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DOI: 10.1055/s-0031-1273232
© Georg Thieme Verlag KG Stuttgart · New York
Zerebraler Morbus Whipple: Welche Diagnostik ist sinnvoll?
Diagnostic Work-Up in Whipple’s Disease with Cerebral InvolvementPublication History
Publication Date:
03 May 2011 (online)

Zusammenfassung
Zum diagnostischen Vorgehen beim Verdacht auf eine zerebrale Beteiligung im Rahmen eines Morbus Whipple gehören bildgebende Verfahren und eine Liquoruntersuchung mit einer PCR auf Tropheryma whipplei. Die Symptome einer zerebralen Manifestation sind oft unspezifisch, weshalb die extrazerebralen Symptome und die diesbezügliche Diagnostik wegweisend sein können. Die vorliegende Kasuistik schildert den Krankheitsverlauf zweier Patienten, bei denen weder endoskopisch noch im Liquor ein Erregernachweis gelang und erst durch eine zerebrale Biopsie die Verdachtsdiagnose gestellt und eine spezifische Therapie eingeleitet werden konnte.
Abstract
The diagnostic work-up in the case of a suspected cerebral involvement of Whipple’s disease involves neuroimaging and analysis of cerebrospinal fluid (CSF) including polymerase chain reaction (PCR) assays for Tropheryma whipplei. As neurological findings may be complex and unspecific, extracerebral symptoms often lead to the suspicion of Whipple’s disease. We report the cases of two patients in whom the suspected diagnosis of Whipple’s disease could not be proved either by endoscopy or by the analysis of CSF. Only by means of a cerebral biopsy was the diagnosis assumed and specific therapy was initiated.
Schlüsselwörter
Morbus Whipple - zerebraler Whipple - Tropheryma whipplei
Keywords
Whipple’s disease - cerebral Whipple - Tropheryma whipplei
Literatur
- 1
Relman D A, Schmidt T M, MacDermott R P et al.
Identification of the uncultured bacillus of Whipple’s disease.
N Engl J Med.
1992;
327
293-301
MissingFormLabel
- 2
Herbay von A, Otto H F, Stolte M et al.
Epidemiology of Whipple’s disease in Germany. Analysis of 110 patients diagnosed in
1965 – 95.
Scand J Gastroenterol.
1997;
32
52-57
MissingFormLabel
- 3
Fenollar F, Puechal X, Raoult D.
Whipple’s disease.
N Engl J Med.
2007;
356
55-66
MissingFormLabel
- 4
Durand D V, Lecomte C, Cathebras P et al.
Whipple disease. Clinical review of 52 cases. The SNFMI Research Group on Whipple
Disease. Societe Nationale Francaise de Medecine Interne.
Medicine.
1997;
76
170-184
MissingFormLabel
- 5
Louis E D, Lynch T, Kaufmann P et al.
Diagnostic guidelines in central nervous system Whipple’s disease.
Ann Neurol.
1996;
40
561-568
MissingFormLabel
- 6
Panegyres P K, Edis R, Beaman M et al.
Primary Whipple’s disease of the brain: characterization of the clinical syndrome
and molecular diagnosis.
QJM.
2006;
99
609-623
MissingFormLabel
- 7
Adler C H, Galetta S L.
Oculo-facial-skeletal myorhythmia in Whipple disease: treatment with ceftriaxone.
Ann Intern Med.
1990;
112
467-469
MissingFormLabel
- 8
Hausser-Hauw C, Roullet E, Robert R et al.
Oculo-facio-skeletal myorhythmia as a cerebral complication of systemic Whipple’s
disease.
Mov Disord.
1988;
3
179-184
MissingFormLabel
- 9
Schwartz M A, Selhorst J B, Ochs A L et al.
Oculomasticatory myorhythmia: a unique movement disorder occurring in Whipple’s disease.
Ann Neurol.
1986;
20
677-683
MissingFormLabel
- 10
Dobbins 3rd W O.
The diagnosis of Whipple’s disease.
N Engl J Med.
1995;
332
390-392
MissingFormLabel
- 11
Raoult D, Birg M L, La Scola B et al.
Cultivation of the bacillus of Whipple’s disease.
N Engl J Med.
2000;
342
620-625
MissingFormLabel
- 12
Muller S A, Vogt P, Altwegg M et al.
Deadly carousel or difficult interpretation of new diagnostic tools for Whipple’s
disease: case report and review of the literature.
Infection.
2005;
33
39-42
MissingFormLabel
- 13
Fenollar F, Raoult D.
Whipple’s disease.
Clin Diagn Lab Immunol.
2001;
8
1-8
MissingFormLabel
- 14
Marth T, Raoult D.
Whipple’s disease.
Lancet.
2003;
361
239-246
MissingFormLabel
- 15
Fenollar F, Fournier P E, Robert C et al.
Use of genome selected repeated sequences increases the sensitivity of PCR detection
of Tropheryma whipplei.
J Clin Microbiol.
2004;
42
401-403
MissingFormLabel
- 16
Ehrbar H U, Bauerfeind P, Dutly F et al.
PCR-positive tests for Tropheryma whippelii in patients without Whipple’s disease.
Lancet.
1999;
353
2214
MissingFormLabel
- 17
La S cola B, Fenollar F, Fournier P E et al.
Description of Tropheryma whipplei gen. nov., sp. nov., the Whipple’s disease bacillus.
Int J Syst Evol Microbiol.
2001;
51
1471-1479
MissingFormLabel
- 18
Maiwald M, Ditton H J, Herbay von A et al.
Reassessment of the phylogenetic position of the bacterium associated with Whipple’s
disease and determination of the 16S-23S ribosomal intergenic spacer sequence.
Int J Syst Bacteriol.
1996;
46
1078-1082
MissingFormLabel
- 19
Black D F, Aksamit A J, Morris J M.
MR imaging of central nervous system whipple disease: a 15-year review.
Am J Neuroradiol.
2010;
31
1493-1497
MissingFormLabel
- 20
Rolain J M, Fenollar F, Raoult D.
False positive PCR detection of Tropheryma whipplei in the saliva of healthy people.
BMC Microbiol.
2007;
7
48
MissingFormLabel
- 21
Fenollar F, Raoult D.
Molecular techniques in Whipple’s disease.
Expert Rev Mol Diagn.
2001;
1
299-309
MissingFormLabel
- 22
Misbah S A, Mapstone N P.
Whipple’s disease revisited.
J Clin Pathol.
2000;
53
750-755
MissingFormLabel
- 23
Schnider P J, Reisinger E C, Gerschlager W et al.
Long-term follow-up in cerebral Whipple’s disease.
Eur J Gastroenterol Hepatol.
1996;
8
899-903
MissingFormLabel
- 24
Keinath R D, Merrell D E, Vlietstra R et al.
Antibiotic treatment and relapse in Whipple’s disease. Long-term follow-up of 88 patients.
Gastroenterology.
1985;
88
1867-1873
MissingFormLabel
- 25 Diener N P, Berlit G D, Elger CE. et al .„Leitlinien für die Diagnostik und Therapie in der Neurologie”. Stuttgart: Thieme; 2008
MissingFormLabel
- 26
Feurle G E, Marth T.
An evaluation of antimicrobial treatment for Whipple’s Disease. Tetracycline versus
trimethoprim-sulfamethoxazole.
Dig Dis Sci.
1994;
39
1642-1648
MissingFormLabel
- 27
Schneider T, Moos V, Loddenkemper C et al.
Whipple’s disease: new aspects of pathogenesis and treatment.
Lancet Infect Dis.
2008;
8
179-190
MissingFormLabel
- 28
Ghigo E, Capo C, Aurouze M et al.
Survival of Tropheryma whipplei, the agent of Whipple’s disease, requires phagosome
acidification.
Infect Immun.
2002;
70
1501-1506
MissingFormLabel
- 29
Davis Jr T D, Mc B J, Borland Jr J L et al.
The effect of antibiotic and steroid therapy in Whipple’s disease.
Gastroenterology.
1963;
44
112-116
MissingFormLabel
- 30
Scharnke W, Dancygier H.
Whipple’s disease – a rare systemic disease. Current status of diagnosis and treatment.
Dtsch Med Wochenschr.
2001;
126
957-962
MissingFormLabel
- 31
Herbay von A, Ditton H J, Schuhmacher F et al.
Whipple’s disease: staging and monitoring by cytology and polymerase chain reaction
analysis of cerebrospinal fluid.
Gastroenterology.
1997;
113
434-441
MissingFormLabel
Dr. Anna Diels
Klinik und Poliklinik für Neurologie, Uniklinik Köln
Kerpener Str. 62
50924 Köln
Email: anna.diels@uk-koeln.de