We report two patients with myopathic dropped head syndrome, a rare and interesting neuromuscular syndrome characterised by a predominant weakness of the neck extensor muscles. The first patient, a middle aged Chinese man, presented with progressive weakness of neck extension but his clinical course later stabilised despite a lack of response to corticosteroids. Muscle biopsy revealed a necrotising myopathy with no evidence of inflammation. This patient supports the existence of an idiopathic restricted non-inflammatory myopathy, a so called isolated neck extensor myopathy syndrome which is recognised to pursue a less progressive, more benign course. Our second patient had histopathological evidence for polymyositis; there was a favourable response to steroids. Our cases underscore the fact that there may be a spectrum of pathological processes associated with the myopathic dropped head syndrome ranging from non-inflammatory muscle necrosis to a full blown inflammatory myositis.
We present a case of cervical necrotising fasciitis in a 56 year old man, secondary to a rare mastoid infection. The patient had coexisting diabetes mellitus and hypertension. He was treated with early surgical debridement followed by neck and chest reconstruction and radical mastoidectomy. Aggressive antibiotic therapy and supportive care was given. He recovered well with minimal residual functional deficit.
A 49 year-old Indian housewife was diagnosed with Hodgkin's disease in 1995. She was given combination chemotherapy comprising Chlorambucil, Vincristine, Procarbazine and Prednisolone. Unfortunately she defaulted after two courses of chemotherapy. One year later, she developed progressive right knee swelling and pain, associated with loss of appetite, loss of weight, intermittent fever, night sweats and pruritus. The right knee swelling measured 15 cm x 20 cm and was warm and tender. A plain radiograph of the right knee revealed osteolytic lesions at the distal end of the right femur and the proximal ends of the right tibia and fibula, associated with gross periosteal reaction and soft tissue swelling. Apart from left cervical lymphoadenopathy, examination of other systems was unremarkable. Pelvic bone marrow biopsy was inconclusive. An open biopsy of the lower end of the right femur was consistent with Hodgkin's disease. She was given salvage combination therapy comprising Chlorambucil, Vincristine, Procarbazine, Prednisolone, Doxorubicin, Bleomycin and Vinblastine. She tolerated the treatment well and responded with significant reduction in the swelling and pain of the right knee. Unfortunately, she again defaulted treatment after 2 courses of chemotherapy. This case illustrates an unusual presentation of Hodgkin's disease in relapse.
The Nipah virus outbreak represented one of several bat-derived paramyxoviruses that has emerged during the last decade to cause severe human and animal disease. The pathogenesis of Nipah infection is associated with its ability to infect blood vessels and extravascular parenchyma in many organs, particularly in the central nervous system. The clinical manifestations of acute Nipah infection range from fever and mild headache to a severe acute encephalitic syndrome in which there is a high mortality. Much remains to be understood about this new disease, including its intriguing ability to cause relapsing encephalitis in some survivors. This review provides an overview of the Nipah outbreak, focussing on what is presently known about it as an infectious disease, including the clinical aspects, pathology and pathogenesis.
Neurotization of the brachial plexus is an established procedure in the upper limb. However, neurotization of the lower limb remains experimental. Brunelli reported the use of the ulnar nerve to neurotize the lower limb. Zhao et al reported the use of intercostals nerve to neurotize the lower limb in rats. The aim of the study was to determine the feasibility of using intercostals nerve to neurotize the femoral nerve in human cadavers and to ascertain the ideal intercostals nerve that has the anatomical course that suit this role. Six fresh cadaveric dissection were performed through an extensile midline incision in the postmortem room and the lower six (T7-T12) were identified and traced from their origin. Their length and course identified and charted. T9 and T10 intercostal nerve was thought to be the most suitable nerve as the donor nerve, and the T11 and T12 intercostal nerve could not be free from its intra-muscular course sufficiently to be use as donor.
A 27-year-old Indian woman at 23 weeks' gestation presented with decompensated liver cirrhosis, coagulopathy, restrictive lung disease with cor pulmonale and preeclampsia. She was diagnosed to have sea-blue histiocyte syndrome (SBHS) at the age of 13 years and was treated conservatively. There was worsening liver, respiratory and bone marrow function as the pregnancy progressed. She underwent a successful pregnancy despite her poor medical condition and advanced disease state. We described the first case of familial SBHS in a pregnant patient from Asia.
Malignant pelvic tumours often present late, hence a high index of suspicion should be maintain in order to arrive at the diagnosis. This is particularly true for those who have unusual symptoms. A proper planning and staging strategies is required to save the limb, and the limb salvage surgery is at present the surgery of choice to achieve local control and restoring optimum functions of the lower limbs as being illustrated by our three cases.
A 43-year-old woman developed abdominal pain and diarrhea following a travel to Malaysia. Examinations in another hospital proved no evidence of infection, an empirical antibiotic therapy with ciprofloxacin yielded no benefit. One and a half year later, the patient was admitted to our department because of persistent diarrhea and wasting. Laboratory tests showed megaloblastic anemia, folate deficiency and steatorrhea. Stool specimens for bacterial pathogens and parasites were negative. Endoscopy and biopsy from the distal portion of the duodenum revealed broadening and shortening of the villi and an increased infiltration of the lamina propria by chronic inflammatory cells (plasma cells and lymphocytes). In conclusion, diagnosis of tropical sprue was established. The therapy comprised of tetracycline for six weeks and folic acid for six months. Subsequently, the diarrhea disappeared, the patient continuously gained weight and was free of any complaints. The complete remission following this regimen proved the suspected diagnosis. Differential diagnosis in any patient who recently returned from the tropics may be a challenge. Tropical sprue predominantly occurs during or after a longer stay in endemic areas. However, if chronic diarrhea and signs of malabsorption develop after a short journey to India, South-East Asia and parts of the Caribbean, tropical sprue has to be considered, too.
The case of a minor salivary gland tumour, arising from the tongue, with nodal metastasis is presented. Biopsy of the tumour and fine-needle aspiration cytology of the neck swelling showed the presence of a clear cell carcinoma with evidence of nodal metastases. A commando operation was performed and the defect was reconstructed using a local tongue flap. The literature review indicated that the neoplasm was rare and its site of occurrence rather unusual.
A body recovered from the water does not necessarily imply that death was due to drowning. The diagnosis of drowning is discussed together with the significance of the "diatom" and biochemical tests.
Matched MeSH terms: Drowning/pathology*; Respiratory System/pathology*; Near Drowning/pathology
A case is reported of retroperitoneal malignant fibrous histiocytoma (MFH) invading into the colon, causing fresh bleeding per rectum. It illustrates the difficulty encountered in the pre-operative diagnosis of this condition, especially in a patient with an atypical presentation of profuse lower gastrointestinal bleeding.
Two groups of three rabbits each were infected with 250 cercariae of the Baling and Koyan strain of Schistosoma malayensis. Changes induced by both strains included periportal hepatocellular necrosis and fibrosis. Vascular changes such as portal phlebitis and thrombophlebitis and varying degrees of pericholangitis were also present. Amyloid deposition was noted. A comparative study of the changes induced in rabbits by S. malayensis, S. mekongi and S. japonicum showed that the hepatic lesions induced by the Baling strain of S. malayensis were similar to that induced by S. japonicum, and were more severe than that induced by S. mekongi or the Koyan strain.
Since it was first described in 1978 the abdominal cocoon continues to be a rare cause of intestinal obstruction. So far this rare condition where the small intestine is encased in a fibrous membrane has been reported only in females. Diagnosis is usually made at laparotomy and the treatment of choice is lysis of adhesions. Proper recognition of this benign condition will result in the correct management of it and prevent unnecessary bowel resections. Five new cases including one male patient, together with a review of previous reports in the English literature, are presented.
Eight cases of clear cell sarcoma of kidney were seen in the Department of Pathology, University Hospital, Kuala Lumpur, Malaysia over the 16-year period from 1973 to 1989. Five of the patients were males. Six patients were Malay, one Chinese and one Indian. The patients' ages ranged from 8 months to 3 years. Clear cell sarcoma was the original diagnosis in two patients while six were diagnosed as blastemal-predominant Wilms' tumours at presentation. Metastases developed in five patients. Metastatic sites included the thoracic vertebra, skull, orbit, humerus, radius, ulna, shoulder, lung and liver. The prolonged survival, of 9 years and 9 months, seen in one patient despite omission of Adriamycin (doxorubicin) from the chemotherapeutic protocol is highlighted. We also emphasise the histological factors which are of help in differentiating clear cell sarcoma from Wilms' tumour.
Vulvar ulceration is a rare manifestation of histiocytosis X. A 13-year-old girl had a nonhealing vulvar ulcer for 1 year. She had been in remission from histiocytosis X and the ulcer was not recognised as a sign of disease recurrence until tissue biopsy was obtained for histopathological and immunohistochemical studies. This article stresses the importance of establishing an accurate diagnosis when chronic vulvar ulcers are encountered and reviews the literature on this uncommon presentation of histiocytosis X.
From 1988 to 1990, eighty operations on eighty knees of seventy-nine patients with a clinical diagnosis of meniscal tear was entered into a prospective study. The clinical diagnosis was correct in 76% of patients. Excellent or good results were achieved in 90% of knees which had only meniscal lesions but dropped to 70.5% when other intraarticular pathologies coexisted with the meniscal lesions. Excellent or good results were achieved in 71% of the knees in the presence of Anterior Cruciate Ligament (ACL) deficiency.
Continent pouch ileostomy is fashioned for patients who need a proctocolectomy. It is usually indicated for cases of ulcerative colitis and familial adenomatous polyposis where the anal sphincter can no longer maintain normal function or has to be removed. A case of familial adenomatous polyposis with features of Gardner's syndrome is reported. The patient presented with carcinoma of the rectum. Abdominoperineal resection followed by completion pancolectomy was performed. A continent pouch ileostomy was fashioned for him. He resumed work as a labourer within six months. The pouch was troublefree, needed to emptied four to six times a day and was fully continent of fluid, flatus and faeces until his demise three years later from liver secondaries. Continent pouch ileostomy is a better alternative than permanent end ileostomy. All care should be taken to rule out the existence of Crohn's disease. It is proposed that continent pouch ileostomy should be offered to patients needing permanent ileostomy if the expertise is available.
A review of gestational trophoblastic disease diagnosed at the Department of Pathology, University Hospital, Kuala Lumpur from January 1989 to December 1990 using established histological criteria showed 25 complete hydatidiform moles (CHM), 11 partial hydatidiform moles (PHM), 1 invasive mole and 2 choriocarcinoma. The ages of the patients with CHM ranged from 21 to 43 years (mean = 28.5 years) and PHM 20 to 33 years (mean = 27.5 years). The invasive mole occurred in a 42-year-old Malay woman. The two patients with choriocarcinoma were both Chinese and 41 and 46-years old respectively. During the same period, 1,062 non-molar abortions and 13,115 births, inclusive of livebirths and stillbirths were recorded at the University Hospital. The incidence rate of hydatidiform moles was thus estimated to be 1:384 pregnancies. PHM constituted 30% of all molar pregnancies. Hydatidiform moles occurred among the Malays, Chinese and Indians at the rate of 2.43, 2.66 and 3.29 per 1,000 pregnancies respectively. It appears that hydatidiform molar pregnancy has the highest prevalence among the Indians, a finding similar to an earlier Singapore study.