11/227. Whipple's disease: benefit of the fine-needle aspiration cytology from lymph nodes. We report a case of Whipple's disease with predominant neurological symptoms and without any clinical digestive sign. The diagnosis was suspected by fine-needle aspiration from an axillary lymph node and confirmed by the duodenal biopsy and ultrastructural results. Whipple's disease is a rare, chronic, systemic infectious disease. Electron microscopy and PCR on paraffin blocks are very helpful when histologic findings are not discriminant. On cytologic examination, the major differential diagnosis is mycobacterial adenitis. Here we highlight the possibility of diagnosis Whipple's disease on mere cytological material. ( info) |
12/227. dermatomyositis and Whipple's disease. A 14-year-old boy presented with a 3-year history of a skin rash typical of juvenile dermatomyositis, and a 2-month history of mild proximal weakness, myalgia, and weight loss. A quadriceps biopsy showed perifascicular fibre atrophy, focal necrosis and regeneration, immunohistochemical labelling for HLA-1 on the surface of the fibres, and focal C5-9 deposition in capillaries. macrophages with diastase-resistant, PAS-positive cytoplasm were present. Ultrastructural studies showed electron dense and membranous debris. The patient's symptoms responded to intravenous immunoglobulin and oral prednisolone. Four months after discontinuing prednisolone, the patient developed cardiac failure, ventricular tachycardia, and a recurrence of his rash. The 16S ribosomal rna specific for tropheryma whippelii was identified by polymerase chain reaction (PCR) analysis in skeletal and cardiac muscle. The myalgia and skin rash responded to prednisolone and oral co-trimoxazole, and the tachycardia is controlled by oral verapamil. This patient appears to have a novel association of juvenile dermatomyositis and Whipple's disease. ( info) |
| BACKGROUND: whipple disease is a rare condition characterized by migratory polyarthralgias, fever, and chronic diarrhea. A subset of patients with the disease may either initially have or eventually develop symptoms of central nervous system involvement. DESIGN AND methods: The cognitive and behavioral functioning of a patient with central nervous system involvement from whipple disease was studied during a 7-month period. Serial neuropsychological evaluations were used to quantify the nature of his cognitive and behavioral profile. SETTING: neurology department of a university medical center. RESULTS: A variety of cognitive impairments were noted, most prominently in the domains of sustained attention, memory, executive function, and constructional praxis. There were striking behavioral manifestations as well, including disinhibition and confabulation. CONCLUSIONS: The case demonstrates a degree of higher-order central nervous system dysfunction rarely observed and quantified in connection with whipple disease, and with important implications for differential diagnosis of certain neurologic conditions. We also call attention to some of the neuroanatomical correlates of this encephalopathic condition. ( info) |
14/227. 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. ( info) |
15/227. An uncommon cause of recurrent strokes: tropheryma whippelii endocarditis. BACKGROUND: Cardiac involvement in Whipple's disease is not an uncommon phenomenon in autopsies, but its clinical occurrence is often overshadowed by gastrointestinal symptoms. We report a very atypical manifestation of this disorder. SUMMARY OF REPORT: An extraordinary presentation of an extremely long-lasting, culture-negative endocarditis caused by tropheryma whippelii is described, the clinical consequence of which has become apparent in recurrent strokes. CONCLUSIONS: Cardiac involvement of Whipple's disease should always be considered in culture and serologically negative endocarditis. The polymerase chain reaction technique may be a useful tool to confirm a presumed diagnosis of T whippelii endocarditis and consequently to apply an effective treatment regimen. ( info) |
16/227. Cerebral Whipple's disease: clinical and cerebrospinal fluid findings. The case of a patient who had a relapse of cerebral Whipple's disease (WD) one year after discontinuation of a two-years' antibiotic treatment is reported. Neither the clinical course nor the results of magnetic resonance imaging (MRI) and routine examination of the cerebrospinal fluid (CSF) allowed the caring physician to predict the relapse. Retrospective analysis of serial specimens of CSF showed that slight CSF leucocytosis and intrathecal synthesis of IgA might have suggested persistence of infection. The decision to stop antibiotic therapy in cerebral WD is difficult, but evaluation of cell counts and of intrathecal synthesis of IgA may help in the decision. Some patients may need to take treatment indefinitely. ( info) |
17/227. Clinical relapse in Whipple's disease despite maintenance therapy. Whipple's disease is a multisystem disorder that was first reported just over 100 years ago. Only recently, the bacillus responsible for the condition was identified and subsequently cultured. However, differences of opinion remain regarding the best antibiotic regimen and duration of therapy at primary diagnosis and there is also great uncertainty about the management of disease relapse. We report a case of clinical relapse of Whipple's disease in a man who was on a prolonged therapy with trimethoprim-sulfamethoxazole. We describe his management and review the literature on the treatment of this condition, with particular reference to the recurrence of the disease. ( info) |
| CONTEXT: culture of tropheryma whippelii has been established only once, in human fibroblast cell lines from a heart valve inoculum. Molecular-based diagnostic techniques, although highly sensitive, may be less specific. New diagnostic tools involving isolation of bacteria from contaminated intestinal biopsies and immunohistological detection need to be developed. OBJECTIVE: To describe a novel method for detection and culture of T whippelii strains. DESIGN, SETTING, AND SUBJECTS: Laboratory analysis of duodenal biopsy specimens from a patient with typical relapsing whipple disease with intestinal involvement, performed Marseille, france, in March 2000. biopsy specimens were decontaminated with antimicrobial agents and inoculated onto cell cultures. Mouse anti-T whippelii polyclonal antibodies were used to detect T whippelii in fixed specimens taken from the patient before and after relapse, compared with specimens from 10 controls. The genotype of the isolate was determined by amplification and sequencing of 2 dna fragments (ITS and 23S rRNA). MAIN OUTCOME MEASURE: Isolation and genotyping of a new strain(s) of T whippelii from the case patient's biopsy specimens. RESULTS: A strain was grown from the case patient's intestinal specimen that has a genotype different from the first strain isolated. During 2 episodes of whipple disease, T whippelii bacteria were detected by immunochemistry in the patient's duodenal biopsy specimens, but not in controls. CONCLUSIONS: A second strain of T whippelii has been isolated and a protocol for isolation from the intestine has been proven to be efficient. Immunodetection of T whippelii in intestinal biopsy specimens may provide a useful tool for the diagnosis and follow-up of patients with whipple disease. Both techniques need further evaluation and confirmation. ( info) |
19/227. Diffuse lesions in the CNS revealed by MR imaging in a case of whipple disease. SUMMARY: We report a case of whipple disease involving the brain, optic chiasm, posterior fossa, and spinal cord. We evaluate the role of MR imaging for initial evaluation and for long-term follow-up of whipple disease involving the CNS. We also discuss the semiologic characteristics of the lesions. ( info) |
20/227. Whipple's disease. Report of five cases with different clinical features. Whipple's disease (WD) is a rare systemic disease of infectious etiology which involves the small intestine but can virtually affect any organ. We present here five cases (four males and one female) ranging in age from 20 to 59 years. All patients had intestinal involvement associated or not with clinical manifestations linked to this organ. Vegetation in the tricuspid valve was observed in one patient, suggesting endocarditis caused by tropheryma whippelii, with disappearance of the echocardiographic alterations after treatment. In one of the male patients the initial clinical manifestation was serologically negative spondylitis, with no diarrhea occurring at any time during follow-up. Ocular involvement associated with intestinal malabsorption and significant weight loss were observed in one case. In the other two cases, diarrhea was the major clinical manifestation. All patients were diagnosed by histological examination of the jejunal mucosa and, when indicated, of extraintestinal tissues by light and electron microscopy. After antibiotic treatment, full remission of symptoms occurred in all cases. A control examination of the intestinal mucosa performed after twelve months of treatment with sulfamethoxazole-trimethoprim revealed the disappearance of T. whippelii in four patients. The remaining patient was lost to follow-up. ( info) |