Cerebral infarct mimicking glioma in Sjogren’s syndrome

ABSTRACT

Hong Kong Med J 2002;8:292-4 | Number 4, August 2002
CASE REPORT
Cerebral infarct mimicking glioma in Sjogren’s syndrome
MS Koh, KYC Goh, C Chen, HS Howe
Department of Neurosurgery, Singapore General Hospital, Outram Road, Singapore
 
 
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.
 
Key words: Cerebral infarction; Glioma; Magnetic resonance spectroscopy; Sjogren’s syndrome
 
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Inferior vena caval filters following deep vein thrombosis in patients with ruptured intracranial aneurysm

ABSTRACT

Hong Kong Med J 2002;8:288-90 | Number 4, August 2002
CASE REPORT
Inferior vena caval filters following deep vein thrombosis in patients with ruptured intracranial aneurysm
KC Wong, R Boet, WS Poon, S Yu
Division of Neurosurgery, Department of Surgery, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong
 
 
Anticoagulant therapy is highly effective and prevents death in more than 95% of patients who have suffered pulmonary embolism following deep vein thrombosis. Inferior vena caval filters provide an alternative to full anti-coagulation in those patients at highest risk of catastrophic haemorrhagic complications. We report on two patients who developed deep vein thrombosis following ruptured unsecured intracranial aneurysms. Inferior vena caval filters were inserted prior to aneurysm clipping, as a prophylactic measure to prevent pulmonary embolism while awaiting surgery. The patients did not receive anticoagulant therapy following successful clipping of the aneurysm. Both patients had clinical resolution of the lower limb swelling and no radiological evidence of propagation of the thrombus. During 18 months of follow-up there were no complications and no evidence of post-thrombotic syndrome.
 
Key words: Intracranial aneurysm; Vena cava filters; Venous thrombosis
 
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Increased levels of platelet-associated immunoglobulin G in a patient with mixed connective tissue disease

ABSTRACT

Hong Kong Med J 2002;8:285-7 | Number 4, August 2002
CASE REPORT
Increased levels of platelet-associated immunoglobulin G in a patient with mixed connective tissue disease
Y Shimizu, Y Nojima, M Mori
Department of Internal Medicine, Nanokaichi Municipal Hospital, 643 Nanokaichi, Tomioka, Gunma 370-2343, Japan
 
 
We report the case of a 71-year-old Japanese woman with mixed connective tissue disease and increased levels of platelet-associated immunoglobulin G. After administration of oral prednisolone, platelet-associated immunoglobulin G levels decreased with a simultaneous increase in the number of platelets, suggesting that the thrombocytopenia observed in this patient was mediated by immune mechanisms. This is the first reported case of increased platelet-associated immunoglobulin G levels in a patient with mixed connective tissue disease.
 
Key words: Immunoglobulin G; Mixed connective tissue disease; Prednisolone; Scleroderma, systemic; Sjogren’s syndrome
 
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Infantile isolated sulphite oxidase deficiency in a Chinese family: a rare neurodegenerative disorder

ABSTRACT

Hong Kong Med J 2002;8:279-82 | Number 4, August 2002
CASE REPORT
Infantile isolated sulphite oxidase deficiency in a Chinese family: a rare neurodegenerative disorder
KY Chan, CK Li, CK Lai, SF Ng, AYW Chan
Department of Paediatrics and Adolescent Medicine, Princess Margaret Hospital, 2-10 Princess, Margaret Hospital Road, Kowloon, Hong Kong
 
 
We report the clinical, biochemical, neuroradiological, and neurophysiological findings of a 4-year-old Chinese girl with infantile isolated sulphite oxidase deficiency. This is the first reported case in our locality. She presented at the age of 5 months with refractory seizures and developmental regression, and progressed rapidly to profound psychomotor retardation, spasticity, dystonia, microcephaly, and blindness. At the age of 3.5 years, she was admitted to the intensive care unit with septic shock. Ophthalmologic examination at this time revealed bilateral dislocation of the lens. Diagnosis of this very rare disorder was made on the basis of increased levels of urinary sulphite, thiosulphate, and sulphocysteine; normal urine xanthine and hypoxanthine; normal plasma uric acid; and low plasma cystine levels. The diagnosis was confirmed by the absence of sulphite oxidase activities in skin fibroblasts. Isolated sulphite oxidase deficiency is a rare inborn error of sulphur metabolism that is difficult to diagnose on clinical features and routine metabolic tests. The presence of ectopia lentis, seizures, and progressive neurological abnormalities should alert clinicians to the diagnosis.
 
Key words: Cerebral palsy; Lens subluxation; Metabolism, inborn errors; Seizures; Sulfites
 
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Paediatric stroke: case series

ABSTRACT

Hong Kong Med J 2002;8:216-20 | Number 3, June 2002
CASE REPORT
Paediatric stroke: case series
WW Cheng, CH Ko, AKH Chan
Department of Paediatrics, Caritas Medical Centre, 111 Wing Hong Street, Shamshuipo
 
 
Five cases of paediatric stroke are reported. Two patients presented with idiopathic stroke, another following vertebral artery dissection, one second-ary to Moyamoya disease, and one patient with the syndrome of mitochon-drial encephalopathy, lactic acidosis, and stroke-like episodes. The presentation, investigation, and management of paediatric stroke are discussed.
 
Key words: Cerebrovascular accident; Moyamoya disease; Pediatrics; Vertebral artery dissection
 
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Malassezia furfur fungaemia in a ventilator-dependent patient without known risk factors

ABSTRACT

Hong Kong Med J 2002;8:212-4 | Number 3, June 2002
CASE REPORT
Malassezia furfur fungaemia in a ventilator-dependent patient without known risk factors
CM Chu, RWM Lai
Department of Medicine and Geriatrics. United Christian Hospital, 130 Hip Wo Street, Kowloon, Hong Kong
 
 
Malassezia furfur is the lipophilic yeast which causes tinea versicolor and is an uncommon cause of fungaemia. It usually occurs in the context of hyperalimentation with lipid emulsion, immunosuppression, or the presence of a central venous catheter. We report a case of a ventilator-dependent patient who developed Malassezia furfur fungaemia in the absence of these known risk factors. A likely risk factor in this patient was receipt of multiple courses of broad-spectrum antibiotics. This case highlights the importance of recognising Malassezia furfur as a cause of fungaemia, as well as the need for special culture techniques to aid identification.
 
Key words: Fungemia; Malassezia; Risk factors
 
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Ammonia detoxification by continuous venovenous haemofiltration in an infant with urea cycle defect

ABSTRACT

Hong Kong Med J 2002;8:207-10 | Number 3, June 2002
CASE REPORT
Ammonia detoxification by continuous venovenous haemofiltration in an infant with urea cycle defect
WKY Chan, WM But, CW Law
Department of Paediatrics, Queen Elizabeth Hospital, 30 Gascoigne Road, Hong Kong
 
 
We report the case of a newborn baby with carbamoyl phosphate synthetase deficiency. He presented at 2 weeks of life, deteriorating to a state of hyperammonaemic coma and respiratory failure. Rapid detoxification was successfully achieved by continuous venovenous haemofiltration while a definitive diagnosis and treatment were determined. The ammonia clearance achieved by continuous venovenous haemofiltration was greater than 20 mL/min/m2 , which is superior to that achieved by peritoneal dialysis and arteriovenous haemofiltration in this age-group.
 
Key words: Carbamoyl-phosphate synthase I deficiency disease; Hemofiltration; Infant
 
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Lytic skull metastasis secondary to thyroid carcinoma in an adolescent

ABSTRACT

Hong Kong Med J 2002;8:149-51 | Number 2, April 2002
CASE REPORT
Lytic skull metastasis secondary to thyroid carcinoma in an adolescent
GKC Wong, R Boet, WS Poon, HK Ng
Division of Neurology, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong: Department of Surgery
 
 
We report an unusual case of skull metastasis secondary to thyroid carcinoma in an adolescent girl. The 18-year-old presented with an occipital scalp swelling of 5 years’ duration. She reported having thyroid surgery in mainland China 10 years previously. Radiological investigations on presentation demonstrated a lytic hypervascular skull lesion. Preoperative angiography and embolisation was followed by surgical excision. Pathological examination showed the lesion to be a thyroid carcinoma with a predominantly follicular pattern and a completion hemithyroidectomy was subsequently performed. Computed tomography of the thorax showed small micronodules in both lung fields compatible with metastases. The patient was given whole body iodine-131 internal radiation treatment and subsequently commenced thyroid-stimulating hormone–suppressive treatment with thyroxine. The management of thyroid carcinoma and subsequent skull metastasis in children and adolescents is reviewed and controversial points are highlighted.
 
Key words: Neoplasm metastasis; Occipital bone; Skull neoplasms; Thyroid neoplasms
 
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Treatment of a ruptured iliac aneurysm with an endoluminal stent graft

ABSTRACT

Hong Kong Med J 2002;8:145-8 | Number 2, April 2002
CASE REPORT
Treatment of a ruptured iliac aneurysm with an endoluminal stent graft
ACW Ting, SWK Cheng, KF Kwok
Division of Vascular Surgery, Department of Surgery, The University of Hong Kong, Queen Mary Hospital, 102 Pokfulam Road, Hong Kong
 
 
The management of a 79-year-old man presenting with a ruptured common iliac aneurysm is described. The patient had multiple medical problems including hypertension, ischaemic heart disease, and atrial fibrillation, as well as a left hemispheric stroke 5 years previously. Traditional open surgery was judged unsuitable in this case because of the predicted poor outcome. The patient was subsequently treated successfully with endoluminal stent grafting.
 
Key words: Aneurysm, ruptured; Aortic aneurysm, abdominal; Iliac aneurysm; Stents
 
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Pulmonary embolism presenting as disseminated intravascular coagulation

ABSTRACT

Hong Kong Med J 2002;8:142-3 | Number 2, April 2002
CASE REPORT
Pulmonary embolism presenting as disseminated intravascular coagulation
SSL Choi, SY Pang, WP Mak, A KO
Department of Medicine and Geriatrics, Caritas Medical Centre, 111 Wing Hong Street, Shamshuipo, Kowloon, Hong Kong
 
 
We report an unusual case of disseminated intravascular coagulation. Occult pulmonary embolism is a recognised cause of disseminated intravascular coagulation. Unexplained shock should prompt the physician to search for a thrombotic cause such as pulmonary thromboembolism.
 
Key words: Disseminated intravascular coagulation; Pulmonary embolism
 
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