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Query: UMLS:C0018681 (headache)
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Primary malignant melanoma of the cerebral nervous system is an uncommon entity. A case of primary intracranial melanoma associated with dermoid cyst is reported. The patient was a 40-year-old man who was admitted to our hospital on May 2, 1979 complaining of headache, double vision, left hemiparesis and Jacksonian seizure. On admission he had a slight degree of papilledema. Left abdominal reflex was absent. Lumbar puncture showed high pressure of the cerebrospinal fluid which was otherwise normal. Plain skull films showed a calcification in the pineal region. CT scan demonstrated multiple high density areas with slight enhancement after contrast medium infusion in the right fronto-parietal region and a well defined high density area associated with an irregular low density area without contrast enhancement in the right pulvinar and the atrium and temporal horn of the right lateral ventricle. However, no tumor stain was seen on angiograms. On May 8, 1979 partial removal of the tumor was performed. Areas of black discoloration were seen in the leptomeninges of the right fronto-parietal lobes. Histopathological examination of this tumor revealed primary intracranial melanoma. Postoperatively DTIC, ACNU and Vincristine were administered intravenously but the tumor showed only little sensitivity. He died 5 months after operation. Autopsy was performed on October 8, 1979. Malignant melanoma in the right fronto-parietal lobes was invading the cerebellar hemisphere and spinal cord, but any tumor was not found in any other parts of the body except for this central nervous system. The tumor in the right pulvinar extending to the atrium and temporal horn of the right lateral ventricle was a dermoid cyst with calcification histopathologically. A case of primary intracranial melanoma associated with dermoid cyst has not been reported in any other associated with dermoid cyst has not been reported in any other literature yet.
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PMID:[An autopsy case of primary intracranial melanoma associated with dermoid cyst (author's transl)]. 709 73

Human ehrlichiosis is a newly recognized tick-borne disease. Since 1935 Ehrlichia canis has been known as a cause of illness in dogs and other canine species, and for a few years it was related with human disease. In 1990, Ehrlichia chaffeensis was isolated from a man suspected of having ehrlichiosis. Partial sequencing of the rRNAS from the human isolate and E. canis, indicated that they are 98.7% related. More recently (May 1994) an "human granulocytic ehrlichiosis" have been reported in USA. PCR amplification and sequence of 16S rDNA, showed that the human isolate was virtually identical to those reported for E. phagocytophila y E. equi, organisms that cause ehrlichiosis in rumiant and in horses. Most patients shows fever, headache, malaise, nausea or vomiting, anorexia and in a minority of cases rash is present. Some of them have complications such as pulmonary infiltrates, gastrointestinal problems, renal dysfunction or failure, hepatoesplenomegaly, neurologic abnormalities, DIC and some times death. Leucopenia, thrombocytopenia and elevated liver enzyme values have been common findings. Tetracycline and cloramphenicol have been using in adults and children as especific theraphy.
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PMID:[Human ehrlichiosis. Review]. 773 23

From January 1986 to April 1991, 100 consecutive patients with APL received oral ATRA at a dose of 60-100 mg/d alone or in combination with chemotherapy. In 84 cases treated with ATRA, 74 (88.1%) achieved CR; in the 16 cases treated with combined therapy, the CR Rate was 75%. Among the 50 patients followed up for a median of 36 months, 10 used ATRA (Group B) as continuation therapy, 10 chemotherapy (Group C), and 30 cases ATRA and chemotherapy alternatively (Group A). The mean survival was 8, 9, 21 months, respectively. For the 29 cases who died, the overall 3-year survival rate was higher in the group A (46.7%) than in the group B and C. ATRA did not provoke or aggravate DIC, nor did it cause bone marrow hypoplasia. The main side effects were dryness of the lip or skin, headache, nausea or vomiting and liver dysfunction. Severe scrotum exfoliative dermatitis with ulceration was seen in one case. In vitro induction of differentiation, GM-CFU, L-CFU assay and cytogenetic studies were performed. The results were discussed together with clinical observation regarding the mechanism of action of ATRA on APL. ATRA used as an inducer of differentiation is an alternative effective drug in the induction of remission in de novo APL as well as in cases in relapse.
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PMID:[Treatment of acute promyelocytic leukemia (APL) with all-trans retinoic acid (ATRA): a report of five-year experience]. 822 22

A case of sinus thrombosis occurring during combination chemotherapy with CDDP and VP-16 (PE) for a suprasellar germ-cell tumor is presented. A 5-year-old girl developed polyuria, polydipsia and headache in April, 1991 and became unconscious on May 10, 1991, when MRI and CT demonstrated a suprasellar tumor and marked hydrocephalus. After a ventriculo-peritoneal shunt operation, radiotherapy and two courses of PE therapy were carried out. During the second course of PE therapy, diabetes insipidus became quite difficult to control and severe hypovolemic hypernatremia developed. While it was being treated, the patient developed a clonic convulsion of her left extremities and visual disturbance. CT scan demonstrated a right parietal hemorrhagic infarction and IV-DSA suggested thrombosis of the superior sagittal sinus. Laboratory data disclosed DIC. The main cause of sinus thrombosis in this patient was considered severe dehydration. It is also possible that cisplatin and steroid played a role. In addition to these, dysfunction of hypothalamus, which is one of the regulatory centers of the plasma concentration of factor VIII, may have contributed to the acceleration of blood coagulation. This case re-emphasized the importance of preventing dehydration and monitoring the blood coagulation fibrinolytic system during PE therapy in patients with a suprasellar germ-cell tumor accompanied with diabetes insipidus.
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PMID:[Sinus thrombosis during CDDP and VP-16 (PE) therapy for suprasellar germ-cell tumor: case report]. 825 77

Thromboembolic (TE) events have been frequently reported in beta-thalassemic patients in association with known risk factors such as diabetes, complex cardiopulmonary abnormalities, hypothyroidism, liver function anomalies, and postsplenectomy thrombocytosis. In a recent survey involving 9 Italian thalassemic centers, we identified 32 patients with TE episodes in a total of 735 subjects, of whom 683 had thalassemia major and 52 thalassemia intermedia, corresponding to 3.95 and 9.61%, respectively. There was a great variation in localization: the main one (16/32) was CNS, with a clinical picture of headache, seizures and hemiparesis. Other localizations were the pulmonary (3 patients), mesenteric (1 patient) and portal (2 patients) sites. There were 6 cases of deep venous thrombosis (2 in the upper limbs, 4 in the lower ones). Intracardiac thrombosis was found in 2 subjects and clinical and laboratory signs of DIC were observed in 2 others during pregnancy. Since our patients with TE events present a statistically significantly higher incidence of associated dysfunction (cardiomyopathy, diabetes, liver function anomalies, hypothyroidism) than those without TE events (50 vs. 13.8%), we suggest close monitoring of those patients who are at higher risk of developing TE events because of the presence of one or more of these predisposing factors.
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PMID:Thromboembolic events in beta thalassemia major: an Italian multicenter study. 985 99

A 39-year-old female, who had splenectomy for idiopathic thrombocytopenic purpura (ITP) in 1988, was admitted to our hospital with high fever, headache, and loss of consciousness on November 29,1997. Neck stiffness and Kernig's sign were present. Examination of cerebrospinal fluid showed pleocytosis up to 506 cells/mm3 with 89% of polymorphonuclear cells and elevated protein to 1,135 mg/dl, and absence of glucose, Streptococcus pneumoniae phagocytosis was detected in the fluid. We diagnosed her as having pneumococcal meningitis as overwhelming postsplenectomy infection (OPSI) syndrome. After administration of dexamethasone (8 mg/day), cefotaxime (4 g/day), and ampicillin (6 g/day), she survived without any complications. Splenectomized patients have been recognized as immunocompromized hosts, and carry high morbidity and mortality risk from fulminant bacterial infections. Therefore, emergency treatment is important to reduce high mortality in such infections. We present an adult case of OPSI syndrome which occurred as pneumococcal meningitis, and we would like to emphasize the importance of prompt use of corticosteroids and high dose of sensitive antibiotics before DIC may occur during the course of illness.
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PMID:[A survival case of fulminant pneumococcal meningitis as overwhelming postsplenectomy infection (OPSI) syndrome]. 1034 48

We experienced a rare case of sinus histiocytosis with massive lymphoadenopathy (Rosai-Dorfman disease) accompanying hypertrophic cranial pachymeningitis. The patient is a 64-year-old woman with an early symptom of rt. peripheral facial palsy. She had a 4-month history of headache with a 5-week history of numbness in the rt. supraorbital nerve area, and lost her weight by 10 kg in 2 months. She developed rt. trochlea nerve palsy and numbness in the lt. mandibular nerve area. Laboratory findings showed that ALP, LDH and CRP were higher than normal. Of CT, MRI and MRA, the images of her head were normal. However, the Gd-enhanced MRI only showed a diffuse pachymeningeal enhancement. After about 3 months from the onset of rt. peripheral facial palsy, she died of DIC of unknown etiology. As a result of examinations in anatomical pathology, she was diagnosed as having sinus histiocytosis with massive lymphoadenopathy (Rosai-Dorfman disease). There were a large number of histiocytes on the pachymeninx. These findings suggest that hypertrophic cranial pachymeningitis caused multiple cranial neuropathy. We emphasize that use of Gd-enhanced MRI in the early stage is important for diagnosis.
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PMID:[Rosai-Dorfman disease accompanying hypertrophic cranial pachymeningitis with an early symptom of right peripheral facial palsy]. 1242 22

Coagulation disorders are common in cancer patients. In patients with solid tumors, a low-grade activated coagulation can result in systemic and cerebral arterial or venous thrombosis. Cancer treatments may also contribute to this coagulopathy, which usually, but not exclusively, occurs in the setting of advanced malignant disease. There may be TIAs or cerebral infarctions. Because of the widespread distribution of cerebral thromboses, there may be a superimposed encephalopathy; sometimes this is the only sign. Concurrent systemic thrombosis is present in many patients and is a useful clue to the diagnosis. In cerebral venous occlusion, the initial symptom is usually a headache. Except for cerebral intravascular coagulation that is unassociated with NBTE, neuriomaging studies usually demonstrate one or more parenchymal infarctions. MRI or MRV may demonstrate venous thrombosis. The laboratory evidence of coagulopathy is difficult to distinguish from the asymptomatic coagulopathy that often accompanies advanced cancer, and the test results must be interpreted cautiously. NBTE can be diagnosed by transesophageal echocardiography. There is no established treatment for the thrombotic coagulopathy associated with cancer, but anticoagulation should be considered. In leukemia and lymphoma, the coagulopathy is typically acute DIC that can lead to systemic and brain hemorrhages. It is especially common in acute myelogenous leukemias. The clinical signs of cerebral hemorrhage are fulminant and may be fatal. The bleeding usually occurs in the brain or subdural compartment, and rarely in the subarachnoid space. The diagnosis can be suspected by the clinical setting and by systemic thrombosis or hemorrhage. It can be established by examination of the peripheral smear, the platelet count, and tests of coagulation function. Therapy of acute DIC is controversial and should be individualized for the clinical setting. Cerebrovascular disorders can complicate metastatic or primary tumor in the brain, skull, dura, or leptomeninges. The clinical signs of infarction are indistinguishable from other causes of stroke, except that tumor-related venous occlusion will usually first produce signs of increased intracranial pressure. The diagnosis of tumor-related infarction can usually be established by neuroimaging studies that show infarction and may show extracerebral sites of tumor. CSF examination is useful in diagnosing leptomeningeal metastasis. A search for lung or cardiac tumor should be performed when embolic tumor infarction is suspected. Primary or metastatic tumors in the brain or dura may hemorrhage, producing the initial clinical signs of the brain tumor or a change in chronic signs induced by the tumor. There are helpful clues to a neoplastic hemorrhage on brain CT or MRI scans. The brain hemorrhage may require evacuation and the underlying tumor will usually require additional antineoplastic treatment. Hyperleukocytosis (extreme elevation of the cell count) in acute myelogenous leukemia is a less common cause of brain hemorrhage in recent years because of improved methods to lower the cell count. Cerebral arterial or venous thrombosis is sometimes the result of cancer therapy. The attribution of thrombosis to chemotherapy in many published cases is only speculative, because carefully conducted prospective studies that include investigation for other thrombotic causes are not available. The best-known associations with thrombosis are L-asparaginase, which is typically used in the induction therapy of acute lymphocytic leukemia, and combination hormonal therapy and chemotherapy for breast cancer. Radiation to the head and neck, typically administered for head and neck epithelial cancers or lymphoma, may result in delayed carotid atherosclerosis. The distribution of stenosis or occlusion is within the radiation portal and is typically more extensive than is atherosclerosis that develops in the absence of radiation. Small clinical series suggest that surgical treatment is equally effective as in nonirradiated carotid atherosclerosis. In children, the cerebral vessels can be affected by brain radiation resulting in stenosis or occlusion. Brain hemorrhages can result from chemotherapy effects on the hemostatic system or a microangiopathic anemia. Hemorrhages from radiation-induced vascular abnormalities are rare. Opportunistic infections, especially fungal infections, can complicate cancer or its treatment. Septic cerebral emboli may result in focal cerebral signs, seizures, or encephalopathy. Sometimes there is an associated hemorrhagic vasculitis or cerebritis. Rarely, mycotic aneurysms may bleed. A high index of suspicion is needed to diagnose fungal infection because of the difficulty in culturing the organism from the blood or CSF. A clinician can usually establish the cause of stroke in the cancer patient by performing a careful review of the clinical setting--including the type and extent of cancer and the type of antineoplastic therapy--in which the stroke occurred. Systemic thrombosis, embolism, or hemorrhage can be a clue to the cause, and appropriate neuroimaging and coagulation studies to aid in the diagnosis are available. Therapy may ameliorate symptoms or prevent further episodes. The identification of one of these unusual stroke syndromes that leads to the diagnosis of an occult and treatable cancer can be particularly rewarding.
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PMID:Cerebrovascular complications in cancer patients. 1269 Jun 49

Scrub typhus is an acute febrile illness caused by infection with Orientia tsutsugamushi transmitted by the bite of larval trombiculid mites (chiggers). A prospective study was conducted in septic shock patients in Maharat Hospital, Nakhon Ratchasima Province, Thailand, from 12 November 2001 to 5 January 2002. Of the 51 septic shock patients studied during the 7 week period, 18 (35.3%) were found to have evidence of scrub typhus infection; 3 patients (16.7%) died. In this study, septic shock caused by Orientia tsutsugamushi is the most prominent (35.3%) in endemic area of scrub typhus. Scrub typhus with septic shock patients results in organ failure: respiratory failure, DIC were predominant, followed by renal and hepatic involvement. Two deaths were due to respiratory failure and one death was as a result of combined respiratory and renal failure. Fever was the most common symptom, followed by headache, myalgia and dyspnea; lymphadenophathy and eschar are common signs. Laboratory findings revealed that almost all of the patients had a mild leukocytosis, reduced hematocrit and thrombocytopenia; SGOT, ALP, direct bilirubin (DB), total billirubin (TB), BUN, Cr were elevated; hypoalbuminemia was noted. Urinalysis showed that 88.9% of the patients had albuminuria. 77.8% of patients had abnormal chest X-rays.
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PMID:Septic shock secondary to scrub typhus: characteristics and complications. 1275 26

Ninety-nine patients with meningcoccal disease were admitted to the medical department of King Fahd Hospital, Medina during the Haj season of 1407H. (August 1987G). Neisseria meningitidis group A (Clone III-I) was responsible for this outbreak. This bacteria was brought into the Kingdom of Saudi Arabia by the pilgrims from South Asia who were carriers of the disease. Middle age and elderly seemed to be vulnerable to meningococcal disease. The majority of the patients presented with fever, headache, vomiting, skin rash, and signs of meningeal irritation. Diabetics afflicted with meningococcal disease fared badly; of 13 diabetics in the study, eight died. The mortality rate in our patients was 12.1%, a figure comparing favorably with other studies. The factors which influenced mortality included age above 50, shock, coma, renal failure, DIC, upper gastrointestinal bleeding, female sex and other associated diseases. The outbreak was successfully controlled by mass vaccination with polyvalent vaccine of the pilgrims and local population along with chemoprophylaxis with rifampicin.
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PMID:Meningococcal disease: Clinical profile of 99 patients. 1759 Jun 68


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