1/17. Analysis of the perception of and reactivity to pain and heat in patients with wallenberg syndrome and severe spinothalamic tract dysfunction.BACKGROUND: The aim of the study was to assess the consequences of severe spinothalamic tract lesions resulting from lateral medullary infarct and to show that a specific pain perception can be elicited by strong thermal stimulation. CASE DESCRIPTIONS: Both patients examined presented with severe thermoalgic dissociation of the limbs contralateral to the lesion, with normal discriminative somatosensory perception and motor strength. They reported pain perception when touching very warm (>50 degrees C to 60 degrees C) objects and a brisk, occasionally uncontrolled withdrawal reaction of the arm and hand under the same conditions, without any perception of the heat nature of the stimulus. Warm stimulation, <45 degrees C, elicited no thermal perception or discrimination. pain perception could be elicited in both patients by increasing the temperature, with a reproducible threshold of 47 degrees C to 49 degrees C. Pain always occurred after a prolonged delay of 8 to 10 seconds in response to threshold heat, and was described as deep and osseous, and clearly different from that perceived on the nonaffected side. The delay was much shorter when the temperature was increased by 4 degrees C to 5 degrees C. Cold stimulation elicited similar pain perception in one patient. Analysis of subjective perception of laser stimulation showed a much higher pain threshold on the affected hand. There were no laser-evoked potentials on this side, which suggested major spinothalamic injury. Assessment of the RIII noxious reflex revealed persistent response withdrawal reactions, with an increased threshold on the affected side, and partial consciousness of the noxious nature of the stimulus. CONCLUSIONS: To our knowledge, this is the first description of the appearance of pain perception of high temperatures in patients with severe spinothalamic injury who are suffering from a complete loss of temperature perception. This implies that noxious thermal stimulation can still be perceived via extra spinothalamic pathways (which are slow and multisynaptic), such as the spinoreticulothalamic tract. patients with Wallenberg syndrome should be informed and made aware of their residual perception of and reactions to noxious stimulation.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/17. Cognitive and behavioral abnormalities in a case of central nervous system whipple disease.BACKGROUND: whipple disease is a rare condition characterized by migratory polyarthralgias, fever, and chronic diarrhea. A subset of patients with the disease may either initially have or eventually develop symptoms of central nervous system involvement. DESIGN AND methods: The cognitive and behavioral functioning of a patient with central nervous system involvement from whipple disease was studied during a 7-month period. Serial neuropsychological evaluations were used to quantify the nature of his cognitive and behavioral profile. SETTING: neurology department of a university medical center. RESULTS: A variety of cognitive impairments were noted, most prominently in the domains of sustained attention, memory, executive function, and constructional praxis. There were striking behavioral manifestations as well, including disinhibition and confabulation. CONCLUSIONS: The case demonstrates a degree of higher-order central nervous system dysfunction rarely observed and quantified in connection with whipple disease, and with important implications for differential diagnosis of certain neurologic conditions. We also call attention to some of the neuroanatomical correlates of this encephalopathic condition.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
3/17. Multifocal eosinophilic granuloma ("hand-Schuller-Christian disease"). Report illustrating H-S-C chronicity and diagnostic challenge.We have described an unusual case of multifocal eosinophilic granuloma ("hand-Schueller-Christian disease") in a middle-aged woman. The case underscores the varied and subtle nature of the disease presentation and the extent to which many organ systems may become involved. Unusual features of her case include atypical bone roentgenograms, cutaneous anergy, panhypopituitarism and evidence of diffuse central nervous system dysfunction. Several features of multifocal eosinophilic granuloma present in the older age group are different from those presenting in children and young adults. Finally, multifocal eosinophilic granuloma may present all the clinical and laboratory features of a progressive, chronic disease.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
4/17. Opsoclonus in a patient with guillain-barre syndrome.A 54 year-old woman showed the unusual association of a guillain-barre syndrome and opsoclonus, a rare eye movement characterized by involuntary bursts of jerking, ataxic and multidirectional saccades, without an intersaccadic interval. Since opsoclonus is a phenomenon described exclusively in CNS diseases, the case reported supports the hypothesis of CNS involvement in some cases of guillain-barre syndrome. The latter is generally considered an immune-mediated disease and an immune pathogenesis is also supposed in opsoclonus associated with systemic malignancies; besides, in the patient reported, the self-limiting nature of the disturbance and the lack of MRI lesions suggest that opsoclonus may result from an immune-related phenomenon causing a functional and transitory dysfunction of the CNS.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
5/17. Nervous system involvement in systemic lupus erythematosus: report of three cases.central nervous system involvement in systemic lupus erythematosus is rather frequent whereas peripheral nervous system involvement is much less common. The three patients studied by us had isolated manifestations uncommon in nature. The first one developed a sensory-motor polineuropathy with signs of axonal degeneration. It responded to the therapeutic association of corticosteroids with an immunosuppressive agent. Satisfactory recovery took place over a time span of a year. The second patient had encephalic and cerebral trunk involvement from which an irreversible dementia resulted. The third patient, who had recurrent aseptic meningitis, is asymptomatic for six months now. patients one and two had no systemic manifestations at the time of nervous system involvement. Suspicion of systemic lupus erythematosus was made on the basis of past inspecific articular symptoms. The neurological and systemic manifestations may be sometimes simultaneous; they are usually followed by serologic changes. Isolated nervous system involvement may be seen with and without sorologic changes, and there may be found antibodies reactive with phospholipids (anticardiolipin, antigangliosides and anticerebrosides). The employment of nonsteroid immunosuppressive drugs associated with corticosteroids in small doses seems to be useful in cases of systemic lupus erythematosus with nervous system involvement.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
6/17. Infections due to Lancefield group C streptococci.Our experience with group C streptococcal infection over the past 15 years demonstrates an important and emerging role for this hemolytic organism as an opportunistic and nosocomial pathogen. Significant risk factors in this predominantly male population included chronic cardiopulmonary disease, diabetes, malignancy, and alcoholism. bacteremia occurred in 74% of cases seen in our series. Nosocomial acquisition of infection was observed in 26%, and infection was frequently polymicrobial in nature with gram-negative enteric bacilli isolated most commonly along with group C streptococci. We observed a broad spectrum of infections including puerperal sepsis, pleuropulmonary infections, skin and soft-tissue infection, central nervous system infection, endocarditis, urinary tract infection, and pharyngeal infections. Several cases of bacteremia of unknown source were observed in neutropenic patients with underlying leukemia. New syndromes of infection due to group C streptococci observed in our series included intra-abdominal abscess, epidural abscess, and dialysis-associated infection. Response to therapy and outcome was related to the underlying disease. While the literature suggests that patients with group C endocarditis respond better to synergistic penicillin-aminoglycoside regimens, patient numbers are too small to draw definite conclusions. The clinical significance of antibiotic tolerant group C streptococci remains uncertain. In patients with serious group C infections including endocarditis, meningitis, septic arthritis, or bacteremia in neutropenic hosts, we advocate the initial use of cell-wall-acting agents in combination with an aminoglycoside.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
7/17. diagnosis of CNS lymphoma using immunofluorescent phenotyping of CSF mononuclear cells.We describe the use of a panel of monoclonal antibodies, directed against leukocyte surface antigens to characterize CSF mononuclear cells with regard to malignancy when cytopathology was inconclusive. Cytocentrifuged preparations from three patients in which traditional modalities had not yielded a diagnosis were studied, utilizing a panel of antibodies for B and T cell antigens. All three patients were found to have B cell lymphoma of the CNS. Rapid institution of the appropriate therapy resulted in marked improvement of CNS symptoms in each case. Our results indicate that in patients with CNS disease and CSF pleocytosis of undefined nature, this technique may provide rapid and precise diagnostic information.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
8/17. Infantile multisystem inflammatory disease: a specific syndrome?We report two patients with infantile onset of evanescent rash, fever, arthropathy with severe deformities, periosteal changes, chronic meningitis, hydrocephalus, convulsions, developmental delay, papilledema, unusual uveitis, and lymphadenopathy. A few patients with similar findings have been previously reported. Although some similarity exists between findings in these patients and in others with systemic juvenile rheumatoid arthritis, they appear to differ both in regard to the nature and severity of the clinical and pathologic features. We suggest that this group of patients has a separate rheumatic disorder not yet included in the standard classifications of the childhood rheumatic diseases.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
9/17. Hereditary neurocutaneous angioma: a new genetic entity?A family pedigree with a possible new genetic syndrome characterised by the presence of angiomas, systemic in nature, affecting particularly the skin and the central nervous system, is described. Angiomas of the CNS seem to have a marked tendency to bleed. The condition shows a clearly dominant mode of transmission, four subjects in three generations being affected. Differentiation from other conditions belonging to the vascular abnormalities subgroup of phakomatoses is presented.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
10/17. central nervous system mass lesions in the acquired immunodeficiency syndrome (AIDS).The authors present the cases of nine patients with acquired immunodeficiency syndrome (AIDS) and intracerebral mass lesions, who were evaluated at the University of california, san francisco, between April, 1979, and July, 1983. Eight patients were confirmed homosexual males, and none was Haitian . Their average age was 38.8 years. Tissue diagnosis was made in all patients from brain biopsy or autopsy material. Three patients initially presented for evaluation of their neurological deficits, while the other six already carried the diagnosis of AIDS at admission. Seven patients presented with multiple intracranial lesions and two had polymicrobial infection. In this series, three patients had toxoplasma gondii brain abscesses, two had primary lymphoma, two had metastatic Kaposi's sarcoma of the central nervous system (CNS), two had focal cytomegalovirus encephalitis and one each had cryptococcal and candida albicans brain abscesses. The clinical presentation, radiological evaluation, and serodiagnostic study of these patients were not helpful in establishing the nature of the CNS lesions. brain biopsy is considered by the authors to be critical for the evaluation and appropriate treatment of mass lesions in patients with AIDS.- - - - - - - - - - ranking = 0.5keywords = nature (Clic here for more details about this article) |
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