Haemophagocytic syndrome (HPS) should be included in the differential diagnosis of pyrexia of unknown origin (PUO). The hallmark of HPS is the accumulation of activated macrophages that engulf haematopoietic cells in the reticuloendothelial system. We describe a patient with unexplained fever in which a final diagnosis of HPS was established in a bone marrow study.
Patients (particularly elderly) undergoing evaluation for peripheral neuropathy of unknown cause should be screened for the presence of a monoclonal protein (M protein). The association of a neuropathy and a paraproteinaemia such as Waldenstrom's Macroglobulinaemia (WM) is not uncommon with the former antedating the haematologic symptoms by several years. Response to treatment has varied from good to very poor. We describe a case of WM presenting as a subacute demyelinating peripheral neuropathy. There was prompt resolution of the neuropathy with intravenous immunoglobulin therapy. Subsequent treatment with cyclophosphamide and plasmapheresis resulted in complete clinical remission with no further neurological relapses.
A fulminant clinical presentation with high fever and hepatosplenomegaly, together with a course of worsening pancytopenia, coagulopathy and liver failure, is suggestive of the haem syndrome (HPS). Bone marrow examination is diagnostic. We present 3 cases of HPS associated with different aetiologies including acute Ebstein Barr virus infection, T cell lymphoma, and malignant histiocytosis. In all the cases, the diagnosis was made late and the patients succumbed before definitive therapy could be administered.
Leukemic infiltration of the optic nerve is rare [1]. [Camera, A., Piccirillo, G., Tranfa, F., Rosa, N., Frigeri, F., Martinelli, V., Rotoloi, B. (1993) "Optic nerve involvement in acute lymphoblastic leukemia", Leuk. Lymph. 11, 153-155]. Radiotherapy should be given urgently to all patients with optic nerve infiltrate to restore their vision [2]. [Rosenthal, A. (1983) "Ocular manifestation of leukemia", Ophthalmology 90, 899-905]. We report a case of a unilateral optic nerve relapse 7 months after diagnosis of acute lymphoblastic leukemia (ALL) in a 17-year-old boy who had been off treatment for 6 weeks. The ocular symptoms was initially diagnosed as primary optic neuritis and treated with corticosteroids resulting in temporary clinical recovery. Radiation therapy for ocular leukemia that was commenced 2 months after the onset of symptoms failed to reverse the visual loss. The lack of a reliable and effective tool to diagnose ocular leukemia at an early stage has resulted in significant treatment delay and poor visual outcome.
Nocardia infection is rare in bone marrow transplant (BMT) recipients with less than 30 cases reported in the literature [1-4]. The majority of the cases occurred late in the post-transplant period. Common clinical presentations included formation of widespread and multiple abscesses. Bone marrow hypoplasia is an uncommon finding. We describe the first case of nocardiosis, diagnosed at day 100 after non-myeloablative allogeneic peripheral blood stem cell transplantation, presenting as pancytopenia and hypocellular marrow. Eradication of the infection with antibiotics resulted in complete hematological recovery.
Previous studies have shown that carbamazepine-induced Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) patients is associated with the HLA-B*1502 allele. Screening for HLA-B*1502 before using carbamazepine can prevent SJS/TEN particularly in populations with high frequency of the allele. The objective of this paper was to describe how the UKM Medical Centre, Malaysia was able to set up a cost effective screening of HLA-B*1502 for patients taking carbamazepine. The cost of in-house HLA-B⁄1502 screening was less than those commercially available, and was sensitive and specific.
Infection is a frequent complication after an acute stroke and may affect stroke outcome. We identified predictors of early infection, type of infection, their relation to initial disability, and the eventual outcome during the inpatient period.
Symptomatic rheumatoid pachymeningitis is a rare extra-articular manifestation of rheumatoid arthritis. Clinical symptoms are non-specific and diagnosis is frequently made by exclusion. We present a 61-year-old woman with a 9-year history of rheumatoid arthritis presenting with deafness and progressive disability over a two month duration. She was diagnosed as having rheumatoid pachymeningitis based on the cerebral magnetic resonance imaging findings.
Retraction notice: Following investigations of duplicate publication in Parkinsonism and Related Disorders 2009; 15: 670–674, we have determined that there is indeed a substantial overlap between the two articles. As such, we fully retract this paper from the published record of the Singapore Medical Journal.
Professor Teo Eng Kiong, Editor, Singapore Medical Journal
http://smj.sma.org.sg/5201/5201rec1.pdf
There are limited studies reporting the frequency of sleep-disordered breathing (SDB) in Parkinson's disease (PD), and the figures quoted are variable, ranging from 2.5 to 66 percent. This study aimed to determine the prevalence and types of SBD in PD patients attending the Universiti Kebangsaan Malaysia Medical Centre neurology clinic, and the correlation between the subjective sleep symptoms using the Parkinson's disease sleep scale (PDSS) and the objective measurements using polysomnography (PSG).
METHODS: This was a cross-sectional study involving 46 PD patients over a period of six months. The patients' demographic data, Hoehn and Yahr staging and PDSS scores were collected. The patients were then subjected to overnight PSG using the Somnomedic system.
RESULTS: There were 27 male and 19 female patients with a mean age of 64.0+/-9.7 years. 29 were Chinese, 15 Malay and 2 Indian. The mean duration of illness was 5.8+/-4.3 years. The mean PDSS score was 120.3+/-13.5. SDB was found in 54.6 percent of the patients (apnoeahypopnoea index [AHI] 5 and above), with 27.3 percent having moderate and severe SDB (AHI 15 and above). The median AHI was 6.7 (range 0-40.4). The prevalence of SDB in PD patients based on the AHI cutoffs were 27.3 percent for mild, 18.2 percent for moderate and 9.1 percent for severe. There were statistically significant positive correlations between the AHI and the neck circumference and between the AHI and the waist-hip ratio. There was no significant correlation between the AHI and PDSS, or the AHI and disease severity.
CONCLUSION: There was a high prevalence of SBD in our PD patients, which was comparable to other studies. Obstructive sleep apnoea was the dominant type of SBD. There was no correlation between the subjective sleep symptoms using the PDSS and the objective measurements using PSG.
Study site: Neurology clinic, Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM)
Leukoaraiosis (LA) is a term that defines an abnormal appearance of the subcortical white matter of the brain on neuroimaging. This study was done to evaluate the predictive value of LA in terms of mortality, disability and cognitive decline at three months post-stroke and also to identify the risk factors that are independently associated with LA in a stroke population.
Migraine is associated with a variety of personality traits. The objective of this study was to reevaluate the personality traits using Minnesota Multiphasic Personality Inventory-2 (MMPI-2) in migraine patients diagnosed by applying the new International Classification of Headache Disorders-2 criteria.
INTRODUCTION: Migraine is a common disabling condition that results in considerable socioeconomic loss. The role of non-steroidal anti-inflammatory drugs (NSAIDs) in acute migraine has been well-established. We compared the efficacy of the cyclooxygenase-2 inhibitor celecoxib with the NSAID, naproxen sodium, in the treatment of acute migraine.
METHODS: This was a randomised, open label, controlled trial. We selected patients with a diagnosis of migraine, based on the International Headache Society revised criteria. 60 patients were randomised to either celecoxib 400 mg (30 patients) or naproxen sodium 550 mg (30 patients). Patients took the study medicine for the first acute migraine episode that occurred during the study period and reported the headache reduction based on a visual analogue score (VAS). Patients were reviewed after a month to check on VAS at one and two hours, compared to the baseline. Any side effects of the medication were also recorded.
RESULTS: Of the 52 patients who completed the study, eight did not experience any headaches. The mean VAS in the celecoxib group improved significantly from baseline (6.48 +/- 1.53) to one hour (4.28 +/- 2.11) and two hours (2.24 +/- 2.57) (p-value is less than 0.0005). The mean VAS in the naproxen sodium group also improved significantly from baseline (7.30 +/- 1.66) to one hour (4.81 +/- 2.50) and two hours (2.63 +/- 2.65) (p-value is less than 0.0005). However, there was no significant difference between the magnitudes of improvement between the treatment groups. The incidence of gastric pain was significantly higher in the naproxen sodium group (p-value is equal to 0.029).
CONCLUSION: In comparison with naproxen sodium, celecoxib was equally effective in relieving pain in acute migraine and caused significantly less gastric pain.
Study site: neurology outpatient clinic in Pusat Perubatan
Universiti Kebangsaan Malaysia (PPUKM)
There is no biological marker that can accurately predict the prognosis after an acute ischaemic stroke. The main objective of this study was to evaluate the prognostic value of tissue factor (thromboplastin) levels in first ischaemic stroke.
There are several studies that reported a higher frequency of sleep-disordered breathing (SDB) among ischaemic stroke patients with increasing evidence linking SDB and cardiovascular complications. Many showed prevalence between 43 percent and 72 percent, taking the apnoea-hypopnoea index (AHI) equal to or greater than ten. The main objective of this study was to determine the frequency of SDB in recent ischaemic stroke patients admitted to Hospital Universiti Kebangsaan Malaysia (HUKM) and the relationship between SDB and known risk factors of ischaemic stroke.
A 41-year-old Malay housewife presented with recurrent chylous ascites and progressive cachexia over 17 years. A diagnosis of lymphangioleiomyomatosis (LAM) was established by laparotomy where biopsy of the liver, peritoneum and adhesions from previous surgery showed smooth muscle proliferation in the blood vessels and lymphatics. Clinically and radiologically, there was no evidence of pulmonary involvement. She was treated with dietary fat restriction and medium-chain triglycerides. This is the first case of LAM reported in Malaysia.
Calcinosis universalis (CU) is characterised by diffuse deposition of insoluble calcium salt in the skin, subcutaneous tissue or organs. Calcium deposits in the breast may be associated with an increased risk for developing breast cancer. We present a case of a 65-year-old woman diagnosed with CU secondary to undifferentiated connective tissue disease. She developed progressive calcification of her skin, which did not improve with oral medications aimed at reducing the calcification. Investigations to look for possible causes of calcification were all unremarkable. During follow-up, calcification was also found in both her breasts. Initial mammography was reported as fibroadenoma. However, 3 years later, she returned with metastatic breast cancer which presented with a massive pleural effusion of the right lung. Calcinosis universalis should now be considered as a risk factor for breast cancer.
Parkinson's disease (PD) is a chronic neurodegenerative movement disorder characterized by the progressive and massive loss of dopaminergic neurons by neuronal apoptosis in the substantia nigra pars compacta and depletion of dopamine in the striatum, which lead to pathological and clinical abnormalities. A numerous of cellular processes including oxidative stress, mitochondrial dysfunction, and accumulation of α-synuclein aggregates are considered to contribute to the pathogenesis of Parkinson's disease. A further understanding of the cellular and molecular mechanisms involved in the pathophysiology of PD is crucial for developing effective diagnostic, preventative, and therapeutic strategies to cure this devastating disorder. Preconditioning (PC) is assumed as a natural adaptive process whereby a subthreshold stimulus can promote protection against a subsequent lethal stimulus in the brain as well as in other tissues that affords robust brain tolerance facing neurodegenerative insults. Multiple lines of evidence have demonstrated that preconditioning as a possible neuroprotective technique may reduce the neural deficits associated with neurodegenerative diseases such as PD. Throughout the last few decades, a lot of efforts have been made to discover the molecular determinants involved in preconditioning-induced protective responses; although, the accurate mechanisms underlying this "tolerance" phenomenon are not fully understood in PD. In this review, we will summarize pathophysiology and current therapeutic approaches in PD and discuss about preconditioning in PD as a potential neuroprotective strategy. Also the role of gene reprogramming and mitochondrial biogenesis involved in the preconditioning-mediated neuroprotective events will be highlighted. Preconditioning may represent a promising therapeutic weapon to combat neurodegeneration.
Neuroinflammation is known as a key player in a variety of neurodegenerative and/or neurological diseases. Brain Toll-like receptors (TLRs) are leading elements in the initiation and progression of neuroinflammation and the development of different neuronal diseases. Furthermore, TLR activation is one of the most important elements in the induction of insulin resistance in different organs such as the central nervous system. Involvement of insulin signaling dysregulation and insulin resistance are also shown to contribute to the pathology of neurological diseases. Considering the important roles of TLRs in neuroinflammation and central insulin resistance and the effects of these processes in the initiation and progression of neurodegenerative and neurological diseases, here we are going to review current knowledge about the potential crosstalk between TLRs and insulin signaling pathways in neuroinflammatory disorders of the central nervous system.