A 10-year-old girl presented with progressive dystonia with diurnal fluctuation. Response to low dose L-Dopa was dramatic and sustained with no complications. Recurrence of symptoms was observed on attempted withdrawal. Because of the dramatic response to therapy, dopa-responsive dystonia must be considered in the differential diagnosis of disorders presenting as gait disorders in childhood.
We report a case of bladder outflow obstruction presenting with upper tract dilatation mistaken initially as pelviureteric junction (PUJ) obstruction. The lower tract obstruction ought to be dealt with first before upper tract obstruction is assessed because the renal pelvic pressure is significantly affected by vesical filling and high bladder pressure.
A 35-year-old Chinese man who was known to have insulin-dependent diabetes mellitus was admitted for fever and weight loss. During his hospital stay, he fell to his death from his ward at the twelfth floor. The clinical features, radiological findings and gross organ changes at autopsy closely simulated miliary tuberculosis. Histology, however, revealed extensive necrosis of the adrenal glands, lungs, spleen, kidneys and thyroid associated with the presence of Histoplasma capsulatum organisms. This case highlights the similarity both clinically and pathologically between histoplasmosis and tuberculosis and emphasizes the need to be aware of this infection in a nonendemic area among patients with a compromised immune system.
A large retroperitoneal liposarcoma presented clinically as an ovarian tumour in an infertile woman. The importance of computed tomography in the differential diagnosis and the appropriate management of retroperitoneal liposarcoma is presented with a review of the relevant literature.
It has been noted in the literature that Osteogenesis Imperfecta is frequently mistaken for non-accidental injury. This article serves to illustrate the difficulty in differentiating between the two conditions and that they can occur concomitantly in one patient.
Five cases of renal angiomyolipoma which underwent spontaneous rupture are described. These patients presented as an "acute abdomen" for which the diagnosis was not initially apparent. A high index of suspicion is required to make the diagnosis even with modern imaging techniques. The treatment of these tumours is discussed.
A 43-year-old lady with long standing non-insulin dependent diabetes mellitus on glibenclamide presented with cholestatic liver disease. Initially she was thought to have developed primary biliary cirrhosis (PBC). When she made a spontaneous recovery following the withdrawal of glibenclamide, it became obvious that the patient had been suffering from drug-induced chronic cholestasis (DICC). The subtle differences between PBC and DICC are highlighted.
A retrospective histological analysis of colonic biopsies received by the Department of Pathology, University of Malaya during the 4-year-period between 1990 and 1993 revealed nine cases of microscopic colitis (MC). The ages of the patients ranged from 18 to 53 years. Seven patients were females with a female to male ratio of 3.5 :1. The main clinical symptom was chronic diarrhoea of duration varying from 4 months to 5 years. None of the patients had any systemic illness or were on any prior medication. Colonoscopy and barium enema observations in all the subjects were essentially normal. Colonic biopsies showed diffuse plasmacytic infiltration of the lamina propria, intraepithelial lymphocytic infiltration and normal crypt pattern. To the best of our knowledge, this is the first documented report on MC from Malaysia. It is envisaged that better recognition of this condition by histopathologists would reduce the numbers in the often diagnosed category of "nonspecific colitis".
Monoclonal plasma cell proliferative diseases such as multiple myeloma and plasmacytoma can involve extramedullary sites at the time of first presentation, or subsequently in the course of the disease. Under such circumstances, they can mimic primary or metastatic carcinomas, neuroendocrine or neuroectodermal tumours and lymphomas, and the pathologist often has to resort to immunohistochemistry as an aid to diagnosis. We studied the morphology and immunohistochemical properties of 10 cases of previously confirmed monoclonal plasma cell proliferative lesions retrieved from the files of the Department of Pathology, University of Malaya. Serial 4u thick paraffin sections were stained with H&E, the Unna-Pappenheim technique for nucleic acid and a panel of antibodies using a standard immunoperoxidase technique. Light chain restriction was demonstrable in most of the cases. Seven (70%) showed kappa and 2 (20%) lambda light chain restriction. The remaining case was not stainable with most of the antibodies in the panel. The majority (80%) of cases showed accompanying IgG heavy chain in the cytoplasm, while 1 case had IgA. Seven (70%) showed membrane positivity with antibody to epithelial membrane antigen (EMA) and 7 (70%) cytoplasmic positivity with antibody to vimentin. This study enhances our awareness that neoplastic plasma cells can be positive for EMA and vimentin, and cautions us from misinterpreting these lesions as carcinomas or sarcomas. Notwithstanding that, immunohistochemical staining for kappa and lambda light chains can be helpful in differentiating monoclonal plasma cell proliferations from polyclonal ones.
A hitherto undescribed group of lesions consisting of cystic bony lesions, exostosis, fibromatous lesion, unilateral tonsillar hypertrophy, epidermoid cyst (cholesteatoma) and hyperplasia of the mandible confined to the left side of the face is reported. The case may represent a variant of the Proteus syndrome.
This study shows that out of 774 patients tested, the house dust mite (Dermatophagoides pteronyssinus) provoked the maximum response (51.81%) the house dust was second (42.81%), and shrimp was third (23.92%). The moderate allergenic extracts were cat fur (12.79%), dog fur (10.72%), cockroach (8.47%) egg white (7.56%) and orris powder (6.30%). Among the low allergenic extracts were Aspergillus fumigatus (5.38%), Staphyloccus aureaus (3.53%) and chicken feathers (3.18%). The authors are of the opinion that the skin sensitivity test is an important diagnostic tool in allergic rhinitis and since this is a preliminary study any short listing of allergens to be used is not recommended yet. Skin sensitivity tests is a useful tool for differentiating allergic rhinitis from vasomotor rhinitis. Estimation of allergen specific IgE concentration in the serum will not offer any major advantages over the intradermal skin test in determining the clinical significance of house dust mite allergy. 21 The development of local materials for skin testing may enhance the usefulness of these investigations.
Left atrial myxoma almost always arises in the inter-atrial septum. A case is described where it arose from the posterior wall of the left atrium. Clinical presentation was suggestive of mitral stenosis and sub-acute bacterial endocarditis and diagnosis was arrived at necropsy.
Two cases of Syndromes of Ophthalmoplegia , Ataxia and Areflexia were reported. A brief discussion on its clinical feature and differential diagnosis was made.
The case records of 64 patients with malaria over a five year period admitted to the University Hospital, Kuala Lumpur were examined. There were 32 cases of P. falciparum, 26 cases of P. vivax and two cases of mixed infections. Four cases of P. malariae were recorded. The clinical findings, biochemical and haematological parameters were examined for any indication of a pernicious syndrome. A high index of suspicion of a malarial infection may be based on the findings of anaemia, thrombocytopaenia, hyponatraemia, renal failure and abnormal liver function tests in the face of a negative blood film. These pernicious syndromes occur more often in malignant tertian malaria (anaemia 50%, hyponatraemia 39.1%) but a high percentage of the other malarial species show these abnormalities (P. vivax anaemia 57.7%, hyponatraemia 19.2%). When these abnormalities are present but blood films for malaria parasites are negative, repeat blood films are warranted until a parasitological diagnosis is achieved and correct treatment may be started.
We report a patient with unusual manifestations of hyperthyroidism which initially suggested lymphoma. The pathophysiology of these features in thyrotoxicosis is discussed. The need to consider thyrotoxicosis in an otherwise unexplained case of lymphoid hyperplasia will be stressed.
A patient with marfanoid habitus was admitted to the General Hospital, Kuala Lumpur in October 1985 for surgical closure of an atrial septal defect. He was suspected to have Marfan Syndrome but there was no involvement of the aorta nor the eye. The clinical features were intermediate between that of the Marfan Syndrome and the Ehler's Danlos Syndrome. It is suggested that this could be a separate distinct entity within the heritable disorders of connective tissue known as the Marfanoid Hypermobility Syndrome.