL after remission of APML is very rare, and only 1 such case has been reported. Possible causes include therapy-related reasons, genetic susceptibility to leukemia, and environmental exposure.

Successful peripheral blood stem cell mobilisation with filgrastim in patients with chronic myeloid leukaemia achieving complete cytogenetic response with imatinib, without increasing disease burden as measured by quantitative real-time PCR.

Imatinib mesylate (Glivec) is a selective inhibitor of bcr-abl tyrosine kinase, the product of the Philadelphia chromosome, which is the hallmark of chronic myeloid leukaemia (CML). With imatinib, complete cytogenetic response (CCR) can be achieved in over 70% of newly diagnosed patients with CML. However, the optimal long-term management of patients who achieve CCR after imatinib is unknown. With longer follow-up, it is anticipated that some patients are likely to progress and become candidates for autologous transplantation. We studied filgrastim (r-metHuG-CSF) mobilisation of peripheral blood stem cells (PBSC) in 32 patients who have achieved CCR with imatinib. Our data demonstrate that (1) the target CD34(+) cell yields of >/=2.0 x 10(6)/kg were attained with filgrastim 10 microg/kg/day, in 9/18 (50%) of patients during uninterrupted imatinib therapy, and in 10/14 (70%) when imatinib was temporarily withheld. The median CD34(+) cell yield per aphaeresis was 0.70 x 10(6)/kg (range 0.14-2.18) and 2.90 x 10(6)/kg (range 0.15-8.71) in the two groups, respectively (P&<0.005). (2) The cell yields did not correlate with the duration of imatinib administration. (3) There was no impact of the mobilisation procedure on the level of leukaemia as measured by serial blood bcr-abl levels using real-time quantitative PCR with either protocol. (4) bcr-abl remained detectable at low levels in the harvests in most but not all patients. In conclusion, filgrastim can safely be used to mobilise PBSC in patients who have achieved CCR with imatinib, but CD34(+) cell yields are significantly improved when imatinib is temporarily withheld.

Bifrontal decompressive craniectomy for acute subdural empyema.

INTRODUCTION: Subdural empyema is an uncommon but serious complication of sinusitis. Despite the use of advanced imaging facilities, modern antibiotic therapy and aggressive neurosurgical protocols, this condition still carries significant morbidity and mortality. CASE REPORT: We report an unusual case of sinusitis-associated acute subdural empyema in a 13-year-old patient, presenting in a catastrophic manner with acutely raised intracranial pressure. Emergency bifrontal decompressive craniectomy was necessary both to reduce the intracranial pressure and to drain the subdural empyema. RESULTS: The full range of intracranial complications subsequently occurred, including brain abscesses, recurrent subdural empyema and ventriculitis. Despite this, the patient's outcome was good, with minimal intellectual deficits. CONCLUSION: In cases of severe intracranial infection, we therefore advocate an aggressive surgical approach coupled with appropriate antibiotics to ensure a good outcome.

Cerebral infarct mimicking glioma in Sjogren's syndrome.

A 50-year-old Chinese woman with a chronic 20-year history of ataxic gait associated with dry eyes and mouth, was admitted to hospital after a single episode of syncope. Magnetic resonance imaging scans showed a large left frontal hypodense lesion suggestive of a glioma. Craniotomy was performed and the lesion excised, with histology showing only infarcted tissue and no malignant cells. Further diagnostic evaluation revealed that the patient had primary Sjogren's syndrome, with demyelinating polyneuropathy. In the absence of risk factors for stroke, it was considered likely that the cerebral infarct was secondary to autoimmune-related vasculitis. Functional neuroimaging, such as magnetic resonance spectroscopy, should be considered in evaluating doubtful or unusual brain lesions in patients with autoimmune disease.

Non-sporulating Chrysosporium: an opportunistic fungal infection in a neutropenic patient.

We report a case of an invasive infection with non-sporulating Chrysosporium species in a patient who was treated with chemotherapy for relapsed acute lymphoblastic leukemia. This patient presented with a persistent lobar pneumonia, skin lesions, and possible involvement of the central nervous system. The patient responded to treatment with amphotericin B and oral itraconazole.

The role of early tracheostomy in critically ill neurosurgical patients.

OBJECTIVE: To determine the value of early tracheostomy (within 7 days) in ventilated neurosurgical patients. METHODS: Retrospective review of intubated patients in the neurosurgical intensive care unit (NICU) who underwent elective open tracheostomies for prolonged ventilation. RESULTS: Thirty patients over a 2-year period were analysed. There were 19 males and 11 females, mean age 53.9 +/- 18.1 years (range 14 to 89), and mean Glasgow Coma Scale (GCS) score on admission 7.1 +/- 3.8 (range 3 to 15). The underlying disease aetiology was cerebrovascular disease in 53% of patients, head trauma in 33% and tumour or infection in 13%. Tracheostomy was performed after a mean period of 8.5 +/- 3.5 days (range 2 to 18), with patients requiring ventilation for a mean duration of 13.5 +/- 6.3 days (range 3 to 31). Complications were minimal; 1 wound infection (3.3%) and 4 tube obstructions (13.3%). Patients who underwent elective early tracheostomy (Group 1 = within 7 days) had poorer GCS on admission (6.3 +/- 2.9 versus 7.7 +/- 4.3 in Group 2, P = 0.271). Tracheostomy was performed after a mean of 5.3 +/- 1.7 days in Group I vs. 10.6 +/- 2.7 days in Group 2. Group 1 patients had faster recovery from nosocomial pneumonia (12.3 +/- 6.2 versus 17.9 +/- 12.5 days, P = 0.168), shorter duration of ventilation (9.8 +/- 5.9 versus 16.0 +/- 5.4 days, P = 0.007), and reduced incidence of multibacterial tracheobronchial colonisation (42% versus 72%, P = 0.098). The most prevalent organisms were Acinetobacter baumanii (43.3%), Pseudomonas (40%), methicillin-resistant Staphylococcus aureus (MRSA) (33%), Klebsiella (30%) and Staphylococcus aureus (26.7%). CONCLUSION: Early tracheostomy in selected neurosurgical patients with poor GCS scores was associated with reduced incidence of tracheobronchial colonisation by multiple pathogens, improvement in chest infections, and rapid weaning from ventilatory support.

An unusual case of traumatic intracranial hemorrhage caused by wakeboarding.

Wakeboarding is a relatively new and exciting sporting activity which is similar to water-skiing. While there have been several reports of water-skiing-related injuries, there has been nothing reported in the literature so far concerning injuries in wakeboarding. We present the case of a 14-year-old male who sustained an unusual intracranial subdural hemorrhage while wakeboarding and postulate on the mechanisms which could have resulted in the injury.

Giant cell arteritis causing bilateral sequential anterior ischaemic optic neuropathy--a case report.

Giant cell arteritis is a chronic granulomatous inflammation of unknown aetiology involving large and medium size arteries in the elderly. It causes acute visual loss from ischaemia to the optic nerves or central retinal artery occlusion. This is a rare cause of anterior ischaemic optic neuropathy in our local population. We present a patient who had bilateral loss of vision from sequential arteritic ischaemic optic neuropathy. She was treated with intravenous steroids immediately. Diagnosis was based on histopathological studies of temporal artery biopsies.

Is decompressive craniectomy for acute cerebral infarction of any benefit?

BACKGROUND: Acute occlusion of the major cerebral arteries results in ischaemic changes to the brain, without time for reperfusion by the collateral circulation. The subsequent cellular events lead to a breakdown of the blood-brain barrier, causing malignant cerebral edema manifested clinically by a rapid neurological deterioration. The aim of this study was to determine the value of surgical decompression in patients who present with acute cerebral infarction. METHODS: Retrospective review of patients with deteriorating consciousness level from massive cerebral ischemia and secondary edema, treated by decompressive craniectomy. RESULTSThere were 10 patients over a 2-year period from 1997-99, consisting of seven male and three female patients (mean age 47.56 years) with a mean preoperative Glasgow Coma Scale (GCS) score of 6/15. Three patients had dominant middle cerebral artery (MCA) infarction, four had nondominant MCA infarction, one had posterior cerebral artery infarction, and the remaining two had cerebellar infarction. At a mean follow-up period of 7 months, two patients had died (20% mortality), four patients (40%) were vegetative or severely disabled, and the remaining four patients (40%) had mild disability or good outcome. Favorable prognostic factors were younger age (less than 50 years) and good initial GCS score (14 or better). CONCLUSION: Decompressive craniectomy in the setting of acute brain swelling from cerebral infarction is a life-saving procedure and should be considered in younger patients who have a rapidly deteriorating neurologic status.

Sympathetic storms in a child with a midbrain glioma: a variant of diencephalic seizures.

The authors report the unusual case of a 7-year-old child, one of the youngest reported to date, who developed repeated episodes of sympathetic hyperactivity after surgical resection of a midbrain glioma. These paroxysmal events were similar to previously described diencephalic seizures. However, there was no evidence of epileptogenic activity on electroencephalography, and radiologic imaging did not reveal hydrocephalus or intraparenchymal hemorrhage. In this report, clinical features are described of this patient, along with the novel use of clonidine--a sympathetic blocking agent--in his treatment, published reports are reviewed on diencephalic seizures, and steps are recommended in the treatment of a patient who presents in this manner. The authors believe that diencephalic seizures can present with a spectrum of autonomic features, and treatment should be tailored with the appropriate pharmacologic blockade.

Intraventricular recombinant tissue plasminogen activator for treatment of spontaneous intraventricular haemorrhage in pregnancy.

Spontaneous intracranial haemorrhage during pregnancy, which is usually subarachnoid or intraparenchymal in nature, causes significant maternal and foetal morbidity and mortality. Solitary intraventricular haemorrhage (IVH) in pregnancy has never been previously reported nor has the use of intraventricular fibrinolysis in this situation. A 28-year-old woman presented at 20 weeks' gestation with status epilepticus from spontaneous severe IVH. Angiography and magnetic resonance imaging did not show the presence of a vascular lesion or aneurysm. Thrombolytic treatment with intraventricular injection of recombinant tissue plasminogen activator (rt-PA) was instituted. Although the foetus aborted spontaneously 6 weeks after the haemorrhage and a ventriculo-peritoneal shunt for hydrocephalus was eventually required, the mother recovered to the level of mild disability at 3 months. Intraventricular fibrinolysis with rt-PA seems to be an effective form of treatment for IVH in pregnancy.