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Biomedical subjects

K A Jaeckle

Publications and source records attributed to K A Jaeckle.

47 records · Page 3Linked to original sources

Central neurogenic hyperventilation: pharmacologic intervention with morphine sulfate and correlative analysis of respiratory, sleep, and ocular motor dysfunction.

Central neurogenic hyperventilation (CNH), for which there is no effective therapy, can eventually result in respiratory fatigue and death. This report describes a patient with CNH due to a brainstem anaplastic astrocytoma who also exhibited disturbances of sleep and ocular motor function. The CNH responded clinically to morphine sulfate and methadone. Analysis of ventilatory response to CO2 before and after morphine demonstrated a depression of ventilatory response (49 to 53% of baseline) and occlusion pressure response (35 to 50% of baseline) to CO2, with a requirement for high doses of naloxone (10 mg IV) to reverse the effect. Polysomnography revealed sustained hyperventilation, elevated O2 saturation, and low end-tidal CO2 throughout all stages of non-rapid eye movement (NREM) sleep, and absence of rapid eye movement (REM) sleep. Ocular motor evaluation disclosed absence of horizontal and reflexive saccades with compensatory head thrusts. Correlation of the clinical and physiologic data with the MRI abnormalities suggested that the lesion responsible for CNH in this patient might reside in the medial tegmental parapontine reticular formation. Since recurrent episodes of hyperventilation responded in a sustained fashion to IV and oral opiates, this treatment may warrant consideration in other patients with CNH.

Adult↗

Anticerebellar antibodies in neurologically normal patients with ovarian neoplasms.

Several groups of investigators have confirmed the occurrence of antibodies to Purkinje and other cerebellar neuronal populations in the serum and spinal fluid of patients with paraneoplastic cerebellar degeneration. Although this antibody response suggests that paraneoplastic cerebellar degeneration may have an autoimmune basis, it is not known what role anticerebellar antibodies play in the pathogenesis of this disorder or whether the presence of antibodies invariably results in cerebellar injury. We identified 3 patients with ovarian malignancies in whom high titers of circulating anticerebellar antibodies were present without clinical evidence of cerebellar disease. We followed these patients clinically and serologically until their deaths from their neoplasms. All 3 patients remained neurologically normal. In 2 of the patients, anticerebellar antibodies persisted at high titer. CSF obtained from 1 of these patients postmortem did not contain detectable levels of anticerebellar antibody, but histopathologic examination of her cerebellum revealed patchy loss of Purkinje cells. In the 3rd patient, antibody titers fell with removal of the primary tumor and chemotherapy but did not rise with tumor recurrence. Indirect immunofluorescence did not reveal anticerebellar antibodies in the serum or CSF of other patients with neoplasms, patients with other cerebellar disease, or normal controls. The present study demonstrates that patients with ovarian malignancies may occasionally develop antibodies that react with cerebellar neuronal antigens and can maintain this antibody response for protracted periods of time without clinically evident cerebellar injury. Tumor recurrence may not be accompanied by rise in titers of anticerebellar antibodies.

Aged↗

Evolution of computed tomographic abnormalities in leptomeningeal metastases.

We obtained serial cranial computed tomographic (CT) scans from 25 patients with documented leptomeningeal metastases in order to correlate the course of clinical symptoms and cerebrospinal fluid abnormalities with the evolution of CT abnormalities. In almost one-third of patients, relatively specific CT findings such as enhancement of the meninges, obliteration of cisterns or sulci, and hydrocephalus with transependymal edema preceded clinical symptoms. By the time patients became symptomatic, 10 of 25 (40%) had specific abnormalities, and 4 more became specifically abnormal with time. The first scan after cerebrospinal fluid tumor cells were discovered showed specific abnormalities in only 6 patients, but 7 more became specifically abnormal with time. Ventriculomegaly occurred in 18 of 25 patients (72%) and was associated with sulcal and/or cisternal obliteration in one-half of the patients. In 4 patients sulcal and cisternal obliteration developed prior to ventricular enlargement. Multiple small, enhancing cortical nodules were detected prior to symptom onset and discovery of leptomeningeal tumor in 5 patients.

Arachnoid↗

Autoimmune response of patients with paraneoplastic cerebellar degeneration to a Purkinje cell cytoplasmic protein antigen.

Sera from 6 of 12 patients with paraneoplastic cerebellar degeneration (PCD) contained anti-Purkinje cell antibodies, as determined by indirect immunofluorescence on frozen sections of normal human cerebellum. Samples of cerebrospinal fluid from 2 of the patients with serum antibodies were tested, and both specimens contained anti-Purkinje cell antibody. The anti-Purkinje cell antibodies were polyclonal, fixed complement, and were present in all patients at serum dilutions of 1:1,000 or greater. Antibody activity could not be suppressed by preabsorption of sera with human or animal brain and tissue powders or with fresh crude human cerebellar extracts. No anti-Purkinje cell antibodies were detected in control sera from 167 neurologically normal cancer patients, 32 normal volunteers, 10 patients with other causes of cerebellar degeneration, or 8 patients with other paraneoplastic neurological diseases. Preliminary evidence suggests that the Purkinje antigen is a protein that is often concentrated in the periphery of the cytoplasm in disc-shaped structures. Patients with antibodies often developed signs of PCD near the time of detection of the tumor and had relentless progression of neurological disease. Patients without antibodies frequently had cancer for months to years before PCD developed, and often had spontaneous stabilization of neurological disease with time. Four patients without and 3 patients with antibodies underwent plasmapheresis without response.

Aged↗

The natural history of lumbosacral plexopathy in cancer.

We studied 85 cancer patients with lumbosacral plexopathy and documented pelvic tumor by CT or biopsy. Three clinical syndromes were delineated: lower (L4-S1), 51%; upper (L1-L4), 31%; and pan-plexopathy (L1-S3), 18%. Seventy percent of patients had the insidious onset of pelvic or radicular leg pain, followed weeks to months later by sensory symptoms and weakness. The quintet of leg pain, weakness, edema, rectal mass, and hydronephrosis suggests plexopathy due to cancer. CT showed pelvic tumor in 96%. On myelography, epidural extension, usually below the conus medullaris, was seen in 45%. With treatment, only 28% of patients had objective responses on CT and 17% on examination.

Electromyography↗

Assessment of tumor markers in cerebrospinal fluid.

In clinical practice, the examination of cerebrospinal fluid from patients with primary or metastatic brain tumors is commonly limited to cytomorphologic and routine chemistry analysis. The relative lack of sensitivity and specificity of these tests has led to a search for markers that can detect nervous system involvement by neoplasms at an earlier stage and even predict the site of origin of the neoplasms. This article summarizes recent investigators of biochemical tumor markers and cytoplasmic and cell surface markers in cerebrospinal fluid from patients with nervous system tumors.

Antibodies, Monoclonal↗

Computed tomography in the diagnosis of congenital cytomegalic inclusion disease.

We present two cases of congenital CID, one in a 4-month-old male infant and the other in a 6-week-old male infant. The diagnoses were confirmed by means of computed tomography, cultures, and serologic methods. The relatively noninvasive character of CT makes it an ideal diagnostic method in the neonate. With the improved visualization afforded by CT, specificity on radiographic grounds alone may become possible.

Calcinosis↗

Pokeweed poisoning.

A 68-year-old man who ingested an improperly prepared extract of pokeweed had sweating, confusion, tremor, weakness, incontinence, and syncope. Analysis of the symptoms from this and similar patients previously reported suggests that the pokeweed plant contains an agent that produces peripheral and central cholinergic stimulation.

Aged↗

Subdural hygroma: diagnosis with computed tomography.

Nine cases are reported that fulfilled the criteria for diagnosis of subdural hygroma. With the availability of serial computed tomographic (CT) scans, it is possible to document non-invasively the appearance of CSF-density subdural fluid where no prior blood accumulation has been present.

Adolescent↗

Phase II study of Didemnin B in central nervous system tumors: a Southwest Oncology Group study.

Didemnin B 6.3 mg/m2 was administered intravenously to 48 patients with recurrent or progressive central nervous system tumors. One patient of 39 (2.9%, 95% confidence limits 0.1 to 13.5) eligible patients had a confirmed partial response utilizing standard solid tumor criteria which lasted 14 months. Toxicity was significant. Nausea and vomiting and lethargy were the most frequent toxicities, but multiple severe toxicities were seen. Further investigation of Didemnin B at this dose is not warranted in patients with central nervous system malignancies.

Adolescent↗

Cerebrospinal fluid cytomorphology in systemic lupus erythematosus with Anton's syndrome.

Although the neuropathologic findings in patients who have died of systemic lupus erythematosus (SLE) are well documented, the cytomorphologic reactions present in the cerebrospinal fluid (CSF) of living patients with transient neurologic disturbances associated with SLE are poorly understood. Short of brain biopsy, a CSF cytomorphologic examination may provide the best clue to the nature of the cellular inflammatory process in these transient CNS disorders. Cells in the CSF from a patient with SLE who developed transient cortical blindness with denial of visual loss (Anton's syndrome) were subjected to detailed morphologic classification. The findings suggest that tissue destruction and subclinical hemorrhage can be confirmed by careful CSF cytologic examination in patients with these transient neurologic disturbances.

Adolescent↗