Cases reported "Whipple Disease"

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1/16. Whipple's disease mimicking progressive supranuclear palsy: the diagnostic value of eye movement recording.

    Treatable causes of parkinsonian syndromes are rare; Whipple's disease is one of them. A patient is described who presented with a parkinsonian syndrome and abnormal vertical gaze. Measurement of eye movements showed marked slowing of upward saccades, moderate slowing of downward saccades, a full range of voluntary vertical eye movements, curved trajectories of oblique saccades, and absence of square wave jerks. These features, atypical of progressive supranuclear palsy, suggested the diagnosis of Whipple's disease, which was subsequently confirmed by polymerase chain reaction analysis of intestinal biopsy material. Precise measurement of the dynamic properties of saccadic eye movements in parkinsonian patients may provide a means of identifying treatable disorders.
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ranking = 1
keywords = supranuclear
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2/16. Whipple's disease with isolated central nervous system symptomatology diagnosed by molecular identification of tropheryma whippelii in peripheral blood.

    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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ranking = 0.025643696299304
keywords = ophthalmoplegia
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3/16. whipple disease with supranuclear ophthalmoplegia diagnosed by polymerase chain reaction of cerebrospinal fluid.

    An elderly man developed acute progressive supranuclear ophthalmoplegia and other central nervous system manifestations that suggested whipple disease. Results of small intestinal biopsy were negative but polymerase chain reaction testing of the cerebrospinal fluid confirmed the diagnosis.
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ranking = 29.401263582234
keywords = supranuclear ophthalmoplegia, supranuclear, ophthalmoplegia
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4/16. Whipple's disease: multiple hospital admissions of a man with diarrhoea, fever and arthralgia.

    Whipple's disease is a rare chronic multi-systemic infectious disorder caused by the Gram-positive bacillus, tropheryma whippelii. infection may involve any organ in the body, and most commonly affects white men in the fourth to sixth decades of life. The most common presenting symptoms are gastrointestinal and include abdominal pain, diarrhoea, anorexia and associated weight loss. However, the variability in presentation is considerable and some patients may present with intermittent low-grade fever, neurological abnormalities (nystagmus, ophthalmoplegia, cranial nerve defects), migratory arthralgia, lymphadenopathy, or involvement of the cardiovascular system. In typical Whipple's disease, the most severe changes are seen in the proximal small intestine and biopsy reveals mucosal and lymph node infiltration with large, foamy histocytes, containing granules that stain positive with periodic acid-Schiff (PAS) reagent and represent intact or partially degraded bacteria. Extended antibiotic treatment (up to 1-year) is indicated. life-long surveillance for recurrence is essential, once primary treatment has been completed. We report the case of a 58-year-old man who developed a rare infection with the actinobacterium, T. whippelii. The patient had suffered intermittent episodes of varying clinical symptoms associated with multiple hospital admissions and clinical diagnoses, spanning a period of 22 years. Historically, arthralgia was the primary manifestation in this patient and also was the chief complaint for which he was first hospitalized 22 years ago. At his most recent admission to our hospital department, his presenting symptoms were persistent fatigue, weight loss, arthralgia and diarrhoea. Thus, it is essential that clinicians retain a high index of suspicion for T. whippelii infection in patients who have a long-term history of arthritis, fever and diarrhoea.
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ranking = 0.025643696299304
keywords = ophthalmoplegia
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5/16. An unusual case of insomnia associated with Whipple encephalopathy: first case reported from greece.

    whipple disease is a relapsing systemic illness caused by tropheryma whippelii. central nervous system involvement occurs in 5%-40% of all patients. Hypothalamic manifestations occur in 31% of Whipple encephalopathy, including polydipsia, hyperphagia, change in libido and insomnia. We report a case of a 48-year-old man with severe insomnia, depression, dementia, dysarthria, myoclonic movements of the limbs and ophthalmoplegia. The diagnosis of Whipple encephalopathy was confirmed by PCR analysis of blood and faeces. He received a full dose of antibiotic treatment. Despite clinical improvement, resolution of the lesions detected in MRI scan of the brain and negative results of the PCR in blood, faeces and cerebrospinal fluid six months later, insomnia persisted and finally subsided after the administration of carbamazepine (600 mg/day). Our case supports the finding that carbamazepine might be useful in the treatment of insomnia associated with Whipple encephalopathy.
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ranking = 0.025643696299304
keywords = ophthalmoplegia
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6/16. Anti-Ma2-associated encephalitis with normal FDG-PET: a case of pseudo-Whipple's disease.

    BACKGROUND: A 39-year-old man presented with a history of several months of progressive personality changes, social withdrawal, bradykinesia, mutism, dysphagia, worsening gait, and difficulty with daily living activities. Examination revealed an atypical parkinsonian appearance with incomplete supranuclear ophthalmoplegia and an unusual oculomotor disorder characterized by both low-amplitude, intermittent opsoclonus, and slow, nystagmoid intrusions. INVESTIGATIONS: Routine laboratory testing, autoimmune and infectious serologies, brain MRI, lumbar puncture, electroencephalogram, whole-body CT scan, paraneoplastic serologies, small bowel biopsy, 18F-fluorodeoxyglucose positron emission tomography CT scan, brain biopsy, and testicular ultrasound. diagnosis: Anti-Ma2 paraneoplastic encephalitis in association with metastatic testicular cancer; initially misdiagnosed as CNS Whipple's disease. MANAGEMENT: Corticosteroids, intravenous immunoglobulins, orchiectomy, muscle relaxants, mycophenolate mofetil, plasmapheresis, and bleomycin, etoposide and platinum chemotherapy.
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ranking = 5.8802527164469
keywords = supranuclear ophthalmoplegia, supranuclear, ophthalmoplegia
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7/16. Whipple's disease confined to the central nervous system.

    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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ranking = 0.025643696299304
keywords = ophthalmoplegia
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8/16. Oculo-facio-skeletal myorhythmia as a cerebral complication of systemic Whipple's disease.

    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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ranking = 5.8802527164469
keywords = supranuclear ophthalmoplegia, supranuclear, ophthalmoplegia
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9/16. Encephalopathy complicating Whipple's disease: failure to respond to antibiotics.

    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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ranking = 0.025643696299304
keywords = ophthalmoplegia
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10/16. Whipple's disease of the central nervous system.

    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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ranking = 0.025643696299304
keywords = ophthalmoplegia
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