On bedside teaching.
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Biomedical subjects
Publications and source records attributed to J R Berger.
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Infection with the human immunodeficiency virus frequently results in a dementing illness which manifests predominantly as a subcortical dementia. Parkinsonian features may be prominent in these patients and may on occasion be the presenting manifestation. These patients are exquisitely sensitive to dopaminergic blocking agents. Radiological studies, metabolic uptake studies and pathological examination of the brain suggest that the basal ganglia are the major target of this infection. Although further studies are necessary to determine appropriate treatment for this condition and to develop an understanding of the underlying pathophysiological mechanisms, available evidence suggests that the response to dopamine agonists may be variable and that viral strains and viral products may specifically target cells within the basal ganglia.
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The spectrum of opportunistic infections occurring in association with human-immunodeficiency virus, type 1, is very broad. These infections develop most frequently in the setting of advanced immunosuppression. There is no part of the neuraxis that is immune to these complications and the concurrence of more than one infectious illness may always be considered. The neuroimaging features, when coupled with the clinical and laboratory findings, often suggest the correct diagnosis and enable the physician to initiate therapy.
Although isolated lower motor neuron disease has been reported as a paraneoplastic complication, it has not been previously described, in association with anti-Hu antibody. We report a 51-year-old man in whom weakness heralded the presence of a small-cell cancer of the lung. His neurological disorder was characterized by an unremitting progression of limb, neck, and chest wall weakness and wasting that commenced and remained predominant in the upper limbs. Electrophysiological studies demonstrated widespread denervation and examination of a muscle biopsy specimen showed evidence of acute and chronic denervation. High titers of anti-Hu antibody were detected in the serum and cerebrospinal fluid. Neither objective measures of strength nor titers of anti-Hu antibody responded to corticosteroids, cyclophosphamide, intravenous immunoglobulins, or plasmapheresis. Death from the complications of motor neuron disease ensued 23 months after the onset of weakness. Autopsy revealed tumor in the lung and on pleural and peritoneal surfaces. There was a loss of anterior horn cells in the spinal cord. Despite the absence of symptomatic cerebellar disease, a decrease in the number of Purkinje cells was also detected.
A wide spectrum of central and peripheral nervous system abnormalities may be associated with HIV infection. These disorders may be caused by HIV infection, result as secondary complications related to immunosuppression, or be a neurotoxic effect of therapeutic agents. The range of neurologic disorders includes dementia, focal cerebral mass lesions, myelopathy, peripheral neuropathies, and myopathy. Early diagnosis and therapy is critical, and may result in substantial improvement in patients' quality and quantity of life. This article reviews the approach to differential diagnosis of these neurologic disorders and presents theories of pathogenesis and current approaches to treatment.
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OBJECTIVE: To evaluate oxandrolone, an oral anabolic steroid with potent anabolic activity and minimal androgenic effects, for the treatment of AIDS-associated myopathy and wasting. METHODS: In a multicenter, double-blind study, 63 HIV-seropositive men with > 10% loss of body weight were randomized to receive either placebo, 5 mg/day oxandrolone, or 15 mg/day oxandrolone for 16 weeks. Body weight, neuromuscular evaluation, and measures of well-being were repeatedly assessed. RESULTS: Patients who received 15 mg/day oxandrolone showed weight gain throughout the 16-week treatment period. Overall, the 5 mg/day oxandrolone group maintained their weight gain over the 16-week period, whereas the placebo group showed continual weight loss. At week 16, significantly more patients in the 15 mg/day dose group reported increases in appetite and activity than those receiving placebo. There were no consistent, dose-related, statistically significant differences from baseline in laboratory values or adverse events. CONCLUSION: Oxandrolone, at a dose of either 5 mg/day or 15 mg/day, in contrast to placebo, had a positive impact on the weight and well-being of HIV-seropositive patients suffering from wasting and weakness. Measurable improvement in muscle strength was not noted at the doses employed in this study. Oxandrolone was well tolerated in all the patients who were enrolled in the study. Based on the results reported here, additional studies using higher doses of oxandrolone seem warranted.
The new onset of headache in a person infected with human immunodeficiency virus (HIV) may be an ominous sign of a significant underlying neurological disorder. This case control study compares the prevalence of headache in HIV-seropositive persons without identifiable neurological disease on study entry to that in an HIV-seronegative control population with similar risk factors for HIV infection. Features of headache that were evaluated included frequency, duration, location, severity, and recent change in characteristics. In the HIV-seropositive population, the presence of headache and its specific features were correlated with the degree of immunosuppression as determined by absolute CD4 counts and Centers for Disease Control stages, the presence of other neurological symptoms and neurological signs, cranial magnetic resonance imaging (MRI) findings, and cerebrospinal fluid (CSF) values. Headaches were common; approximately 50% of all subjects reported headache at study entry and 2 years later. Headache frequency and characteristics were not different between HIV-seropositive and HIV-seronegative subjects. Headache was neither more frequent nor different in HIV-seropositive individuals with advanced immunosuppression. There was no correlation between headache and abnormal CSF parameters, cranial MRI abnormalities, including the presence of sinusitis, or the use of zidovudine.
We report a case of rapidly progressive encephalopathy and generalized chorea due to HIV encephalitis. The patient was a 24-year-old man known to be HIV-seropositive for 4 years. The severity of the movement disorder resulted in rhabdomyolysis. Sepsis developed and he died after a 21-day hospitalization. Pathologic study revealed prominent neuronal loss and gliosis of subcortical regions. Acute encephalopathy with generalized chorea may be a rare consequence of HIV encephalitis.
Although the use of rigid fixation techniques has become widespread for the treatment of mandible fractures, indications still remain for the utilization of biphasic external pin fixation in patients who suffer cranio-maxillofacial trauma. The treatment of continuity defects of the mandible secondary to avulsive injuries or ablative surgery is a primary indication for the utilization of biphasic external pin fixation. The placement of biphasic external pins requires an understanding of the surgical anatomy surrounding the mandible. In this article, the authors discuss the indications for, contraindications for, and complications associated with the use of biphasic external pin fixation, describe the procedure, and present a case report of a patient with parotid salivary fistula secondary to the placement of external pin fixation.
We describe a clinical picture similar to Parkinson's disease in a patient with midbrain encephalitis due to cysticercosis. The clinical, radiographic, and cerebrospinal fluid findings suggested an inflammatory reaction to a dying cyst that involved midbrain structures. The encephalopathy appeared to worsen temporarily after the institution of anticysticercus therapy. A discordance between worsening parkinsonian symptoms and improving pyramidal features was noted. Virtually a complete clinical recovery eventually ensued.
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We report a 47-year-old man who presented with partial seizures complicating focal cerebral vasculitis of the right temporal lobe. Excisional biopsy of the mass lesion revealed fibrinoid necrosis of small vessels. These vessels were infiltrated by neutrophils, eosinophils, lymphocytes, and plasma cells. Despite extensive evaluation, no etiology was apparent for the vasculitis. No immunosuppressive agents were administered, and 4 1/2 years after the diagnosis, he remains healthy except for an incongruous left homonymous hemianopia. Follow-up cranial magnetic resonance images revealed only postoperative changes. This case demonstrates that focal lesions and a benign course may represent one end of the spectrum of primary angiitis of the central nervous system.
While the dentigerous cyst is not uncommon, its development as a result of an impacted deciduous tooth is rare. According to the authors' search, this case is only the fifth of its type reported in the dental literature. The authors review the presentation and treatment of a deciduous dentigerous cyst.
Progressive multifocal leukoencephalopathy, a formerly rare disease that chiefly occurred in persons with underlying lymphoma and chronic lymphocytic leukemia, is now seen with increasing frequency in the era of acquired immunodeficiency syndrome. Progressive multifocal leukoencephalopathy is currently estimated to arise in 5% of all human immunodeficiency virus-infected individuals. The clinical features of the disorder in patients with acquired immunodeficiency syndrome do not appear to be significantly different from progressive multifocal leukoencephalopathy occurring in association with other immunosuppressive disorders. Radiographically, the appearance of HIV dementia on magnetic resonance imaging is sometimes confused with that of progressive multifocal leukoencephalopathy. Among the characteristics that are helpful in distinguishing between the two disorders are the presence of focal findings, the rate of disease progression, the specific magnetic resonance imaging attributes, including the location of the lesions, and certain cerebrospinal fluid parameters, including surrogate markers for human immunodeficiency virus dementia and the presence of myelin basic protein. The remarkable increase in the burden of progressive multifocal leukoencephalopathy has provided a vital impetus for its study, particularly with respect to diagnosis and therapy. Establishing an unequivocal diagnosis of progressive multifocal leukoencephalopathy currently requires brain biopsy. The application of polymerase chain reaction for JC virus amplification to cerebrospinal fluid samples suggests that it may provide an alternative means of diagnosis. Recent in vitro studies of cytosine arabinoside and camptothecin suggest that they, or similar agents, may prove useful in the treatment of this illness and well-designed clinical trials are underway.
PURPOSE: To evaluate and describe the neuroimaging findings of patients with neurosyphilis. METHODS: The neuroimaging studies of 35 patients with documented neurosyphilis were reviewed. Diagnosis was established in 34 patients with cerebrospinal fluid for a Venereal Disease Research Laboratory test, complemented by autopsy in 1 and brain biopsy in 1. All patients had reactive fluorescent treponemal antibody tests with absorption in their sera. Imaging studies included plain and contrast-enhanced CT of the brain, plain and gadolinium-enhanced MR, MR angiography, and conventional angiography. Imaging findings were also correlated with the relevant pathologic findings at autopsy in three additional patients with neurosyphilis who did not have brain imaging studies. RESULTS: Of the 35 patients with imaging studies, 32 tested human immunodeficiency virus (HIV)-seropositive, and 3 were HIV-seronegative. Eleven (31%) of 35 patients had normal radiographic findings. Cerebral infarctions were seen in 8 (23%) of 35 patients, and nonspecific white matter lesions in 7 (20%) of 35. Cerebral gummas and extraaxial enhancement indicating meningitis were noted in 2 (6%) of 35 patients, respectively. Arteritis was demonstrated in 2 (50%) of 4 patients who underwent either MR angiography or conventional angiography. The 3 subjects who had autopsy but not imaging studies were found to have manifestations of meningovascular syphilis, including syphilitic leptomeningitis and an obliterative endarteritis. CONCLUSION: We conclude that findings of vascular occlusive disease manifested as infarction or arteritis, enhancing cortical lesions with or without adjacent meningeal enhancement, focal or diffuse extraaxial enhancement, and white matter disease, although nonspecific, in the proper clinical setting should prompt appropriate testing for neurosyphilis, a treatable disease, in patients with and without HIV infection.