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Biomedical subjects

J C Trautmann

Publications and source records attributed to J C Trautmann.

At least 19 recordsLinked to original sources

Visual prognosis in giant cell arteritis.

PURPOSE: The visual prognosis in giant cell arteritis (GCA) was evaluated over a 5-year period. METHODS: The authors reviewed the records of all patients with a diagnosis of GCA established at the Mayo Clinic over a 5-year period regarding visual status. Follow-up data for these patients were obtained 5 years later. RESULTS: Of the 245 patients studied, 34 (14%) permanently lost vision because of GCA. In 32 of these patients, the visual deficit developed before glucocorticoid therapy for GCA was begun; in the 2 other patients, the visual loss occurred after the diagnosis was made and therapy was started. Visual loss progressed in three patients after initiation of oral glucocorticoids, and in five other patients vision improved. After 5 years, the probability of loss of vision developing after initiating oral glucocorticoid treatment was determined to be 1% (Kaplan-Meier technique), and the probability of additional loss was 13% in patients with GCA who had a visual deficit at the time therapy was begun. CONCLUSION: The development or progression of visual loss was rare after the initiation of glucocorticoid therapy.

Aged↗

Detection of Epstein-Barr virus in lymphoid tissues of patients with infectious mononucleosis by in situ hybridization.

Although the immunological response during infectious mononucleosis (IMN) has been studied in detail, little is known about the spread of Epstein-Barr virus (EBV) in lymphoid organs or the topographical distribution of the infected cells. In this study, EBV was detected in 11 lymph nodes, 4 tonsils, and 1 spleen of 16 patients with IMN. The predominant cell type positive for the EBV genome was identified as small lymphocytes localized chiefly within typical T areas, preferentially in perifollicular and interfollicular regions of the lymph node. A few endothelia of epithelioid venules were also found to be positive. Furthermore, a small number of sinus lining cells of lymph nodes exhibited labelling. Altogether, only a small number of cells, not exceeding 1 per cent of all cells, were infected with EBV. Our results show that only a small number of lymphocytes carry the EBV and that besides B lymphocytes, other cell constituents of lymphatic tissues are infected by EBV during IMN.

Autoradiography↗

Late juvenile-onset Krabbe's disease.

Krabbe's disease is an autosomal recessive leukodystrophy characterized by a lack of galactocerebroside beta-galactosidase activity. In contrast to the classic early infantile-onset form of Krabbe's disease, less recognized, late-onset variants exist. The authors present a case of late juvenile-onset Krabbe's disease, including the associated magnetic resonance imaging (MRI) findings. Most patients with late-onset Krabbe's disease present with visual loss due to optic atrophy. Associated gait abnormalities and parental consanguinity should increase the clinician's suspicion that a child may have late-onset Krabbe's disease. Because of the prolonged survival in late-onset Krabbe's disease, the recent development of bone marrow transplantation for these patient makes diagnosis of this disorder particularly important.

Adolescent↗

Optic nerve sheath meningoceles. Clinical and radiographic features in 13 cases with a review of the literature.

Thirteen patients with dilated intraorbital optic nerve sheaths with an expanded, patulous cerebrospinal fluid (CSF) space were studied with high-resolution computed tomography (CT) or magnetic resonance imaging (MRI). Eleven patients had bilateral findings. Headache or visual complaints, or both, were present in all patients. Signs of optic nerve dysfunction were present in eight patients. Three patients had visual acuity worse than 20/200. Cerebrospinal fluid pressure was mildly elevated in two patients. Three patients underwent a surgical procedure; visual acuity improved in one. The authors propose the term meningocele for this condition and suggest MRI with fat-suppression techniques and off-axis sagittal views as the radiographic procedure of choice.

Adolescent↗

Familial cavernous malformations of the central nervous system and retina.

We studied a family in which 4 persons from three generations had multiple cavernous malformations ("angiomas") of the central nervous system (CNS) and/or retina and found accounts in the literature of sixteen other families with this condition. In these families with familial cavernous malformation of the CNS and retina, 92% of pathologically documented vascular malformations were cavernous; 50% of those subjects affected had multiple CNS and/or retinal vascular malformations and 68% (excluding probands) were symptomatic. Cutaneous vascular lesions were an inconsistant manifestation. Autosomal dominant inheritance with high penetrance was confirmed.

Adolescent↗

Minimal oculomotor nerve paresis secondary to unruptured intracranial aneurysm.

In 12 patients with minimal oculomotor nerve deficits due to unruptured intracranial aneurysm, the nerve-related findings were incomplete and at least one element (ptosis, mydriasis, or extraocular muscle weakness) was spared in every patient. Although symptomatic, the affected cranial nerve III functions were only partially lost. Six patients had ptosis and mydriasis, three had ptosis and diplopia, two had mydriasis and diplopia, and one had mydriasis alone. Eleven patients had accompanying headaches that were remarkably variable and difficult to categorize. Cerebral angiography showed the aneurysms, but computed tomography missed one third of them. The symptomatic aneurysm arose from the internal carotid artery in six patients, and from the distal basilar artery in six. Fragments of a cranial nerve III palsy associated with recent onset and ipsilateral headache suggest an enlarging internal carotid or distal basilar artery aneurysm.

Adult↗

Chronic idiopathic anhidrosis.

We describe the cases of eight patients with chronic idiopathic anhidrosis. These patients were heat intolerant and became hot, flushed, dizzy, dyspneic, and weak but did not sweat when the ambient temperature was high or when they exercised. Four patients had preganglionic sudomotor lesions and in the remaining 4 the lesion appeared to be postganglionic. The patients did not have orthostatic hypotension, other evidence of generalized autonomic failure, or symptomatic somatic neuropathy. One patient regained thermoregulatory sweat function and no patient's condition progressed to generalized autonomic failure. Chronic idiopathic anhidrosis appears to be distinctly different from other autonomic neuropathies that tend to carry much poorer prognoses.

Adult↗

Magnetic resonance imaging for neurotoxicity in long-term survivors of carcinoma.

Neurotoxicity is a potential complication of combined chemotherapy and whole-brain radiotherapy in long-term survivors of carcinoma. Clinical features of this neurotoxicity are similar to those manifested in the leukoencephalopathy of pediatric patients with leukemia who have been treated prophylactically with whole-brain radiotherapy and chemotherapy. Magnetic resonance imaging, because of its ability to distinguish cortical gray matter and white matter and its utility for studying demyelinating diseases, was used in the assessment of five long-term survivors of carcinoma who had clinical evidence of neurotoxicity. On magnetic resonance examinations, all five patients had profound abnormalities in the periventricular white matter. These changes were considerably more pronounced than those seen on computed tomographic scanning. Thus, magnetic resonance imaging may serve as a useful procedure for early detection of neurotoxicity in patients with carcinoma who have received cerebral radiotherapy and chemotherapy.

Aged↗

Acute panautonomic neuropathy.

Two patients with acute panautonomic neuropathy had severe impairment of sympathetic and parasympathetic function of acute onset. Autonomic tests suggesting a postganglionic lesion and direct evidence of loss of unmyelinated and small myelinated fibers on nerve biopsy were found in Patient 1. There was a selective loss of small myelinated and unmyelinated nerve fibers; C potential was absent in the in vitro compound action potential; dopamine-beta-hydroxylase activity was unmeasurable. No definite abnormalities were found on the nerve biopsy of Patient 2. The acute panautonomic neuropathies appear to comprise a spectrum of somatic and autonomic involvement.

Acute Disease↗

Visual status after transsphenoidal surgery at the Mayo Clinic, 1971-1982.

In 1,003 patients who underwent transsphenoidal surgery for sellar and parasellar lesions, the surgical mortality was 0.7% (seven patients). Only 25 patients experienced visual complications. Pituitary adenomas were found in 851 patients, of whom 714 underwent visual acuity and visual field testing both before and after their transsphenoidal operations. Among this group, visual acuity had been reduced in 115; postoperatively it was restored to normal or improved in 53, remained the same in 60, and became worse in two. Visual fields, which had been abnormal preoperatively in 230 cases, were restored to normal or improved in 168, remained the same in 52, and became worse in ten. There were also five patients whose vision was normal preoperatively but not postoperatively; thus, the total of those whose sight became worse after transsphenoidal surgery was 17. Surgical mortality in the group with adenomas was 0.5% (four of 851 patients). Nine adenomas recurred during follow-up. Of 40 patients with craniopharyngiomas, 21 had abnormal vision preoperatively; postoperatively, nine were improved and two were worse. In 40 cases surgical exploration failed to disclose any abnormality. There were 12 patients with chordomas and 60 with miscellaneous sellar and parasellar tumors.

Hospitals, Proprietary↗

[Laennec. The bicentenary of the birth of the author of "auscultation mèdiate" (author's transl)].

R. T. H. Laennec (17. 2. 1781-13. 8. 1826), the founder of the principles of auscultation, started from poor beginnings to become the leading clinician of France. In the prime of life, holder of two chairs of clinical medicine and physician to prominent persons in public life, Laennec contracted pulmonary tuberculosis, which led to his death at his home in Brittany at the early age of 45 years. Laennec is still remembered by posterity as the inventor of the stethoscope and author of a pioneering textbook on diseases of the heart and lungs, which is still worth reading today.

Auscultation↗

Computer-assisted perimetry in neuro-ophthalmic disease.

A computer-assisted perimeter has become available commercially and has been evaluated and compared with standard kinetic field testing in patients with neuro-ophthalmic disease. Threshold testing by static means has provided evidence of field defects at a stage usually too subtle to record by standard methods. The machine is quite well standardized and controlled and has had only a few technical problems. Newer programs can be added by means of a change in floppy disk, and this capability promises additional avenues of clinical investigation.

Adult↗

Dominantly inherited cystoid macular edema.

Four patients of Greek ancestry had dominantly inherited cystoid macular edema. Characteristics of this syndrome include the following: an early onset and prolonged course of cystoid changes in the macula, followed by atrophy of the macula in later stages. Some patients also show leakage of fluorescein from the optic disc capillaries, subnormal EOG Lp/Dt ratios, elevated rod dark adaptation thresholds, red-green and blue-yellow color deficiencies, normal ERG findings, hyperopia, peripheral pigmentary retinopathy, and vitreous opacities. Dominantly inherited cystoid macular edema is a distinct genetic trait among the dominantly inherited macular dystrophies.

Adult↗

Osteoma: an unusual cause of amaurosis fugax.

A 16-year-old white boy presented with a history of amaurosis fugax associated with an afferent pupillary defect, proptosis, discomfort of the left eye. Tomograms of the skull and of the orbit revealed a lobulated 4-cm ivory osteoma arising from the left ethmoid sinus and extending into the maxillary sinus and left orbit. After surgical extirpation, visual acuity returned to normal and the papilledema resolved after 4 months. This patient is 1 of 37 patients with osteoma of the paranasal sinuses or of the orbit (or of both) who underwent surgical treatment at the Mayo Clinic between the years 1948 and 1977. Twelve of these patients had orbital involvement, and only these patients had ocular signs and symptoms.

Adolescent↗

Combined nuclear and supranuclear defects in ocular motility. A clinicopathologic study.

A 65-year-old man suffered a midbrain infarct after coronary artery bypass surgery. He was left with a severe neuro-ophthalmologic deficit consisting of paralysis of upward and downward vertical gaze, weakness of adduction of the left eye, a dilated fixed left pupil, and partial right Horner's syndrome. He died 31 months after the episode. Postmortem examination disclosed an infarct involving parts of both oculomotor nuclei as well as supranuclear structures thought to be involved in the mediation of vertical eye movements.

Aged↗

Cavernous hemangioma of the retina.

Cavernous hemangioma of the retina is recognized as a distinct clinical entity. Although neurocutaneous findings are sometimes associated with these retinal tumors, in most of the cases reported to date, systemic features have not been described. This report describes two patients with cavernous hemangioma of the retina. One had neurologic symptoms and signs that were highly suggestive of a hemangioma of the brain stem, and the other had major congenital cardiovascular anomalies. During a five-year interval, the second patient had spontaneous incomplete sclerosis of the retinal hemangioma.

Adult↗

Transsphenoidal decompression of the optic nerve and chiasm. Visual results in 62 patients.

A review of recent experience with transsphenoidal for lesion in and about the sella turcica establishes the value of this approach for the management of patients which visual loss. The lesions encountered consisted of pituitary adenoma in 45 cases, craniopharyngioma in 10 cases, and miscellaneous tumours involving the sella in the remaining seven cases. Sixty of the 62 patients in this series had quantitative determination of preoperative and postoperative visual status; after surgery, vision was improved in 81%, unchanged in 11%, and worse in 5%. Two patients (3%) died during the immediate postoperative period before their visual status could be evaluated.

Adenoma, Chromophobe↗