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How is Whipple’s disease diagnosed?  return to first page of Whipples

Because Whipple’s disease is rare, the doctor may first try to rule out more common conditions with similar symptoms, including
inflammatory rheumatic disease
celiac disease
various neurologic disorders
intra-abdominal lymphoma
Mycobacterium avium complex in people with AIDS

Whipple’s disease is diagnosed through a careful evaluation of symptoms, endoscopy, and biopsy with tissue staining. Electron microscopy and polymerase chain reaction (PCR) testing are used to confirm a diagnosis.



Endoscopy will be used to examine the lining of the small intestine. An endoscope—a thin, flexible, lighted tube with a small camera on the tip—is inserted through the mouth and stomach and into the small intestine. The endoscope transmits images taken inside the small intestine to a video monitor where a health care professional can view them.

A biopsy is performed during endoscopy to collect samples of tissue from the lining of the small intestine for examination using periodic acid-Schiff (PAS) staining. PAS is a magenta-colored stain that can reveal T. whipplei-infected cells from thinly cut tissues when viewed with a light microscope. Because PAS staining is nonspecific, meaning it can also stain cells infected with other types of bacteria and fungi, many doctors choose to confirm results with a second diagnostic test, such as electron microscopy or PCR testing.

Electron microscopy, which has a much greater resolution than light microscopy, can be used to see T. whipplei bacteria inside infected cells in the tissue taken through biopsy. T. whipplei have a unique appearance easily identified by experienced laboratories.

PCR testing can detect and identify extremely low levels of bacterial DNA in tissues and body fluids. The presence of T. whipplei DNA in cerebrospinal fluid is an indication of neurologic Whipple’s disease. PCR testing for Whipple’s disease is relatively new; therefore, results should be supported by PAS staining or electron microscopy.


How is Whipple’s disease treated?

Whipple’s disease is treated with long-term antibiotics that kill T. whipplei bacteria.

Standard therapy for Whipple’s disease involves initial treatment with intravenous (IV) antibiotics for 2 weeks, followed by daily oral antibiotic treatment for 1 to 2 years. IV antibiotics are delivered through a needle inserted into a vein. IV antibiotics used to treat Whipple’s disease include ceftriaxone (Rocephin) and penicillin G (Pfizerpen) plus streptomycin. Trimethoprim/sulfamethoxazole (Septra, Bactrim), a combination oral antibiotic that can enter the cerebrospinal fluid and brain, is commonly used to treat Whipple’s disease.

An alternative treatment for Whipple’s disease is a combination of Doxycycline (Vibramycin) plus the antimalarial drug hydroxychloroquine (Plaquenil) taken for 12 to 18 months. Supporters of this approach recommend that people with neurologic Whipple’s disease also take long-term antibiotics that can enter the cerebrospinal fluid and brain, such as sulfamethoxazole.


What is the likely outcome for people with Whipple’s disease?


After treatment, the likely outcome for most people with Whipple’s disease is good. Most symptoms disappear in about 1 month. Relapse is common, however, highlighting the need to closely watch for a return of symptoms. Endoscopy with small intestinal biopsy followed by PAS staining and electron microscopy or PCR testing should be repeated 1 year after the start of treatment.

People with neurologic Whipple’s disease who relapse tend to have much poorer health outcomes, including serious neurologic symptoms and even death; therefore, some scientists argue that all cases of Whipple’s disease should be considered neurologic. Relapsing neurologic Whipple’s disease is sometimes treated with a combination of antibiotics and weekly injections of interferon gamma (IFNγ)—a substance made by the body that activates the immune system.


Points to Remember

Whipple’s disease is a rare bacterial infection primarily affecting the small intestine. It can also affect the heart, lungs, brain, joints, and eyes.
Bacteria called Tropheryma whipplei (T. whipplei) cause Whipple’s disease.
Scientists are unsure how T. whipplei infects people.
Whipple’s disease is most common in middle-aged Caucasian men.
Classic signs and symptoms of Whipple’s disease include joint pain, chronic diarrhea, weight loss, abdominal pain and bloating, fever, fatigue, and anemia.
Neurologic symptoms of Whipple’s disease can mimic those of almost any other neurologic condition.
Whipple’s disease is diagnosed through a careful evaluation of symptoms, endoscopy, and biopsy with tissue staining. Electron microscopy and polymerase chain reaction (PCR) testing are used to confirm a diagnosis.
Whipple’s disease is treated with long-term antibiotics that kill T. whipplei bacteria.
After treatment, the likely outcome for most people with Whipple’s disease is good.
People with neurologic Whipple’s disease who relapse tend to have much poorer health outcomes, including serious neurologic symptoms and even death.

 Please  continue to neurological  symptoms of Whipples and treatment with IVIg

Peripheral neuropathy in Whipples disease: a case report.

Rusina R, Keller O, Síma R, Zámečník J.

Source

Department of Neurology, Thomayer Teaching Hospital, Prague, Czech Republic.

Abstract

Whipples disease is a chronic multisystem inflammatory disease with predominantly gastrointestinal manifestations due to Tropheryma whipplei infection. Typical neurological abnormalities include dementia, eye movement abnormalities, hypothalamic dysfunction and oculomasticatory myorhythmias. The literature on peripheral neuropathy in Whipples disease is sparse and the involvement of peripheral nerves in Whipples disease has not been documented convincingly so far. We present a case of Whipples disease presenting by axonal peripheral neuropathy without gastrointestinal involvement. The diagnosis was confirmed by a sural nerve biopsy and consequent PCR of the sample. All clinical signs disappeared progressively during the antibiotic therapy. Two years after the T. whipplei infection, the patient developed dopa-sensitive Parkinson's disease, although these two events seem to be unrelated. This case illustrates the value of peripheral nerve biopsy in cases of axonal neuropathy of unexplained origin and extends the clinical spectrum of Whipples disease to a new modality.

 

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