Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029089 (ophthalmoplegia)
 3,338 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Whipple's disease presenting as a neurological disease without gastrointestinal symptoms is an unusual occurrence. A 40 year old man suffered hypersomnia, memory loss and progressive ophthalmoplegia for 6 months prior to death. The nature of this disease was not established during life. Extensive granulomatous inflammation affecting the hypothalamus, hippocampus and periaqueductal gray matter of the brain was found to represent Whipple's disease by electron microscopy. Characteristic lesions were also present in spleen, mesenteric lymph nodes, small intestine and myocardium. Bacillary bodies and membranous inclusions similar to those seen in visceral lesions of Whipple's disease were present in macrophages. The findings supported the theory of direct involvement of the central nervous system by bacilli rather than a metabolic origin for the lesions.
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PMID:Whipple's disease of the central nervous system. 6 98

Four patients with Whipple's disease which had responded to antibiotic therapy, later developed neurologic disease identical to that seen in patients with Whipple's disease who died without treatment. Dementia, myoclonus, ataxia, and supranuclear ophthalmoplegia were the main neurologic features. Restarting antibiotics has been followed by stabilization of disease in all four. Two have improved. In three, the previously diagnosed and treated Whipple's disease was not considered as a possible cause of the neurologic disease until the symptoms and signs were far advanced. It is advisable to periodically evaluate all patients with Whipple's disease, even after successful treatment. Signs of neurologic disease should be considered a possible recurrence of Whipple's disease and antibiotics restarted.
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PMID:Neurologic disease in patients with treated Whipple's disease. 6 1

A case of Whipple's disease with central nervous system (CNS) involvement has been retrospectively diagnosed. At the age of 50 and after six years of recurrent bouts of pyrexia, anorexia and loss of weight, a man developed a subacute encephalitis. Two episodes of acute loss of vision were followed by changes in mental status, supranuclear ophthalmoplegia and oculo-facio-cervical myorhythmias which remained present until death, one year later. Combined light- and electron microscopic studies have demonstrated: No. 1 the presence in the CNS of nodules containing large amounts of Sieracki cells; No. 2 the existence of bacteria in various stages of degeneration in macrophages or in the neuropile. A review of CNS complications in Whipple's disease is made.
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PMID:Cerebral manifestations of Whipple's disease. 9 79

Progressive hypersomnia, memory disturbance, and vertical ophthalmoplegia developed in a 63-year-old woman. The diagnosis of Whipple's disease of the central nervous system was suggested by her presentation and results of studies using magnetic resonance imaging. Despite a one-month course of antibiotics, active Whipple's disease, localized to the central nervous system, was found at autopsy.
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PMID:Whipple's disease confined to the central nervous system. 243 22

A fifth case of oculomasticatory myorhythmia associated with cerebral Whipple's disease is reported. This peculiar abnormal movement has never been described in association with cerebral dysfunction other than Whipple's disease. The present case exhibited rhythmic convergence of the eyes and synchronous (1-2 Hz) contractions of the masticatory muscles and of the proximal and distal skeletal muscles. These abnormal movements occurred 13 years after the beginning of the disease. They were persistent and unchanged until the death of the patient 3 months later. No treatment was effective to suppress the involuntary movements (clonazepam, baclofen, antibiotics). Associated neurological signs included global supranuclear ophthalmoplegia, facial weakness, bilateral ptosis, absent gag reflex, and intellectual deterioration.
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PMID:Oculo-facio-skeletal myorhythmia as a cerebral complication of systemic Whipple's disease. 246 61

Progressive dementia, vertical ophthalmoplegia, and prominent hypothalamic dysfunction developed in one patient with documented intestinal Whipple's disease despite ongoing antibiotic therapy with intestinal remission. A clinical diagnosis of central nervous Whipple's disease was made on the basis of the patient's presentation. High-dose parenteral penicillin and chloramphenicol were administered for 4 1/2 weeks. There was no improvement in results of daily mental status examination or neuropsychologic testing. Deterioration was noted in the electroencephalographic findings after therapy. Vigorous antibiotic therapy with agents that cross the blood-brain barrier had no immediate beneficial effect. Irreversible neurologic damage or a slow, delayed response may account for this observation.
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PMID:Encephalopathy complicating Whipple's disease: failure to respond to antibiotics. 616 Jul 97

A 47-year-old white man with dementia, supranuclear ophthalmoplegia, and myoclonic ocular and facial jerks died in 1931. The case report in 1936 by Ford and Walsh diagnosed encephalitis. In 1993, we made a clinical diagnosis of Whipple's disease on the basis of the 1936 publication. We restudied the pathologic material and found, in addition to extensive encephalitis, PAS-positive material in only the eye, brain, spinal cord, and pituitary. Electron microscopy demonstrated free and intracytoplasmic microorganisms in the eye and brain. We review the history of cerebral ocular Whipple's disease and the implications from this case, which occurred before the development of antibiotics.
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PMID:Cerebral ocular Whipple's disease: a 62-year odyssey from death to diagnosis. 753 2

Pure neurologic Whipple's disease (WD) may be suspected by same clinical data (dementia-ophthalmoplegia-myoclonus triad, oculomasticatory myorhythmia) with support of MRI. Diagnosis is confirmed by intestinal and/or brain biopsy. Early recognition is critical in a disease that can lead to irreversible neurologic sequelae and that can potentially be cured. Despite therapy, relapses in patients with WD are common, being neurologic recurrence the most frequent and serious. Antibiotics that do not cross the blood-brain barrier are not adequate initial therapy for WD, because they predispose to neurologic relapse. Patients with WD should be treated for one year with antibiotics that cross the blood-brain barrier (such as parenteral penicillin+streptomycin, followed by oral trimethoprim-sulfamethoxazole). CNS relapse is usually resistant to therapy.
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PMID:[Neurologic manifestations of Whipple disease]. 851 45

A 65-year-old man was suffering from recurrent manic psychosis accompanied by weight loss. He also had a history of pleural effusion, aspecific migratory non-deforming seronegative polyarthritis, sensorineural hearing loss and semicircular canal paresis. Whipple's disease (WD) had been diagnosed at the age of 63 years. On admission to hospital) he had weight loss, diarrhoea in combination with an organic brain syndrome, hemiparesis and ophthalmoplegia, including internuclear ophthalmoplegia (INO). A clinical diagnosis of central nervous system (CNS) WD was made. MRI revealed a thalamus lesion that halved in size during sulfamethoxazole-trimethoprim treatment. The organic brain syndrome and ophthalmoplegia diminished also, as did the cerebrospinal fluid (CSF) IgG level. A review of CNS WD is presented and implications for treatment are discussed.
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PMID:Whipple's disease and the central nervous system. A case report and a review of the literature. 893 Apr 20

We report a new case of Whipple's disease (WD) confined to the central nervous system. The patient presented with ataxia, ophthalmoplegia, hypersomnia, hemiparesis and generalized myorhythmia. The diagnosis was confirmed by identification of specific sequences of the causal agent of WD, the actinobacteria Tropheryma whippelii (TW), by PCR of DNA extracted from peripheral blood. An epidemiological survey of TW in patients with dementia suggests that WD is an uncommon cause of dementia in our population. Molecular methods may allow rapid identification of TW in peripheral fluids, and non-invasive diagnosis of this disorder.
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PMID:Whipple's disease with isolated central nervous system symptomatology diagnosed by molecular identification of Tropheryma whippelii in peripheral blood. 1084 86


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