[iko (OP)]

Working in a laboratory, I see very few 'real patients'
and follow some cases through clinical reports from my
colleagues and friends.
Then I read med. literature and case reports, of course.
In the last few years Whipple's Disease (WD) has drawn
my attention for various reasons:

- Intriguing clinical presentations.
- technical difficulties in making diagnosis.
- cheap, easy and instantly accessible cure (when caught on time).
- defined as a 'very rare' disease, but underdiagnosed frequently.
- many cases reported from Europe (many in U.K., Germany and Switzerland?).
- first observed one century ago (1907), it is still a sort of 'mystery'.


"Earlier diagnosis leads to earlier treatment and hopefully the prevention of chronic disabling complications and needless mortality from this once uniformly fatal condition."    James DG and Lipman MC, 2002 (see below)



I open the discussion with simple bits and pieces plus whipple-pictures
Hope many of the NKSforum readers will partecipate in this open thread.
 

   
http://www.scienceimage.csiro.au/index.cfm?event=site.image.thumbnail&id=188&viewfile=f&divid=PP
http://www.sanger.ac.uk/Info/Press/gfx/030221_T_whipplei_300.jpg

I overstressed on purpose intriguing reports from medical literature to open the discussion on how to find a cheap and easy way to avoid these treatable ailments going undetected for months and years, with all the human suffering involved.

In a few words, modern PCR technology will eventually allow us -in the near future- to develop special inexpensive microchips to detect by one simple blood test most of the germs that we carry 'on board'.

An infectious disease will be diagnosed in seconds starting from the germ this time, not from patients' medical history, signs and symptoms.

Quite a little revolution in clinical medicine, but today we do already use other 'screening' tests.
Of course we'll need top notch sensitivity and the highest number of bugs searched through a single test, to make it reliable, easy and cheap.
Unfortunately, these days we see some of this magic hightech show used mostly in forensic medicine.

ikodnotes   [^]

      
http://images.the-scientist.com/content/figures/0890-3670-050411-44-1-1.jpg
http://www.five.tv/media/image/11970633.jpg

The Bug
What is it?
Where is it found?
How is it transmitted?
What diseases does it cause?
Who/what is at risk of infection?

click here for free full-text!
http://microbiology.mtsinai.on.ca/bug/TW/tw-bug.shtml 

[Karen W.]

Is whipples disease absolutely without any symptoms Like this post! LOL LOL.. Hugs IKO

[iko (OP)]

Yes Karen,

Whipple's disease is so variegated in terms of symptoms
that goes from nothing to any sign or symptom you can imagine!
This makes it useless to report their list.

 
http://www.whipplesdisease.net/Bacterium/a_T_whippelii.jpg
http://giornale.regione.marche.it/archivio/num0203/speciale/foto12bg.jpg

A visible bacterium which is reluctant to common cultivation

By means of electron microscopy, the bacterium of Whipple´s disease is well characterized as rod-shaped, approximately 2,5 um in length, with a diameter of 0,25 um.

Its most peculiar ultrastructural feature is a trilaminar membrane with a broad electron dense outer layer. ( 1, 2 )
 
 Since the bacterial nature of Whipple´s disease was recognized in 1961, several attempts were performed to culture the associated bacterium. Some of them were initially assumed to have been successful, but their results could never be reproduced by others. ( 3, 4, 5, 6 )

In 2000, a first case of successful cultivation associated with human fibroblast cell lines was reported, which eventually could be reconfirmed. ( 7, 8; 6 ) In 2003, the new knowledge of the genome provided information  for a first in vitro cultivation without host cells ( 9 ). (see: Bacterium/Growth )

more from: http://www.whipplesdisease.net/Bacterium/hauptteil_bacterium.html

[iko (OP)]

The Bug
What is it?
Where is it found?
How is it transmitted?
What diseases does it cause?
Who/what is at risk of infection?

click here for free full-text!
http://microbiology.mtsinai.on.ca/bug/TW/tw-bug.shtml 

1 Century Ago!

http://www.whipplesdisease.net/

   
http://www.whipplesdisease.net/History/Historical_Case/Whipple1907-1.jpg
http://www.whipplesdisease.net/

[Karen W.]

Working in a laboratory, I see very few 'real patients'
and follow some cases through clinical reports from my
collegues and friends.
Then I read med. literature and case reports, of course.
In the last few years WD draw my attention for various
reasons:

- Intriguing clinical presentations.
- technical difficulties in making diagnosis.
- cheap, easy and instantly accessible cure (when caught on time).
- reported as a 'very rare' disease, but underdiagnosed frequently.
- cases found more often in Europe (many in Germany and Switzerland?).


I open the discussion with simple bits and peaces plus whipple-pics
Hope many of the NKSforum readers will partecipate in this open thread.
 

http://www.sanger.ac.uk/Info/Press/gfx/030221_T_whipplei_300.jpg

YAYYYYYYYYYY! thanks Iko it is actually very interesting. So symptoms can range from nothing to tons of stuff! That must make diagnosing very difficult indeed!

[iko (OP)]

5.0 - Questions about Whipples in Medicine
5.1 - What is Whipple's Disease?
Whipple's disease is a multisystem disorder caused by chronic infection with a bacterium, Tropheryma whippelii. Many patients have malabsorption, which means an impairment of the body's ability to absorb certain nutrients. The disease frequently causes weight loss, irregular breakdown of carbohydrates and fats, resistance to insulin. Most patients have dysfunctions of the immune system. When recognized and treated, Whipple's disease can be cured. Untreated, the disease is usually fatal. Read more at the National Digestive Diseases Clearing House or at Whipple´s Disease Online.

Whipple's disease was named after George Hoyt Whipple, who first observed the disease in 1907 at Johns Hopkins University in Baltimore, Maryland. Whipple was a staff member there from 1905 until 1914. He won the 1934 Nobel Prize in physiology and medicine. (See Blaine Whipple's article on this site and George Hoyt Whipple's ancestry in the WhippleGenWeb.)

(This question was answered with the help of Dr. med. Axel von Herbay, Privatdozent für Pathologie, Pathologisches Institut, Universitätsklinikum, Im Neuenheimer Feld 220, 69120 Heidelberg, Germany. Dr. von Herbay can be reached by e-mail at Axel_von_Herbay@med.uni-heidelberg.de, on the Web at www.WhipplesDisease.net, by telephone at +49 6221 562675, or by fax at +49 6221 562675.) 

from: http://www.whipple.org/docs/faqs.html#5.1

http://www.biomedcentral.com/content/figures/1472-6823-6-3-3-l.jpg
full-text article on "Reversible Hypothyroidism...": http://www.biomedcentral.com/content/pdf/1472-6823-6-3.pdf

 

[iko (OP)]

I hope you now have got the 'basics' of Whipple Disease,
I will add many more links later, don't worry.
Everybody is invited to post whipple bits here.
The final story has to be written still.

Then we'll collect a series of abnormal presentations
of the very same disease...looking like something else!

Whipple's disease: a granulomatous masquerader.

James DG, Lipman MC.
Department of Medicine, Royal Free School of Medicine, Rowland Hill Street, London NW3 2PF, UK.

Whipple's disease is a multisystemic infection that affects middle-aged white men. It typically presents with fever, polyarthritis, diarrhea, steatorrhea, and weight loss.
Many other systems can be involved, however, including the central nervous system, heart, lymphatics, lungs, bone marrow, and skin. Recent work has demonstrated the causative organism to be a complex bacteria, Tropheryma whipplei. The diagnosis is established most securely by periodic acid-Schiff staining of foamy monocyte-macrophages in biopsy tissue and body fluids, by electron microscopy, which reveals bacilli within membrane-bound vesicles, and by molecular amplification techniques using polymerase chain reaction of tissues and body fluids. The differential diagnosis includes chronic multisystemic infections and granulomatous disorders, because Whipple's disease is a fascinating blend of both. The condition can resemble sarcoidosis and mycobacterial disease and fungal, protozoal, and bacillary infections. Earlier diagnosis leads to earlier treatment and hopefully the prevention of chronic disabling complications and needless mortality from this once uniformly fatal condition.

Clin Chest Med. 2002 Jun;23(2):513-9, xi-xii.

http://www.2camels.com/images/festival-photos/venice-carnevale6.jpg

[iko (OP)]

Here is one of the most intriguing episodes of Whipple hunting:
the ultra-advanced treatment for a common disorder (Rheumatoid
Arthritis, or Still's disease) makes the real rare disease come OUT!
...and it's a report from Germany.

Exacerbation of Whipple's disease associated with infliximab treatment.

Kneitz C, Suerbaum S, Beer M, Müller J, Jahns R, Tony HP.
Department of Clinical Immunology and Rheumatology, Medizinische Poliklinik, University of Würzburg, Germany. kneitz_C@klinik.uni-wuerzburg.de

A 34-year-old man with chronic inflammatory joint disease and recurrent fever over 6 years was diagnosed as having Still's disease. Treatment with corticosteroids and azathioprine was ineffective. Therefore, infliximab/ methotrexate was started. The patient subsequently developed a wasting disease with rapid weight loss, erythema nodosum, diarrhoea, progressive lymph node enlargement, and a sigmoido-vesical fistula. Histological analysis of several enlarged lymph nodes, the margins of the fistula, and the small bowel established the diagnosis of Whipple's disease (WD). The presence of Tropheryma whipplei (Tw) DNA in the tissues was confirmed by polymerase chain reaction (PCR). Careful re-evaluation of biopsies taken from the ileum and the liver 2 years earlier, which at that time was not judged to be diagnostic for WD, retrospectively showed subtle histological signs of WD and were positive for Tw DNA.
In summary, infliximab treatment seems to increase the risk of exacerbation of WD.
WD should be carefully ruled out prior to application of tumour necrosis factor-alpha (TNF-alpha) blockade.

Scand J Rheumatol. 2005 Mar-Apr;34(2):148-51.

http://www.annals.org/cgi/content/full/126/3/214

http://cmr.asm.org/cgi/reprint/14/3/561

http://www.biomedcentral.com/content/pdf/1472-6823-6-3.pdf

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2003000300009

http://jnnp.bmj.com/cgi/content/abstract/73/3/336

http://stroke.ahajournals.org/cgi/content/abstract/31/8/2002

http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=12701398&ordinalpos=3&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17258677&ordinalpos=2&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

[iko (OP)]

Sorry, I just found a major whipple-website,
"the" thing to start safely from here:
it had been temporarily lost in my notes.
Ready to click and read.
Enjoy

http://www.whipplesdisease.net/

 
http://www.whipplesdisease.net/a_Whipple.jpg
http://gsm.about.com/pictures/photo_us/029/sulf008i.jpg

The treatment:  Sulfamethoxazole; Trimethoprim, SMX-TMP Tablet (Tab 400;80 mg;mg) for 1-2 years.

[iko (OP)]

Before disappearing for a while and going again
through my messy notes about Whipple's disease,
I post another mysterious case for the affectionate
readers of 'fastidious' persistent infections:
here Whipple was dressed up as dermatomyositis,
an 'autoimmune' disorder of uncertain etiology...
...a pediatric case, quite rare indeed.

Actually it's easy: WD&DM give only this 1 citation on PubMed!  []

Dermatomyositis and Whipple's disease.

Helliwell TR, Appleton RE, Mapstone NC, Davidson J, Walsh KP.
Department of Pathology, University of Liverpool, UK.

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.

Neuromuscul Disord. 2000 Jan;10(1):46-51.

http://www.virtualitalia.com/travel/veneto_cold.gif

Even Toxoplasma gondii has been involved in
several cases of autoimmune dermatomyositis.

[Karen W.]

Thanks Iko.. interesting reading indeed...Thank you...

[iko (OP)]

Early diagnosis of Whipple's disease is crucial to
lower the number of useless autoptic diagnoses.
This is one "just on time" case in point:
(USA, 10 years ago!)

Cardiac Whipple Disease: Identification of Whipple Bacillus
by Electron Microscopy in the Myocardium of a Patient before Death.

by Silvestry FE et al.
University of Pennsylvania Medical Center, Philadelphia, USA.
Ann Intern Med. 1997 Feb 1;126(3):214-6.
 

http://www.annals.org/cgi/content/full/126/3/214
Free full-text available!

http://www.ecodibergamo.it/EcoOnLine/Assets/SPETTACOLO/ATTORI_TEATRI_SPETTACOLI/BURATTINI/arlecchino--480x398.jpg

[iko (OP)]

New patient-friendly technologies for Whipple's disease hunting:
fiberoptic endoscopy cannot inspect the entire small intestine,
but only the first part of it, called duodenum, and a bit further...

In some 'occult' cases, whipple bacteria may hide in unreachable
sites of the small intestine.  Now they have to deal with micro-
electronics progress!

Refractory Whipple's disease with anaemia: first lessons from capsule endoscopy.

Fritscher-Ravens A, Swain CP, von Herbay A.Endoscopy Unit, St Mary's Hospital, London, UK.

Whipple's disease is a chronic multisystem disorder caused by infection with the rod-shaped bacterium, Tropheryma whippelii. We report the case of a 65-year-old woman with intestinal Whipple's disease that had been refractory to monotherapy with a number of antibiotics over a 2-year period. The patient then presented with watery diarrhoea, cachexia (body mass index 18 kg/m (2)) and chronic anaemia (haemoglobin 7.6 g/dl). Wireless capsule endoscopy showed that the disease affected the entire small intestine. Focal occult areas of bleeding were observed in different parts of the jejunum. The capsule's transit time through the small intestine was 2 hours 43 minutes. Capsule endoscopy allows novel insights into the pathophysiology of Whipple's disease.

Endoscopy. 2004 Jul;36(7):659-62.

    
http://comm.uea.ac.uk/mind/getimg.asp?id=256&x=800&y=800
http://www.yalemedicalgroup.org/news/images/proctor1.jpg
http://www.uihealthcare.com/news/currents/vol5issue1/images/5crohnsulcer3.jpg

[iko (OP)]

Everything and its opposite, these are the 'typical' extremely vague symptoms
of Whipple disease according to clinical reports in medical literature.
As insomnia is rarely found, even hypersomnia has been recently reported:

Hypersomnia in Whipple disease: case report.

Maia LF, Marta M, Lopes V, Rocha N, Lopes C, Martins-da-Silva A, Monteiro L.
Department of Neurological Disordes and Senses, Hospital Geral de Santo António, Largo Prof. Abel Salazar, 4099-001 Porto, Portugal.

Whipple disease (WD) is a rare systemic infection caused by Tropheryma whippelii. Neurological involvement has been recognised in 40% of patients, either as initial manifestations or during the course of the disease. We report on a 45 years-old man with WD with initial, persistent and irresistible episodes of daytime somnolence. The patient was HLA-DQB1*0602 positive (genetic marker for narcolepsy). WD diagnosis was suspected on clinical and MRI basis and confirmed by histological and immunohistochemical study of duodenal biopsy. Forty months later all clinical features improved, narcoleptic-like episodes disappeared and cerebral MRI and CSF normalised. Longitudinal neurophysiological studies revealed persistent sleep pattern abnormalities with sleep fragmentation, paucity of slow wave and of REM sleep. The disruption of the hypocretin circuitry in the hypothalamic - diencephalic region triggered by the infection was the probable cause of the hypersomnia and narcopleptic symptoms. WD should be added to the list of causes of secondary hypersomnia.

Arq Neuropsiquiatr. 2006 Sep;64(3B):865-8.

[Karen W.]

Could you explain Hypersomnia better please?

[iko (OP)]

Could you explain Hypersomnia better please?

Sorry Karen,

I was sleeping!  []

Hypersomnia should be 'super'sleep, too much sleep and somnolence during daytime.
This patient had a genetic predisposition to narcolepsy too...
Well, narcolepsy is...(wiki-wiki-wiki! [])

Narcolepsy
Narcolepsy is a neurological condition most characterized by Excessive Daytime Sleepiness (EDS). A narcoleptic will most likely experience disturbed nocturnal sleep, confused with insomnia, and disorder of REM or rapid eye movement sleep. It is a type of dyssomnia.

The main characteristic of narcolepsy is overwhelming excessive daytime sleepiness (EDS), even after adequate night time sleep. A person with narcolepsy is likely to become drowsy or to fall asleep, often at inappropriate times and places. Daytime naps may occur with or without warning and may be physically irresistible. These naps can occur several times a day. They are typically refreshing, but only for a few hours. Drowsiness may persist for prolonged periods of time. In addition, night-time sleep may be fragmented with frequent awakenings.

Four other classic symptoms of narcolepsy, which may not occur in all patients, are cataplexy, sleep paralysis, hypnogogic hallucinations, and automatic behavior. Cataplexy is an episodic condition featuring loss of muscle function, ranging from slight weakness (such as limpness at the neck or knees, sagging facial muscles, or inability to speak clearly) to complete body collapse. Episodes may be triggered by sudden emotional reactions such as laughter, anger, surprise, or fear, and may last from a few seconds to several minutes. The person remains conscious throughout the episode. Sleep paralysis is the temporary inability to talk or move when waking up. It may last a few seconds to minutes. This is often frightening but is not dangerous. Hypnagogic hallucinations are vivid, often frightening, dream-like experiences that occur while dozing, falling asleep and/or while awakening. Automatic behavior means that a person continues to function (talking, putting things away, etc.) during sleep episodes, but awakens with no memory of performing such activities. It is estimated that up to 40 percent of people with narcolepsy experience automatic behavior during sleep episodes. Daytime sleepiness, sleep paralysis, and hypnagogic hallucinations also occur in people who do not have narcolepsy, more frequently in people who are suffering from extreme lack of sleep. Cataplexy is generally considered to be unique to narcolepsy.

from wikipedia:   http://en.wikipedia.org/wiki/Narcolepsy


MyGoodness, me hope never get this narcoPEPSI!    []

...actually that seems to be a typical case of whipplesomnia...

[iko (OP)]

...other cases of whipplesomnia:

Insomnia for 5 years.

Lieb K, Maiwald M, Berger M, Voderholzer U.
Department of Psychiatry, University of Freiburg, Germany. klaus_lieb@psyalig.ukl.uni-freiburg.de

...no abstract available on PubMed Database.
you have to trust me: it was Whipple!

Lancet. 1999 Dec 4;354(9194):1966.

http://www.carnivalofvenice.com/MediaGallery/Gallery/Eventi/Carnevale/2005/FEB01/AUT19058.JPG

An unusual case of insomnia associated with Whipple encephalopathy: first case reported from Greece.
Papadopoulou M, Rentzos M, Vagiakis E, Zouvelou V, Potagas C, Bonakis A, Chatzipanagiotou S, Ioannidis A, Nicolaou C, Vassilopoulos D.Department of Neurology, University of Athens Medical School, Eginition Hospital, 74 Vas. Sophias Av., 11528, Athens, Greece. marpapgr@yahoo.co.uk

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.

Neurol Sci. 2005 Jul;26(3):174-7.

http://guide.dada.net/psicoterapia_ericksoniana/myimg/196435_1.jpg

[iko (OP)]

This time Whipple was dressed up as sarcoidosis,
a mysterious infectious-like granulomatous disorder
of uncertain etiology...but Tropheryma whippelii
had been hiding there for years.

Weight loss, joint pain and abdominal lymphadenopathy
as signs of sarcoidosis, but also of Whipple's disease  [Article in Dutch]

Deeren D, Vanderschueren S, Ectors N, Blockmans D, Bobbaers H.
Afd. Interne Geneeskunde, Universitaire Ziekenhuizen Leuven, België. driesdeeren@hotmail.com

Two patients, a woman aged 66 and a man aged 56 years, with an inflammatory syndrome, weight loss, joint pain and abdominal lymphadenopathy received long-term treatment with corticosteroids for alleged sarcoidosis. No long-term remission was induced and the patients were referred for a second opinion. Eventually the diagnosis of Whipple's disease was established 5 years after the appearance of the first symptoms in the case of the female patient and 4 years after in the case of the male patient. Both patients showed a marked clinical improvement after treatment with trimethoprim-sulfamethoxazole.
An atypical presentation of alleged sarcoidosis should suggest the possibility of Whipple's disease, especially in the case of gastrointestinal symptoms and the failure to respond to corticosteroids, and warrants duodenal biopsy. The presence of granulomas with an elevated angiotensin-converting enzyme level is not pathognomonic for sarcoidosis. It is vitally important to distinguish the two disorders, as Whipple's disease is an infectious disorder that requires antibiotic therapy to prevent a fatal outcome.

Ned Tijdschr Geneeskd. 2003 Mar 29;147(13):620-4.

http://www.carnevalevenezia.com/images/carnevale/maschere_piazza_san_marco_venezia.jpg

In this unfortunate case -several years ago- it was simply too late...

Chronic interstitial nephritis in Whipple's disease.

Schlumpf A, Marbet UA, Stöcklin E, Wegmann W, Lämmle B, Mujagic M, Jösch W, Thiel G, Thölen H, Olivieri W, Gudat F, Torhorst J, Zollinger HU, Mihatsch MJ.
Report is given on a 68-year-old man who suffered primarily from progressive weight loss and repeated episodes of fever and arthralgia.
Later, liver dysfunction and renal insufficiency developed. Liver and kidney biopsies disclosed granulomatous hepatitis and nephritis. Because of the morphologic and clinical findings, the diagnosis of Boeck's disease (sarcoidosis) was made. Shortly before death, diarrhea developed.
Autopsy revealed a massive systemic involvement in Whipple's disease proven by light and electron microscopy and immunofluorescence. Tuberculoid and epitheloid cell granulomas and isolated giant cells were found in addition to the biopsy findings in skeleton muscles, the small intestine, lymphnodes and bronchi. At autopsy, the kidney showed chronic interstitial nephritis. The literature of kidney involvement in Whipple's disease is reviewed. This is the first case with granulomatous interstitial nephritis and chronic renal insufficiency in an inadequately treated Whipple's disease.

Klin Wochenschr. 1983 Jan 3;61(1):25-33. 
 

[Karen W.]

 Gosh this disease seems to be masquerading around as if it could be many different things ..I am glad  I am not a doctor! LOL So confusing..

[iko (OP)]

Whipple was masked as meningoencephalitis in a young woman here.
Infectious etiology was suspected and PENICILLIN administered...
without finding any germ!  But of course it worked -empirically- for
a few months.
Then Whipple came back as a severe cardiovascular problem, and
again managed to hide until a brain biopsy was performed.
A simple PCR test would have made it earlier and more safely.

http://www.pimpo.com/carnevale/venezia.jpg

Cerebral Whipple's disease with a stroke-like presentation and cerebrovascular pathology.

Peters G, du Plessis DG, Humphrey PR.
Department of Neurology, Walton Centre for Neurology and Neurosurgery, Liverpool, UK.

Although neurological symptoms are common in Whipple's disease, patients rarely have a purely neurological presentation and involvement restricted to the central nervous system is uncommon. A 39 year old woman presented with a meningoencephalitic illness, which responded to penicillin.
Eleven months later she developed recurrent stroke-like episodes. Patchy enhancing meningeal, cortical, and subcortical lesions thought to be vascular in origin developed within nine days of the onset of symptoms. No evidence was found of a cardiovascular source of emboli, vasculitis, or thrombophilic condition. A brain biopsy showed meningoencephalitic features suspicious of Whipple's disease associated with leptomeningeal arterial fibrosis and thrombosis.
DNA polymerase chain reaction confirmed Tropheryma whippelii in both blood and brain tissue.
The neurological manifestations of cerebral Whipple's disease are varied and very rarely include stroke-like symptoms. The pathogenesis of cerebral infarction in Whipple's disease is not well established but arterial fibrosis and endocarditis complicated by embolisation have been reported. This case emphasises the importance of early brain biopsy in unusual cases of stroke and illustrates the clinical utility of polymerase chain reaction to confirm Whipple's disease.

J Neurol Neurosurg Psychiatry. 2002 Sep;73(3):336-9.