NADPH diaphorase histochemistry of the human hypothalamus.

The morphology and distribution of NADPH diaphorase reactive neurons was studied in the normal human hypothalamus. Reactive neurons were divided into three categories on the basis of perikaryal size. Small neurons (8-20 microns) were oval or fusiform, and pale staining. Intermediate neurons (20-30 microns) were fusiform, triangular or pyramidal with a wide range of staining intensity. Large neurons (greater than 30 microns) were triangular or pyramidal with moderate to dark staining. Reactive neurons were found in four major regions: medial preoptic, ventromedial, lateral, and perifornical. Scattered positive neurons were found in several other hypothalamic areas. Reactive fibers were present in the supraoptic decussation, medial forebrain bundle, and stria medullaris thalami. The localization of NADPH diaphorase neurons in hypothalamic nuclei affected by Alzheimer's disease and other degenerative disorders suggests that further studies of this neuronal subset are warranted.

Angiocentric T-cell lymphoma of the pancreas presenting as late-onset diabetes mellitus with diabetic retinopathy.

Pancreatic lymphoma presenting with clinical diabetes mellitus (DM) is rare. We report angiocentric T-cell lymphoma of the pancreas in a 65-year-old Thai woman who presented with progressive deterioration of visual acuity of both eyes. She had diabetic retinopathy (DR) diagnosed only 20 months after the diagnosis of DM at the age of 63. She later developed erythematous rashes, fever, and deterioration of consciousness; she eventually died of shock. A skin biopsy and bone marrow examination revealed angiocentric T-cell lymphoma. At autopsy, the pancreas and both eyes were extensively infiltrated by lymphoma. Widespread involvement of nearly all organs but superficial lymphadenopathy was detected. In contrast to other typical cases of long-standing DM, only mild atherosclerosis was noted, and no DR was found. To the best of our knowledge, this is the first case of lymphoma involving the pancreas and both eyes producing clinical DM and DR.

Spontaneous granulomatous amebic encephalitis: report of four cases from Thailand.

Granulomatous amebic encephalitis (GAE), or meningoencephalitis caused by Acanthamoeba sp and leptomyxid amebae are uncommon CNS infections that usually occur in an immunocompromised host. From 1990 to 1992, 4 patients with GAE were treated at Siriraj Hospital, Bangkok. One case was diagnosed antemortem, from a brain biopsy. The other three cases were diagnosed as GAE postmortem. Pathological findings included acute and subacute granulomatous inflammation with extensive cerebral necrosis, angiitis, fibrinoid necrosis and fibrin thrombi. One patient had a chronic skin ulcer in which free-living amebic trophozoites were found. No visceral involvement was observed. All patients developed "spontaneous" GAE, but we suspect an undiagnosed abnormality in cell mediated immunity or a deficient humoral immune response.

Mitochondrial genome analysis in Kearns-Sayre syndrome.

We analysed the mitochondrial genome of one patient with chronic and progressive bilateral ophthalmoplegia. This patient also had abnormal EKG showing cardiac conduction defects and pigmentary retinopathy, suggestive of the Kearns-Sayre syndrome. On muscle biopsy, with Gomori trichrome stain, the fibers showed an increase in red-staining material in the intermyofibrillary network and the subsarcolemmal region. On electron microscopy, aggregations of abnormal mitochondria were demonstrated, confirming the diagnosis of mitochondrial myopathy. Analysis of mitochondrial DNA (mtDNA) from the patient and her mother showed no deleted mtDNA.

Pasteurella multocida infective endocarditis: a case report.

A 17-year-old man presented with acute febrile illness with jaundice, embolic skin lesion, heart murmur, renal insufficiency and abnormal CSF. Pasteurella multocida was isolated from blood cultures. In spite of adequate antibiotic treatment for endocarditis of the mitral valve, he developed a fatal ruptured cerebral mycotic aneurysm. Post mortem examination revealed an atrial septal defect, vegetation at the anterior mitral leaflet, intraventricular, subarachnoid and intracerebral hemorrhage.

Bilateral high radial nerve compressions: a case report.

A 40-year-old woman with bilateral high radial nerve compressions by non-traumatic cause was reported. It occurred first at the right radial nerve which was explored after a period of investigation and conservative treatment. Two constricted sites 2.0 cm apart of the right radial nerve crossed by branches of the radial collateral artery beneath the lateral head of the triceps were found. The constricted sites including tissue in between was resected and replaced with a sural nerve graft. One year later the patient had the same episode on the left side. The operative finding was the same as the previous one. Sural nerve graft was performed after neurolysis had failed. The patient's normal radial nerve function returned in one year. This is the first reported case in the literature of bilateral high radial nerve compressions by branches of the radial collateral artery.

Brainstem germinoma presenting as intermittent apnoea: a rare entity: report of one patient and review of literature.

A 17-year-old man who had repeated pneumonia and presented with intermittent apnoea was described clinically and pathologically. Neurological examination and computed axial tomography revealed a calcified mass over the lower brainstem. Neurological intervention was done with removal of the tumour and the pathological section showed the classical picture of germinoma. The patient was given irradiation treatment afterwards and he was weaned off the respirator within a week. Unfortunately, he died because of recurrent chest infection after a long hospital admission of 92 days. Reviewing the literature showed the rarity of the germinoma at the brainstem location.

Successful medical treatment of multiple cryptococcomas: report of a case and literature review.

We report a 35-year-old man diagnosed as having CNS cryptococcosis with multiple cryptococcomas, presenting with headache, papilloedema and impaired mental function in a previously healthy man. Cerebrospinal fluid (CSF) examination revealed lymphocytic pleocytosis with low glucose level. Gram's stain, acid fast bacilli stain and Indian ink examination were all negative. CSF cryptococcal antigen was positive, however, several fungal cultures were negative. Early cranial CT scan showed focal cerebritis over the right temporal lobe while subsequent imaging studies showed multiple contrast-enhancing masses with severe surrounding brain oedema over bilateral frontoparietal areas. Brain biopsy showed cryptococcal granulomatous lesions. Treatment was successful with antifungal agents and steroids without surgical removal.

Ketoconazole and Flutamide in the treatment of disseminated intravascular clotting from prostate cancer: a case report and review.

Disseminated intravascular clotting (DIC) is a well-recognized complication of malignancy. Prostatic cancer can produce chronic DIC as well as acute severe DIC. Treatment of DIC are general supportive measures including heparin, transfusion of blood, platelets and clotting factors, but the most important aspect is correction of underlying malignant diseases i.e. cancer of the prostate gland. For metastatic prostatic cancer presenting with an emergency oncologic condition, the treatment of choice is surgical orchiectomy, but surgery may not be possible in the presence of severe DIC. Ketoconazole and Flutamide are drugs with different mechanisms for hormonal manipulation of this cancer. Due to severe DIC, we combined both drugs trying to put maximum therapeutic effect on this life threatening profound DIC patient.

The analysis of peri-tumor necrosis following the subcutaneous implantation of autologous tumor cells transfected with an episome transcribing an antisense insulin-like growth factor 1 RNA in a glioblastoma multiforme subject.

A subject inflicted with glioblastoma multiforme who received partial tumor resection and radiotherapy was recruited for an ex vivo gene therapy protocol using irradiated autologous tumor cells that had been engineered to suppress the expression of insulin-like growth factor I as the tumor vaccine. After subcutaneous injection for 8 weeks, the subject developed peri-tumor necrosis with mass effect. The authors wondered whether this event could have resulted from the tumor vaccine. The tissue section bordering the necrotic tumor tissue to the viable normal tissue was examined for nature of any infiltrated cells and their activities. Lymphocytes, macrophages, and a small number of neutrophils diffused into the necrotic tumor tissue were found. The infiltrated lymphocytes consisted of both CD4+ and CD8+ T cells. The functional activity of these lymphocytes was demonstrated by the active production of interferon y and tumor necrosis factor alpha based on the respective immunofluorescent staining localized to these cells. This finding is compatible with the proposed mechanism underlying the tumor vaccination. However, the contribution of radiation treatment to this event cannot be clearly ruled out.

An unusual family with Leber's hereditary optic neuropathy and facioscapulohumeral muscular dystrophy.

We performed an observational prospective analysis to study the clinical characteristics as well as a molecular genetic analysis of 17 members of a Thai family who had visual loss and/or muscle weakness. Their blood mitochondrial DNA were examined for the presence of the G11778A Leber's hereditary optic neuropathy (LHON) mutation. Facioscapulohumeral muscular dystrophy (FSHD) DNA analysis was performed in four members who had visual loss. Of 17 family members, the eight members who had the 11778 LHON mutation were all from branch 'a'. Three of these eight members had FSHD with a 17-27-kb deletion of a tandem repeat in the 4q35 subtelomere, and two had been clinically diagnosed as FSHD. Four of six examined members in branch 'b' showed muscular dystrophy clinically diagnosed as FSHD. No correlation of blood DNA analysis between LHON and FSHD in affected members was found. We describe the first family with FSHD and G11778A LHON in which a mutation in mitochondrial DNA at nucleotide position 11778 of branch 'a' was found to be the origin of the mutation.

Microglial and neural differentiation in human teratomas.

Ten human teratomas arising outside the central nervous system (CNS) were studied using a panel of immunohistochemical, and lectin histochemical stains to determine the relationship of the presence of microglia to markers of neural maturity or differentiation. Microglia, identified by silver carbonate, Ricinus communis agglutinin-1 (RCA-1), or both were found in eight out of ten cases. They were common in mature areas which also had S-100, glial fibrillary acidic protein, vimentin, neurofilament, and synaptophysin immunostaining. Microglia were distinguished from macrophages in necrotic foci. Cells which were RCA-1 positive and silver carbonate positive were found in immature neural tissues but these lacked all typical features of microglia. These observations indicate that true microglia are frequent in nonCNS teratomas and that they are found in association with other indicators of neural maturation. The presence of possible precursors in immature areas suggests that microglia undergo maturation concurrent with neural differentiation in these tumors.

Primary thyroid carcinoma and thyrotoxicosis.

A 60-year-old man noticed rapid enlargement of a long-standing thyroid goitre, with dysphagia and difficulty in breathing. Thyrotoxicosis was diagnosed. Chest X-ray revealed multiple pulmonary metastases. He underwent near-total thyroidectomy. The histopathology revealed an undifferentiated thyroid carcinoma with some areas of papillary carcinoma and its follicular variant. Postoperative 131I total body scan showed residual thyroid tissue in the neck and one functioning metastasis in the right rib, posteriorly. The patient's condition deteriorated rapidly and he died from pneumonia. The autopsy showed widespread metastases of undifferentiated thyroid carcinoma. Only the right rib contained the follicular variant of papillary carcinoma.

A unique 3.5-kb deletion of the mitochondrial genome in Thai patients with Kearns-Sayre syndrome.

Kearns-Sayre syndrome is one of the neurological diseases caused by a defect in the energy-producing system of mitochondria. Keams-Sayre is known to be associated with a deletion in the mitochondrial genome and is usually detected in muscle biopsies of the patients. In this study, we report the molecular lesion of mitochondrial DNA (mtDNA) in four Thai patients admitted to hospital with encephalomyopathies. The 3.5-kb deletion of mtDNA was detected by Southern analysis, mapped by amplification with five primer pairs covering almost the total mitochondrial genome, and confirmed by PCR primer shift analysis. The deleted position was localized to nt 10208/13765 or nt 10204/13761 spanning the coding area of subunits 3 (ND3), 4L (ND4L), 4 (ND4), and 5 (ND5) of respiratory chain enzyme complex I and the tRNA genes for histidine, serine, leucine, and arginine. The sequence flanking the deletion was a 4-bp repeat of TCCC. All four patients have exactly the same 3558-bp mtDNA deletion; this is the only deleted position in their mtDNA but is different from those reported in the literature. The deletion seems to be found only in Thai patients, although they present with different clinical manifestations and none of them is not related.