Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Isolated angiitis of the central nervous system is a rare disease affecting mainly adults of both sex; about 210 cases have been reported. Contrary to other inflammatory arteritis, arthralgia, myalgia, weight loss and fever are exceptional and symptoms are mainly neurologic, but none is specific. The diagnosis is evoked in case of headaches and cognitive impairment, associated or not with multifocal neurologic signs. Evolution is acute, subacute or chronic. Elevated sedimentation rate and cerebrospinal fluid pleiocytosis are present in 2/3 of cases. CT scan and brain MRI generally demonstrate multifocal ischemic lesions involving cortex, white matter, basal ganglia and brainstem. Cerebral arteriography is the key investigation, showing segmental stenoses alternating with fusiform dilatations of blood vessels, which are highly suggestive but not specific. It can be normal and its repetition is then recommended. Certain diagnosis is obtained from cerebromeningeal biopsy, showing a segmental angiitis of small vessels, which is granulomatous in 88 p.100 and non granulomatous in 12 p.100 of cases. The pathogenesis is unknown. Spontaneous evolution is generally fatal. Cyclophosphamide associated with prednisone considerably improves the prognosis, especially when initiated early in the course of the disease.
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PMID:[Isolated angiitis of the central nervous system. Report of two cases and review of the literature]. 1245 39

Kikuchi-Fujimoto disease is a rare disease first described in 1972 by Kikuchi and Fujimoto et al. (1,2). Clinically the disease presents with lymphadenitis usually in the cervical region. Most reported cases of Kikuchi-Fujimoto disease have been of Asian origin. The cause is unknown and the condition is self-limiting. Some kind of viral or postviral etiology has been implicated. Bacterial and protozoal organisms as well as various other antigens, chemical, physical and neoplastic, have also been postulated. An association with systemic lupus erythematosus has also been shown. Lymphadenitis, hepatomegaly and splenomegaly as well as leukopenia, elevated erythrocyte sedimentation rate and hepatic abnormalities are common findings. Fever, malaise, fatigue, headache, night sweats, nausea, vomiting, weight loss, cutaneous manifestations, and even neurological symptoms are other complaints. Histologically the lymph nodes show partial involvement with patchy irregular areas of necrosis in the paracortical area with absence of neutrophils. We describe four cases of Kikuchi-Fujimoto disease observed in Greece. Their characteristics are discussed, whilst a review of the literature is attempted.
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PMID:Kikuchi-Fujimoto disease in Greece. A study of four cases and review of the literature. 1249 69

To clarify the clinical manifestations of cat scratch disease (CSD), we evaluated a total of 130 seropositive patients with CSD. The patients' ages ranged from 1 to 68 years; 103 (79.2%) were under 18 years of age. CSD occurred predominantly in the fall and winter months. Regional lymphadenopathy was noted in 110 (84.6%) of the cases, and the most common sites were the neck (33%), axillary (27%), and inguinal (18%) regions. One hundred of the patients (77%) had general symptoms, such as fever, headache, and malaise. The clinical manifestations of CSD showed a wide spectrum from typical or classical CSD, with regional lymphadenopathy, to atypical or systemic CSD. Of the 130 cases, 103 (79.2%) were typical CSD and 27 (20.8%) were atypical CSD. Atypical cases of CSD were commonly reported as fever of unknown origin (37.0%), neuroretinitis (22.2%), encephalopathy (14.8%), hepatosplenic granuloma (11.1%), and Parinaud's oculoglandular syndrome (7.4%). Fever of unknown origin or prolonged fever lasting more than 14 days was evident in 27 (20.8%) of the 130 cases in this study. Eleven of the 27 cases lacked lymphadenopathy. Our findings suggest that CSD is not a rare disease in Japan. The indirect fluorescent antibody (IFA) test to detect Bartonella species may provide a prompt diagnosis of CSD and facilitate appropriate therapy.
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PMID:Cat scratch disease: analysis of 130 seropositive cases. 1252 97

Mollaret's meningitis (MM) is a rare disease of benign nature characterized by recurrent episodes of aseptic meningitis. Cerebrospinal fluid (CSF) examination remains the sole diagnostic modality. Eighteen CSF samples from 14 patients were studied along with the clinical data. Specimens were prepared by cytocentrifugation and Millipore filtration and were stained with Diff-Quik and Papanicolaou stains. Eight patients were men and six were women, with an age range of 17-74 yr (mean age 37 yr). Most common clinical presentation was recurrent episodes of headaches and photophobia followed by a sustained mild fever lasting 5-7 days. The CSF showed markedly increased cellularity with pleocytosis. The differential count showed predominant monocytosis ranging from 84% to 100% (mean 96). In our series, two patients had herpes simplex virus type 2 (HSV-2) DNA detected by polymerase chain reaction (PCR) in the CSF. The monocytes were seen predominantly singly, but three cases showed a strong tendency to aggregate in small groups. Phenotypically, these cells had bean-shaped bilobed nuclei as well as multiple deep nuclear clefts depicting the so-called "footprint" appearance. In four cases, multiple blunt-tipped cytoplasmic pseudopods were noted. Degenerated monocytes with the appearance of the so-called "ghost cells" were noted in one-half of the cases. Background cells were mostly small mature lymphocytes; however, one-half of cases showed a significant amount of plasma cells and/or polymorphonuclear leukocytes (PMNs). Lysed blood with hemosiderin-laden macrophages and numerous leptomeningeal cells were seen in two cases. CSF examination of MM presents a spectrum of cytomorphologic features. When interpreted in light of the appropriate clinical setting. the latter, although nonspecific, provides an accurate diagnosis. The differential diagnosis includes various degenerative, inflammatory/infectious, and lymphoproliferative disorders of the central nervous system.
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PMID:Mollaret's meningitis: cytopathologic analysis of fourteen cases. 1272 16

Spontaneous intracranial hypotension (SIH) is a rare disease which is associated with variety of symptoms and signs including cranial neuropathies. Though diplopia occurred reportedly in about one fourth of SIH cases, trochlear nerve palsy has been reported only one case in the literature. A 71-year-old previously healthy male developed postural headache and nausea. After 15 days, he began to have diplopia caused by right trochlear nerve palsy. He consulted our hospital 2 months later because only diplopia was not recovered. Magnetic resonance imaging (MRI) showed bilateral thin chronic subdural hematoma (CSDH), brain sagging and downward brain stem displacement, but not ischemic change in brain stem. We suspected SIH for right trochlear nerve palsy, and he had symptomatic therapy. Two months later, he had burr hole surgery because of disturbance of consciousness and right hemiparesis due to progressive bilateral-CSDH. To say nothing of disturbance of consciousness and right hemiparesis, his trochlear nerve palsy was completely recovered after surgery at once. Follow-up MRI showed brain sagging and downward brain stem displacement were recovered.
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PMID:[Spontaneous intracranial hypotension associated with trochlear nerve palsy and bilateral chronic subdural hematoma]. 1509 63

Fibrous dysplasia (FD) of the sphenoidal sinus is a rare disease, especially during adulthood. We report a case of FD of the right sphenoidal sinus in an adult male patient who presented with nonspecific symptoms limited to headache localized to the right temporal area and to the inferior orbital rim of both sides. Magnetic resonance imaging revealed a dense mass that occupied the entire right sphenoidal sinus and skull base with typical ground-glass opacification and bony sclerosis of the whole sphenoidal wall. The diagnosis of FD was confirmed on pathological examination of a biopsy taken through sphenotomy. The patient underwent a subcranial craniotomy for tumor resection. After more than 4 years of follow-up, the patient was disease-free. On the basis of these clinical features, it is important to consider sphenoidal FD in both young and adult patients complaining of an unexplained headache, because it may present unusually with headache localized to the temporal region or the inferior orbital rim.
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PMID:Fibrous dysplasia of the sphenoid sinus and skull base presents in an adult with localized temporal headache. 1516 46

In mast cell (MC) disorders (mastocytosis), clinical symptoms are caused by the release of chemical mediators from MCs, the pathologic infiltration of neoplastic MCs in tissues, or both. Cutaneous mastocytosis is a benign disease in which MC infiltration is confined to the skin. In pediatric cases cutaneous mastocytosis might regress spontaneously. Systemic mastocytosis (SM) is more frequently diagnosed in adults and is a persistent (clonal) disease of bone marrow-derived myelomastocytic progenitors. The somatic c-kit mutation D816V is found in the majority of such patients. The natural clinical course in SM is variable. Whereas most patients remain at the indolent stage for many years, some have aggressive SM (ASM) at diagnosis. Other patients have an associated clonal hematologic non-MC lineage disease (AHNMD). MC leukemia (MCL) is a rare disease variant characterized by circulating MCs and fatal disease progression. The diagnoses of ASM, SM-AHNMD, and MCL might be confused with a variety of endocrinologic, vascular, or immunologic disorders. It is therefore of particular importance to be aware of the possibility of an underlying (malignant) MC disease in patients with unexplained vascular instability, unexplained (anaphylactoid) shock, idiopathic flushing, diarrhea, headache, and other symptoms that might be mediator related. An important diagnostic clue in such cases is an increased serum tryptase level. The current review provides an overview of mastocytosis and its subvariants and a practical guide that might help to delineate mastocytosis from unrelated systemic disorders.
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PMID:Diagnosis and classification of mast cell proliferative disorders: delineation from immunologic diseases and non-mast cell hematopoietic neoplasms. 1524 37

A 49-year-old right-handed woman was admitted for an 8-month history of unusual headache and transient diplopia. Clinical examination and brain CT scan were normal. Two months later, symptoms of raised intracranial pressure developed and a brain CT scan showed small lateral ventricles and sulci without any abnormal contrast enhancement or tumor mass. Brain MRI with T2-weighted spin echo sequences revealed a hyperintense signal in the right temporoparietal region, whereas only a slight enlargement of this region was noted on T1 spin echo. The patient deteriorated rapidly and died with uncontrollable raised cerebrospinal fluid pressure. The diagnosis of gliomatosis cerebri was made at necropsy. Gliomatosis cerebri is a rare intracranial neoplasm of neuroepithelial origin. Spread of this tumor is particularly fast in the white matter compared with the gray matter and nuclei. Clinical symptoms are not specific. The diagnosis can be suspected by MFU showing an isointense or hypointense signal in the deep white matter on T1-weighted images, and largely a hyperitense signal on T2-weighted sequences. The diagnosis is confirmed by stereotactic biopsy or necropsy. No curative treatment is currently available. Radiotherapy can delay the rapidly fatal outcome. Our case illustrates the possible onset of this rare disease by isolated cephalgia with normal early CT scan.
Headache 1998 Oct
PMID:Gliomatosis cerebri: a rare cause of progressive headache. 1561 85

Cavernous hemangioma of the calvaria is a very rare disease, and patients usually present with headaches or a visible skull deformity. Few reports of patients presenting with intradiploic or epidural hemorrhages are found in the literature. No case of an intradural hemorrhage from a cavernous hemangioma of the skull has been reported to date. The authors present the case of a 50-year-old man in whom a symptomatic subdural hematoma (SDH) resulting from a cavernous hemangioma of the calvaria had hemorrhaged and eroded through the inner table of the skull and dura mater. The patient underwent surgery for evacuation of the SDH and resection of the calvarial lesion. Postoperatively, the patient experienced immediate relief of his symptoms and had no clinical or radiological recurrence. Calvarial cavernous hemangiomas should be considered in the differential diagnosis of nontraumatic SDHs. Additionally, skull lesions that present with intracranial hemorrhages must be identified and resected at the time of hematoma evacuation to prevent recurrences.
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PMID:Cavernous hemangioma of the skull presenting with subdural hematoma. Case report. 1563 93

Cranial fascitis is a rare disease that is considered as a subset of nodular fascitis. Lauer and Enzinger described the disease for the first time in 1980 as a cranial fascitis of childhood (CFC), based on pathologic findings in nine cases of children from three weeks to six year old. The disease is benign proliferation of immature fibroblasts infiltrating the cranial bones with tendency to form tumor like shapes. Surgery excision gives total cure and recurrence is very rare. We present the case of a twenty years old male patient with head trauma in medical history and two years history of recurrent forehead headache. Performed cerebral computerized tomography and skeleton scintigraphy showed erosion of the lateral parts of both frontal skull bones and upper-lateral parts of the right and left orbital cavity. In general anesthesia, biopsy specimen was taken. After histopathological examination the diagnosis of cranial fascitis was established. Eight months after the diagnosis we observed progression of the skull bones destruction. Therefore our recommended treatment was surgical excision but we did not obtain patient agreement.
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PMID:[Cranial fascitis--case report and literature review]. 1577 17


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